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CYSTIC DISEASE OF KIDNEY

CYSTIC DISEASE OF KIDNEY. Classification of renal cyst Adult polycystic disease ( Autosomal dominant disease) Childhood polycystic disease ( Autosomal recessive disease)

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CYSTIC DISEASE OF KIDNEY

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  1. CYSTIC DISEASE OF KIDNEY Dr S Chakradhar

  2. Classification of renal cyst • Adult polycystic disease (Autosomal dominant disease) • Childhood polycystic disease (Autosomal recessive disease) • Cystic renal dysplasia - Disorganized development of kidney characterized by cyst (primitive glomeruli, tubules & mesenchyme) • Medullary cystic disease - limited to medullary pyramids and papillae. • Acquired cystic disease – dialysis associated / uraemic • Simple cyst - Non neoplastic cyst lined by single cuboidal or flattened epithelium Dr S Chakradhar

  3. ADULT POLYCYSTIC KIDNEY DISEASE (APCKD) • Autosomal dominant Pathological defect thought to be due to: Results from mutations in either the PKD-1 or PKD-2 gene. • Abnormally weak basement membrane leading to segmental distention of tubule or vessel and cyst formation • Proliferation of tubular epithelium Dr S Chakradhar

  4. Abnormal basement membrane also predisposes to cyst formation in other organs • liver - 33% • cerebral artery aneurysm - 10% • other associations: Diverticulosis and Mitral valve prolapse • less common: pancreas, spleen, thyroid, ovary, endothelium, seminal vesicles, and aorta Dr S Chakradhar

  5. Clinical features: • Polycystic changes are bilateral and present any time from early childhood to as late as 80 years of age • The kidneys are normal at birth, symptoms are rare before 20Years • kidneys may enlarge to 10 times normal volume ❏ symptoms • Often asymptomatic; discovered incidentally on imaging • Acute abdominal pain/lumbar pain • Hematuria , UTI • Rarely extra-renal presentation (e.g. Ruptured berry aneurysm) ❏ signs • HTN (up to 75% of adults) • ± Palpable kidneys Dr S Chakradhar

  6. Investigations : • BUN, Creatinine : To assess for progressive renal failure • Urine RME: To assess for Hematuria • Abdomen USG (best modality): Enlarged kidneys, multiple cysts throughout renal parenchyma, lobulated kdney • CT abdomen with contrast Dr S Chakradhar

  7. Diagnosis: • Positive family history • Ultrasound: cysts are usually detectable by age 20 • Differential diagnosis: Multiple simple cysts (not progressive like APCKD) Dr S Chakradhar

  8. Complications: • UTI • Infected cysts most common in women due to ascending infection • Focal compression of intra-renal arteries by cysts increased reninproduction  HTN • If untreated will ACCELERATE progression to ESRD • Nephrolithiasisin 5-15% of APCKD (may form due to poor drainage from distorted calyceal system)usually Uratestones Dr S Chakradhar

  9. Management Goal: to preserve renal function: • Must treat UTI early • Screen for HTN, treat aggressively with Antihypertensives (e.g. ACE inhibitors) • Adequate hydration to prevent stone formation • Instrumentation of the Genito Urinary tract should be avoided • Should avoid contact sports due to greater risk of injury if kidneys are large • As ESRD develops, treat with renal replacement therapy • Screen for cerebral aneurysms • Must provide genetic counselling: 50% chance of transmission by affected parents Dr S Chakradhar

  10. CYSTIC DISEASE of Renal Medulla MEDULLARY CYSTIC DISEASE • Rare Autosomal recessive disorder Pathologically • Cysts restricted to the renal medulla or corticomedullary border, as well as a triad of • Tubular atrophy, tubular basement membrane disintegration, and interstitial fibrosis • Often results in ESRD during Adolescence/Childhood Cysts difficult to image due to small size Dr S Chakradhar

  11. MEDULLARY SPONGE KIDNEY • Multiple cystic dilatations in the papillary collecting ducts (medulla) • Presents in the 4th to 6th decades usually as passage of renal stones • Benign with respect to the development of renal insufficiency • Increased incidence of renal calculi, infections, and HTN Nephrocalcinosis may be seen on X-ray, Medullary sponge defect seen on IVP Dr S Chakradhar

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