Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes.
In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin-dependent diabetes mellitus, and subsequently developed PHH caused by diffuse nesidioblastosis. Mutations of the MEN1 and Mody (2/3) genes were ruled out. Preoperative diagnostic procedures, the histopathological criteria and the surgical treatment options of adult nesidioblastosis are discussed. So far only one similar case of adult nesidioblastosis subsequent to diabetes mellitus II has been reported in the literature. In case of conversion of diabetes into hyperinsulinemic hypoglycemia syndrome, nesidioblastosis in addition to insulinoma should be considered.
Raffel
A
A
Department of General and Visceral Surgery, Heinrich-Heine University, Düsseldorf, Germany.
Anlauf
M
M
Hosch
S-B
SB
Krausch
M
M
Henopp
T
T
Bauersfeld
J
J
Klofat
R
R
Bach
D
D
Eisenberger
C-F
CF
Kloppel
G
G
Knoefel
W-T
WT
D000328:Adult; D004252:DNA Mutational Analysis; D003922:Diabetes Mellitus, Type 1 / Q000150:complications*; D051538:Hepatocyte Nuclear Factor 1-alpha / Q000235:genetics; D006801:Humans; D006946:Hyperinsulinism / Q000209:etiology*; D007003:Hypoglycemia / Q000209:etiology*; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics; D046768:Nesidioblastosis / Q000150:complications* / Q000235:genetics; D010179:Pancreas / Q000473:pathology
C496487:HNF1A protein, human; D051538:Hepatocyte Nuclear Factor 1-alpha