Comment confirmer le diagnostic de stéatose hépatique aiguë gravidique en urgence ?

Acute fatty liver of pregnancy (AFLP) is a rare disease of which prognosis could be adverse if diagnosis is delayed. Certain diagnosis is sometimes made complex because of undercurrent symptoms with pre-eclampsia or hemolysis, elevated liver enzyme, low platelet (HELLP) syndrome. Several reports announce an increase of incidence and illustrate cases confirmed by non-invasive methods. They permit early diagnosis and improve morbidity and mortality. Reviewing seven of the most important series of AFLP, we demonstrate how to use ultrasonography or computed tomography scan to confirm AFLP. However, liver biopsy should be realised after delivery in case of uncertain diagnosis.     

Severe liver disease in pregnancy

Objective: To determine the clinical characteristics of patients with fulminant hepatitis of pregnancy (FHP) and acute fatty liver of pregnancy (AFLP) and analyze their correlation with pregnancy outcome. Methods: Of 55 pregnant women with severe liver disease, 41 had FHP and 14 had AFLP. Results: Jaundice was the primary manifestation for both FHP and AFLP and hepatic encephalopathy was the most significant complication for both. Disseminated intravascular coagulation, albuminuria, and prothrombin activity were found to be independent risk factors of maternal mortality for both. However, the rates of preterm labor, fetal demise, and neonatal asphyxia were lower in the FHP group. Conclusion: Women with FHP or AFLP are at risk for severe complications and adverse pregnancy outcome. Since the 2 conditions are managed differently, early diagnosis is essential.     

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. About 22 cases

OBJECTIVE: To report the clinical experience, biochemical findings, complications and maternal outcome in patients with acute fatty liver of pregnancy (AFLP). PATIENTS AND METHODS: Retrospective study over a period of 11 years (1993-2003). The diagnosis of AFLP was confirmed by liver biopsy in 15 women. However, in 7 women a medical committee that took into account clinical symptoms, and laboratory findings assessed the diagnosis. RESULTS: Were included in this study, 22 women with a mean age of 30+/-5.4 years. Only 22.7% of cases were primigravid. The mean gestational age was 36+/-2.76 weeks (range 31-41 weeks). The fetus was a male infant in 75% of cases. Ten women were admitted in the hospital without jaundice. However 15 women had developed an icterus since their hospital admission or during ICU stay. The mean SAPS II on the ICU admission was of 24.86+/-11.2 points. Biological disturbances observed were mainly: liver cytolysis in 91% of cases, a trend to hypoglycaemia in 86%, a hypoprotidemia in 66.7% and CIVD in 32%. During their ICU stay, 19 women (86.4%) developed one or several organ failures associated to the hepatic failure and 18 women required blood transfusion. After an average stay of 7.5 days, evolution was marked by the death of seven patients (31.8%). Factors correlated with a poor prognosis were: the delay of medical consultation, the development of jaundice, the development of encephalopathy, respiratory or a circulatory failure. DISCUSSION AND CONCLUSION: AFLP is a rare but life-threatening complication. Furthermore AFLP shares features with other more common and less perilous illnesses. An early diagnosis and appropriate therapy of this pathology should improve the poor prognosis in our country.     

深圳地区妊娠期急性脂肪肝的发病状况与母婴结局

目的了解深圳地区妊娠期急性脂肪肝(AFLP)的发病率、临床特点及母婴结局。 方法统计深圳地区2003年至2010年8年间分娩孕产妇数,收集符合诊断为妊娠期急性脂肪肝的病例,分析其发病率、临床特点及母婴结局。 结果8年间深圳地区分娩孕产妇982 761例次,符合妊娠期急性脂肪肝诊断标准的病例44例,发病率为1/22 335。44例妊娠合并急性脂肪肝患者中,合并妊娠期高血压疾病的有23例(52%);平均年龄(27.7±5.7)岁,发病孕周为30^＋2～40^＋3周,初产妇占33例(75%),多胎妊娠占9例(20%)。37例(84%)首发症状为不同程度的消化道症状,40例(91%)出现黄疸并进行性加重,39例(89%)病例出现凝血功能障碍,25例(57%)出现肾功能不全,9例(20%)合并肝性脑病,5例(11%)出现多器官功能障碍。孕产妇痊愈39例,死亡5例,孕产妇死亡率为11%;分娩胎儿55例,死胎9例,新生儿死亡1例,围生儿死亡率为18%。 结论深圳地区妊娠期急性脂肪肝以晚孕期消化道症状为首发症状,同时伴有黄疸、肝酶升高、凝血功能障碍,并出现早发肾功能不全为主要临床特点。早期诊断,及时终止妊娠可改善母婴结局。     

Acute fatty liver of pregnancy: an unusual presentation.

We describe a case of acute fatty liver that developed during the third trimester of pregnancy. Acute fatty liver of pregnancy (AFLP) is a well-documented medical condition and is important due to its implications on maternal and fetal morbidity and mortality. AFLP is managed with supportive care and close surveillance is needed in patients with a previous history of this disease. Long-term sequelae such as brain damage may also occur. This case was unusual because the patient presented due to a co-incident uterine scar rupture and was subsequently diagnosed with AFLP. Although no association between scar rupture and AFLP has been reported in the scientific literature, the association of abdominal scar dehiscence and liver disease has been reported previously.     

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.     

妊娠急性脂肪肝35例临床分析

目的探讨妊娠急性脂肪肝（acute fatty liver of pregnancy,AFLP）的早期诊断方法和治疗原则。方法回顾性分析了1999年1月至2009年12月在北京地坛医院收治的35例AFLP患者的临床资料,并全部进行了随访。结果 AFLP临床表现以消化道症状、肝损害、凝血功能障碍为主要特征,严重者伴多脏器功能受损。孕产妇及围产儿结局：3例孕产妇死亡,5例围产儿死亡。结论早期诊断和及时终止妊娠,加强支持疗法及综合治疗是关键,可有效地降低母婴死亡率。     

Acute fatty liver of pregnancy

Objective Acute fatty liver of pregnancy (AFLP) is a rare and serious entity associated with significant maternal and neonatal mortality and morbidity. We describe our experience with the clinical diagnosis, management and course patients with AFLP.Study design Medical records of patients with AFLP were reviewed over a 10-year period. Records were reviewed for symptoms, laboratory findings, clinical course, and maternal and perinatal outcomes.Results The incidence of AFLP was 1 in 7,270 births. The mean gestational age at onset was 34.6 ± 4.9 weeks. Eight percent of the patients were multiparous with more than three pregnancies. The prodromic phase was variable; patients complained of nausea, abdominal pain, malaise, polyuria-polydipsia syndrome and headaches followed by jaundice. The laboratory results indicated coagulopathy, liver function abnormalities and hypoglycemia. Emergency cesarean section was performed in two cases. The diagnosis has been assessed by transcutaneous hepatic biopsies processed in all patients between the 4th and 15th day (8.4 ± 4.3 days). Maternal morbidity included hypoglycaemia (40%), coagulopathy (50%) encephalopathy (30%) and renal failure (40%). There were no maternal deaths but fetal mortality is high 66%.Conclusion The early recognition of AFLP cases and prompt progressive management, including early termination of pregnancy and large dose infusion of fresh frozen plasma, the prognosis of AFLP is obviously improved.     

Sarcoidosis mimicking recurrent endometrial cancer

BACKGROUND: Sarcoidosis is a multisystem disease and can be confused with benign or malignant tumors. In patients with recurrent gynecologic cancer, liver and intrathoracic lesions should undergo a biopsy to rule in metastatic malignancy, as clinical findings and CAT scan results may represent other disease processes. CASE: A 67 year old woman had a total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and periaortic lymphadenectomy, and peritoneal cytology in 2001 for Stage I B grade 1 adenocarcinoma of the endometrium. She developed a vaginal recurrence in 2005. A CT scan of lungs, abdomen, and pelvis revealed extensive mediastinal adenopathy and multiple space occupying hepatic lesions worrisome for metastatic disease. A needle biopsy of the largest liver lesion revealed sarcoidosis. CONCLUSION: Sarcoid lesions may mimic metastatic disease in patients with malignancy, potentially leading to delayed and/or inappropriate therapy.     

Acute fatty liver in the second trimester of pregnancy

Objective: Acute fatty liver of pregnancy (AFLP) is an uncommon, potentially fatal disorder that usually occurs in the late third trimester of pregnancy. We present the first reported case of acute fatty liver in the second trimester of pregnancy.Methods: We report the clinical and laboratory findings in a patient with AFLP who presented in the second trimester of pregnancy.Results: A 37-year-old G5P4 woman presented at 22 weeks gestation (by 18 weeks ultrasound) with nausea and vomiting. She was normotensive, had no proteinuria, had elevated SGOT and SGPT (266 and 261, respectively), negative hepatitis studies and a normal platelet count. She was managed conservatively for presumed cholelithiasis until 24 weeks gestation when she was transferred to our facility because of worsening SGPT and SGPT (368 and 505, respectively), jaundice (total bilirubin of 8.9 mg/dL), hypoglycemia, and laboratory evidence of disseminated intravascular coagulation (DIC) (PT = 18.6, PTT = 56, hypofibrinogenemia and presence of fibrin split products). Ultrasound showed singleton fetus (EFW 450 g) with total placenta previa. Computed tomography scan of the abdomen revealed decreased hepatic density consistent with AFLP. Delivery of a nonviable fetus was effected after transfusion of fresh frozen plasma. Postoperatively, the patient had rapid resolution of DIC, jaundice, and hypoglycemia; liver transaminases normalized 5 days postoperatively and the patient was discharged home in good condition 5 days later.Conclusion: It has been traditionally stated that AFLP occurs in the late third trimester of pregnancy. This case demonstrates that, even in the second trimester of pregnancy, the diagnosis of AFLP should be considered as a cause of deteriorating liver function, jaundice, and DIC.     

Acute fatty liver in pregnancy: treatment, prognosis, rules of management

Acute fatty liver of pregnancy (AFLP), rare and potentially fatal disease, is considered as obstetric emergency. The perinatologist must be familiar with this complication of pregnancy because early diagnosis, prompt delivery and supportive care is the mainstay of therapy. When pregnant woman has nausea or vomiting, abdominal pain and jaundice, increased transaminase activity, coagulopathy and hypoglycemia during third trimester AFLP should be suspected. Mothers who have experienced AFLP must be informed of the possibility recurrence and undergo close surveillance in the next pregnancy.     

An unusual cause of neonatal coagulopathy and liver disease.

Congenital thyrotoxicosis is a rare and potentially fatal illness. We report a case in a preterm infant delivered to a mother known to have autoimmune endocrine disease. Diagnosis was difficult because the infant's presenting symptoms and signs closely resembled congenital viral infection with co-existent hepatic dysfunction and coagulopathy. The associated hepatic dysfunction was so severe that liver biopsy was scheduled before the diagnosis emerged. A high degree of clinical suspicion coupled with prenatal identification of pregnancies at risk of complication by congenital thyrotoxicosis is imperative to facilitate prompt diagnosis and treatment.     

A technique for safe, frequent biopsy of the liver after hepatic transplantation

Valuable information can be obtained from the hepatic biopsy in patients undergoing hepatic transplantation. Biopsy of the liver can be repeatedly performed safely even in patients with severely deranged clotting function by using the technique described herein. This simple technique provides the window through which the immunologic and metabolic causes of hepatic dysfunction may be more clearly viewed and intelligently studied.     

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.     

Disease progression in a patient with nonalcoholic steatohepatitis

Nonalcoholic steatohepatitis (NASH) is a severe form of nonalcoholic fatty liver disease (NAFLD). The prevalence and clinical significance of NAFLD/NASH have been increasingly recognized in Western countries but much less known in Asian countries, including Taiwan. Here, we report the case of a 43-year-old man who had abnormal liver tests for 18 years. Retrospective evaluation of his initial clinical, laboratory and histologic findings indicated that the hepatic disorder was compatible with the diagnosis of NASH. Although his liver biochemical tests improved after taking lipid-lowering agents, a liver biopsy 17 years later demonstrated histologic progression of intralobular necroinflammation and perivenular fibrosis. These facts suggest that NASH, albeit mild and slowly progressive, indeed exists in Taiwan. After the control of chronic hepatitis B and C and westernization of the lifestyle in Taiwan, an increasing burden of NAFLD/NASH is anticipated and active prophylactic measures should be implemented.     

Mononucleosis and hepatic failure associated with diphenylhydantoin treatment in an infant.

Diphenylhydantoin-induced hepatitis and mononucleosis are uncommon in children. The occurrence of these two diseases in the same individual, with progression to hepatic failure is rare and has not been reported in infants. This report represents a 6-month-old male infant who developed an infectious mononucleosis-like syndrome and hepatic failure 16 days after diphenylhydantoin administration. He took this anticonvulsant for controlling seizures after a head injury. Fever, skin rash, hepatosplenomegaly, lymphadenopathy, and atypical lymphocytosis led to the initial diagnosis of infectious mononucleosis. However, negative heterophil antibody did not support the diagnosis. Jaundice ensued in the following course and became more and more profound. Meanwhile, physical examination showed shrinking in liver size. Negative virology studies, including Epstein-Barr virus, cytomegalovirus, and hepatitis B virus, excluded them as causative agents. The patient lapsed into a stage I hepatic coma, but gradually recovered clinically and biochemically after eight successive exchange transfusions and supportive care. Two liver biopsies were performed 20 and 50 days after the onset of disease, respectively. Remarkable hepatic parenchymal loss, cholestasis, and fatty change were found on histologic examination of the first biopsy specimen, and portal fibrosis was noted on the second.     

Acute fatty liver of pregnancy: underestimated complication of the third trimester?

Two cases of acute fatty liver of pregnancy (AFLP) are described. The liver function abnormalities have been ignored in the both cases. The review literature on this rare clinical entity unique to pregnancy is presented. The importance of clinical and biochemical findings in the diagnosis of disease is emphasized. Although etiology is unknown, deficiency of fatty acid beta-oxidation has been suggested.     

Transient remnant removal disease in acute fatty liver of pregnancy.

OBJECTIVE: To elucidate the potential role of an altered lipid metabolism in the pathophysiology of acute fatty liver of pregnancy (AFLP). CASE REPORT: We report on two otherwise healthy women in the 34th gestational week who presented with symptoms of AFLP. Besides characteristic symptoms like nausea, abdominal pain, highly elevated serum amino transferase levels, and increased creatinine concentrations, the patients' clotting system showed consumption and/or decreased synthesis of coagulation factors. Pregnancies were terminated by elective cesarean section because of worsening symptoms. Blood tests normalized quickly and both the mothers and their baby boys could be dismissed in healthy condition. STUDY DESIGN: Blood samples were collected shortly before delivery and 5, 15, 70, and 110 days afterwards. Lipids and apolipoproteins (apo) were analyzed in whole plasma as well as in very low density, intermediate density, low density (LDL), and high density lipoprotein. Total LDL was further separated into 6 LDL subfractions by equilibrium density ultracentrifugation. RESULTS: Before delivery, the LDL subfraction pattern was characterized by the virtual absence of intermediate and most dense LDL. Lipoprotein electrophoresis showed the presence of beta-migrating VLDL. Within days after delivery, the distribution of apoB-containing lipoproteins returned to normal. Genetic variations of apoE, lipoprotein lipases, and the long-chain 3-hydroxyacyl-coenzyme A dehydrogenase were not detected in any of the patients. CONCLUSIONS: The lipoprotein metabolism in the acute phase of AFLP was reminiscent of hepatic lipase deficiency, a disorder characterized by impaired removal of lipoprotein remnants. As these triglyceride-rich particles cause endothelial dysfunction, they may contribute to the pathophysiology of AFLP.