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4 Defects in cholesterol biosynthesis,dentified in the last decade. One of these, the Smith-Lemli-Opitz syndrome is fairly common with an incidence of perhaps 1 in 20,000. Defects in 3.-hydroxysteroid Δ.-reductase are responsible for this syndrome. In severely affected SLO patients, cholesterol levels are 10 mg/dl or lower. Administrati