Answer to Pathology MCQ 155

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.

Category: Pathology MCQs