Nesidioblastosis (Nesidioblastosis of Pancreas)

An inherited autosomal recessive syndrome characterized by the disorganized formation of new islets in the PANCREAS and PERSISTENT HYPERINSULINEMIA HYPOGLYCEMIA OF INFANCY. It is due to focal hyperplasia of pancreatic ISLET CELLS budding off from the ductal structures and forming new islets of Langerhans. Mutations in the islet cells involve the potassium channel gene KCNJ11 or the ATP-binding cassette transporter gene ABCC8, both on CHROMOSOME 11.

Also Known As:

Networked: 217 relevant articles (6 outcomes, 15 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Diabetes Mellitus
2.	Insulinoma
3.	Hypoglycemia (Reactive Hypoglycemia)
4.	Hyperplasia
5.	Neoplasms (Cancer)

Experts

1.	Perren, Aurel: 3 articles (12/2015 - 12/2006)
2.	Service, F John: 3 articles (11/2015 - 07/2005)
3.	Thompson, Geoffrey B: 3 articles (11/2015 - 07/2005)
4.	Schmid, Christoph: 2 articles (12/2015 - 12/2006)
5.	Andrews, James C: 2 articles (11/2015 - 07/2005)
6.	Grant, Clive S: 2 articles (11/2015 - 02/2009)
7.	Vella, Adrian: 2 articles (11/2015 - 02/2009)
8.	Rumilla, Kandelaria M: 2 articles (11/2015 - 02/2009)
9.	Damani, Tanuja: 2 articles (01/2013 - 06/2011)
10.	Lloyd, Ricardo V: 2 articles (02/2009 - 07/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nesidioblastosis:

1.	Diazoxide (Hyperstat)FDA Link 02/01/2006 - "This is the first Japanese adult case of nesidioblastosis treated successfully with diazoxide. " 02/01/2006 - "A rare case of adult-onset nesidioblastosis treated successfully with diazoxide." 02/01/2006 - "This case report suggests that diazoxide may be effective for adult-onset nesidioblastosis in a manner similar to that described for pediatric cases." 08/31/2008 - "Our cases suggest that diazoxide therapy is an effective and safe alternative in the treatment of adult-onset nesidioblastosis." 05/01/1985 - "Complications of diazoxide in the treatment of nesidioblastosis."
2.	Insulin (Novolin)FDA Link 05/01/1996 - "In vitro electrophysiological studies using patch clamp techniques on isolated pancreatic beta cells characterised the ionic basis for insulin secretion in nesidioblastosis. " 05/01/1993 - "During the 3-year period, fasting blood insulin increased during the last half of the study, and this was associated with islets that were 1,300% larger than in control animals (nesidioblastosis). " 09/01/2012 - "In a very small number of patients, the cause is not dietary indiscretions but, instead, factitious insulin administration or nesidioblastosis. " 01/01/2009 - "However, nesidioblastosis was undiscovered until the development of hypoglycaemia after delivery, being previously masked by pregnancy-related changes in insulin sensitivity." 04/01/2008 - "Mcl-1, an anti-apoptotic Bcl-2 family member, essentially overlaps with insulin-producing cells in neonatal nesidioblastosis."
3.	Glucose (Dextrose)FDA LinkGeneric 12/01/1987 - "We hypothesize that this may be the mechanism by which some patients with nesidioblastosis maintain normal serum glucose levels." 12/01/1987 - "This demonstrates that some sensitivity to glucose is present in B cells from the nesidioblastosis tissue, despite the unresponsiveness to glucose alone. " 01/01/2013 - "Medical treatments led to only a mild reduction in the intravenous glucose requirement; an 85-90% pancreatectomy was performed and histological "diffuse nesidioblastosis" was confirmed. " 12/01/1987 - "The inhibitory effects of SRIF and SMS in the culture from the nesidioblastosis tissue, could be reversed by the addition of 11.2 mmol glucose/l, but not in one of the insulinoma cultures. " 06/09/1977 - "We treated a two-month-old infant with servere intractable hypoglycemia and nesidioblastosis with continuous glucose infusions (0.75 g per kilogram per hour) via a central venous catheter. "
4.	Phosphopyruvate Hydratase (Enolase)IBA 07/01/1991 - "Histological classification of nesidioblastosis: efficacy of immunohistochemical study of neuron-specific enolase."
5.	Growth Hormone (Somatotropin)IBA 07/01/1986 - "Growth hormone was effective in reducing the glucose requirement in an infant with nesidioblastosis. " 12/01/1988 - "Five infants had hyperinsulinism secondary to nesidioblastosis or to a beta-cell adenoma of the pancreas, one infant had neonatal sepsis due to staphylococcal infection and one infant congenital growth hormone (HGH) and adrenocorticotropic hormone (ACTH) deficiency. "
6.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 05/01/1999 - "The somatostatin-producing cells arise from these ducts, very much in the fashion of ductulo-endocrine complexes in nesidioblastosis." 12/01/1987 - "Somatostatin secretion in cultured human islet cells from patients with nesidioblastosis: a compensatory mechanism?" 01/22/1987 - "Long-term treatment of an infant with nesidioblastosis using a somatostatin analogue." 08/01/1985 - "Infants suffering from nesidioblastosis require 1.0-4.5 micrograms/kg/h somatostatin and a concomitant carbohydrate supply of 0.3-0.48 g/kg/h in order to maintain normoglycemia. " 12/18/1981 - "[Somatostatin in a case of nesidioblastosis (author's transl)]."
7.	Glucagon (Glukagon)FDA Link 05/01/2003 - "Nesidioblastosis with glucagon-reactive islet cell hyperplasia: a case report." 04/01/2014 - "Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review." 04/01/2014 - "[A case of alpha-cell nesidioblastosis and hyperplasia with multiple glucagon-producing endocrine cell tumor of the pancreas]." 01/01/1982 - "High-titer (1:30,000) antiglucagon antibodies were detected in a 14-month-old patient with nesidioblastosis who had been treated by intramuscular long-acting glucagon since age 5 months. " 11/01/1990 - "Nesidioblastosis was a prominent feature in all cases but one, with a positivity for insulin in 11 cases, for glucagon in 13, for somatostatin in 6 and for PP in 17. "
8.	CalciumIBA 11/01/1997 - "Selective intra-arterial calcium injection in the investigation of adult nesidioblastosis: a case report." 11/01/2006 - "All of the patients showed a combination of postprandial neuroglycopaenia, negative prolonged fasts (except one patient) and negative localization studies for insulinoma, but positive calcium stimulation tests and nesidioblastosis in the gradient-guided resected pancreas. " 11/01/2006 - "Noninsulinoma pancreatogenous hypoglycaemia syndrome (NIPHS), characterized by postprandial neuroglycopaenia, negative prolonged fasts and negative perioperative localization studies for insulinoma, but positive selective arterial calcium stimulation tests and nesidioblastosis in the gradient-guided resected pancreas, is a rare hypoglycaemic disorder of undetermined aetiology. " 12/01/2000 - "We recently reported a novel hypoglycemic disorder in 5 patients (patients 1 to 5) with postprandial neuroglycopenia, negative 72-hour fasts, negative perioperative imaging studies, but positive calcium stimulation tests and islet hypertrophy and nesidioblastosis in the gradient-guided resected pancreata. " 11/01/2015 - "It is not known whether the biochemical results of selective arterial calcium stimulation (SACST) with hepatic venous sampling can differentiate insulinoma from diffuse nesidioblastosis. "
9.	Pancreatic Polypeptide (Pancreatic Polypeptide (PP))IBA 06/01/1987 - "We describe a 63-yr-old man with disseminated medullary carcinoma of the thyroid and pancreatic nesidioblastosis and microadenosis with pancreatic polypeptide (PP) hypersecretion. " 03/01/1994 - "We herein describe two unique patients with adult nesidioblastosis with gastrin and pancreatic polypeptide secretion. " 06/01/1987 - "Medullary carcinoma of the thyroid, pancreatic nesidioblastosis and microadenosis, and pancreatic polypeptide hypersecretion: a new association and clinical and hormonal responses to long-acting somatostatin analog SMS 201-995." 06/01/2002 - "Ten cases of normal pancreatic tissue, two cases of nesidioblastosis, five insulin-producing tumours and one case of multiple endocrine neoplasia 1 syndrome, containing an insulin-, a somatostatin- and several glucagon-producing tumours, as well as tiny foci of endocrine dysplasia with different predominance of the secreted hormones (mainly glucagon and pancreatic polypeptide) were included in the study. " 01/01/1986 - "Therefore, in this study morphologic, immunohistologic (to detect insulin, glucagon, somatostatin, and pancreatic polypeptide), and morphometric features were evaluated in 1) normal pancreases (from fetal to adult; n = 49); 2) pancreases from patients with nesidioblastosis (n = 5); and 3) tumor-associated pancreases (TAP) from patients with insulin-producing islet cell tumors (n = 8). "
10.	Octreotide (Sandostatin)FDA LinkGeneric 05/01/2004 - "We report the case of a 36-yr-old woman with nesidioblastosis treated throughout pregnancy with high doses of octreotide. " 05/01/1994 - "In nesidioblastosis and other hyperinsulinemic conditions, timely treatment with octreotide can protect cerebral function and may reduce mortality. " 11/01/2001 - "Octreotide, administered by continuous intravenous infusion or intermittent bolus dosing, was used in the treatment of gastrointestinal bleeding in four patients, pancreatitis in three patients, chylous leaks in two patients, and hypoglycemia related to nesidioblastosis in one patient. " 05/01/2004 - "Normal pregnancy in a woman with nesidioblastosis treated with somatostatin analog octreotide." 05/01/1988 - "Long-term use of somatostatin analogue SMS 201-995 in the treatment of hypoglycaemia due to nesidioblastosis."

Therapies and Procedures

1.	Pancreatectomy 04/01/2011 - "The histopathology was consistent with nesidioblastosis and gradient guided pancreatectomy relieved the hypoglycemic episodes." 01/01/1986 - "A subtotal pancreatectomy was performed during SMS treatment, and the diagnosis of nesidioblastosis was confirmed by immunocytologic and electron-microscopic studies. " 01/01/2015 - "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. " 12/01/2014 - "Subtotal and 90% pancreatectomy were performed for nesidioblastosis at the ages 2 months and 8 years, respectively. " 10/01/2013 - "She was diagnosed nesidioblastosis and total pancreatectomy was performed leading to symptom resolution. "
2.	Gastric Bypass (Roux-en-Y Gastric Bypass) 01/01/2008 - "The cause of adult nesidioblastosis is not known, but the recently observed association with gastric bypass surgery in obese patients suggests that a reactive process possibly unmasks or induces a defect in the beta cell, resulting in its hyperfunction." 07/21/2005 - "Gastric bypass and nesidioblastosis--too much of a good thing for islets?" 04/01/2007 - "Laparoscopic spleen-preserving distal pancreatectomy as treatment for nesidioblastosis after gastric bypass surgery." 02/01/2015 - "A rare complication of gastric bypass (weight loss) surgery: nesidioblastosis." 11/01/2014 - "An association between post-Roux-en-Y gastric bypass (RYGB) hypoglycemia and nesidioblastosis was reported in 2005 and may cause serious neuroglycopenic symptoms. "
3.	Splenectomy 09/01/1984 - "Conventional 85% pancreatectomy with splenectomy performed for management of hypoglycemia of neonatal nesidioblastosis has been followed by a dismal prognosis characterized by post-splenectomy sepsis, recurrent hypoglycemia, permanent brain damage, and a high mortality. " 08/01/1989 - "Two patients on steroids had unsuspected PI identified as an incidental operative finding during pancreatectomy for nesidioblastosis (1) and splenectomy for hemolytic anemia (1), respectively. "
4.	Catheters 06/09/1977 - "We treated a two-month-old infant with servere intractable hypoglycemia and nesidioblastosis with continuous glucose infusions (0.75 g per kilogram per hour) via a central venous catheter. "
5.	Bariatric Surgery 01/01/2014 - "Since pancreatic islets in nesidioblastosis overexpress insulin-like growth factor 1 (IGF-1) receptor α and administration of recombinant IGF-1 provokes hypoglycemia, our main objective was to investigate the occurrence of post-load hypoglycemia one year after bariatric surgery and its relation to pre- and post-operative IGF-1 serum concentrations. "