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- Budd-Chiari Syndrome and Acute Liver Failure: An Uncommon Presentation of Acute Myeloid LeukaemiaPublication . Costa, Ana Rita Gonçalves; Freitas, Inês; Raposo, Joana; Barbosa, Gustavo; Pessegueiro Miranda, Helena; Nery, Filipe GaioAcute liver failure (ALF) is a rare entity, particularly in the context of Budd-Chiari syndrome (BCS). BCS is an uncommon disorder with multiple risk factors, most commonly myeloproliferative disorders. In BCS, active search and exclusion of underlying malignancy is mandatory, particularly in the context of ALF, as it may contraindicate liver transplantation (LT). We present the case of a healthy 29-year-old male, without known risk factors for liver disease, who presented to the emergency department with abdominal pain, ascites, and jaundice. BCS with consequent severe acute liver injury with rapid progression to ALF was diagnosed. The patient was listed for LT. The study of peripheral blood finally revealed myeloid blasts, and flow cytometry showed a population of blast cells with abnormal immunophenotypic profile (CD33+ and myeloperoxidase, MPO+). The bone marrow biopsy showed morphological and immunophenotypic aspects of acute myeloid leukaemia (AML) FAB M1. This diagnosis was considered a formal contraindication to LT, so the patient was delisted. ALF contraindicated rescue chemotherapy and AML contraindicated LT. The patient died 48 h after ICU admission. The search for underlying neoplasia is mandatory in the context of BCS, moreover with associated ALF, as it may limit lifesaving treatments and interventions to supportive and palliative.
- Severe Acute Liver Injury Related to Heat StrokePublication . Ribeiro, Fani; Bibi, Mário; Pereira, Marta; Ferreira, Sofia; Pessegueiro Miranda, Helena; Araújo, RuiHeat stroke (HS) is a life-threatening condition characterized by hyperthermia and multiple organ failure. Mild to moderate hepatocellular injury is a well-documented complication but severe liver injury and acute liver failure are rare. There are neither established criteria nor optimal timing for liver transplantation and conservative management seems to be the cornerstone treatment. The authors report a case of a patient with severe liver injury related to HS who recovered completely under conservative treatment. Learning points: Hyperthermia, neurologic dysfunction and recent exposure to hot weather or physical exertion should raise the suspicion of heat stroke (HS).Fast and effective cooling is the cornerstone of treatment, along with support of organ dysfunction. Antipyretics have no role in HS management.Conservative treatment has been described as being successful in the management of patients with HS that manifest severe acute liver injury (ALI) and acute liver failure (ALF). However, early referral to a liver transplantation centre is essential to guide treatment.
- The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case SeriesPublication . Cravo, Marcia; Oliveira, Daniel G; Guimas, Arlindo; Vita, Pedro; Rego, Ana Luisa; Alves, Rute; Valadares, Diana; Carvalheiras, Graziela; Ricardo, Miguel; Pinto, Alexandre; Pessegueiro Miranda, Helena; Nery, Filipe GaioBudd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach.
- Liver Transplantation for Acute Hepatic Failure Following Intentional Iron OverdosePublication . Eça, Rosário; Ferreira, Sofia; Gandara, Judit; Pessegueiro Miranda, Helena; Daniel, JorgeThe majority of acute iron toxicity cases occur in young children from accidental ingestion. In adults, iron poisoning is rare and mostly due to intentional ingestion. Physicians, particularly those who do not routinely treat pediatric patients, are often unfamiliar with the clinical manifestation of iron poisoning, its management, and its potential for multiple organ failure, especially liver damage. Severe acute hepatotoxicity treated with liver transplantation is rare in adults, with very limited published literature. We report a case of a severe iron tablet overdose with suicidal intent that progressed to fulminant hepatic failure despite medical treatment, ultimately treated with liver transplantation.
- Palliative Care in End-Stage Liver Disease Patients Awaiting Liver Transplantation: ReviewPublication . Vieira Silva, Sara; Freire, Elga; Pessegueiro Miranda, HelenaIntroduction: End-stage liver disease (ESLD) is the advanced phase of most liver diseases. The cure is liver transplantation (LT), only available for a minority of patients. This review summarizes the evidence regarding palliative care (PC) in ESLD patients awaiting LT. Methods: Review of the literature available in Medline, Scopus and Web of Knowledge, with keywords ESLD and PC. Results: Fifteen of the 230 articles reviewed met the inclusion criteria. Ten main themes were addressed: symptom burden; perspectives of life-sustaining treatment and comfort for patients, families and health professionals; goals of care discussions; patient and family needs; quality of life; PC and survival; referral to PC, barriers and opportunities; integration of PC; outpatient care and cost-effectiveness analysis. The referral of patients to PC was only evaluated in a few studies, all of which reported low referral rates. Better knowledge of how PC professionals can support other professionals was considered important, and also better ways to integrate PC were considered essential. Conclusion: ESLD patients awaiting LT have a significant need for PC and, despite the insufficient response, were reported to benefit from this type of care. Future research is essential to determine the means to overcome barriers and better integrate PC for ESLD patients awaiting LT.