Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis

Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Intestinal angioedema is another important and incapacitating presentation that may be the main or only manifestation during an attack. In this article, a group of experts from the “Associação Brasileira de Alergia e Imunologia (ASBAI)” and the “Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH)” has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema

Poor Regenerative Outcome after Skeletal Muscle Necrosis Induced by Bothrops asper Venom: Alterations in Microvasculature and Nerves

artículo (arbitrado) -- Universidad de Costa Rica, Instituto de Investigaciones Clodomiro Picado. 2011Background: Viperid snakebite envenoming is characterized by prominent local tissue damage, including muscle necrosis. A frequent outcome of such local pathology is deficient skeletal muscle regeneration, which causes muscle dysfunction, muscle loss and fibrosis, thus provoking permanent sequelae that greatly affect the quality of life of patients. The causes of such poor regenerative outcome of skeletal muscle after viperid snakebites are not fully understood. Methodology/Principal Findings: A murine model of muscle necrosis and regeneration was adapted to study the effects of the venom and isolated toxins of Bothrops asper, the medically most important snake in Central America. Gastrocnemius muscle was injected with either B. asper venom, a myotoxic phospholipase A2 (Mtx), a hemorrhagic metalloproteinase (SVMP), or saline solution. At various time intervals, during one month, tissue samples were collected and analyzed by histology, and by immunocytochemical and immunohistochemical techniques aimed at detecting muscle fibers, collagen, endothelial cells, myoblasts, myotubes, macrophages, TUNEL-positive nuclei, and axons. A successful regenerative response was observed in muscle injected with Mtx, which induces myonecrosis but does not affect the microvasculature. In contrast, poor regeneration, with fibrosis and atrophic fibers, occurred when muscle was injected with venom or SVMP, both of which provoke necrosis, microvascular damage leading to hemorrhage, and poor axonal regeneration. Conclusions/Significance: The deficient skeletal muscle regeneration after injection of B. asper venom is likely to depend on the widespread damage to the microvasculature, which affects the removal of necrotic debris by phagocytes, and the provision of nutrients and oxygen required for regeneration. In addition, deficient axonal regeneration is likely to contribute to the poor regenerative outcome in this model.This study was supported by NeTropica (grant 2-N-2008), by Vicerrectoría de Investigación, Universidad de Costa Rica (project 741-A7-604). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.UCR::Vicerrectoría de Investigación::Unidades de Investigación::Ciencias de la Salud::Instituto Clodomiro Picado (ICP

Comparison of Bothropoides jararaca bites with and without envenoming treated at the Vital Brazil Hospital of the Butantan Institute, State of São Paulo, Brazil Comparação dos acidentes causados por Bothropoides jararaca com e sem envenenamento atendidos no Hospital Vital Brazil do Instituto Butantan, Estado de São Paulo

INTRODUCTION: This study analyses the cases of all bites (including dry bites) caused by Bothropoides jararaca attended at the Vital Brazil Hospital of the Butantan Institute, State of São Paulo, Brazil. METHODS: A retrospective study was conducted of patients bitten by Bothropoides jararaca (n=792) from January 1990 to December 2004. The characteristics of the snake specimen, data related to the accident and clinical manifestations on admission were obtained from patient medical records. RESULTS: The majority of the cases in this study were caused by female and juvenile snakes. No stomach contents were found in 93.4% of the snake specimens after dissection. No statistical difference was observed between the occurrence of dry bites and the maturity or sex of the snake. The median SVL of snakes in mild and moderate cases was 40.5cm and in severe cases, SVL increased to 99cm. Necrosis was more common in the digits of the feet and hands (4.8%) compared to the other body regions (1.8%). A significant difference was verified between severity and a time interval greater than six hours from the bite to hospital admission. A significant association was verified between gingival bleeding and abnormal blood coagulability. In accidents caused by adult snakes, necrosis was more frequent (7.2%) compared to accidents caused by juvenile snakes (1%). CONCLUSIONS: In this work, the association between certain epidemiological data and the evolution of biological parameters in the clinical course of Bothrops sensu latu accidents were highlighted, contributing to the improvement of snake bite assistance.<br>INTRODUÇÃO: Neste estudo, analisou-se todos os casos de picadas (incluindo picadas secas) causadas por Bothropoides jararaca atendidos no Hospital Vital Brazil do Instituto Butantan, São Paulo, Brasil. MÉTODOS: Estudo retrospectivo em que foram incluídos pacientes atendidos no Hospital Vital Brazil do Instituto Butantan, picados por serpentes da espécie Bothropoides jararaca (nº=792) entre 1990 a 2004. Os dados foram obtidos através de prontuários médicos. RESULTADOS: No presente estudo, a maioria dos acidentes foi causada por serpentes fêmeas e filhotes. Não havia presença de conteúdo estomacal em 93,4% das serpentes dissecadas. Não houve diferença estatística entre a ocorrência de picada seca e o sexo da serpente. O comprimento rostro cloacal das serpentes nos casos leves e moderados foram 40,5cm e nos casos severos 99cm. Necrose foi mais comum nos dedos dos pés e das mãos (4,8%) em comparação com outras regiões do corpo (1,8%). Houve diferença estatística entre a gravidade e o intervalo de tempo entre a picada e a admissão hospitalar superior a seis horas. Encontramos uma associação significativa entre gengivorragia e incoagulabilidade sanguínea. Nos acidentes causados por serpentes adultas, a necrose foi mais frequente (7,2%) quando comparado aos acidentes causados por serpentes filhotes (1%). CONCLUSÕES: Neste estudo, destaca-se a associação entre os dados epidemiológicos e biológicos em relação à evolução do quadro clínico nos acidentes botrópicos, contribuindo para a melhoria da assistência nos acidentes ofídicos