An uncommon cause of recurrent presyncope, dizziness, and tachycardia: a case report of diffuse, adult-onset nesidioblastosis/non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)

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Abstract: Neurovegetative and autonomic symptoms are common presentations of various diseases, ranging from psychosomatic to severe organic disorders. A 23-year-old man presented with a history of recurrent presyncope, dizziness, and tachycardia. Repeated diagnostic work-up in various clinical settings could not identify any definite cause for approximately eight years. However, the incidental detection of postprandial and exercise-induced hypoglycemia was suggestive of an insulin-related disorder. A 72 h plasma glucose fasting test revealed endogenous hyperinsulinism. Upon imaging studies, no tumor mass potentially indicating insulinoma could be detected. 68Ga-DOTA-Exendin-4 PET/CT showed diffuse tracer enrichment throughout the whole pancreas. A subtotal pancreatectomy was performed, and the diagnosis of diffuse, adult-onset nesidioblastosis was established histopathologically. This corresponds to the clinical findings of a functional β-cell disorder, also known as non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). After nine months, the symptoms recurred, making complete pancreatectomy necessary. Postoperative laboratory evaluation exhibited no residual endogenous C-peptide production. This case illustrates the diagnostic challenges in patients presenting with unspecific, neurovegetative and autonomic symptoms with a severe and rare underlying cause

Location: Deutsche Nationalbibliothek Frankfurt am Main

Extent: Online-Ressource

Language: Englisch

Notes: Biomedicines. - 11, 6 (2023) , 1741, ISSN: 2227-9059

Event: Veröffentlichung

(where): Freiburg

(who): Universität

(when): 2023

Creator: Dieterle, Martin P.
Husari, Ayman
Prozmann, Sophie Nicole
Wiethoff, Hendrik
Stenzinger, Albrecht
Röhrich, Manuel
Pfeiffer, Uwe
Kießling, Wolfgang Rüdiger
Engel, Helena
Sourij, Harald
Steinberg, Thorsten
Tomakidi, Pascal
Kopf, Stefan
Szendrödi, Julia

DOI: 10.3390/biomedicines11061741

URN: urn:nbn:de:bsz:25-freidok-2375618

Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.

Last update: 14.08.2025, 11:00 AM CEST

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Associated

Dieterle, Martin P.
Husari, Ayman
Prozmann, Sophie Nicole
Wiethoff, Hendrik
Stenzinger, Albrecht
Röhrich, Manuel
Pfeiffer, Uwe
Kießling, Wolfgang Rüdiger
Engel, Helena
Sourij, Harald
Steinberg, Thorsten
Tomakidi, Pascal
Kopf, Stefan
Szendrödi, Julia
Universität

Time of origin

2023

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An uncommon cause of recurrent presyncope, dizziness, and tachycardia: a case report of diffuse, adult-onset nesidioblastosis/non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)

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Other Objects (12)

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