"Abderhalden-Kaufmann-Lignac syndrome"
"cystinosis"
-Def:- it is an autosomal recessive disorder involving deposits of cystine crystals in lysosomes of liver,spleen,bone marrow,cornea, and renal tubules due to abnormal metabolism of cystine ."cystinosis"
-Incidence :- 1 : 100,000- 1 :200,000.
-It is the most common cause of Fanconi syndrome in the pediatric age group.
-Clinical picture:-
