Page 478 - Concise Pathology for Exam Preparation ( PDFDrive )

16 Diseases of the Kidney and Lower Urinary Tract 463

Q. Enumerate the cystic lesions of kidney.

Ans. Cysts of the kidney include
• Polycystic kidney (adult and infantile type)
• Medullary cystic disease (medullary sponge kidney and nephronophthisis)
• Localized or simple renal cyst
• Multicystic renal dysplasia
• Acquired (dialysis-associated) cystic disease
• Renal cysts associated with tuberous sclerosis
• Calyceal or pyelogenic cyst
• Pelvic cyst
• Perinephric cyst
• Cystic degeneration in tumours

Q. Differentiate between adult and childhood polycystic kidney
disease.
Ans. Differences between adult and childhood polycystic kidney disease are tabulated in
Table 16.2.

TABLE 16.2. Differences between adult and childhood polycystic kidney disease
Features Adult Childhood
Inheritance Autosomal dominant, caused by a mutation Autosomal recessive with a mutation in the
in the genes encoding polycystin 1 and 2. gene encoding fibrocystin, ie, PKHD1
Defective gene is PKD1 or PKD2
Frequency More common Less common
Presentation • Presents after fourth decade; may be asso- • Presents in perinatal/neonatal age group
ciated with a cystic liver, berry aneurysm, with splenomegaly and hepatic fibrosis
subarachnoid haemorrhage, colonic diver- (oesophageal varices may be seen as a
ticulii and mitral valve prolapse consequence of hepatic fibrosis)
• Large, multicystic kidney • External surface of the kidney is smooth;
cut surface shows numerous small cysts in
the cortex and medulla (dilated elongated
channels at right angles to the cortical
surface)
Origin of cysts May arise from any level of nephron from Arises from collecting ducts; lined uniformly
tubules to collecting ducts; lining variable cuboidal cells
Clinical features Haematuria, flank pain, hypertension and Bilateral abdominal mass
urinary infection
Outcome Chronic renal failure begins at age of 40–60 • Young infants usually die of hepatic and
years renal failure
• Patients who survive develop congenital
hepatic fibrosis

Q. Enumerate the various glomerular syndromes.
Ans. Based on clinical manifestations, renal diseases are classified into the following
major glomerular syndromes:
1. Acute nephritic syndrome: Haematuria, azotaemia, variable proteinuria, oliguria,
oedema and hypertension
2. Rapidly progressing glomerulonephritis (RPGN): Acute nephritis, proteinuria and
acute renal failure
3. Nephrotic syndrome: Proteinuria . 3.5 g/day, hypoalbuminaemia, hyperlipidaemia
and lipiduria
4. Chronic renal failure (CRF): Azotaemia and uraemia progressing over years
5. Asymptomatic haematuria/proteinuria: Haematuria and subnephrotic proteinuria

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