Manejo conservador de un hematoma subcapsular hepático en un síndrome de HELLP

To improve morbidity and mortality in patients with subcapsular liver hematoma, clinical suspicion, a rapid diagnosis and multidisciplinary treatment are fundamental. The treatment of this complication includes expectant management with clinical and radiological observation, embolization of the hepatic arteries, and surgical treatment if there is hemodynamic instability, persistent bleeding or increasing pain. We report a case of postpartum HELLP syndrome that was complicated by a subcapsular liver hematoma, successfully treated by embolization of the right hepatic artery.     

Síndrome ELLP,un diagnóstico diferencial complicado

HELLP syndrome (Hemolysis, Elevated Liver enzymes, and Low Platelet count) is a thrombotic microangiopathy of pregnancy. This syndrome may be incomplete, with manifestations of only a few of its parameters: EL (elevated liver enzymes), ELLP (elevated liver enzymes and thrombocytopenia) and LP (thrombocytopenia alone). ELLP syndrome is a difficult differential diagnosis in which the main causes of thrombocytopenia in pregnancy and other diseases must be excluded. In current clinical practice, the management of ELLP is similar to that of complete HELLP syndrome. However, maternal and fetal morbidity is lower in ELLP syndrome, which may suggest the need to evaluate different protocols for these two variants of the same disease.     

Tratamiento quirúrgico conservador del síndrome de HELLP (ELLP) asociado a hematoma hepático subcapsular

Severe hemorrhagic complications associated with pregnancy usually occur within the third trimester and are frequently linked to preeclampsia and HELLP syndrome.     

Rotura hepática asociada a preeclampsia y síndrome HELLP,con resultados catastróficos

We present the case of a 41 years old pregnant, diagnosed of preeclampsia in her 39 weeks with catastrophic result, ending in maternal death. We show the deterioration of this patient since the diagnosis of preeclampsia, with evolution to eclampsia, HELLP syndrome, respiratory distress, septic shock and finally, maternal death.     

Rotura hepática espontánea en el síndrome HELLP: tratamiento mediante packing

Spontaneous hepatic rupture associated with HELLP syndrome (hemolysis, elevated liver enzyme levels and low platelet count) is a very rare phenomenon that is frequently associated with a substantial rate of complications and mortality. In these cases, urgent management based on prompt delivery, blood transfusion and hepatic hemostasis is required.     

Drugs in pregnancy. Gastrointestinal disease.

Gastrointestinal diseases in pregnancy can be divided into diseases specific to pregnancy, for example, hyperemesis gravidarum, obstetric cholestasis, HELLP syndrome and acute fatty liver of pregnancy, and diseases incidental to pregnancy, for example, inflammatory bowel disease, dyspepsia, peptic ulcer disease and viral hepatitis. Disorders in the second category may present for the first time in pregnancy. This chapter considers the drug management of each of these conditions, with the exception of HELLP syndrome and acute fatty liver. The preferred drug treatment and the known complications associated with their use in pregnancy are also described. Where possible, studies relating to the safety of different therapeutic options are discussed.     

Gastrointestinal disease

Gastrointestinal diseases in pregnancy can be divided into diseases specific to pregnancy, for example, hyperemesis gravidarum, obstetric cholestasis, HELLP syndrome and acute fatty liver of pregnancy, and diseases incidental to pregnancy, for example, inflammatory bowel disease, dyspepsia, peptic ulcer disease and viral hepatitis. Disorders in the second category may present for the first time in pregnancy. This chapter considers the drug management of each of these conditions, with the exception of HELLP syndrome and acute fatty liver. The preferred drug treatment and the known complications associated with their use in pregnancy are also described. Where possible, studies relating to the safety of different therapeutic options are discussed.     

Púrpura trombótica trombocitopénica versus síndrome de HELLP: un reto diagnóstico durante la gestación

Thrombocytopenia is relatively frequent during pregnancy and can occur in a range of syndromes. Among these, particular attention should be paid to thrombotic thrombocytopenic purpura (TTP) and HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count). It can be difficult to differentiate between these conditions during pregnancy, and their management and complications may differ widely. It is therefore essential to recognize their causes and perform a correct differential diagnosis, particularly in light of their possible serious consequences, which include maternal and/or fetal death.     

Manifestación atípica de hígado graso agudo del embarazo

Acute fatty liver of pregnancy is an uncommon but highly serious entity. The classical features are abdominal pain, nausea, vomiting, and elevated liver enzymes. Acute fatty liver of pregnancy can also present with atypical features with insidious onset and other clinical manifestations that are infrequent in this entity.     

Diabetes insípida transitoria en gestante con disfunción hepática y embarazo gemelar. Revisión del síndrome polidipsia-poliuria en gestación

Diabetes insipidus (DI) is a rare endocrine complication of pregnancy. Traditionally, DI has been considered to be either central or nephrogenic. However, in pregnancy, a specific type has been described: transient DI or gestational DI. This entity occurs mainly in the third trimester of pregnancy and resolves spontaneously between the first and fourth week after delivery. The physiopathology of gestational DI has a placental origin, with excessive levels of placental vasopressinase leading to excessive catabolism of vasopressin and secondarily to polydipsia-polyuria syndrome in the pregnant woman. Screening for subclinical pregestational DI (central or nephrogenic) or transient gestation is mandatory whenever this entity is suspected clinically. Diagnosis is simple and treatment with desmopressin acetate significantly decreases the maternal and fetal risks of this disease. It is also necessary to exclude other frequently associated disorders (preeclampsia, HELLP syndrome, liver dysfunction) and to carry out close fetal monitoring, because of the risk of intrauterine growth retardation secondary to placental dysfunction.     

Hyperemesis gravidarum,gastrointestinal and liver disease in pregnancy

Women of childbearing age may be affected by diseases of the gastrointestinal tract or liver; some have no effect on obstetric outcome, some are improved in pregnancy and some deteriorate. Gastrointestinal or liver disease may be caused by pregnancy and resolve following delivery e.g. hyperemesis gravidarum, pre-eclampsia, acute fatty liver of pregnancy, HELLP syndrome and obstetric cholestasis, or may present for the first time in pregnancy e.g. inflammatory bowel disease, cholelithiasis and hepatitis. These examples and other common gastrointestinal and liver disorders will be discussed giving details of diagnosis, management and effects on pregnancy outcome and also background information on normal liver physiology in pregnancy.     

Manifestación de leucemia promielocítica aguda en el embarazo

Acute promyelocytic leukemia, also known as M3 in the French-American-British classification, presents as severe hemorrhagic syndrome in 80% of affected pregnant women, posing a lethal threat to both mother and fetus. The results of multicenter studies suggest that the most effective treatment consists of the concomitant use of all-trans retinoic acid plus idarubicin. We present the case of a 32-year-old woman at 33 weeks of pregnancy with severe coagulation abnormalities due to acute promyelocytic leukemia, who was admitted to the intensive care unit for respiratory insufficiency due to an acute pulmonary lesion. The interest of this case lies in its infrequency. We describe the therapeutic management and initial course of the disease, as well as the prognostic implications of treatment during pregnancy.     

Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications.

Fetal-maternal interactions are critical determinants of maternal health during pregnancy and perinatal outcome. This review explores the causative relationship of a fetal disorder of mitochondrial fatty acid oxidation, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, and the serious maternal liver diseases of pregnancy-preeclampsia, the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet counts), and acute fatty liver of pregnancy. Features of the metabolic adaptation necessitated during the fetal-neonatal transition; common phenotypes of pediatric fatty acid oxidation disorders, including neonatal hypoketotic, hypoglycemia and hepatic crisis; and clinical abnormalities of HELLP and acute fatty liver of pregnancy are presented. Evidence that a common mutation in the alpha-subunit (LCHAD) of trifunctional protein, E474Q, is always one of the mutant alleles in fetal isolated LCHAD deficiency associated with these disorders of pregnancy that cause high maternal, fetal, and newborn morbidity and mortality is reviewed. Recommendations for molecular testing for LCHAD deficiency in families with life-threatening maternal liver disease are given.     

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.     

Síndrome de bridas amnióticas: caso clínico y revisión del tema

Amniotic band syndrome is a set of congenital birth defects consisting of constriction rings and limb or digit amputations, associated with the presence of amniotic bands. The incidence of this complication is low and its occurrence is sporadic. This syndrome usually causes constriction rings in the distal end of limbs or digits, which, in severe cases, can lead to complete amputation of the limbs or other malformations. Only 29-50% of cases are diagnosed prenatally. We report the case of a patient with an ultrasonographic diagnosis of amniotic band syndrome in week 12 of pregnancy.     

妊娠期高血压疾病并发HELLP综合征终止妊娠时机及方式的探讨

目的:探讨妊娠期高血压疾病并发溶血、肝酶升高及血小板减少综合征(HELLP综合征)终止妊娠的时机和方式对母儿的影响。方法:对我院11年来31例HELLP综合征患者的临床资料进行回顾性分析。结果:31例患者当中阴道分娩仅2例,其中1例母体产后并发DIC死亡,围生儿1例死产,1例新生儿死亡。剖宫产29例,剖宫产率93.5%,其中1例母体术后并发急性肾功能衰竭死亡,1例术后并发DIC、多器官衰竭(MOST)死亡,余27例均存活,未发现明显后遗症;围生儿31例(双胎2例),其中胎死宫内3例,死产1例,新生儿死亡4例。结论:HELLP综合征严重威胁母儿的安全,适时终止妊娠是治疗HELLP综合征极其重要的手段,终止妊娠的方式首选剖宫产;终止妊娠的时机为诊断后24~48小时,具体还应依病情的严重程度及孕周的大小作出综合判断。     

妊娠期急性肾损伤的相关进展

妊娠期间肾脏损伤风险至少增加2倍,妊娠期急性肾损伤(acute kidney injury in pregnancy,P-AKI)是一种高风险产科疾病,严重威胁母亲和胎儿的健康。尽管P-AKI在过去30年中发病率下降,我国仍有较高的发病率及致死率。P-AKI是妊娠期严重的并发症,有起病急、进展快、病情重的临床特点,常伴有呼吸、心血管及消化等系统的症状。P-AKI的相关疾病,如子痫前期、妊娠期急性脂肪肝、HELLP综合征和血栓性微血管病等临床表现相似,容易出现诊断困难。现总结近年来国内外文献,从P-AKI病理生理变化、不同地区发病率及诱因、诊断标准、P-AKI的相关疾病及预防治疗措施等进行分析,并对P-AKI的透析指征进一步分析,旨在提高大家对疾病的了解、预防和诊治,降低发病率、病死率及致残率,改善母婴结局。     

Lebererkrankungen in der Schwangerschaft

Liver diseases in pregnancy can result in maternal as well as fetal complications. Intrahepatic cholestasis in pregnancy may lead to a high fetal risk of IUD, meconium-stained amniotic fluid, premature delivery, asphyxia and arrhythmia. Concerning a good fetal outcome there is no evidence-based therapy. Pruritus and elevated cholic acid can be treated and controlled well by ursodeoxycholic acid. Since there is a higher risk of intrauterine death, most common at 37+ weeks of gestation, delivery is recommended starting at 36+ weeks, even earlier when excessively high cholic acid levels occur. Acute fatty liver of pregnancy is linked to higher maternal and fetal mortality. There are regular crossovers to the HELLP syndrome. Cholelithiasis causes 6% of all jaundice in pregnancy and thus has to be considered as another differential diagnosis particularly in multiparity. If operative treatment is required for cholecystolithiasis during pregnancy the best fetal outcome is achieved in the second trimester. It is likely that in the future chronic liver diseases such as Wilson??s disease and autoimmune hepatitis will be seen more often during pregnancy since there are increasingly better options for treatment. The same applies to pregnant women who have already undergone liver transplantation. An interdisciplinary approach with hepatologists in these high-risk pregnancies is mandatory.     

Jaundice as the first symptom of HELLP syndrome--case report

Jaundice is a rare symptom of the HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count) and is diagnosed in only 5% of the patients with this condition. However jaundice is related with sever presentation of the disease and associated with higher mortality The aim of this paper was to present a case of 24-year-old patient with jaundice as the first symptom of severe HELLP syndrome. A review of the literature about symptoms and treatment of HELLP syndrome and differential diagnosis of jaundice in pregnancy was done as well.     

Disección aórtica aguda tipo A en gestante de 33 semanas afectada del síndrome de Marfan

Although aortic dissection is uncommon in young women, it occurs more frequently during pregnancy, especially in the last trimester. Predisposing factors for aortic dissection are Marfan syndrome and other collagen diseases, chronic hypertension, aortic coarctation and cocaine abuse.