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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.
Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia.
Biomedicines. 2023; 11(6): 1732 Doi: 10.3390/biomedicines11061732 [OPEN ACCESS]
Web of Science PubMed PUBMED Central FullText FullText_MUG

 

Co-Autor*innen der Med Uni Graz
Sourij Harald
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Abstract:
Differential diagnosis of hypoglycemia in the non-diabetic adult patient is complex and comprises various diseases, including endogenous hyperinsulinism caused by functional β-cell disorders. The latter is also designated as nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). Clinically, this rare disease presents with unspecific adrenergic and neuroglycopenic symptoms and is, therefore, often overlooked. A combination of careful clinical assessment, oral glucose tolerance testing, 72 h fasting, sectional and functional imaging, and invasive insulin measurements can lead to the correct diagnosis. Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective. Therefore, nearly all patients currently undergo surgical resection of parts or the entire pancreas. Consequently, apart from faster diagnosis, more elaborate and less invasive treatment options are needed to relieve the patients from the dangerous and devastating symptoms. Based on a case of a 23-year-old man presenting with this disease in our department, we performed an extensive review of the medical literature dealing with this condition and herein presented a comprehensive discussion of this interesting disease, including all aspects from epidemiology to therapy.

Find related publications in this database (Keywords)
nesidioblastosis
hyperinsulinism
hypoglycemia
hyperinsulinemic hypoglycemia
congenital hyperinsulinism
positron-emission tomography
insulinoma
pancreatectomy
hyperplasia
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