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Kidney

Congenital anomalies1. Renal Dysplasias and Hypoplasias = 20% of CRF in children2. Autosomal-dominant PKD= Adults, 10% of CRF in adults3. Hypoplasia= failure of kidney development, no scars If bilateral? childhood renal failure4. OligoMegaNephronia= small kidney with hypertrophied Nephrons5.

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Kidney

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    1. Kidney

    2. Congenital anomalies 1. Renal Dysplasias and Hypoplasias = 20% of CRF in children 2. Autosomal-dominant PKD= Adults, 10% of CRF in adults 3. Hypoplasia= failure of kidney development, no scars If bilateral? childhood renal failure 4. OligoMegaNephronia= small kidney with hypertrophied Nephrons 5. Ectopic Kidneys= At low levels (above pelvic brim or within pelvis) Causes kinking or tortuosity of the ureters? obstruction to urinary flow? infections 6.Renal Agenesis = complete absence If bilateral? incompatible with life (Stillborn babies) If Unilateral ?associated with limb defects, hypoplastic lungs, normal life but compensatory hypertrophy of other kidney and CRF in some patients 7. Horseshoe Kidneys= Fusion poles (MC in lower pole – 90%) Causes kinking and torsion of Ureters

    3. Cystic Diseases =Hereditary or Nonhereditary, developmental or acquired ,Confused with malignant tumors, Cause CRF Cystic renal dysplasia Polycystic kidney disease (PKD)= HTN, Berry aneurysms, Autosomal-dominant (adult) types (ADPKD1 & ADKKD 2) Autosomal-recessive (childhood) -ARPKD Medullary cystic disease Medullary sponge kidney Nephronophthesis Acquired= dialysis-associated) cystic disease 5. Localized (simple) renal cysts 6. Renal cysts in hereditary malformation syndromes (Tuberous sclerosis) 7. Glomerulocystic disease 8. Extraparenchymal renal cysts (pyelocalyceal cysts, hilar lymphangitic cysts) 1. CYSTIC RENAL DYSPLASIA = Abnormal metanephric differentiation may cause ureteropelvic obstruction, ureteral agenesis or atresia

    4. Renal dysplasia

    5. Cystic Diseases 2(a). ADPKD = multiple cysts of both kidneys ,AD with high penetrance universally Bilateral, Basic defect - disruption in the regulation of intracellular Ca2+ levels Gross appearance= Bilaterally enlarged kidneys (4 kg each) Microscopic examination = Functioning Nephrons Prognosis= Rapid Progression in dark skinned, males, HTN pts. Extra-renal congenital anomalies= Cysts (spleen, pancreas, lungs, liver) & Berry aneurysms Death is due to = CAD or HTN (40%), Infections ( 25%), Subarachnoid hemorrhage from ruptured berry aneurysm (15% )

    6. ADPKD PKD1 gene chromosome16p13.3 MC -80% Cases Most severe Early ESRD or Death (53 yrs.) Gene encodes membrane protein polycystin-1 localized to tubular epithelial cells PKD2 gene 4q21 Less Common Less severe Late onset ESRD or Death (69 yrs.) polycystin-2 localized to all segments of the renal tubules & extra-renal tissues.

    7. Cystic Diseases 2(b). ARPKD = Perinatal, Neonatal (MC), infantile, and juvenile subcategories Genetically homogeneous, Chromosome – 6? Gene- PKHD1 ?Protein- Fibrocystin (expressed in adult and fetal kidney and also in liver & pancreas) Gross= Bilateral Enlarged kidneys with Smooth external appearance Cut section= Numerous small cysts in cortex and medulla, Sponge like Kidney ,Dilated elongated collecting tubules at right angles to the cortical surface Congenital hepatic fibrosis = periportal fibrosis with proliferation of biliary ducts Seen in ARPKD

    8. ADPKD & ARPKD ADPKD AD Irregular Flattened Liver cysts (40%) ARPKD AR Smooth Cuboidal Liver cysts (100%)

    9. ADPKD & ARPKD ADPKD PKD 1,2 Polycystin -1 ,2 16 , 4 Occasional ARPKD PKHD 1 Fibrocystin 6 Frequent (mainly in infantile & Juvenile forms)

    10. ADPKD & ARPKD

    11. Cystic Diseases 3. Medullary cystic disease =(Medullary sponge kidney &Nephronophthesis) Medullary sponge kidney= incidental finding in adults with normal renal function Nephronophthesis =MC genetic cause of ESRD in children and young adults Mc type Familial juvenile Nephronophthesis (40-50%), - AR present first with Polyuria and polydipsia Sodium wasting and tubular acidosis progression to ESRD during a period of 5 to 10 years Disease is strongly considered in children or adolescents with unexplained CRF Positive family history Chronic Tubulointerstitial nephritis on biopsy

    12. Medullary sponge kidney & Nephronophthesis Medullary sponge kidney common Adults Incidental No Not known medulla Nephronophthesis Less common Children Symptomatic Yes Genetic (AD, AR) CM junction

    13. Medullary cystic disease

    14. Cystic Diseases 4. Acquired (dialysis-associated) cystic disease Numerous cortical and Medullary cysts contain calcium oxalate crystals Most ominous complication is Renal cell carcinoma -7% in 10 years 5. Simple cysts = common postmortem findings without clinical significance

    15. The worst thing (in one’s life) is self cheating The best thing (in one’s life) is self realization Krishna T

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