ABSTRACT
Resumen Se describe el caso de un paciente de 75 años sin antecedentes de relevancia, que fue diagnosticado con insulinoma maligno en estado avanzado, con metástasis hepáticas, con síntomas por hipoglucemia hiperinsulinémica refractaria al tratamiento, y con diazóxido y octreotide de acción corta. El paciente presentó una respuesta clínica poco esperada a la embolización transarterial de metástasis hepáticas, pues a pesar de que persisten las lesiones tumorales, desarrolló hiperglucemia persistente y requirió manejo con insulina. Adicionalmente, se hace una breve revisión de la literatura sobre las opciones terapéuticas disponibles para el tratamiento sintomático de la hipoglucemia hiperinsulinémica.
Abstract The case is presented of a 75 year-old man who was diagnosed with malignant insulinoma in an advanced stage with diffuse liver metastases and symptoms due to hyperinsulinaemic hypoglycaemia refractory to treatment with diazoxide and short-acting octreotide. The patient had an unexpected clinical response to trans-arterial embolisation of liver metastases, since, despite still having the tumour, he developed persistent hyperglycaemia that required insulin treatment. A brief review of the literature is also presented on the treatment options for hyperinsulinaemic hypoglycaemia.
Subject(s)
Humans , Male , Aged , Hyperglycemia , Hypoglycemia , Insulinoma , Neoplasm MetastasisABSTRACT
Background : Hyperinsulinemic hypoglicemia with severe neuroglycopenic symptoms has been identified as a late and rare complication in patients submitted to Roux-en-Y gastric bypass. However, the potential gravity of its manifestations requires effective treatment of this condition. The absence of treatment makes it necessary to develop more effective clinical or surgical methods. Aim : To present one surgical option to revisional surgery in the treatment of hyperinsulinemic hypoglicemia Methods : The procedure consists in reconstituting alimentary transit through the duodenum and proximal jejunum, while keeping the restrictive part of the gastric bypass. As an additional strategy to maintain weight loss, is realized gastric fundus resection, aiming to suppress ghrelin production more effectively. Results : It was used in three patients with successful results in one year of follow-up. Conclusion : The procedure to reconstruct the food transit through the duodenum and proximal jejunum, keeping the restrictive component of gastric bypass in the treatment of hyperinsulinemic hypoglycemia showed good initial results and validated its application in other cases with this indication.
Racional : Hipoglicemia hiperinsulinêmica com sintomas neuroglicopênicos severos tem sido identificada como complicação tardia e rara em pacientes submetidos à gastroplastia com bypass em Y-de-Roux. Porém, a gravidade potencial de suas manifestações exige tratamento definitivo desta condição. A falta de tratamento efetivo gera a necessidade de desenvolver métodos clínicos ou cirúrgicos mais eficazes. Objetivo: Apresentar proposta de operação revisional para o tratamento da síndrome de hipoglicemia hiperinsulinêmica. Métodos : O procedimento consiste em reconstituir o trânsito alimentar pelo duodeno e jejuno proximal, mantendo o componente restritivo do by-pass gástrico. Como estratégia adicional de manutenção ponderal, é realizada ressecção do fundo gástrico, visando intensificar a supressão da grelina. Resultado: O procedimento foi realizado em três pacientes com bom resultado em seguimento de um ano. Conclusão : O procedimento de reconstituir o trânsito alimentar pelo duodeno e jejuno proximal, mantendo o componente restritivo do by-pass gástrico no tratamento da hipoglicemia hiperinsulinêmica apresentou bons resultados iniciais podendo validar sua indicação para outros casos.
Subject(s)
Adult , Female , Humans , Gastric Bypass/adverse effects , Hyperinsulinism/etiology , Hyperinsulinism/surgery , Hypoglycemia/etiology , Hypoglycemia/surgery , ReoperationABSTRACT
OBJETIVO: Rever a apresentação dos casos de hipoglicemia hiperinsulinêmica da infância (HHI), tratamento e histologia nos serviços de endocrinologia pediátrica no Brasil. MATERIAIS E MÉTODO: Os serviços receberam protocolo para resgatar dados de nascimento, resultados laboratoriais, tipo de tratamento instituído, necessidade de pancreatectomia e histologia. RESULTADOS: Vinte e cinco casos de HHI de seis centros foram resgatados, 15 do sexo masculino, 3/25 nascidos de parto normal. A mediana de idade do diagnóstico foi 10,3 dias. As dosagens de glicose e insulina na amostra sérica crítica apresentaram mediana de 24,7 mg/dL e 26,3 UI/dL. A velocidade de infusão de glicose endovenosa foi superior a 10 mg/kg/min em todos os casos (M:19,1). Diazóxido foi utilizado em 15/25, octreotide em 10, corticoide em 8, hormônio de crescimento em 3, nifedipina em 2 e glucagon em 1. Quarenta por cento (10/25) foram pancreatectomizados, nos quais a análise histológica revelou a forma difusa da patologia. CONCLUSÃO: Primeira análise crítica de uma amostra brasileira de portadores de HHI congênita. Arq Bras Endocrinol Metab. 2012;56(9):666-71.
OBJECTIVE: To review the presentation of hyperinsulinemic hypoglycemia of the infancy (HHI), its treatment and histology in Brazilian pediatric endocrinology sections. MATERIALS AND METHOD: The protocol analyzed data of birth, laboratory results, treatment, surgery, and pancreas histology. RESULTS: Twenty-five cases of HHI from six centers were analyzed: 15 male, 3/25 born by vaginal delivery. The average age at diagnosis was 10.3 days. Glucose and insulin levels in the critical sample showed an average of 24.7 mg/dL and 26.3 UI/dL. Intravenous infusion of the glucose was greater than 10 mg/kg/min in all cases (M:19,1). Diazoxide was used in 15/25 of the cases, octreotide in 10, glucocorticoid in 8, growth hormone in 3, nifedipine in 2 and glucagon in 1. Ten of the cases underwent pancreatectomy and histology results showed the diffuse form of disease. CONCLUSION: This is the first critic review of a Brazilian sample with congenital HHI. Arq Bras Endocrinol Metab. 2012;56(9):666-71.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Male , Congenital Hyperinsulinism/diagnosis , Congenital Hyperinsulinism/therapy , Brazil , Blood Glucose/analysis , Congenital Hyperinsulinism/etiology , Insulin/blood , Medical Records , PancreatectomyABSTRACT
El hiperinsulinismo congénito (HC) es la causa más común de hipoglicemia persistente en el primer año de vida. Se acompaña de riesgo elevado de daño neurológico irreversible. En los últimos años se ha profundizado en el conocimiento sobre su patogenia, destacándose la heterogenicidad clínica, histológica y genética, con claras implicancias en el diagnóstico y tratamiento. Se describe el caso de un lactante portador de HC que debuta con hipoglicemias sintomáticas a los 5 meses de vida. El objetivo de esta comunicación es, a la luz de los conocimientos actuales, analizar los criterios diagnósticos y terapéuticos de esta patología. Se destaca la importancia de aplicar un algoritmo para guiar el estudio paraclínico y descartar las etiologías más frecuentes asociadas con la hipoglicemia.
Congenital hyperinsulinism (HC) is the most common etiology of persistent hypoglycemia in infants. It is associated with a high risk of irreversible neurological damage. Knowledge about its pathogenesis has improved in the past few years. It is characterized by a heterogeneous clinical presentation, histology and genetic which leads to special diagnosis and treatment features. This is a case report of a 5 month old with HC who had symptomatic hypoglycemia. The objective of this communication is to analyze the diagnosis and treatment criteria of HC. The need to follow a clinical and laboratory guideline for diagnosis will help exclude other causes of hypoglycemia.