Congenital hyperinsulinism and surgical outcome in a single tertiary center in Brazil

Abstract Objective: Congenital hyperinsulinism (CHI) is a heterogeneous genetic disease characterized by increased insulin secretion and causes persistent hypoglycemia in neonates and infants due to dysregulation of insulin secretion by pancreatic β cells. Babies with severe hypoglycemia and for whom medical treatment has been ineffective usually require surgical treatment with near-total pancreatectomy. To evaluate the clinical and surgical aspects affecting survival outcomes in babies diagnosed with CHI in a single tertiary care center. Methods: Retrospective Cohort study involving a single university tertiary center for the treatment of CHI. The authors study the demographics, clinical, laboratory, and surgical outcomes of this casuistic. Results: 61 % were female, 39 % male, Birth weight: 3576 g (±313); Age of onset of symptoms: from the 2nd hour of life to 28 days; Time between diagnosis and surgery ranged between 10 and 60 days; Medical clinical treatment, all patients received glucose solution with a continuous glucose infusion and diazoxide. 81 % of the patients used corticosteroids, 77 %. thiazide, 72 % octreotide, 27 % nifedipine; Neurological sequelae during development and growth: 54 % had some degree of delay in neuropsychomotor development, 27 % obesity. Surgery was performed open in 6 and 12 minimally invasive surgery (MIS). Histopathology: 2 focal and 16 diffuse, Length of stay (days) was lower in MIS (p < 0.05). Survival was 100 %. Conclusions: CHI is a rare and difficult-to-manage tumor that must be performed in a multidisciplinary and tertiary center. Most surgical results are good and the laparoscopic approach to disease has been the best choice for patients.

Congenital hyperinsulinism and surgical outcome in a single tertiary center in Brazil.

OBJECTIVE: Congenital hyperinsulinism (CHI) is a heterogeneous genetic disease characterized by increased insulin secretion and causes persistent hypoglycemia in neonates and infants due to dysregulation of insulin secretion by pancreatic ß cells. Babies with severe hypoglycemia and for whom medical treatment has been ineffective usually require surgical treatment with near-total pancreatectomy. To evaluate the clinical and surgical aspects affecting survival outcomes in babies diagnosed with CHI in a single tertiary care center. METHODS: Retrospective Cohort study involving a single university tertiary center for the treatment of CHI. The authors study the demographics, clinical, laboratory, and surgical outcomes of this casuistic. RESULTS: 61 % were female, 39 % male, Birth weight: 3576 g (±313); Age of onset of symptoms: from the 2nd hour of life to 28 days; Time between diagnosis and surgery ranged between 10 and 60 days; Medical clinical treatment, all patients received glucose solution with a continuous glucose infusion and diazoxide. 81 % of the patients used corticosteroids, 77 %. thiazide, 72 % octreotide, 27 % nifedipine; Neurological sequelae during development and growth: 54 % had some degree of delay in neuropsychomotor development, 27 % obesity. Surgery was performed open in 6 and 12 minimally invasive surgery (MIS). HISTOPATHOLOGY: 2 focal and 16 diffuse, Length of stay (days) was lower in MIS (p < 0.05). Survival was 100 %. CONCLUSIONS: CHI is a rare and difficult-to-manage tumor that must be performed in a multidisciplinary and tertiary center. Most surgical results are good and the laparoscopic approach to disease has been the best choice for patients.

Circumcision as treatment for Zoon's balanitis in an HIV-positive teenage patient.

In this report we describe a case of the Zoon's balanitis in a boy with HIV (AIDS B2). The clinical presentation, failure of topical treatment, cure by circumcision, and the histopathology findings are presented.

Phimotic ring topical corticoid cream (0.1% mometasone furoate) treatment in children.

BACKGROUND/PURPOSE: Phimosis, owing to the presence of a preputial fibrotic ring, is surgically treated in 1% of children. During the last decade, however, topical steroid treatment has been proposed for phimosis. METHODS: We present a double-blind study comparing 0.1% mometasone furoate topical cream vs moisturizing cream (placebo) for the treatment of phimosis. Children aged from 2 to 13 years (n = 110) presenting with phimosis (Kikiro's classification grade 5) and scheduled for circumcision were included in this trial. The patients were evaluated after 8 weeks of topical treatment with moisturizing cream (n = 54) or steroid cream (n = 56). Nonresponders from both groups received an additional 8 weeks of steroid cream treatment. RESULTS: In the steroid group, the ring disappeared and glans exposure was obtained in 49 (88%) of 56 patients vs 28 (52%) of 54 patients in the placebo group (P < .05). After a second treatment, in the steroid group, 5 of the 7 patients were finally cured vs 22 of the 26 in the placebo group (P < .05). Two children with persisting phimosis (Kikiro's retractability grade 5 and appearance grade 3) in the steroid group (4%) vs 4 children in the placebo group (7%) ended up receiving postectomy. CONCLUSIONS: The present investigation adds up and supports the effectiveness of phimosis topical corticoid treatment. Nevertheless, hygiene and preputial traction, when appropriately performed, seem to play an important role in the disappearance of the phimotic ring as well. New studies are necessary to confirm if this is true or not.

Hepatectomia parcial em cirurgia pediátrica / Partial hepatectomy in pediatric surgery

OBJETIVO: Embora, atualmente, as indicações de hepatectomias em crianças sejam menos frequentes, em alguns casos elas constituem a melhor opção terapêutica. O objetivo deste trabalho é relatar a experiência de dez anos com grandes ressecções hepáticas em pacientes pediátricos. MÉTODO: Foram analisados os dados de doze pacientes submetidos a lobectomia hepática nos serviços de Cirurgia Pediátrica do Hospital da Clínicas da Faculdade de Medicina da Universidade de São Paulo e do Hospital Santa Lydia, em Ribeirão Preto (SP) de 1985 a 1995. RESULTADOS: Foram realizadas oito lobectomias esquerdas e quatro lobectomias direitas. Dez crianças foram operadas por neoplasia e duas por complicações de traumatismo hepático. A idade das dez crianças portadoras de neoplasia variou de seis dias a dezesseis meses, sendo em média 3,8 meses. O diagnóstico histopatológico foi hemangioendotelioma em cinco (50 por cento), hepatoadenoma em dois (20 por cento), hepatoblastoma em dois (20 por cento) e hepatocarcinoma em um (10 por cento). O peso do tumor correspondeu em média a 7,1 por cento do peso do paciente. A duração média da cirurgia foi de 2 horas e 58 minutos. O seguimento pós-operatório variou até 141 meses, sendo em média 76,5 meses. Sete pacientes receberam transfusão de sangue intra-operatória, correspondente a 23,3 por cento de sua volemia, em média. Um deles apresentou recidiva de tumor, necessitando reoperação. Nenhum dos doze pacientes apresentou complicações pós-operatórias. CONCLUSÃO: A hepatectomia parcial é um procedimento difícil tecnicamente, que, no entanto, pode ser realizado com segurança, mesmo em hospitais que não disponham de recursos tecnológicos sofisticados, desde que o cirurgião esteja bem preparado para enfrentar suas dificuldades.

BACKGROUND: Although less frequent in childhood nowadays, hepatectomies may still be the best choice in some cases. Our objective is to describe a 10-year experience with large hepatic resections in pediatric patients. METHODS: A retrospective review of clinical and surgical data of 12 patients submitted to hepatic lobectomy, at Pediatric Surgery Service of Hospital das Clinicas da Faculdade de São Paulo and Hospital Santa Lydia, Ribeirão Preto (Brazil), between 1985 and 1995. RESULTS: There were eight left lobectomies and four right lobectomies. The indication of surgery were neoplastic diseases in 10 children, and complicated hepatic trauma in two. The age range was six days to 16 months in children with hepatic tumors, and the mean age was 3,8 months. Histopathological diagnosis were hemangioendothelioma in five (50 percent), hepatic adenoma in two (20 percent), hepatoblastoma in two (20 percent), and hepatic carcinoma in one (10 percent). Mean tumor weight was 7,1 percent of body weight. Mean surgical time was 2h 58m. Postoperative follow-up was up to 141 months, and mean follow-up was 76,5 months. Seven patients received intraoperative blood transfusion, corresponding to 23,3 percent of volemy. Only one patient had a late reoperation for resection of a new tumor. There were no postoperative complications. CONCLUSION: Although hepatic lobectomy is a major surgery, and undoubtedly technically difficult, it can be safely performed without expensive devices and sophisticated methods (some of them not always available in common hospitals) if the surgeon is skilled and well prepared for such a procedure.

Atresia de vias biliares: estudo de 19 casos / Biliary Atresia: study of 19 patients

O estudo foi realizado através da análise e coleta de dados dos prontuários de 19 pacientes internados no Hospital das Clínicas da Faculdade de Medicina de Ribeiräo Preto, no período de 1982 a 1992, com o diagnóstico de atresia de vias biliares, os quais foram submetidos à cirurgia de derivaçäo biliar, sendo 12 meninas e 7 meninos. A cirurgia de derivaçäo foi realizada em 16 pacientes e, sempre que possível, as crianças foram operadas antes de 12 semanas de vida. Os examespré-operatórios que contribuíram para o diagnóstico säo apresentados. Concluiu-se que um adequado protocolo para acompanhamento dos pacientes com icterícia colestática é muito necessário, colaborando para um diagnóstico precoce e uma busca de melhor sobrevida para essas crianças.

Cirurgia de urgência no recém-nascido / Emergency surgery in the newborn

O tratamento cirúrgico das urgências neonatais exige recursos técnicos e humanos altamente especializados, e constitui importante segmento da Cirurgia Pediátrica. Dentre inúmeras afecçöes seräo abordadas, por sua incidência e gravidade, a atresia de esôfago, a hérnia diafragmática, as obstruçöes duodenais congênitas (atresia duodenal, pâncreas anual, a má-rotaçäo intestinal e membrana duodenal), as atresias jejuno-ileais, a doença de Hirschsprung, as anomalias ano-retais em suas várias formas, o íleo e a peritonite meconiais, a onfalocele, a gastrosquise e a enterite necrotizante neonatal. Aspectos clínicos e da fisiologia, bem como a conduta diagnóstica e os cuidados pré-, intra e pós operatórios säo analisados aqui, de maneira sucinta e objetiva

Urgências abdominais näo traumáticas na criança / Non-traumatic abdominal emergencies in children

As urgências abdominais näo traumáticas da criança apresentam características próprias, relacionadas às diferentes faixas etárias. Discutem-se, neste artigo, a orientaçäo diagnóstica e os cuidados pré-, intra- e pós-operatórios de algumas dessas urgências, tais como apendicite aguda, hérnia inguinal encarcerada, estenose hipertrófica de piloro, intussuscepçäo intestinal, divertículo de Meckel e obstruçäo por bolo de áscaris