Wolman syndrome

Patients with this autosomal recessive mutation are deficient in the lysosomal acid lipase that hydrolyzes cholesteryl esters and triglycerides derived from plasma lipoproteins [106]. This deficiency results in the accumulation of cholesteryl esters in the lysosomes of all body tissues [16,107]. Wolman syndrome patients usually do not survive the first year of life. 

Cholesteryl ester storage disease is a less severe form of Wolman syndrome in which a residual amount (1-5%) of lysosomal acid lipase activity is expressed [108,109]. Patients with this disease may survive until young adulthood. 

Amyloidosis, fatty liver, glycogenoses, Wolman s syndrome, hyperchylomicronaemia, Wilson s disease, Zellweger s cerebrohepato renal syndrome, Niemann-Pick disease, mucopolysaccharidoses, etc. 

Wilson s disease Wolman s disease Zellweger syndrome... 

Dickinson EJ, Wolman RL. Sicca syndrome associated with amiodarone therapy. BMJ (CUn Res Ed) 1986 293 510. 

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