Cystic diseases of kidney

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benign and malignant cystic renal disease including hydronephrosis

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Cystic diseases of kidney

  1. 1. CYSTIC DISEASES OF KIDNEY DR. ARPITA SAHA
  2. 2. KIDNEY CYST • A FLUID-FILLED SAC • LINED BY AN EPITHELIUM • ARISING FROM A DILATATION IN ANY PART OF THE NEPHRON OR COLLECTING DUCT
  3. 3. 1. MULTICYSTIC RENAL DYSPLASIA 2. POLYCYSTIC KIDNEY DISEASE A. AUTOSOMAL-DOMINANT (ADULT) POLYCYSTIC DISEASE B. AUTOSOMAL-RECESSIVE (CHILDHOOD) POLYCYSTIC DISEASE 3. MEDULLARY CYSTIC DISEASE A. MEDULLARY SPONGE KIDNEY B. NEPHRONOPHTHISIS 4. ACQUIRED CYSTIC DISEASE A. DIALYSIS-ASSOCIATED B. HYDATID CYST C. TUBERCULOSIS D. NEOPLASM ASSOCIATED 5. LOCALIZED (SIMPLE) RENAL CYSTS 6. RENAL CYSTS IN HEREDITARY MALFORMATION SYNDROMES (E.G., TUBEROUS SCLEROSIS, VHL) 7. GLOMERULOCYSTIC DISEASE 8. EXTRAPARENCHYMAL RENAL CYSTS A. PYELOCALYCEAL CYSTS B. HILAR LYMPHANGITIC CYSTS
  4. 4. ADPKD • HEREDITARY • AGE OF PRESENTATION- USUALLY 4TH TO 5TH DECADE • COMMON • 1/400- 1000 LIVE BIRTH • M/C MUTATION-PKD1 IN CHROMOSOME 16 (85% CASES, MORE SEVERE) • POLYCYSTIN 1 • PKD 2 IN CHROMOSOME 4 (LESS SEVERE) • POLYCYSTIN 2
  5. 5. ADPKD • GROSS: • USUALLY BILATERAL ENORMOUS ENLARGEMENT • SIZES; WEIGHTS • EXTERNAL SURFACE: MASS OF CYSTS WITHNOINTERVENING PARENCHYMA • CYSTS: CLEAR/SEROUS FLUID/TURBID REDTOBROWN/HEMORRHAGIC FLUID • ENLARGEMENT PRESSURE EFFECTS ON CALYCES ANDPELVIS . .+/-
  6. 6. MICROSCOPY • CYSTS: LINEDBY CUBOIDAL/FLATTENEDEPITHELIUM • INTERVENENING AREA: ISCHEMIC ATROPHY FUNCTIONINGNEPHRONS INTERSTITIAL SCARRING TUBULARATROPHY • PELVICALYCEAL SYSTEM: PRESSURE EFFECT
  7. 7. CLINICAL FEATURES • ASYMPTOMATIC / RENAL INSUFFICIENCY > 30 YRS • PAIN: HAEMORRHAGE/PROGRESSIVE DILATION OF CYSTS • RENAL COLIC: EXCRETION OF BLOOD CLOTS CAUSES. • ABDOMINAL PALPATION: HUGELY ENLARGED KIDNEYS • HEMATURIA • FEATURES OF PROGRESSIVE CHRONIC KIDNEY DISEASE: • PROTEINURIA , POLYURIA, AND HYPERTENSION.
  8. 8. CLINICAL FEATURES….. • PATIENTS WITH PKD2 MUTATIONS: >OLDER AGE AT ONSET >LATER DEVELOPMENT OF RENAL FAILURE. • PROGRESSION IS ACCELERATED IN: BLACKS (LARGELY CORRELATED WITH SICKLE-CELL TRAIT) MALES IN THE PRESENCE OF HYPERTENSION
  9. 9. DIAGNOSIS USUALLY ESTABLISHED BY USG • REVEALS DIFFUSE HYPERECHOGENICITY, BILATERALLY ENLARGED KIDNEYS WITH CYSTS • CRITERIA FOR ADPKD: <30 YEARS: AT LEAST 2 CYST IN ONE KIDNEY • IF EITHER PARENT HAS ADPKD FINDING OF ENLARGED ECHOGENIC KIDNEYS IN FETUS CONFIRMS PRENATAL DIAGNOSIS
  10. 10. EXTRARENAL CONGENITAL ANOMALIES • POLYCYSTIC LIVERDISEASE • SPLEEN • PANCREAS • LUNGS • INTRACRANIAL BERRY ANEURYSMS • PINEAL GLAND • SEMINAL VESICLE • MITRAL VALVE PRO LAPSE AND OTHER CARDIAC VALVULAR ANOMALIES • COLONIC DIVERTICULA • SKELETAL ABNORMALITY
  11. 11. CLINICAL OUTCOME • LONG RUN ESRD • CAUSE OF DEATH • 40% CORONARY/HYPERTENSIVE HEART DISEASE • 25% INFECTION • 15% RUPTURED BERRY ANEURYSM • REST OTHER CAUSES
  12. 12. ARPKD • RARE 1: 40,000 LIVE BIRTH • PKHD1 GENE IN CHROMOSOME 6 • FIBROCYSTIN
  13. 13. GROSS • Enlarged • Smooth external appearance. • RETAIN RENIFORM SHAPE • C/S- numerous small cysts in the cortex and medulla kidney spongelike • Dilated elongated channels are present at RIGHT ANGLES TO THE CORTICAL SURFACE • Complete REPLACEMENT OF the medulla and cortex
  14. 14. MICROSCOPY • CYLINDRICAL DILATION OF ALL COLLECTING TUBULES. • CYSTS - UNIFORM LINING BY CUBOIDAL CELLS LIVER • CYSTS • ASSOCIATED WITH PORTAL FIBROSIS • PROLIFERATION OF PORTAL BILE DUCTS.
  15. 15. CLINICAL FEATURE • LARGE ABDOMINAL MASS AT BIRTH • POTTER PHENOTYPE • FACIES D/T OLIGOHYDROMNIOS • JOINT DEFORMATION • PULMONARY HYPOPLASIA • SEVERE- NEWBORN DIE SHORTLY AFTER BIRTH • OLDER CHILDREN (4-8 YRS) HEPATIC DISEASE • APPROX 23% EXPERIENCE VARICEAL BLEEDING
  16. 16. MULTICYSTIC RENAL DYSPLASIA • M.C.CAUSE OF PALPABLE ABDOMINAL LUMP IN NEONATE • M.C. CAUSE OF CYSTIC DISEASE IN CHILDREN • SPORADIC DISEASE • DUE TO ABNORMAL METANEPHRIC DIFFERENTIATION
  17. 17. MRD • A/W • URETEROPELVIC ANOMALY • CARDIOVASCULAR ABNORMALITY • GROSS • ENLARGED • EXREMELY IRREGULAR • MULTICYSTIC KIDNEY
  18. 18. MRD • CYST LINED BY FLATTTENED EPITHELIAL LINIG • SURROUNDED BY UNDIFFERENTIATED MESENCHYME, CARTILAGE, IMMATURE COLLECTING DUCT • NORMAL NEPHRONS ARE THERE BUT MANY OF THEM HAVE IMMATURE CD
  19. 19. UROLITHIASIS
  20. 20. TYPES • FOURMAIN TYPES • (1) CALCIUMSTONES - CALCIUMOXALATE ORCALCIUMOXALATE MIXEDWITH CALCIUMPHOSPHATE • (2) TRIPLE STONES ORSTRUVITE STONES- MAGNESIUMAMMONIUM PHOSPHATE • (3) URIC ACIDSTONES • (4) CYSTINE
  21. 21. CAUSES • OBSCURED • PREDISPOSING FACTORS • CONC OF SOLUTE • CHANGES IN pH • BACTERIAL INFECTIONS • LACK OF INHIBITORS OF CRYSTAL FORMATION IN URINE
  22. 22. CALCIUM OXALATE STONE • MC CAUSE HYPERCALCIURIA 1. ABSORPTIVE 2. RENAL • ALKALINE URINE PREDISPOSE
  23. 23. STRUVITE STONE • ALKALINE URINE • POST UTI (P. vulg aris ) • AVITAMINOSIS A
  24. 24. URIC ACID STONE • GOUT • DISEASES INVOLVING RAPID CELL TURNOVER EG, LEUKEMIA • ACIDIC URINE
  25. 25. CYSTINE STONE • ACIDIC URINE • A/W IMPAIRED RENAL CYSTINE TRANSPORT
  26. 26. MORPHOLOGY • USUALY UNILATERAL • RENAL PELVIS & CALYCES > BLADDER >……. • SMALL/ LARGE • SMOOTH/ JAGGED • STAGHORN CALCULI
  27. 27. CLINICALLY • ASYMPTOMATIC/ SIGNIFICANT RENAL DAMAGE • COLIC • OBSTRUCT URINE FLOW ULCERATION, BLEEDING (GROSS HEMATURIA)  BACTERIAL INFECTION

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