Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Dieterle, MP; Husari, A; Prozmann, SN; Wiethoff, H; Stenzinger, A; Röhrich, M; Pfeiffer, U; Kießling, WR; Engel, H; Sourij, H; Steinberg, T; Tomakidi, P; Kopf, S; Szendroedi, J.
An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS).
Biomedicines. 2023; 11(6): 1741
Doi: 10.3390/biomedicines11061741
(- Case Report)
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- Co-Autor*innen der Med Uni Graz
- Sourij Harald
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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.
- Find related publications in this database (Keywords)
- nesidioblastosis
- hyperinsulinism
- hypoglycemia
- hyperinsulinemic hypoglycemia
- congenital hyperinsulinism
- positron emission tomography
- insulinoma
- pancreatectomy
- hyperplasia