Abstract
Neonatal and infantile hypoglycemia due to insulin excess has been associated with a spectrum of anatomic changes, i.e., nesidioblastosis, endocrine cell dysplasia, islet cell hyperplasia, and adenomatosis. The mechanism of hyperinsulinemia in these patients has been attributed to: (a) an increase in islet cell mass, (b) abnormal islet cell architecture with alternation in paracrine function, (c) imbalance of alpha, beta, and delta cell secretion, and (d) a regulatory defect of insulin secretion. Morphometric, immunocytochemical, and electron microscopy studies have not substantiated an increase in endocrine cell mass in many patients. Nesidioblastosis is believed to be a normal phase of fetal development of the pancreas rather than a pathologic entity. A regulatory defect in insulin storage and release is the best explanation of hyperinsulinism in these patients.
We report experience with 165 neonates and infants including 6 of our own collected during the last decade. Subtotal pancreatectomy (79–90%) was carried out as the initial operative procedure in 76% of patients. Twenty-eight percent required a second operation of near-total to total pancreatectomy. Eleven percent required a third procedure. A near-total pancreatectomy (95–98%) was performed as the initial operation in 24% of the patients. Five percent of these patients required a second operation.
There is a clear advantage of performing an extensive pancreatectomy (95–98%) at the initial operative procedure. In the 40 patients so treated, the incidence of mental retardation was 12.5% and seizures, 2.5%. Ten percent required diazoxide for further management, and 7.5% required insulin on a long-term basis. Mortality was 2.5%. In our report, covering experience from 1934 to 1971, the mortality was 8.3% and mental retardation, 52%.
Résumé
Dans l'hypoglycémie du nouveau-né et de l'enfant en rapport avec un excès de sécrétion d'insuline, on a incrinminé une variété de changements anatomiques: nésidioblastose, dysplasie cellulaire endocrine, hyperplasie des îlots, adénomatose pour n'en nommer que quelques uns. Le mécanisme d'hyper-insulinisme a été attribué à: (a) une augmentation de la masse cellulaire des îlots, (b) une anomalie de l'architecture cellulaire des îlots retentissant sur la fonction endocrine, (c) un déséquilibre des sécrétionsα, β, etδ, et, (d) un défaut de la régulation de la sécrétion de l'insuline. Des études morphométriques, immunocytochimiques, et en microscopie élecronique n'ont pas confirmé d'augmentation cellulaire de la masse endocrine chez bien des patients. On croit maintenant que la nésidioblastose est une phase normale du développement foetal du pancréas plutôt qu'une entité pathologique. Un défaut de la régulation du stockage et du relargage de l'insuline est la meilleure explication de l'hyperinsulinisme chez ce groupe de patients.
Nous ajoutons notre expérience de 6 nouveau-nés et enfants à 159 autres cas colligés pendant cette dernière décennie. L'acte chirurgical initial était une pancréatectomie subtotale (à 79–90%) dans 76% des cas. Vingt-huit pour cent des patients ont nécessité une 2e intervention, allant d'une pancréatectomie quasi totale à une pancréatectomie totale. Une 3e intervention était nécessaire chez 11% des patients. Une pancréatectomie presque totale (95–98%) a été l'intervention initiale chez 24% des patients. Cinq pour cent seulement ont nécessité une 2e intervention.
Une pancréatectomie la plus large possible (95–98%) est préconisée comme intervention initiale. Chez les 40 patients ainsi traités, la fréquence de retard mental était de 12.5%, et celle des convulsions, de 2.5%. Dix pourcent des patients ont nécessité un traitement par la diazoxide, et 7.5%, de l'insuline à long terme. La mortalité était de 2.5%. Dans les cas colligés entre 1934 et 1971, la mortalité était de 8.3% et le retard mental de 52%.
Resumen
Las hipoglicemias neonatal e infantil debida a exceso de insulina ha estado asociada con una variedad de cambios anatomopatológicos: nesidioblastosis, displasia de células endocrinas, hiperplasia de células insulares, y adenomatosis. El mecanismo de la hiperinsulinemia en estos pacientes ha sido atribuido a: (a) un aumento en la masa de células insulares, (b) arquitectura anormal de las células insulares con alteración en su función paracrina, (c) desequilibrio en la secreción de las células alpha, beta, y delta, y (d) un defecto regulatorio en la secreción de insulina. Estudios morfométricos, inmunocitoquímicos, y de microscopía electrónica no han logrado comprobar un aumento en la masa celular endocrina en muchos pacientes. La nesidioblastosis es considerada como una fase normal del desarrollo fetal del páncreas más que como una entidad patológica. Un defecto regulatorio de la secreción de insulina parece ser la mejor explicatión del hiperinsulinismo en estos pacientes.
Este informe se refiere a la experiencia con 165 neonatos e infantes, incluyendo 6 de una serie propia, recolectados en el curso de la última década. Se practicó pancreatectomía subtotal (79–90%) como el procedimiento inicial en 76% de los pacientes; 28% requirieron una segunda Operatión consistente en pancreatectomía casi total o total; 11% requirieron un tercer procedimiento. Una pancreatectomía casi total (95–98%) fue realizada como operación inicial en 24% de los pacientes; 5% de ellos requirieron una segunda operación.
Existe una clara ventaja en realizar una pancreatectomía extensa (95–98%) como procedimiento inicial. En los pacientes así tratados, la incidencia de retardo mental fue 12.5%, y de convulsiones, 2.5%. Diez por ciento requirieron diazóxido en el manejo ulterior, y 7.5% requirieron insulina a largo término. La mortalidad fue 2.5%. En nuestro informe, que cubre la experiencia entre 1934 y 1971, la mortalidad fue 8.3%, y el retardo mental, 52%.
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Thomas, C.G., Cuenca, R.E., Azizkhan, R.G. et al. Changing concepts of islet cell dysplasia in neonatal and infantile hyperinsulinism. World J. Surg. 12, 598–607 (1988). https://doi.org/10.1007/BF01655860
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DOI: https://doi.org/10.1007/BF01655860