Adult diffuse nesidioblastosis: genetically or environmentally induced?

Klöppel, Günter; Anlauf, Martin; Raffel, Andreas; Perren, Aurel; Knoefel, Wolfram Trudo

doi:10.1016/j.humpath.2007.09.010

Cited by 96 publications

(113 citation statements)

References 29 publications

(47 reference statements)

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“…Glucagon-like peptide1 has been proposed as a possible causative mechanism of nesidioblastosis because of its effects on islet cell neogenesis and b-cell apoptosis leading to islet cell hyperplasia in rodents. 44 Weight loss after gastric bypass in severely obese patients might unmask a b-cell defect that promotes hyperinsulism and islet cell hyperplasia. 3,44 We observed a higher prevalence of women patients with postgastric bypass nesidioblastosis, which most likely reflects the women predominance among individuals who have undergone gastric bypass for morbid obesity.…”

Section: Discussionmentioning

confidence: 99%

“…44 Weight loss after gastric bypass in severely obese patients might unmask a b-cell defect that promotes hyperinsulism and islet cell hyperplasia. 3,44 We observed a higher prevalence of women patients with postgastric bypass nesidioblastosis, which most likely reflects the women predominance among individuals who have undergone gastric bypass for morbid obesity. 45,46 In summary, IGF2, IGF1Ra, and TGFbR3 are overexpressed pancreatic tissues from patients with nesidioblastosis.…”

Section: Discussionmentioning

confidence: 99%

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Hyperinsulinemic hypoglycemia with nesidioblastosis: histologic features and growth factor expression

et al. 2009

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Hypoglycemia secondary to nesidioblastosis is rare in adults, and the pathogenesis of this condition is unknown. To determine factors leading to nesidioblastosis in adults, we analyzed 36 cases of nesidioblastosis including 27 cases of postgastric bypass nesidioblastosis and 9 cases of idiopathic nesidioblastosis in adults by immunohistochemistry using antibodies to insulin-like growth factor1, insulin-like growth factor2 (IGF2), insulin-like growth factor one receptor-a epidermal growth factor receptor, transforming growth factor-b1 and 2, and transforming growth factor-b receptor type 3. Fifty-two surgically excised pancreatic specimens from patients with benign exocrine tumors and no evidence of hypoglycemia were used as controls. There was increased IGF2, insulin-like growth factor receptor1 receptor-a and transforming growth factor-b receptor 3 expression in islets from nesidioblastosis patients compared to controls. Peliosis-type vascular ectasia was more common in nesidioblastosis patients compared to controls. These findings suggest that increased production of growth factors and growth factor receptors may contribute to the development of nesidioblastosis in adults.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Hyperinsulinemic hypoglycemia with nesidioblastosis: histologic features and growth factor expression

et al. 2009

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“…The cause of adult nesidioblastosis is unknown, but it may be genetically induced, as in congenital hyperinsulinism in neonates, or be a response to metabolic and hormonal changes, such as those following gastric bypass surgery and weight loss (3). A paradoxical elevation of glucagon-like peptide 1 reported following gastric bypass surgery (8) may influence islet cell neogenesis and apoptosis, thus producing islet hyperplasia and PHH (9).…”

Section: Discussionmentioning

confidence: 99%

“…Nesidioblastosis is the major cause of PHH in infants and children, but in adults it is usually a consequence of a solitary insulinoma. Nesidioblastosis has been reported infrequently in adults (1)(2)(3). It should be noted that in pediatric patients nesidioblastosis may be classified histologically as either diffuse or focal, but only diffuse lesions have been reported in adult patients with histologically-confirmed nesidioblastosis.…”

Section: Introductionmentioning

confidence: 99%

A rare case of focal nesidioblastosis causing adult-onset hypoglycemia

Qin

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Nesidioblastosis is a major cause of persistent hyperinsulinemic hypoglycemia of infancy and is caused by hypertrophy of the pancreatic endocrine islands. The disease can be categorized histologically into diffuse and focal forms. The condition rarely occurs in adults and only one adult case of suspected, but not histologically confirmed, focal nesidioblastosis has been reported. The present study describes the case of a 62-year-old man suffering from symptomatic hypoglycemia for 3 years and exhibiting a nodule in the pancreatic tail. Pathological evaluation following surgical enucleation of the pancreatic body and tail revealed focal nesidioblastosis. The hypoglycemic symptoms of the patient disappeared postoperatively. To the best of our knowledge, this is the first histologically-confirmed case of focal adult nesidioblastosis, suggesting that the possibility of nesidioblastosis should be taken into account in adult patients with persistent hypoglycemia. IntroductionHypoglycemia in nondiabetic patients is not a common clinical problem and can be a diagnostic and therapeutic challenge (1). Persistent hyperinsulinemic hypoglycemia (PHH) is a functional disorder caused by aberrant insulin release by pancreatic β cells (2). Nesidioblastosis is the major cause of PHH in infants and children, but in adults it is usually a consequence of a solitary insulinoma. Nesidioblastosis has been reported infrequently in adults (1-3). It should be noted that in pediatric patients nesidioblastosis may be classified histologically as either diffuse or focal, but only diffuse lesions have been reported in adult patients with histologically-confirmed nesidioblastosis. A single case of suspected focal nesidioblastosis in an adult was reported by McElroy et al in 2010 (4), but was not confirmed histologically. Due to the lack of evidence, most physicians do not take a possibility of focal nesidioblastotosis into account when confronted with an adult patient with PHH..The present study focused on the case of a 62-year-old man with a 3-year history of intermittent episodes of symptomatic hypoglycemia. A 72-h fasting test, elevated levels of insulin and C-peptide, concomitant with decreased blood glucose levels and imaging, led to the discovery of a nodule in the pancreatic tail. The pancreatic corpus and tail were enucleated laparoscopically and the presence of focal nesidioblastosis was confirmed histologically. We propose that focal nesidioblastosis should be taken into consideration when confronted with PHH, even in middle-aged patients. Case reportCase presentation. A 62-year-old man with a body mass index of 26.99 presented with a 3-year history of intermittent episodes of dizziness, weakness and sweating, which were apparently associated with work load and subsided upon food intake. Previous clinical evaluations had not included blood glucose measurements. The patient had no history of diabetes, pancreatic diseases, von Hippel-Lindau or multiple endocrine neoplasia syndromes and denied using insulin or medications assoc...

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“…In 15 adults with nesidioblastosis, blinded interobserver analysis showed beta-cell hypertrophy with enlarged and hyperchromatic nuclei; the interobserver analysis revealed 100% specificity and 87.7% sensitivity for these criteria (Anlauf et al 2005b). The degree and extent of these features vary much more from patient to patient than in newborns (Kloppel et al 2008). The interpretation of histological findings is not unequivocal, as shown by the divergent conclusions regarding 6 patients with reactive insulin-mediated hypoglycaemia after bypass surgery Meier et al 2006).…”

Section: Nesidioblastosismentioning

confidence: 99%