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)

Two adult patients with unilateral hypoplastic optic nerves, absent septa pellucida and hypopituitarism are described. Patient 1, aged 20, presented with diabetes insipidus due to partial vasopressin deficiency. Patients 2, aged 29, presented with focal epilepsy. Both had short stature. They showed absent growth hormone (GH) response to insulin-hypoglycaemia or glucagon, but responded to 100 micrograms growth hormone releasing factor (GRF-44) with a rise in circulating GH, suggesting a hypothalamic defect in GH release though a co-existing pituitary defect cannot be excluded. Other hypothalamic-pituitary functions were normal. These two patients probably represent the milder form of the clinical spectrum of septo-optic dysplasia which, with the extensive use of CT brain scans, will be increasingly encountered by physicians attending adult patients.
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PMID:Hypothalamic defects in two adult patients with septo-optic dysplasia. 375 51

Neonatal hypoglycemia caused by islet cell dysplasia (ICD), sometimes called nesidioblastosis, may lead to psychomotor retardation and neurologic dysfunction in up to 50% of patients who are not given early aggressive treatment. In 1979, we adopted a more aggressive protocol for treating this condition that consists of the following steps: immediate insertion of a silastic central venous line for reliable venous access; continuous intravenous infusion of glucose and glucagon to maintain euglycemia; oral diazoxide; and near total pancreatectomy if the first steps fail to overcome the hypoglycemia or the patient cannot be weaned off intravenous therapy. Twelve consecutive patients who underwent pancreatectomy for control of hypoglycemia between 1979 and 1984 were recalled and evaluated for growth delay, neurologic dysfunction, and psychomotor retardation using the Revised Yale Developmental Schedules, the Peabody Picture Vocabulary, and the Draw a Man Test. Follow-up ages ranged from 1.2 to 6.0 years with a median of 3.6 years. No significant growth abnormalities were identified. No patient exhibited focal neurologic dysfunction, although some demonstrated soft neurologic signs, which did not appear to be related to their earlier hypoglycemia. Psychomotor function for the group as a whole was normal, with a mean developmental quotient (DQ) of 99.2. The DQ was average for five patients and above average for four; no patient had a DQ in the frankly subnormal range. Psychomotor development correlated better with the family's socioeconomic and educational status than with the neonatal hypoglycemia. These children are developmentally and neurologically normal despite severe neonatal hypoglycemia. Continued follow-up will be necessary to detect any late sequelae.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Growth and development in patients operated on for islet cell dysplasia. 379 87

We have reviewed ten children who underwent surgical therapy for persistent neonatal hypoglycemia over a 5-year period. All had inappropriately high insulin levels in the face of hypoglycemia, and all failed medical management with intravenous glucose, frequent feeds, diazoxide and glucagon. Two groups of five patients each were analysed retrospectively. Group 1 underwent 95% pancreatectomy, leaving a small amount of pancreatic tissue on the duodenum and common bile duct. The only major complication in this group was in one patient with common duct obstruction requiring choledochoduodenostomy. All these children are developing normally, without diabetes, steatorrhea, or recurrent hypoglycemia. Group 2 underwent 85% pancreatectomy, leaving the uncinate process in situ. Two of these children are well. Two required conversion to 95% resection because of recurrent hypoglycemia; one of these required a subsequent total pancreatectomy, at which time the pancreatic remnant had significantly regenerated. The other Group II patient was normoglycemic but died at age 3 from pneumonia. Pathology in nine cases showed islet cell dysplasia; 5 of these also had microadenomatosis. One case had a histologically normal pancreas. We conclude that 95% pancreatectomy is a safe operation with a lower failure rate than less radical resections, and should be used early in the management of this condition.
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PMID:Surgical management of persistent neonatal hypoglycemia due to islet cell dysplasia. 639 33

Glicentin-containing cells (Glic. cells) in intestinal metaplasia, adenoma and carcinoma of the stomach were examined using immuno-histochemical techniques. Glic. cells first occurred in the gastric mucosa of the transitional area between metaplastic and intact gastric glands. They frequently showed hyperplasia or micronoduli in the budding area of the deeper metaplastic glands, but in completely intestinalized mucosa these endocrine cells decreased remarkably. Gastric adenomas with mild dysplasia had a good number of glicentin-immunoreactive cells which were located in the deeper adenoma glands. Gastrin- and somatostatin-positive cells were also detected in the adenomas. The incidence of glicentin-positive tumor cells was significantly higher in well differentiated adenocarcinoma than in poorly differentiated adenocarcinoma. Among the seven cases of scirrhous argyrophil cell carcinoma, three showed glicentin- and glucagon-immunoreactivity in the same area of the tumor. These findings suggest that the selective increase of Glic. cells in intestinal metaplasia may be closely related to the development of gastric adenoma. Glicentin positive tumor cells in gastric carcinomas can be regarded to be an expression of intestinal or fetal markers.
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PMID:Glicentin-containing cells in intestinal metaplasia, adenoma and carcinoma of the stomach. 643 45

14 cases of hypopituitarism associated with mid-line defects are reported: 7 with septo-optic dysplasia, 5 with agenesis of corpus callosum and septum pellucidum without optical lesion (2 with cleft palate), 1 with familial pituitary aplasia and 1 with mediofrontal cutaneous aplasia. The most striking features in these patients are: precocious signs of pituitary deficiency, mainly hypoglycemia; micropenis and cryptorchidism in males; decrease of growth velocity 2 months to 6 years after birth. Neuroradiological investigations, evaluation of somatotropic and corticotropic secretions with glucagon test, and evaluation of thyrotropin and prolactin secretion with thyroliberin test, offer in the youngest patients the best way to precocious diagnosis and treatment.
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PMID:[Congenital hypopituitarism associated with mid-line defects (author's transl)]. 729 48

Mice carrying a Moloney murine sarcoma virus-(MSV) simian virus 40 large T transgene develop heritable tumors including endocrine pancreatic tumors. We have established several independent transgenic mouse lines expressing this transgene. One of these lines, designated MSV125, is characterized by the development of congenital cataracts and either pancreatic or brain tumors. The development and histopathology of the pancreatic tumors were studied by light microscopy and immunocytochemistry for large T antigen, neuron-specific enolase, insulin, proinsulin, glucagon, somatostatin, pancreatic polypeptide, gastrin, and serotonin. The 23 tumors examined were similar to human endocrine pancreatic tumors with respect to their macroscopic and histological features. We classified 91% of the tumors as insulinomas based on the predominance of insulin immunoreactivity. In newborn and young transgenic animals, nesidioblastosis and islet cell proliferation, consisting mostly of insulin containing beta cells, was obvious and persisted into adulthood. In transgenic animals more than 2 months old, islet hyperplasia and dysplasia predominated from which single tumors developed. Hyperplastic and dysplastic islets were composed mostly of beta cells. Large T antigen was detectable not only in tumor cells, but also in cells of normal and hyperplastic islets and in islet anlagen of newborn transgenic mice, indicating expression of the transgene in the endocrine part of the pancreas. Large T antigen-immunoreactivity was restricted to the beta cells. Insulinomas of the MSV-simian virus 40 T antigen-derived MSV125 transgenic mouse line may represent a valuable model for the study of the development and biology of insulinoma.
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PMID:Endocrine pancreatic tumors in MSV-SV40 large T transgenic mice. 838 73

Tissue kallikreins are thought to be present in the pancreatic islets of Langerhans and to aid in the conversion of proinsulin to insulin. In recent immunohistochemical studies, we observed strong staining of the newly identified human kallikreins 6 and 10 (hK6 and hK10) in the islets of Langerhans. Here, we examine hK6 and hK10 immunoexpression in different types of islet cells of the endocrine pancreas, in order to obtain clues for hK6 and hK10 function in these cells. 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. A streptavidin--biotin--peroxidase and an alkaline phosphatase protocol, as well as a sequential immunoenzymatic double staining method were performed, using specific antibodies against hK6, hK10, insulin, glucagon, somatostatin, pancreatic polypeptide, and serotonin. hK6 and hK10 immunoexpression was observed in the islets of Langerhans, including the pancreatic polypeptide-rich islets, in the normal pancreas. Scattered hK6 and hK10 positive cells were localized in relationship with pancreatic acinar cells. In the exocrine pancreas, a cytoplasmic and/or brush border hK6 and hK10 immunoexpression was observed in the median and small sized pancreatic ducts, while the acinar cells were negative. Foci of nesidioblastosis and endocrine dysplasia expressed both kallikreins. hK6 and hK10 were also strongly and diffusely expressed throughout all insulin-, glucagon- and somatostatin-producing tumours. The double staining method revealed co-localization of each hormone and hK6/hK10 respectively, in the same cellular population, in the normal as well as in the diseased pancreas. Our results support the view that hK6 and hK10 may be involved in insulin and other pancreatic hormone processing and/or secretion, as well as in physiological functions related to the endocrine pancreas.
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PMID:Immunohistochemical localization of human kallikreins 6 and 10 in pancreatic islets. 1276 63

Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.
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PMID:Irreversible intestinal failure. 1507 23

We describe a Japanese brother and sister with Martsolf syndrome. They had short stature, severe mental retardation, cataract, hypogonadism, craniofacial dysmorphism, and bone and joint symptoms including scoliosis, lax finger joints, and talipes valgus. Previously undescribed findings included proximal femoral epiphyseal dysplasia reminiscent of Legg-Calve-Perthes disease in both patients, and Klippel-Feil malformation and osteopathia striata in one patient. Brain MRI showed mild frontal and temporal lobe atrophy, and mild ventricular enlargement. Severe GH deficiency was demonstrated after insulin tolerance and glucagon/propranolol tolerance tests. No responses to serum LH and FSH after a gonadotropin-releasing hormone (GnRH) test suggested secondary hypogonadism, that is, hypogonadotropic hypogonadism, due to hypothalamus-pituitary axis insufficiency in both patients.
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PMID:Martsolf syndrome in Japanese siblings. 1739 1

Glucagon-like peptide-2 (GLP-2) is a nutrient-dependent intestinotropic hormone that promotes intestinal growth, via increased intestinal proliferation and decreased apoptosis, as well as increases in nutrient absorption and barrier function. The long-acting analog h(Gly(2))GLP-2[1-33] is currently being tested for treatment of short bowel syndrome and Crohn's disease. However, the role of GLP-2 in colon carcinogenesis is controversial. To assess the intestinotropic effects of exogenous and endogenous GLP-2, C57BL6/J mice were injected with 1 microg h(Gly(2))GLP-2[1-33]; 30 or 60 ng hGLP-2[3-33], a GLP-2 receptor antagonist; or PBS (4 wk, twice a day, sc). Chronic h(Gly(2))GLP-2[1-33] increased small intestinal weight/body weight (P < 0.001), villus height (P < 0.001), crypt depth (P < 0.001), and crypt cell proliferation, as measured by expression of the proliferative marker Ki67 (P < 0.05-0.01). In contrast, chronic hGLP-2[3-33] decreased small intestinal weight/body weight (P < 0.05) and colon weight/body weight (P < 0.05). To assess the carcinogenic effects of endogenous and exogenous GLP-2, separate mice were injected with azoxymethane (10 mg/kg, 4 wk, every 7 d, ip), followed by 1.5 microg h(Gly(2))GLP-2[1-33], 30 ng hGLP-2[3-33], or PBS (4 wk, twice a day, sc) 2 or 12 wk thereafter. At 10 or 46 wk after azoxymethane treatment, the numbers of aberrant crypt foci increased with h(Gly(2))GLP-2[1-33] (P < 0.001) and decreased with hGLP-2[3-33] (P < 0.01-0.05) treatment. Furthermore, mucin-depleted aberrant foci, consistent with progressive dysplasia, were almost exclusively present in h(Gly(2))GLP-2[1-33]-treated mice (P < 0.01-0.001). Additionally, adenocarcinomas developed in h(Gly(2))GLP-2[1-33]-treated mice but not in those receiving hGLP-2[3-33] or PBS. Taken together, these studies indicate that chronic treatment with GLP-2 enhances colon carcinogenesis, whereas antagonism of the GLP-2 receptor decreases dysplasia, with possible implications for human therapy.
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PMID:Carcinogenic effects of exogenous and endogenous glucagon-like peptide-2 in azoxymethane-treated mice. 1949 74


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