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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)

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...

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Main Authors: Dieterle, Martin P. (Author) , Husari, Ayman (Author) , Prozmann, Sophie Nicole (Author) , Wiethoff, Hendrik (Author) , Stenzinger, Albrecht (Author) , Röhrich, Manuel (Author) , Pfeiffer, Uwe (Author) , Kießling, Wolfgang Rüdiger (Author) , Engel, Helena (Author) , Sourij, Harald (Author) , Steinberg, Thorsten (Author) , Tomakidi, Pascal (Author) , Kopf, Stefan (Author) , Szendrödi, Julia (Author)
Format: Article (Journal)
Language:English
Published: 2023
In: Biomedicines
Year: 2023, Volume: 11, Issue: 6, Pages: 1-14
ISSN:2227-9059
DOI:10.3390/biomedicines11061741
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.3390/biomedicines11061741
Verlag, kostenfrei, Volltext: https://www.mdpi.com/2227-9059/11/6/1741
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Author Notes:Martin Philipp Dieterle, Ayman Husari, Sophie Nicole Prozmann, Hendrik Wiethoff, Albrecht Stenzinger, Manuel Röhrich, Uwe Pfeiffer, Wolfgang Rüdiger Kießling, Helena Engel, Harald Sourij, Thorsten Steinberg, Pascal Tomakidi, Stefan Kopf and Julia Szendroedi
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Summary: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.
Item Description:Veröffentlicht: 17. Juni 2023
Gesehen am 14.08.2023
Physical Description:Online Resource
ISSN:2227-9059
DOI:10.3390/biomedicines11061741

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