Literature DB >> 24075522

Nesidioblastosis. A case of hyperplasia of the islets of Langerhans in the adult.

Esther Pilar García-Santos1, María del Carmen Manzanares-Campillo, David Padilla-Valverde, Pedro Villarejo-Campos, Aurora Gil-Rendo, Virginia Muñoz-Atienza, Susana Sánchez-García, Ana María Puig-Rullán, José Luis Rodríguez-Peralto, Jesús Martín-Fernández.   

Abstract

INTRODUCTION: Nesidioblastosis is a rare disease caused by hyperplasia of pancreatic islets, developing a state of hypoglycemia due to an increase in the insulin production. It is the leading cause of hyperinsulinic hypoglycemia in childhood, whereas in adults it only represents the 0.5-5% of cases. The pathogenesis is still unknown. We have studied several genetic mutations associated with dependent potassium channel of ATP present in the beta cells of the pancreas, as well as in patients underwent bariatric surgery because of the metabolic changes involved. REPORT: Woman (38 years old) attends consultation of General Surgery derived from Endocrinology before symptoms of persistent hypoglycemia. Factitious hypoglycemia and syndromes of neuroendocrine origin were ruled out. Imaging tests failed to identify space-occupying lesions. The medical treatment failed, persisting hypoglycemia symptoms. Before the given analytical and radiological findings obtained, and the persistence of symptoms affecting the quality of life of the patient, we opted for surgical treatment performing a pancreatectomy of the 80% of the gland. The final pathologic diagnosis was nesidioblastosis. DISCUSSION: Nesidioblastosis is a rare pathology, but it must be present in the differential diagnosis of hypoglycemia symptoms with endogenous hyperinsulinism in adults, once the intake of sulfonylureas and possible pancreatic neoformations have been ruled out.
Copyright © 2013 IAP and EPC. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Hyperinsulinism; Hypoglycemia; Insulinoma; Nesidioblastosis

Mesh:

Year:  2013        PMID: 24075522     DOI: 10.1016/j.pan.2013.06.009

Source DB:  PubMed          Journal:  Pancreatology        ISSN: 1424-3903            Impact factor:   3.996


  6 in total

1.  Total pancreatectomy for the management of refractory post-gastric bypass hypoglycemia.

Authors:  Gaya Spolverato; Feriyl Bhaijee; Robert Anders; Kelsey Salley; Joan Parambi; Todd Brown; Timothy M Pawlik
Journal:  Dig Dis Sci       Date:  2014-10-26       Impact factor: 3.199

Review 2.  Nesidioblastosis Associated with Pancreatic Heterotopia as a Differential Diagnosis of Hypoglycemia: A Literature Review and Case Report.

Authors:  Aline A Lopes; Ana C Miranda; Marcelo S Maior; Roberto V de Mello; Francisco A Bandeira
Journal:  Am J Case Rep       Date:  2020-07-06

3.  Anesthetic management of a case of nesidioblastosis.

Authors:  Ravindra K Pandey; Udismita Baruah; Ripul Oberoi; Neha Pangasa; Nandinie Hamshi
Journal:  Saudi J Anaesth       Date:  2018 Oct-Dec

4.  Adult-Onset Focal Nesidioblastosis With Nodular Formation Mimicking Insulinoma.

Authors:  Shunsuke Doi; Takatsugu Yamada; Yoshinori Kito; Shinsaku Obara; Yusuke Fujii; Takao Nishimura; Tatsushi Kato; Hiroyuki Nakayama; Masahiro Tsutsumi; Ryuji Okamura
Journal:  J Endocr Soc       Date:  2021-12-11

5.  Second MAFA Variant Causing a Phosphorylation Defect in the Transactivation Domain and Familial Insulinomatosis.

Authors:  Christian Fottner; Stefanie Sollfrank; Mursal Ghiasi; Anke Adenaeuer; Thomas Musholt; Arno Schad; Matthias Miederer; Simin Schadmand-Fischer; Matthias M Weber; Karl J Lackner; Heidi Rossmann
Journal:  Cancers (Basel)       Date:  2022-04-01       Impact factor: 6.639

6.  Insulin autoimmune syndrome induced by exogenous insulin injection: a four-case series.

Authors:  Yimin Shen; Xiaoxiao Song; Yuezhong Ren
Journal:  BMC Endocr Disord       Date:  2019-12-28       Impact factor: 2.763

  6 in total

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