Literature DB >> 19078586

Duchenne muscular dystrophy and glycerol kinase deficiency: a rare contiguous gene syndrome.

A A Amato1.   

Abstract

Duchenne muscular dystrophy (DMD), glycerol kinase deficiency (GKD), and adrenal hypoplasia congenita (AHC) can occur together as part of a contiguous gene syndrome located at chromosome Xp21, GKD can manifest with recurrent episodes of vomiting, acidemia, mental retardation, or stupor. Involvement of the AHC gene can produce life-threatening adrenal insufficiency, sexual ambiguity, and electrolyte abnormalities. These associated conditions can make the diagnosis of DMD difficult. Neuromuscular specialists need to be aware of this contiguous gene syndrome because the potential life-threatening complications of GKD and AHC can be treated.

Entities:  

Year:  2000        PMID: 19078586     DOI: 10.1097/00131402-200006000-00006

Source DB:  PubMed          Journal:  J Clin Neuromuscul Dis        ISSN: 1522-0443


  1 in total

1.  Case Report: A Novel Truncating Variant of NR0B1 Presented With X-Linked Late-Onset Adrenal Hypoplasia Congenita With Hypogonadotropic Hypogonadism.

Authors:  Feng Zhu; Min Zhou; Xiuling Deng; Yujuan Li; Jing Xiong
Journal:  Front Endocrinol (Lausanne)       Date:  2022-06-16       Impact factor: 6.055

  1 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.