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Adult nesidioblastosis is characterized by endogenous hyperinsulinemia typically causing post-prandial hypoglycemia, and most commonly occurs post-Rouxen-Y gastric bypass.
Case Report NESIDIOBLASTOSIS IN AN ADULT WITH SHORT GUT SYNDROME AND TYPE DIABETES Mimi Wong, BSc, MBBS(Hons)1,2; Luke Conway, MBBS, FRACP1; Caroline Cooper, MBBS(Hons), FRCPA2,3; Ashim Sinha, MD, FRACP, FACE1,4; Nirjhar Nandi, FRACP1 ABSTRACT Objective: Adult nesidioblastosis is characterized by endogenous hyperinsulinemia typically causing post-prandial hypoglycemia, and most commonly occurs post-Rouxen-Y gastric bypass Methods: We report a unique case of nesidioblastosis occurring in a 67-year-old female Results: A 5-year history of symptomatic hypoglycemia occurred in a patient with short bowel syndrome and type diabetes mellitus (T2DM) managed previously with a glucagon-like peptide (GLP-1) agonist, which achieved significant weight loss Continuous glucose monitoring captured 42 hypoglycemia episodes in a 2-week period, and following an oral glucose tolerance test there was the suggestion of a hyperinsulinemia state She was managed with an open distal pancreatectomy, and subsequently required medical therapy to maintain euglycemia Conclusion: We present the first case of nesidioblastosis occurring in a patient with short bowel syndrome, pre-existing T2DM managed with a GLP-1 agonist which achieved significant weight loss, all of which we speculate could have predisposed to hypoglycemia and development of nesidioblastosis (AACE Clinical Case Rep 2019;5:e375-e379) Submitted for publication May 26, 2019 Accepted for publication August 2, 2019 From 1Department of Diabetes and Endocrinology, Cairns Hospital, Queensland, Australia, 2School of Medicine, University of Queensland, Australia, 3Pathology Queensland, Princess Alexandra Hospital, Queensland, Australia, and 4Department of Medicine, James Cook University, Queensland, Australia Address correspondence to Dr Mimi Wong, Department of Medicine, 165 Esplanade, Cairns City, QLD, 4870 Australia E-mail: mimi.wong@uqconnect.edu.au DOI: 10.4158/ACCR-2019-0243 To purchase reprints of this article, please visit: www.aace.com/reprints Copyright © 2019 AACE Copyright © 2019 AACE Abbreviations: BSL = blood sugar level; GLP-1 = glucagon-like peptide 1; MMT = mixed meal test; RYGB = Rouxen-Y gastric bypass; T2DM = type diabetes mellitus INTRODUCTION Adult nesidioblastosis is a rare hyperinsulinemic state, classically associated with post-prandial hypoglycemia (1) Typically, hypoglycemia is provoked with a mixedmeal test (MMT) and localizing studies are invariably negative (2) It is not possible to diagnose nesidioblastosis clinically, with imaging, or biochemically Histopathologic features of adult nesidioblastosis are more variable than the more common newborn setting, and include exclusion of an insulinoma, the presence of conspicuous islet cells with enlarged, hyperchromatic nuclei, and islet hypertrophy and hyperplasia Formation of ductuloinsular complexes is not a distinctive feature in adults but is well reported In some cases the histopathologic changes are minimal and distinction from normal pancreas is difficult (3,4) The pathophysiology of adult nesidioblastosis remains to be elucidated Genetic factors, trophic factors, and receptor expression on islet cells have been suggested to be involved Roux-en-Y gastric bypass (RYGB) has been linked to nesidioblastosis, and it is thought that elevated glucagon-like peptide (GLP-1) and gastric inhibitory peptide may unmask a b-cell defect (1,3) CASE REPORT A 67-year-old female was referred to our institution in 2018 In 2009, she had a motor vehicle accident which led to total colectomy, resection of 75% of her small bowel, and formation of an end ileostomy This was complicated AACE CLINICAL CASE REPORTS Vol No November/December 2019 e375 e376 Nesidioblastosis and Short Bowel, AACE Clinical Case Rep 2019;5(No 6) by high-output stoma and malnutrition She was referred to a dietician, and dietary advice included having small and frequent meals with high fiber In addition, she had trailed multiple pharmacologic therapies which had limited effects, including loperamide and buscopan Enteral feeding had never been used In 2013, she was diagnosed with type diabetes mellitus (T2DM) which coincided with weight gain, from a baseline of 45 to 50 kg (body mass index [BMI] 19), to 75 kg (BMI 29.7) Hemoglobin A1c (HbA1c) and oral glucose tolerance test (OGTT) at diagnosis were not available, though blood sugar levels (BSLs) through glucometer peaked to 23 mmol/L Initially she was managed with metformin for a month, and subsequently with a GLP-1 agonist (exenatide, 10 mcg twice a day) in 2013 for 18 months, which led to a dramatic weight reduction to 45 kg Lifestyle optimization and malabsorption also likely Copyright © 2019 AACE contributed to this significant weight loss Following this, exenatide was ceased Her diabetes has since been managed with lifestyle measures with good glycemic control (HbA1c 5.3%, 34 mmol/mol), and her weight has been between 55 to 59 kg in the past few years Since 2013 she reported having episodes of symptomatic hypoglycemia, with BSLs less than mmol/L, which consisted of sweating, tremor, light-headedness and lethargy Initial BSL monitoring revealed fasting and postprandial hypoglycemia to as low as 2.3 mmol/L These episodes resolved within to 10 minutes of correction Initially hypoglycemia occurred once every months; however, in the last 12 months she had increased episodes, and in 2018, an episode resulted in loss of consciousness which was complicated by a tibial fracture At the time her BSL was 3.6 mmol/L, and occurred 30 minutes following a meal A B Fig Flash glucose monitor readings Episodes of symptomatic hypoglycemia occurred with BSL less than mmol/L, and each hypoglycemic episode was treated A, Readings prior to subtotal pancreatectomy B, Readings following subtotal pancreatectomy and commencement of 50 mg octreotide times a day BSL = blood sugar level; M = Main meals of breakfast, lunch, and dinner Copyright © 2019 AACE Nesidioblastosis and Short Bowel, AACE Clinical Case Rep 2019;5(No 6) e377 Fasting sample Table Preoperative Evaluation of Hypoglycemia Serum glucose (mmol/L) 8.1 Insulin (