Nesidioblastosis associated with hyperinsulinemic hypoglycemia in adults: review of the literature

Jabri, Ahmad; Bayard, Cecilia

doi:10.1016/j.ejim.2004.06.012

Cited by 45 publications

(46 citation statements)

References 51 publications

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“…Mutations of SUR1 and Kir6.2 genes encoding the subunits of the pancreatic ATP-sensitive potassium channel responsible for glucose-induced insulin secretion, have been reported in some hereditary form of PHHI (2, 3). However, adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, currently recognized as NIPHS (6,7), is a very rare entity, representing only 0.5-5% of cases of organic hyperinsulinemia (8); since the first reported series of adult-onset nesidioblastosis by Harness et al in 1981 (9), only 71 cases have been reported to date.…”

Section: Discussionmentioning

confidence: 99%

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome: A Rare Case of Adult-onset Nesidioblastosis

et al. 2005

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The most common cause of hyperinsulinemic hypoglycemia in adults is insulinoma. Nesidioblastosis is a rare, but well-recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemic syndrome (NIPHS), has been reported. Here, we describe an extremely rare case of NIPHS in an elderly man. A 78-year-old man was admitted to our hospital due to hypoglycemic coma. During the previous 3 months, he noticed excessive sweating at midafternoon. His low fasting plasma glucose level (27 mg/dl) and high immunoreactive insulin level (11.1 U/ml) were consistent with the possible presence of insulinoma. Localizing studies including computed tomography of the abdomen and celiac arteriography were negative, but selective arterial calcium infusion (SACI) test suggested the presence of insulinoma in the body and tail of the pancreas. Surgical exploration by palpation and intraoperative ultrasonography failed to detect any mass in the pancreas, and 60% distal pancreatectomy was performed. Postoperatively, his hypoglycemic episodes completely disappeared. Histological examination of the resected pancreas revealed diffuse islet cell hyperplasia consistent with a pathological diagnosis of nesidioblastosis. Thus, our case is a very rare case of NIPHS, or adult-onset nesidioblastosis, in which SACI test was proven to be a useful diagnostic tool for localization of the pancreatic lesion. Key words: hyperinsulinemic hypoglycemia, nesidioblastosis, noninsulinoma pancreatogenous hypoglycemia syndrome (NPHS), selective arterial calcium injection (SACI)

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Section: Discussionmentioning

confidence: 99%

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome: A Rare Case of Adult-onset Nesidioblastosis

et al. 2005

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“…Therefore a clinical diagnosis of insulinoma is strongly suspected. However, it is also possible that adult onset nesidioblastosis associated with PHH, which is very rare and represents 0.5-5.0% of cases of organic hyperinsulinemia [21], can be a causal pathology of this patient. Surgical resection of the pancreas and careful histological analyses are necessary to arrive at a final diagnosis for this case.…”

Section: Discussionmentioning

confidence: 99%

A Case of Hyperinsulinemia of Undetermined Origin, Successfully Treated with Long-acting Octreotide

et al. 2005

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Abstract. Major causes of fasting hypoglycemia in adults are insulinoma, factitious hypoglycemia and nesidioblastosis. The primary treatment for insulinoma is surgical removal of the tumor, but there are cases with hyperinsulinemia that cannot undergo surgery. Somatostatin analogue is one of the treatments used in such cases of insulinoma or persistent hyperinsulinemic hypoglycemia. We report here a patient who had undetermined hyperinsulinemia and was successfully treated with a long-acting somatostatin analogue, which had recently become available. The patient, a 72-year-old female, who had previously been diagnosed as insulinoma and undergone partial pancreatectomy, was admitted complaining of the recurrence of hypoglycemic attacks after an interval of ten years. On admission, hypoglycemia (42 mg/dl), hyperinsulinemia (IRI: 79.3 mU/m) and low HbA1c (3.6%) were present. In 75g-OGTT at 30 min after load, IRI reached 6623 mU/ml, while plasma glucose level was 88 mg/dl. The anti-insulin antibody was not present. Since attempts at tumor localization by imaging techniques failed and the patient refused further examinations or surgical treatment, we recommended her to take a medication with a somatostatin analogue. Insulin suppression test using 50 mg of octreotide improved plasma glucose and IRI levels, suggesting the usefulness of the treatment, and a monthly administration of 20 mg of long-acting octreotide has successfully controlled her symptoms of hypoglycemia for 10 months. Our case demonstrated the utility of the long-acting somatostatin analogue for long-term treatment of undetermined hyperinsulinemia. A preliminary loading test using short-acting octreotide may be useful to determine appropriate medication, especially in cases who cannot receive surgical treatment.

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“…Genetic defects involving the subunits of the ATPsensitive potassium channel in pancreatic beta cells are known to be responsible for development of persistent hyperinsulinaemic hypoglycaemia of infancy [2], and some of them could also be associated with development of the disease in adults. Despite this, pathogenesis of nesidioblastosis in adults is still unclear, even if it may be associated with bariatric surgery and its metabolic changes, especially in patients who underwent a gastric bypass with Roux-en-Y reconstruction [19].…”

Section: Opis Przypadkumentioning

confidence: 99%

“…Nesidioblastosis is the primary cause of persistent hyperinsulinaemic hypoglycaemia in infants, but it accounts for only 0.5% to 7% of all cases of hyperinsulinaemic hypoglycaemia in adults [2]. Moreover, while infant nesidioblastosis is mainly caused by mutations of genes encoding subunits of pancreatic ATP-sensitive potassium channel [3,4], the cause of adult nesidioblastosis is still unknown.…”

Section: Introductionmentioning

confidence: 99%

Przetrwała hipoglikemia hiperinsulinemiczna współistniejąca z nieaktywnym wyspiakiem trzustki

Valli

Blandamura

Pastorelli³

et al. 2015

Endokrynologia Polska

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The most common cause of hyperinsulinaemic hypoglycaemia in adult is insulinoma. Although nesidioblastosis is a rare but well-recognised disorder of persistent hypoglycaemia in infants, it is extremely rare in adults. We present a case of a 59-year-old woman with small neuroendocrine tumour of the tail of the pancreas, diagnosed by CT scans and MRI, and hypoglycaemic syndrome. Laparoscopic distal pancreatectomy was performed, and pathologic examination showed a well-differentiated, non-functioning endocrine tumour of the pancreas and diffuse nesidioblastosis in the remnant gland. In the early postoperative period, recurrent hypoglycaemia occurred in spite of oral diazoxide therapy. Plasma proinsulin levels were extremely high. 18F-DOPA positron emission tomography showed a pathologic uptake of tracer in the head and the uncinate process of the pancreas. Subtotal pancreatectomy was suggested but the patient refused operation: she is taking diazoxide 100 mg three times daily. Coexistence of nesidioblastosis with a neuroendocrine tumour makes preoperative diagnosis and management of severe hypoglycaemia more difficult. Nesidioblastosis should be considered in differential diagnosis of hypoglycaemic syndrome, but histological examination is necessary for a definitive tissue diagnosis. StreszczenieNajczęstszą przyczyną hipoglikemii hiperinsulinemicznej u osób dorosłych jest insulinoma. Chociaż przetrwała hipoglikemia hiperinsulinemiczna to rzadkie, lecz łatwo rozpoznawalne zaburzenie przetrwałej hipoglikemii u noworodków, bardzo rzadko występuje u osób dorosłych. Autorzy badania przedstawiają przypadek 59-letniej kobiety z niewielkim guzem neuroendokrynnym ogona trzustki, zdiagnozowanym dzięki tomografii komputerowej oraz rezonansowi magnetycznemu, oraz zespołem hipoglikemicznym. Wykonano laparoskopową dystalną pankreatektomię, a badanie patologiczne wykazało wysoko zróżnicowanego, nieaktywnego, endokrynnego wyspiaka trzustki oraz rozlaną przetrwałą hipoglikemię hiperinsulinemiczną w pozostałej części gruczołu. We wczesnym okresie pooperacyjnym wystąpiła nawracająca hipoglikemia, mimo stosowania doustnego leczenia diazoksydem. Stężenie proinsuliny w osoczu był bardzo wysoki. Pozytonowa tomografia emisyjna z 18F-DOPA wykazała patologiczną absorpcję znacznika w głowie i wyrostku haczykowatym trzustki. Sugerowano subtotalną pankreatektomię, lecz pacjentka nie zgodziła się na operację: przyjmowała dawkę 100 mg diazoksydu trzy razy dziennie. Współistnienie przetrwałej hipoglikemii hiperinsulinemicznej z nowotworem neuroendokrynnym utrudnia diagnostykę przedoperacyjną i leczenie ciężkiej hipoglikemii. Przetrwała hipoglikemia hiperinsulinemiczna powinna być brana pod uwagę w diagnozie różnicującej zespół hipoglikemiczny, lecz badanie histologiczne jest niezbędne do definitywnej diagnozy tkanek. (Endokrynol Pol 2015; 66 (4): 356-360)

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Nesidioblastosis associated with hyperinsulinemic hypoglycemia in adults: review of the literature

Cited by 45 publications

References 51 publications

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome: A Rare Case of Adult-onset Nesidioblastosis

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome: A Rare Case of Adult-onset Nesidioblastosis

A Case of Hyperinsulinemia of Undetermined Origin, Successfully Treated with Long-acting Octreotide

Przetrwała hipoglikemia hiperinsulinemiczna współistniejąca z nieaktywnym wyspiakiem trzustki

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