Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia
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,
Julia Szendroedi
Affiliations
Martin Philipp Dieterle
Division of Oral Biotechnology, Center for Dental Medicine, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Ayman Husari
Department of Orthodontics, Center for Dental Medicine, Medical Center–University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Sophie Nicole Prozmann
Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Hendrik Wiethoff
Institute of Pathology, University Hospital Heidelberg, 69120 Heidelberg, Germany
Albrecht Stenzinger
Institute of Pathology, University Hospital Heidelberg, 69120 Heidelberg, Germany
Manuel Röhrich
Department of Nuclear Medicine, University Hospital Heidelberg, 69120 Heidelberg, Germany
Uwe Pfeiffer
Pfalzklinikum for Psychiatry and Neurology AdÖR, Weinstr. 100, 76889 Klingenmünster, Germany
Wolfgang Rüdiger Kießling
Independent Researcher of Neurology and Psychiatry, 72250 Freudenstadt, Germany
Helena Engel
Cancer Immune Regulation Group, German Cancer Research Center, Im Neuenheimer Feld 280, 69120 Heidelberg, Germany
Harald Sourij
Division of Endocrinology and Diabetology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria
Thorsten Steinberg
Division of Oral Biotechnology, Center for Dental Medicine, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Pascal Tomakidi
Division of Oral Biotechnology, Center for Dental Medicine, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Stefan Kopf
Department of Internal Medicine I and Clinical Chemistry, University of Heidelberg, 69120 Heidelberg, Germany
Julia Szendroedi
Department of Internal Medicine I and Clinical Chemistry, University of Heidelberg, 69120 Heidelberg, Germany
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.
