Late-Onset Ornithine Carbamoyltransferase Deficiency Accompanying Acute Pancreatitis and Hyperammonemia

Hyperammonemia related to urea cycle disorders is a rare cause of potentially fatal encephalopathy that is encountered in intensive care units (ICUs). Left undiagnosed, this condition may manifest irreversible neuronal damage. However, timely diagnosis and treatment initiation can be facilitated simply by increased awareness of the ICU staff. Here, we describe a patient with acute severe pancreatitis who developed hyperammonemia and encephalopathy without liver disease. Urea cycle disorder was suspected and hemodialysis was initiated. Following reduction of ammonia levels, subsequent treatment included protein restriction and administration of arginine and sodium benzoate. The patient was discharged to home after 47 days with plasma ammonia within normal range and without neurological symptoms. In clinical care settings, patients with neurological symptoms unexplained by the present illness should be assessed for serum ammonia levels to disclose any urea cycle disorders to initiate timely treatment and improve outcome

Nutritional impact of inflammatory bowel diseases on children and adolescents* *Study conducted at the Service of Pediatric Gastroenterology and Hepatology (Complexo HUPES-CPPHO) and Faculdade de Medicina da Bahia, Universidade Federal da Bahia, Salvador, BA, Brazil.

AbstractOBJECTIVETo perform a sistematiy review of the literature about the nutritional impact of inflammatory bowel diseases in children and adolescents.DATA SOURCESA systematic review was performed using PubMed/MEDLINE, LILACS and SciELO databases, with inclusion of articles in Portuguese and in English with original data, that analyzed nutritional aspects of inflammatory bowel diseases in children and adolescents. The initial search used the terms “inflammatory bowel diseases” and “children” or “adolescents” and “nutritional evaluation” or “nutrition deficiency”. The selection of studies was initially performed by reading the titles and abstracts. Review studies and those withouth data for pediatric patients were excluded. Subsequently, the full reading of the articles considered relevant was performed.RESULTS237 studies were identified, and 12 of them were selected according to the inclusion criteria. None of them was performed in South America. During the analysis of the studies, it was observed that nutritional characteristics of patients with inflammatory bowel disease may be altered; the main reports were related to malnutrition, growth stunting, delayed puberty and vitamin D deficiency.CONCLUSIONThere are nutritional consequences of inflammatory bowel diseases in children and adolescents, mainly growth stunting, slower pubertal development, underweight and vitamin deficiencies. Nutritional impairments were more significant in patients with Crohn's disease; overweight and obesity were more common in patients with ulcerative rectocolitis. A detailed nutritional assessment should be performed periodically in children and adolescents with inflammatory bowel disease

The impact of multivisceral liver resection on short- and long-term outcomes of patients with colorectal liver metastasis: A systematic review and meta-analysis

The impact of Multivisceral Liver Resection (MLR) on the outcome of patients with Colorectal Liver Metastasis (CRLM) is unclear. The present systematic review aimed to compare patients with CRLM who underwent MLR versus standard hepatectomy regarding short- and long-term outcomes. MLR is a feasible procedure but has a higher risk of major complications. MLR did not negatively affect long-term survival, suggesting that an extended resection is an option for potentially curative treatment for selected patients with CRLM

Two decades of liver resection with a multidisciplinary approach in a single institution: What has changed? Analysis of 1409 cases

Objectives: To evaluate results of patients undergoing liver resection in a single center over the past two decades with a particular look at Colorectal Liver Metastasis (CRLM) and Hepatocellular Carcinoma (HCC). Method: Patients were divided into two eras, from 2000 to 2010 (Era 1) and 2011 to 2020 (Era 2). The most frequent diagnosis was CRLM and HCC, with 738 (52.4%) and 227 (16.1%) cases respectively. An evaluation of all liver resection cases and a subgroup analysis of both CRLM and HCC were performed. Preoperative and per operative variables and long-term outcomes were evaluated. Results: 1409 liver resections were performed. In Era 2 the authors observed higher BMI, more: minimally invasive surgeries, Pringle maneuvers, and minor liver resections; and less transfusion, less ICU necessity, and shorter length of hospital stay. Severe complications were observed in 14.7% of patients, and 90-day mortality was 4.2%. Morbidity and mortality between eras were not different. From 738 CRLM resections, in Era 2 there were significantly more patients submitted to neoadjuvant chemotherapy, bilateral metastases, and smaller sizes with significantly less transfusion, the necessity of ICU, and shorter length of hospital stay. More pedicle clamping, minimally invasive surgeries, and minor resections were also observed. From 227 HCC resections, in Era 2 significantly more minimally invasive surgeries, fewer transfusions, less necessity of ICU, and shorter length of hospital stay were observed. OS was not different between eras for CRLM and HCC. Conclusions: Surgical resection in a multidisciplinary environment remains the cornerstone for the curative treatment of primary and metastatic liver tumors

O conhecimento de estudantes universitários do sexo masculino sobre o aleitamento materno e o papel do pai na amamentação

Objetivo: avaliar o conhecimento de estudantes universitários do sexo masculino sobre o aleitamento materno e o papel do pai no processo da amamentação. Metodologia: abordagem quantitativa, corte transversal, mediante aplicação de um questionário a cem estudantes universitários, sexo masculino, do primeiro ano de graduação nas diversas áreas da Universidade Federal da Bahia, Salvador. Resultados: a média de idade dos estudantes entrevistados foi de 21,8 anos; 92,6% eram solteiros e 5,3% tinham filhos. Analisando o conhecimento sobre o aleitamento materno, 33,0% afirmaram que a amamentação deve ser iniciada na primeira hora de vida; 91,5% reconhecerem a existência de um período de amamentação exclusiva, mas apenas 51,2% responderam corretamente a duração deste período; 40,4% dos estudantes responderam que a duração do aleitamento total é de 2 anos ou mais. Em relação aos benefícios da amamentação, 55,3% desconhecem a existência de benefícios maternos, 93,6% reconhecem a existência de benefícios para a criança e 72,3% desconhecem a existência de benefícios para a família. Quanto ao papel do pai, 58,5% dos estudantes acreditam que o pai pode exercer um papel importante na amamentação; 98,9% apoiariam seus filhos no processo de amamentação. Conclusões: os estudantes apresentam conhecimento sobre amamentação, porém não possuem aprofundamento sobre benefícios, além de poucos reconhecerem a importância do pai neste processo

HEPATOSPLENIC SCHISTOSOMIASIS-ASSOCIATED CHRONIC PORTAL VEIN THROMBOSIS: RISK FACTOR FOR HEPATOCELLULAR CARCINOMA?

ABSTRACT BACKGROUND: Hepatosplenic schistosomiasis is an endemic disease prevalent in tropical countries and is associated with a high incidence of portal vein thrombosis. Inflammatory changes caused by both parasitic infection and portal thrombosis can lead to the development of chronic liver disease with potential carcinogenesis. AIMS: To assess the incidence of portal vein thrombosis and hepatocellular carcinoma in patients with schistosomiasis during long-term follow-up. METHODS: A retrospective study was conducted involving patients with schistosomiasis followed up at our institution between 1990 and 2021. RESULTS: A total of 126 patients with schistosomiasis were evaluated in the study. The mean follow-up time was 16 years (range 5–31). Of the total, 73 (57.9%) patients presented portal vein thrombosis during follow-up. Six (8.1%) of them were diagnosed with hepatocellular carcinoma, all with portal vein thrombosis diagnosed more than ten years before. CONCLUSIONS: The incidence of hepatocellular carcinoma in patients with schistosomiasis and chronic portal vein thrombosis highlights the importance of a systematic long-term follow-up in this group of patients

The prognostic impact of poorly differentiated clusters and tumor budding in colorectal liver metastases

INTRODUÇÃO: A ressecção hepática é o único tratamento potencialmente curativo para as metástases hepáticas de câncer colorretal (MHCCR), porém com taxas de recidiva entre 60 e 70%. Desta forma, faz-se necessário um melhor entendimento das vias de disseminação e recidiva da doença. Os brotamentos tumorais (BT) e grupamentos pouco diferenciados (GPD), relacionados à transição epitélio-mesenquimal, são fatores prognósticos para o câncer colorretal; entretanto sua presença e importância nas MHCCR ainda não estão estabelecidas. Os objetivos deste trabalho foram avaliar a presença dos BT e GPD nas MHCCR, determinar sua importância prognóstica e sua relação com outros fatores patológicos conhecidos. MÉTODOS: Foram avaliados, retrospectivamente, 229 pacientes submetidos a ressecção de MHCCR com intuito curativo, entre janeiro de 2004 e junho de 2014. Nos espécimes cirúrgicos das MHCCR ressecadas, foi realizada análise anatomopatológica através de lâminas coradas em hematoxilina e eosina (HE), para avaliação dos BT, GPD, infiltrado inflamatório peritumoral, presença de pseudocápsula e tipo de borda; e por imuno-histoquímica, por meio de anticorpos anti-AE1/AE (BT e GPD), anti-D2-40 (invasão linfática) e anti-CD34 (invasão venosa portal). Também foram analisadas as variáveis clínicas relacionadas ao prognóstico. RESULTADOS: O seguimento médio após a hepatectomia foi de 43 meses. As taxas de sobrevida global e livre de doença em 1, 3 e 5 anos foram de, respectivamente, 94,1%, 66,7% e 45,5% e 53,6%, 31,5% e 29,6%. Os BT estiveram presentes em 61,1% dos pacientes na avaliação pelo AE1/AE3 e em 48,9% pelo HE, enquanto os GPD estiveram presentes em 42,8% dos pacientes na avaliação pelo AE1/AE3 e em 49,3% pelo HE. Na análise univariada, os BT e os GPD grau 3 ( > 9 GPD) mostraram significância prognóstica, tanto na avaliação pelo AE1/AE3 quanto pelo HE. Na análise múltipla, os fatores independentes para sobrevida global foram: a presença de GPD grau 3 (pelo HE), presença de pseudocápsula, invasão venosa portal e presença de 4 ou mais nódulos. Os fatores independentes para sobrevida livre de doença foram: GPD grau 3 (pelo HE), quimioterapia prévia, presença de 4 ou mais nódulos, infiltrado inflamatório peritumoral ausente ou leve, invasão venosa portal e borda infiltrativa. Os BT e GPD não estiveram associados à recidiva hepática. Os BT foram associados a: GPD, MHCCR sincrônicas, tumores de até 5 cm, ausência de pseudocápsula, borda infiltrativa, e presença de invasão venosa portal. Os GPD foram associados a: BT, infiltrado inflamatório peritumoral ausente ou leve, ausência de pseudocápsula e borda infiltrativa. CONCLUSÕES: Os BT e os GPD são frequentes nas MHCCR e ambos, na análise univariada, são fatores de pior prognóstico na sobrevida global e livre de doença, estando associados à recidiva extra-hepática. A presença de GPD grau 3 avaliada pelo HE é fator prognóstico independente na sobrevida global e livre de doença, sugerindo que este é um importante mecanismo de disseminação tumoral na MHCCRINTRODUCTION: The only potentially curative treatment for colorectal liver metastases is surgical resection. However, about 60 to 70% of patients will recur, showing the necessity of a better knowledge regarding spread and disease recurrence pathways. Tumor budding and poorly differentiated clusters, markers related to epithelial-mesenchymal transition, are prognostic factors for colorectal cancer, but their presence and significance in colorectal liver metastases is not yet established. The aims of this study were to evaluate the presence of tumor budding and poorly differentiated clusters in colorectal liver metastases, to determine their prognostic value and to relate them to other known pathological factors. METHODS: A total of 229 patients that underwent hepatic resection for colorectal liver metastases between January 2004 and June 2014 were retrospectively evaluated. Resected specimens of colorectal liver metastases were submitted to pathological evaluation by hematoxilin and eosin staining, to analyze tumor budding, poorly differentiated clusters, peritumoral inflammatory infiltrate, presence of tumor pseudocapsule and tumor growth pattern; and by immunohistochemical staining with antibodies anti- AE1/AE3 (tumor budding and poorly differentiated clusters), anti-D2-40 (lymphatic invasion) and anti-CD-34 (portal vein invasion). Clinical variables related to prognosis were also evaluated. RESULTS: Mean follow up after hepatectomy was 43 months. Overall and disease-free survival rates at 1, 3 and 5 years were 94.1%, 66.7% and 45.5% and 53.6%, 31.5% and 29.6%, respectively. Tumor budding was present in 61.1% of patients in the evaluation by AE1/AE3 staining and in 48.9% by hematoxilin and eosin, while poorly differentiated clusters were present in 42.8% of patients by AE1/AE3 staining and 49.3% by hematoxilin and eosin. At univariate analysis, tumor budding and poorly differentiated clusters grade 3 ( > 9 poorly differentiated clusters) by AE1/AE3 and hematoxilin and eosin staining showed prognostic significance. On multiple analysis, independent factors for overall survival were: presence of poorly differentiated clusters grade 3 (by hematoxilin and eosin), presence of tumor pseudocapsule, portal vein invasion and presence of 4 or more nodules. Independent factors for disease-free survival were: poorly differentiated clusters grade 3 (by hematoxilin and eosin), preoperative chemotherapy, portal vein invasion, presence of 4 or more nodules, none/mild peritumoral inflammatory infiltrate and infiltrative tumor border. Tumor budding and poorly differentiated clusters were not related to hepatic recurrence. Tumor budding was associated with: presence of poorly differentiated clusters, synchronous colorectal liver metastases, tumor size up to 5 cm, absence of tumor pseudocapsule, infiltrative tumor border and presence of portal vein invasion. Poorly differentiated clusters were associated with: presence of tumor budding, none/mild peritumoral inflammatory infiltrate, absence of tumor pseudocapsule and infiltrative tumor border. CONCLUSIONS: Tumor budding and poorly differentiated clusters are frequent in colorectal liver metastases and, on univariate analysis, are prognostic factors for overall and disease-free survival, being associated to extrahepatic recurrence. Presence of poorly differentiated clusters grade 3 stained by hematoxilin and eosin is an independent prognostic factor for overall and disease-free survival, suggesting that it is an important spread pathway in colorectal liver metastase

Nutritional impact of inflammatory bowel diseases on children and adolescents

OBJECTIVE: To perform a sistematiy review of the literature about the nutritional impact of inflammatory bowel diseases in children and adolescents.DATA SOURCES: A systematic review was performed using PubMed/MEDLINE, LILACS and SciELO databases, with inclusion of articles in Portuguese and in English with original data, that analyzed nutritional aspects of inflammatory bowel diseases in children and adolescents. The initial search used the terms "inflammatory bowel diseases" and "children" or "adolescents" and "nutritional evaluation" or "nutrition deficiency". The selection of studies was initially performed by reading the titles and abstracts. Review studies and those withouth data for pediatric patients were excluded. Subsequently, the full reading of the articles considered relevant was performed.RESULTS: 237 studies were identified, and 12 of them were selected according to the inclusion criteria. None of them was performed in South America. During the analysis of the studies, it was observed that nutritional characteristics of patients with inflammatory bowel disease may be altered; the main reports were related to malnutrition, growth stunting, delayed puberty and vitamin D deficiency.CONCLUSION: There are nutritional consequences of inflammatory bowel diseases in children and adolescents, mainly growth stunting, slower pubertal development, underweight and vitamin deficiencies. Nutritional impairments were more significant in patients with Crohn's disease; overweight and obesity were more common in patients with ulcerative rectocolitis. A detailed nutritional assessment should be performed periodically in children and adolescents with inflammatory bowel disease

Prospecção Tecnológica sobre Sistemas de Valoração de Tecnologias Protegidas por Patentes e/ou Registros de Programa de Computador

Based on the existing difficulties in the technology valuation processes, including internal demand from NIT/UNIVASF, this research aims to prospect systems of valuation of technologies to solve this necessity. To this, scientific literature review was carried out, as well as the search for national and international patents, in addition to the search for software registered in Brazil and on public bases. No patents were identified, nor were Computer Program Registries (RPC), which allow the valuation of new technologies, the process of which includes steps to help users and allow interaction between the innovation manager and the inventors. From the technological SWOT and ROADMAP matrix, there is a favorable environment for development and market opportunity for a system that implements a friendly valuation process for users from various graduation.A partir das dificuldades identificadas nos processos de valoração de tecnologias, incluindo demanda interna do NIT/Univasf, esta pesquisa tem como objetivo prospectar sistemas de valoração de tecnologias de forma a atender a essa necessidade. Para tanto, foram realizadas revisão de literatura científica sobre o tema, busca de patentes nacionais e internacionais, além da procura de softwares registrados no Brasil e em bases públicas. Não foram identificadas patentes e nem Registros de Programa de Computador (RPC) que permitam a valoração de novas tecnologias, cujo processo inclua etapas de apoio aos usuários e permita interação do gestor de inovação com os inventores. A partir da matriz SWOT e de Roadmap tecnológico, verificou-se ambiente favorável ao desenvolvimento e oportunidade de mercado para um sistema que implemente um processo de valoração amigável para usuários de várias áreas de formação