2012 primary renal disease (prd) codes - era-edta reg · 2012 primary renal disease (prd) codes...

2012

Primary Renal Disease (PRD) codes

(version 1.0)

ERA‐EDTA Registry Academic Medical Center Department of Medical Informatics PO Box 22700 1100 DE Amsterdam The Netherlands Telephone +31 20 566 7637 Fax +31 20 691 9840 E‐mail [email protected]

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originally created by The College of American Pathologists. “SNOMED” and “SNOMED CT” are registered

trademarks of the IHTSDO.

Unless an IHTSDO Affiliate License to use SNOMED CT is held, the user of the SNOMED CT content included in this

Mapping Spreadsheet is required to accept the following restrictions:

a) The use is limited to SNOMED CT concept codes, descriptions and expressions contained in the Mapping

Spreadsheet;

b) The use is limited to purposes related to diagnosis, treatment and management of renal disorders;

c) The use does not involve or provide access to SNOMED CT relationships, except indirectly through inclusion of

expressions in the Mapping Spreadsheet;

d) The use does not permit download or use of portions of SNOMED CT that are not covered by this agreement;

e) The use does not involve more than a total of six‐hundred (600) SNOMED CT concepts.

Furthermore, the use has limitations as stated in the Affiliate license as they apply to sub‐licensees

(www.ihtsdo.org/license.pdf).

Disclaimer

This ERA‐EDTA Coding system for Primary Renal Disease and the related web‐based PRD search tool are tools

assisting in recording the diagnosis of a renal disease. They are offered for free to those who want to use it for

research, teaching and for improvement of the quality of care for patients after a diagnosis has been made by the

nephrologist caring for the patient. In publications users are kindly requested to cite the related paper by Venkat‐

Raman G. et al. in Nephrology Dialysis Transplantation 2013.

ERA‐EDTA, the members of its Coding and Definitions Working Group, and its Registry housed at AMC

(Amsterdam, The Netherlands) disclaim any express or implied warranty of fitness for such use and shall not be

liable for any direct, indirect or consequential loss; personal injury; special or punitive damages; loss of profits,

loss of savings and loss of revenue; loss of business, loss of reputation and loss of goodwill; and loss of data

arising from the use of the Coding system and/or the related web‐based PRD search tool.

http://www.ihtsdo.org/license.pdf

I GLOMERULAR DISEASE

1

3749 Glomerulonephritis ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Urine analysis ‐ Imaging

Old ERA‐EDTA PRD code

‐ 10 ‐ Glomerulonephritis; histologically NOT examined

SNOMED CT concept identifier and fully specified name ‐ 36171008 ‐ Glomerulonephritis (disorder)

ICD‐10 code and term

‐ N059 ‐ Unspecified nephritic syndrome, Unspecified

2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0


2

1003 Adult nephrotic syndrome ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Biochemistry

Additional notes/criteria

‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code

‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name

‐ 52254009 ‐ Nephrotic syndrome (disorder) ICD‐10 code and term

‐ N049 ‐ Nephrotic syndrome, unspecified



3

1019 Nephrotic syndrome of childhood ‐ steroid sensitive ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ Defined by response to steroid therapy. Old ERA‐EDTA PRD code


‐ 445119005 ‐ Steroid sensitive nephrotic syndrome of childhood (disorder) ICD‐10 code and term




4

3604 Nephrotic syndrome of childhood ‐ steroid resistant ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/600995 about nephrotic syndrome, type 2; NPHS2 = nephrotic syndrome, steroid‐resistant, autosomal recessive; SRN1

‐ http://omim.org/entry/610725 about nephrotic syndrome, type 3; NPHS3 = nephrotic syndrome, early‐onset, type 3

Criteria for diagnosis [all to be satisfied]



‐ No remission despite a therapeutic trial of corticosteroids. Old ERA‐EDTA PRD code


‐ 800991000000107 ‐ Steroid resistant nephrotic syndrome of childhood (disorder) ICD‐10 code and term




5

3615 Nephrotic syndrome of childhood ‐ no trial of steroids ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ Nephrotic syndrome in childhood. No trial of steroids, no histology, no information to allow a more detailed PRD to be selected.



‐ 445119005 ‐ Steroid sensitive nephrotic syndrome of childhood (disorder) ICD‐10 code and term




6

1026 Congenital nephrotic syndrome (CNS) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/256300 about nephrotic syndrome, type 1; NPHS1 ‐ http://omim.org/entry/602716 about nephrin; NPHS1 ‐ http://omim.org/entry/600995 about nephrotic syndrome, type 2; NPHS2 = nephrotic

syndrome, steroid‐resistant, autosomal recessive; SRN1 ‐ http://omim.org/entry/604766 about podocin; NPHS2 ‐ http://omim.org/entry/610725 about nephrotic syndrome, type 3; NPHS3 = nephrotic

syndrome, early‐onset, type 3 ‐ http://omim.org/entry/256370 about nephrotic syndrome, type 4; NPHS4 ‐ http://omim.org/entry/614199 about nephrotic syndrome, type 5, with or without

ocular abnormalities; NPHS5 ‐ http://omim.org/entry/614196 about nephrotic syndrome, type 6; NPHS6




‐ Defined by response to steroid therapy. ‐ Clinical history: Onset in 1st 3 months after birth.



‐ 48796009 ‐ Congenital nephrotic syndrome (disorder) ICD‐10 code and term




7

1035 Congenital nephrotic syndrome (CNS) ‐ Finnish type ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/256300 about nephrotic syndrome, type 1; NPHS1 ‐ http://omim.org/entry/602716 about nephrin; NPHS1


‐ Clinical history ‐ Biochemistry ‐ Gene test


‐ Mutation in the gene encoding nephrin (NPHS1) on chromosome 19q13.1. ‐ Clinical history: Onset in 1st 3 months after birth.



‐ 197601003 ‐ Finnish congenital nephrotic syndrome (disorder) ICD‐10 code and term




8

1042 Congenital nephrotic syndrome (CNS) ‐ Finnish type ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/256300 ‐ http://omim.org/entry/602716 about nephrin; NPHS1


‐ Histology ‐ Clinical history ‐ Biochemistry ‐ Gene test


‐ Mutation in the gene encoding nephrin (NPHS1) on chromosome 19q13.1. ‐ Clinical history: Onset in 1st 3 months after birth.


‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name

‐ 197601003 ‐ Finnish congenital nephrotic syndrome (disorder) ICD‐10 code and term




9

1057 Congenital nephrotic syndrome (CNS) ‐ diffuse mesangial sclerosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/256370 about nephrotic syndrome, type 4; NPHS4 ‐ http://omim.org/entry/607102 about WT1 gene; WT1




‐ Often associated with mutation in the Wilms tumor suppressor gene (WT1) on chromosome 11p13.

‐ Clinical history: Onset in 1st 3 months after birth. Old ERA‐EDTA PRD code






10

1061 Congenital nephrotic syndrome (CNS) ‐ focal segmental glomerulosclerosis (FSGS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/600995 Criteria for diagnosis [all to be satisfied]



‐ Clinical history: Onset in 1st 3 months after birth. ‐ Often associated with NPHS2 mutations.


‐ 11 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in children SNOMED CT concept identifier and fully specified name

‐ 236384008 ‐ Congenital nephrotic syndrome with focal glomerulosclerosis (disorder) ICD‐10 code and term

‐ N071 ‐ Focal and segmental glomerular lesions



11

1074 Denys‐Drash syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/194080 ‐ http://omim.org/entry/607102 about WT1 gene; WT1


‐ Clinical history ‐ Clinical exam ‐ Gene test


‐ Mutation in the WT1 gene. ‐ Clinical history: Onset in 1st 3 months after birth.


‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name

‐ 236385009 ‐ Drash syndrome (disorder) ICD‐10 code and term

‐ N048 ‐ Nephrotic syndrome, other



12

1088 Congenital nephrotic syndrome (CNS) ‐ congenital infection Criteria for diagnosis [all to be satisfied]



‐ Clinical history: Onset in 1st 3 months after birth. Infection as a cause of congenital nephrotic syndrome is rare, especially in Europe.

‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease, both of which are coded elsewhere.







13

1090 Minimal change nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Biochemistry Additional notes/criteria

‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code


‐ 44785005 ‐ Minimal change disease (disorder) ICD‐10 code and term

‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality



14

1100 Minimal change nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied] ‐ Histology ‐ Biochemistry Additional notes/criteria ‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code ‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name ‐ 44785005 ‐ Minimal change disease (disorder) ICD‐10 code and term ‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality



15

1116 IgA nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/161950 ‐ http://omim.org/entry/613944


‐ Clinical history Additional notes/criteria

‐ In the absence of a renal histopathology, consider alternative PRDs (e.g. postinfection glomerulonephritis) before choosing this code.

‐ Note that Henoch‐Schönlein nephritis has a separate PRD. Old ERA‐EDTA PRD code


‐ 236407003 ‐ IgA nephropathy (disorder) ICD‐10 code and term

‐ N028 ‐ Recurrent and persistent haematuria, other



16

1128 IgA nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Histology Additional notes/criteria

‐ IgA must be demonstrated by renal histopathology. ‐ IgA in skin histopathology or raised serum IgA concentration are not sufficient for this

PRD. ‐ Note that Henoch‐Schönlein nephritis is coded separately.


‐ 12 ‐ IgA nephropathy (proven by immunofluorescence, not code 76 or 85) SNOMED CT concept identifier and fully specified name

‐ 236407003 ‐ IgA nephropathy (disorder) ICD‐10 code and term




17

1137 Familial IgA nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Clinical history ‐ Family history


‐ With histological evidence in a first degree relative and a compatible clinical setting in the patient, this PRD allows nephrologists to record their preferred diagnosis of IgA nephropathy even in the absence of a renal histopathology.



‐ 445404003 ‐ Familial immunoglobulin A nephropathy (disorder) ICD‐10 code and term




18

1144 Familial IgA nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Histology ‐ Family history


‐ IgA must be demonstrated by renal histopathology. ‐ IgA in skin histopathology or raised serum IgA concentration are not sufficient for this

PRD. Old ERA‐EDTA PRD code


‐ 445404003 ‐ Familial immunoglobulin A nephropathy (disorder) ICD‐10 code and term




19

1159 IgA nephropathy secondary to liver cirrhosis ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history Old ERA‐EDTA PRD code


‐ 282364005 ‐ IgA nephropathy associated with liver disease (disorder) ICD‐10 code and term

‐ N028 ‐ Recurrent and persistent haematuria, other AND ‐ K746 ‐ Other and unspecified cirrhosis of liver



20

1163 IgA nephropathy secondary to liver cirrhosis ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical history



‐ 282364005 ‐ IgA nephropathy associated with liver disease (disorder) ICD‐10 code and term

‐ N028 ‐ Recurrent and persistent haematuria, other AND ‐ K746 ‐ Other and unspecified cirrhosis of liver



21

1171 IgM ‐ associated nephropathy Criteria for diagnosis [all to be satisfied]


‐ The presence of IgM should be demonstrated by IF and the presence of deposits compatible with Immunoglobulins in the same region of the glomerulus should be seen on EM.



‐ 236411009 ‐ IgM nephropathy (disorder) ICD‐10 code and term

‐ N053 ‐ Diffuse mesangial proliferative glomerulonephritis



22

1185 Membranous nephropathy ‐ idiopathic Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/604939 about phospholipase A2 receptor 1; PLA2R1 Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Immunology


‐ May be associated with antibodies to the phospholipase A2 receptor. ‐ This PRD should not be used for SLE related nephropathies which are coded

elsewhere. Old ERA‐EDTA PRD code

‐ 14 ‐ Membranous nephropathy SNOMED CT concept identifier and fully specified name

‐ 197590001 ‐ Nephrotic syndrome with membranous glomerulonephritis (disorder) ICD‐10 code and term

‐ N042 ‐ Nephrotic syndrome, diffuse membranous glomerulonephritis



23

1192 Membranous nephropathy ‐ malignancy associated Criteria for diagnosis [all to be satisfied]








24

1205 Membranous nephropathy ‐ drug induced Criteria for diagnosis [all to be satisfied]








25

1214 Membranous nephropathy ‐ infection associated Criteria for diagnosis [all to be satisfied]








26

1222 Mesangiocapillary glomerulonephritis type 1 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Often associated with cryoglobulinaemia. Old ERA‐EDTA PRD code

‐ 15 ‐ Membrano‐proliferative GN; type I (proven by immunofluorescence / electron microscopy, not code 84 or 89)

SNOMED CT concept identifier and fully specified name

‐ 75888001 ‐ Mesangiocapillary glomerulonephritis, type I (disorder) ICD‐10 code and term

‐ N055 ‐ Diffuse mesangiocapillary glomerulonephritis



27

1233 Mesangiocapillary glomerulonephritis type 2 (dense deposit disease) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Histology Old ERA‐EDTA PRD code

‐ 13 ‐ Dense deposit disease; membrano‐proliferative GN; type II (proven by immunofluorescence / electron microscopy)

SNOMED CT concept identifier and fully specified name

‐ 59479006 ‐ Mesangiocapillary glomerulonephritis, type II (disorder) ICD‐10 code and term

‐ N056 ‐ Unspecified nephritic syndrome, dense deposit disease



28

1246 Mesangiocapillary glomerulonephritis type 3 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ 236409000 ‐ Mesangiocapillary glomerulonephritis type III (disorder) ICD‐10 code and term

‐ N055 ‐ Diffuse mesangiocapillary glomerulonephritis



29

1251 Idiopathic rapidly progressive (crescentic) glomerulonephritis Criteria for diagnosis [all to be satisfied]


‐ This PRD should not be used for other specific PRDs e.g. IgA, vasculitis or Goodpasture's syndrome.


‐ 16 ‐ Crescentic (extracapillary) glomerulonephritis (type I, II, III) SNOMED CT concept identifier and fully specified name

‐ 236398000 ‐ Crescentic glomerulonephritis (disorder) ICD‐10 code and term

‐ N057 ‐ Diffuse concentric glomerulonephritis



30

1267 Primary focal segmental glomerulosclerosis (FSGS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6



‐ 17 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in adults SNOMED CT concept identifier and fully specified name

‐ 236403004 ‐ Focal segmental glomerulosclerosis (disorder) ICD‐10 code and term




31

1279 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal recessive ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Family history ‐ Gene test


‐ This PRD can be used if there is a compatible family history with renal histological evidence in a first degree relative but not in the patient.


‐ 50 ‐ Hereditary/Familial nephropathy ‐ type unspecified SNOMED CT concept identifier and fully specified name

‐ 445388002 ‐ Autosomal recessive focal segmental glomerulosclerosis (disorder) ICD‐10 code and term




32

1280 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal recessive ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Histology ‐ Family history ‐ Gene test


‐ This PRD should be used if there is a compatible family history and renal histological evidence in the patient.



‐ 445388002 ‐ Autosomal recessive focal segmental glomerulosclerosis (disorder) ICD‐10 code and term




33

1298 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal dominant ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6 ‐ http://omim.org/entry/604638 about Actinin




‐ This PRD can be used if there is a compatible family history with renal histological evidence in a first degree relative but not in the patient.



‐ 444977005 ‐ Autosomal dominant focal segmental glomerulosclerosis (disorder) ICD‐10 code and term




34

1308 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal dominant ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6 ‐ http://omim.org/entry/604638 about Actinin




‐ This PRD should be used if there is a compatible family history and renal histological evidence in the patient.



‐ 444977005 ‐ Autosomal dominant focal segmental glomerulosclerosis (disorder) ICD‐10 code and term




35

1312 Focal segmental glomerulosclerosis (FSGS) secondary to obesity ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Clinical exam ‐ Biochemistry


‐ Obesity on clinical examination. ‐ This PRD should be used with great caution. ‐ It allows a nephrologist to give an opinion in the absence of the histological proof

which is required to substantiate a diagnosis of FSGS. ‐ An alternative PRD e.g. 'Adult Nephrotic syndrome ‐ no histology' should be

considered. Old ERA‐EDTA PRD code






36

1320 Focal segmental glomerulosclerosis (FSGS) secondary to obesity ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Histology ‐ Clinical exam ‐ Biochemistry


‐ Obesity on clinical examination. Old ERA‐EDTA PRD code






37

1331 Diffuse endocapillary glomerulonephritis Criteria for diagnosis [all to be satisfied]


‐ Typical of post‐infectious glomerulonephritis. Old ERA‐EDTA PRD code


‐ 3704008 ‐ Diffuse endocapillary proliferative glomerulonephritis (disorder) ICD‐10 code and term

‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.



38

1349 Mesangial proliferative glomerulonephritis Criteria for diagnosis [all to be satisfied]


‐ This PRD should not be used for SLE related nephropathies which are coded elsewhere.



‐ 35546006 ‐ Mesangial proliferative glomerulonephritis (disorder) ICD‐10 code and term

‐ N033 ‐ Diffuse mesangial proliferative glomerulonephritis



39

1354 Focal and segmental proliferative glomerulonephritis Criteria for diagnosis [all to be satisfied]



‐ 83866005 ‐ Focal and segmental proliferative glomerulonephritis (disorder) ICD‐10 code and term




40

1365 Glomerulonephritis ‐ secondary to other systemic disease Criteria for diagnosis [all to be satisfied]


‐ Examples of the systemic conditions include malignancy and liver disease but this PRD should not be used if a more accurate one is available.



‐ 36171008 ‐ Glomerulonephritis (disorder) ICD‐10 code and term

‐ N059 ‐ Unspecified nephritic syndrome, unspecified



41

1377 Glomerulonephritis ‐ histologically indeterminate Criteria for diagnosis [all to be satisfied]


‐ Use this PRD if no other seems appropriate even after considering all the evidence and the renal histopathology report.



‐ 36171008 ‐ Glomerulonephritis (disorder) ICD‐10 code and term




42

1383

Systemic vasculitis ‐ ANCA negative ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name

‐ 46956008 ‐ Systemic vasculitis (disorder) ICD‐10 code and term

‐ I776 ‐ Arteritis, unspecified



43

1396 Systemic vasculitis ‐ ANCA positive ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Immunology


‐ Must be clinical evidence to suggest a vasculitis. Old ERA‐EDTA PRD code

‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name

‐ 46956008 ‐ Systemic vasculitis (disorder) ICD‐10 code and term

‐ I776 ‐ Arteritis, unspecified



44

1401 Granulomatosis with polyangiitis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Clinical exam ‐ Immunology


‐ Involvement of upper or lower respiratory tract or the oral cavity in addition to renal involvement.



‐ 195353004 ‐ Wegener's granulomatosis (disorder) ICD‐10 code and term

‐ M313 ‐ Wegener's granulomatosis



45

1417 Granulomatosis with polyangiitis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ In a compatible clinical setting, histological diagnosis from nasal or respiratory tract can support this PRD if renal histology is not available.



‐ 195353004 ‐ Wegener's granulomatosis (disorder) ICD‐10 code and term

‐ M313 ‐ Wegener's granulomatosis



46

1429 Microscopic polyangiitis ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 239928004 ‐ Microscopic polyarteritis nodosa (disorder) ICD‐10 code and term

‐ M300 ‐ Polyarteritis nodosa



47

1438 Churg‐Strauss syndrome ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Immunology


‐ Peripheral blood eosinophilia is often found. Old ERA‐EDTA PRD code


‐ 82275008 ‐ Allergic granulomatosis angiitis (disorder) ICD‐10 code and term

‐ M301 ‐ Polyarteritis with lung involvement [Churg‐Strauss]



48

1440 Churg‐Strauss syndrome ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 82275008 ‐ Allergic granulomatosis angiitis (disorder) ICD‐10 code and term

‐ M301 ‐ Polyarteritis with lung involvement [Churg‐Strauss]



49

1455 Polyarteritis nodosa Criteria for diagnosis [all to be satisfied]

‐ Imaging Additional notes/criteria

‐ ANCA may be positive but specific tests for anti MPO and anti PR3 are negative. ‐ PAN can be secondary to other disorders. ‐ Use this PRD for classical PAN proven with imaging. ‐ Do not use this PRD for any type of microscopic polyangiitis.


‐ 73 ‐ Renal vascular disease due to polyarteritis SNOMED CT concept identifier and fully specified name

‐ 155441006 ‐ Polyarteritis nodosa (disorder) ICD‐10 code and term




50

1464 Anti‐Glomerular basement membrane (GBM) disease / Goodpasture's syndrome ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Immunology Old ERA‐EDTA PRD code

‐ 86 ‐ Goodpasture's Syndrome SNOMED CT concept identifier and fully specified name

‐ 236506009 ‐ Goodpasture's disease (disorder) ICD‐10 code and term

‐ M310 ‐ Hypersensitivity angiitis



51

1472 Anti‐Glomerular basement membrane (GBM) disease / Goodpasture's syndrome ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov



‐ 86 ‐ Goodpasture's Syndrome SNOMED CT concept identifier and fully specified name

‐ 50581000 ‐ Goodpasture's syndrome ICD‐10 code and term

‐ M310 ‐ Hypersensitivity angiitis



52

1486 Systemic lupus erythematosus / nephritis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Clinical history ‐ Immunology


‐ Evidence of renal disease manifested by at least either proteinuria or haematuria. Old ERA‐EDTA PRD code

‐ 84 ‐ Lupus erythematosus SNOMED CT concept identifier and fully specified name

‐ 68815009 ‐ Systemic lupus erythematosus glomerulonephritis syndrome (disorder) ICD‐10 code and term

‐ M321D ‐ Systemic lupus erythematosus with organ or sys involv



53

1493 Systemic lupus erythematosus / nephritis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ 84 ‐ Lupus erythematosus SNOMED CT concept identifier and fully specified name

‐ 68815009 ‐ Systemic lupus erythematosus glomerulonephritis syndrome (disorder) ICD‐10 code and term

‐ M321D ‐ Systemic lupus erythematosus with organ or sys involv



54

1504 Henoch‐Schönlein purpura / nephritis ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Clinical exam


‐ Evidence of renal disease manifested by at least either proteinuria or haematuria. ‐ Must be clinical evidence or history compatible with HSP.


‐ 85 ‐ Henoch‐Schoenlein purpura SNOMED CT concept identifier and fully specified name

‐ 191306005 ‐ Henoch‐Schönlein purpura (disorder) ICD‐10 code and term

‐ D690 ‐ Allergic purpura



55

1515 Henoch‐Schönlein purpura / nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical history ‐ Clinical exam


‐ Must be clinical evidence or history compatible with HSP. Old ERA‐EDTA PRD code

‐ 85 ‐ Henoch‐Schoenlein purpura SNOMED CT concept identifier and fully specified name

‐ 191306005 ‐ Henoch‐Schönlein purpura (disorder) ICD‐10 code and term

‐ D690 ‐ Allergic purpura



56

1527 Renal scleroderma / systemic sclerosis ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ Cutaneous and systemic symptoms with autoantibodies. Old ERA‐EDTA PRD code

‐ 87 ‐ Systemic sclerosis (scleroderma) SNOMED CT concept identifier and fully specified name

‐ 89155008 ‐ Systemic sclerosis ICD‐10 code and term

‐ M349 ‐ Systemic sclerosis, unspecified



57

1536 Renal scleroderma / systemic sclerosis ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ Cutaneous and systemic symptoms with autoantibodies. Old ERA‐EDTA PRD code

‐ 87 ‐ Systemic sclerosis (scleroderma) SNOMED CT concept identifier and fully specified name

‐ 236502006 ‐ Renal involvement in scleroderma (disorder) ICD‐10 code and term

‐ M348 ‐ Other forms of systemic sclerosis



58

1543 Essential mixed cryoglobulinaemia ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Biochemistry Old ERA‐EDTA PRD code

‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name

‐ 239947001 ‐ Essential mixed cryoglobulinemia (disorder) ICD‐10 code and term

‐ D891 ‐ Cryoglobulinaemia



59

1558 Essential mixed cryoglobulinaemia ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Biochemistry



‐ 239947001 ‐ Essential mixed cryoglobulinemia (disorder) ICD‐10 code and term

‐ D891 – Cryoglobulinaemia



60

1562 Cryoglobulinaemia secondary to hepatitis C ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Immunology Old ERA‐EDTA PRD code


‐ 30911005 ‐ Cryoglobulinemia (disorder) ICD‐10 code and term

‐ D891 ‐ Cryoglobulinaemia AND B171 ‐ Acute hepatitis C OR ‐ D891 ‐ Cryoglobulinaemia AND B182 ‐ Chronic hepatitis C



61

1570 Cryoglobulinaemia secondary to hepatitis C ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:






‐ D891 ‐ Cryoglobulinaemia AND B171 ‐ Acute hepatitis C OR ‐ D891 ‐ Cryoglobulinaemia AND B182 ‐ Chronic hepatitis C



62

1589 Cryoglobulinaemia secondary to systemic disease ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Immunology Additional notes/criteria

‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease. Alternative PRDs are available.




‐ D891 ‐ Cryoglobulinaemia




63

1591 Cryoglobulinaemia secondary to systemic disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease. Alternative PRDs are available.




‐ D891 – Cryoglobulinaemia

II TUBULOINTERSTITIAL DISEASE

64

1602 Primary reflux nephropathy ‐ sporadic Criteria for diagnosis [all to be satisfied]


‐ A positive family history excludes this diagnosis. ‐ This PRD should not normally be used if there is a positive family history. In that case

use PRD 'Familial reflux nephropathy'. Old ERA‐EDTA PRD code

‐ 24 ‐ Pyelonephritis due to vesico‐ureteric reflux without obstruction SNOMED CT concept identifier and fully specified name

‐ 197764002 ‐ Non‐obstructive reflux‐associated chronic pyelonephritis (disorder) ICD‐10 code and term

‐ N110 ‐ Nonobstructive reflux‐associated chronic pyelonephritis



65

1618 Familial reflux nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/193000 about VUR1 ‐ http://omim.org/entry/610878 about VUR2 ‐ http://omim.org/entry/613674 about VUR3


‐ Family history ‐ Imaging


‐ A positive family history is required for this PRD. Old ERA‐EDTA PRD code

‐ 24 ‐ Pyelonephritis due to vesico‐ureteric reflux without obstruction SNOMED CT concept identifier and fully specified name

‐ 522551000000101 ‐ Familial non‐obstructive reflux‐associated chronic pyelonephritis (disorder)


‐ N110 ‐ Nonobstructive reflux‐associated chronic pyelonephritis



66

1625 Congenital dysplasia / hypoplasia Criteria for diagnosis [all to be satisfied]

‐ Imaging Old ERA‐EDTA PRD code

‐ 60 ‐ Renal hypoplasia (congenital) ‐ type unspecified SNOMED CT concept identifier and fully specified name

‐ 204949001 ‐ Renal dysplasia (disorder) ICD‐10 code and term

‐ Q614 ‐ Renal dysplasia



67

1639 Multicystic dysplastic kidneys Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/143400 ‐ http://omim.org/entry/602868 about cell division cycle 5, s. pombe, homolog of;

CDC5L Criteria for diagnosis [all to be satisfied]

‐ Imaging ‐ Gene test


‐ Associated with the HNF1B gene. Old ERA‐EDTA PRD code

‐ 40 ‐ Cystic kidney disease ‐ type unspecified SNOMED CT concept identifier and fully specified name

‐ 82525005 ‐ Congenital cystic kidney disease (disorder) ICD‐10 code and term

‐ Q619 ‐ Cystic kidney disease, unspecified



68

1641 Dysplasia due to fetal ACE‐inhibitor exposure Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Clinical history ‐ Imaging



‐ 519331000000100 ‐ Renal dysplasia due to fetus affected by maternal use of angiotensin converting enzyme inhibitor (disorder)


‐ Q614 ‐ Renal dysplasia



69

3627 Renal cysts and diabetes syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Immunology ‐ Imaging ‐ Gene test




‐ 446641003 ‐ Renal cysts and diabetes syndrome (disorder) ICD‐10 code and term

‐ E142 ‐ Unspecified diabetes mellitus with renal complications



70

1656 Glomerulocystic disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Gene test




‐ 253864004 ‐ Familial hypoplastic, glomerulocystic kidney (disorder) ICD‐10 code and term

‐ Q605 ‐ Renal hypoplasia, unspecified



71

1660 Congenital pelvi‐ureteric junction obstruction Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 373584008 ‐ Congenital pelviureteric junction obstruction (disorder) ICD‐10 code and term

‐ Q621 ‐ Atresia and stenosis of ureter



72

1673 Congenital vesico‐ureteric junction obstruction Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/193000 about VUR1 ‐ http://omim.org/entry/610878 about VUR2 ‐ http://omim.org/entry/613674 about VUR3




‐ 373585009 ‐ Congenital ureterovesical obstruction (disorder) ICD‐10 code and term

‐ Q621 ‐ Atresia and stenosis of ureter



73

1687 Posterior urethral valves Criteria for diagnosis [all to be satisfied]



‐ 253900005 ‐ Congenital posterior urethral valves (disorder) ICD‐10 code and term

‐ Q643 ‐ Other atresia and stenosis of urethra and bladder neck



74

1694 Syndrome of agenesis of abdominal muscles ‐ prune belly syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov



‐ Clinical exam ‐ Imaging


‐ 66 ‐ Syndrome of agenesis of abdominal muscles (Prune Belly) SNOMED CT concept identifier and fully specified name

‐ 5187006 ‐ Prune belly syndrome ICD‐10 code and term

‐ Q794 ‐ Prune belly syndrome



75

1706 Congenital neurogenic bladder Criteria for diagnosis [all to be satisfied]


‐ Investigation will usually include urodynamic studies. Old ERA‐EDTA PRD code


‐ 445387007 ‐ Congenital neurogenic urinary bladder (finding) ICD‐10 code and term

‐ N319 ‐ Neuromuscular dysfunction of bladder, unspecified



76

1710 Bladder exstrophy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Clinical exam Old ERA‐EDTA PRD code


‐ 61758007 ‐ Exstrophy of bladder sequence (disorder) ICD‐10 code and term

‐ Q641 ‐ Exstrophy of urinary bladder



77

1723 Megacystis‐megaureter Criteria for diagnosis [all to be satisfied]


‐ Bilateral hydroureteronephrosis; large, smooth, thin‐walled bladder without urethral obstruction, gross reflux.



‐ 253904001 ‐ Megacystis‐megaureter syndrome (disorder) ICD‐10 code and term

‐ Q622 ‐ Congenital megaloureter



78

1734 Oligomeganephronia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ Reduced number of enlarged nephrons. Old ERA‐EDTA PRD code

‐ 61 ‐ Oligomeganephronic hypoplasia SNOMED CT concept identifier and fully specified name

‐ 18417009 ‐ Oligomeganephronic hypoplasia of kidney (disorder) ICD‐10 code and term




79

1747 Renal papillary necrosis ‐ cause unknown Criteria for diagnosis [all to be satisfied]


‐ 99 ‐ Other identified renal disorder SNOMED CT concept identifier and fully specified name

‐ 90241004 ‐ Papillary necrosis (disorder) ICD‐10 code and term

‐ N172 ‐ Acute renal failure with medullary necrosis



80

1752 Acquired obstructive uropathy / nephropathy Criteria for diagnosis [all to be satisfied]


‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name

‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term

‐ N138 ‐ Other obstructive and reflux uropathy OR

‐ N139 ‐ Obstructive and reflux uropathy, unspecified



81

1768 Acquired obstructive nephropathy due to neurogenic bladder Criteria for diagnosis [all to be satisfied]



‐ The diagnosis is made by imaging plus either clinical examination or urodynamic studies.




‐ N319 ‐ Neuromuscular dysfunction of bladder, unspecified



82

1775 Obstructive nephropathy due to prostatic hypertrophy Criteria for diagnosis [all to be satisfied]




‐ N138 ‐ Other obstructive and reflux uropathy AND N40X ‐ Hyperplasia of prostate



83

1781 Obstructive nephropathy due to prostate cancer Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ N138 ‐ Other obstructive and reflux uropathy AND C61X ‐ Malignant neoplasm of prostate



84

1799 Obstructive nephropathy due to bladder cancer Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ N138 ‐ Other obstructive and reflux uropathy AND C679 ‐ Malignant neoplasm of bladder, unspecified



85

1809 Obstructive nephropathy due to other malignancies Criteria for diagnosis [all to be satisfied]




‐ N138 ‐ Other obstructive and reflux uropathy



86

1813 Idiopathic retroperitoneal fibrosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ 197808006 ‐ Idiopathic retroperitoneal fibrosis (disorder) ICD‐10 code and term

‐ N135 ‐ Kinking and stricture of ureter without hydronephrosis



87

1821 Retroperitoneal fibrosis secondary to malignancies Criteria for diagnosis [all to be satisfied]




‐ 236017004 ‐ Malignant retroperitoneal fibrosis (disorder) ICD‐10 code and term




88

3689 Retroperitoneal fibrosis secondary to drugs Criteria for diagnosis [all to be satisfied]




‐ 236015007 ‐ Drug‐induced retroperitoneal fibrosis (disorder) ICD‐10 code and term




89

3670 Retroperitoneal fibrosis secondary to peri‐aortitis Criteria for diagnosis [all to be satisfied]



‐ 49120005 ‐ Retroperitoneal fibrosis (disorder) ICD‐10 code and term




90

1832 Calculus nephropathy / urolithiasis Criteria for diagnosis [all to be satisfied]


‐ 25 ‐ Pyelonephritis due to urolithiasis SNOMED CT concept identifier and fully specified name

‐ 95566004 ‐ Urolithiasis (disorder) ICD‐10 code and term

‐ N209 ‐ Urinary calculus, unspecified



91

1845 Calcium oxalate urolithiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Biochemistry ‐ Imaging



‐ 444717006 ‐ Calcium oxalate urolithiasis (disorder) ICD‐10 code and term




92

1850 Enteric hyperoxaluria Criteria for diagnosis [all to be satisfied]




‐ 37497004 ‐ Enteric hyperoxaluria (disorder) ICD‐10 code and term

‐ E748 ‐ Other specified disorders of carbohydrate metabolism



93

1866 Magnesium ammonium phosphate (struvite) urolithiasis Criteria for diagnosis [all to be satisfied]



‐ Associated with chronic infections with urease producing organisms. Old ERA‐EDTA PRD code

‐ 25 ‐ Pyelonephritis due to urolithiasis SNOMED CT concept identifier and fully specified name

‐ 444690001 ‐ Magnesium ammonium phosphate urolithiasis (disorder) ICD‐10 code and term




94

1878 Uric acid urolithiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:






‐ 267441009 ‐ Uric acid urolithiasis (disorder) ICD‐10 code and term

‐ M100D ‐ Idiopathic gout



95

1884 Tubulointerstitial nephritis ‐ no histology Additional notes/criteria

‐ This PRD can be used in an appropriate clinical setting, if there is convincing evidence of tubular dysfunction, e.g. Fanconi syndrome, tubular proteinuria.


‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name

‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term

‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic



96

1897 Tubulointerstitial nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]








97

1907 Familial interstitial nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/161900 about progression of renal failure Criteria for diagnosis [all to be satisfied]



‐ This PRD may be used if the diagnosis has been confirmed with a renal histology in at least one affected family member.

‐ This PRD should not be used for familial nephropathies which have a more accurate PRD.

‐ A genetic test may help to make the diagnosis but is not mandatory for this PRD. Old ERA‐EDTA PRD code


‐ 28689008 ‐ Interstitial nephritis (disorder) ICD‐10 code and term




98

1911 Familial interstitial nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/161900 about progression of renal failure Criteria for diagnosis [all to be satisfied]



‐ This PRD should not be used for familial nephropathies which have a more accurate PRD.



‐ 28689008 ‐ Interstitial nephritis (disorder) ICD‐10 code and term




99

1924 Tubulointerstitial nephritis associated with autoimmune disease ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ A genetic test may help to make the diagnosis but is not mandatory for this PRD. Old ERA‐EDTA PRD code



‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND M359 ‐ Systemic involvement of connective tissue, unspecified



100

1930 Tubulointerstitial nephritis associated with autoimmune disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical history ‐ Biochemistry




‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND M359 ‐ Systemic involvement of connective tissue, unspecified



101

1948 Tubulointerstitial nephritis with uveitis (TINU) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical exam Additional notes/criteria

‐ To use this PRD, there must be evidence of past or current uveitis. Old ERA‐EDTA PRD code



‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND H209 ‐ Iridocyclitis, unspecified



102

1953 Tubulointerstitial nephritis with uveitis (TINU) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Clinical exam


‐ To use this PRD, there must be evidence of past or current uveitis. Old ERA‐EDTA PRD code



‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND H209 ‐ Iridocyclitis, unspecified



103

1969 Renal sarcoidosis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Any standard method of diagnosing sarcoid is acceptable. Old ERA‐EDTA PRD code


‐ 37061001 ‐ Granulomatous sarcoid nephropathy (disorder) ICD‐10 code and term

‐ D868D ‐ Sarcoidosis of other and combined sites



104

1976 Renal sarcoidosis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ Histology from non renal tissue is acceptable. Old ERA‐EDTA PRD code


‐ 37061001 ‐ Granulomatous sarcoid nephropathy (disorder) ICD‐10 code and term

‐ D868D ‐ Sarcoidosis of other and combined sites



105

1982 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ 94 ‐ Balkan nephropathy SNOMED CT concept identifier and fully specified name

‐ 236514003 ‐ Toxic nephropathy (disorder) ICD‐10 code and term

‐ N144 ‐ Toxic nephropathy, not elsewhere classified



106

1995 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ Compatible renal histopathology evidence is required to use this PRD but alone is insufficient because the appearances are not diagnostic.

‐ A clinical history consistent with exposure to Aristolochia or residence in an area in which this condition is endemic is also necessary.


‐ 94 ‐ Balkan nephropathy SNOMED CT concept identifier and fully specified name

‐ 236514003 ‐ Toxic nephropathy (disorder) ICD‐10 code and term




107

2005 Drug‐induced tubulointerstitial nephritis ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name

‐ 439990003 ‐ Drug‐induced tubulointerstitial nephritis (disorder) ICD‐10 code and term

‐ N142 ‐ Nephropathy induced by unspec drug medicament or biol subs



108

2014 Drug‐induced tubulointerstitial nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 439990003 ‐ Drug‐induced tubulointerstitial nephritis (disorder) ICD‐10 code and term

‐ N142 ‐ Nephropathy induced by unspec drug medicament or biol subs



109

2022 Nephropathy due to analgesic drugs ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ 31 ‐ Nephropathy (interstitial) due to analgesic drugs SNOMED CT concept identifier and fully specified name

‐ 59400006 ‐ Analgesic nephropathy (disorder) ICD‐10 code and term

‐ N140 ‐ Analgesic nephropathy



110

2033 Nephropathy due to analgesic drugs ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical history ‐ Imaging


‐ 31 ‐ Nephropathy (interstitial) due to analgesic drugs SNOMED CT concept identifier and fully specified name

‐ 59400006 ‐ Analgesic nephropathy (disorder) ICD‐10 code and term

‐ N140 ‐ Analgesic nephropathy



111

2046 Nephropathy due to ciclosporin ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ 33 ‐ Nephropathy (interstitial) due to cyclosporin A SNOMED CT concept identifier and fully specified name

‐ 519481000000106 ‐ Nephropathy induced by ciclosporin (disorder) ICD‐10 code and term

‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs



112

2051 Nephropathy due to ciclosporin ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ 33 ‐ Nephropathy (interstitial) due to cyclosporin A SNOMED CT concept identifier and fully specified name

‐ 519481000000106 ‐ Nephropathy induced by ciclosporin (disorder) ICD‐10 code and term




113

2067 Nephropathy due to tacrolimus ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ 519491000000108 ‐ Nephropathy induced by tacrolimus (disorder) ICD‐10 code and term




114

2079 Nephropathy due to tacrolimus ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 519491000000108 ‐ Nephropathy induced by tacrolimus (disorder) ICD‐10 code and term




115

2080 Nephropathy due to aminoglycosides ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ 519501000000102 ‐ Nephropathy induced by aminoglycosides (disorder) ICD‐10 code and term




116

2098 Nephropathy due to aminoglycosides ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 519501000000102 ‐ Nephropathy induced by aminoglycosides (disorder) ICD‐10 code and term




117

2108 Nephropathy due to amphotericin ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ 519511000000100 ‐ Nephropathy induced by amphotericin (disorder) ICD‐10 code and term




118

2112 Nephropathy due to amphotericin ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 519511000000100 ‐ Nephropathy induced by amphotericin (disorder) ICD‐10 code and term




119

2120 Nephropathy due to cisplatin ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ 32 ‐ Nephropathy (interstitial) due to cis‐platinum SNOMED CT concept identifier and fully specified name

‐ 53556002 ‐ Cis‐platinum nephropathy (disorder) ICD‐10 code and term




120

2131 Nephropathy due to cisplatin ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ 32 ‐ Nephropathy (interstitial) due to cis‐platinum SNOMED CT concept identifier and fully specified name

‐ 53556002 ‐ Cis‐platinum nephropathy (disorder) ICD‐10 code and term




121

2149 Nephropathy due to lithium ‐ no histology Criteria for diagnosis [all to be satisfied]



‐ 4390004 ‐ Lithium nephropathy (disorder) ICD‐10 code and term




122

2154 Nephropathy due to lithium ‐ histologically proven Criteria for diagnosis [all to be satisfied]




‐ 4390004 ‐ Lithium nephropathy (disorder) ICD‐10 code and term




123

2165 Lead induced nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ To use this PRD there should be biochemical evidence of chronic lead intoxication (e.g. EDTA chelation) plus hyperuricaemia and hypertension.

‐ A single measurement of blood lead is insufficient. Old ERA‐EDTA PRD code

‐ 34 ‐ Lead induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name

‐ 519521000000106 ‐ Nephropathy induced by lead (disorder) ICD‐10 code and term

‐ N143 ‐ Nephropathy induced by heavy metals



124

2177 Lead induced nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ To use this PRD there should be biochemical evidence of chronic lead intoxication (e.g. EDTA chelation) plus hyperuricaemia and hypertension.

‐ A single measurement of blood lead is insufficient. Old ERA‐EDTA PRD code

‐ 34 ‐ Lead induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name

‐ 519521000000106 ‐ Nephropathy induced by lead (disorder) ICD‐10 code and term

‐ N143 ‐ Nephropathy induced by heavy metals



125

2183 Acute urate nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ 92 ‐ Gout SNOMED CT concept identifier and fully specified name

‐ 236496000 ‐ Acute urate nephropathy (disorder) ICD‐10 code and term

‐ E790 ‐ Hyperuricaem without sign inflamm arthritis+tophaceous dis



126

2196 Acute urate nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ Histology ‐ Clinical history ‐ Biochemistry



‐ 236496000 ‐ Acute urate nephropathy (disorder) ICD‐10 code and term

‐ E790 ‐ Hyperuricaem without sign inflamm arthritis+tophaceous dis



127

3636 Chronic urate nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:






‐ 190829000 ‐ Chronic urate nephropathy (disorder) ICD‐10 code and term




128

2203 Chronic urate nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ Requires the presence of parenchymal kidney damage resulting from urate deposits. ‐ Should be distinguished from Uromodulin‐associated nephropathy (PRD 2827).



‐ 190829000 ‐ Chronic urate nephropathy (disorder) ICD‐10 code and term




129

3662 Hypercalcaemic nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ Use a more accurate PRD if appropriate. Old ERA‐EDTA PRD code

‐ 93 ‐ Nephrocalcinosis and hypercalcaemic nephropathy SNOMED CT concept identifier and fully specified name

‐ 33763006 ‐ Hypercalcemic nephropathy (disorder) ICD‐10 code and term

‐ E835 ‐ Disorders of calcium metabolism



130

2219 Radiation nephritis Criteria for diagnosis [all to be satisfied]


‐ To use this PRD, there should be a history of radiation with the radiation field including the kidneys.



‐ 7725007 ‐ Radiation nephritis (disorder) ICD‐10 code and term

‐ T66X ‐ Unspecified effects of radiation



131

2226 Renal / perinephric abscess Criteria for diagnosis [all to be satisfied]



‐ 3321001 ‐ Renal abscess (disorder) OR

‐ 80640009 ‐ Perirenal abscess (disorder) ICD‐10 code and term

‐ N151 ‐ Renal and perinephric abscess



132

2235 Renal tuberculosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ A diagnosis of past or present tuberculosis of the kidney must have been made. Old ERA‐EDTA PRD code

‐ 91 ‐ Tuberculosis SNOMED CT concept identifier and fully specified name

‐ 44323002 ‐ Tuberculosis of kidney (disorder) ICD‐10 code and term

‐ A181D ‐ Tuberculosis of genitourinary system



133

2242 Leptospirosis Additional notes/criteria

‐ A diagnosis of leptospirosis must have been made. The diagnosis is normally supported by serology, culture or PCR.



‐ 77377001 ‐ Leptospirosis (disorder) ICD‐10 code and term

‐ A279 ‐ Leptospirosis, unspecified



134

2257 Hantavirus nephropathy Criteria for diagnosis [all to be satisfied]



‐ Diagnosis of hantavirus infection should have been confirmed by PCR. Old ERA‐EDTA PRD code


‐ 102455002 ‐ Hemorrhagic nephroso‐nephritis (disorder) ICD‐10 code and term

‐ A985D ‐ Haemorrhagic fever with renal syndrome



135

2261 Xanthogranulomatous pyelonephritis Criteria for diagnosis [all to be satisfied]



‐ 38898003 ‐ Xanthogranulomatous pyelonephritis (disorder) ICD‐10 code and term

‐ N119 ‐ Chronic tubulo‐interstitial nephritis, unspecified



136

2274 Nephropathy related to HIV ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/609423 ‐ http://omim.org/entry/612551 ‐ http://omim.org/entry/607832


‐ This PRD requires evidence of HIV infection. Old ERA‐EDTA PRD code


‐ 90708001 ‐ Kidney disease (disorder) ICD‐10 code and term

‐ N289 ‐ Disorder of kidney and ureter, unspecified AND B24X ‐ Unspecified human immunodefiency virus [HIV] disease



137

2288 Nephropathy related to HIV ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/609423 ‐ http://omim.org/entry/612551 ‐ http://omim.org/entry/607832





‐ N289 ‐ Disorder of kidney and ureter, unspecified AND B24X ‐ Unspecified human immunodefiency virus [HIV] disease



138

2290 Schistosomiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ This PRD requires microbiological evidence of urinary tract infection with Schistosomiasis.

‐ This PRD should not be used for immune complex nephropathy secondary to Schistosomiasis.



‐ 236706006 ‐ Urinary schistosomiasis (disorder) ICD‐10 code and term

‐ B650D ‐ Schistosom due Schis haematobium [urin schistosom]




139

2300 Other specific infection Additional notes/criteria

‐ This PRD requires evidence of infection with the putative organism. ‐ This PRD should only be used if there is evidence of infection directly causing renal

impairment not listed elsewhere. Old ERA‐EDTA PRD code


‐ 40733004 ‐ Infectious disease (disorder) ICD‐10 code and term


III SYSTEMIC DISEASE AFFECTING THE KIDNEY

140

2316 Diabetic nephropathy in type I ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ A diagnosis of type I diabetes mellitus must have been made. ‐ For a diagnosis of diabetic nephropathy without evidence from renal histopathology,

proteinuria must have been documented at some point in the patient's history. ‐ A PRD of diabetic nephropathy is not mandatory in the presence of DM with

proteinuria and alternative diagnoses can be considered. ‐ In the absence of renal histopathology the differential diagnosis will include 'Chronic

kidney disease (CKD) / chronic renal failure (CRF) aetiology uncertain / unknown (with or without histology)', Ischaemic nephropathy, renovascular disease and atheroembolic renal disease.


‐ 80 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type I SNOMED CT concept identifier and fully specified name

‐ 421893009 ‐ Renal disorder associated with type I diabetes mellitus (disorder) ICD‐10 code and term




141

2328 Diabetic nephropathy in type I diabetes ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ A diagnosis of type I diabetes mellitus must have been made. ‐ Histopathological features most compatible with diabetic nephropathy must be

present. Old ERA‐EDTA PRD code

‐ 80 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type I SNOMED CT concept identifier and fully specified name

‐ 421893009 ‐ Renal disorder associated with type I diabetes mellitus (disorder) ICD‐10 code and term




142

2337 Diabetic nephropathy in type II diabetes ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ A diagnosis of type II diabetes mellitus must have been made. ‐ For a diagnosis of diabetic nephropathy, proteinuria must have been documented at

some point in the patient's history. ‐ A PRD of diabetic nephropathy is not mandatory in the presence of DM with

proteinuria and alternative diagnoses can be considered. ‐ In the absence of renal histology the differential diagnosis will include 'Chronic kidney

disease (CKD) / chronic renal failure (CRF) aetiology uncertain / unknown (with or without histology)', ischaemic nephropathy, renovascular disease and atheroembolic renal disease.

‐ Distinguish from: Inherited / genetic diabetes mellitus type II. Old ERA‐EDTA PRD code

‐ 81 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type II SNOMED CT concept identifier and fully specified name

‐ 420279001 ‐ Renal disorder associated with type II diabetes mellitus (disorder) ICD‐10 code and term




143

2344 Diabetic nephropathy in type II diabetes ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ A diagnosis of type II diabetes mellitus must have been made. ‐ Histopathological features most compatible with diabetic nephropathy must be

present. Old ERA‐EDTA PRD code

‐ 81 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type II SNOMED CT concept identifier and fully specified name

‐ 420279001 ‐ Renal disorder associated with type II diabetes mellitus (disorder) ICD‐10 code and term




144

2359 Chronic hypertensive nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/145500 about essential hypertension ‐ http://omim.org/entry/161900 about progression of renal failure


‐ Clinical history ‐ Clinical exam


‐ The patient must have a history of hypertension. ‐ Other PRDs will usually have been considered before accepting this PRD.


‐ 72 ‐ Renal vascular disease due to hypertension SNOMED CT concept identifier and fully specified name

‐ 38481006 ‐ Hypertensive renal disease (disorder) ICD‐10 code and term

‐ I120 ‐ Hypertensive renal disease with renal failure



145

2363 Chronic hypertensive nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/145500 about essential hypertension ‐ http://omim.org/entry/161900 about progression of renal failure


‐ Histology ‐ Clinical history ‐ Clinical exam


‐ 72 ‐ Renal vascular disease due to hypertension SNOMED CT concept identifier and fully specified name

‐ 38481006 ‐ Hypertensive renal disease (disorder) ICD‐10 code and term




146

2371 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Clinical exam ‐ Biochemistry


‐ Lacks evidence of any other PRD responsible for the hypertension and renal failure. Old ERA‐EDTA PRD code

‐ 71 ‐ Renal vascular disease due to malignant hypertension SNOMED CT concept identifier and fully specified name

‐ 65443008 ‐ Malignant hypertensive renal disease (disorder) ICD‐10 code and term




147

2385 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical exam


‐ Lacks evidence of systemic sclerosis. Old ERA‐EDTA PRD code

‐ 71 ‐ Renal vascular disease due to malignant hypertension SNOMED CT concept identifier and fully specified name

‐ 65443008 ‐ Malignant hypertensive renal disease (disorder) ICD‐10 code and term




148

2392 Ageing kidney ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Imaging


‐ If the patient develops stage 4 CKD, consider a PRD of ischaemic nephropathy. Old ERA‐EDTA PRD code


‐ 445108007 ‐ Age related reduction of renal function (finding) ICD‐10 code and term




149

2407 Ischaemic nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]




‐ 75 ‐ Ischaemic renal disease/cholesterol embolism SNOMED CT concept identifier and fully specified name

‐ 519581000000107 ‐ Ischaemic nephropathy (disorder) ICD‐10 code and term

‐ N280 ‐ Ischaemia and infarction of kidney



150

2411 Ischaemic nephropathy / microvascular disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]





‐ 519581000000107 ‐ Ischaemic nephropathy (disorder) ICD‐10 code and term




151

2424 Renal artery stenosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ 302233006 ‐ Renal artery stenosis (disorder) ICD‐10 code and term

‐ I701 ‐ Atherosclerosis of renal artery



152

2430 Atheroembolic renal disease ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:


‐ Clinical history ‐ Clinical exam ‐ Imaging


‐ Eosinophilia or complement consumption are suggestive. Old ERA‐EDTA PRD code


‐ 51677000 ‐ Atheroembolism of renal arteries (disorder) ICD‐10 code and term




153

2448 Atheroembolic renal disease ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ 51677000 ‐ Atheroembolism of renal arteries (disorder) ICD‐10 code and term




154

2453 Fibromuscular dysplasia of renal artery Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 2900003 ‐ Hyperplasia of renal artery (disorder) ICD‐10 code and term

‐ I773 ‐ Arterial fibromuscular dysplasia



155

2469 Renal arterial thrombosis / occlusion Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ A source of emboli or a hypercoagulable state are usually identified. Old ERA‐EDTA PRD code


‐ 236488005 ‐ Renal artery occlusion (disorder) ICD‐10 code and term




156

2476 Renal vein thrombosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ If there is an underlying nephrotic syndrome causing the thrombosis, the underlying renal diagnosis should take precedence.



‐ 15842009 ‐ Thrombosis of renal vein (disorder) ICD‐10 code and term

‐ I823 ‐ Embolism and thrombosis of renal vein



157

2482 Cardiorenal syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ Intractable heart failure with severe renal impairment without evidence of parenchymal kidney disease or renal vascular disease.

‐ Corresponds to CardioRenal Syndrome type 2 as described by the Acute Dialysis Quality Initiative group (Ronco C et al J Am Coll Cardiol 2008; 52; 1527‐1539).



‐ 445236007 ‐ Cardiorenal syndrome (disorder) ICD‐10 code and term

‐ I139 ‐ Hypertensive heart and renal disease, unspecified



158

2495 Hepatorenal syndrome Criteria for diagnosis [all to be satisfied]



‐ A renal biopsy is not normally done in this clinical situation. ‐ Renal histology is essentially normal. ‐ Patients with liver disease are predisposed to IgA nephropathy and where this is

found, the alternative PRD 'IgA nephropathy secondary to liver cirrhosis' should be considered.



‐ 51292008 ‐ Hepatorenal syndrome (disorder) ICD‐10 code and term

‐ K767 ‐ Hepatorenal syndrome



159

2509 Renal amyloidosis Criteria for diagnosis [all to be satisfied]


‐ Histological proof of amyloid from another tissue is an adequate substitute for renal histology.


‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name

‐ 48713002 ‐ Amyloid nephropathy (disorder) ICD‐10 code and term

‐ E854 ‐ Organ‐limited amyloidosis



160

2513 AA amyloid secondary to chronic inflammation Criteria for diagnosis [all to be satisfied]


‐ Histological proof of amyloid from another tissue is an adequate substitute for renal histology.



‐ 274945004 ‐ AA amyloidosis (disorder) ICD‐10 code and term

‐ E853 ‐ Secondary systemic amyloidosis



161

2521 AL amyloid secondary to plasma cell dyscrasia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ There should be evidence of a monoclonal light chain (e.g. biochemical or immunological).

‐ Histological proof of amyloid from another tissue is a substitute for renal histology. Old ERA‐EDTA PRD code


‐ 23132008 ‐ AL amyloidosis (disorder) ICD‐10 code and term

‐ E859 ‐ Amyloidosis, unspecified



162

2532 Familial amyloid secondary to protein mutations ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Family history Additional notes/criteria

‐ Amyloid should have been demonstrated histologically in at least one affected family member.



‐ 66451004 ‐ Familial visceral amyloidosis, Ostertag type (disorder) ICD‐10 code and term

‐ E850 ‐ Non‐neuropathic heredofamilial amyloidosis



163

2545 Familial amyloid secondary to protein mutations ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Family history



‐ 66451004 ‐ Familial visceral amyloidosis, Ostertag type (disorder) ICD‐10 code and term




164

2550 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/142680; for background information see: ‐ http://omim.org/entry/608107


‐ Clinical history ‐ Family history ‐ Gene test



‐ 367528006 ‐ Amyloid of familial Mediterranean fever (disorder) ICD‐10 code and term




165

2566 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/142680; for background information see: ‐ http://omim.org/entry/608107


‐ Histology ‐ Clinical history ‐ Family history ‐ Gene test



‐ 367528006 ‐ Amyloid of familial Mediterranean fever (disorder) ICD‐10 code and term




166

2578 Myeloma kidney ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:



‐ A diagnosis of myeloma must have been made with biochemical, haematological or immunological confirmation.


‐ 82 ‐ Myelomatosis / light chain deposit disease SNOMED CT concept identifier and fully specified name

‐ 32278006 ‐ Myeloma kidney (disorder) ICD‐10 code and term

‐ C900D ‐ Multiple myeloma



167

2584 Myeloma cast nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ A diagnosis of myeloma must have been made with biochemical, haematological or immunological confirmation.



‐ 32278006 ‐ Myeloma kidney (disorder) ICD‐10 code and term

‐ C900D ‐ Multiple myeloma



168

2597 Light chain deposition disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ A diagnosis of light chain disease must have been made with biochemical, haematological or immunological confirmation.



‐ 373604002 ‐ Light chain deposition disease (disorder) ICD‐10 code and term

‐ D808 ‐ Other immunodeficiencies with predominantly antibody defects



169

2606 Immunotactoid / fibrillary nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ 73305009 ‐ Fibrillary glomerulonephritis (disorder) ICD‐10 code and term




170

2610 Haemolytic uraemic syndrome (HUS) ‐ diarrhoea associated Criteria for diagnosis [all to be satisfied]


‐ This PRD is usually associated with E. coli 0157 infection. Old ERA‐EDTA PRD code

‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name

‐ 373421000 ‐ Diarrhea‐associated hemolytic uremic syndrome (disorder) ICD‐10 code and term

‐ D593 ‐ Haemolytic‐uraemic syndrome



171

2623 Atypical haemolytic uraemic syndrome (HUS) ‐ diarrhoea negative Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/235400 about AHUS1 ‐ http://omim.org/entry/612922 about AHUS2 ‐ http://omim.org/entry/612923 about AHUS3 ‐ http://omim.org/entry/612924 about AHUS4 ‐ http://omim.org/entry/612925 about AHUS5 ‐ http://omim.org/entry/612926 about AHUS6




‐ If there is evidence of a genetic mutation, or of a family history of haemolytic uraemic syndrome, then the code 'Congenital haemolytic uraemic syndrome' or 'Familial haemolytic uraemic syndrome' should be used.



‐ 373422007 ‐ Diarrhea‐negative hemolytic uremic syndrome (disorder) ICD‐10 code and term




172

2634 Thrombotic thrombocytopenic purpura (TTP) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ ADAMTS13 deficiency or autoantibodies to ADAMTS13 may be demonstrated (not mandatory).



‐ 78129009 ‐ Thrombotic thrombocytopenic purpura (disorder) ICD‐10 code and term




173

2647 Haemolytic uraemic syndrome (HUS) secondary to systemic disease Criteria for diagnosis [all to be satisfied]


‐ This PRD includes HUS secondary to drugs or malignancies. Old ERA‐EDTA PRD code


‐ 111407006 ‐ Hemolytic uremic syndrome (disorder) ICD‐10 code and term




174

2652 Congenital haemolytic uraemic syndrome (HUS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 444976001 ‐ Congenital hemolytic uremic syndrome (disorder) ICD‐10 code and term




175

2668 Familial haemolytic uraemic syndrome (HUS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Family history ‐ Biochemistry ‐ Gene test


‐ This PRD includes complement factor H or I abnormalities. Old ERA‐EDTA PRD code


‐ 373420004 ‐ Upshaw‐Schulman syndrome (disorder) ICD‐10 code and term

‐ M311 ‐ Thrombotic microangiopathy



176

2675 Familial thrombotic thrombocytopenic purpura (TTP) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ ADAMTS13 deficiency (not mandatory). Old ERA‐EDTA PRD code


‐ 373420004 ‐ Upshaw‐Schulman syndrome (disorder) ICD‐10 code and term

‐ M311 ‐ Thrombotic microangiopathy



177

2681 Nephropathy due to pre‐eclampsia / eclampsia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/189800 about PEE1 ‐ http://omim.org/entry/609402 about PEE2 ‐ http://omim.org/entry/609403 about PEE3 ‐ http://omim.org/entry/609404 about PEE4






‐ N289 ‐ Disorder of kidney and ureter, unspecified AND O159 ‐ Eclampsia, unspecified as to time period OR

‐ N289 ‐ Disorder of kidney and ureter, unspecified AND O149 ‐ Pre‐eclampsia, unspecified



178

2699 Sickle cell nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:




‐ 13886001 ‐ Sickle cell nephropathy (disorder) ICD‐10 code and term

‐ D571D ‐ Sickle‐cell anaemia without crisis




179

2702 Sickle cell nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:





‐ 13886001 ‐ Sickle cell nephropathy (disorder) ICD‐10 code and term

‐ D571D ‐ Sickle‐cell anaemia without crisis

IV FAMILIAL / HEREDITARY NEPHROPATHIES

180

2718 Autosomal dominant (AD) polycystic kidney disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/173900 about PKD1 ‐ http://omim.org/entry/613095 about PKD2 ‐ http://omim.org/entry/600666 about PKD3


‐ Family history ‐ Imaging ‐ Gene test


‐ 41 ‐ Polycystic kidneys; adult type (dominant) SNOMED CT concept identifier and fully specified name

‐ 28728008 ‐ Polycystic kidney disease, adult type (disorder) ICD‐10 code and term

‐ Q612 ‐ Polycystic kidney, adult type



181

2725 Autosomal dominant (AD) polycystic kidney disease type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/173900 about PKD1 ‐ http://omim.org/entry/601313 about polycystin 1 ‐ http://omim.org/entry/606702 about PKDH1





‐ 253878003 ‐ Adult type polycystic kidney disease type 1 (disorder) ICD‐10 code and term




182

2739 Autosomal dominant (AD) polycystic kidney disease type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/613095 about PKD2 ‐ http://omim.org/entry/173910 about polycystin 2





‐ 253879006 ‐ Adult type polycystic kidney disease type 2 (disorder) ICD‐10 code and term




183

2741 Autosomal recessive (AR) polycystic kidney disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 42 ‐ Polycystic kidneys; infantile (recessive) SNOMED CT concept identifier and fully specified name

‐ 28770003 ‐ Polycystic kidney disease, infantile type (disorder) ICD‐10 code and term

‐ Q611 ‐ Polycystic kidney, infantile type



184

2756 Alport syndrome ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/301050 about alport syndrome, X‐ linked; ATS ‐ http://omim.org/entry/303630 about collagen, type IV, alpha‐5; COL4A5 ‐ http://omim.org/entry/203780 about alport syndrome, autosomal recessive ‐ http://omim.org/entry/104200 about alport syndrome, autosomal dominant ‐ http://omim.org/entry/308940 about leiomyomatosis, diffuse, with alport syndrome;

DL‐ATS ‐ http://omim.org/entry/300194 about alport syndrome, mental retardation, midface

hypoplasia, and elliptocytosis for background information see: ‐ http://omim.org/entry/609469 about nephropathy, progressive, with deafness


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test


‐ 51 ‐ Hereditary nephritis with nerve deafness (Alport's Syndrome) SNOMED CT concept identifier and fully specified name

‐ 399340005 ‐ Hereditary nephritis (disorder) ICD‐10 code and term

‐ N079 ‐ Unspecified morphological changes



185

2760 Alport syndrome ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/301050 about alport syndrome, X‐ linked; ATS ‐ http://omim.org/entry/303630 about collagen, type IV, alpha‐5; COL4A5 ‐ http://omim.org/entry/203780 about alport syndrome, autosomal recessive ‐ http://omim.org/entry/104200 about alport syndrome, autosomal dominant ‐ http://omim.org/entry/308940 about leiomyomatosis, diffuse, with alport syndrome;

DL‐ATS ‐ http://omim.org/entry/300194 about alport syndrome, mental retardation, midface

hypoplasia, and elliptocytosis for background information see: ‐ http://omim.org/entry/609469 about nephropathy, progressive, with deafness


‐ Histology ‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test


‐ 51 ‐ Hereditary nephritis with nerve deafness (Alport's Syndrome) SNOMED CT concept identifier and fully specified name

‐ 399340005 ‐ Hereditary nephritis (disorder) ICD‐10 code and term




186

2773 Benign familial haematuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Urine analysis


‐ The clinical history can be useful because it does NOT suggest any other nephropathy. ‐ Haematuria can be demonstrated by any means e.g. dip stick or urine microscopy.



‐ 236421001 ‐ Benign familial hematuria (disorder) ICD‐10 code and term

‐ N029 ‐ Recurrent and persistent haematuria, unspecified



187

2787 Thin basement membrane disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 236418003 ‐ Thin basement membrane disease (disorder) ICD‐10 code and term

‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality



188

2794 Cystic kidney disease Criteria for diagnosis [all to be satisfied]


‐ 40 ‐ Cystic kidney disease ‐ type unspecified SNOMED CT concept identifier and fully specified name

‐ 82525005 ‐ Congenital cystic kidney disease (disorder) ICD‐10 code and term

‐ Q619 ‐ Cystic kidney disease, unspecified



189

2804 Medullary cystic kidney disease type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Urine analysis ‐ Imaging ‐ Gene test


‐ Usually autosomal dominant family history, bland urine and without heavy proteinuria.


‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name

‐ 444699000 ‐ Medullary cystic kidney disease type 1 (disorder) ICD‐10 code and term

‐ Q615 ‐ Medullary cystic kidney



190

2815 Medullary cystic kidney disease type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test


‐ Genetically identical to uromodulin‐associated nephropathy, but characterised by presence of cysts plus gout.



‐ 445503007 ‐ Medullary cystic kidney disease type 2 (disorder) ICD‐10 code and term




191

2827 Uromodulin‐associated nephropathy (familial juvenile hyperuricaemic nephropathy) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/162000 about HNFJ1 ‐ http://omim.org/entry/613092 about HNFJ2 ‐ http://omim.org/entry/614227 about HNFJ3 for background information see: ‐ http://omim.org/entry/191845 about uromodulin


‐ Histology ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test


‐ Autosomal dominant inheritance, high incidence of gout, but no cysts. Old ERA‐EDTA PRD code

‐ 49 ‐ Cystic kidney disease ‐ other specified type SNOMED CT concept identifier and fully specified name

‐ 46785007 ‐ Familial juvenile gout (disorder) ICD‐10 code and term

‐ E798 ‐ Other disorders of purine and pyrimidine metabolism



192

2836 Nephronophthisis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/609254 about senior‐loken syndrome 5; SLSN5 and NPHP ‐ http://omim.org/entry/612013 about coiled‐coil and C2 domains‐containing protein

2A; CC2D2A ‐ http://omim.org/entry/613159 about NPHPL1 ‐ http://omim.org/entry/609237 about IQ motif‐containing protein B1; IQCB1 =

nephrocystin 5; NPHP5 ‐ http://omim.org/entry/610142 about centrosomal protein, 290‐KD; CEP290 =

nephrocystin 6; NPHP6 ‐ http://omim.org/entry/611498 about NPHP7 ‐ http://omim.org/entry/610937 about RPGRIP1‐like; RPGRIP1L = nephrocystin 8;

NPHP8 ‐ http://omim.org/entry/613824 about NPHP9 ‐ http://omim.org/entry/613524 about serologically defined colon cancer antigen 8;

SDCCAG8 = NPHP10 gene ‐ http://omim.org/entry/613550 about NPHP11 ‐ http://omim.org/entry/613820 about NPHP12


‐ Clinical history ‐ Family history



‐ 204958008 ‐ Nephronophthisis (disorder) ICD‐10 code and term

‐ Q618 ‐ Other cystic kidney diseases



193

2843 Nephronophthisis ‐ type 1 (juvenile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/256100 about NPHP1 ‐ http://omim.org/entry/607100 about nephrocystin 1; NPHP





‐ 444830001 ‐ Juvenile nephronophthisis (disorder) ICD‐10 code and term




194

2858 Nephronophthisis ‐ type 2 (infantile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/602088 about NPHP2 Criteria for diagnosis [all to be satisfied]




‐ 444558002 ‐ Infantile nephronophthisis (disorder) ICD‐10 code and term




195

2862 Nephronophthisis ‐ type 3 (adolescent type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/604387 about NPHP3 ‐ http://omim.org/entry/608002 about nephrocystin 3; NPHP3





‐ 444749006 ‐ Adolescent nephronophthisis (disorder) ICD‐10 code and term




196

2870 Nephronophthisis ‐ type 4 (juvenile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/606966 about NPHP4 ‐ http://omim.org/entry/607215 about nephrocystin 4; NPHP4





‐ 446989009 ‐ Nephronophthisis type 4 (disorder) ICD‐10 code and term




197

2889 Nephronophthisis ‐ type 5 Criteria for diagnosis [all to be satisfied]








198

2891 Nephronophthisis ‐ type 6 Criteria for diagnosis [all to be satisfied]








199

2901 Primary Fanconi syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/134600 about FRTS1 ‐ http://omim.org/entry/613388 about FRTS2



‐ Clinical syndrome associated with genetic mutations. Old ERA‐EDTA PRD code

‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name

‐ 236466005 ‐ Congenital Fanconi syndrome (disorder) ICD‐10 code and term

‐ E720 ‐ Disorders of amino‐acid transport



200

2917 Tubular disorder as part of inherited metabolic diseases Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/551200 about chronic tubulointerstitial nephropathy Criteria for diagnosis [all to be satisfied]

‐ Family history ‐ Biochemistry



‐ 197744007 ‐ Renal tubulo‐interstitial disorders in metabolic diseases (disorder) ICD‐10 code and term

‐ N163A ‐ Renal tubulo‐interstitial disorders in metabolic diseases



201

2929 Dent disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Imaging ‐ Gene test


‐ X linked recessive nephrolithiasis + mutation in gene CLCN5. ‐ Clinical: low MW proteinuria, raised urinary calcium:creatinine ratio. ‐ May have nephrocalcinosis, nephrolithiasis, hypophosphatemic rickets.



‐ 444645005 ‐ Dent's disease (disorder) ICD‐10 code and term

‐ N398 ‐ Other specified disorders of urinary system



202

2938 Lowe syndrome (oculocerebrorenal syndrome) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test


‐ Genetic mutation in OCR1. ‐ Similar renal phenotype to that seen in Dent disease.



‐ 79385002 ‐ Lowe syndrome (disorder) ICD‐10 code and term




203

2940 Inherited aminoaciduria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/ search for [amino AND aciduria] Criteria for diagnosis [all to be satisfied]




‐ 522601000000103 ‐ Inherited aminoaciduria (disorder) ICD‐10 code and term

‐ E729 ‐ Disorder of amino‐acid metabolism, unspecified



204

2955 Cystinuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/220100 ‐ http://omim.org/entry/606407 about hypotonia‐cystinuria syndrome


‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Imaging ‐ Gene test


‐ Urinary cystine concentration / nephrolithiasis / genetic mutations. Old ERA‐EDTA PRD code


‐ 85020001 ‐ Cystinuria (disorder) ICD‐10 code and term




205

2964 Cystinosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/219800 ‐ http://omim.org/entry/219900 about cystinosis late‐onset juvenile or adolescent

nephropathic type Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Gene test


‐ Systemic deposit cystine / mutation in CTNS gene. Old ERA‐EDTA PRD code

‐ 52 ‐ Cystinosis SNOMED CT concept identifier and fully specified name

‐ 190681003 ‐ Cystinosis (disorder) ICD‐10 code and term




206

2972 Inherited renal glycosuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Family history ‐ Biochemistry ‐ Urine analysis



‐ 226309007 ‐ Familial renal glucosuria (disorder) ICD‐10 code and term




207

2986 Hypophosphataemic rickets X‐linked (XL) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/307800 about hypophosphatemic rickets, X‐linked dominant; XLHR

‐ http://omim.org/entry/300554 about hypophosphatemic rickets, X‐linked recessive Criteria for diagnosis [all to be satisfied]




‐ 82236004 ‐ Familial x‐ linked hypophosphatemic vitamin D refractory rickets (disorder) ICD‐10 code and term

‐ E833 ‐ Disorders of phosphorus metabolism



208

2993 Hypophosphataemic rickets autosomal recessive (AR) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ Imaging shows rickets. Old ERA‐EDTA PRD code


‐ 90505000 ‐ Autosomal recessive hypophosphatemic vitamin D refractory rickets (disorder)


‐ E833 ‐ Disorders of phosphorus metabolism



209

3000 Primary renal tubular acidosis (RTA) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/267200 about renal tubular acidosis III Criteria for diagnosis [all to be satisfied]



‐ 1776003 ‐ Renal tubular acidosis (disorder) ICD‐10 code and term

‐ N258 ‐ Other disorders resulting from impaired renal tubular funct



210

3016 Proximal renal tubular acidosis (RTA) ‐ type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/179830 ‐ http://omim.org/entry/604278 about renal tubular acidosis, proximal, with ocular

abnormalities and mental retardation Criteria for diagnosis [all to be satisfied]



‐ 24790002 ‐ Proximal renal tubular acidosis (disorder) ICD‐10 code and term




211

3028 Distal renal tubular acidosis (RTA) ‐ type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/179800 about renal tubular acidosis, distal, autosomal dominant

‐ http://omim.org/entry/602722 about renal tubular acidosis, distal, autosomal recessive; RTADR

‐ http://omim.org/entry/611590 about renal tubular acidosis, distal, with hemolytic anemia


‐ Biochemistry ‐ Imaging ‐ Gene test


‐ May have nephrocalcinosis. Old ERA‐EDTA PRD code


‐ 236461000 ‐ Distal renal tubular acidosis (disorder) ICD‐10 code and term




212

3037 Distal renal tubular acidosis with sensorineural deafness ‐ gene mutations Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/267300 about renal tubular acidosis, distal, with progressive nerve deafness


‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test


‐ May have nephrocalcinosis. Old ERA‐EDTA PRD code


‐ 236461000 ‐ Distal renal tubular acidosis (disorder) ICD‐10 code and term

‐ N258 ‐ Other disorders resulting from impaired renal tubular funct AND H905 ‐ Sensorineural hearing loss, unspecified



213

3044 Nephrogenic diabetes insipidus Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/304800 about diabetes insipidus, nephrogenic, X‐ linked ‐ http://omim.org/entry/125800 about diabetes insipidus, nephrogenic, autosomal ‐ http://omim.org/entry/221995 about diabetes insipidus, nephrogenic, with mental

retardation and intracerebral calcification Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Gene test



‐ 111395007 ‐ Nephrogenic diabetes insipidus (disorder) ICD‐10 code and term

‐ N251 ‐ Nephrogenic diabetes insipidus



214

3059 Lesch Nyhan syndrome ‐ hypoxanthine guanine phosphoribosyl transferase deficiency Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/300322 ‐ http://omim.org/entry/300323 for background information see: ‐ http://omim.org/entry/308000 ‐ http://omim.org/entry/308950





‐ 10406007 ‐ Lesch‐Nyhan syndrome (disorder) ICD‐10 code and term

‐ E791 ‐ Lesch‐Nyhan syndrome



215

3063 Phosphoribosyl pyrophosphate synthetase (PRPPS) superactivity Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test



‐ 35759001 ‐ Ribose‐phosphate pyrophosphokinase overactivity (disorder) ICD‐10 code and term




216

3071 Alagille syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/118450 about alagille syndrome 1; ALGS1 ‐ http://omim.org/entry/610205 about alagille syndrome 2; ALGS2


‐ Clinical history ‐ Clinical exam ‐ Imaging ‐ Gene test


‐ Renal dysplasia, renal artery stenosis, and cystic kidney disease have been described in this condition.



‐ 31742004 ‐ Arteriohepatic dysplasia (disorder) ICD‐10 code and term

‐ Q447 ‐ Other congenital malformations of liver



217

3085 Bartter syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/601678 about type 1 ‐ http://omim.org/entry/241200 about type 2 ‐ http://omim.org/entry/607364 about type 3 ‐ http://omim.org/entry/602522 about type 4a ‐ http://omim.org/entry/613090 about type 4b


‐ Biochemistry ‐ Gene test



‐ 71275003 ‐ Pseudoprimary aldosteronism (disorder) ICD‐10 code and term

‐ E268 ‐ Other hyperaldosteronism



218

3092 Gitelman syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov





‐ 3188003 ‐ Familial hypokalemia‐hypomagnesemia (disorder) ICD‐10 code and term

‐ E876 ‐ Hypokalaemia



219

3102 Liddle syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov





‐ 71275003 ‐ Pseudoprimary aldosteronism (disorder) ICD‐10 code and term




220

3118 Apparent mineralocorticoid excess Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ 237770005 ‐ Syndrome of apparent mineralocorticoid excess (disorder) ICD‐10 code and term

‐ E278 ‐ Other specified disorders of adrenal gland



221

3125 Glucocorticoid suppressible hyperaldosteronism Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov





‐ 237743003 ‐ Glucocorticoid‐suppressible hyperaldosteronism (disorder) ICD‐10 code and term




222

3139 Inherited / genetic diabetes mellitus type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/125853 about DM II and its rene relationships Criteria for diagnosis [all to be satisfied]

‐ Histology ‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Gene test


‐ Multiple / variable mutations. ‐ Previously called 'Maturity onset diabetes in young people (MODY)'. ‐ MODY types 1‐6 (HNF‐1alpha mutation accounts for 65%).



‐ 237604008 ‐ Diabetes mellitus autosomal dominant type II (disorder) ICD‐10 code and term

‐ E139 ‐ Other specified diabetes mellitus without complications



223

3141 Pseudohypoaldosteronism type 1 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/177735 about Pseudohypoaldosteronism type I, autosomal dominant PHA1A

‐ http://omim.org/entry/264350 about Pseudohypoaldosteronism type I, autosomal recessive PHA1B





‐ 43941006 ‐ Pseudohypoaldosteronism, type 1 (disorder) ICD‐10 code and term

‐ E274 ‐ Other and unspecified adrenocortical insufficiency



224

3156 Pseudohypoaldosteronism type 2 (Gordon syndrome) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov





‐ 15689008 ‐ Pseudohypoaldosteronism, type 2 (disorder) ICD‐10 code and term

‐ E878 ‐ Other disorders of electrolyte and fluid balance NEC



225

3160 Familial hypocalciuric hypercalcaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/145980 about HHC1 ‐ http://omim.org/entry/145981 about HHC2 ‐ http://omim.org/entry/600740 about HHC3





‐ 237885008 ‐ Familial hypocalciuric hypercalcemia (disorder) ICD‐10 code and term




226

3173 Familial hypercalciuric hypocalcaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov






‐ 237885008 ‐ Familial hypocalciuric hypercalcemia (disorder) ICD‐10 code and term




227

3187 Familial hypomagnesaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/602014 about HOMG1 ‐ http://omim.org/entry/154020 about HOMG2 ‐ http://omim.org/entry/248250 about HOMG3 ‐ http://omim.org/entry/611718 about HOMG4 ‐ http://omim.org/entry/248190 about HOMG5 ‐ http://omim.org/entry/613882 about HOMG6




‐ A number of syndromes with different mutations have been identified. Old ERA‐EDTA PRD code


‐ 80710001 ‐ Primary hypomagnesemia (disorder) ICD‐10 code and term

‐ E834 ‐ Disorders of magnesium metabolism



228

3194 Primary hyperoxaluria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/259900 about hyperoxaluria, primary, type I; HP1 ‐ http://omim.org/entry/260000 about hyperoxaluria, primary, type II; HP2 ‐ http://omim.org/entry/613616 about hyperoxaluria, primary, type III; HP3


‐ Histology ‐ Biochemistry ‐ Imaging


‐ 53 ‐ Primary oxalosis SNOMED CT concept identifier and fully specified name

‐ 17901006 ‐ Primary hyperoxaluria (disorder) ICD‐10 code and term




229

3207 Primary hyperoxaluria type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Biochemistry ‐ Imaging ‐ Gene test



‐ 65520001 ‐ Primary hyperoxaluria type I (disorder) ICD‐10 code and term




230

3211 Primary hyperoxaluria type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov





‐ 40951006 ‐ Primary hyperoxaluria, type II (disorder) ICD‐10 code and term




231

3731 Primary hyperoxaluria type III Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ Associated with mutations in an uncharacterized gene (DHDPSL) on chromosome 10. Old ERA‐EDTA PRD code


‐ 828971000000101 ‐ Primary hyperoxaluria, type III (disorder) ICD‐10 code and term




232

3224 Fabry disease ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Gene test


‐ 54 ‐ Fabry's disease SNOMED CT concept identifier and fully specified name

‐ 16652001 ‐ Fabry's disease (disorder) ICD‐10 code and term

‐ E752 ‐ Other sphingolipidosis



233

3230 Fabry disease ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Histology ‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Gene test


‐ 54 ‐ Fabry's disease SNOMED CT concept identifier and fully specified name

‐ 16652001 ‐ Fabry's disease (disorder) ICD‐10 code and term

‐ E752 ‐ Other sphingolipidosis



234

3248 Xanthinuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/278300 about xanthinuria, type I ‐ http://omim.org/entry/603592 about xanthinuria, type II


‐ Biochemistry ‐ Gene test



‐ 190919008 ‐ Xanthinuria (disorder) ICD‐10 code and term




235

3253 Nail‐patella syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test



‐ 236527004 ‐ Nail patella‐like renal disease (disorder) ICD‐10 code and term

‐ N078 ‐ Hereditary nephropathy, not elsewhere classified, other



236

3269 Rubinstein‐Taybi syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/180849 about Rubinstein‐Taybi syndrome 1; RSTS1 ‐ http://omim.org/entry/613684 about Rubinstein‐Taybi syndrome 2; RSTS2





‐ 45582004 ‐ Rubinstein‐Taybi syndrome (disorder) ICD‐10 code and term

‐ Q872 ‐ Cong malformation syndromes predominantly involving limbs



237

3276 Tuberous sclerosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/191100 about tuberous sclerosis 1; TSC1 ‐ http://omim.org/entry/605284 about TSC1 gene; TSC1 ‐ http://omim.org/entry/613254 about tuberous sclerosis 2; TSC2 ‐ http://omim.org/entry/191092 about TSC2 gene; TSC2


‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test



‐ 7199000 ‐ Tuberous sclerosis syndrome (disorder) ICD‐10 code and term

‐ Q851 ‐ Tuberous sclerosis



238

3282 Von Hippel‐Lindau disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/193300 about Von Hippel‐Lindau syndrome; VHL ‐ http://omim.org/entry/608537 about VHL gene





‐ 46659004 ‐ Von Hippel‐Lindau syndrome (disorder) ICD‐10 code and term

‐ Q858 ‐ Other phakomatoses, not elsewhere classified



239

3295 Medullary sponge kidneys Criteria for diagnosis [all to be satisfied]



‐ 236443009 ‐ Medullary sponge kidney (disorder) ICD‐10 code and term




240

3305 Horse‐shoe kidney Criteria for diagnosis [all to be satisfied]



‐ 41729002 ‐ Horseshoe kidney (disorder) ICD‐10 code and term

‐ Q631 ‐ Lobulated, fused and horseshoe kidney



241

3314 Frasier syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Clinical exam ‐ Imaging ‐ Gene test



‐ 445431000 ‐ Frasier syndrome (disorder) ICD‐10 code and term

‐ Q998 ‐ Other specified chromosome abnormalities



242

3658 Renal coloboma syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov


‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test


‐ Associated with the Pax2 gene. Old ERA‐EDTA PRD code


‐ 446449009 ‐ Renal coloboma syndrome (disorder) ICD‐10 code and term




243

3322 Branchio‐oto‐renal syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/113650 about branchiootorenal syndrome 1; BOR1 ‐ http://omim.org/entry/610896 about branchiootorenal syndrome 2; BOR2





‐ 290006 ‐ Melnick‐Fraser syndrome (disorder) ICD‐10 code and term

‐ Q870 ‐ Cong malform syndromes predom affect facial appearance



244

3333 Williams syndrome Criteria for diagnosis [all to be satisfied]

‐ Clinical exam ‐ Gene test



‐ 63247009 ‐ Williams syndrome (disorder) ICD‐10 code and term

‐ Q878 ‐ Other specified congenital malformation syndromes NEC



245

3346 Townes‐Brocks syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov




‐ SALL1 is the only gene known to be associated with TBS (2011). Old ERA‐EDTA PRD code


‐ 523411000000105 ‐ Townes‐Brocks syndrome (disorder) ICD‐10 code and term




246

3351 Lawrence‐Moon‐Biedl / Bardet‐Biedl syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/245800 about Laurence‐Moon syndrome ‐ http://omim.org/entry/209900 about Bardet‐Biedl syndrome; BBS





‐ 232059000 ‐ Laurence‐Moon syndrome (disorder) ICD‐10 code and term

‐ Q878 ‐ Other specified congenital malformation syndromes NEC



247

3367 Mitochondrial cytopathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:

‐ http://omim.org/entry/251900 about mitochondrial myopathy Criteria for diagnosis [all to be satisfied]

‐ Gene test Additional notes/criteria

‐ Usually diagnosed by muscle histopathology and mitochondrial enzyme complex functional assay.



‐ 240096000 ‐ Mitochondrial cytopathy (disorder) ICD‐10 code and term

‐ G713 ‐ Mitochondrial myopathy, not elsewhere classified




248

3379 Familial nephropathy Criteria for diagnosis [all to be satisfied]

‐ Family history Additional notes/criteria

‐ A clear and compatible family history must be present that cannot be assigned to any more specific familial disorder.



‐ 236419006 ‐ Progressive hereditary glomerulonephritis without deafness (disorder) ICD‐10 code and term


V MISCELLANEOUS RENAL DISORDERS

249

3380 Acute kidney injury Criteria for diagnosis [all to be satisfied]



‐ For a brief definition of acute and chronic renal failure when RRT is required, see 'Notes for users'.

‐ This super concept PRD should only be used if it is not possible to choose a more accurate one.


‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name

‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term

‐ N179 ‐ Acute renal failure, unspecified



250

3398 Acute kidney injury due to hypovolaemia Criteria for diagnosis [all to be satisfied]




‐ N179 ‐ Acute renal failure, unspecified AND E86X ‐ Volume depletion



251

3403 Acute kidney injury due to circulatory failure Criteria for diagnosis [all to be satisfied]




‐ N179 ‐ Acute renal failure, unspecified AND R579 ‐ Shock, unspecified



252

3419 Acute kidney injury due to sepsis Criteria for diagnosis [all to be satisfied]




‐ N179 ‐ Acute renal failure, unspecified



253

3426 Acute kidney injury due to rhabdomyolysis Criteria for diagnosis [all to be satisfied]





‐ N179 ‐ Acute renal failure, unspecified AND M628 ‐ Other specified disorders of muscle



254

3435 Acute kidney injury due to nephrotoxicity Criteria for diagnosis [all to be satisfied]



‐ 236428007 ‐ Nephrotoxic acute renal failure (disorder) ICD‐10 code and term




255

3442 Acute cortical necrosis Criteria for diagnosis [all to be satisfied]



‐ Either imaging or histological evidence is acceptable. Old ERA‐EDTA PRD code


‐ 197650009 ‐ Acute cortical necrosis (disorder) ICD‐10 code and term

‐ N171 ‐ Acute renal failure with acute cortical necrosis



256

3457 Acute pyelonephritis Criteria for diagnosis [all to be satisfied]


‐ Either evidence of urinary tract infection with white cell casts or histology. Old ERA‐EDTA PRD code

‐ Not available in previous coding system SNOMED CT concept identifier and fully specified name

‐ 36689008 ‐ Acute pyelonephritis (disorder) ICD‐10 code and term

‐ N10X ‐ Acute tubulo‐interstitial nephritis



257

3461 Kidney tumour Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/144700 about renal cell carcinoma, nonpapillary; RCC ‐ http://omim.org/entry/150800 about hereditary leiomyomatosis and renal cell

cancer; HLRCC ‐ http://omim.org/entry/609322 about rhabdoid tumor predisposition syndrome 1;

RTPS1 ‐ http://omim.org/entry/267000 about renal hamartomas, nephroblastomatosis, and

fetal gigantism Criteria for diagnosis [all to be satisfied]


‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name

‐ 126880001 ‐ Neoplasm of kidney (disorder) ICD‐10 code and term

‐ D410 ‐ Neoplasm uncert / unkn behav kidney



258

3474 Renal cell carcinoma ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/605074 about renal cell carcinoma, papillary, 1; RCCP1 ‐ http://omim.org/entry/613253 about hereditary renal cancer, associated 1; HRCA1 ‐ http://omim.org/entry/606423 about disrupted in renal carcinoma 1; DIRC1 ‐ http://omim.org/entry/602773 about disrupted in renal carcinoma 2; DIRC2 ‐ http://omim.org/entry/608262 about disrupted in renal carcinoma 3; DIRC3 ‐ http://omim.org/entry/300854 about renal cell carcinoma, xp11‐associated; RCCX1 ‐ http://omim.org/entry/179755 about papillary renal cell carcinoma translocation‐

associated gene; PRCC ‐ http://omim.org/entry/605642 about thyroid carcinoma, papillary, with papillary renal

neoplasia ‐ http://omim.org/entry/144700 about renal cell carcinoma, nonpapillary; RCC ‐ http://omim.org/entry/150800 about hereditary leiomyomatosis and renal cell

cancer; HLRCC Criteria for diagnosis [all to be satisfied]



‐ 254915003 ‐ Clear cell carcinoma of kidney (disorder) ICD‐10 code and term

‐ C64X ‐ Malignant neoplasm of kidney, except renal pelvis



259

3488 Transitional cell carcinoma ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/109800 about bladder cancer. Refers to TCC of the renal pelvis Criteria for diagnosis [all to be satisfied]



‐ 408642003 ‐ Transitional cell carcinoma of kidney (disorder) ICD‐10 code and term




260

3490 Wilms tumour ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov

‐ http://omim.org/entry/194070 about Wilms tumor 1; WT1 ‐ http://omim.org/entry/605442 about Wilms tumor 1‐associating protein; WTAP ‐ http://omim.org/entry/194071 about Wilms tumor 2; WT2 ‐ http://omim.org/entry/194090 about Wilms tumor 3; WT3 ‐ http://omim.org/entry/601363 about Wilms tumor 4; WT4 = familial wilms tumor 1;

FWT1 ‐ http://omim.org/entry/601583 about Wilms tumor 5; WT5 ‐ http://omim.org/entry/194072 about Wilms tumor, aniridia, genitourinary anomalies,

and mental retardation syndrome; WAGR ‐ http://omim.org/entry/612469 about Wilms tumor, aniridia, genitourinary anomalies,

mental retardation, and obesity syndrome; WAGRO Criteria for diagnosis [all to be satisfied]



‐ 302849000 ‐ Nephroblastoma (disorder) ICD‐10 code and term




261

3501 Mesoblastic nephroma ‐ histologically proven Criteria for diagnosis [all to be satisfied]



‐ 307604008 ‐ Mesoblastic nephroma (disorder) ICD‐10 code and term

‐ D410 ‐ Neoplasm uncert / unkn behav kidney



262

3517 Single kidney identified in adulthood Criteria for diagnosis [all to be satisfied]


‐ Diagnosis codes expressing the presence of a kidney (left or right) are not suitable because they do not imply absence of the contralateral kidney.



‐ 824131000000108 ‐ Solitary kidney (finding) ICD‐10 code and term




263

3529 Chronic kidney disease (CKD) / chronic renal failure (CRF) caused by tumour nephrectomy Criteria for diagnosis [all to be satisfied]


‐ Causing kidney disease due to loss of renal mass in the absence of another identified primary renal disease.


‐ 96 ‐ Traumatic or surgical loss of kidney SNOMED CT concept identifier and fully specified name

‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term

‐ N189 ‐ Chronic renal failure, unspecified



264

3538 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to traumatic loss of kidney Criteria for diagnosis [all to be satisfied]




‐ 96 ‐ Traumatic or surgical loss of kidney SNOMED CT concept identifier and fully specified name





265

3540 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to donor nephrectomy Criteria for diagnosis [all to be satisfied]




‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name





266

3555 Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ no histology Criteria for diagnosis [all to be satisfied]


‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.

‐ It does not mean that no information is available. In that case, consider using PRD 3643 'Chronic renal failure'.







267

3564 Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ histologically proven Criteria for diagnosis [all to be satisfied]










268

3572 Haematuria and proteinuria ‐ no histology Criteria for diagnosis [all to be satisfied]

‐ Biochemistry ‐ Urine analysis






‐ 53298000 ‐ Hematuria syndrome (disorder) AND 29738008 ‐ Proteinuria (disorder) ICD‐10 code and term

‐ R31X ‐ Unspecified haematuria AND N391 ‐ Persistent proteinuria, unspecified



269

3712 Isolated haematuria ‐ no histology Criteria for diagnosis [all to be satisfied]





‐ Alternative PRDs are available for 'Isolated proteinuria ‐ no histology' and for 'Haematuria and proteinuria ‐ no histology'.



‐ 53298000 ‐ Hematuria syndrome (disorder) ICD‐10 code and term

‐ R31X ‐ Unspecified haematuria



270

3720 Isolated proteinuria ‐ no histology Criteria for diagnosis [all to be satisfied]





‐ Alternative PRDs are available for 'Isolated haematuria ‐ no histology' and for 'Haematuria and proteinuria ‐ no histology'.



‐ 29738008 ‐ Proteinuria (disorder) ICD‐10 code and term

‐ N391 ‐ Persistent proteinuria, unspecified



271

3643 Chronic renal failure due to systemic infection Criteria for diagnosis [all to be satisfied]

‐ Clinical history ‐ Biochemistry ‐ Imaging



‐ 90688005 ‐ Chronic renal failure syndrome (disorder) ICD‐10 code and term




272

3691 Renal failure Criteria for diagnosis [all to be satisfied]



‐ This super concept PRD should only be used if it is not possible to specify even whether the patient has acute or chronic renal failure.



‐ 42399005 ‐ Renal failure syndrome (disorder) ICD‐10 code and term

‐ N19X ‐ Unspecified renal failure




273

3708 Chronic renal failure Additional notes/criteria

‐ This super concept PRD should be used: 1) when the investigations that have been undertaken do not allow any more

granular PRD to be selected or 2) very infrequently when a full diagnosis has been made which can not be assigned

to another PRD. In this case, please send an email to the ERA‐EDTA Registry office, highlighting the PRD that is missing.

‐ Where a patient has been investigated appropriately but it is not possible to choose a more granular PRD, consider using: ‐ PRD 3555 = 'Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ no histology, or ‐ PRD 3564 'Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ histologically proven'.

‐ See 'Notes for users'. Old ERA‐EDTA PRD code


‐ 90688005 ‐ Chronic renal failure syndrome (disorder) ICD‐10 code and term


2012 primary renal disease (prd) codes - era-edta reg · 2012 primary renal disease (prd) codes...

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