Nesidioblastosis in adults.

The persistent hyperinsulinemic hypoglycemia may be caused either by a solitary tumor of the pancreas secreting excessive amount of insulin, known as insulinoma or, rarely, by nesidioblastosis. Nesidioblastosis is a rare cause of persistent hyperinsulinemic hypoglycemia in adults. The incidence of nesidioblastosis in adults is unknown, but it is generally thought to be very low. The ß cell changes in adult nesidioblastosis suggest a dysregulation of the function of the cell. The cause of the functional dysregulation in adults is unknown. The pathogenesis of adult nesidioblastosis may be different from infantile congenital hyperinsulinism caused by a genetic effect. Histologically nesidioblastosis is almost always characterized by a proliferation of abnormal ß cells throughout the entire pancreas. Clinically and biochemically , it is not possible to distinguish between diffuse nesidioblastosis and insulinoma. If all highly selective noninvasive imaging techiques fail to identify a tumor, selective arterial calcium stimulation testing should be performed. The final diagnosis relies on the histopathologic evaluation. The treatment of adult nesidioblastosis is surgical resection of the pancreas.

Nesidioblastosis: hipoglucemia hiperinsulínica persistente en un recién nacido / Nesidioblastosis: persistent hyperinsulinemic hypoglycemia in a newborn

Se presenta el caso clínico de un recién nacido a término por parto distócico (cesárea), debido a una toxemia gravídica, con antecedente de polihidramnios, que manifestó dificultad respiratoria a los pocos minutos del nacimiento y fue ingresado en el Hospital Infantil Norte Dr Juan de la Cruz Martínez Maceira de Santiago de Cuba. Le fueron detectadas cifras de glucemia muy bajas que, evolutivamente, trajeron aparejadas convulsiones tónico-clónicas generalizadas, cuyas frecuencias no se lograban disminuir. Se le diagnosticó una hipoglucemia hiperinsulínica persistente (nesidioblastosis) y fue trasladado a La Habana para recibir tratamientos clínico y quirúrgico definitivos. Actualmente es atendido por un equipo médico multidisciplinario

The case of a term infant by dystocia (cesarean section) due to pregnancy toxemia with history of polyhydramnios is presented, who had respiratory distress a few minutes after birth and he was admitted to Dr Juan de la Cruz Martínez Maceira Northern Children Hospital of Santiago de Cuba. Very low blood glucose levels were detected that progressively caused tonic-clonic seizures, which frequencies could not be reduced. He was diagnosed with persistent hyperinsulinemic hypoglycemia (nesidioblastosis) and was transferred to Havana to receive definitive clinical and surgical treatments. Currently, he is treated by a multidisciplinary medical team

Nesidioblastosis del adulto coexistente con insulinoma / Adult Nesidioblastosis Concurrent with Insulinoma

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.

Adult diffuse nesidioblastosis: genetically or environmentally induced?

Persistent hyperinsulinemic hypoglycemia in adults is usually caused by solitary benign insulinomas. Nesidioblastosis, a term that has been used to designate a functional disorder of the beta cells, is a rare cause of persistent hyperinsulinemic hypoglycemia in adults, but seems to have increased in frequency. The histopathologic criteria for adult nesidioblastosis are similar to those used for the diagnosis of the diffuse form of congenital hyperinsulinism in infants and include beta-cell hypertrophy, islet hyperplasia, and an increase in the beta-cell mass. However, the degree and extent of these features vary much more from patient to patient than in newborns. The cause of adult nesidioblastosis is not known, but the recently observed association with gastric bypass surgery in obese patients suggests that a reactive process possibly unmasks or induces a defect in the beta cell, resulting in its hyperfunction.

[Endogenous persistent hypoglicemia of adult: case report]. / Hipoglicemia hiperinsulinêmica persistente endógena no adulto: relato de caso.

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): 60-99 mg/dl; Serum insulin: 70.9 mcU/ml / RI: < 29.1 mcU/ml; e C peptide: 7.1 ng/ml / RI: 1.1-5.0 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.

Hipoglicemia hiperinsulinêmica persistente endógena no adulto: relato de caso / Endogenous persistent hypoglicemia of adult: case report

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.

[Adult nesidioblastosis]. / Nesidioblastose do adulto.

In adults, endogenous hyperinsulinemic hypoglycaemia is almost invariably due to insulinoma. The authors describe a 29 year old patient with repeated postprandial symptomatic hypoglycaemias and negative prolonged fast test. Distal pancreatectomy was performed, directed by positive arteriographic testing. Histological examination revealed pancreatic nesidioblastosis and no tumoral lesion. Distinct pathogenetic, clinical, diagnostic and therapeutic aspects of this rare disease in the adult are reviewed.