renal problems in children dr. rim el-rifai consultant paediatrician qmhc october 2005
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Renal Problems in children
Dr. Rim El-Rifai
Consultant Paediatrician
QMHC
October 2005
Outline
Renal malformations and Common Urological problems
Common Renal Problems Summary
Introduction
• Developmental disorders account for a wide spectrum of kidney diseases that cause considerable morbidity and mortality in the first years of life
• Childhood kidney disorders can predispose to adult morbidity and mortality
• Chronic renal diseases can affect growth
Renal Malformations and Common Urological Problems
Renal malformations
The major causes of end-stage renal failure in children
Can be diagnosed Antenatally Can be part of a syndrome Some have a genetic basis
– Vesico-ureteric reflux
Types of abnormalities detected antenatally
Abnormalities in the size of the kidneys
Abnormalities in the texture of the renal parenchyma
Visible cysts Hydronephrosis
Abnormal renal size Large:
– Polycystic kidney disease– Multicysticdysplastic– Cystic dysplasia– Congenital nephrotic syndrome– Renal tumour– Compensatory hypertrophy
Small:– Renal dysplasia/hypoplasia– Damage from obstructive uropathy
Abnormal Parenchyma
Echobright:– Polycystic disease– Cystic dysplasia– Damage from obstructive uropathy– Glomerulo-cystic disease– Congenital Nephrotic syndrome
Macrocysts:– AD PCKD– TS– Multicystic dysplastic– Cystic dysplasia
Hydronephrosis
• Obstruction: • PUJO• VUJO/ Megaureter• PUV• Ureterocele
• Non-obstruction: • VUR, • prune-belly s.
Other renal problems detected antenatally
Duplication of the upper tract Renal agenesis Renal fusion and ectopia
Diagnosis And Management of Antenatal Hydronephrosis
Diagnosis:– Antenatal screening– Postnatal KUB Ultrasound – MCUG– MAG3 renogram/ DMSA
Management:– Prophylactic antibiotics – Early treatment of UTI and complications– surgery
Urinary Tract Infections
Urinary tract infections
One of the most common bacterial infections in childhood (7% of girls- 2% of boys)
Most present to the primary care physician Complications can result in end-stage renal
disease and hypertension Can be as a result of underlying anatomical
abnormalities E-Coli cause 80-90% of first time UTI
Risk Factors associated with permanent renal damage
Obstruction Vesico-ureteric reflux with dilatation Younger age (< 4 years) Delay of treatment Number of pyelonephritis attacks Uncommon bacteria
Long-term consequences of reflux nephropathy
Chronic renal failure– Reflux nephrophathy in 13% of patients 5-44 years of
age with ESRF (Australia and New Zealand)– 30% of CRF in children (Wales)– 39% of renal transplants (Ireland)
Hypertension– Need for annual BP monitoring for life
Complications of pregnancy– UTI during pregnancy– Pregnancy induced hypertension– Complicated pregnancies, and worse fetal outcome
Diagnosis and management of childhood UTI
MSU Early antibiotic therapy Prophylactic antibiotics Imaging: USS, MCUG, DMSA, other Surgery Monitoring of BP annually
Voiding Dysfunction and The Wet Child
Normal Sequence of Developing Bladder and Bowel Control
Nocturnal bowel control
Daytime bowel control
Daytime bladder control
Nocturnal bladder control
Classification: Primary vs secondary
Primary mono-symptomatic nocturnal enuresis Primary Diurnal enuresis
– structural urological abnormalities.– Neuropathic Bladder
Secondary Diurnal enuresis– UTI– Dysfunctional voiding– Concentration abnormalities: IDDM, Diabetes
insipidus– Neuropathic bladder
Characterization of Voiding Dysfunction
Storage Problem: Failure to Store normal volumes of urine at low pressure & without leakage– Non compliant bladder– overactive bladder– Inadequate sphincter tone during filling
Emptying Problem: Failure to empty completely, on command, efficiently at low pressures– Failure of neurological control of bladder– Bladder muscle failure– Failure of sphincter relaxation during voiding
Evaluation of Dysfunctional Voiding
History Physical Exam Laboratory Tests Imaging and Urodynamics
Management of the wet child Treatment of underlying causes:
– UTI– anatomical abnormalities
Bladder training (and bowel) Drugs:
– Anticholinergics – Desmopressin
Other:– Alarms, star charts, – surgery
Common Renal Problems
Features of Renal problems Clinical:
– Oedema– Polyuria and polydypsia– Failure to thrive/ short stature– Hypertension
Abnormal investigations– Blood:
» U&E, Albumin, Bone
– Urinalysis» Proteinuria» Haematuria
Haematuria
Detection of Haematuria
Visual examination Dipsticks Microscopy
Causes of gross Haematuria in 150 children: Pediatrics 1977
UTI– Proven 39– Suspected 35
Perineal irritation 16 Trauma 10 Acute nephritis 6 Coagulopathy 5 Stones 3 Tumour 1 Other 35
IgA Nephropathy
Male predominance More common in 2nd- 3rd decades 2-10% of glomerulonephritides in
UK
IgA Nephropathy Diagnosis:
– macroscopic haematuria, – asymptomatic microscopic haematuria and
proteinuria, – acute nephritis, – nephrotic syndrome, – mixed nephritic-nephrotic synd.
Renal biopsy: deposits of IgA (plasma IgA raised in 20%)
Prognosis: – clinical course variable, – 50% recurrence in transplanted kidney
Alport Syndrome
1. Inherited nephritis, 2. Sensori-neural deafness, 3. Occular defects, 4. less commonly large Platelets.
80-90% X-linked dominant, 10-20% AR
Diagnosis: renal biopsy.
Alport Syndrome
Treatment: – ACE inhib. or Angiotensin recept. Blockers, – blood pressure control, – Renal transplantation
Prognosis Poor in boys: – proteinuria, hypertension and renal
impairment in late teens. – Hearing loss in adolescent years
Thin Basement membrane nephropathy
Haematuria Variable clinical course Biopsy DD:
–Alports S.
–benign familial haematuria
Benign familial haematuria
Microscopic haematuria AD inheritance Normal biopsy Good prognosis
Proteinuria
Proteinuria in Renal disease
Dipsticks: correlate better with level of albuminuria
24 hour urinary protein >60mg/m2/day Early morning Urine Protein/creatinine
ratio > 10 –25 mg/mmol Exercise and age related in normal
children
Causes of proteinuria on dipstick
Artefect: alk. Urine, contamination by vag. Secretions
Benign: – Functional: exercise, cold, fever, congestive heart
failure– Idiopathic: incidental finding: Transient- Intermittent– Orthostatic: transient- fixed (<2 g/24 hr)
Persistent/ non-benign:– Persistent isolated– Disease related
Disease related Proteinuria
Glomerular mechanisms: increased protein filtration – Damage to basement membrane– Loss of glomerular anion– Increased glomerular permeability
Tubular causes: decreased protein reabsorption
other
Proteinuria evaluation Exclude non-renal causes History, examination, urinalysis Documentation of proteinuria:
– Dipstick diary: BD for 1/52– 24 hr urine collection– Random Pr:Cr ratio
MSU GFR measurement: est. GFR (Schwartz formula) Immunology/serology: C3-C4, ASOT, anti-hyaloronidase,
ANA, anti-DNA. Blood chemistry: prot, alb, cholest.
Disease related proteinuria
Renal disease:– Glomerular causes– Tubular causes: hereditary, ATN, heavy metal
poisoning– Secretory proteinuria: neonates, lower urinary tract
Other diseases– Overflow proteinuria– Histuria– other
Nephrotic Syndrome
Idiopathic Nephrotic syndrome
More common in boys More common in Arabs and people
from Indian subcontinent Peak incidence 2-5 years Minimal change disease the most
common Genetic component
Clinical features
Oedema: – gravity related, effusions
BP: – usually normal or low, – paradoxically elevated in 20%
abdominal pain: – hypovolaemia, peritonitis
Lab. findings
Urine: – large amounts of albumin (>50 mg/kg/day)– microscopic haematuria (23%),
Blood: – Hypoalbuminaemia (<25 g/l), – low IgG, – increased cholesterol, – usually normal U & E, – reduced total calcium, – raised or decreased Hb and Hct
Investigations of Nephrotic Syndrome
Urine:– Urinalysis and MC&S– Quantification of proteinuria: Early morning Pr:Cr ratio, 24 hr
collection– U- Na if hypovolaemia suspected
Plasma:– U, Cr & E– Albumin, T. Prot., Ca, Phos.– C3, C4, ASOT, ANA– Hep B serology– Varicella IgG status
FBC
Treatment of SSNS
Admit to hospital Treat associated infection: Penicillin Steroids:
– evidence that longer initial course ( 6-7 mo vs 2-3 mo) protects from frequent relapses
Supporting treatments: diuretics Diet:
– no-added salt, healthy eating, fluid restriction
Complications of SSNS Infection:
– Low IgG and serum factor B (C3 proactivator), impaired opsonisation and lymphocyte transformation, immunosupression
Thrombosis: – Thrombocytosis, increased clotting factors
(V, VII, VIII, X, Fibrinogen), reduced Antothrombin III, hypovolaemia, steroids
Complications of SSNS
Acute renal failure: – pre-renal commonly, less common ATN
Hyperlipidaemia: – mechanism poorly understood
Malnutrition Side effects of treatment:
– steroids, alkylating agents, Cyclosporin A, Levamisole
Indications for renal biopsy in NS
Age < 12 months (continuous or congenital/ infantile NS)
Age > 16 years Persistent hypertension macroscopic haematuria Impaired renal function unresponsive to
correction of hypovolaemia Low C3, C4 Failure to respond to initial course of steroids
Other Nephrotic Syndromes
Steroid-resistant NS:– Focal segmental glomerulosclerosis and
minimal change disease: rising incident in African-American children
– Membranoproliferative (mesangiocapillary) glomerulonephritis: uncommon
– Membranous nephropathy: very rare Congenital/ Infantile NS
Acute Nephritis Syndrome
Acute Nephritis Syndrome
Acute glomerular injury and inflammation with decreased GFR and Na and water retention
Urinalysis: – Haematuria + Albuminuria + red cell casts
Most common cause: – Acute post-streptococcal Glomerulo-
nephritis (APSGN) 80%
Other causes of acute nephritis
IgA Nephropathy HSP Membranoproliferative Glomerulonephritis Lupus nephritis ANCA-positive vasculitis Chronic infections:
– Shunt nephritis, Infective endocarditis, Hep B, Hep C, HIV nephropathy
Acute post-Streptococcal Gromelunephritis
(APSGN)
APSGN
Post throat or skin infection – nephritogenic group A beta-haemolytic
streptococcus Risk of APSGN is 10-15%, (40% within
families)
AB’s do not prevent GN but important to prevent further spread of bacteria
APSGN
Age: 5-15 years More common in males Antigen-antibody related nephritis Abrupt onset 7-14 days after throat
infection and 3-6 weeks after skin infection
Renal involvement in APSGN
Mild asymptomatic Haematuria
Acute renal failure with oligo-anuria
(rarely necessitating dialysis)
Clinical features of APSGN Acute fluid overload
– Peripheral oedema– Pulmonary oedema– Congestive heart failure
Hypertension Haematuria (micro +/- macro) Proteinuria Renal function impairment
– Oliguria– Elevated plasma creatinine
Investigations
Urinalysis:– M C &S, – early morning Pr: Cr ratio
Bacteriology: – throat swab, – ASOT, – Streptozyme essay ( strptolysin O,
streptokinase, DNAse B, Hyaloronidase, NADase antibodies)
Investigations
Immunology: – C3 and C4– (ANCA, ANA and double straded DNA Ab, GBM
Ab)
Renal function: – U Cr &E, acid-base, plasma proteins, Ca and
phos.
Haematology: – FBC, blood film
Management
Eradication of organism Treatment of renal failure: supportive
Indications for in-patient management: Hypertension Oedema Oliguria Elevated plasma creatinine Electrolyte abnormalities
Clinical course and long term prognosis
Most symptoms subside in 2-3 weeks C3 back to normal in 8-12 weeks Microscopic haematuria +/- low grade
proteinuria can persist for 1-2 years
Excellent prognosis overall in children
Renal manifestations of Systemic Disorders
Childhood vasculitis Systemic symptoms: malaise, fever weight
loss– purpuric skin rash– Haematuria and red cell casts– Arthropathy– Serositis– Unexplained cardiac or pulmonary disease
Lab: anaemia, leukocytosis, thrombocytosis, raised ESR or CRP, ANCA +ve
Henoch-Schönlein Purpura
Multisystem small vessel systemic vascultitis
Prominent cutaneous component
Most common vasculitis in children
14/100 000 of children Most favourable outcome
HSP nephritis
Incidence varies greatly (20-61%)
Up to 2 months from presentation
Isolated haematuria- acute nephritis picture
Treatment supportive Steroids in severe GI, Immunosuppressant for
severe renal involvement
Prognosis: HSP nephritis
Risk of chronic renal impairment <2% overall
CRF – up to 10% in patients referred to Nephrologist– Picture of nephritic/nephrotic nature and
crescentic changes on biopsy– Late deterioration in renal function well
recognised: long term FU needed
Other vasculitis syndromes
SLE Kawasaki disease Takayasu’s arteritis Polyarteritis nodosa Wegenr’s granulomatosis
Other renal manifestations of systemic diseases
Cystic Fibrosis– Nephrotoxic drugs– Tubulo-interstitial nephritis– IgA Nephropathy
Diabetes Mellitus– microalbuminuria
Summary
Antenatal screening can detect a significant number of renal and urological abnormalities
Prevention and treatment of reflux nephropathy can prevent ESRD
Nephro-Urological problems in childhood are reasonably common
Spectrum of childhood nephro-urological problems extend through adolescent age necessitating close collaboration with adult nephrologists
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