陈欣+孟丽丽+付帅+刘梅兰+张建平
[摘要] 意图 剖析妊娠期急性脂肪肝患者的临床体现特征,并追寻妊娠结局,了解疾病的转归。 办法 回忆性剖析妊娠期急性脂肪肝14例,记载临床材料,包含一般状况如年纪、孕周、是否初产妇等以及临床体现、相关查看、并发症及妊娠结局等。 成果 妊娠期急性脂肪肝患者的发病孕周为(34.72±2.51)周,城市人口较多,占78.6%。患者中大部分为初产妇,占92.9%。临床体现以黄疸、血肌酐升高、消化道症状、胆酶别离等为主;并发症则体现为急性肾功用不全的发作率最高,其次为弥散性血管内凝血(DIC)、肝性脑病、产后出血等。妊娠结局:孕母逝世、早产、死胎、新生儿窒息的发作率较高。 定论 妊娠期急性脂肪肝好发于妊娠晚期,常伴有严峻的并发症,对母胎影响大,在临床工作中应留意及时确诊、医治。
[关键词] 妊娠期急性脂肪肝;妊娠;弥散性血管内凝血
[中图分类号] R714.255 [文献标识码] B [文章编号] 1674-4721(2014)09(c)-0162-03
Clinical analysis of 14 cases with acute fatty liver of pregnancy
CHEN Xin MENG Li-li FU Shuai LIU Mei-lan ZHANG Jian-ping
Department of Gynaecology and Obstetrics,Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University,Guangzhou 510120,China
[Abstract] Objective To analyze the clinical manifestation feature of patients with acute fatty liver of pregnancy,and track the pregnancy outcome and know the prognosis of the disease. Methods 14 cases with acute fatty liver of pregnancy were retrospectively analyzed,with clinical data including the general situation(such as age,gestational age,whether primipara, etc),clinical manifestations,relevant examination,complications and pregnancy outcomes and so on were recorded. Results Pregnancy gestational age at onset in patients with acute fatty liver was (34.72±2.51) weeks,the urban population was more(accounting for 78.6%).Most of the patients are primiparas (accounting for 92.9%).The main clinical manifestations were jaundice,elevated serum creatinine,gastrointestinal symptoms,bile enzyme separation and soo on,and complications are characterized by the highest incidence rate of acute renal insufficiency,followed by disseminated intravascular coagulation,hepatic encephalopathy,postpartum hemorrhage,etc.Pregnancy outcomes:the incidence rate of motherhood death,premature birth,stillbirth,and neonatal asphyxia were higher. Conclusion Acute fatty liver of pregnancy should be paid attention to timely diagnosis treatment in clinical work,and it appears mostly at late pregnancy often associating with serious complications,and it has a greater impact on mother and the fetus.
[Key words] Acute fatty liver of pregnancy;Pregnancy;Disseminated intravascular coagulation
妊娠期急性脂肪肝(acute fatty liver of pregnancy,AFLP)是妊娠期特有疾病之一。1996年报导其发病率为1/13 000,2002年则为1/7000[1-2]。临床以肝功用急剧衰竭、黄疸为主要特征,可伴有脑、肾等多脏器功用危害,严峻危及孕产妇及围产儿的生命安全,病死率高。现在较为一致的观念是,AFLP一经确诊应立即停止妊娠,因而,清晰确诊、及时处理在临床工作中尤为重要。本研讨搜集、剖析了14例AFLP。
1 材料与办法
1.1 一般材料
搜集1993年1月~2013年12月中山一院、中山大学孙逸仙留念医院、中山三院、南边医院、广东省人民医院、广州市第二人民医院、广医附二院、番禺何贤医院、江门中心医院、中山博爱医院、惠州中心医院、粤北人民医院、深圳保健院、市妇幼韶关市第二人民医院、清远市人民医院出院确诊为AFLP的患者14例。
1.2 确诊规范
①妊娠晚期发作消化道症状、黄疸、肝功用危害和(或)暴发性肝衰竭;②肝脏印象学查看契合AFLP的改动;③如有肝脏病理学查看,应契合AFLP改动:肝细胞弥漫性脂肪变性,炎症坏死不明显[3]。
1.3 研讨内容
①患者的基本材料:年纪、生育史、既往肝脏反常史;②临床体现及妊娠进程:发病孕周、主要症状及妊娠结局;③实验室查看:血尿惯例、肝脾超声查看、肝穿刺病检、肝炎病原学查看等。
2 成果
2.1 一般状况
14例患者的平均年纪为(27.36±4.62)岁,发病孕周为(34.72±2.51)周。其间城市人口合计11例,占78.6%,农村人口3例,占21.4%;初产妇13例,占92.9%,经产妇1例,占7.1%;定时产检者6例,余8例未定时产检。14例患者既往均无肝脏反常病史。
2.2 临床材料状况
AFLP患者的黄疸发作率为100.0%,血肌酐升高、消化道症状、胆酶别离发作率也较高,均≥50.0%。14例患者中,7例剖宫产停止妊娠(表1)。
表1 临床材料状况
2.3 并发症发作状况
急性肾功用不全的发作率最高,其次为DIC、肝性脑病、产后出血等(表2)。
表2 并发症发作状况
2.4 妊娠结局
APLA患者有很高的母儿逝世率、发病率,孕母逝世率为50.0%,早产率为57.1%。活产儿8例,其间3例呈现新生儿窒息,占活产儿的37.5%(表3)。
表3 妊娠结局
3 评论
我国肝炎病毒感染率高,因而,一旦孕期呈现肝病体现,临床医师首要考虑的确诊往往是重症肝炎,但需求警觉的是,AFLP作为妊娠期罕见的并发症,近年来,发病率呈升高趋势。现在,AFLP的发病率远高于以往的知道。
AFLP是妊娠期特有的疾病,发病原因和机制尚不清晰。现在以为,最常见的病由于雌、孕激素代谢妨碍。妊娠期雌、孕激素水平增高,使三羧酸循环化中棕榈酸氧化作用活性下降,加之雌激素可使肝细胞线粒体超微结构发作改动,一旦呈现代谢妨碍,可导致很多脂肪微滴滋润肝细胞,堵塞胆怯管,形成肝内胆汁淤积,乃至是小片状坏死。此外,AFLP发病尚可能与养分、感染、药物中毒、肥壮及遗传等有关[4-9]。
AFLP发病初始症状没有特异性,多为疲惫、厌恶、吐逆,特别是仅有消化道症状,易与胃肠炎相混杂,较难作出确诊[10-11],但病况发展往往急骤、严峻,可敏捷呈现肝功用反常。本研讨显现,AFLP多发作于妊娠晚期,以初产妇为主,肝炎病原学检测阳性率低,提示关于初产妇假如妊娠晚期呈现肝功用反常,且病原学查看为阴性,应高度警觉AFLP的可能。有一些特征如高血压、蛋白尿、高尿酸、高肌酐发作率高,能够帮忙进行辨别确诊。本研讨一切患者皆呈现黄疸,其他体现还有昏倒、少尿等。
AFLP患者肝细胞在短时期内很多坏死,所以往往很快呈现并发症[12-13]。本研讨发现急性肾功用不全的发作率最高,其次为DIC、肝性脑病、产后出血等,且产后出血、DIC是孕妈妈逝世的主要原因[14]。
AFLP患者中,妊娠妇女逝世、死胎、早产、新生儿窒息的发作率都很高。正常妊娠早产率仅为5%~15%,新生儿窒息率为3%~10%,而在AFLP患者中早产率高达87.5%,新生儿窒息率达37.5 %,可能原由于AFLP患者存在代谢紊乱、能量缺乏,胎盘运送血氧及养分功用下降[15]。为改进AFLP患者及新生儿预后,应活跃处理原发病,进行归纳医治,防治多脏器功用衰竭,并及时停止妊娠。
关于临产方法,一般建议不能短时间经阴道临产的患者,采纳剖宫产相对较安全,但手术是损害性操作,存在很多出血可能,所以临床工作中应根据具体状况决议产科处理计划,轻柔的手术操作及切当的止血特别重要。
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(收稿日期:2014-07-11 本文修改:许俊琴)
[7] Shekhawat P,Bennett MJ,Sadovsky Y,et al.Human placenta metabolizes fatty acids:implications for fetal fatty acid oxidation disorders and maternal liver diseases[J].Am J Physiol Endocrinol Metab,2003,284(6):E1098-E1105.
[8] Tank PD,Nadanwar YS,Mayadeo NM.Outcome of pregnancy with severe liver disease[J].Int J Gynaecol Obstet,2002, 76(1):27-31.
[9] 张璐,张素云.妊娠期急性脂肪肝6例临床剖析[J].浙江临床医学,2010,12(2):175-176.
[10] Vigil-De Gracia P,Lavergne JA.Acute fatty liver of pregnancy[J].Int J Gynaecol Obstet,2001,72(2):193-195.
[11] 屈在卿.妊娠合并上腹痛2例报导[J].重庆医学, 2012, 41 (1): 103-104.
[12] Qiu DK.Complications of chronic liver disease-current conception of diagnosis and treatment[M].Shanghai:Shanghai Science and Technology Publishing House,2001:76.
[13] Steingrub JS.Pregnancy-associated severe liver dysfunction[J].Crit Care Clin,2004,20(4):763-776.
[14] Hamid SS,Jafri SM,Khan H,et al.Fulminant hepatic failure in pregnant women:acute fatty liver or acute viral hepatitis?[J].J Hepatol,1996,25(1):20-27.
[15] Blish KR,Ibdah JA.Maternal heterozygosity for a mitochondrial trifunctional protein mutation as a cause for liver disease in pregnancy[J].Med Hypotheses,2005,64(1):96-100.
(收稿日期:2014-07-11 本文修改:许俊琴)
[7] Shekhawat P,Bennett MJ,Sadovsky Y,et al.Human placenta metabolizes fatty acids:implications for fetal fatty acid oxidation disorders and maternal liver diseases[J].Am J Physiol Endocrinol Metab,2003,284(6):E1098-E1105.
[8] Tank PD,Nadanwar YS,Mayadeo NM.Outcome of pregnancy with severe liver disease[J].Int J Gynaecol Obstet,2002, 76(1):27-31.
[9] 张璐,张素云.妊娠期急性脂肪肝6例临床剖析[J].浙江临床医学,2010,12(2):175-176.
[10] Vigil-De Gracia P,Lavergne JA.Acute fatty liver of pregnancy[J].Int J Gynaecol Obstet,2001,72(2):193-195.
[11] 屈在卿.妊娠合并上腹痛2例报导[J].重庆医学, 2012, 41 (1): 103-104.
[12] Qiu DK.Complications of chronic liver disease-current conception of diagnosis and treatment[M].Shanghai:Shanghai Science and Technology Publishing House,2001:76.
[13] Steingrub JS.Pregnancy-associated severe liver dysfunction[J].Crit Care Clin,2004,20(4):763-776.
[14] Hamid SS,Jafri SM,Khan H,et al.Fulminant hepatic failure in pregnant women:acute fatty liver or acute viral hepatitis?[J].J Hepatol,1996,25(1):20-27.
[15] Blish KR,Ibdah JA.Maternal heterozygosity for a mitochondrial trifunctional protein mutation as a cause for liver disease in pregnancy[J].Med Hypotheses,2005,64(1):96-100.
(收稿日期:2014-07-11 本文修改:许俊琴)