Literature DB >> 16677980

Sudden death in a young woman from medium chain acyl-coenzyme A dehydrogenase (MCAD) deficiency.

Ginger W Wilhelm1.   

Abstract

Medium chain acyl-coenzyme A dehydrogenase (MCAD) deficiency is an inherited disorder of fatty acid metabolism that usually presents in early childhood. This case report describes a 19-year-old woman who presented with lethargy, disorientation, and vomiting. She had a cardiopulmonary arrest from which she could not be resuscitated 24h after the onset of the illness. Pre-mortem blood studies confirmed MCAD deficiency. An MCAD deficiency and other metabolic disorders lie within the differential diagnosis of a patient presenting with acutely altered mental status. The inheritance of MCAD deficiency and its clinical presentation, pathophysiology, treatment, and prevention are discussed.

Entities:  

Mesh:

Substances:

Year:  2006        PMID: 16677980     DOI: 10.1016/j.jemermed.2005.05.030

Source DB:  PubMed          Journal:  J Emerg Med        ISSN: 0736-4679            Impact factor:   1.484


  5 in total

Review 1.  The clinical manifestation of MCAD deficiency: challenges towards adulthood in the screened population.

Authors:  Ulrich A Schatz; Regina Ensenauer
Journal:  J Inherit Metab Dis       Date:  2010-06-08       Impact factor: 4.982

Review 2.  Adult presentations of medium-chain acyl-CoA dehydrogenase deficiency (MCADD).

Authors:  T F Lang
Journal:  J Inherit Metab Dis       Date:  2009-10-11       Impact factor: 4.982

Review 3.  Fatty acid oxidation disorders: outcome and long-term prognosis.

Authors:  Bridget Wilcken
Journal:  J Inherit Metab Dis       Date:  2010-01-05       Impact factor: 4.982

4.  Medium-chain acyl-CoA dehydrogenase deficiency associated with a novel splice mutation in the ACADM gene missed by newborn screening.

Authors:  Sarah C Grünert; A Wehrle; P Villavicencio-Lorini; E Lausch; B Vetter; K O Schwab; S Tucci; U Spiekerkoetter
Journal:  BMC Med Genet       Date:  2015-07-30       Impact factor: 2.103

5.  Inherited Metabolic Diseases and Cardiac Pathology in Adults: Diagnosis and Prevalence in a CardioMetabo Study.

Authors:  Marina Brailova; Guillaume Clerfond; Romain Trésorier; Régine Minet-Quinard; Julie Durif; Grégoire Massoullié; Bruno Pereira; Vincent Sapin; Romain Eschalier; Damien Bouvier
Journal:  J Clin Med       Date:  2020-03-04       Impact factor: 4.241

  5 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.