Nesidioblastosis en una escolar, a propósito de un caso
Date
Subject
Nesidioblastosis
hypoglycemia
hyperinsulinism
pancreatectomy
Nesidioblastosis
hipoglucemia
hiperinsulinismo
pancreatectomía
hypoglycemia
hyperinsulinism
pancreatectomy
Nesidioblastosis
hipoglucemia
hiperinsulinismo
pancreatectomía
Language:
Journal Title
Journal ISSN
Volume Title
Publisher
Intituto Tecnológico de Santo Domingo (INTEC)
Introducción: La nesdioblastosis se define como proliferación de las células endocrinas pancreáticas, que comporta alteraciones en su distribución y diferenciación. El número de los islotes de Langerhans es menor de lo normal, encontrándose las células endocrinas distribuidas, anárquicamente, por el parénquima pancreático, en forma de pequeños nidos o cordones1.
Objetivo: Describir un caso clínico de nesidioblastosis en una paciente en edad escolar, con historia de convulsiones tonicoclónicas desde 1 año de edad, tratado con Oxcarbazepina. Presentando hipoglucemias sintomáticas desde 1 año y medio, por lo que fue referida a la consulta de endocrinología.
Presentación del caso clínico: Paciente femenina de 7 años y 5 meses de edad cronológica, referida al departamento de endocrinología con historia de enfermedad actual, caracterizada diaforesis y mareos; presenta movimientos tónico-clónicos generalizados, retroversión ocular con pérdida del estado de conciencia (en 2 oportunidades), es trasladada a centro médico donde evidencian glucemia en 18-11-32-16 mg/dl respectivamente, colocan solución dextrosa con mejoría momentánea. Permanece por 9 días sin recuperación completa y aparecen fiebre de 3 días de evolución; Es trasladada al Hospital Infantil Robert Reid Cabral (RRC), donde se estudia y se confirman las hipoglucemias, analíticas que revelan hormonas contrarreguladoras elevadas, niveles de insulina en 2,700 mui/ml. Se estabiliza con octreotide y se decide llevar a cirugía para pancreactectomia, realizándose laparotomía abierta. Biopsia trans-operatoria reportó: hiperplasia de las células de los islotes de Langerhans. Por lo que se extirpa un 95% de tejido pancreático, presenta hiperglicemias y se inicia tratamiento con análogos de insulina de acción lenta (0.14 ud/kg/d), enzimas pancreáticas 10,000ud, 15 min previo a comidas y multivitamínicos.
Al mes de la cirugía la paciente vuelve a presentar signos de hipoglicemias, se suspende la insulina y se ingresa, se verifican las hipoglicemias y se realiza una segunda intervención quirúrgica. También se realiza excéresis de 5% de páncrea; segundada porción del duodeno, con anastomosis terminal. Actualmente responde al tx con insulina análoga.
Conclusión: La nesidioblastosis en una causa rara de hipoglucemia por hiperinsulinismo endógeno en el escolar, clínicamente es indistinguible del insulinoma; bioquímicamente es necesario documentar la hipoglucemia hiperinsulinémica durante el ayuno, el tratamiento de elección es la cirugía y seguimiento médico a los déficits secundarios a la misma.
Introduction: Necidioblastosis is defined as proliferation of pancreatic endocrine cells, which involves alterations in their distribution and differentiation. The number of the islets of Langerhans is lower than normal, with the endocrine cells distributed anarchically by the pancreatic parenchyma in the form of small nests or cords1. Objective: To describe a clinical case of nesidioblastosis in a patient of school age, with a history of tonic-clonic seizures from 1 year of age, treated with Oxcarbazepine. Presenting symptomatic hypoglycaemia from 1 year and a half, so it was referred to the endocrinology clinic. Presentation of the clinical case: Female patient of 7 years and 5 months of chronological age, referred to the department of endocrinology with a history of current illness, characterized by diaphoresis and dizziness; presents generalized tonic-clonic movements, ocular retroversion with loss of consciousness (on 2 occasions), is transferred to a medical center where they demonstrate glycemia at 18-11-32-16 mg / dl respectively, place dextrose solution with momentary improvement. It remains for 9 days without complete recovery and fever appears 3 days of evolution and is transferred to the Robert Reid Cabral Children's Hospital, where it is studied and confirmed hypoglycemia, laboratory tests that reveal high counter-regulating hormones, insulin levels in 2,700 mui / ml, stabilizes With octreotide and it is decided to carry out surgery for pancreactectomy, open laparotomy is performed. Trans-operative biopsy reported: hyperplasia of the islet cells of Langerhans. So 95% of pancreatic tissue is removed, presents hyperglycemia and treatment is started with slim-action insulin analogs (0.14 ud / kg / d) pancreatic enzymes 10,000d, 15 min prior to meals, multivitamin. One month after surgery, the patient presented signs of hypoglycemia again, insulin was discontinued and the patient was admitted, hypoglycemia was verified, and second surgical intervention was performed. An excess of 5% of the pancreas and 2nd portion of the duodenum was performed with an end-to-end anastomosis. Currently responding to tx with analogous insulin. Conclusion: Nesidioblastosis in a rare cause of hypoglycaemia due to endogenous hyperinsulinism in the school, clinically is indistinguishable from insulinoma; biochemically it is necessary to document hyperinsulinemic hypoglycaemia during fasting, the treatment of choice is surgery and medical treatment to the deficits 2nd to it.
Introduction: Necidioblastosis is defined as proliferation of pancreatic endocrine cells, which involves alterations in their distribution and differentiation. The number of the islets of Langerhans is lower than normal, with the endocrine cells distributed anarchically by the pancreatic parenchyma in the form of small nests or cords1. Objective: To describe a clinical case of nesidioblastosis in a patient of school age, with a history of tonic-clonic seizures from 1 year of age, treated with Oxcarbazepine. Presenting symptomatic hypoglycaemia from 1 year and a half, so it was referred to the endocrinology clinic. Presentation of the clinical case: Female patient of 7 years and 5 months of chronological age, referred to the department of endocrinology with a history of current illness, characterized by diaphoresis and dizziness; presents generalized tonic-clonic movements, ocular retroversion with loss of consciousness (on 2 occasions), is transferred to a medical center where they demonstrate glycemia at 18-11-32-16 mg / dl respectively, place dextrose solution with momentary improvement. It remains for 9 days without complete recovery and fever appears 3 days of evolution and is transferred to the Robert Reid Cabral Children's Hospital, where it is studied and confirmed hypoglycemia, laboratory tests that reveal high counter-regulating hormones, insulin levels in 2,700 mui / ml, stabilizes With octreotide and it is decided to carry out surgery for pancreactectomy, open laparotomy is performed. Trans-operative biopsy reported: hyperplasia of the islet cells of Langerhans. So 95% of pancreatic tissue is removed, presents hyperglycemia and treatment is started with slim-action insulin analogs (0.14 ud / kg / d) pancreatic enzymes 10,000d, 15 min prior to meals, multivitamin. One month after surgery, the patient presented signs of hypoglycemia again, insulin was discontinued and the patient was admitted, hypoglycemia was verified, and second surgical intervention was performed. An excess of 5% of the pancreas and 2nd portion of the duodenum was performed with an end-to-end anastomosis. Currently responding to tx with analogous insulin. Conclusion: Nesidioblastosis in a rare cause of hypoglycaemia due to endogenous hyperinsulinism in the school, clinically is indistinguishable from insulinoma; biochemically it is necessary to document hyperinsulinemic hypoglycaemia during fasting, the treatment of choice is surgery and medical treatment to the deficits 2nd to it.
Description
Type
info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
info:eu-repo/semantics/publishedVersion
Source
Science and Health; Vol 2 No 3 (2018): Science and Health, september-december; 63-68
Ciencia y Salud; Vol. 2 Núm. 3 (2018): Ciencia y Salud, septiembre-diciembre; 63-68
2613-8824
2613-8816
10.22206/cysa.2018.v2i3
Ciencia y Salud; Vol. 2 Núm. 3 (2018): Ciencia y Salud, septiembre-diciembre; 63-68
2613-8824
2613-8816
10.22206/cysa.2018.v2i3