Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants.
Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants.
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DOI：
10.1016/j.adnc.2003.12.001
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发表时间：
2004-02-01
期刊：
影响因子：
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通讯作者：
Bellig, Linda L
中科院分区：
文献类型：
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作者：
Bellig, Linda L
自20世纪80年代以来，妊娠期急性脂肪肝一直被认为是一个临床问题。在过去的8年里，这种疾病与婴儿先天性代谢错误的联系已经被认识到。这种疾病的杂合子女性通常是没有症状的，直到她们肝脏代谢游离脂肪酸(FFA)的能力被纯合子胎儿压倒为止。先天性代谢缺陷，即长链3-羟基酰基辅酶A脱氢酶(LCHAD)缺乏症，在婴儿中可能不会立即被识别出来。婴儿的症状通常是由饮食中长链脂肪酸负荷增加或由导致内源性脂肪分解的疾病引发的。下面的案例研究回顾了这一围产期健康问题的临床病理生理学，并强调了照顾患有妊娠急性脂肪肝的母亲所生婴儿的优先事项。
Acute fatty liver disease of pregnancy has been recognized as a clinical problem since the 1980s. In the past 8 years, the association of this disease with a genetic inborn error of metabolism in the infant has been recognized. Women who are heterozygous for this disorder are usually asymptomatic until the capacity of their livers to metabolize free fatty acids (FFA) is overwhelmed by a homozygous fetus. The inborn error of metabolism, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency, may not be immediately recognizable in the infant. Symptoms in the infant are often triggered by an increased long-chain fatty acid load in the diet, or by illness that results in breakdown of endogenous fat. The following case study reviews the clinical pathophysiology of this perinatal health problem and highlights the priorities for the care of infants born to mothers with acute fatty liver disease of pregnancy.