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1.
BMJ Case Rep ; 17(3)2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38521515

RESUMO

This case report presents a primigravida in her 20s with a history of seizure disorder and chronic cholecystitis, who presented at 30 weeks and 6 days of gestation with upper abdominal pain, fever and vomiting. Initially diagnosed with acute calculous cholecystitis, the patient's condition rapidly deteriorated, resulting in fetal demise and the development of severe complications. Subsequent investigations revealed an enlarged fatty liver and signs of acute liver failure. The diagnosis of acute fatty liver of pregnancy was initially considered but later ruled out, and the patient was diagnosed with hepatitis E based on positive anti-hepatitis E virus IgM antibodies. Prompt termination of pregnancy was performed, followed by intensive care management. After a prolonged hospital stay, the patient recovered and was discharged in stable condition. This case emphasises the importance of considering hepatitis E as a potential cause of acute liver failure in pregnant women and the need for early recognition and multidisciplinary management to achieve favourable outcomes.


Assuntos
Colecistite , Fígado Gorduroso , Hepatite E , Falência Hepática Aguda , Complicações na Gravidez , Feminino , Humanos , Gravidez , Colecistite/complicações , Fígado Gorduroso/complicações , Hepatite E/complicações , Hepatite E/diagnóstico , Falência Hepática Aguda/complicações , Adulto
2.
Resuscitation ; 197: 110136, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38336284

RESUMO

RATIONALE: Apart from hypoxic hepatitis (HH), the hepatic consequences of out-of-hospital cardiac arrest (OHCA) have been little studied. This cohort study aimed to investigate the characteristics of liver dysfunction resulting from OHCA and its association with outcomes. METHODS: Among the conventional static liver function tests used to define acute liver failure (ALF), we determined which one correlated more closely with the reference indocyanine green (ICG) clearance test in a series of OHCA patients from the CYRUS trial (NCT01595958). Subsequently, we assessed whether ALF, in addition to HH (i.e., acute liver injury), was an independent risk factor for death in a large cohort of OHCA patients admitted to two intensive care units between 2007 and 2017. RESULTS: ICG clearance, available for 22 patients, was impaired in 17 (77.3%) cases. Prothrombin time (PT) ratio was the only static liver function test that correlated significantly (r = -0.66, p < 0.01) with ICG clearance and was therefore used to define ALF, with the usual cutoff of < 50%. Of the 418 patients included in the analysis (sex ratio: 1.4; median age: 64 [53-75] years; non-shockable rhythm: 73%), 67 (16.0%) presented with ALF, and 61 (14.6%) had HH at admission. On day 28, 337 (80.6%) patients died. Following multivariate analysis, ALF at admission, OHCA occurring at home, absence of bystander, non-cardiac cause of OHCA, low-flow duration ≥ 20 min, and SOFA score excluding liver subscore at admission were independently associated with day 28 mortality. CONCLUSIONS: ALF occurred frequently after OHCA and, unlike HH, was independently associated with day 28 mortality.


Assuntos
Reanimação Cardiopulmonar , Hepatite , Falência Hepática Aguda , Parada Cardíaca Extra-Hospitalar , Humanos , Pessoa de Meia-Idade , Parada Cardíaca Extra-Hospitalar/terapia , Parada Cardíaca Extra-Hospitalar/complicações , Estudos de Coortes , Falência Hepática Aguda/complicações , Hepatite/complicações
3.
Transpl Infect Dis ; 26(1): e14200, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38010711

RESUMO

BACKGROUND: Acute liver failure (ALF) is a component of multisystem organ failure that causes severe liver dysfunction in patients without underlying chronic liver disease. The patients with ALF are prone to have infections, including bacteremia. However, studies of the infectious impact for post liver transplantation (LT) in pediatric ALF are limited. We aimed to evaluate our current practice for pediatric LT cases of ALF with preoperative bacteremia. METHODS: The records of all patients under 18 years old undergoing LT for ALF in our center from November 2005 to December 2021 were collected. They were divided into two groups: those with a preoperative bloodstream infection (BSI) and those without (NBSI). We compared the preoperative status and also reviewed the details of the BSI group. Intraoperative course and postoperative outcomes were also compared. RESULTS: There were 19 BSI patients and 66 NBSI patients. One BSI case was detected on the day of LT. This patient had no changes in vital signs and general condition. After evaluation and therapeutic intervention by pediatric infectious disease specialists, LT was performed on the same day. Five cases developed septic shock at the time of detection of BSI. All BSI patients were in stable condition on the operation day with proper interventions. There were no significant differences in mortality and hospital stay between both groups. CONCLUSIONS: LT might be able to be performed for pediatric ALF even with positive blood cultures. In addition, appropriate therapeutic intervention by specialists and patient's stable condition before LT are essential.


Assuntos
Bacteriemia , Doenças Transmissíveis , Falência Hepática Aguda , Transplante de Fígado , Sepse , Humanos , Criança , Adolescente , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Bacteriemia/etiologia , Falência Hepática Aguda/cirurgia , Falência Hepática Aguda/complicações
4.
Clin J Gastroenterol ; 17(1): 84-92, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37773425

RESUMO

A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration. Brain-dead donor liver transplantation was performed on hospital day 30. Since then, the patient has remained well.


Assuntos
Síndrome de Budd-Chiari , Falência Hepática Aguda , Transplante de Fígado , Masculino , Humanos , Adulto Jovem , Adulto , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/efeitos adversos , Doadores Vivos , Falência Hepática Aguda/cirurgia , Falência Hepática Aguda/complicações , Encéfalo
5.
Ann Hepatol ; 29(2): 101167, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37802415

RESUMO

INTRODUCTION AND OBJECTIVES: Acute liver failure, also known as fulminant hepatic failure (FHF), includes a spectrum of clinical entities characterized by acute liver injury, severe hepatocellular dysfunction and hepatic encephalopathy. The objective of this study was to assess cerebral autoregulation (CA) in 25 patients (19 female) with FHF and to follow up with seventeen of these patients before and after liver transplantation. PATIENTS AND METHODS: The mean age was 33.8 years (range 14-56, SD 13.1 years). Cerebral hemodynamics was assessed by transcranial Doppler (TCD) bilateral recordings of cerebral blood velocity (CBv) in the middle cerebral arteries (MCA). RESULTS: CA was assessed based on the static CA index (SCAI), reflecting the effects of a 20-30 mmHg increase in mean arterial blood pressure on CBv induced with norepinephrine infusion. SCAI was estimated at four time points: pretransplant and on the 1st, 2nd and 3rd posttransplant days, showing a significant difference between pre- and posttransplant SCAI (p = 0.005). SCAI peaked on the third posttransplant day (p = 0.006). Categorical analysis of SCAI showed that for most patients, CA was reestablished on the second day posttransplant (SCAI > 0.6). CONCLUSIONS: These results suggest that CA impairment pretransplant and on the 1st day posttransplant was re-established at 48-72 h after transplantation. These findings can help to improve the management of this patient group during these specific phases, thereby avoiding neurological complications, such as brain swelling and intracranial hypertension.


Assuntos
Encefalopatia Hepática , Falência Hepática Aguda , Transplante de Fígado , Humanos , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Transplante de Fígado/efeitos adversos , Encefalopatia Hepática/diagnóstico por imagem , Encefalopatia Hepática/etiologia , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/cirurgia , Falência Hepática Aguda/complicações , Homeostase/fisiologia
6.
BMJ Case Rep ; 16(11)2023 Nov 02.
Artigo em Inglês | MEDLINE | ID: mdl-37918944

RESUMO

We present the case of a female patient in her 40s who presented with jaundice, orthopnoea, paroxysmal nocturnal dyspnoea and bilateral pedal oedema. After extensive investigations, she was diagnosed with hepatic dysfunction, dilated cardiomyopathy (DCM) and coeliac axis thrombosis. Her case was further complicated with episodes of torsades de pointes due to metabolic disturbance, with consequent sudden cardiac arrest. In this case report, we explore the clinical features, pathophysiology and treatment of acute hepatic failure and coeliac axis thrombosis, secondary to DCM and alcoholic liver disease.


Assuntos
Cardiomiopatia Dilatada , Insuficiência Cardíaca , Falência Hepática Aguda , Trombose , Torsades de Pointes , Humanos , Feminino , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/diagnóstico , Torsades de Pointes/complicações , Morte Súbita Cardíaca/etiologia , Trombose/complicações , Falência Hepática Aguda/etiologia , Falência Hepática Aguda/complicações , Insuficiência Cardíaca/etiologia
7.
Medicine (Baltimore) ; 102(45): e35931, 2023 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-37960809

RESUMO

Acute kidney injury (AKI) is a common complication of acute liver failure (ALF); but its pathogenesis is unknown. ALF was divided into 2 subgroups; ALF with hepatic coma, which corresponds to ALF in the US and Europe, and ALF without hepatic coma. AKI has been shown to worsen the prognosis of ALF patients with hepatic coma; however, its prognostic significance in ALF without hepatic coma remains unknown. A single-center retrospective study of 174 patients with ALF was performed. AKI was defined according to KDIGO criteria. AKI developed in 29 (66.0%) of 44 ALF patients with hepatic coma and 27 (38.5%) of 130 ALF patients without hepatic coma. Systemic inflammatory response syndrome (SIRS) was found to be significantly associated with AKI incidence in ALF patients (P < .001). Tumor necrosis factor-alpha (TNF-α) was found to be significantly associated with the presence and severity of AKI (P = .0039 and P = .0140, respectively). On multivariate analysis, TNF-α was an independent risk factor linked with AKI (P = .0103). Even in the absence of hepatic coma, the transplant-free survival rate of ALF was significantly associated with the presence and severity of AKI. Even when hepatic coma is absent, AKI complicated in ALF is strongly associated with TNF-α and worsens the transplant-free survival rate. Before the onset of hepatic coma, plasma exchange, or extracorporeal blood purification to remove inflammatory cytokines should be considered in ALF patients.


Assuntos
Injúria Renal Aguda , Encefalopatia Hepática , Falência Hepática Aguda , Humanos , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/patologia , Falência Hepática Aguda/complicações , Prognóstico , Estudos Retrospectivos , Síndrome de Resposta Inflamatória Sistêmica/complicações , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Fator de Necrose Tumoral alfa
8.
PLoS Negl Trop Dis ; 17(10): e0011352, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37844119

RESUMO

BACKGROUND: Leptospirosis is a tropical disease associated with life threatening complications. Identifying clinical and investigation-based parameters that predict mortality and morbidity is vital to provide optimal supportive care. METHODS: We conducted an observational study in an endemic setting, in the southern Sri Lanka. Consecutive patients having complicated leptospirosis were recruited over 18 months. Clinical, investigational and treatment data were collected and the predictors of mortality were analysed. RESULTS: Out of 88 patients having complicated leptospirosis, 89% were male. Mean age was 47yrs (±16.0). Among the total major complications 94.3% had acute kidney injury, 38.6% pulmonary haemorrhages, 12.5% fulminant hepatic failure, 60.2% hemodynamic instability and 33% myocarditis. An acute significant reduction of haemoglobin (Hb) was observed in 79.4% of patients with pulmonary haemorrhage. The mean of the highest haemoglobin reduction in patients with pulmonary haemorrhage was 3.1g/dL. The presence of pulmonary haemorrhage (PH) and hemodynamic instability within first 48 hours of admission significantly predicted mortality (p<0.05) in severe leptospirosis. Additionally, within first 48 hours of admission, elevated SGOT (AST), presence of atrial fibrillation, presence of significant haemoglobin reduction, higher number of inotropes used, prolonged shock, invasive ventilation and admission to ICU significantly predicted mortality. Out of major complications during the first week after admission, pulmonary haemorrhage and fulminant hepatic failure (FHF) combination had significant adjusted odds of mortality (OR = 6.5 and 4.8, p<0.05). Six patients with severe respiratory failure due to PH underwent ECMO and four survived. The overall mortality in complicated leptospirosis was 17%. In PH and FHF, the mortality rate was higher reaching 35.4% and 54.5%, respectively. CONCLUSIONS: Within first 48 hours of admission, major complications such as pulmonary haemorrhage and haemodynamic instability and other parameters such as atrial fibrillation, acute haemoglobin reduction, elevated SGOT level could be used as early parameters predictive of mortality in severe leptospirosis. PH and FHF during the first week of admission in leptospirosis are associated with high morbidity and mortality requiring prolonged ICU care and hospitalisation. Above parameters could be used as parameters indicating severity for triaging and intensifying treatment. Using ECMO is a plausible treatment option in patients with severe pulmonary haemorrhage.


Assuntos
Fibrilação Atrial , Leptospirose , Falência Hepática Aguda , Pneumopatias , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Aspartato Aminotransferases , Fibrilação Atrial/complicações , Causas de Morte , Hemoglobinas , Hemorragia/etiologia , Leptospirose/epidemiologia , Falência Hepática Aguda/complicações , Pneumopatias/complicações , Sri Lanka/epidemiologia , Adulto
9.
Am J Case Rep ; 24: e941270, 2023 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-37870994

RESUMO

BACKGROUND Acute liver failure (ALF) associated with malignant lymphoma is a rare condition with non-specific clinical and radiological features. Here, we describe an unusual case of ALF due to DLBCL with an image negative on presentation posing diagnostic difficulty. CASE REPORT A 74-year-old man was admitted to our hospital with abdominal pain. Radiological and laboratory investigations revealed lymphadenopathy with mildly elevated transaminitis and alkaline phosphates levels. A right upper-quadrant ultrasound showed heterogeneous hepatic parenchyma. Eight days later, he had worsening abdominal pain. He was found to have altered mental status and asterixis. His liver function was worsened with ALT 101, AST 328, lactic acid 4.2, total bilirubin 2.5, INR 6.35, and ammonia level 117 µmol/L. He continued to deteriorate with worsening lactic acidosis, coagulopathy, severe anemia, elevated liver enzymes, and thrombocytopenia. Unfortunately, the patient died of multi-organ failure on the 14th day of hospitalization. The autopsy findings revealed DLBCL involving multiple organs, including the liver, lung, bone marrow, and multiple lymph nodes. Despite an extensive diagnostic workup, an underlying diagnosis was unable to be established antemortem. CONCLUSIONS We describe a case of ALF linked to DLBCL discovered at autopsy. The non-specific clinical and radiological features of this condition make diagnosis challenging, and the prognosis is often poor. Further research and awareness are needed to improve the early detection and management of ALF associated with malignant lymphoma. By expanding the literature on this topic, we aim to improve outcomes and optimize patient care in similar clinical scenarios.


Assuntos
Acidose Láctica , Falência Hepática Aguda , Linfoma Difuso de Grandes Células B , Masculino , Humanos , Idoso , Acidose Láctica/diagnóstico , Acidose Láctica/etiologia , Falência Hepática Aguda/etiologia , Falência Hepática Aguda/complicações , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico , Dor Abdominal
10.
BMJ Case Rep ; 16(10)2023 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-37813556

RESUMO

Hepatitis A is one of the most common causes of acute viral hepatitis in children. Immunological manifestations involving the nervous system are rare with hepatitis A infection. We report a case of a toddler who presented with acute liver failure secondary to hepatitis A infection. The child showed clinical and laboratory improvement initially with conservative management. However, after the initial improvement, she developed acute-onset ptosis along with areflexia. Serological and neurophysiological tests revealed the occurrence of ocular variant Guillain-Barré syndrome and ocular myasthenia gravis, which was only partially responsive to treatment (intravenous immunoglobulin and pyridostigmine). A sudden clinical deterioration was noted after the onset of ptosis. She succumbed on day 40 of hospitalisation due to hospital-acquired infection along with the primary hepatic pathology. This is a rare coincidental presentation of acute viral hepatitis A infection with autoimmune manifestations.


Assuntos
Blefaroptose , Síndrome de Guillain-Barré , Vírus da Hepatite A , Hepatite A , Falência Hepática Aguda , Miastenia Gravis , Feminino , Humanos , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/diagnóstico , Hepatite A/complicações , Hepatite A/diagnóstico , Miastenia Gravis/complicações , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamento farmacológico , Blefaroptose/complicações , Doença Aguda , Falência Hepática Aguda/complicações
12.
Crit Care Med ; 15(5): 657-676, 20230501.
Artigo em Inglês | BIGG - guias GRADE | ID: biblio-1434968

RESUMO

To develop evidence-based recommendations for clinicians caring for adults with acute liver failure (ALF) or acute on chronic liver failure (ACLF) in the ICU. The guideline panel comprised 27 members with expertise in aspects of care of the critically ill patient with liver failure or methodology. We adhered to the Society of Critical Care Medicine standard operating procedures manual and conflict-of-interest policy. Teleconferences and electronic-based discussion among the panel, as well as within subgroups, served as an integral part of the guideline development. In part 2 of this guideline, the panel was divided into four subgroups: neurology, peri-transplant, infectious diseases, and gastrointestinal groups. We developed and selected Population, Intervention, Comparison, and Outcomes (PICO) questions according to importance to patients and practicing clinicians. For each PICO question, we conducted a systematic review and meta-analysis where applicable. The quality of evidence was assessed using the Grading of Recommendations Assessment, Development, and Evaluation approach. We used the evidence to decision framework to facilitate recommendations formulation as strong or conditional. We followed strict criteria to formulate best practice statements. We report 28 recommendations (from 31 PICO questions) on the management ALF and ACLF in the ICU. Overall, five were strong recommendations, 21 were conditional recommendations, two were best-practice statements, and we were unable to issue a recommendation for five questions due to insufficient evidence. Multidisciplinary, international experts formulated evidence-based recommendations for the management ALF and ACLF patients in the ICU, acknowledging that most recommendations were based on low quality and indirect evidence.


Assuntos
Humanos , Adulto , Falência Hepática Aguda/complicações , Falência Hepática Aguda/tratamento farmacológico , Antibioticoprofilaxia , Hiperamonemia/sangue , Solução Salina Hipertônica/uso terapêutico , Albuminas/uso terapêutico
13.
Pediatr Transplant ; 27(4): e14524, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37013855

RESUMO

BACKGROUND: Neurological impairment is not rare in infants with acute liver failure (ALF). This study aimed to investigate the perioperative risk factors for neurological impairment following liver transplantation (LT) in infantile ALF. METHODS: Retrospective analysis was performed in infants who were younger than 1 year with ALF who subsequently underwent LT at our hospital between January 2005 and December 2016. Patients were considered to have neurological impairment if the Pediatric Cerebral Performance Category score was between 2 and 5 at the age of 6 years. A comparison between the groups of infants with and without neurological impairment was performed, and factors with p < .10 in the comparison were analyzed using univariate logistic regression analysis for neurological impairment. RESULTS: Twenty-six infants survived until 6 years of age, and 31% (8/26) of them had neurological impairment. Patients with neurological impairment were significantly younger in age at ALF onset, had significantly higher pre-LT bilirubin and prothrombin time/international normalized ratio, and stayed significantly longer in the intensive care unit than those without neurological impairment. Total bilirubin (odds ratio (OR) = 1.12, 95% confidence interval (CI) 1.02-1.22, p = .012), indirect bilirubin (OR = 1.10, 95% CI 1.01-1.20, p = .025), direct bilirubin (OR = 1.22, 95% CI 1.01-1.47, p = .040), and age in month at ALF (OR = 0.76, 95% CI 0.58-0.999, p = .049) showed significant association with neurological impairment. CONCLUSIONS: High pre-LT peak bilirubin value and younger age at ALF onset can be perioperative risk factors for neurological impairment after LT in infantile ALF.


Assuntos
Falência Hepática Aguda , Transplante de Fígado , Humanos , Criança , Lactente , Transplante de Fígado/efeitos adversos , Resultado do Tratamento , Estudos Retrospectivos , Fatores de Risco , Falência Hepática Aguda/complicações , Falência Hepática Aguda/cirurgia , Bilirrubina , Prognóstico
14.
J Hepatol ; 79(1): 43-49, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36822480

RESUMO

BACKGROUND & AIMS: In the year 2022, an outbreak of indeterminate acute hepatitis and indeterminate paediatric acute liver failure (ID-PALF) in association with adenoviraemia in immunocompetent children was reported in the UK. We postulate that this association is not a new disease in immunocompetent children. METHODS: Children with acute hepatitis during the outbreak who were referred to King's College Hospital, London for advice and management were included in the study. Data on the frequency of ID-PALF in 2022, as well as transplantation rates and the association with adenovirus infection, were obtained from electronic health records. The clinical presentation, histology and outcomes of children with ID-PALF and adenoviraemia in 2017-2021 were compared with those in 2022. RESULTS: From January to June 2022, 65 patients with acute hepatitis were referred. Ten children were admitted with ID-PALF. ID-PALF constituted 26% of all PALF cases in 2017-2021, in contrast to 58.8% during the 2022 outbreak. During the outbreak, adenoviraemia was present in 52% of children with acute hepatitis without liver failure (in whom adenoviraemia test results were available) and in 100% of ID-PALF cases. Adenoviraemia was seen in immunocompetent children in 6/13 (46%) of all ID-PALF cases between 2017-2019, with a clear absence of adenoviraemia in the 6 ID-PALF cases during 2020-2021. Compared to ID-PALF with adenoviraemia in 2017-2019 (n = 6), ID-PALF with adenoviraemia during the outbreak (n = 10) was associated with more frequent hepatic encephalopathy, hypotension requiring vasoactive medications and higher plasma ammonia levels (admission and peak), with similar native liver survival. CONCLUSIONS: The recent outbreak of ID-PALF with adenoviraemia in immunocompetent children does not appear to be a new disease, contrary to perception and other reports. The frequency of such cases over the years could be linked to background rates of adenovirus infections. IMPACT AND IMPLICATIONS: Indeterminate paediatric acute liver failure (ID-PALF) associated with adenoviraemia in immunocompetent children is not a new disease specific to 2022. The exclusive role of human adenovirus infection in the causation of this outbreak of acute hepatitis seems unlikely. Indeed, we provide histological data from explants in transplanted patients that do not support direct viral cytotoxicity. The disease is probably mediated by immunological injury directed towards adenovirus infection and/or adeno-associated virus-2.


Assuntos
Infecções por Adenoviridae , Hepatite , Falência Hepática Aguda , Humanos , Criança , Falência Hepática Aguda/etiologia , Falência Hepática Aguda/complicações , Infecções por Adenoviridae/complicações , Doença Aguda , Surtos de Doenças
15.
Zhonghua Er Ke Za Zhi ; 61(1): 66-69, 2023 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-36594124

RESUMO

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.


Assuntos
Falência Hepática Aguda , Atrofia Óptica , Anomalia de Pelger-Huët , Criança , Masculino , Humanos , Feminino , Lactente , Pré-Escolar , Estudos Retrospectivos , Proteínas de Neoplasias/genética , Atrofia Óptica/genética , Anomalia de Pelger-Huët/genética , Falência Hepática Aguda/genética , Falência Hepática Aguda/cirurgia , Falência Hepática Aguda/complicações
16.
Clin J Gastroenterol ; 16(2): 254-262, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36449216

RESUMO

Acute pancreatitis is an uncommon occurrence in acute liver failure. Furthermore, such cases are rarely complicated by parenchymal hemorrhages. Herein, we report the case of a 69-year-old male patient with multiple pancreatic parenchymal hemorrhages concomitant with acute liver failure. The patient underwent conservative treatment for acute liver failure caused by hepatitis B virus infection. Plain computed tomography on the 30th day revealed two high-density mass lesions in the pancreatic body and tail, which were suspected to be multiple pancreatic parenchymal hemorrhages. Despite restarting gabexate mesylate, the patient died of multiple organ failure on the 49th day. The clinical information of the present case and our literature review of 61 similar cases in 43 case reports identified via a systematic keyword search of the PubMed database, which described acute pancreatitis concomitant with acute hepatitis and acute liver failure, will aid physicians in the diagnosis and treatment of this rare condition.


Assuntos
Falência Hepática Aguda , Pancreatite , Masculino , Humanos , Idoso , Pancreatite/complicações , Pancreatite/diagnóstico por imagem , Doença Aguda , Pâncreas , Hemorragia , Falência Hepática Aguda/complicações
17.
Chinese Journal of Pediatrics ; (12): 66-69, 2023.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-970238

RESUMO

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.


Assuntos
Criança , Masculino , Humanos , Feminino , Lactente , Pré-Escolar , Estudos Retrospectivos , Proteínas de Neoplasias/genética , Atrofia Óptica/genética , Anomalia de Pelger-Huët/genética , Falência Hepática Aguda/complicações
18.
BMC Infect Dis ; 22(1): 938, 2022 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-36514003

RESUMO

BACKGROUND: Dengue haemorrhagic fever is a severe form of acute dengue infection characterized by leakage of plasma through capillaries into body spaces resulting in circulatory insufficiency leading to shock. Despite varying degrees of liver involvement occurring in acute dengue infection, intrahepatic cholestasis is very rare in the literature with only two cases reported so far. We report a challenging case of a middle-aged woman with DHF complicated by acute liver failure, coagulopathy, acute renal failure and prolonged intrahepatic cholestasis. She was successfully managed in the intensive care unit with supportive therapy, Cytosorb® and therapeutic plasma exchange. CASE PRESENTATION: A 54-year-old Sri Lankan obese woman with multiple comorbidities presented with fever, headache, vomiting and generalized malaise for 3 days and was diagnosed with dengue haemorrhagic fever. Despite the standard dengue management, she clinically deteriorated due to development of complications such as, acute liver injury, intrahepatic cholestasis and acute renal injury. Acute liver failure was evidenced by transaminitis, lactic acidosis, coagulopathy with pervaginal bleeding and severe encephalopathy necessitating elective intubation and mechanical ventilation. She was immediately transferred to intensive care facilities where she underwent supportive management for liver failure, continuous renal replacement therapy coupled with cytosorb and therapeutic plasma exchange with which she made a remarkable recovery. CONCLUSION: Acute liver failure with a prolonged phase of intrahepatic cholestasis is a very rare complication of acute dengue illness which is sparsely documented in medical literature so far. This patient was managed successfully with supportive therapy, aided by cytoSorb hemo-adsorption and therapeutic plasma exchange.


Assuntos
Injúria Renal Aguda , Colestase Intra-Hepática , Dengue , Falência Hepática Aguda , Dengue Grave , Pessoa de Meia-Idade , Feminino , Humanos , Dengue Grave/complicações , Dengue Grave/terapia , Dengue Grave/diagnóstico , Troca Plasmática/efeitos adversos , Falência Hepática Aguda/complicações , Falência Hepática Aguda/terapia , Colestase Intra-Hepática/complicações , Colestase Intra-Hepática/terapia , Plasmaferese/efeitos adversos , Injúria Renal Aguda/terapia , Injúria Renal Aguda/complicações , Dengue/complicações , Dengue/terapia
19.
World J Gastroenterol ; 28(39): 5735-5749, 2022 Oct 21.
Artigo em Inglês | MEDLINE | ID: mdl-36338891

RESUMO

BACKGROUND: The coronavirus disease 2019 (COVID-19) was perhaps the most severe global health crisis in living memory. Alongside respiratory symptoms, elevated liver enzymes, abnormal liver function, and even acute liver failure were reported in patients suffering from severe acute respiratory disease coronavirus 2 pneumonia. However, the precise triggers of these forms of liver damage and how they affect the course and outcomes of COVID-19 itself remain unclear. AIM: To analyze the impact of liver enzyme abnormalities on the severity and outcomes of COVID-19 in hospitalized patients. METHODS: In this study, 684 depersonalized medical records from patients hospitalized with COVID-19 during the 2020-2021 period were analyzed. COVID-19 was diagnosed according to the guidelines of the National Institutes of Health (2021). Patients were assigned to two groups: those with elevated liver enzymes (Group 1: 603 patients), where at least one out of four liver enzymes were elevated (following the norm of hospital laboratory tests: alanine aminotransferase (ALT) ≥ 40, aspartate aminotransferase (AST) ≥ 40, gamma-glutamyl transferase ≥ 36, or alkaline phosphatase ≥ 150) at any point of hospitalization, from admission to discharge; and the control group (Group 2: 81 patients), with normal liver enzymes during hospitalization. COVID-19 severity was assessed according to the interim World Health Organization guidance (2022). Data on viral pneumonia complications, laboratory tests, and underlying diseases were also collected and analyzed. RESULTS: In total, 603 (88.2%) patients produced abnormal liver test results. ALT and AST levels were elevated by a factor of less than 3 in 54.9% and 74.8% of cases with increased enzyme levels, respectively. Patients in Group 1 had almost double the chance of bacterial viral pneumonia complications [odds ratio (OR) = 1.73, P = 0.0217], required oxygen supply more often, and displayed higher biochemical inflammation indices than those in Group 2. No differences in other COVID-19 complications or underlying diseases were observed between groups. Preexisting hepatitis of a different etiology was rarely documented (in only 3.5% of patients), and had no impact on the severity of COVID-19. Only 5 (0.73%) patients experienced acute liver failure, 4 of whom died. Overall, the majority of the deceased patients (17 out of 20) had elevated liver enzymes, and most were male. All deceased patients had at least one underlying disease or combination thereof, and the deceased suffered significantly more often from heart diseases, hypertension, and urinary tract infections than those who made recoveries. Alongside male gender (OR = 1.72, P = 0.0161) and older age (OR = 1.02, P = 0.0234), diabetes (OR = 3.22, P = 0.0016) and hyperlipidemia (OR = 2.67, P = 0.0238), but not obesity, were confirmed as independent factors associated with more a severe COVID-19 infection in our cohort. CONCLUSION: In our study, the presence of liver impairment allows us to predict a more severe inflammation with a higher risk of bacterial complication and worse outcomes of COVID-19. Therefore, patients with severe disease forms should have their liver tests monitored regularly and their results should be considered when selecting treatment to avoid further liver damage or even insufficiency.


Assuntos
COVID-19 , Falência Hepática Aguda , Pneumonia Viral , Estados Unidos , Humanos , Masculino , Feminino , COVID-19/complicações , COVID-19/epidemiologia , SARS-CoV-2 , Pneumonia Viral/complicações , Falência Hepática Aguda/complicações , Inflamação/complicações
20.
BMC Gastroenterol ; 22(1): 433, 2022 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-36229799

RESUMO

BACKGROUND: Liver diseases post-COVID-19 vaccination is extremely rare but can occur. A growing body of evidence has indicated that portal vein thrombosis, autoimmune hepatitis, raised liver enzymes and liver injuries, etc., may be potential consequence of COVID-19 vaccines. OBJECTIVES: To describe the results of a systematic review for new-onset and relapsed liver disease following COVID-19 vaccination. METHODS: For this systematic review, we searched Proquest, Medline, Embase, PubMed, CINAHL, Wiley online library, Scopus and Nature through the Preferred Reporting Items for Systematic Reviews and Meta Analyses PRISMA guideline for studies on the incidence of new onset or relapsed liver diseases post-COVID-19 vaccination, published from December 1, 2020 to July 31, 2022, with English language restriction. RESULTS: Two hundred seventy-five cases from one hundred and eighteen articles were included in the qualitative synthesis of this systematic review. Autoimmune hepatitis (138 cases) was the most frequent pathology observed post-COVID-19 vaccination, followed by portal vein thrombosis (52 cases), raised liver enzymes (26 cases) and liver injury (21 cases). Other cases include splanchnic vein thrombosis, acute cellular rejection of the liver, jaundice, hepatomegaly, acute hepatic failure and hepatic porphyria. Mortality was reported in any of the included cases for acute hepatic failure (n = 4, 50%), portal vein thrombosis (n = 25, 48.1%), splanchnic vein thrombosis (n = 6, 42.8%), jaundice (n = 1, 12.5%), raised liver enzymes (n = 2, 7.7%), and autoimmune hepatitis (n = 3, 2.2%). Most patients were easily treated without any serious complications, recovered and did not require long-term hepatic therapy. CONCLUSION: Reported evidence of liver diseases post-COIVD-19 vaccination should not discourage vaccination against this worldwide pandemic. The number of reported cases is relatively very small in relation to the hundreds of millions of vaccinations that have occurred and the protective benefits offered by COVID-19 vaccination far outweigh the risks.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Hepatite Autoimune , Falência Hepática Aguda , Trombose Venosa , Humanos , Doença Crônica , COVID-19/epidemiologia , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Hepatite Autoimune/complicações , Hepatite Autoimune/etiologia , Falência Hepática Aguda/complicações , Vacinação/efeitos adversos , Trombose Venosa/complicações , Trombose Venosa/etiologia
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