妊娠期急性脂肪肝临床管理指南(2022)

中华医学会妇产科学分会产科学组

doi:10.3760/cma.j.cn112141-20210907-00499

妊娠期急性脂肪肝临床管理指南(2022)

DOI: 10.3760/cma.j.cn112141-20210907-00499

中华医学会妇产科学分会产科学组

利益冲突：所有作者声明无利益冲突。

张卫社，中南大学湘雅医院妇产科，1471674914@qq.com；杨慧霞，北京大学第一医院妇产科，yanghuixia@bjmu.edu.cn
[本文首次发表于中华妇产科杂志, 2022, 57(1): 13-24]

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出版历程
- 收稿日期: 2022-02-02
- 录用日期: 2022-03-04
- 出版日期: 2022-04-20

Clinical management guidelines for acute fatty liver of pregnancy in China (2022)

Obstetrics Subgroup, Chinese Society of Obstetrics and Gynecology, Chinese Medical Association

摘要

摘要: 妊娠期急性脂肪肝(AFLP)是一种罕见但病情危急的产科特有疾病，致死率高，对母儿安全构成严重威胁。为规范临床管理，改善母儿结局，特制订本指南。基于前期多次的临床问卷调查环节，指南制订小组确定了临床医师最关注的9个临床问题，并对其逐一给出了推荐意见，包括：AFLP的门诊筛查、诊断、术前风险评估、分娩方式选择、麻醉方式选择、围产期并发症、人工肝治疗的指征、预后的评估及治疗期间如何监测，涵盖了AFLP诊治相关的热点问题。
- 脂肪肝 /
- 孕妇 /
- 临床管理 /
- 诊疗准则(主题)
Abstract: Acute fatty liver of pregnancy (AFLP) is a rare but critical obstetric disease with a high mortality rate, and it poses a serious threat to the safety of the mother and the child. The guidelines are developed to standardize clinical management and improve the outcome of the mother and the child. Based on multiple previous clinical questionnaire surveys, the guideline development group identified the nine clinical issues that clinicians are most concerned with and gave recommendations for each issue, including the outpatient screening for AFLP, diagnosis, preoperative risk assessment, selection of mode of delivery, selection of anesthesia method, perinatal complications, indication for artificial liver therapy, evaluation of prognosis, and monitoring during treatment, which covers the hot topics associated with the diagnosis and treatment of AFLP.
- Fatty Liver /
- Pregnant Women /
- Clinical Governance /
- Pratice Guidelines As Topic

HTML全文

图 1 AFLP孕妇的临床管理流程

注：AFLP表示妊娠期急性脂肪肝；MDT表示多学科团队；INR表示国际标准化值；PTA表示凝血酶原活动度；TBil表示总胆红素；AKI表示急性肾功能损伤；DIC表示弥漫性血管内凝血；MODS表示多器官功能障碍综合征。

Figure 1. Clinical management process of AFLP pregnant women

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表 1 证据质量与推荐强度的GRADE分级表

Table 1. GRADE classification table of evidence quality and recommended strength

类别	分级	具体描述
证据质量分级	高(A)	非常有把握观察值接近真实值
	中(B)	对观察值有中等把握：观察值有可能接近真实值，但也有可能差别很大
	低(C)	对观察值的把握有限：观察值可能与真实值有很大差别
	极低(D)	对观察值几乎没有把握：观察值与真实值可能有极大差别
推荐强度分级	强(1)	明确显示干预措施利大于弊或弊大于利
	弱(2)	利弊不确定或无论质量高低的证据均显示利弊相当
	GPS	基于非直接证据或专家意见、经验形成的推荐
注：GRADE表示推荐意见的分级评估、制订和评价；GPS表示良好实践声明。

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表 2 文献中AFLP孕妇的临床特征、实验室及超声检查的异常率

Table 2. Clinical characteristics, laboratory and ultrasound bnormalities of AFLP pregnant women in the literature

类别	朱特选等^[9] (中国湖南，n=78)	Gao等^[10] (中国多中心，n=133)	Nelson等^[4] (美国，n=51)	Knight等^[11] (英国，n=57)
孕周[x或x±s(范围)]	35.6(29.0~42.0)	36.1±2.7(21.0~41.0)	37.0±2.6(31.7~40.9)	36.0(22.0~40.0)
临床表现(%)
恶心呕吐	64.1	42.9	57.0	60.0
上腹不适	24.4	30.8	53.0	56.0
黄疸	79.5	47.4	33.0	-
肝性脑病	26.9	28.6	16.0	9.0
烦渴或多尿	21.8	-	-	12.0
实验室检查(%)
白细胞计数升高	88.5	77.4	98.0	98.0
血小板计数减少	-	42.1	69.0	65.0
转氨酶升高	93.6	91.7	100.0	100.0
高胆红素血症	98.7	93.2	100.0	100.0
PT延长或凝血功能障碍	87.0	69.2	48.0	87.0
血氨升高	43.3	-	-	50.0
血肌酐升高	80.8	60.9	96.0	58.0
血糖异常	34.7(低血糖)	57.1(低血糖)，9.8(高血糖)	18.0(低血糖)	78.0(低血糖或高血糖)
超声检查(%)
明亮肝或腹水	83.1	57.1	27.0	27.0
注：-无此项；AFLP表示妊娠期急性脂肪肝；PT表示凝血酶原时间。

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表 3 AFLP的Swansea诊断标准^[13]

Table 3. Swansea diagnostic criteria for AFLP^[13]

类别	诊断标准
临床症状	呕吐
	腹痛
	烦渴或多尿
	肝性脑病
生化指标	胆红素>14 μmol/L(0.8 mg/dL)
	血糖 < 4 mmol/L(72 mg/dL)
	尿酸>340 μmol/L(5.7 mg/dL)
	白细胞计数>11×10⁹/L
	转氨酶>42 U/L
	血氨>47 μmol/L(27.5 mg/dL)
	血清肌酐>150 μmol/L(1.7 mg/dL)
	PT>14 s或APTT>34 s
超声检查	腹水或明亮肝
肝组织活检	微泡性脂肪变性
注：表中所有指标的异常以检测实验室所定标准进行界定，符合6个及以上的条目诊断为AFLP；AFLP表示妊娠期急性脂肪肝；PT表示凝血酶原时间；APTT表示部分凝血活酶时间。

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