Resumen
Introducción: La nesidioblastosis es una enfermedad poco frecuente en adultos causada por hiperplasia de los islotes pancreáticos, produciendo hipoglicemia por incremento de la producción de insulina. La patogenia es poco conocida, sin embargo se ha descrito un cuadro clínico caracterizado por hipoglicemia con niveles elevados de insulina y péptido C sin ninguna lesión pancreática detectable, su diagnóstico final es histopatológico.
Caso clínico: Se reporta el caso de una paciente de 36 años que cursa con episodios recurrentes de hipoglicemia sintomática a predominio postprandial, asociada a elevación de los niveles de insulina y péptido C, se le realizaron exámenes de imágenes, siendo negativas para insulinoma. Las pruebas posteriores incluyeron la estimulación intraarterial con calcio, revelando marcada liberación de insulina en más de un segmento pancreático; la paciente fue sometida a pancreatectomía corporocaudal confirmandose en el estudio histopatológico el diagnóstico de nesidioblastosis.
Conclusiones: La nesidioblastosis, si bien es bastante rara en poblaciones adultas, se debe considerar como diagnóstico diferencial en pacientes con hipoglicemia persistente; el diagnóstico es difícil y generalmente requiere confirmación histopatológica.
Abstract
Background: Nesidioblastosis is a rare disease in adults caused by pancreatic islet hyperplasia, producing hypoglycemia due to an increase in insulin production. The pathogenesis is poorly understood, however a clinical picture characterized by hypoglycemia with high levels of insulin and C-peptide without any detectable pancreatic lesion has been described, its final diagnosis is histological.
Case report: We report a case of a 36-year-old female patient who presents with recurrent episodes of hypoglycemia symptomatic predominantly postprandial, associated with insulin and C-peptide elevation. Images were made (triphasic computed tomography, magnetic resonance imaging, echoendoscopy and octreoscan), being negative for insulinoma. Subsequent tests included calcium stimulation of the pancreas, revealing marked insulin release in more than one pancreatic segment. The patient was subjected to distal pancreatectomy confirming in the histological study the diagnosis of nesidioblastosis.
Conclusions: Nesidioblastosis accounts for less than 5% of the cases of cases of hyperinsulinemic hypoglycemia in adult patients, should be considered as a differential diagnosis in patients with persistent hypoglycemia, the diagnosis is difficult and usually requires histological confirmation.
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