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Topic: Congenital hyperinsulinism

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Hyperinsulinemic hypoglycemia

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Hypoglycemic

	Sur1 Hyperinsulinism
		Congenital Hyperinsulinism is the most frequent cause of severe, persistent hypoglycaemia in newborn babies and children.
		Hyperinsulinism causes a particularly damaging form of hypoglycaemia because it denies the brain of all the fuels on which it is critically dependent.
		The identification of genetic defects responsible for hyperinsulinism and the improved understanding of the mechanism of abnormal insulin secretion have permitted the development of insulin secretion studies aimed at identifying the specific type of hyperinsulinism a child may have.
	www.sur1.org /hyperinsulism.htm (3486 words)

	Medscape MEDLINE search: Hyperinsulinism
		Congenital hyperinsulinism and mosaic abnormalities of the ploidy.
		Hyperinsulinism is a heterogeneous disorder characterised by severe hypoglycaemia due to an inappropriate oversecretion of insulin.
		Congenital hyperinsulinism is a rare pancreatic endocrine cell disorder that has been categorized histologically into diffuse and focal forms.
	search.medscape.com /uslclient/searchMedline.do?queryText=Hyperinsulinism (1026 words)

	Hyperinsulinism - Wikipedia, the free encyclopedia
		Hyperinsulinism can be associated with several types of medical problems, which can be roughtly divided into two broad categories: those tending toward high blood glucose levels (hyperglycemia) and those tending toward low glucose levels (hypoglycemia).
		The principal treatments of hyperinsulinism due to insulin resistance are measures that improve insulin sensitivity, such as weight loss, physical exercise, and drugs such as thiazolidinediones or metformin.
		The treatment of this form of hyperinsulinism depends on the cause and the severity of the hyperinsulinism, and may include surgical removal of the source of insulin, or a drug such as diazoxide or octreotide that reduces insulin secretion.
	en.wikipedia.org /wiki/Hyperinsulinism (549 words)

	APSA :: Resources for Parents : Hyperinsulinism (via CobWeb/3.1 planetlab2.isi.jhu.edu) (Site not responding. Last check: 2007-11-03)
		Congenital hyperinsulinism, also called nesidioblastosis, is a problem in babies in which insulin control is abnormal, causing low blood sugar.
		Congenital hyperinsulinism is a rare problem of sugar control in the body that has an incidence of 1 in 50,000 live births.
		This article analyzes a large experience with congenital hyperinsulinism from the standpoint of genetics, medical and surgical treatment, and long-term risk of diabetes.
	www.eapsa.org.cob-web.org:8888 /parents/hyperinsulinism.cfm (413 words)

	Enzyme Defect Leads To Hyperinsulinism
		Congenital hyperinsulinism is a group of genetic disorders that cause hypoglycemia in infants and children.
		The majority of cases of congenital hyperinsulinism appear to be due to defects in insulin secretion by pancreatic cells.
		This enzyme is stimulated by the amino acid leucine, meaning that protein meals that contain leucine lead to activation of glutamate dehydrogenase, which in turn triggers the release of insulin from pancreatic cells.
	www.sciencenewsden.com /2006/enzymedefectleadstohyperinsulinism.shtml (440 words)

	Medical-surgical teamwork cures severe insulin condition in newborns
		As determined by both preoperative testing and an operative biopsy, all the children had the focal form of congenital HI, in which the abnormal tissue is confined to a limited area of the pancreas, the organ that produces insulin.
		While some forms of HI in infants are transient and relatively common, congenital HI is a rare genetic disease, with several subtypes, based on specific mutations.
		The most severe forms of congenital HI affect an estimated 100 to 200 infants annually in the U.S. Of that number, approximately two-thirds may have the focal type, as found by the Children's Hospital team.
	www.eurekalert.org /pub_releases/2003-05/chop-mtc052103.php (738 words)

	Hyperinsulinism, genetics
		Severe congenital hyperinsulinism caused by a mutation in the Kir6.2 subunit of the adenosine triphosphate-sensitive potassium channel impairing trafficking and function.
		Congenital hyperinsulinism (HI), the most important cause of hypoglycaemia in early infancy, is a heterogeneous disease with two types of histological lesions, focal and diffuse, with major consequences in terms of surgical approaches.
		Congenital hyperinsulinism (HI) is the most important cause of hypoglycaemia in early infancy (Aynsley-Green et al 2000; Cornblath et al 1990; Pagliara et al 1973; Thomas et al 1977).
	lib.bioinfo.pl /meid:7639 (4381 words)

	congenital hyperinsulinism - humpath.com (Site not responding. Last check: 2007-11-03)
		hyperinsulinemic hypoglycemia in congenital disorder of glycosylation Ia (CDG-Ia)
		Hyperinsulinism in infancy: from basic science to clinical disease.
		Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations.
	www.humpath.com /article.php3?id_article=3137 (143 words)

	Congenital hyperinsulinism
		Adults with the mildest degrees of congenital hyperinsulinism may simply have a decreased tolerance for prolonged fasting.
		In many cases the first evidence of hyperinsulinism is an usually high requirement for intravenous glucose.
		The initial pancreatectomy became a prolonged process of repeated microscopic examination of small pieces removed from various parts of the pancreas while the infant was under anesthesia in the operating room, with an experienced surgeon and pathologist attempting to identify a focal region of overactive cells which could be completely removed.
	www.mrsci.com /Endocrinology/Congenital_hyperinsulinism.php (1098 words)

	Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin ... (Site not responding. Last check: 2007-11-03)
		Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation.
		Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation.This study evaluated whether focal vs. diffuse hyperinsulinism could be diagnosed by acute insulin response (AIR) tests and whether arterial calcium stimulation/venous sampling (ASVS) could localize focal lesions.
		Recessive mutations of the ATP-dependent plasma membrane potassium channel (K(ATP)) genes, SUR1 and K(ir)6.2, cause diffuse hyperinsulinism.
	www.ihop-net.org /UniPub/iHOP/gp/10392903.html (244 words)

	HI, low blood sugar, hypoglycemia, PHHI in children \| Congenital Hyperinsulinism Center - Children's Hospital of ...
		Congenital Hyperinsulinism Center - Children's Hospital of Philadelphia
		Congenital Hyperinsulinism (HI) is a disorder that causes low blood sugar (hypoglycemia) in infants and children.
		The Children's Hospital of Philadelphia is currently the only medical center in the United States that conducts the acute insulin response test for congenital HI and is the only American center that uses the insulin stimulation test to avoid the need for near total pancreatectomy in the treatment of HI.
	www.chop.edu /consumer/jsp/division/service.jsp?id=47690 (260 words)

	Abstracts for References 1-4 of 'Pathogenesis, clinical features, and diagnosis of persistent hyperinsulinemic ...
		In the diffuse form of CHI, the hyperinsulinism is due to a recessive mutation of both alleles of these genes (rare dominant mutations have been described).
		Congenital hyperinsulinism is one of the most common causes of recurrent hypoglycaemia in early infancy.
		Diagnosis of infancy-onset hyperinsulinism was often delayed because of less profound hypoglycaemia and better tolerance to hypoglycaemia.
	patients.uptodate.com /abstract.asp?TR=pediendo/14471&viewAbs=1~2~3~4&title=1-4 (1194 words)

	DiabetoValens.com - The Diabetes Guide
		Primary hyperinsulinism is a rare but important cause of hypoglycemia in infants and children.
		Hyperinsulinism usually resolves spontaneously when the infant is aged several weeks or months.
		Infants with hyperinsulinism are at higher risk of neurologic sequelae than infants with hypoglycemia from other causes.
	my.diabetovalens.com /nwly_dgsd/hinsulin.asp (774 words)

	Congenital Hyperinsulinism International (CHI) is a charitable organization dedicated to improving the lives of ... (Site not responding. Last check: 2007-11-03)
		Congenital Hyperinsulinism International (CHI) is a charitable organization dedicated to improving the lives of children, adults, and families living with congenital hyperinsulinism.
		Advocate on behalf of children and adults with congenital hyperinsulinism.
		Create and provide educational resources about congenital hyperinsulinism to patients, families, medical professionals, school personnel, and any other interested parties.
	www.congenitalhi.org (116 words)

	Preoperative Evaluation of Infants with Focal or Diffuse Congenital Hyperinsulinism by Intravenous Acute Insulin ... (Site not responding. Last check: 2007-11-03)
		hypoglycemia in 86% of the infants with focal hyperinsulinism.
		The Diagnosis of Ectopic Focal Hyperinsulinism of Infancy with [18F]-Dopa Positron Emission Tomography
		Noninvasive Diagnosis of Focal Hyperinsulinism of Infancy With [18F]-DOPA Positron Emission Tomography
	jcem.endojournals.org /cgi/content/full/89/1/288 (5090 words)

	eMedicine - Hyperinsulinemia : Article by Robert J Ferry, Jr, MD (Site not responding. Last check: 2007-11-03)
		Prolonged hyperinsulinism in infants who are SGA and asphyxiated newborns: Infants who are SGA, experience maternal toxemia, or have birth asphyxia are at increased risk for developing hypoglycemia.
		Patients with severe hyperinsulinism may be refractory to medical therapy and may require excision of a portion of or the entire pancreas.
		Stanley CA, Lieu YK, Hsu BY: Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
	www.emedicine.com /ped/topic1075.htm (5472 words)

	Diabetes Week -- Your Online Diabetes Newsletter (Site not responding. Last check: 2007-11-03)
		Researchers at Children's Hospital of Philadelphia have developed a surgical process that carries a high cure rate for a congenital condition in infants known as focal hyperinsulinism without causing diabetes.
		At most centers where surgery is performed for hyperinsulinism, the pancreas is almost completely removed, which leaves the child at risk of developing diabetes.
		The most severe forms of congenital hyperinsulinism affect an estimated 100 to 200 infants annually in the United States.
	www.diabetesweek.org /members/vol1/news/011101.htm (307 words)

	DanforthCenter.org
		The enzyme is a homohexamer that is tightly regulated by a large number of positive and negative allosteric effectors as well as by cooperative interactions between subunits.
		Congenital hyperinsulinism is the most common cause of hypoglycemia in early infancy and can result in seizures or coma (Stanley and Baker 1976).
		The GTP molecule is shown as a ballandstick figure, the C-alpha backbone is shown as a stick model (with color ramped from red->blue as you go from the N to C terminus), and the sidechains that are mutated in this disorder are shown as stick figures extending from the backbone.
	www.danforthcenter.org /smith/gdh.htm (1263 words)

	DiabetoValens.com - The Diabetes Guide
		Congenital hyperinsulinism (HI) is the most common cause of persistent hypoglycemia in neonates.
		Accurate diagnosis is important because one type of HI is diffuse, occurring throughout the pancreas and requiring that 95 percent of the organ be removed, a procedure called near-total pancreatectomy.
		Medical tests performed by interventional radiologists were used to guide the diagnosis and the delicate surgery on the infant's pancreas, an organ the size of an adult's little finger.
	my.diabetovalens.com /nwly_dgsd/newborns.asp (721 words)

	Hypoglycemia
		Congenital hypopituitarism Congenital hyperinsulinism inborn errors of carbohydrate metabolism such as glycogen storage disease
		Suspected mechanisms have included increased glucose utilization by a large or active tumor, cachexia and depletion of glycogen reserves, inhibited hepatic gluconeogenesis or glycogenolysis, blunted counterregulatory hormone responses, autoimmune stimulation of insulin receptors, and production by the tumor of peptides (e.g., insulin-like growth factor 2) or antibodies capable of activating insulin receptors.
		Hyperinsulinism Hypoglycemia due to endogenous insulin Congenital hyperinsulinism Transient neonatal hyperinsulinism Due to maternal factors Maternal diabetes Erythroblastosis fetalis Excessive intravenous glucose during labor Maternal sulfonylurea Sympathomimetic tocolytic agents (e.g.
	www.doggy411.com /show_article.do?id=234 (4396 words)

	SSIEM (Site not responding. Last check: 2007-11-03)
		This two-day international symposium will feature recent exciting advances in research related to congenital hyperinsulinism, neonatal diabetes and disorders of ancreatic islet regulation in children.
		The role of beta-cell potassium channel defects in congenital hyperinsulinism and permanent neonatal diabetes mellitus.
		Hyperinsulinism disorders of glucokinase and glutamate dehydrogenase and current concepts of fuel-mediated insulin secretion.
	www.ssiem.org /news.asp?id=7 (202 words)

	eMedicine - Persistent Hyperinsulinemic Hypoglycemia of Infancy : Article by Robert S Gillespie, MD, MPH (Site not responding. Last check: 2007-11-03)
		Hyperinsulinism with hyperammonemia and elevated levels of FFA suggests a fatty acid oxidation disorder.
		Hyperinsulinism with hyperammonemia and normal levels of FFA suggest the diagnosis of hyperinsulinism with hyperammonemia, a clinically and genetically distinct variant of persistent hyperinsulinemic hypoglycemia of infancy.
		Persistent hyperinsulinism, then, may represent a derangement of the developmental process or the extreme end of a spectrum of endocrine cell function.
	www.emedicine.com /ped/topic1565.htm (7264 words)

	Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism.
		RESULTS: Children with diffuse SUR1(-/-) disease and infants with congenital focal hyperinsulinism responded to CaAIR, whereas the normal control group, patients with hyperinsulinism/hyperammonemia syndrome, and SUR1(+/-) carriers did not.
		OBJECTIVES: To identify infants with hyperinsulinism caused by defects of the beta-cell adenosine triphosphate-dependent potassium channel complex and to distinguish focal and diffuse forms of hyperinsulinism caused by these mutations.
		Three infants with congenital focal disease, 1 with diffuse hyperinsulinism, and the child with insulinoma underwent selective pancreatic intra-arterial calcium stimulation with hepatic venous sampling.
	www.ihop-net.org /UniPub/iHOP/gp/8427181.html (226 words)

	eMedicine - Pediatrics, Hypoglycemia : Article by Hilarie Cranmer, MD, MPH, FACEP (Site not responding. Last check: 2007-11-03)
		Hyperinsulinism, or persistent hyperinsulinemic hypoglycemia of infancy (PHHI), is the most common cause of hypoglycemia in the first 3 months of life.
		Congenital hyperinsulinism most commonly is associated with an abnormality of beta-cell regulation throughout the pancreas.
		Remission of congenital hyperinsulinism generally does not occur, but the severity of the disease may decrease with time.
	www.emedicine.com /emerg/topic384.htm (2716 words)

	Diazoxide \| Congenital Hyperinsulinism Center - Children's Hospital of Philadelphia
		Hyperinsulinism is caused by many different genetic defects.
		The second most common cause of hyperinsulinism is the hyperinsulinism/hyperammonemia syndrome, in which the gene defect has nothing to do with the potassium channel, but instead interferes with the metabolism of glutamate in the cell, causing excessive insulin secretion.
		The third type of hyperinsulinism, which is extremely rare, is glucokinase hyperinsulinism.
	www.chop.edu /consumer/jsp/division/generic.jsp?id=71070 (507 words)

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