Lathosterolosis

Lathosterolosis is an inborn error of cholesterol biosynthesis caused by a deficiency of the enzyme 3-beta-hydroxysteroid-delta-5-desaturase.This leads to a flaw in the conversion of lathosterol to 7-dehydrocholesterol.Characteristics include facial dysmorphism, congenital malformations, failure to thrive, developmental delay, and liver illness.[2] Brunetti-Pierri et al. originally described Lathosterolosis in 2002.This article about an endocrine, nutritional, or metabolic disease is a stub.
LathosterolSymptomsFacial dysmorphismcongenital malformationsfailure to thrivedevelopmental delayliver illnessDifferential diagnosisSmith-Lemli-Opitz syndromeinborn error of cholesterol biosynthesis3-beta-hydroxysteroid-delta-5-desaturase7-dehydrocholesterolOrphanetInborn errors of steroid metabolismMevalonatepathwayHMG-CoA lyase deficiencyHyper-IgD syndromeMevalonate kinase deficiencycholesterolHydrops-ectopic calcification-moth-eaten skeletal dysplasiaCHILD syndromeConradi–Hünermann syndromeSmith–Lemli–Opitz syndromedesmosterolDesmosterolosisSteroidsCorticosteroidaldosteroneGlucocorticoid remediable aldosteronismcortisolcortisoneCAH 17α-hydroxylaseCAH 11β-hydroxylaseCAH 3β-dehydrogenaseCAH 21-hydroxylaseApparent mineralocorticoid excess syndrome/11β-dehydrogenaseSex steroidandrogens17α-Hydroxylase deficiency17,20-Lyase deficiencyCytochrome b5 deficiency3β-Hydroxysteroid dehydrogenase deficiency17β-Hydroxysteroid dehydrogenase deficiency5α-reductase 2 deficiencyestrogensAromatase deficiencyAromatase excess syndromeX-linked ichthyosisAntley–Bixler syndrome