Inborn errors of steroid metabolism

An inborn error of steroid metabolism is an inborn error of metabolism due to defects in steroid metabolism.[citation needed] A variety of conditions of abnormal steroidogenesis exist due to genetic mutations in the steroidogenic enzymes involved in the process, of which include: In addition, several conditions of abnormal steroidogenesis due to genetic mutations in receptors, as opposed to enzymes, also exist, including:[citation needed] No activating mutations of the GnRH receptor in humans have been described in the medical literature,[3] and only one of the FSH receptor has been described, which presented as asymptomatic.[4][5]
SpecialtyMedical geneticsendocrinologyinborn error of metabolismsteroid metabolismconditionssteroidogenesisgenetic mutationssteroidogenic enzymes20,22-Desmolase (P450scc) deficiencysteroid hormonescholesterol3β-Hydroxysteroid dehydrogenase 2 deficiencyprogestogenandrogenestrogensglucocorticoidsmineralocorticoidsCombined 17α-hydroxylase/17,20-lyase deficiencyCytochrome P450 oxidoreductase deficiencysex steroidsmetabolic reactionsIsolated 17,20-lyase deficiencyCytochrome b5 deficiencymethemoglobinmethemoglobinemia17β-Hydroxysteroid dehydrogenase 3 deficiency5α-Reductase 2 deficiencytestosteronedihydrotestosteroneAromatase deficiencyAromatase excess21-Hydroxylase deficiency11β-Hydroxylase 1 deficiencyglucocorticoid deficiency11β-Hydroxylase 2 deficiency18-Hydroxylase deficiency18-Hydroxylase overactivityreceptorsGonadotropin-releasing hormone (GnRH) insensitivityFollicle-stimulating (FSH) hormone insensitivityLuteinizing hormone (LH) insensitivityLuteinizing hormone (LH) oversensitivitymedical literatureDisorders of sex developmentCongenital adrenal hyperplasiaAdrenal insufficiencyHypogonadismhypoandrogenismhypoestrogenismHypergonadismhyperandrogenismhyperestrogenismDelayed pubertyprecocious pubertyIntersexSteroid hormoneCorticosteroidglucocorticoidmineralocorticoidSex steroidestrogenNeuroactive steroidHypothalamuspituitary glandAdrenal cortextesticleHPA axisHPG axisMevalonatepathwayHMG-CoA lyase deficiencyHyper-IgD syndromeMevalonate kinase deficiency7-DehydrocholesterolHydrops-ectopic calcification-moth-eaten skeletal dysplasiaCHILD syndromeConradi–Hünermann syndromeLathosterolosisSmith–Lemli–Opitz syndromedesmosterolDesmosterolosisSteroidsaldosteroneGlucocorticoid remediable aldosteronismcortisolcortisoneCAH 17α-hydroxylaseCAH 11β-hydroxylaseCAH 3β-dehydrogenaseCAH 21-hydroxylaseApparent mineralocorticoid excess syndrome/11β-dehydrogenaseandrogens17α-Hydroxylase deficiency17,20-Lyase deficiency3β-Hydroxysteroid dehydrogenase deficiency17β-Hydroxysteroid dehydrogenase deficiencyAromatase excess syndromeX-linked ichthyosisAntley–Bixler syndromeAdrenal gland disorderHyperfunctionHyperaldosteronismPrimary aldosteronismConn syndromeBartter syndromeLiddle's syndrome17α CAHPseudohypoaldosteronismCushing's syndromePseudo-Cushing's syndromeSteroid-induced osteoporosisSex hormones21α CAH11β CAHHypoaldosteronismLipoidAdrenal crisisAdrenalitisXanthogranulomatousAddison's diseaseWaterhouse–Friderichsen syndrome