Liver transplantation for the treatment of acute liver failure in 3 cases with NBAS gene deficiency and literature review / 中华儿科杂志

Objective: To investigate the clinical efficacy of liver transplantation in the treatment of acute liver in children with NBAS gene deficiency disease and their outcome. Methods: This retrospective study enrolled children with NBAS gene deficiency who were admitted to the Children's Hospital of Fudan University for liver transplantation from January 2013 to June 2022. The clinical data were collected and analyzed. Medical literature published before June 2022 was searched with the keywords of "NBAS" "neuroblastoma amplified sequence recurrent" "acute liver failure" "SOPH syndrome" "short stature with optic nerve atrophy" "Pelger-Huët anomaly" in PubMed, China National Knowledge Infrastructure and Wanfang database. Results: Liver transplantation was performed in 3 patients (2 males and 1 female) with NBAS deficiency. All patients presented with fever-triggered recurrent acute liver failure. The genetic detection found compound heterozygous NBAS gene pathogenic variants in them. The total episodes of acute liver failure before liver transplantation were 11, 2, and 4 respectively, and the age at liver transplantation was 3.5, 2.3, and 2.0 years respectively. During liver transplantation, patient 1 was in the convalescent phase of acute liver failure, patient 2 was in the acute phase, presenting with hepatic encephalopathy (grade V) and respiratory failure, and patient 3 was considered to be in the acute phase. After liver transplantation, patient 1 recovered normal liver function within 1 month and had no liver transplantation-related complications. Patient 2 had secondary epilepsy, intellectual disability, movement disorder, and transiently elevated transaminases. Patient 3 died of severe infection within 1 month. There was no literature in Chinese, 6 in English, 8 NBAS-deficient patients who were treated with liver transplantation. Total 11 patients presented with fever-triggered recurrent acute liver failure. Their age at liver transplantation ranged from 0.9 to 5.0 years. Postoperative complications occurred in 3 patients. Until the last visit, they were followed up for 0.7 to 14.0 years. Total 2 patients died and the 9 surviving patients did not develop acute liver failure. Conclusions: Liver transplantation is effective for the treatment of acute liver failure associated with NBAS gene disease. However, postoperative complications of liver transplantation may occur. The timing of liver transplantation still needs further research.

ACG Clinical Guideline: disorders of the hepatic and mesenteric circulation

Disorders of the mesenteric, portal, and hepatic veins and mesenteric and hepatic arteries have important clinical consequences and may lead to acute liver failure, chronic liver disease, noncirrhotic portal hypertension, cirrhosis, and hepatocellular carcinoma. Although literature in the field of vascular liver disorders is scant, these disorders are common in clinical practice, and general practitioners, gastroenterologists, and hepatologists may benefit from expert guidance and recommendations for management of these conditions. These guidelines represent the official practice recommendations of the American College of Gastroenterology. Key concept statements based on author expert opinion and review of literature and specific recommendations based on PICO/GRADE analysis have been developed to aid in the management of vascular liver disorders. These recommendations and guidelines should be tailored to individual patients and circumstances in routine clinical practice.

Fulminant hepatic failure caused by herbal supplies

The consumption of botanicals for therapeutic purposes has increased significantly in recent years. Drug-induced liver disease (DILI) is a frequent cause of acute liver injury, around 50% in the United States, and about 1% is secondary to the use of phytotherapeuticals and herbal supplies. Ruellia bahiensis, a plant species of the Acanthaceae family, is a tropical plant distributed in Northeastern Brazil. In folk medicine in the state of Bahia, the species is known as "mãe-boa" and is commonly used. L.S.S, a 23-year old, female, patient was admitted at University Hospital of Bahia-Brazil with signs and symptoms of acute hepatitis. She had made daily use of an herbal supply popularly known as "mãe-boa" for at least two years prescribed by a physician. Diagnostic investigation was negative for viral and autoimmune hepatitis, leptospirosis, dengue, and CMV (cytomegalovirus). The patient had to undergo liver transplantation. Explant revealed massive hepatic necrosis. According to histological findings, and after exclusion of other etiologies, liver damage was assigned to herbal supply. The prolonged use of Ruellia bahiensis infusions may have caused the liver dysfunction.

Cerebral hemodynamic and metabolic changes in fulminant hepatic failure / Modificações da hemodinâmica e metabolismo cerebral na insuficiência hepatica fulminante

ABSTRACT Intracranial hypertension and brain swelling are a major cause of morbidity and mortality of patients suffering from fulminant hepatic failure (FHF). The pathogenesis of these complications has been investigated in man, in experimental models and in isolated cell systems. Currently, the mechanism underlying cerebral edema and intracranial hypertension in the presence of FHF is multi-factorial in etiology and only partially understood. The aim of this paper is to review the pathophysiology of cerebral hemodynamic and metabolism changes in FHF in order to improve understanding of intracranial dynamics complication in FHF.

RESUMO O edema cerebral e a hipertensão intracraniana (HIC) são as principais causas de morbidade e mortalidade de pacientes com insuficiência hepática fulminante (IHF). A patogênese dessas complicações tem sido investigada no homem, em modelos experimentais e em sistemas celulares isolados. Atualmente, o mecanismo subjacente ao edema cerebral e HIC na presença de IHF é multifatorial em etiologia e pouco compreendido na literatura. O objetivo deste trabalho é revisar a fisiopatologia das alterações hemodinâmicas e metabólicas cerebrais na IHF, visando melhorar a compreensão da complicação da hemodinâmica encefálica na IHF.

Hipotermia terapêutica como ponte até transplante em pacientes com falência hepática fulminante / Therapeutic hypothermia as a bridge to transplantation in patients with fulminant hepatic failure

Os tópicos mais importantes na falência hepática fulminante são o edema cerebral e a hipertensão intracraniana. Dentre todas as opções terapêuticas, tem sido relatado que a hipotermia sistêmica induzida em níveis entre 33 - 34ºC reduz a elevação da pressão e aumenta o tempo durante o qual os pacientes podem tolerar um enxerto. Esta revisão discutiu as indicações e os efeitos adversos da hipotermia.

The most important topics in fulminant hepatic failure are cerebral edema and intracranial hypertension. Among all therapeutic options, systemic induced hypothermia to 33 - 34ºC has been reported to reduce the high pressure and increase the time during which patients can tolerate a graft. This review discusses the indications and adverse effects of hypothermia.

Clinical Features and Risk Factors for Severe Complications among Patients with Acute Hepatitis A Virus Infection in The Jeonbuk Province of Korea / 대한소화기학회지

BACKGROUND/AIMS: The frequency of symptomatic acute HAV infections in adulthood are increasing in Korea. This study analyzes the clinical severity in patients with acute HAV infection and investigates risk factors associated with three severe complications: prolonged cholestasis, acute kidney injury, and acute liver failure. METHODS: We performed a retrospective analysis of 726 patients diagnosed from January 2006 to December 2010 at three tertiary hospitals in Jeonbuk Province, Republic of Korea with acute HAV infection. RESULTS: In the group of 726 patients, the mean age was 30.3 years, 426 (58.6%) were male, and 34 (4.7%) were HBsAg positive. Severe complications from acute HAV infection occurred as follows: prolonged cholestasis in 33 (4.6%), acute kidney injury in 17 (2.3%), and acute liver failure in 16 (2.2%). Through multivariate analysis, age > or =40 years (OR 2.63, p=0.024) and peak PT (INR) > or =1.5 (OR 5.81, p=0.035) were found to be significant risk factors for prolonged cholestasis. Age > or =40 years (OR 5.24, p=0.002) and female gender (OR 3.11, p=0.036) were significant risk factors for acute kidney injury. Age > or =40 years (OR 6.91, p=0.002), HBsAg positivity (OR 5.02, p=0.049), and peak total bilirubin (OR 1.11, p=0.001) were significant risk factors for acute liver failure. CONCLUSIONS: Age > or =40 years, female gender, HBsAg positivity, peak PT (INR) > or =1.5, and peak total bilirubin were significant risk factors for severe complications in acute HAV infections.

Terapia com células da medula óssea em modelo experimental de insuficiência hepática aguda induzida por acetominofen / Therapy with bone marrow cells in an experimental model of heart failure liver induced by acute acetaminophen

Introdução e objetivos: a insuficiência hepática aguda (IHA), apesar de rara, permanece como uma condição rapidamente progressiva e frequentemente fatal. A intoxicação por acetaminofen (APAP) induz necrose hepática maciça e frequentemente leva à morte por edema cerebral. Terapias celulares são de grande interesse como potenciais tratamentos para IHA. Neste projeto foi avaliado o potencial terapêutico das células mononucleares da medula óssea (CMMO) em um modelo experimental de IHA induzida por APAP em camundongos. Métodos: A IHA foi induzida em camundongos C57Bl/6, previamente submetidos à dieta alcoólica por três semanas, através da administração de APAP na dose de 300 mg/kg por via intraperitoneal. Após a indução da IHA, os camundongos foram transplantados, por via endovenosa, com 107 CMMO...

Introduction and objectives: a cute liver failure (IHA), although rare, remains a rapidly progressive and often fatal condition. Poisoning by acetaminophen (APAP) induces a massive hepatic necrosis and often leads to death by cerebral edema. Cell therapies are of great interest as potential treatments for IHA. In this project we evaluated the therapeutic potential of bone marrow mononuclear cells (BMC) in an experimental model of IHA induced by APAP in mice. Methods: The IHA was induced in C57BL/6 mice previously submitted to the alcohol diet for three weeks by the administration of APAP at a dose of 300 mg / kg, intraperitoneally. After induction of IHA, the mice were transplanted intravenously with 107 BMC...

Acute liver failure: an update

Acute liver failure is a serious medical emergency resulting from various insults that liver sustains. In our setting, viral etiology is the commonest cause. The syndrome results in significant liver damage leading to hepatic encephalopathy, coagulopathy and several other serious clinical consequences. Sepsis, cardio vascular failure and renal failure can result in multi system organ failure and fatal consequences. Management requires identification of etiologic agent as soon as possible. Nursing care in intensive care unit [ICU] setting is recommended. Attention towards correction of electrolyte imbalance, coagulopathy, encephalopathy and systemic complication along with judicious use of antibiotics help in obtaining a favorable outcome. Transplant remains the ultimate management

A new experimental model for acute hepatic failure in rats / Novo modelo experimental em ratos para insuficiência hepática aguda

PURPOSE: To develop a reliable surgical model of acute hepatic failure and hyperammonemia in rats that avoids porto-systemic shunt and bile duct ligation, applicable to hepatic encephalopathy research. METHODS: The pedicles of right lateral and caudate lobes were exposed and clamped. One hour later, the animal was reopened, clamps were released and anterior subtotal hepatectomy (resection of median and left lateral lobes) was performed, comprising 75 percent of liver removal. Four hours after hepatectomy, blood samples and liver tissues were collected from ALF and control groups. RESULTS: Differences between ALF and control groups were significant for ALT, AST, total and direct bilirubin, sodium, potassium, alkaline phosphatasis, gamma-glutamyltransferase and most important, ammonia. Histologically, significant differences were noticed between groups. CONCLUSION: The model is useful for the study of specific aspects of ALF and the development of new therapeutic approaches.

OBJETIVO: Desenvolver um modelo cirúrgico de IHA e hiperamonemia em ratos, que evita o shunt porto-sistêmico e a ligadura do ducto biliar, que seja aplicável à pesquisa de encefalopatia hepática. MÉTODOS: Após anestesia geral e laparotomia mediana, os pedículos dos lobos laterais direito e caudado foram isolados e clampeados. Após 1 hora, o animal foi reaberto, os clampes retirados e foi realizada hepatectomia anterior subtotal (ressecção dos lobos médio e lateral esquerdo), compreendendo a remoção de 75 por cento do parênquima. Quatro horas após a hepatectomia, amostras de sangue e tecido hepático foram coletadas nos grupos IHA e controle. RESULTADOS: Diferenças entre os grupos IHA e controle foram significativas para ALT, AST, bilirrubina total e direta, sódio, potássio, fosfatase alcalina, gama glutamiltransferase e principalmente amônia. Histologicamente, diferenças significativas foram observadas entre os grupos. CONCLUSÃO: O modelo é útil para o estudo de aspectos específicos da IHA e o desenvolvimento de novas abordagens terapêuticas.

Curso de atualização em emergências médicas: aula 18: insuficiência hepatica aguda / Course of updating in medical emergencies: class 18: acute hepatic failure

A insuficiência hepática aguda (IHA) é uma síndrome clínica extremamente grave, de diagnóstico precoce difícil, evolução rápida e alta mortalidade. Nesta revisão buscamos reunir as informações mais atuais sobre classificação, etiologia, diagnóstico e tratamento, discutindo as diversas controvérsias sobre o tema. O diagnóstico da IHA é difícil e engloba o quadro clínico e laboratorial de hepatite aguda (grave), tempo de protrombina alargado, com qualquer alteração do sensório, além de pesquisa cuidadosa na história do paciente, incluindo o uso de medicações ou ervas e presença de diagnóstico prévio de hepatopatia. O diagnóstico etiólogico inclui infecções virais, medicamentos e toxinas, causas cardíacas e vasculares, metabólicas, além da hepatite autoimune, doenças de Wilson e neoplasias. O tratamento da IHA é dado em duas etapas, sendo a primeira constituída pelas medidas de suporte, prevenção e tratamento das complicações, que devem ser oferecidas a todos os pacientes, e a segunda pelas medidas específicas, que serão direcionadas dependendo da etiologia. O transplante hepático é a única terapia definitiva para os pacientes que não conseguem o restabelimento da função hepática.

Acute hepatic failure (AHF) is one extremely serious clinical syndrome of difficult pre-emptive diagnosis, rapid evolution and high mortality. In this review we summarized the current information regarding its classification, etiology, diagnosis and treatment, and discussed the controversies about the issue. The diagnosis of the AHF is difficult and includes laboratorial and clinical findings of severe acute hepatitis, increased prothrombin time and presence of hepatic encephalopathy. It is necessary that a careful history of the patient be obtained especially with respect to utilization of medications, herbs as well as the presence of previous diagnosis of liver disease. The possible etiologies include viral infections, cardiac and vascular affections, medications and toxins, metabolic causes, auto-immune hepatitis, Wilson's disease and neoplasm. The treatment of AHF requires support measures, prevention and treatment of complications that must be offered all patients and specific measures which should be offered according to the etiology of AHF. Liver transplant is the only definitive therapy for patients who do not recover the hepatic function.

Clinical features of patients with fulminant hepatitis A requiring emergency liver transplantation: comparison with acute liver failure due to other causes / 대한간학회지

BACKGROUND/AIMS: According to recent prevalence of hepatitis A virus (HAV) infection, acute liver failure (ALF) due to HAV infection is observed frequently in parallel. The aim of this study was to elucidate the clinical, laboratory, and pathologic features of patients who have undergone emergency liver transplantation (LT) due to fulminant HAV infection. METHODS: Clinical, laboratory, and pathologic data of 11 transplant recipients with anti-HAV IgM-positive ALF between December 2007 and May 2009 were analyzed, and compared with data of 10 recipients who underwent LT for the management of ALF due to other causes. RESULTS: The median age of the patients with HAV-related ALF was 34 years (range: 15-43 years). The levels of hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and creatinine were higher and the level of bilirubin was lower in the HAV-related ALF group than in the other group (P=0.005, 0.001, 0.001, 0.010, and 0.003, respectively). The time from the onset of initial symptoms to the development of encephalopathy was shorter in the HAV-related ALF group than in the other group (median 5 days, range: 4-13 days; P<0.001). In patients with HAV-related ALF, laboratory findings and clinical prognostic parameters including the Acute Liver Failure Study Group prognostic index, King's College criteria, and model for endstage liver disease (MELD) and Child-Pugh scores were not associated with the grade of hepatic encephalopathy or time of progression to encephalopathy. CONCLUSIONS: The results of this study indicate that the clinical condition of patients with HAV-related ALF requiring emergency LT aggravates rapidly. Prognostic parameters are not sufficient for discriminating transplant candidates in patients with fulminant hepatitis A.

A case of hemophagocytic syndrome complicated by acute viral hepatitis A infection / 대한간학회지

Hemophagocytic syndrome (HPS) is a rare but serious condition that is histopathologically characterized by activation of macrophage or histiocytes with hemophagocytosis in bone marrow and reticuloendothelial systems. Clinically it presents with high fever, hepatosplenomegaly, pancytopenia, liver dysfunction, and hyperferritinemia. Hepatitis A virus is a very rare cause of secondary HPS. We report a case of a 22-year-old woman infected by hepatitis A virus who was consequently complicated with HPS. She presented typical clinical features of acute hepatitis A, and showed clinical and biochemical improvements. However, HPS developed as a complication of acute hepatitis A and the patient died of intraperitoneal bleeding caused by hepatic decompensation and disseminated intravascular coagulation.

Hipotermia intravascular inducida en el manejo de la hipertensión intracraneana en insuficiencia hepática aguda: caso clínico / Intravascular hypothermia for the management of Intracranial hypertension in acute liver failure: case report

Acute liver failure has a mortality rate in excess of 80 percent. Most deaths are attributed to brain edema with intracranial hypertension and herniation of structures, where ammonium plays a major role in its generation. We report an 18 year-old female with a fulminant hepatic failure caused by virus A infection. The patient developed a profound sopor and required mechanical ventilation. A CT scan showed the presence of brain edema and intracranial hypertension. A Raudemic® catheter was inserted to measure intracranial pressure and brain temperature. Intracranial hypertension became refractory and intravascular hypothermia was started, reducing brain temperature to 33°C. Seventy two hours later, a liver transplantation was performed. After testing graft perfusion, rewarming was started, completing 122 hours of hypothermia at 33°C. The patient was discharged in good conditions after 69 days of hospitalization.

Intraparenchymal intracranial pressure monitoring in patients with acute liver failure / Monitoreo intraparenquimatoso de presión intracraneana en pacientes con falla hepática aguda

BACKGROUND: Elevated intracranial pressure (ICP) is a common cause of death in acute liver failure (ALF) and is determinant for decision-making regarding the timing of liver transplantation. The recommended type ICP monitoring device is controversial in ALF patients. Epidural devices had less risk of hemorrhagic complications, but they are less reliable than intraparenchymal ones. METHOD: Twenty-three patients with ALF were treated, and 19 of them received a liver transplant. Seventeen patients had ICP monitoring because of grade III-IV encephalopathy. All patients received fresh plasma (2-3 units) before and during placing the intraparenchymal device. RESULTS: Eleven cases (64.7 percent) had elevated ICP, and 6 patients (35.2 percent) had normal values. One patient (5.9 percent) had an asymptomatic small intraparenchymal haemorrhage <1cm³ in CTscan, which did not prevent the liver transplantation. CONCLUSION: In our experience, intraparenchymal ICP monitoring in patients with ALF seems to be an accurate method with a low risk of complications.

ANTECEDENTES: La presión intracraneana elevada (PIC) es una causa frecuente de muerte en la falla hepática aguda (FHA) y es determinante para la toma de decisiones respecto del momento del transplante hepático. El tipo de dispositivo para el monitoreo de OIC es controversial em los pacientes em FHA. Los dispositivos epidurales tienen menos riesgo de complicaciones hemorrágicas, pero son menos confiables que los intraparenquimatosos. MÉTODO: Veintitrés pacientes con FHA fueron tratados, y 19 de ellos recibieron un transplante hepático. diecisiete pacientes tuvieron monitoreo de PIC debido a encefalopatía grado III-IV. Todos los pacientes recibieron plasma fresco (2-3 unidades) antes y durante la colocación de la fibra intraparenquimatosa. RESULTADOS: Once casos (64.7 por ciento) tuvieron PIC elevada, y 6 pacientes (35.2 por ciento) tuvieron valores normales. Un paciente (5.9 por ciento) tuvo una pequeña hemorragia intraparenquimatosa asintomática <1cm³ en TAC, la cual no impidió el transplante hepático. CONCLUSIÓN: En nuestra experiencia, el monitoreo intraparenquimatoso de presión intracraneana en pacientes con FHA parece ser un método preciso y con bajo riesgo de complicaciones.

Combined liver-kidney transplantation in a patient with acute liver failure coexisting with acute renal failure.

Acute renal failure (ARF) is common among patients with liver failure awaiting liver transplantation due to the increased waiting time for available liver grafts and extended survival from improved intensive care. The role of combined liver and kidney transplantation (LKT) in this situation is quite controversial. A case of acute liver failure (ALF) complicated with ARF is reported. Non-A, non-B hepatitis was the cause of ALF. He had hemodialysis for one month before transplantation. Combined LKT was performed because of prolonged pre-transplant hemodialysis and the potential of irreversible renal failure. Severe impairment of both native kidneys was confirmed by renal scan at 6 months after transplantation. Combined LKT may be needed for patients with acute liver failure complicated with prolonged acute renal failure.

Dengue viral infections as a cause of encephalopathy.

The aim of this study was to determine the clinical characteristics and poor prognostic factors associated with high mortality in dengue encephalopathy. Fifteen patients with confirmed dengue infections, who developed encephalopathy, were recruited from two tertiary care hospitals in Colombo, Sri Lanka. Among the factors that contributed to encephalopathy were: Acute liver failure (73%), electrolyte imbalances (80%) and shock (40%). Five (33.3%) patients developed seizures. Disseminated intravascular coagulation was seen in five (33.3%). Secondary bacterial infections were observed in 8 (53.3%) of our patients. The overall mortality rate was 47%.

Factores pronósticos en insuficiencia hepática aguda / Prognoses factors in acute hepatic insufficiency

Introducción. La insuficiencia hepática aguda (IHA) es un síndrome poco frecuente aunque con elevada mortalidad. El objetivo de este estudio fue determinar las características clínico-epidemiológicas de la IHA. Materiales y métodos: Estudio abierto, prospectivo, descriptivo de pacientes con diagnóstico de IHA hospitalizados en la Unidad de Hígado del HNERM desde Febrero 1999 hasta Enero 2003. Resultados. Se estudiaron 15 casos. La edad promedio fue 63 años (30-81), la relación M/F fue 2/1. La etiología fue hepatitis viral B (53.3 por ciento), reacciones tóxicas por idiosincracia (20 por ciento), indeterminada (20 por ciento) y hepatitis A (6.7 por ciento). En el momento del diagnóstico el 80 por ciento presentó encefalopatía Grado I y el 20 por ciento encefalopatía III. El 13.3 por ciento no tuvo ascitis y el 86.7 por ciento ascitis leve-moderada. El 53.3 por ciento tenía como antecedente una enfermedad crónica (diabetes, insuficiencia renal crónica, cardiopatía y otros). Los valores de laboratorio fueron: albúmina media 2.5 gr./dl, bilirrubina media 25.9 mg/dl, tiempo de protrombina media 29'' y Factor V media 40.7 por ciento. Las complicaciones más frecuentes fueron la sepsis y el edema cerebral. La mortalidad global fue 80 por ciento. El tiempo de sobrevida media fue 16.6 días. Conclusiones. La mayoría de casos tuvieron más de 60 años. La principal causa de IHA fue la hepatitis viral B, esta enfermedad puede prevenirse con la inmunización activa. La infección y el edema cerebral fueron causas importantes de muerte. La IHA aunque rara, es una entidad rápidamente progresiva y fatal.

Introduction. Acute Hepatic Insufficiency (AHI) is a rare syndrome but has a highmortality rate. The purpose of this study was to determine the clinico-epidemiological characteristics of AHI. Materials and Methods. Open study, prospective, descriptive of patients diagnosed with AHI in the Liver Unit of the Edgardo Rebagliati Martins State Hospital (HNERM) from February 1999 until January 2003. Results. Fifteen (15) cases were studied. The average age was 63 (30-81), the M-Fratio was 2/1. The diagnosis was viral Hepatitis B (53.3 per cent), toxic idiosyncratic reactions(20 per cent), undetermined (20 per cent) and Hepatitis A (6.7 per cent). At the time of diagnosis 80 per cent had Grade I encephalopathyand 20 per cent Grade III encephalopathy; 13.3 per cent did not have ascites and 86.7 per cent had mild-moderate ascites; 53.3 per cent had a history of chronic illness (diabetes, chronic renal insufficiency, cardiopathy and others). Average laboratory values were: albumin 2.5 gr./dl, bilirubin 25.9 mg/dl, prothrombin time 29” and Factor V 40.7 per cent. The most frequent complications were sepsis and cerebral oedema. Global mortality was 80 per cent. The average survival time was 16.6 days.Conclusions. In most cases the patients were over 60 years of age. The main cause of AHI was viral Hepatitis B, a disease which can be prevented with active immunisation. Infection and cerebral oedema were common causes of death. AHI, although rare, is a rapidly degenerative and fatal condition.

Acute liver failure.

Acute liver failure in children is a rare but potentially fatal disease. Causes of ALF in neonatal period are different from those in early or late childhood. Despite the improvement in the paediatric intensive care, liver transplantation remains the only effective treatment. Use of newer treatment modalities (liver assist devices and hepatocyte transplantation) is still in experimental phase. Management requires early recognition, prompt diagnosis of treatable condition, supportive therapy and prevention of complications hence these children should ideally be treated in a specialist unit.

Hepatite fulminante: do diagnóstico ao transplante de fígado / Fulminant hepatitis: from diagnosis to liver transplantation

A insuficiência aguda grave do fígado é rara, e potencialmente letal. A identificação precoce dessa síndrome, o manejo adequado dentro do ambiente de terapia intensiva e a transferência para centros transplantadores são mandatórios. A realização do transplante hepático nos pacientes com mau prognóstico salva esses indivíduos de uma morte quase certa. O autor revisa as estratégias atuais do suporte à vida nesses pacientes críticos de maneira multidisciplinar, enfatizando a monitorização invasiva para definir o momento oportuno do transplante hepático.