Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Morphology of pancreas (either esocrine, either endocrine) was studied in 29 cases of surgically treated chronic pancreatitis (27 cases of chronic calcifying pancreatitis and 2 cases of chronic obstructive pancreatitis). Parenchymal sclerosis in chronic calcifying pancreatitis (CCP) which represents the goal of our study was graded as mild (10 cases), moderate (10) and severe (7). Immunoperoxidase staining (PAP method) for insulin, glucagon, somatostatin, pancreatic polipeptide (PP), vasoactive intestinal polipeptide (VIP) and gastrin, was used to investigate endocrine pancreas. Acinar sclerosis and endocrine damage were closely related. Progression of sclerosis into islet appears to follow vascular pedicles producing a fragmentation into small cell groups as final result. In all cases of moderate or severe sclerosis, A/B cell ratio was increased due to the reduction of insulin positive cells. "Adenoma-like complexes", i.e., apparent concentration of islets, resulting from the loss of the acinar component, were observed in 7 cases with moderate or severe sclerosis. 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. No positivity for gastrin was observed, while VIP was detected in a few ganglia. An increased amount of PP cells in islet and budding from the ducts was noticed and their presence outside the pancreatic head was demonstrable in 4 out of the 7 distal pancreatectomy specimens. Our data confirm the secondary involvement of the endocrine pancreas in the sclerotic acinar process.
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PMID:[Anatomopathologic pictures of chronic pancreatitis]. 209 39

The clinicopathologic features of an adult with insulinoma and pancreatic islet cell hyperplasia, who presented with hyperinsulinemic hypoglycemia are reported, together with in vitro studies on the patient's pancreatic islets. Islet cell hyperplasia with ductal proliferation and budding and beta cell degranulation was demonstrated by immunochemical means. The in vitro studies of cultured hyperplastic islet cells support the clinicopathologic features. Thus, in comparison with control islets maintained in culture for up to 14 days, hyperplastic islets could be cultured for up to 60 days, during which time cell overgrowth required subculture on three occasions. Furthermore, in contrast to control islets the release of both insulin and somatostatin from cultured hyperplastic islets was refractory to glucose, glucagon, and tolbutamide; theophylline was the only secretagogue to stimulate insulin and somatostatin release from hyperplastic islets in vitro. Indirect immunofluorescence revealed the presence of islet cell surface autoantibodies in the plasma of this patient reactive with both normal human islets and a rat insulinoma line (RIN-m5F). These studies demonstrate the proliferative capacity and relatively undifferentiated functional state of hyperplastic islets in vitro. They provide further evidence that islet cell division is capable of being stimulated in adult life. The pathogenic significance of islet cell surface autoantibodies in hyperplastic islet cell disease and insulinoma warrants further investigation.
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PMID:Nesidioblastosis and multifocal pancreatic islet cell hyperplasia in an adult. Clinicopathologic features and in vitro pancreatic studies. 286 76

One hundred pancreatic tumors ranging in size from 0.3 to 7 cm were studied in 28 patients (17 male and 11 female patients; mean age 35 years) with multiple endocrine neoplasia, type I. An immunohistochemical study was performed on deparaffinized sections using the following antibodies: neuron-specific enolase, chromogranin A or synaptophysin, insulin, glucagon, somatostatin, pancreatic polypeptide (PP), vasoactive intestinal peptide (VIP), gastrin, adrenocorticotropic hormone, alpha-subunit of human chorionic gonadotropin, gonadotropin-releasing factor, serotonin, and calcitonin. Among the 100 tumors (all multiple), seven were unclassified, 10 were plurihormonal, and 83 produced a predominant hormonal secretion (with 50-90% of the same cell type), including 37 "A-cell tumors" (glucagon), 27 "B-cell tumors" (insulin), 11 PP-cell tumors, one G-cell tumor (gastrin) and one vasoactive intestinal peptide (VIP)-cell tumor. These multiple tumors had a different predominant hormonal secretion in the same patient in 23 of the 28 cases. There was a preferential association of A-cell tumor and B-cell tumor. Hyperplasia of the islets of Langerhans was not detected in adjacent pancreas. Nesidioblastosis was observed in 30% of cases.
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PMID:Immunohistochemical study of 100 pancreatic tumors in 28 patients with multiple endocrine neoplasia, type I. 889 42

Nesidioblastosis associated with progressive weight loss and hyperglycemia was diagnosed in two mid-adult, wild-caught, male squirrel monkeys (Saimiri sciureus). Hyperglycemia, glucosuria, and abnormal glucose tolerance test results were found when the monkeys were presented for clinical evaluation for chronic weight loss, episodic dehydration, hypothermia, and lethargy. Immunohistochemical studies of the pancreatic tissue demonstrated that the proliferating endocrine cells stained predominantly glucagon-positive in the most severely affected monkey.
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PMID:Nesidioblastosis associated with hyperglycemia in two squirrel monkeys (Saimiri sciureus). 902 1

A 95% pancreatectomy became the treatment of choice for persistent hyperinsulinemic hypoglycemia of the neonate (PHHN, Nesidioblastosis) at the author's institution, when lesser resections failed to prevent hypoglycemia in 25% to 50% of cases. With few outcome data available in the literature, the authors reviewed their 25-year experience to assess the efficacy and the long-term consequences of this procedure. Since 1971, 27 infants underwent a 95% pancreatectomy for the treatment of PHHN. None had responded to medical treatment (glucose infusion, glucagon, octreotide, diazoxide), and two had 85% pancreatectomy that failed. The procedure consisted of resecting the pancreas including the uncinate process, leaving only the gland lying between the common bile duct (CBD) and the duodenum and a small rim of pancreas along the duodenal sweep. Hyperinsulinemia and hypoglycemia recurred in nine children (33%), all within 2 to 5 days. Seven of them were subsequently cured with near-total pancreatic resection. Partial pancreatic regrowth was evident at reoperation. In two cases hypoglycemia was controlled with diazoxide and frequent feedings because reoperation was refused. The gross anatomic findings and the histopathology were not predictive of treatment failure. Perioperative complications occurred in four of 27 children (15%) after 95% pancreatectomy and in four of seven children (57%) after near-total pancreatectomy. Clinical follow-up ranged from 0.5 to 18 years (mean, 8 years; median, 8 years). To date, diabetes has developed in 15 children (56%), nine of 20 (45%) after 95% pancreatectomy (mean age, 9.7 years) and six of seven (86%) after a near-total pancreatectomy (mean age, 1.7 years). After 95% pancreatectomy, the incidence of diabetes increased with age, developing in nine of the 13 (69%) children followed up for more than 4 years. The failure of 95% pancreatectomy to prevent hypoglycemia in one third of children with PHHN and the ultimate development of diabetes in a minimum of two-thirds, indicates that an alternative treatment strategy is needed for this disease.
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PMID:Is 95% pancreatectomy the procedure of choice for treatment of persistent hyperinsulinemic hypoglycemia of the neonate? 904 50

The histopathology of Fibrocalculous Pancreatic Diabetes (FCPD) has been extensively studied, but there are no reports on alteration in patterns of hormone secreting cells using immunohistochemistry in islets of FCPD patients. In this study, we report on the histopathology and immunohistochemistry of islets of FCPD patients and its possible correlation with the clinical picture. Pancreatic biopsies were carried out in six patients with FCPD at the time of surgery for abdominal pain. Routine histopathology and immunohistochemistry studies were carried out with six primary antibodies namely insulin, glucagon, pancreatic polypeptide (PP), somatostatin, vasoactive intestinal peptide and gastrin. Histopathology of the pancreas showed a spectrum of changes ranging from moderate to severe atrophy, fibrosis of the parenchyma and degeneration of the ducts. Nesidioblastosis was present in three patients. Immunohistochemical studies showed a decrease in the number of islets but some patients showed evidence of hyperplasia. There was an overall decrease in the percent of insulin cells and the positivity in the islets correlated with plasma C-peptide levels and the duration of diabetes. There was no consistent relationship with glucagon with some patients showing increased and other decreased positivity. There was a marked decrease in PP and somatostatin positivity, the significance of which is not clear. The reduction, but partial preservation of insulin positivity is consistent with the ketosis resistance shown by patients with Fibrocalculous Pancreatic Diabetes.
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PMID:Histopathology and immunohistochemistry of pancreatic islets in fibrocalculous pancreatic diabetes. 1113 79

OBJECTIVE: To report a case of Persistent Hyperinsulinemic Hypoglycemia in twins which is a situation not yet reported in the literature. METHODS: Report of seizures in identical twins, from consanguineous parents, with persistent hypoglycemia as cause of the seizures. Laboratory tests, performed for etiological investigation of the hypoglycemia, included thyroid hormones (T4/TSH), insulin, cortisol, growth hormone, stimulation test with glucagon (to evaluate the insulin/glucose relation), and histopathological study of the pancreas. RESULTS: Laboratorial investigation revealed a persistent hypoglycemia with hyperinsulinism which were confirmed with the stimulation test with glucagon. The histopathological exam showed a persistence of first generation pancreatic islet, confirming the diagnosis of Persistent Hyperinsulinemic Hypoglycemia in Infancy (the new denomination of Nesidioblastosis). CONCLUSION: Although rare, this condition must be early suspected early in the evaluation of hypoglycemia of the young infant, even out of the neonatal period, specially if the parents are consanguineous. The adequate therapy must be quickly initiated in order to prevent neurological damage.
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PMID:[Persistent hyperinsulinemic hypoglycemia of infancy: case report] 1464 92

The forkhead gene family, named after the founding gene member in Drosophila, is characterized by a unique DNA-binding domain. This so-called forkhead box encodes a winged-helix DNA-binding motif, the name of which describes the structure of the domain when bound to DNA. The three Fox (forkhead box) group A genes, Foxa1, Foxa2 and Foxa3, are expressed in embryonic endoderm, the germ layer that gives rise to the digestive system, and contribute to the specification of the pancreas and the regulation of glucose homoeostasis. Deletion of the Foxa2 gene in pancreatic beta-cells in mice results in a phenotype resembling PHHI (persistent hyperinsulinaemic hypoglycaemia of infancy). Molecular analyses have demonstrated that Foxa2 is an important regulator of the genes encoding Sur1, Kir6.2 and Schad (short chain L-3-hydroxyacyl-CoA dehydrogenase), mutation of which causes PHHI in humans. Foxa1 was shown to be an essential activator of glucagon gene expression in vivo. An additional winged-helix protein, Foxo1, contributes to pancreatic beta-cell function by regulating the Pdx1 gene, which is required for pancreatic development in cooperation with Foxa2.
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PMID:Winged-helix transcription factors and pancreatic development. 1563 23

We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas. The patient's clinical presentation, diagnosis, treatment, pancreas pathology, and follow-up are reviewed. A 60-year-old patient was incidentally found to harbor a pancreatic mass with markedly elevated glucagon levels but without glucagonoma syndrome. She was initially diagnosed with glucagonoma, and the tumor was resected. Pathological examination demonstrated that the tumor was a nonfunctioning islet cell tumor and revealed nesidioblastosis and hyperplasia of alpha cells and microglucagonoma in the apparently normal surgical margin. The patient still had high postoperative glucagons levels which were suppressed by somatostatin analog treatment. No pancreatic tumors recurred 36 months after surgery. This is the third case of alpha-cell nesidioblastosis reported in the English literature. Nesidioblastosis and hyperplasia of alpha cells should be considered in the differential diagnosis of hyperglucagonemia. Somatostatin analog may be used to suppress glucagon secretion in alpha-cell hyperplasia.
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PMID:Nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas: review of the literature. 1843 91

Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells. It also means maldistribution of islet cells within the ductules of exocrine pancreas. Generally, nesidioblastosis occurs in beta-cell and causes neonatal hyperinsulinemic hypoglycemia or adult noninsulinoma pancreatogenous hypoglycemia syndrome. Alpha-cell nesidioblastosis and hyperplasia is an extremely rare disorder. It often accompanies glucagon-producing marco- and mircoadenoma without typical glucagonoma syndrome. A 35-year-old female was referred to our hospital with recurrent acute pancreatitis. On radiologic studies, 1.5 cm sized mass was noted in pancreas tail. Cytological evaluation with EUS-fine-needle aspiration suggested serous cystadenoma. She received distal pancreatectomy. The histologic examination revealed a 1.7 cm sized neuroendocrine tumor positive for immunohistochemical staining with glucagon antibody. Multiple glucagon-producing micro endocrine cell tumors were scattered next to the main tumor. Additionally, diffuse hyperplasia of pancreatic islets and ectopic proliferation of islet cells in centroacinar area, findings compatible to nesidioblastosis, were seen. These hyperplasia and almost all nesidioblastic cells were positive for glucagon immunochemistry. Even though serum glucagon level still remained higher than the reference value, she has been followed-up without any evidence of recurrence or hormone related symptoms. Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review.
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PMID:[A case of alpha-cell nesidioblastosis and hyperplasia with multiple glucagon-producing endocrine cell tumor of the pancreas]. 2475 52


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