怀孕和胎儿脂肪酸氧化障碍的急性脂肪肝:系统性审查
Dante Varotsis1, Sarah Araji1, Rebecca Horgan1
1Department of Obstetrics and Gynecology and Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania; Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine and Department of Pediatrics, Division of Genetics, University of Mississippi Medical Center, Jackson, Mississippi; Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Eastern Virginia Medical School, Norfolk, Virginia; Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, New Jersey; and Department of Obstetrics and Gynecology, Maimonides Medical Center, Brooklyn, New York.
妊娠期急性脂肪肝 (AFLP) 在约三分之一的病例中与胎儿脂肪酸氧化 (FAO) 障碍有关,最常见的是LCHAD缺乏症. 这两种情况对母亲和新生儿都有很高的风险,因此在AFLP评估中需要进行LCHAD测试.
科学领域:
- 孕产妇和胎儿的医学
- 代谢障碍 代谢障碍 代谢障碍
- 遗传学 是一个遗传学.
背景情况:
- 妊娠期急性脂肪肝 (AFLP) 是一种罕见但严重的疾病.
- 胎儿脂肪酸氧化 (FAO) 障碍是一种遗传代谢条件.
- AFLP与胎儿FAO疾病之间的关联需要进一步调查.
研究的目的:
- 评估母体AFLP和胎儿粮农组织疾病之间的关联.
- 确定患有AFLP的母亲及其受FAO疾病影响的胎儿的临床和遗传特征.
主要方法:
- 对报告的AFLP病例进行FAO疾病遗传检测的系统文献审查.
- 搜索了多个数据库,包括PubMed,MEDLINE,Cochrane图书馆,CINAHL,Scopus和clinicalTrials.gov.gov. 这些数据库.
- 包括研究详细的怀孕特征和粮农组织障碍工作.
主要成果:
- 77例AFLP怀孕中的27例 (35.1%) 与已确认的胎儿粮农组织疾病有关.
- 最常见的粮农组织疾病是LCHAD缺陷 (81.5%),其次是MCADD,SCADD和CPT-I缺陷.
- 在LCHAD缺乏症的AFLP病例中观察到高发病率和死亡率,包括死产和婴儿死亡率.
结论:
- 在约三分之一的病例中,AFLP与胎儿FAO疾病有关.
- 在这种情况下,LCHAD缺乏是最常见的粮农组织疾病.
- 在AFLP诊断工作中建议对胎儿和母亲的LCHAD缺乏症进行评估.
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