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Diffuse, adult-onset nesidioblastosis/non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS): review of the literature of a rare cause of hyperinsulinemic hypoglycemia

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 (N...

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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: 16 June 2023
In: Biomedicines
Year: 2023, Volume: 11, Issue: 6, Pages: 1-42
ISSN:2227-9059
DOI:10.3390/biomedicines11061732
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.3390/biomedicines11061732
Verlag, lizenzpflichtig, Volltext: https://www.mdpi.com/2227-9059/11/6/1732
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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: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.
Item Description:Gesehen am 21.09.2023
Physical Description:Online Resource
ISSN:2227-9059
DOI:10.3390/biomedicines11061732

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