摘要
RESUMEN Introducción: La Nesidioblastosis es una rara afección pancreática que provoca hipoglucemia por hipersinsulinismo endógeno en la infancia. Es poco habitual en el adulto; solo se han publicado casos aislados desde su descripción. Objetivo: Caracterizar la presentación de una hipoglucemia hiperinsulínica en un paciente adulto con Nesidioblastosis. Caso clínico: Paciente adulto de 35 años, blanco, sexo masculino, sospecha de insulinoma, con episodios de hipoglucemia en ayunas o tras un ejercicio que revertía con la ingesta de alimentos o soluciones glucosadas. Se le realizó pancreatectomía de un 85 por ciento y en el estudio histológico se detectó una Nesidioblastosis. Conclusiones: Es infrecuente en el adulto, realizar su diagnóstico es difícil, se llega a la cirugía con el conocimiento de un estado hiperinsulínico endógeno, sin la certeza de su origen(AU)
ABSTRACT Introduction: Nesidioblastosis is a rare pancreatic condition that causes hypoglycemia due to endogenous hypersinsulinism in childhood. It is unusual in adults; only isolated cases have been published including its description. Objective: To characterize a case of hyperinsulinic hypoglycemia, in an adult patient with nesidioblastosis. Clinical case: A 35-year-old, white, male, adult patient with suspected insulinoma, with episodes of hypoglycemia in the fasting state or after exercise that was reversed with ingestion of food or glucose solutions. 85 percent pancreatectomy was performed and nesidioblastosis was detected in the histological study. Conclusions: It is rare in adults, making its diagnosis is difficult, and surgery is reached with the knowledge of an endogenous hyperinsulinic state, without the certainty of its origin(AU)
Subject(s)
Humans , Male , Adult , Pancreatectomy/methods , Nesidioblastosis/diagnosis , Hypoglycemia/diagnostic imaging , Insulinoma/therapy摘要
A 41-year-old woman, multiparous of 4, with personal or familiar history of diabetes, with a history of Nissen fundoplication due to pathological gastro-oesophageal reflux, is refer to an endocrinologjst during her post-operatiye follow up, 4 months after her surgery for a 14 kg weight loss in 10 months and symtomatic hypoglycemia to repetition. A positive prolonged fasting test for hypoglycemia was performed. In addition, abdominal computed axial tomography was performed, which resulted normal and endosonography, showing a lesion on the head of the pancreas. Octreoscan positive for pancreatic head focal lesion with positive somatostatin receptors compatible with insulinoma. Whipple surgery was performed where surgeon palpated pancreatic tumor, biopsy showed tissue compatible with diffuse nesidioblastosis. In the postoperative period, the patient decreased frequency and intensity of hypoglycemic episodes compared to their previous stage. Control prolonged fasting test and Octreoscan were within normal ranges. However, 4 months after surgery, the patient presented hypoglycemia of lower intensity and frequency than before surgery. Currently he remains in control with nutritionist and endocrinologist, mainly adjusting diet and with good control glycemias.
Subject(s)
Humans , Adult , Pancreas/pathology , Nesidioblastosis/diagnosis , Pancreatic Neoplasms/diagnosis , Nesidioblastosis/surgery , Nesidioblastosis/complications , Diagnosis, Differential , Hyperinsulinism/etiology , Hypoglycemia/etiology , Insulinoma/diagnosisSubject(s)
Chromogranins/analysis , Female , Histocytochemistry , Humans , Immunohistochemistry , Infant, Newborn , Microscopy , Nesidioblastosis/complications , Nesidioblastosis/diagnosis , Nesidioblastosis/pathology , Pancreas/pathology , Phosphopyruvate Hydratase/analysis , Seizures/complications , Seizures/diagnosis , Seizures/pathology摘要
La nesidioblastosis es poco común, pero ampliamente reconocida como causa de la hipoglucemia hiperinsulinémica persistente de la infancia. En adultos, el insulinoma es la principal causa de hipoglucemia hiperinsulinémica, pero también se han identificado casos de nesidioblastosis, los cuales se denominan síndrome de hipoglucemia pancreatógena no insulinoma. Los primeros casos de nesidioblastosis del adulto que fueron descritos se asociaron con otras condiciones, como insulinoma y tumores neuroendocrinos pancreáticos y MEN-1. En este artículo se describe un caso de nesidioblastosis coexistente con insulinoma en una paciente de 20 años; se hace una revisión de la literatura; se plantea la discusión sobre su posible etiología, presentación clínica, diagnóstico, tratamiento y, finalmente, se presenta un algoritmo para su estudio.
Nesidioblastosis is infrequent; however, it is widely recognized as the cause of persistent infant hyperinsulinemic hypoglycemia. Among adults, insulinoma is the major cause of hyperinsulinemia hypoglycemia, but identification has also been made of cases of nesidioblastosis which are designated as ´non insulinoma pancreatogena hypoglycemic syndrome´. The first case descriptions of adult nesidioblastosis were associated with other conditions such as insulinoma and neuroenodocrinal pancreatic tumors, and MEN-1. This article describes a case of nesidioblastosis concurrent with insulinoma in a 20-year old patient; the literature is reviewed; discussion is provided on possible etiology, clinical manifestation, diagnosis, treatment, and a concluding algorithm.
Subject(s)
Humans , Female , Young Adult , Hyperinsulinism , Neurodegenerative Diseases , Nesidioblastosis/diagnosis , Nesidioblastosis/etiology , Nesidioblastosis/therapy , Colombia摘要
A hipoglicemia hiperinsulinêmica persistente endógena em adultos é, na maioria dos casos, causada por insulinoma. A Nesidioblastose, uma hiperinsulinemia funcional rara por hiperplasia das células beta do pâncreas, tem sido descrita principalmente em neonatos. Apresentamos o caso de uma paciente de 34 anos com quadro clínico-laboratorial compatível com hipoglicemia hiperinsulinêmica endógena (Glicemia jejum: 54 mg/dl / Valor de referência (VR): 6099 mg/dl; Insulina sérica: 70,9 mcU/ml / VR: < 29,1 mcU/ml; e Peptídeo C: 7,1 ng/ml / VR: 1,15,0 ng/ml - simultâneos à glicemia). Foi aventada a hipótese de insulinoma. Em função da ausência de imagem característica aos exames radiológicos (ultra-som e tomografia de abdome), optou-se pela laparotomia exploradora, onde também não foi evidenciado tumor pancreático macroscopicamente. Os exames histopatológico e imuno-histoquímico evidenciaram hiperplasia de células beta, de segmento distal do pâncreas, compatível com nesidioblastose. A paciente evoluiu com estabilidade clínica por cerca de dois meses, quando se verificou recidiva das crises hipoglicêmicas, refratárias ao uso de Octreotide. Optou-se pela realização de "octreosan", que indicou nesidioblastose difusa, sendo procedida pancreatectomia parcial extensa. Seguiu-se o uso contínuo de Octreotide, com controle eficaz das crises hipoglicêmicas. Uma vez que esse é um diagnóstico raro no adulto, objetiva-se, nesse artigo, divulgar o manejo diagnóstico-terapêutico em casos de hipoglicemia hiperinsulinêmica endógena.
Persistent Hyperinsulinemic Endogenous hypoglycemia in adults is, in most cases, due to Insulinoma. Nesidioblastosis, a peculiar functional hyperinsulinemia from hypertrophic beta cells, has been described mainly in newborns. This article describes a 34-year-old patient who presented hyperinsulinemic endogenous hypoglycemia clinical and laboratorial situation (Fasting glycemia: 54 mg/dl / Reference Interval (RI): 6099 mg/dl; Serum insulin: 70.9 mcU/ml / RI: < 29.1 mcU/ml; e C peptide: 7.1 ng/ml / RI: 1.15.0 ng/ml). It was suspected Insulinoma. Because of the lack of typical images in radiologic exams (ultrasonography and computerized tomography) it had been decided to do laparotomy, but it was not found any macroscopic pancreatic tumor. Histological and histochemistry examination of a distal pancreatic segment showed alteration suitable to nesidioblastosis. The patient presented clinical stability during the next two months, however, after that, there was a recurrence of a hypoglycemia crisis, refractory to Octreotide administration. It was done "octreoscan", which showed expanded nesidioblastosis, being done extensive partial pancreatectomy. Octreotide was used again, with a good control of the hypoglycemia crisis. As it is an uncommon diagnosis in an adult, the objective of this article is to describe the diagnostic and therapeutic aspects in cases of hyperinsulinemic endogenous hypoglicemia.