modified by faysal massad - ju medicine · 2020-04-08 · 3-autosomal dominant (adult) polycystic...
TRANSCRIPT
![Page 1: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/1.jpg)
1
CYSTIC DISEASES of THE
KIDNEY
Dr. Nisreen Abu Shahin
Modified by Faysal Massad
cysts aredilatedtubulesusually
due Tiresussure or due to othercauses
![Page 2: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/2.jpg)
Types of cysts
1-Simple Cysts2-Dialysis-associated acquired cysts 3-Autosomal Dominant (Adult) Polycystic Kidney Disease 4-Autosomal Recessive (Childhood) Polycystic Kidney Disease 5-Medullary Cystic Disease
2
![Page 3: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/3.jpg)
Simple renal Cysts
![Page 4: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/4.jpg)
1-Simple Cysts�Multiple or single
�1-5 cm in diameter�filled with clear fluid.
�confined to the cortex.
�no clinical significance. �Usually discovered incidentally or because of hemorrhage and pain
�Importance: to differentiate from kidney tumors
4
Follow up byimaging techniques
A A
Not very Seriousmild complications
2
![Page 5: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/5.jpg)
Cysts associated with chronic dialysispatients with CRF I
Possible complicationsSI e frinton
![Page 6: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/6.jpg)
2-Dialysis-associated acquired cysts
�in patients with renal failure who have prolonged dialysis.
�both cortex and medulla�Complications: hematuria; pain
�Increased risk of renal carcinomas (100 times greater than in the general population)
6
P risk of infection3 Stonefomention
y rapture of cyst hemorrhage 4 malignancy
Those cysts are significantinsidecyst
multiple cells variablein size
may be numerousabnormalunction
Pmffffaptiongrowtuzator secretionepithelialproliferation
tubularkidney transplantation is the optimum solution
![Page 7: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/7.jpg)
Autosomal Dominant (Adult) Polycystic Kidney Disease
7
1 a y Irasp Id p un
GIT f onset of diseaseCause manifestations
cortex
T is lost
weight parenchyma
lost1.5kg sofuretionnormal j
so CRF150g aurine
hence causing abdominalmassesimpairment
![Page 8: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/8.jpg)
3- Autosomal Dominant (Adult) Polycystic Kidney Disease
�multiple bilateral cysts
�eventually destroy the renal parenchyma.
�Incidence (1: 500-1000) persons�10% of chronic renal failure.
�inheritance of one of 2 autosomal dominant genes:
�(1)- PKD1: 85-90% (encodes polycystin-1)�(2)- PKD2 :10-15% (encodes polycystin- 2).
8
gr
in cortex medulla
Common
by the age of 50yrs
tubules are abnormal from early time but progress isslow 12 hence manifestations appear
![Page 9: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/9.jpg)
3-Autosomal Dominant (Adult) Polycystic Kidney Disease – cont.Clinical presentation :
�asymptomatic until the 4th decade
�Symptoms: flank pain , heavy dragging sensation, abdominal mass, hemorrhage, obstruction, Intermittent gross hematuria
Complications
1- hypertension ( 75% )
2- urinary infection
3- vascular aneurysms of circle of Willis (10% -30%)Æ (subarachnoid hemorrhage ).
4- renal failure at age 509
S be a T pi e disease was always found duringadulthood
due to slow progression
small of patients
Berry
Transplantation is needed
![Page 10: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/10.jpg)
4-Autosomal Recessive (Childhood) Polycystic Kidney Disease
�autosomal recessive
�1:20,000 live births.
�Types: perinatal, neonatal, infantile, and juvenile.�Associated with liver cysts
�Mutations in PKHD1 gene coding for fibrocystin.
�Fibrocystin may be involved in the function of cilia in tubular epithelial cells .
10
manfestationsappear early
since AR4 types
extra renal earlier worsePrognosis
![Page 11: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/11.jpg)
Normal term infant kidneys
![Page 12: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/12.jpg)
Autosomal Recessive (Childhood) Polycystic Kidney Disease:
12
fstaffecystTreatmentis also cortex
transplantation medulla
porandymalboss
![Page 13: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/13.jpg)
5- Medullary Cystic Disease¾2 major types:
1-medullary sponge kidney
¾- common and innocent condition.2-nephronophthisis-medullary cystic disease complex
¾- almost always associated with renal dysfunction.
¾- usually begins in childhood.¾- Cysts are at cortico-medullary junction
13
cysts only in medulla
Usuallywithoutdangerousclinicaleffects
Geneticallyey Tasting determined
bymany
Tubules2 interstitial abnormalitiesGenes
![Page 14: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/14.jpg)
5- Medullary Cystic Disease
o Clinical features:o polyuria and polydipsia (↓tubular function). o renal failure over 5-10-yearo A positive family history and unexplained chronic renal failure in young patients should lead to suspicion of medullary cystic disease.
14
tubular diluted urineAtrophy
nd interstitialFibrosisleadingto
Renal
failure
![Page 15: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/15.jpg)
URINARY OUTFLOW OBSTRUCTION
15
an obstruction anywhere in the outflowtrack
![Page 16: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/16.jpg)
•Renal Stones (Urolithiasis)
•stone formation at any level in the urinary collecting system.•Most common in kidney. •(1%) of all autopsies.•Symptomatic more common in men•Familial tendency toward stone formation•unilateral in 80%•Variable sizes
16
The most important
Can be asymptomatic
![Page 17: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/17.jpg)
Stone = inorganic salt (98%) + organic matrix (2%)
�Types are according to inorganic salt: 1- calcium oxalate/ calcium oxalate+ calcium phosphate-- (80%) .2- Struvite (magnesium ammonium phosphate) (<10%)3- uric acid (6-7%) 4- cystine stones (2%)
17
Moffatt from cells or bacterialcolonies
least common
![Page 18: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/18.jpg)
Causes of Renal Stones 1-increased urine concentration of stone's constituents exceeds solubility in urine (supersaturation).
�50% of calcium stones pts have hypercalciuria with no hypercalcemia.�5% to 10% Æ hypercalcemia and hypercalciuria.
18
nude
![Page 19: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/19.jpg)
2-The presence of a nidus�Urates provide a nidus for calcium deposition.
�Desquamated epithelial cells�Bacterial colonies
3-urine pH¾Magnesium ammonium phosphate (struvite) stones occur with alkaline urine due to UTIs. ¾Uric acid stones form in acidic urine (under pH 5.5).
4-infectionsurea-splitting bacteria (Proteus vulgaris and staph).
organic nucleus
have urease enzyme
![Page 20: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/20.jpg)
Hydronephrosis
20
![Page 21: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/21.jpg)
Hydronephrosis•dilation of the renal pelvis and calyces due to obstruction, with accompanying atrophy of kidney parenchyma. •sudden or insidious•Obstruction at any level from the urethra to the renal pelvis.•The most common causes are :
21
due tobadoutflowof urine
ice ureter urinary bladderurethra
![Page 22: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/22.jpg)
22
Hydronephrosis of the kidney, with marked dilation of the pelvis and calyces and thinning of renal parenchyma.
![Page 23: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/23.jpg)
1-Congenital:examples •Atresia of urethra•Valve formations in ureter or urethra •Aberrant renal artery compressing ureter •Renal ptosis with torsion or kinking of ureter
23
![Page 24: Modified by Faysal Massad - JU Medicine · 2020-04-08 · 3-Autosomal Dominant (Adult) Polycystic Kidney Disease multiple bilateral cysts eventually destroy the renal parenchyma](https://reader033.vdocument.in/reader033/viewer/2022042408/5f2350d34ef67d54585def63/html5/thumbnails/24.jpg)
2-Acquired: �Examples:�Foreign bodies: Calculi, necrotic apillae�Tumors: prostatic hyperplasia, prostate cancer, bladder tumors, cervix or uterus cancer.�Inflammation: Prostatitis, ureteritis, urethritis, �Neurogenic: Spinal cord damage�Normal pregnancy: rare, mild and reversible
Stones