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ketoacidotic during pregnancy

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https://www.readbyqxmd.com/read/24706027/ketone-body-metabolism-and-its-defects
#1
REVIEW
Toshiyuki Fukao, Grant Mitchell, Jörn Oliver Sass, Tomohiro Hori, Kenji Orii, Yuka Aoyama
Acetoacetate (AcAc) and 3-hydroxybutyrate (3HB), the two main ketone bodies of humans, are important vectors of energy transport from the liver to extrahepatic tissues, especially during fasting, when glucose supply is low. Blood total ketone body (TKB) levels should be evaluated in the context of clinical history, such as fasting time and ketogenic stresses. Blood TKB should also be evaluated in parallel with blood glucose and free fatty acids (FFA). The FFA/TKB ratio is especially useful for evaluation of ketone body metabolism...
July 2014: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/23430882/three-japanese-patients-with-beta-ketothiolase-deficiency-who-share-a-mutation-c-431a-c-h144p-in-acat1-subtle-abnormality-in-urinary-organic-acid-analysis-and-blood-acylcarnitine-analysis-using-tandem-mass-spectrometry
#2
Toshiyuki Fukao, Shinsuke Maruyama, Toshihiro Ohura, Yuki Hasegawa, Mitsuo Toyoshima, Antti M Haapalainen, Naomi Kuwada, Mari Imamura, Isao Yuasa, Rik K Wierenga, Seiji Yamaguchi, Naomi Kondo
Mitochondrial acetoacetyl-CoA thiolase (T2) deficiency affects both isoleucine catabolism and ketone body metabolism. The disorder is characterized by intermittent ketoacidotic episodes. We report three Japanese patients. One patient (GK69) experienced two ketoacidotic episodes at the age of 9 months and 3 years, and no further episodes until the age of 25 years. She had two uncomplicated pregnancies. GK69 was a compound heterozygote of the c.431A>C (H144P) and c.1168T>C (S390P) mutations in T2 (ACAT1) gene...
2012: JIMD Reports
https://www.readbyqxmd.com/read/1852536/accumulation-of-odd-numbered-long-chain-fatty-acids-in-fetuses-and-neonates-with-inherited-disorders-of-propionate-metabolism
#3
U Wendel, R Baumgartner, S B van der Meer, L J Spaapen
Fetuses affected with propionic acidemia incorporate great amounts of odd-numbered long-chain fatty acids (OLCFA) into their body lipids. This is due to abundant supply with precursor amino acids of propionyl-CoA throughout pregnancy. After birth, the lower provision of precursor amino acids during dietary treatment compared with fetal life results in a decline of propionyl-CoA production and therefore OLCFA synthesis. However, the observed decrease of OLCFA may also partly reflect the recovery from acute ketoacidotic episodes that the patients experienced soon after birth as long as they were undiagnosed...
April 1991: Pediatric Research
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