If you find the material on this website useful, you will find that the two books ACES for PACES and KEYS to SUCCESS in Medicine complement the material on these sites and will enhance your studying and revision

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ACES for PACES

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BOF 53
Cystinosis, all are true except
1-Cystine accumulates in the kidneys
2-it is an autosomal recessive disease due to failure of cystine export out of lysosomes
3-renal stones are common
4-Fanconi syndrome is seen
5-cysteamine is effective at reducing cystine accumulation but unfortunately unpleasant

Answer 3
1-and reticuloendothelial and other tissues
2-causing wide spread intra-lysosomal accumulation of cystine
3-false. Unlike CYSTINURIA, no stones
4-true with severe hypophosphataemic rickets
5- And rarely tolerated

Revision Tip

Revise cystinosis KEYS to SUCCESS in Medicine page 64

 

 

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