妊娠合并急性脂肪肝的临床分析

目的为提高早期诊断和正确处理轻型妊娠急性脂肪肝(AFLP)，降低孕产妇及围生儿的死亡率。方法对1991年12月至2001年12月在我院产科住院分娩的4例AFLP患者的临床资料进行回顾性分析。结果4例患者均为初产妇，发病孕周为32—38周，单胎3例，双胎1例，入院前1—2周均有程度不一的乏力、恶心、厌食、腹泻及上腹部不适等临床表现，全部患者肝酶升高，谷丙转氨酶(ALT)升高为111—579IU／L，平均为253IU／L：天冬氨酸转氨酶(AST)升高达72—561IU／L，平均为219IU／L：血清胆红素升高在42．2—190．6umol／L，平均为93．3umol／L；伴有凝血酶时间延长、(3例较正常对照延长3倍以上)低蛋白血症、低纤维蛋白原及不同程度的出血倾向，表现为皮肤的瘀点、瘀斑、牙龈出血、呕血及伤口渗血。并发症：肝性脑病3例，DIC3例，低血糖2例，消化道出血2例。孕产妇死亡2例，病死亡率为50％，围生儿死亡3例，围生儿死亡率为60％。结论提高对AFLP的警惕及认识，根据其临床表现，结合实验室检查及B超检查，可作出早期诊断，对AFLP患者及时终止妊娠，配合积极有效的支持疗法，可大大改善AFLP的母儿预后。     

妊娠急性脂肪肝六例临床分析

妊娠急性脂肪肝是妊娠后期特有的急危重病 ,起病急而凶险 ,死亡率高达 85 %。早期诊断 ,积极有效的治疗与及时合理的产科处理 ,可明显改善预后。本研究对我院收治的 6例妊娠急性脂肪肝患者的临床资料进行回顾性分析 ,现报道如下。一、临床资料1.资料来源及诊断 :1996年 6月至 2 0 0 1年 6月我院共收治 6例妊娠急性脂肪肝患者 ,其诊断符合下列标准而最后临床诊断为妊娠急性脂肪肝 :(1)孕晚期出现恶心、呕吐、厌食、乏力 ,黄疸及肝功能损害 (6例 )。 (2 )Ｂ超检查符合脂肪肝显像 (2例 )。 (3)肝组织活检符合妊娠急性脂肪肝病理改变 (1例 )。 (…     

妊娠急性脂肪肝七例临床分析

妊娠急性脂肪肝 (AFL P)是妊娠晚期特发的疾病 ,发生率低 (1/ 13 32 3) ,母婴死亡率高达 75 % [1 ,2 ]。目前病因尚不明确 ,可能与孕期激素异常、缺乏某些营养物质、代谢障碍、胎儿等因素有关。Strauss等 [3 ]指出基因缺陷是 AFL P的病因。　　一、临床资料1982年 1月～ 1999年 12月 ,我院共收治 AFL P患者 7例。初产妇 6例 ,经产妇1例 ,其中双胎 2例。发生率为 3.88/10 0 0 0 ,孕产妇死亡率 85 .71% ,围产儿死亡率为 6 6 7‰ ,为我院 18年孕产妇死亡首位原因。6例否认肝病史 ,1例少年时患甲肝治愈。主要临床表现见表 1。实验室检查 :…     

妊娠合并急性脂肪肝7例分析

妊娠急性脂肪肝 (ＡＦＬＰ)是妊娠特发性疾病 ,临床上少见 ,但预后不佳 ,母婴病死率极高。如能早期诊断及时终止妊娠 ,每能挽救母儿生命[1] ,降低母婴病死率。本院收治ＡＦＬＰ 7例 ,报告如下。1　临床资料1.1　一般资料　 1983年 1月至 1999年 12月在我院产科住院分娩产妇 3985例 ,其中妊娠合并急性脂肪肝 7例。 7例患者中 ,年龄 2 3～ 2 9岁。初产妇 5例 ,经产妇 2例。单胎 6例 ,双胎 1例。孕产妇 4例存活 ,3例死亡 ,病死率为42 86 %。围生儿存活 6例 ,2例胎死宫内 ,围生儿病死率为 2 5 0 0 % ,临床资料见表 1。作者单位 :南京市第二医…     

妊娠合并急性脂肪肝6例诊疗体会

妊娠合并急性脂肪肝６例诊疗体会厉建兰１陈建梅１殷彩云２（１日照市人民医院２禹城市卫生防疫站）１临床资料妊娠急性脂肪肝（ａｃｕｔｅｆａｔｌｙｌｉｖｅｒ，ＡＦＬ）６例，年龄２４～２７ｙ，平均２５．８ｙ，均为初产妇，双胎４例，单胎２例，发病孕龄３３～３７＋...     

妊娠急性脂肪肝6例分析

妊娠急性脂肪肝 (acute fatty liver of pregnaney,AFL P)是妊娠晚期特有的严重疾病 ,发病率由百万分之一至万分之一不等 ,近年报道逐渐增多 [1 ,2 ] ,我院近 5年来收治AFL P患者 6例 ,现分析总结如下 :一、资料与结果1.诊断标准 :(1)孕晚期突发消化道症状、黄疸、肝功能损害 ;(2 )血清学检查排除甲、乙、丙、丁、戊型肝炎及药物性、中毒性或其他肝病 ;(3)肝活检者 ,病理符合 AFL P改变。2 .一般资料 :1996年 7月 1日至 2 0 0 1年 6月 30日 ,我院共发生 6例 AFL P,同期住院分娩孕产妇共 910 3例 ,发生率为 0 .0 7% ,孕妇年龄 2 4～ 3…     

妊娠期急性脂肪肝4例临床分析

目的:探讨妊娠期急性脂肪肝的诊断、治疗和预后.方法:对4例妊娠期急性脂肪肝患者的临床资料进行回顾性分析.结果:4例患者中3例初产妇,1例经产妇,孕龄33～40周.4例患者均有产后大出血、凝血功能障碍,剖宫产3例,其中2例行全子宫切除术,1例因多器官功能衰竭死亡,另2例抢救成功;1例患者因胎死宫内,行水囊引产后死于肝性脑病.新生儿存活2例,死亡2例.结论:妊娠期急性脂肪肝母儿死亡率高,早期识别和诊断是关键,及时终止妊娠能提高母儿生存率.     

急性妊娠脂肪肝12例临床分析

急性妊娠脂肪肝（acute fatty liver of pregnancy,AFLP）是一种非常少见的妊娠期并发症,它发病骤急,病情凶险,国外报道的AFLP发病率为1/6692～1/13 328,国内尚无确切的文献报道.但已证实AFLP对孕妇和胎儿的围产结局有致命的影响,若处理不及时很可能造成母婴死亡.为了提高对AFLP发病特点的认识,探讨如何进行早期诊断和治疗,我们对收治的AFLP患者的临床资料进行了回顾性分析.现将结果报道如下.     

妊娠急性脂肪肝患者的妊娠结局和随访分析

目的回顾性分析妊娠急性脂肪肝（acute fatty liver of pregnancy,AFLP）的临床特点,探讨早期诊断和治疗与妊娠结局及产后随访结局的关系。方法对5年内收治的6例AFLP患者的临床资料进行回顾性分析,并进行了1~5年的随访。结果临床表现有厌食、喜冷饮、恶心、呕吐、上腹不适、乏力及进行性加重的黄疸等。实验室检查肝转氨酶和血清胆红素升高、纤维蛋白原下降、凝血酶原时间延长。B超和CT显示脂肪肝影像。孕产妇并发症有低蛋白血症、肾功能损害、肝性脑病、低血糖等。发病至终止妊娠时间超过10d2例,其围产儿死亡,孕产妇病情危重。无孕产妇死亡病例发生。随访1～5年,产妇和存活的婴儿均健康,2例再次妊娠无再发病。结论掌握该病的临床特点,早期诊断、尽快终止妊娠及支持治疗,是提高母婴预后的关键,治愈后无远期不良结局。     

妊娠期急性脂肪肝的诊断与治疗

妊娠期急性脂肪肝(AFLP)是妊娠晚期一种特发性的严重并发症,孕产妇及围产儿死亡率高。该病缺乏特异性临床表现,需与妊娠期肝内胆汁淤积症等相鉴别,早期易误诊。肝活检是诊断AFLP的金标准,但根据病史、临床表现结合实验室及影像学检查,不必肝脏穿刺也可以做出临床诊断。迅速终止妊娠并给予支持治疗是主要治疗措施,对改善AFLP患者母胎结局至关重要。     

Acute fatty liver of pregnancy: a retrospective study of 32 cases in South China

Abstract

Objective: To describe 32 cases of acute fatty liver of pregnancy (AFLP) and identify the potential clinical predictors of maternal and fetal outcomes in South China.

Methods: Thirty-two cases of AFLP previously treated in the First Affiliated Hospital of Nanchang University in the past 10 years were enrolled in the current study.

Results: Sex of fetus and delivery method was the potential clinical predictor of fetal outcome. The main manifestations of AFLP were jaundice (n?=?32), nausea and vomiting (n?=?28), malaise (n?=?25), and ascites (n?=?25), and its complications included acute renal failure (n?=?26), hepatic encephalopathy (n?=?17), infection (n?=?10), and postpartum hemorrhage (n?=?7). Evidence of fatty changes of liver was demonstrated by B ultrasound and computed tomography (CT). Eighteen patients were recovered due to rapid diagnosis, early termination of pregnancy and supportive treatments.

Conclusions: Male sex and vaginal delivery was the risk factor of fetal outcome. Detailed history taking and proper management of potential predictors are important in making the decision of prompt delivery and choosing the appropriate delivery method. Further studies are needed to expand our knowledge on this disease.     

Clinical characteristics of acute fatty liver of pregnancy in a tertiary Indonesian hospital

Acute fatty liver of pregnancy (AFLP) is a rare, often autosomal recessive disorder with a major risk for maternal and perinatal mortality and morbidity. In order to achieve a more favorable outcome, awareness of its clinical signs and symptoms and early recognition are of pivotal importance. Over a 5-year period, 18 patients were diagnosed with AFLP (one twin, 19 babies). The most common sign and symptoms were jaundice, hypoglycemia, nausea and vomiting, encephalopathy, and hypertension. Abnormal laboratory test results included elevated total/conjugated (direct) bilirubin, AST, ALT, PT, APTT, creatinine, leukocyte count, and hypoalbuminemia. Maternal and fetal mortality rate was high: 66.7% resulted in a maternal death and 57.9% in an intrauterine fetal demise (IUFD). The number of complications was found to correlate with maternal death (p?=?.042). Surviving AFLP patients had ≤3 complications, while patients with >3 complications on presentation had a high risk of maternal death (OR?=?5.0; 95% CI: 0.55–45.4). The presence of hypertension significantly increased the risk of maternal death (OR: 24.5; 95% CI: 1.1–542.8; p?=?.01). The risk of IUFD was primarily related to gestational age at delivery and birth weight. The high rate of jaundice as presenting symptom of AFLP suggests that Indonesian primary maternity care providers may often miss its important earlier signs and symptoms, in particular de novo onset of nausea and vomiting in late pregnancy.     

Diagnosis of early acute fatty liver of pregnancy

Acute fatty liver of pregnancy (AFLP) is usually diagnosed at autopsy, with severe hepatic failure, or after the appearance of extrahepatic disease. We present an atypical patient with mild AFLP in whom hepatic biopsy allowed earlier diagnosis. A low threshold for liver biopsy in patients not experiencing fulminant disease can afford diagnostic information prior to the onset of serious sequelae.     

Three cases of acute fatty liver of pregnancy: Postpartum clinical course depends on interval between onset of symptoms and termination of pregnancy

We report our experience with three cases of acute fatty liver of pregnancy. Case 1 complained of hydrodipsia 4 days before delivery. Case 2 presented with nausea, vomiting and dizziness 6 days before delivery. Case 3 developed loss of appetite and general fatigue with jaundice 10 days before delivery. They underwent termination of pregnancy after diagnosis was made. Case 3 still developed hepatic encephalopathy, and finally she required liver transplantation. We hypothesise that the interval between the onset of symptoms and termination of pregnancy is an important factor for acuity of the disorder and patient morbidity or mortality.     

Acute fatty liver of pregnancy associated with pancreatitis: a life-threatening complication

OBJECTIVE: Inadequate data describe pancreatitis as a complication in women with acute fatty liver of pregnancy. Our purpose was to report our experience with this complication, which included maternal and perinatal outcomes. STUDY DESIGN: Medical records of patients with acute fatty liver of pregnancy and/or pancreatitis were reviewed over a 15-year period. Records were reviewed for symptoms, laboratory findings, clinical course, and maternal and perinatal outcomes. RESULTS: Persistent nausea and vomiting were the symptoms in 10 of 12 patients (83%). The average maternal age was 26.3 years (range, 21-37 years). There were two maternal deaths (17%); one death occurred despite liver transplantation. Maternal morbidity included encephalopathy (50%), acute respiratory distress syndrome (17%), and renal failure (33%) that resulted from acute tubular necrosis (3 cases) and acute cortical necrosis (1 case) that required renal transplantation. Of the 12 patients, 11 patients (91%) had an elevated serum lipase level, and another patient had an elevated serum amylase level (9%). Eight of 12 patients (67%) underwent radiologic studies, of which 7 patients (88%) were diagnostic for pancreatitis. Radiographic and autopsy findings noted pancreatic inflammation (3 cases), pseudocyst formation (3 cases), and hemorrhagic pancreatitis (1 case). The radiographic and serum laboratory abnormalities occurred after the onset of hepatic and renal abnormalities in all cases. CONCLUSION: Pancreatitis is a potentially lethal complication of acute fatty liver of pregnancy, and all patients with this diagnosis should undergo screening for the abnormality. Pancreatic abnormalities typically appear after hepatic and renal dysfunction.     

The association between acute fatty liver of pregnancy and fatty acid oxidation disorders

Acute fatty liver of pregnancy is a relatively rare but potentially fatal liver disorder of late pregnancy. Recent advances in molecular diagnostic procedures provide evidence of a genetic basis for this condition and a link to offspring disorders in fatty acid oxidation. This relationship implies the need for genetic testing and follow-up of at-risk women and their neonates.     

Maternal mortality and severe maternal morbidity from acute fatty liver of pregnancy in the Netherlands

Objective

To assess maternal death and severe maternal morbidity from acute fatty liver of pregnancy (AFLP) in the Netherlands.

Study design

A retrospective study of all cases of maternal mortality in the Netherlands between 1983 and 2006 and all cases of severe maternal morbidity in the Netherlands between 2004 and 2006, in which all 98 maternity units in the Netherlands participated. Maternal mortality ratio (MMR) and incidence of severe maternal morbidity were the main outcome measures.

Results

The MMR from direct maternal mortality from AFLP was 0.13 per 100,000 live births (95% CI 0.05-0.29). The incidence of severe maternal morbidity from AFLP was 3.2 per 100,000 deliveries (95% CI 1.8-5.7).

Conclusions

AFLP is a rare condition which still causes severe maternal morbidity and in some cases mortality. Referral to a tertiary care hospital for treatment of this uncommon disease should be considered.     

Noninvasive Swansea criteria are valuable alternatives for diagnosing acute fatty liver of pregnancy in a Chinese population

Background: This study aims to assess the diagnostic and prognostic value of Swansea criteria in diagnosing acute fatty liver of pregnancy (AFLP) in a Chinese population.

Methods: A retrospective study was conducted on 52 Chinese women diagnosed with AFLP. All selected cases were reassessed using the Swansea criteria with special focus on the noninvasive criteria, since performing a liver biopsy for this indication is rare in a Chinese population.

Results: Ninety point four percent of patients fulfilled five or more of the Swansea criteria. Thirty-one cases were positive for six or more Swansea criteria, but there were no significance differences between patients when using a cutoff criteria <6 or >6. When patients were positive for less than seven criteria, frequency of stillbirth, continuous blood purification (CBP) treatment, hysterectomy, and postpartum hemorrhage were not increased. However, patients who were positive for seven or more criteria had a significantly higher risk of stillbirth and a higher rate of CBP treatment (p?p?=?0.040). We observed a significantly elevated postpartum hemorrhage along with positivity of the Swansea criteria (p?=?0.040).

Conclusions: Swansea criteria without liver biopsy are good screening tools for AFLP diagnosis, and may be useful for assessing disease severity.