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CONGENITAL MALFORMATION OF KIDNEY

CONGENITAL MALFORMATION OF KIDNEY. 1 .RENAL HYPOPLASIA –DEFICIENT RENAL PARENCHYMA 2.SUPERNUMERARY KIDNEYS. HYPOPLASTIC KIDNEY. SUPERNUMERARY KIDNEYS(FUSED). CONGENITAL MALFORMATION OF KIDNEY. 3.ABNORMALITIES OF POSITION,FORM & ORIENTATION PELVIC KIDNEY ,HORSESHOE KIDNEY,

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CONGENITAL MALFORMATION OF KIDNEY

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  1. CONGENITAL MALFORMATION OF KIDNEY 1.RENAL HYPOPLASIA –DEFICIENT RENAL PARENCHYMA 2.SUPERNUMERARY KIDNEYS

  2. HYPOPLASTIC KIDNEY

  3. SUPERNUMERARY KIDNEYS(FUSED)

  4. CONGENITAL MALFORMATION OF KIDNEY 3.ABNORMALITIES OF POSITION,FORM & ORIENTATION PELVIC KIDNEY ,HORSESHOE KIDNEY, PERSISTENT RENAL LOBULATION 4.ABNORMALITIS OF DIFFERENTIATION - CYSTIC DISEASE OF THE KIDNEY

  5. FUSED KIDNEY (HORSESHOE)

  6. MULTICYSTIC RENAL DYSPLASIA DISORGANISED METANEPHROGENIC DIFFERENTIATION MOST COMMON FORM OF CYSTIC RENAL DISEASE IN THE NEWBORN &INFANTS. SPORADIC OR FAMILIAL & PART OF A SYNDROME OF OTHER ANOMALIES

  7. MULTICYSTIC RENAL DYSPLASIA COMMONLY ASSOCIATED WITH OBSTRUCTIVE ABNORMALITIES LIKE URETERAL ATRESIA,URETHRAL OBSTRUCTION,OBSTRUCTION OF PELVIURETERIC JUNCTION

  8. MULTICYSTIC RENAL DYSPLASIA MORPHOLOGY - UNILATERAL, BILATERAL, OR FOCAL GROSS – ALMOST ALWAYS CYSTIC –AFFECTED PART IS REPLASED BY DISORDERLY MASS OF MULTIPLE CYSTS RESEMBLING A BUNCH OF GRAPES - CALYSES & PELVIS MAY NOT BE RECOGNISED.URETER IS INVARIABLY ABNORMAL,EITHER ABSENT OR ATRETIC

  9. MULTICYSTIC RENAL DYSPLASIA HISTOLOGY –UNDIFFERENTIATING MESENCHYME COMPOSED OF SMOOTH MUSCLE CARTILAGE, IMMATURE COLLECTING DUCTS. CYSTS IN THE MASS REPRESENTS DILATED TUBULES SURROUNDED BY CONCENTRIC LAYERS OF CONNECTIVE TISSUE GLOMERULI, TUBULES ARE SCANTY OR PRIMITIVE OR ABSENT.

  10. MULTICYSTIC RENAL DYSPLASIA

  11. RENAL DYSPLASIA

  12. RENAL DYSPLASIA

  13. MULTICYSTIC RENAL DYSPLASIA CLINICAL FEATURES – UNILATERAL DYSPLASIA PRESENTS AS FLANK MASS IN NEWBORN &INFANTS PROGNOSIS OF UNILATERAL RENAL DYSPLASIA FOLLOWING REMOVAL OF ABNORMAL KIDNEY IS EXCELLENT PROGNOSIS IN BILATERAL RENAL DYSPLASIA ?

  14. CYSTIC DISEASES OF THE KIDNEY POLYCYSTIC KIDNEY DISEASE(PKD) 2 TYPES 1. AN ADULT TYPE- AN AUTOSOMAL DOMINANT DISEASE 2.AN INFANTILE TYPE –AN AUTOSOMAL RECESSIVE DISORDER

  15. AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE INCIDENCE- ADPKD –COMMON ,1:500 TO 1:1000 GENETICALLY HETEROGENOUS

  16. AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE 90% - PKD1 GENE ON SHORT ARM OF CHROMOSOME 16 10% CASES-PKD 2 GENE ON CHROMOSOME 4. PKD 1 & PKD 2 GENES FORM HETERODIMERS-MUTATION OF EITHER GENE - ADPKD

  17. ADPKD OCCURS DUE TO MUTATION OF PKD GENES ALWAYS BILATERAL DIFFUSE EVENTHOUGH KIDNEYS ARE ABNORMAL AT BIRTH RENAL FUNCTION IS RETAINED TILL 3RD TO 5TH DECADE.

  18. MORPHOLOGY GROSS-- SYMMETRIC BILATERAL ENLARGED KIDNEYS. OUTER SURFACE – BOSSELATED CUT SURFACE SHOWS VARYING SIZED CYSTS RANGING FROM TINY TO 4- 5cms CLEAR TO BLOOD TINGED FLUID

  19. ADPKD

  20. MORPHOLOGY RENAL PELVIS & CALYCES ARE PRESENT BUT GREATLY DISTORTED CYSTS DO NOT COMMUNICATE WITH PELVIS OF THE KIDNEY. MICROSCOPY- CYSTS ARISE FROM ALL PARTS OF NEPHRON FROM BOWMAN’S CAPSULE TO COLLECTING DUCTS.

  21. HISTOLOGY NORMAL PARENCHYMA IS SEEN IN BETWEEN THE CYSTS. WITH ADVANCING AGE ACQUIRED LESIONS ARE DETECTED

  22. CLINICAL FEATURES DULL ACHING PAIN IN THE LUMBAR REGION WITH LARGE ABDOMINAL MASSES. OTHER PRESENTING COMPLAINTS ARE HAEMATURIA ,RENAL COLIC HYPERTENSION,URINARY TRACT INFECTION, PROGRESSIVE CHRONIC RENAL FAILURE.

  23. CLINICAL FEATURES 10 -30% OF CASES HAVE BERRY ANEURYSM LESS FREQUENTLY ASSOCIATED WITH CYSTS IN THE LUNG,SPLEEN,LIVER ,PANCREAS.

  24. INFANTILE POLYCYSTIC KIDNEY DISEASE AUTOSOMAL RECESSIVE CHILDHOOD POLYCYSTIC KIDNEY DISEASE(ARPKD)

  25. INFANTILE POLYCYSTIC KIDNEY DISEASE RARE, PERINATAL, NEONATAL,INFANTILE & JUVENILE SUBCATAGORIES.FIRST 2 ARE MORE COMMON. PRESENT AT BIRTH DIE QUICKLY FROM PULMONARY ? OR RENAL FAILURE EARLY IN CHILDHOOD.

  26. MORPHOLGY GROSS- BILATERAL,SPONGY WITH SMOOTH EXTERNAL SURFACE, NORMAL SHAPE. C/S - SMALL FUCIFORM OR CYLINDRICAL CYSTS FORMED FROM DILATATION OF THE COLLECTING DUCTS, EXTEND RADIALLY TO THE OUTER CORTEX.

  27. MORPHOLOGY OF ARPKD NO NORMAL RENAL PARENCHYMA IS SEEN PELVIS ,CALYCES & URETER ARE NORMAL

  28. ADULT & INFANTILE POLYCYSTIC KIDNEY DISEASE

  29. HISTOLOGY TOTAL NUMBER OF NEPHRONS ARE NORMAL ALL COLLECTING DUCTS SHOW CYLINDRICAL OR SACCULAR DILATATIONS & ARE LINED BY CUBOIDAL TO LOW COLUMNAR EPITHELIUM.

  30. CLINICAL FEATURES DEPENDS ON AGE OF THE CHILD IN SEVERE FORM BILATERAL CYSTIC RENAL ENLARGEMENT MAY INTERFERE WITH DELIVERY.

  31. CLINICAL FEATURES RENAL FAILURE MAY MANIFEST EARLY. ALMOST ALL CASES ARE ASSOCIATED WITH CYSTS IN THE LIVER

  32. CLINICAL FEATURES OLDER CHILDREN –ASSOCIATED CONGENITAL HEPATIC FIBROSIS WITH PORTAL HYPERTENSION & SPEENOMEGALY.

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