Search

Introdução O Angioedema Hereditario (AEH) associado à deficiência do inibidor de C1 (C1-INH) é uma doença genética rara, debilitante e potencialmente fatal. As crises são imprevisíveis e podem ocorrer espontaneamente ou serem desencadeadas por estresse, trauma, procedimentos cirúrgicos, infecções e medicamentos. No Brasil, o Protocolo Clínico e Diretrizes Terapêuticas do Ministério da Saúde preconiza que o tratamento das crises é predominante hospitalar sem especificar nenhum tratamento medicamento. Todavia, atraso na resolutividade das crises ocasiona maior risco de óbito, aumento nos atendimentos de urgência e hospitalizações, cirurgias desnecessárias e impacto negativo na vida familiar. Neste contexto, afim de prevenir tais desfechos desfavoráveis, a Secretaria Estadual de Saúde do Espírito Santo estabeleceu um programa de assistência aos portadores de AEH e critérios de utilização do medicamento Icatibanto no tratamento das crises. Após 10 anos de implementação deste programa, este trabalho objetiva avaliar as características epidemiológicas dos pacientes atendidos no programa de assistência aos portadores de AEH no ES e a frequência de tratamento com Icatibanto nas crises de AEH. Métodos Estudo observacional retrospectivo, com levantamento de dados clínicos, epidemiológicos, laboratoriais e dos atendimentos em emergência de pacientes com diagnóstico de AEH cadastrados no programa de assistência aos pacientes com AEH da SESA/ES no período de 2012 à 2022. Resultados No período estudado foram diagnosticados 137 pacientes com AEH, sendo todos cadastrados no centro de referência ambulatorial. Desses, 117 (85,4%) com AEH por deficiência quantitativa do C1-INH, 8 (5,8%) com AEH por deficiência funcional de C1-INH e 12 (8,8%) com AEH com C1-INH normal, por variantes no gene F12. A idade variou de 7 a 97 anos (média: 40 anos) e 74 (54%) eram do sexo feminino. O tempo entre o início dos sintomas e o diagnóstico variou de 1 a 48 anos (média: 14,7 anos). Nos 10 anos de programa, 81 pacientes (59,1%) apresentaram 822 crises que necessitaram atendimento em emergência para aplicação de icatibanto. O tratamento das crises foi estabelecido em serviços de referência hospitalares a partir da geodistribuição dos casos diagnosticados afim agilizar o acesso. A localização de crise mais comum foi a abdominal (40%), seguida de face (27%), extremidades (26%) e laringe (13%). Quanto ao número de crises/ano/paciente, 48% apresentaram 1 crise/ano, 36% 2 a 5 crises/ano e 16% mais de 5 crises/ano. A maioria das crises (95%) necessitou de apenas uma dose do icatibanto para controle do edema. Nenhum paciente necessitou de intubação, hospitalização ou evoluiu para óbito. Faziam tratamento preventivo de crises com danazol ou ácido tranexâmico 67,9% desses pacientes. Discussão e conclusões A implantação de um centro de referência estadual para paciente com AEH realizando triagem dos familiares, mesmo em assintomáticos, contribuiu para o diagnóstico e recomendações de manejo da doença. O acesso otimizado ao tratamento das crises utilizando o medicamento icatibanto em referências hospitalares não registrou nenhum óbito por edema de laringe e cirurgias abdominais por suspeita de abdômen agudo. A frequência de crises dos pacientes diagnósticos neste programa estadual demonstra a necessidade de implementação de políticas de saúde voltadas para esta doença rara, especialmente no tratamento das crises.

Chronic kidney disease (CKD) has always been a complicated global challenge, ranking as the 12th leading cause of death worldwide. Hemodialysis, being one of the most opted renal replacement therapies (RRTs) for patients with end-stage renal disease (ESRD), still possesses some limitations in preventing complications, such as malnutrition and mineral bone disease (CKD-MBD). While efforts have focused on controlling CKD-MBD parameters like calcium and phosphate, less attention has been given to dietary interventions. Moreover, the adoption of low-phosphorus diets for hemodialysis patients is very complex due to potential conflicts with the guideline-recommended high-protein dietary approach. This study sought to investigate the relationship between dietary phosphorus intake and nutritional status in CKD patients undergoing regular hemodialysis.

Power is not enough to make us strong ETQ FAL The heart must also sing the human song. --Syl Lowhar (1965) First allied by the British in 1889, the islands [...]

A 71-year-old female with painless jaundice was admitted for treatment. After failed biliary drainage by ERCP, an EUS-guided choledochoduodenostomy (EUS-CDS) was performed using a lumen-apposing metal stent with an electrocautery-enhanced delivery system (EC-LAMS). However, there was a misdeployment of the stent, resulting in a choledochoduodenal perforation. To address this complication, the patient underwent EUS-guided gallbladder drainage (EUS-GBD) for biliary decompression, followed by the closure of the duodenal wall defect using a clip. The management of the misdeployment was effective. [Extracted from the article]

The performance of an Iranian Ta'ziyeh play in the US testifies to the vitality of traditional Persian drama in exile. The exiled director Mohammad Ghaffari staged 'Moses and the Wandering Dervish' in 1988, transforming it to fit a mixed audience which included Iranians. The play is from the 13th century, written by the poet Rumi as a religious drama. Modern scenes were interjected into the play, such as from the Vietnam War.

This article discusses a case study of an 86-year-old female who underwent EUS-guided gallbladder drainage (EUS-GBD) using a lumen apposing-metal stent (LAMS) for acute lithiasic cholecystitis. However, two months later, she was readmitted for another episode of acute cholecystitis, and a computed tomography scan revealed obstruction of the LAMS and two large lithiasis. The authors describe the successful use of endoscopic gallbladder lithotripsy after EUS-GBD to treat the patient, suggesting that this approach may be necessary for selected patients with large cholelithiasis. The article includes a video demonstrating the procedure. [Extracted from the article]

This article discusses a case study involving a 74-year-old female with distal biliary obstruction caused by unresectable pancreatic cancer. The patient initially underwent a procedure called EUS-guided cholecystogastrostomy, but later developed acute cholangitis due to obstruction of the cholecystogastric fistula. Attempts to complete biliary drainage using other methods were unsuccessful, so an EUS-CDS procedure was performed. The article concludes that EUS-CDS was effective in managing the patient's malignant distal biliary obstruction. The article includes a video demonstrating the procedure. [Extracted from the article]

This article discusses a case study of a 51-year-old female with liver cirrhosis and recurrent alcohol-induced pancreatitis who underwent an endoscopic ultrasound-guided drainage procedure for a large pancreatic pseudocyst. During the procedure, there was a complete misdeployment of a lumen-apposing metal stent (LAMS), which required the placement of a second LAMS. The retrieval of the misdeployed LAMS was delayed, but after seven days, it was successfully removed endoscopically. Four weeks later, a CT scan showed resolution of the pseudocyst, and the LAMS was removed. The study concludes that delayed endoscopic salvage of a misdeployed LAMS during EUS-guided pancreatic pseudocyst drainage was safe and effective. [Extracted from the article]

The COVID-19 pandemic impacts on eating habits among adolescents may be more relevant in pediatric patients with immunocompromised chronic diseases. This case-control study conducted between June and October 2020 aimed to: (i) describe dietary patterns of adolescents with chronic conditions compared to healthy controls and (ii) determine associations between food consumption, health-related quality of life (HRQL) and sleep quality during the COVID-19 pandemic. Participants (184 immunocompromised and 58 healthy adolescents, aged 14.3 [SD 2.5]) responded to HRQL and sleep validated instruments (PedsQL and PSQI) and three 24 h food recalls via online software. Adjusted linear and logistic regressions were used to assess differences in dietary patterns and associations between food consumption (according to Nova classification) and HRQL and sleep quality. Adolescents with gastrohepatic, rheumatic, and kidney diseases had an improved dietary pattern vs. their healthy peers, showing greater consumption of unprocessed and minimally processed foods (unstandardized coefficient (b) = 7.35%[95%CI 1.59; 13.1]; b = 15.10%[95%CI 7.00; 23.1]; and b = 11.2%[95%CI 5.68; 16.8]), and lower consumption of ultraprocessed foods (b = −7.53%[95%CI-12.90; −2.18]; b = −11.4%[95%CI-18.90; −3.94]; b = −10.8%[95%CI-16.00; −5.68]). Consumption of culinary ingredients was associated with reduced psychological HRQL in controls (standardized coefficient (β) = −0.26[95%CI-0.52; −0.004]), and processed food consumption was associated with improved sleep latency in immunocompromised participants (β = 0.16[95%CI 0.01; 0.31]). These findings suggest diet quality may play a role in HRQL and sleep quality in this population, and may be relevant for clinical practitioners and policy makers when considering the importance of dietary quality in immunocompromised youths.