Smith-Strang disease


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Smith-Strang disease

 [smith´ strāng]
a methionine malabsorption syndrome that results in a characteristic smell of the urine; individuals with this condition have white hair and varying degrees of mental retardation.

methionine malabsorption syndrome

An autosomal recessive condition (OMIM:250900) characterised by albinism, hyperpnoea, convulsions and mental retardation after methionine loading, with increased alpha-hydroxybutyric acid, which causes the oasthouse odour.

Smith-Strang disease

(smith'strang')
[Allan J. Smith, contemporary Brit. physician; Leonard B. Strang, Brit. physician, b. 1925]
Methionine malabsorption syndrome.

Smith,

Allan J., 20th century English physician.
Smith-Strang disease - autosomal recessive disease with onset of loss of response to stimuli, mental deterioration, edema, and white hair shortly after birth. Synonym(s): methionine malabsorption syndrome; oasthouse urine disease

Strang,

Leonard Birnie, English physician, 1925–.
Smith-Strang disease - see under Smith, Allan J