Diaphanospondylodysostosis

Alternative Names

  • Vertebral Ossification, Defect in, with Nephrogenic Rests
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Congenital malformations and deformations of the musculoskeletal system

OMIM Number

608022

Mode of Inheritance

Autosomal recessive

Gene Map Locus

7p14.3

Description

Diaphanospondylodysostosis is a rare, recessively inherited, perinatal lethal skeletal disorder. The primary skeletal characteristics include small chest, abnormal vertebral segmentation, and posterior rib gaps containing incompletely differentiated mesenchymal tissue. Consistent craniofacial features include ocular hypertelorism, epicanthal folds, depressed nasal bridge with short nose, and low-set ears. The most commonly described extraskeletal finding is nephroblastomatosis with cystic kidneys, but other visceral findings have been described in some cases. [From OMIM]

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
608022.1Saudi ArabiaFemaleYes Abnormal vertebral morphologyNM_133468.5:c.712T>GHomozygousAutosomal, RecessiveMonies et al. 2017
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