Conradi–Hünermann syndrome

Possible signs and symptoms may include[citation needed] Conradi–Hünermann syndrome is a form of chondrodysplasia punctata, a group of rare genetic disorders of skeletal development involving abnormal accumulations of calcium salts within the growing ends of long bones.While evidence suggests that Conradi–Hünermann syndrome predominantly occurs in females and is usually inherited as an X-linked dominant trait, rare cases in which males were affected have also been reported.[citation needed] The genetics of Conradi–Hünermann syndrome have perplexed medical geneticists, pediatricians and dermatologists for some time, but a number of perplexing features of the genetics of the syndrome have now been resolved, including the fact that the disease is caused by mutations in a gene, and these mutations are simple substitutions, deletions or insertions and are therefore not "unstable".[citation needed] An important test to confirm a diagnosis of Conradi–Hünermann syndrome is evaluating the plasma for elevated levels of a substance known as sterols.Most female patients with the syndrome can live a long and normal life, while males have only survived in rare cases.
SpecialtyMedical geneticschondrodysplasia punctataEBP genescoliosiskyphosisredness of the skinalopeciaIchthyosisgenetic disordersintellectual disabilityX-linked dominantgeneticistspediatriciansdermatologistsmutationssterolsgas chromatographymass spectrometryFetal warfarin syndromeList of cutaneous conditionsList of radiographic findings associated with cutaneous conditionsDiseasesDBSNOMED CTOsteochondrodysplasiasOsteodysplasiaosteodystrophyDiaphysisCamurati–Engelmann diseaseMetaphysisMetaphyseal dysplasiaJansen's metaphyseal chondrodysplasiaSchmid metaphyseal chondrodysplasiaEpiphysisSpondyloepiphyseal dysplasia congenitaMultiple epiphyseal dysplasiaOtospondylomegaepiphyseal dysplasiaOsteosclerosisRaine syndromeOsteopoikilosisOsteopetrosisBoomerang dysplasiaOpsismodysplasiaPolyostotic fibrous dysplasiaMcCune–Albright syndromeChondrodysplasiachondrodystrophydwarfismOsteochondromaosteochondromatosisHereditary multiple exostosesChondromaenchondromaenchondromatosisOllier diseaseMaffucci syndromeGrowth factor receptorAntley–Bixler syndromeAchondroplasiaHypochondroplasiaThanatophoric dysplasiaCOL2A1collagen diseaseAchondrogenesistype 2HypochondrogenesisSLC26A2type 1BAutosomal recessive multiple epiphyseal dysplasiaAtelosteogenesis, type IIDiastrophic dysplasiaRhizomelic chondrodysplasia punctataFibrochondrogenesisShort rib – polydactyly syndromeMajewski's polydactyly syndromeLéri–Weill dyschondrosteosisX-linkedX-linked recessiveImmuneChronic granulomatous disease (CYBB)Wiskott–Aldrich syndromeX-linked severe combined immunodeficiencyX-linked agammaglobulinemiaHyper-IgM syndrome type 1X-linked lymphoproliferative diseaseProperdin deficiencyHematologicHaemophilia AHaemophilia BX-linked sideroblastic anemiaEndocrineAndrogen insensitivity syndromeSpinal and bulbar muscular atrophyKAL1 Kallmann syndromeX-linked adrenal hypoplasia congenitaMetabolicAmino acidOrnithine transcarbamylase deficiencyOculocerebrorenal syndromeDyslipidemiaAdrenoleukodystrophyCarbohydrate metabolismGlucose-6-phosphate dehydrogenase deficiencyPyruvate dehydrogenase deficiencyDanon disease/glycogen storage disease Type IIbLipid storage disorderFabry diseaseMucopolysaccharidosisHunter syndromePurine–pyrimidine metabolismLesch–Nyhan syndromeMineralMenkes diseaseOccipital horn syndromeNervous systemX-linked intellectual disabilityCoffin–Lowry syndromeMASA syndromeAlpha-thalassemia mental retardation syndromeOcular albinismNorrie diseaseChoroideremiaCharcot–Marie–Tooth disease (CMTX2-3)Pelizaeus–Merzbacher diseaseDyskeratosis congenitaHypohidrotic ectodermal dysplasia (EDA)X-linked ichthyosisX-linked endothelial corneal dystrophyNeuromuscularBecker muscular dystrophyDuchenneCentronuclear myopathy (MTM1)Emery–Dreifuss muscular dystrophy 1UrologicAlport syndromeDent's diseaseX-linked nephrogenic diabetes insipidusAMELX Amelogenesis imperfectaBarth syndromeMcLeod syndromeSmith–Fineman–Myers syndromeSimpson–Golabi–Behmel syndromeMohr–Tranebjærg syndromeNasodigitoacoustic syndromeX-linked hypophosphatemiaFocal dermal hypoplasiaFragile X syndromeAicardi syndromeIncontinentia pigmentiRett syndromeCHILD syndromeLujan–Fryns syndromeOrofaciodigital syndrome 1Craniofrontonasal dysplasiaInborn errors of steroid metabolismMevalonatepathwayHMG-CoA lyase deficiencyHyper-IgD syndromeMevalonate kinase deficiencycholesterol7-DehydrocholesterolHydrops-ectopic calcification-moth-eaten skeletal dysplasiaLathosterolosisSmith–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 syndrome