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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.
- 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.
- Use of albumin infusion for cirrhosis-related complications: An international position statementPublication . Bai, Zhaohui; Méndez-Sánchez, Nahum; Romeiro, Fernando Gomes; Mancuso, Andrea; Philips, Cyriac Abby; Tacke, Frank; Basaranoglu, Metin; Primignani, Massimo; Ibrahim, Mostafa; Wong, Yu Jun; Nery, Filipe Gaio; Teschke, Rolf; Ferreira, Carlos Noronha; Muñoz, Alberto E.; Pinyopornpanish, Kanokwan; Thevenot, Thierry; Singh, Shivaram Prasad; Mohanty, Arpan; Satapathy, Sanjaya K.; Ridola, Lorenzo; Maruyama, Hitoshi; Cholongitas, Evangelos; Levi Sandri, Giovanni Battista; Yang, Li; Shalimar, null; Yang, Yongping; Villa, Erica; Krag, Aleksander; Wong, Florence; Jalan, Rajiv; O’Brien, Alastair; Bernardi, Mauro; Qi, XingshunBackground & aims: Numerous studies have evaluated the role of human albumin (HA) in managing various liver cirrhosis-related complications. However, their conclusions remain partially controversial, probably because HA was evaluated in different settings, including indications, patient characteristics, and dosage and duration of therapy. Methods: Thirty-three investigators from 19 countries with expertise in the management of liver cirrhosis-related complications were invited to organise an International Special Interest Group. A three-round Delphi consensus process was conducted to complete the international position statement on the use of HA for treatment of liver cirrhosis-related complications. Results: Twelve clinically significant position statements were proposed. Short-term infusion of HA should be recommended for the management of hepatorenal syndrome, large volume paracentesis, and spontaneous bacterial peritonitis in liver cirrhosis. Its effects on the prevention or treatment of other liver cirrhosis-related complications should be further elucidated. Long-term HA administration can be considered in specific settings. Pulmonary oedema should be closely monitored as a potential adverse effect in cirrhotic patients receiving HA infusion. Conclusions: Based on the currently available evidence, the international position statement suggests the potential benefits of HA for the management of multiple liver cirrhosis-related complications and summarises its safety profile. However, its optimal timing and infusion strategy remain to be further elucidated.Impact and implications: Thirty-three investigators from 19 countries proposed 12 position statements on the use of human albumin (HA) infusion in liver cirrhosis-related complications. Based on current evidence, short-term HA infusion should be recommended for the management of HRS, LVP, and SBP; whereas, long-term HA administration can be considered in the setting where budget and logistical issues can be resolved. However, pulmonary oedema should be closely monitored in cirrhotic patients who receive HA infusion.
- Improvement of Central Nervous System Vasculitis in a Patient with Chronic Hepatitis C Virus Infection after Treatment with an Interferon-Free RegimenPublication . Lima, S.; Faria, Raquel; Nery, Filipe GaioBackground: Neurosarcoidosis is a rare manifestation of hepatitis C virus (HCV) infection, mainly in patients exposed to interferon-based therapies. Although we are living in a new era of HCV treatment, there is still little data concerning the treatment of extrahepatic complications of the disease with direct antiviral agents, especially rare ones such as neurosarcoidosis. Summary: We present a rare case of central nervous system vasculitic lesions in the context of chronic HCV infection associated with mixed cryoglobulinemia, elevated angiotensin-converting enzyme (ACE) levels, and documentation of viral RNA in the cerebrospinal fluid in a treatment-naïve chronic HCV patient. Successful treatment with an interferon-free regimen improved all clinical manifestations, reduced the levels of serum ACE, and reduced the cryoglobulin levels to undetectable. Messages: Neurosarcoidosis and cryoglobulinemia are rare but well-recognized complications of HCV infection, even in treatment-naïve patients. Direct antiviral agents can be useful in the management of this condition.