Literature DB >> 19450972

Management and communication problems in a patient with succinyl-CoA transferase deficiency in pregnancy and labour.

S Merron1, R Akhtar.   

Abstract

Succinyl-CoA transferase deficiency is a high-risk condition that pre-disposes the sufferer to severe and life-threatening ketoacidosis. An 18-year-old woman with succinyl-CoA transferase deficiency was admitted to the delivery suite for induction of labour at 38 weeks of gestation. Her management included adequate calorie intake in order to avoid fatty acid metabolism and adequate hydration along with rigorous electrolyte balance and minimisation of physiological stress by the use of epidural analgesia. The needs of the woman's condition had to be balanced against the desire to minimise gastric volume in case emergency obstetric intervention was required.

Entities:  

Mesh:

Substances:

Year:  2009        PMID: 19450972     DOI: 10.1016/j.ijoa.2009.02.001

Source DB:  PubMed          Journal:  Int J Obstet Anesth        ISSN: 0959-289X            Impact factor:   2.603


  5 in total

1.  Successful Management of Pregnancies in Patients with Inherited Disorders of Ketone Body Metabolism.

Authors:  Raashda Ainuddin Sulaiman; Maha Al-Nemer; Rubina Khan; Munirah Almasned; Bedour S Handoum; Zuhair N Al-Hassnan
Journal:  JIMD Rep       Date:  2017-05-10

2.  Heterozygous carriers of succinyl-CoA:3-oxoacid CoA transferase deficiency can develop severe ketoacidosis.

Authors:  Hideo Sasai; Yuka Aoyama; Hiroki Otsuka; Elsayed Abdelkreem; Yasuhiro Naiki; Mitsuru Kubota; Yuji Sekine; Masatsune Itoh; Mina Nakama; Hidenori Ohnishi; Ryoji Fujiki; Osamu Ohara; Toshiyuki Fukao
Journal:  J Inherit Metab Dis       Date:  2017-07-10       Impact factor: 4.982

3.  A neonatal-onset succinyl-CoA:3-ketoacid CoA transferase (SCOT)-deficient patient with T435N and c.658-666dupAACGTGATT p.N220_I222dup mutations in the OXCT1 gene.

Authors:  Toshiyuki Fukao; Tomohiro Ishii; Naoko Amano; Petri Kursula; Masaki Takayanagi; Keiko Murase; Naomi Sakaguchi; Naomi Kondo; Tomonobu Hasegawa
Journal:  J Inherit Metab Dis       Date:  2010-07-21       Impact factor: 4.982

4.  A Case of Succinyl-CoA:3-Oxoacid CoA Transferase Deficiency Presenting with Severe Acidosis in a 14-Month-Old Female: Evidence for Pathogenicity of a Point Mutation in the OXCT1 Gene.

Authors:  Daniel J Zheng; Michael Hooper; Michele Spencer-Manzon; Richard W Pierce
Journal:  J Pediatr Intensive Care       Date:  2017-07-19

Review 5.  Ketone body metabolism and its defects.

Authors:  Toshiyuki Fukao; Grant Mitchell; Jörn Oliver Sass; Tomohiro Hori; Kenji Orii; Yuka Aoyama
Journal:  J Inherit Metab Dis       Date:  2014-04-08       Impact factor: 4.982

  5 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.