Acute fatty liver of pregnancy and long‐chain 3‐hydroxyacyl–coenzyme A dehydrogenase deficiency
Acute fatty liver of pregnancy and long‐chain 3‐hydroxyacyl–coenzyme A dehydrogenase deficiency
复制标题
妊娠期急性脂肪肝与长链3-羟酰辅酶A脱氢酶缺乏症
作者：
W. Treem;P. Rinaldo;D. Hale;C. Stanley;D. Millington;J. Hyams;S. Jackson;D. Turnbull
急性妊娠脂肪肝的发病机制尚不清楚，但其临床表现和肝脏组织学表现与肝脏线粒体内β氧化途径代谢缺陷的儿童相似，表明肝脏脂肪酸氧化障碍可能起作用。我们报告了一名患有急性妊娠脂肪肝的妇女，她生下了一个看似正常的足月婴儿，在4个月大时出现低血糖、昏迷和严重的肝脂肪变性。婴儿有脂肪酸氧化缺陷，长链3 -羟基酰基辅酶a脱氢酶缺乏症，母亲被证明是杂合的。我们假设，受影响的胎儿与女性杂合子的相互作用在晚期晚期的脂肪酸氧化缺陷解释了一些急性脂肪肝的妊娠病例。(肝脏病学19:339 1994;345)。
The pathogenesis of acute fatty liver of pregnancy is unknown, but similarities in the clinical presentation and the histological appearance of the liver with those found in children with metabolic defects in the intra‐mitochondrial β‐oxidation pathway of the liver suggest that a disturbance in hepatic fatty acid oxidation may play a role. We report a woman with acute fatty liver of pregnancy who gave birth to a seemingly normal full‐term infant who was seen at 4 mo of age with hypoglycemia, coma and profound hepatic steatosis. The infant had a defect in fatty acid oxidation, long‐chain 3‐hydroxyacyl–coenzyme A dehydrogenase deficiency, and the mother proved to be heterozygous for this metabolic condition. We hypothesize that the interaction of an affected fetus with a female heterozygous for this defect in fatty acid oxidation in the late third trimester accounts for some cases of acute fatty liver of pregnancy. (Hepatology 1994;19:339–345).
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影响因子：
39.2
作者：
Riely,CA;Latham,PS;Romero,R;Duffy,TP
通讯作者：
Duffy,TP
影响因子：
15.9
作者：
TONSGARD, JH;GETZ, GS
通讯作者：
GETZ, GS
L-3-羟酰辅酶A脱氢酶缺乏症。
DOI：
--
发表时间：
1990
期刊：
Progress in clinical and biological research
影响因子：
--
作者：
Hale,DE;Thorpe,C;Braat,K;Wright,JH;Roe,CR;Coates,PM;Hashimoto,T;Glasgow,AM
通讯作者：
Glasgow,AM
串联质谱在脂肪酸氧化紊乱诊断中的作用。
DOI：
--
发表时间：
1992
期刊：
Progress in clinical and biological research
影响因子：
--
作者：
Millington,DS;Terada,N;Chace,DH;Chen,YT;Ding,JH;Kodo,N;Roe,CR
通讯作者：
Roe,CR