Severe Acute Liver Injury due to Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.

Severe acute liver injury (ALI) is mostly triggered by viral infections and hepatotoxic drugs; however, it can also be seen in systemic diseases. Hemophagocytic lymphohistiocytosis (HLH) is a rare, immune-mediated syndrome that presents as a life-threatening inflammatory disorder affecting multiple organs. Secondary causes occur mainly in the set of malignancy, infection, and autoimmune disease, and are seldom triggered by vaccination. Although liver involvement is common, presentation as severe ALI is rare. We describe a case of a 65-year-old male with history of low-risk chronic lymphocytic leukemia and rheumatoid arthritis treated with prednisolone who presented with persistent fever and jaundice 1 week after COVID-19 vaccination. The diagnosis was challenging given the predominant liver impairment, characterized by hyperbilirubinemia, transaminases over 1,000 U/L, and prolonged INR, which prompted an extensive investigation and exclusion of autoimmune, toxic, and viral causes of hepatitis. Laboratory workup revealed bicytopenia, hyperferritinemia, which together with organ failure and evidence of hemophagocytosis in bone marrow suggested the diagnosis of HLH. After excluding infectious etiologies, flare of rheumatological disease, and the progression of hematological disease, HLH was diagnosed. He was successfully treated with etoposide and corticosteroids, with dramatic improvement of liver tests. After exclusion of other causes of secondary HLH, the recent vaccination for COVID-19 was the likely trigger. We report a case of double rarity of HLH, as it presented with severe liver dysfunction which was probably triggered by vaccination. In this case, the predominant liver involvement urged extensive investigation of liver disease, so a high index of suspicion was required to make an early diagnosis. Clinicians should consider HLH in patients with unexplained signs and symptoms of systemic inflammatory response and multiorgan involvement, including severe liver involvement as the first presentation.

A lesão hepática aguda (LHA) grave é desencadeada principalmente por infeções virais e hepatotóxicos; contudo, pode ocorrer em condições com envolvimento sistémico. A linfohistiocitose hemofagocítica (LHH) é uma síndrome inflamatória, rara, imunomediada, potencialmente fatal, que pode afetar múltiplos órgãos. A LHH secundária ocorre em contexto de neoplasias, infeções e doenças autoimunes, podendo raramente ser precipitada pela vacinação. Embora seja frequente o envolvimento hepático na LHH, a apresentação como LHA grave é rara. Os autores descrevem o caso de um homem de 65 anos com história de leucemia linfocítica crónica de baixo risco e artrite reumatóide sob prednisolona de 65 anos, que se apresentou com febre persistente e icterícia uma semana após a primeira dose da vacina COVID-19. O diagnóstico constituiu um desafio dado o envolvimento hepático predominante, caracterizado por hiperbilirrubinemia, transaminases acima de 1000 U/L e INR prolongado, o que condicionou uma extensa investigação e exclusão das causas autoimunes, tóxicas, e virais de doença hepática. A presença de bicitopenia e hiperferritinemia, conjuntamente com o desenvolvimento de falências de órgão e evidência de hemofagocitose na medula óssea sugeriram o diagnóstico de LHH. Após exclusão infeções, agudização da doença reumatológica e progressão da doença hematológica, foi feito o diagnóstico de LHH.O doente foi tratado com etoposido e corticosteróides com sucesso, verificando-se uma melhoria dramática das provas hepáticas. Após a exclusão de outras causas de LHH secundária, a recente vacinação foi assumida como provável fator desencadeante. Relatamos um caso raro de LHH, quer pela apresentação com lesão hepática grave, quer pela vacinação como presumível desencadeante. Neste caso, o envolvimento hepático predominante promoveu a uma investigação extensa da doença hepática, tendo sido necessário um elevado índice de suspeição para um diagnóstico atempado. Os médicos devem considerar o diagnóstico de LHH em doentes com sinais e sintomas de resposta inflamatória sistémica, inexplicados que se acompanham por disfunção multiorgânica, nomeadamente disfunção hepática grave como apresentação clínica.

Physicians and nurses professional relationship with criminal investigation in dealing with survivors of sexual abuse: a scoping review.

Survivors of sexual abuse and their families seek help from criminal law enforcement agencies and health professionals to obtain justice and health care. Many communities have implemented multi-professional collaborative models so that the victim's well-being is assured and the truth is established. However, there is a general lack of evidence on how to best articulate these teams with the healthcare professionals caring for the survivors.Therefore, this Scoping Review was conducted in order to analyze and to map the barriers and facilitators of the relationship between health professionals and the criminal investigation team in the care of survivors of sexual abuse. The methodology proposed by the Joanna Briggs Institute for Scoping Reviews was used, and the Bronstein five dimension model of interprofessional collaboration served as the basis for the analysis of barriers and facilitators. Quantitative, qualitative and mixed studies, primary and secondary sources, text and opinion documents were included. Content analysis was performed on the main findings of the collected studies. Twelve articles were identified and analyzed. Collaboration, communication, hierarchy, skills, confidentiality, and leadership emerged as key themes. Multidisciplinary Sexual Assault Nurse Examiner (SANE) and Sexual Assault Response Teams (SARTs) were implemented to coordinate care, but conflicting goals and values among professionals posed challenges. Communication failures and inadequate information sharing hindered collaboration. Neutral leaders who coordinate teams, minimize groupthink, and improve decision-making were found to be valuable. Engaging across disciplinary boundaries and addressing power dynamics were challenging but could be addressed through facilitation and conflict resolution. This review highlights the importance of effective collaboration and interaction within teams and with other professionals in the care of sexual abuse survivors.

Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension.

Mastocytosis is a clonal neoplastic disorder of the mast cells (MC) that can be limited to the skin (cutaneous mastocytosis) or involve one or more extracutaneous organs (systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis (ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

Victims of sexual offences: medicolegal examinations in emergency settings.

The aim of the study was to present some data concerning the examinations of victims of sexual offences in emergency settings conducted by medicolegal examiners of the Lisbon Department of the National Institute of Legal Medicine (NILM) over a two-year period (2002-2003). The study was based on 352 alleged victims of sexual offences referred by investigating police authorities and physicians working at hospital emergency rooms. Examination records were reviewed and data collected according to the extended medicolegal protocol adopted by NILM, which includes sociodemographic variables, and medical and laboratory findings. The results show that examinations in emergency settings represented about 43% of the total examinations of victims of sexual offences, 44% of them being performed at hospital emergency rooms. Victims' ages ranged from a minimum of 93 days to a maximum of 86 years. The mean age was 17.5 years. Females represented about 92% with a large over-representation of those aged from 0 to 19 years (61% of the total). Victims were mainly girls of school age (36%) or under 6 years old (25%). Offenders were male, referred to mainly, as acquaintances/neighbours (32%), friends (24%) or cohabiting family members (20%), a large majority belonging to the victim's social or family circle (85%). Of the examinations requested as "urgent cases" only 61% were reported as having occurred within 72h prior to the examination. Traumatic lesions on the whole body were found in only 28% of the cases, while findings on the genitalia and/or the anus were present in 31%. Medical and laboratory findings were in accordance with some sort of sexual offence in 34% of the cases. Our findings show the great heterogeneity of the victims, with a high proportion of children as well as the interplay between examination requests in emergency settings and the need to define more accurate criteria and proceedings for legal authorities and physicians who are usually the first line of intervention before specialists in legal medicine.

[Thrombasthenia of Glanzmann. Vaginal hysterectomy treated with recombinant factor VIIa]. / Trombastenia de Glanzmann. Histerectomia vaginal de urgência tratada com factor VIIa recombinante.

In the present article, regarding a clinical case of Thrombasthenia of Glanzmann with severe anaemia by menometrorrhagia, the authors propose the vaginal hysterectomy and the administration of the recombinant factor VIIa on the immediate pre and postoperative as effective therapeutical alternatives.