A rare case of non-insulinoma pancreatic hypoglycaemia syndrome (niphs) in an adult due to localised islet cell hyperplasia–successfully managed by enucleation

Persistent hyperinsulinemic hypoglycaemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, localised islet cell hyperplasia is a rare disorder characterised by localised proliferation of islet cells. The authors present the case of a previously health...

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Bibliographic Details
Published in:BMJ case reports 2011-10, Vol.2011 (oct11 1), p.bcr0720114554-bcr0720114554
Main Authors: Nayak, Hemanta K, Sothwal, Arpit, Raizaida, Nishant, Daga, Mradul kumar, Agarwal, Anil kumar, Durga, Garima
Format: Article
Language:English
Subjects:
Adults
Asia
Diabetes
Diagnosis, Differential
Female
Glucose
Humans
Hyperinsulinism - etiology
Hyperplasia - complications
Hyperplasia - pathology
Hyperplasia - surgery
Hypoglycemia - etiology
Indian Subcontinent
Insulin
Insulinoma - diagnosis
Islets of Langerhans - diagnostic imaging
Islets of Langerhans - pathology
Islets of Langerhans - surgery
Laboratories
Middle Aged
Pancreas
Pancreatic Neoplasms - diagnosis
Patients
Population
Rare Disease
Surgery
Syndrome
Ultrasonic imaging
Ultrasonography
Veins & arteries
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Summary:Persistent hyperinsulinemic hypoglycaemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, localised islet cell hyperplasia is a rare disorder characterised by localised proliferation of islet cells. The authors present the case of a previously healthy non-obese middle aged female with new-onset severe hypoglycaemia. Laboratory findings confirmed a case of hyperinsulinemic hypoglycaemia. Endoscopic ultrasonography, intra-arterial calcium stimulation test and intraoperative ultrasonography showed a lesion in the uncinate process that was enucleated. Rest of the pancreas was normal. Histological examination and immunostaining of the resected tissue revealed pancreatic tissue with maintained acinar pattern consistent with diagnosis of localised islet cell hyperplasia. The patient did not have further episodes of hypoglycaemia following the procedure. Localised islet cell hyperplasia with such a very high insulin level is exceedingly rare in adult populations and not reported in literature. This diagnosis cannot be easily made through routine diagnostic laboratory or radiological procedures and likely requires a histological diagnosis. Management of this rare entity is by enucleation.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr.07.2011.4554