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Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis

The patient is a 28-year-old Japanese man diagnosed with severe congenital hyperinsulinemic-hypoglycemia six months after birth. Clinical records revealed no imaging evidence of pancreatic tumor at the time of diagnosis. Subsequently, he had developmental disorders and epilepsy caused by recurrent h...

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Autores principales: Inoue, Chisa, Nishihama, Kota, Hayasaki, Aoi, Okano, Yuko, Hayashi, Akinobu, Eguchi, Kazuhito, Uemura, Mei, Suzuki, Toshinari, Yasuma, Taro, Inoue, Takeshi, Yorifuji, Tohru, Mizuno, Shugo, Gabazza, Esteban C., Yano, Yutaka
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8578890/
https://www.ncbi.nlm.nih.gov/pubmed/34777243
http://dx.doi.org/10.3389/fendo.2021.731071
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author Inoue, Chisa
Nishihama, Kota
Hayasaki, Aoi
Okano, Yuko
Hayashi, Akinobu
Eguchi, Kazuhito
Uemura, Mei
Suzuki, Toshinari
Yasuma, Taro
Inoue, Takeshi
Yorifuji, Tohru
Mizuno, Shugo
Gabazza, Esteban C.
Yano, Yutaka
author_facet Inoue, Chisa
Nishihama, Kota
Hayasaki, Aoi
Okano, Yuko
Hayashi, Akinobu
Eguchi, Kazuhito
Uemura, Mei
Suzuki, Toshinari
Yasuma, Taro
Inoue, Takeshi
Yorifuji, Tohru
Mizuno, Shugo
Gabazza, Esteban C.
Yano, Yutaka
author_sort Inoue, Chisa
collection PubMed
description The patient is a 28-year-old Japanese man diagnosed with severe congenital hyperinsulinemic-hypoglycemia six months after birth. Clinical records revealed no imaging evidence of pancreatic tumor at the time of diagnosis. Subsequently, he had developmental disorders and epilepsy caused by recurrent hypoglycemic attacks. The patient’s hypoglycemia improved with oral diazoxide. However, he developed necrotizing acute pancreatitis at 28 years of age, thought to be due to diazoxide. Discontinuation of diazoxide caused persistent hypoglycemia, requiring continuous glucose supplementation by tube feeding and total parenteral nutrition. A selective arterial secretagogue injection test revealed diffuse pancreatic hypersecretion of insulin. He underwent subtotal distal (72%) pancreatectomy and splenectomy. There was no intraoperative visible pancreatic tumor. His hypoglycemia improved after the surgical procedure. The histopathological study revealed a high density of islets of Langerhans in the pancreatic body and tail. There were large islets of Langerhans and multiple neuroendocrine cell nests in the whole pancreas. Nests of neuroendocrine cells were also detected in lymph nodes. The pathological diagnosis was grade 1 neuroendocrine tumor (microinsulinomas) with lymph node metastases. This patient is a difficult-to-diagnose case of hyperinsulinemic hypoglycemia surgically treated after developing acute pancreatitis. We believe this is a unique case of microinsulinomas with lymph metastases diagnosed and treated as congenital hyperinsulinemic hypoglycemia for almost 28 years.
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spelling pubmed-85788902021-11-11 Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis Inoue, Chisa Nishihama, Kota Hayasaki, Aoi Okano, Yuko Hayashi, Akinobu Eguchi, Kazuhito Uemura, Mei Suzuki, Toshinari Yasuma, Taro Inoue, Takeshi Yorifuji, Tohru Mizuno, Shugo Gabazza, Esteban C. Yano, Yutaka Front Endocrinol (Lausanne) Endocrinology The patient is a 28-year-old Japanese man diagnosed with severe congenital hyperinsulinemic-hypoglycemia six months after birth. Clinical records revealed no imaging evidence of pancreatic tumor at the time of diagnosis. Subsequently, he had developmental disorders and epilepsy caused by recurrent hypoglycemic attacks. The patient’s hypoglycemia improved with oral diazoxide. However, he developed necrotizing acute pancreatitis at 28 years of age, thought to be due to diazoxide. Discontinuation of diazoxide caused persistent hypoglycemia, requiring continuous glucose supplementation by tube feeding and total parenteral nutrition. A selective arterial secretagogue injection test revealed diffuse pancreatic hypersecretion of insulin. He underwent subtotal distal (72%) pancreatectomy and splenectomy. There was no intraoperative visible pancreatic tumor. His hypoglycemia improved after the surgical procedure. The histopathological study revealed a high density of islets of Langerhans in the pancreatic body and tail. There were large islets of Langerhans and multiple neuroendocrine cell nests in the whole pancreas. Nests of neuroendocrine cells were also detected in lymph nodes. The pathological diagnosis was grade 1 neuroendocrine tumor (microinsulinomas) with lymph node metastases. This patient is a difficult-to-diagnose case of hyperinsulinemic hypoglycemia surgically treated after developing acute pancreatitis. We believe this is a unique case of microinsulinomas with lymph metastases diagnosed and treated as congenital hyperinsulinemic hypoglycemia for almost 28 years. Frontiers Media S.A. 2021-10-27 /pmc/articles/PMC8578890/ /pubmed/34777243 http://dx.doi.org/10.3389/fendo.2021.731071 Text en Copyright © 2021 Inoue, Nishihama, Hayasaki, Okano, Hayashi, Eguchi, Uemura, Suzuki, Yasuma, Inoue, Yorifuji, Mizuno, Gabazza and Yano https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Inoue, Chisa
Nishihama, Kota
Hayasaki, Aoi
Okano, Yuko
Hayashi, Akinobu
Eguchi, Kazuhito
Uemura, Mei
Suzuki, Toshinari
Yasuma, Taro
Inoue, Takeshi
Yorifuji, Tohru
Mizuno, Shugo
Gabazza, Esteban C.
Yano, Yutaka
Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title_full Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title_fullStr Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title_full_unstemmed Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title_short Case Report: A Difficult-to-Diagnose Case of Hyperinsulinemic Hypoglycemia Surgically Treated After Developing Acute Pancreatitis
title_sort case report: a difficult-to-diagnose case of hyperinsulinemic hypoglycemia surgically treated after developing acute pancreatitis
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8578890/
https://www.ncbi.nlm.nih.gov/pubmed/34777243
http://dx.doi.org/10.3389/fendo.2021.731071
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