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Pathology Glomerular Diseases 17c14e4f

Glomerulonephritis associated sensory neuronal deafness is seen in -

A
Nail patella syndrome
B
Alport syndrome
C
Down syndrome
D
Fabry's disease
High-Yield Explanation
Ans. is 'b' i.e., Alport's syndromeAlport's syndromeo Alport's syndrome is a type of hereditary nephritis characterized bvGlomerulonephritis progressing to chronic renal failure.Nerve deafnessiii) Eye defects - lenticonus, lens dislocation, posterior cataract, corneal dystrophy.o Most commonly it is inherited asX-linked form.o Rare autosomal - recessive and autosomal-dominant pedigrees also exist.Pathogenesiso There is defective GBM synthesis because of production of abnormal collagen type IV underlies the renal lesions,o The defect is caused by mutation in the gene encoding a-chain of collagen type IV.

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