International Latin American survey on pediatric intestinal failure team

There is little data on the experience of managing pediatric Intestinal Failure (IF) in Latin America. This study aimed to identify and describe the current organization and practices of the IF teams in Latin America and the Caribbean. An online survey was sent to inquire about the existence of IF teams that managed children on home parenteral nutrition (HPN). Our questionnaire was based on a previously published European study with a similar goal. Twenty-four centers with pediatric IF teams in eight countries completed the survey, representing a total number of 316 children on HPN. The median number of children on parenteral nutrition (PN) at home per team was 5.5 (range 1–50). Teams consisted of the following members: pediatric gastroenterologist and a pediatric surgeon in all teams, dietician (95.8%), nurse (91.7%), social worker (79.2%), pharmacist (70.8%), oral therapist (62.5%), psychologist (58.3%), and physiotherapist (45.8%). The majority of the centers followed international standards of care on vascular access, parenteral and enteral nutrition, and IF medical and surgical management, but a significant percentage reported inability to monitor micronutrients, like vitamins A (37.5%), E (41.7%), B1 (66.7%), B2 (62.5%), B6 (62.5%), active B12 (58.3%); and trace elements—including zinc (29.2%), aluminum (75%), copper (37.5%), chromium (58.3%), selenium (58.3%), and manganese (58.3%). Conclusion: There is wide variation in how IF teams are structured in Latin America—while many countries have well-established Intestinal rehabilitation programs, a few do not follow international standards. Many countries did not report having an IF team managing pediatric patients on HPN

Fatores determinantes para a escolha da especialidade médica no Brasil

Fundamento. A escolha da especialidade médica é complexa e multifatorial. A importância dos diferentes fatores varia mundialmente e está associada a diferentes valores e sistemas de educação. O objetivo do estudo é identificar os fatores que determinam essa escolha no Brasil. Métodos. Um questionário foi enviado, via internet (eletronic mail), para graduandos dos seis anos do curso de medicina, solicitando informações demográficas e a avaliação de fatores que influenciam na escolha da especialidade médica. Resultados. A idade média foi de 22 anos. Quanto à influência de familiares, 42,5% dos alunos têm pelo menos um médico como parente próximo, sendo as especialidades mais comuns desse parente: Pediatria, Ginecologia e Obstetrícia e Oftalmologia. A quantidade de respondentes que declararam certeza da carreira a ser seguida ao entrar na faculdade foi 19%, enquanto essa certeza foi de 22,2% na ocasião da pesquisa. Afinidade pela especialidade e estilo de vida foram fatores avaliados como importantes ou muito importantes por mais de 95% dos respondentes. Urgência em ganhar dinheiro rápido, tempo curto de residência, alto rendimento inicial e influência familiar foram os fatores avaliados como menos importantes pelos respondentes na escolha de sua especialidade. Quase metade dos respondentes (49,7%) descartaram uma especialidade que cogitaram fortemente, as principais razões para isso foram ter descoberto não ter afinidade pela especialidade pela qualidade de vida. Conclusões. Afinidade e estilo de vida são os fatores determinantes da escolha e da rejeição de especialidade médica em nosso meio.Background. Choosing medical specialty is complex and depends on multiple factors. The importance of the different factors varies across the world, according to different values and systems of education. The purpose of this study is to identify the determining factors involved in this choice among medical students in Brazil. Methods. A questionnaire was sent by email to medical students from first to sixth year of a selected institution, asking demographic information and the evaluation of factors that may influence the choice of the medical specialty. Results. The mean age was 22 years. Regarding the influence of relatives, 42.5% of the students has at least one physician as a close relative, the most common specialties of this relative were: Pediatrics, Gynecology and Obstetrics and Ophthalmology. 19% of the respondents declared being sure of the career when they entered University, while 22.2% of them were sure about the choice at the occasion of the study. Affinity for the specialty and lifestyle were evaluated as important or very important by more than 95% of respondents. Urge to earn money fast, short time of medical residency, high initial profit and family influence were the factors evaluated as less important by the respondents. Almost half of the respondents (49.7%) rejected a specialty they had strongly cogitated once. The main reasons for this were the lifestyle and the fact that they discovered not having affinity for the specialty. Conclusions. Affinity and medical lifestyle are the defining factors in choosing and rejecting a medical specialty in Brazil

Current Predictors of achievement of Enteral Autonomy in children with Pediatric Intestinal Failure secondary to Short Bowel Syndrome

INTRODUÇÃO: Nas últimas décadas, houve avanços significativos no manejo da Falência Intestinal (FI), que resultou em melhora dos desfechos. A obtenção da autonomia enteral (AE) é o objetivo final do tratamento na FI pediátrica. OBJETIVOS: Nosso objetivo foi avaliar os fatores prognósticos atuais para obtenção da AE na Síndrome do Intestino Curto (SIC) pediátrica e explorar o impacto do comprimento residual do intestino delgado e do cólon na obtenção da AE, em uma coorte de pacientes de seis programas de reabilitação intestinal pediátrica. MÉTODOS: Análise retrospectiva de uma coorte multicêntrica de lactentes <12 meses com SIC (n= 367) diagnosticados entre 2010 e 2015 (2017 em um centro) e acompanhados até 2019. A coorte foi classificada de acordo com a obtenção de AE vs. dependência de nutrição parenteral (NP). Os testes estatísticos incluíram um teste t-Student, qui-quadrado, log-rank para análise univariada e regressão Cox Proportional Hazards para análise multivariada. Para explorar o impacto do comprimento residual do intestino delgado e do colón no alcance da AE, um gráfico de dispersão da porcentagem do intestino residual (esperado para a idade) dividiu os pacientes em quatro quadrantes anatômicos - 50% vs. <50% de intestino delgado e cólon. Uma curva de Kaplan-Meier foi criada com base nesses estratos anatômicos. Uma análise do tempo até o evento também foi realizada comparando a EA de acordo com a etiologia (enterocolite necrosante vs. outras etiologias). RESULTADOS: A AE foi alcançada em 229 pacientes (62,3%). Na análise univariada, os pacientes que atingiram a AE apresentavam maior porcentagem de intestino delgado remanescente (54,4% vs. 30,3; p<0,0001) e comprimento residual do cólon (87,9 vs. 66,8%; p<0,001), e uma maior porcentagem desses pacientes teve a válvula ileocecal (VIC) preservada (68 vs. 26%; p<0,001). Na análise multivariada, a porcentagem de comprimento residual do cólon (Hazards ratio - HR= 1,02; Intervalo de Confiança - IC 95% 1,011,02) e do intestino delgado (HR= 1,01; IC 95% 1,011,02) e preservação da VIC (HR= 2,02; IC 95% 1,41 2,88) foram positivamente associados com o alcance da AE. A capacidade de atingir AE foi significativamente diferente entre os subgrupos anatômicos com base no teste de Log-rank (p<0,001): 80,4% dos lactentes com 50% do cólon e delgado (tempo mediano para AE - 209 dias); 62,5% com 50% do delgado e <50% do cólon (tempo mediano - 397 dias); 58,3% com <50% do delgado e 50% do cólon (tempo mediano - 1192 dias), 25,9% das crianças com <50% de ambos os intestinos. O diagnóstico de enterocolite necrosante não foi associado a uma melhor obtenção de AE (ECN vs. outras etiologias; teste Log-rank p+= 0,33). CONCLUSÕES: No geral, 62% dos lactentes com FI secundária à SIC alcançaram AE em um tempo médio de acompanhamento de 2,3 anos. A extensão da ressecção intestinal e a consequente anatomia intestinal são fatores prognósticos críticos na SIC pediátricaINTRODUCTION: Over the past decades, there have been significant advancements in the management of Intestinal failure (IF), resulting in improvement of outcomes. Achievement of Enteral Autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure. AIMS: We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA, in a cohort of patients from six pediatric intestinal rehabilitation programs. METHODS: Retrospective analysis of a multicenter cohort of infants <12 months with SBS (n=367) diagnosed between 2010 and 2015 (2017 in 1 center) and followed until 2019. The cohort was stratified according to the achievement of EA vs. parenteral nutrition (PN) dependency. Statistical testing included a two-sample t-test, Chi-Square, log-rank for univariate analysis, and Cox Proportional Hazards regression for multivariable analysis. To explore the impact of residual small bowel (SB) and large bowel (LB) length on EA, a scatterplot of percent residual bowel (adjusted for age) divided patients into four anatomical quadrants 50% vs. <50% of LB and SB. A KaplanMeier curve was created based on these anatomical strata. A time-to-event analysis was also performed comparing EA according to etiology (necrotizing enterocolitis vs. other etiologies). RESULTS: EA was achieved in 229 patients (62.3%). In univariate analysis, patients who achieved EA were more likely to have shorter percent residual SB (54.4 vs 30.3 %; p<0.0001) and residual LB length (87.9 vs 66.8%; p<0.001), and less likely to have an ileocecal valve (ICV, 68 vs 26%; p<0.001). In the multivariable analysis, percentage of residual LB (HR=1.02; 95% Confidence Interval - CI 1.011.02) and SB (HR= 1.01; 95% CI 1.011.02) length, and presence of ICV (HR= 2.02; 95% CI 1.412.88) were positively associated with EA. Capacity to achieve EA was significantly different between the anatomical subgroups based on the Log-rank test (p<0.001): 80.4% of infants with 50% SB and LB (median time to achieve EA - 209 days); 62.5% with 50% SB and<50% LB (median time - 397 days); 58.3% with <50% SB and 50% LB (median time - 1192 days), 25.9% of children with < 50% SB and LB. Diagnosis of NEC was not associated with a better achievement of EA (necrotizing enterocolitis vs. other etiologies; Log-rank test p = 0.33). CONCLUSIONS: Overall 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. The degree of bowel resection and the consequent bowel anatomy are critical prognostic factors in pediatric SB

Nutritional treatment of a young infant with cystic fibrosis presenting with severe kwashiorkor dermatosis

Kwashiorkor in infancy is typically associated to an underlying disease. Edema, a striking feature of this type of malnutrition, can be difficult to assess in this age group. The typical dermatosis of Kwashiorkor is not fully explained the deficiency of one isolated vitamin or micronutrient. This article presents an infant with cystic fibrosis, who developed Kwashiorkor in the third month of life with extensive cutaneous manifestations. An early, individualized and aggressive nutritional intervention with optimized supplementationof sulfur amino acids, vitamins and micronutrients was established, with impressively recovery of overall nutrition and skin manifestations in a relatively short period of time65663463

Artrogripose renal colestática diagnosticada a partir de colestase neonatal: relato de caso

Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare autosomal recessive syndrome, with multisystemic manifestations and mainly characterized by arthrogriposis, renal dysfunction and cholestasis. The prognosis is poor and most patients die within the first year of life. This is a case report of a female infant, 37 days old, referred to a tertiary hospital due to neonatal cholestasis. Upon evaluation, she also presented with Fanconi syndrome, arthrogryposis, malnutrition, ichthyosis and agranular platelets, thus receiving the clinical diagnosis of ARC syndrome. The liver biopsy showed signs of neonatal hepatitis. Her admission was complicated by dehydration, worsening of metabolic acidosis and acute respiratory failure. The infant was discharged at the age of 3 months, in palliative care, and later died. As it is a very rare disease, knowledge of its characteristics is crucial for appropriated diagnostic evaluation and differential diagnosis with other causes of cholestasis, as well as adequate management

Can the learning of laparoscopic skills be quantified by the measurements of skill parameters performed in a virtual reality simulator?

Purpose To ensure patient safety and surgical efficiency, much emphasis has been placed on the training of laparoscopic skills using virtual reality simulators. The purpose of this study was to determine whether laparoscopic skills can be objectively quantified by measuring specific skill parameters during training in a virtual reality surgical simulator (VRSS). Materials and Methods Ten medical students (with no laparoscopic experience) and ten urology residents (PGY3-5 with limited laparoscopic experience) were recruited to participate in a ten-week training course in basic laparoscopic skills (camera, cutting, peg transfer and clipping skills) on a VRSS. Data were collected from the training sessions. The time that individuals took to complete each task and the errors that they made were analyzed independently. Results The mean time that individuals took to complete tasks was significantly different between the groups (p < 0.05), with the residents being faster than the medical students. The residents' group also completed the tasks with fewer errors. The majority of the subjects in both groups exhibited a significant improvement in their task completion time and error rate. Conclusion The findings in this study demonstrate that laparoscopic skills can be objectively measured in a VRSS based on quantified skill parameters, including the time spent to complete skill tasks and the associated error rate. We conclude that a VRSS is a feasible tool for training and assessing basic laparoscopic skills

Does Training Laparoscopic Skills in a Virtual Reality Simulator Improve Surgical Performance?

Background and Purpose: Several different methods of teaching laparoscopic skills have been advocated, with virtual reality surgical simulation (VRSS) being the most popular. Its effectiveness in improving surgical performance is not a consensus yet, however. The purpose of this study was to determine whether practicing surgical skills in a virtual reality simulator results in improved surgical performance. Materials and Methods: Fifteen medical students recruited for the study were divided into three groups. Group I (control) did not receive any VRSS training. For 10 weeks, group II trained basic laparoscopic skills (camera handling, cutting skill, peg transfer skill, and clipping skill) in a VRSS laparoscopic skills simulator. Group III practiced the same skills and, in addition, performed a simulated cholecystectomy. All students then performed a cholecystectomy in a swine model. Their performance was reviewed by two experienced surgeons. The following parameters were evaluated: Gallbladder pedicle dissection time, clipping time, time for cutting the pedicle, gallbladder removal time, total procedure time, and blood loss. Results: With practice, there was improvement in most of the evaluated parameters by each of the individuals. There were no statistical differences in any of evaluated parameters between those who did and did not undergo VRSS training, however. Conclusion: VRSS training is assumed to be an effective tool for learning and practicing laparoscopic skills. In this study, we could not demonstrate that VRSS training resulted in improved surgical performance. It may be useful, however, in familiarizing surgeons with laparoscopic surgery. More effective methods of teaching laparoscopic skills should be evaluated to help in improving surgical performance.University of Sao Paulo (USP) Medical Schoo

International Latin American Survey on Pediatric Intestinal Failure Team

There is little data on the experience of managing pediatric Intestinal Failure (IF) in Latin America. This study aimed to identify and describe the current organization and practices of the IF teams in Latin America and the Caribbean. An online survey was sent to inquire about the existence of IF teams that managed children on home parenteral nutrition (HPN). Our questionnaire was based on a previously published European study with a similar goal. Twenty-four centers with pediatric IF teams in eight countries completed the survey, representing a total number of 316 children on HPN. The median number of children on parenteral nutrition (PN) at home per team was 5.5 (range 1–50). Teams consisted of the following members: pediatric gastroenterologist and a pediatric surgeon in all teams, dietician (95.8%), nurse (91.7%), social worker (79.2%), pharmacist (70.8%), oral therapist (62.5%), psychologist (58.3%), and physiotherapist (45.8%). The majority of the centers followed international standards of care on vascular access, parenteral and enteral nutrition, and IF medical and surgical management, but a significant percentage reported inability to monitor micronutrients, like vitamins A (37.5%), E (41.7%), B1 (66.7%), B2 (62.5%), B6 (62.5%), active B12 (58.3%); and trace elements—including zinc (29.2%), aluminum (75%), copper (37.5%), chromium (58.3%), selenium (58.3%), and manganese (58.3%). Conclusion: There is wide variation in how IF teams are structured in Latin America—while many countries have well-established Intestinal rehabilitation programs, a few do not follow international standards. Many countries did not report having an IF team managing pediatric patients on HPN