Adult-Onset Nesidioblastosis Causing Hypoglycemia

Witteles, Ronald; Straus, Francis H.; Sugg, Sonia L.; Koka, Mahalakshmana Rao; Costa, Eduardo; Kaplan, Edwin L.

doi:10.1001/archsurg.136.6.656

Cited by 93 publications

(44 citation statements)

References 31 publications

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“…Other cases of nesidioblastosis in adults appear to happen spontaneously [32], but in fact, some are diagnosed years after surgery for an insulinoma at another institution [33]. Such cases are diagnosed based on morphology alone.…”

Section: Comparison With Adult Nesidioblastosismentioning

confidence: 99%

Neonatal hyperinsulinism: clinicopathologic correlation

Delonlay¹,

Simon²,

Galmiche-Rolland³

et al. 2007

Human Pathology

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Section: Comparison With Adult Nesidioblastosismentioning

confidence: 99%

Neonatal hyperinsulinism: clinicopathologic correlation

Delonlay¹,

Simon²,

Galmiche-Rolland³

et al. 2007

Human Pathology

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“…We considered the possibility that overnutrition had masked hypoglycemia from congenital hyperinsulinism, leading to obesity. However, adult-onset cases of hyperinsulinism have been reported (5), so childhood-onset disease in this patient is probable. Examination of the literature yielded very little information on management of focal or diffuse hyperinsulinism in this patient's age group.…”

Section: Discussionmentioning

confidence: 77%

Hyperinsulinism Presenting In Childhood and Treatment by Conservative Pancreatectomy

Patterson

Mao²,

Yeh³

et al. 2012

Endocrine Practice

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Objective To describe the uncommon presentation of hyperinsulinism in an 8-year-old boy. Methods We describe the patient's clinical findings, results from biochemical and imaging studies, surgical approach, and outcome. The discussion encompasses a review of literature that provided the basis for the diagnostic and surgical approach applied to this patient's case. Results An obese 8.5-year-old boy initially presented with hypoglycemic seizures after initiation of dietary changes to treat obesity. Biochemical analysis indicated hyperinsulinism. Endoscopic ultrasonography showed no pancreatic lesions suggestive of insulinoma. Genetic studies identified no known mutations in the ABCC8, KCNJ11, GCK, or GLUD1 genes. Selective arterial calcium stimulation and hepatic venous sampling did not document a focal source for hyperinsulinism in the pancreas, and positron emission tomography with 18-fluoro-L-3,4-dihydroxyphenylalanine showed diffusely increased uptake in the pancreas. The patient ultimately required partial pancreatectomy because of continued hypoglycemia while taking diazoxide and octreotide. Intraoperative glucose monitoring directed the extent of surgical resection. A 45% pancreatectomy was performed, which resolved the hypoglycemia but led to impaired glucose tolerance after surgery. Conclusion The unusual presentation of hyperinsulinism in childhood required a personalized approach to diagnosis and surgical management using intraoperative glucose monitoring that resulted in a conservative pancreatectomy.

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“…Though uncommon but PHHI is clinically important cause of hypoglycemia in neonates, and must be considered in a patient with a presumptive pre-operative diagnosis of insulinoma. [14]…”

Section: Resultsmentioning

confidence: 99%

Anesthetists approach in a neonate with nesidioblastoma undergoing pancreatectomy

Patel

Shikare

Chavan

et al. 2013

J Anaesthesiol Clin Pharmacol

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Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is rare, but an important cause of hypoglycemia in infants, associated with a number of structural abnormalities of the endocrine pancreas is collectively termed as “Islet cell dysmaturation syndrome.” We present the anesthetic management in a clinically diagnosed case of PHHI in a 22 days old full term child, undergoing Subtotal Pancreatectomy. We have discussed the challenges faced in the intra-operative period in managing this neonate for pancreatic resection surgery with focus on intra-operative management of blood glucose levels.

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Adult-Onset Nesidioblastosis Causing Hypoglycemia

Cited by 93 publications

References 31 publications

Neonatal hyperinsulinism: clinicopathologic correlation

Neonatal hyperinsulinism: clinicopathologic correlation

Hyperinsulinism Presenting In Childhood and Treatment by Conservative Pancreatectomy

Anesthetists approach in a neonate with nesidioblastoma undergoing pancreatectomy

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