Answer to Pathology MCQ 155

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The Correct option is B

Fanconi Syndrome (Toni-Debre-Fanconi Syndrome)

Fanconi syndrome is a generalized disorder of the PT. The defect can lead to glycosuria, aminoaciduria, phosphaturia, bicarbonaturia and excessive urinary loss of K+, Na+, Ca++, Mg++, uric acid and other organic acids.

It appears that the common denominator in most of the inherited forms is that a toxin builds up in the tubular cells (e.g. cystinosis--cystine in lysosomes, Wilson’s disease-copper). In the idiopathic forms, the presumed tubular toxin is unknown. Because of the number of different transport mechanisms involved in Fanconi syndrome, it is likely that individual transporters themselves are affected. It is speculated that a generalized membrane defect exists (permeability theory), or alternatively, there is a defect in intracellular energy production or utilization (energy theory). In experimentally induced Fanconi syndrome (maleic acid), decreased intracellular ATP has been noted.

Clinical signs of Fanconi syndrome include polyuria and those secondary to electrolyte and bicarbonate losses. In children growth retardation and rickets may develop. Idiopathic Fanconi syndrome often progresses to chronic renal failure.

Category: Pathology MCQs



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