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Topic: Hyperinsulinism


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  Sur1 Hyperinsulinism
Hyperinsulinism causes a particularly damaging form of hypoglycaemia because it denies the brain of all the fuels on which it is critically dependent.
The diagnosis of hyperinsulinism may be quite difficult if one relies on demonstrating an elevated blood insulin concentration at the time of hypoglycaemia because insulin levels fluctuate widely over time in patients with hyperinsulinism.
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)

  
  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   (547 words)

  
 HI, low blood sugar, hypoglycemia, PHHI in children : Congenital Hyperinsulinism Center - Children's Hospital of ...
Congenital Hyperinsulinism (HI) is a disorder that causes low blood sugar (hypoglycemia) in infants and children.
Help you network with other families as well as with the Hyperinsulinism Team at The Children's Hospital of Philadelphia.
Nathaniel traveled from Georgia to Children's Hospital for surgery to treat his Focal KATP Hyperinsulinism.
www.chop.edu /consumer/jsp/division/service.jsp?id=47690   (304 words)

  
 HighBeam Encyclopedia - hyperinsulinism   (Site not responding. Last check: )
HYPERINSULINISM [hyperinsulinism] presence in the system of an above-normal amount of insulin, the substance secreted by the pancreas and needed by the body to utilize sugar.
The cause of oversecretion of insulin may be organic, i.e., a tumor of the pancreas, impaired liver function, or endocrine disorders, or it may be functional, e.g., unusual muscular exertion, pregnancy, or lactation.
In diabetics, hyperinsulinism is known as insulin shock and may occur from overdosage with insulin in the course of treatment.
www.encyclopedia.com /html/h/hyperins.asp   (304 words)

  
 Congenital hyperinsulinism - Wikipedia, the free encyclopedia
Congenital hyperinsulinism is a medical term referring to a variety of congenital disorders in which hypoglycemia is caused by excessive insulin secretion.
Congenital hyperinsulinism often becomes apparent later in the first year of life, sometimes by a failure to tolerate increasing feeding intervals and an inability to sleep through the night.
In many cases the first evidence of hyperinsulinism is an usually high requirement for intravenous glucose.
en.wikipedia.org /wiki/Congenital_hyperinsulinism   (1130 words)

  
 AllRefer.com - hyperinsulinism (Pathology) - Encyclopedia
hyperinsulinism, presence in the system of an above-normal amount of insulin, the substance secreted by the pancreas and needed by the body to utilize sugar.
Where there is some organic cause for hyperinsulinism, surgery may be required to eliminate it.
Functional hyperinsulinism and insulin shock are treated by dietary measures designed to bring the insulin-sugar ratio into better balance.
reference.allrefer.com /encyclopedia/H/hyperins.html   (243 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.
Hyperinsulinism is a heterogeneous disorder characterised by severe hypoglycaemia due to an inappropriate oversecretion of insulin.
patients.uptodate.com /abstract.asp?TR=pediendo/14471&viewAbs=1~2~3~4&title=1-4   (1194 words)

  
 Practical management of hyperinsulinism in infancy -- Aynsley-Green et al. 82 (2): 98 -- Archives of Disease in ...
Hyperinsulinism in infancy is one of the most difficult problems to manage in contemporary paediatric endocrinology.
Hyperinsulinism is the most common cause of persistent or recurrent hypoglycaemia in infancy.
Hyperinsulinism and hyperammonaemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
fn.bmjjournals.com /cgi/content/full/82/2/F98   (6301 words)

  
 Hyperinsulinism of the newborn.
Hyperinsulinism of the newborn.Also, a new syndrome of hyperinsulinism associated with benign hyperammonemia was recently described and found to be caused by activating mutations in the glutamate dehydrogenase (GDH) gene (GLUD-1).
Although in most families, the disease is transmitted as an autosomal recessive trait, a novel form of transmission, resulting in focal involvement of the pancreas has recently been described.
Neonatal hyperinsulinism (HI) is a clinical syndrome of pancreatic beta-cell dysfunction characterized by failure to suppress insulin secretion in the presence of hypoglycemia.
www.ihop-net.org /UniPub/iHOP/gp/8457862.html   (253 words)

  
 Exercise induced hypoglycaemic hyperinsulinism -- Meissner et al. 84 (3): 254 -- Archives of Disease in Childhood
Hyperinsulinism in childhood is often caused by genetic defects involving the regulation of insulin secretion leading to recurrent
Hyperinsulinism throughout the day was confirmed by frequent blood sampling.
Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
adc.bmjjournals.com /cgi/content/full/84/3/254   (1701 words)

  
 Hyperinsulinism: molecular aetiology of focal disease -- Ryan et al. 79 (5): 445 -- Archives of Disease in Childhood
mutation on the paternal allele in a patient with hyperinsulinism.
We propose a reduction to homozygosity of an SUR-1 mutation as
The Diagnosis of Ectopic Focal Hyperinsulinism of Infancy with [18F]-Dopa Positron Emission Tomography
adc.bmjjournals.com /cgi/content/full/79/5/445   (1549 words)

  
 eMedicine - Hyperinsulinemia : Article by Kenneth Kwok-Chun Chan, MD
Prolonged hyperinsulinism in infants who are small for gestational age (SGA) and asphyxiated newborns: Infants who are SGA, experience maternal toxemia, or have birth asphyxia are at increased risk for developing hypoglycemia.
Patients with hyperinsulinism usually have elevated levels of insulin for their glucose concentration (ie, even if they do not have hypoglycemia, their insulin level is inappropriately high for their glucose levels).
Patients with severe hyperinsulinism may be refractory to medical therapy and require excision of a portion or the entire pancreas.
www.emedicine.com /PED/topic1075.htm   (4205 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.eurekalert.org /pub_releases/2006-05/asfb-edl052406.php   (653 words)

  
 Murine model of human hyperinsulinism
Familial hyperinsulinism (HI) is a rare inherited disorder that results from the unregulated release of insulin by pancreatic b cells.
As published in G&D, researchers from UPENN Medical Center have generated a strain of mice in which the gene Foxa2 is specifically deleted in pancreatic b cells.
Previous attempts to investigate the role of Foxa2 in b cell physiology were thwarted by the fact that mice which are completely deficient for Foxa2 lack a pancreas.
www.eurekalert.org /pub_releases/2001-06/CSHL-Mmoh-3006101.php   (363 words)

  
 Endotext.com - Insulinomas
An accurate diagnosis of organic hyperinsulinism can be established with near certainty in all cases (252).
We now believe that the combination of a secretin test to determine the nature of the hyperinsulinism (e.g., distinction of hyperplasia from adenoma or multiple adenomatosis) with PTHVS to localize provides the best means of establishing the specific cause of organic hyperinsulinism with near certainty.
Patients with diffuse hyperinsulinism for whom an operation is planned first should have a trial of treatment with diazoxide and a natriuretic benzothiadiazine.
www.endotext.org /guthormones/guthormone4/guthormone4.htm   (2327 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)

  
 Fast DNA Mapping
Neurologist Vivian G. Cheung, M.D., and colleagues have already used the new method to map the segment of DNA known to be responsible for a rare disease called congenital hyperinsulinism.
Congenital hyperinsulinism is an autosomal recessive disease that occurs at relatively high frequency among Ashkenazi Jews.
The GMS-selected DNA samples were found to hybridize to only a few segments on the microarray, thus localizing the gene for hyperinsulinism to a relatively small region of chromosome 11.
www.accessexcellence.org /WN/SUA12/fastdna398.html   (543 words)

  
 Clinical and Molecular Characterization of a Dominant Form of Congenital Hyperinsulinism Caused by a Mutation in the ...   (Site not responding. Last check: )
Clinical and Molecular Characterization of a Dominant Form of Congenital Hyperinsulinism Caused by a Mutation in the High-Affinity Sulfonylurea Receptor -- Thornton et al.
Congenital hyperinsulinism in children is a disorder of persistent
adjacent to the delPhe1388 mutation that causes recessive hyperinsulinism
diabetes.diabetesjournals.org /cgi/content/full/52/9/2403   (4959 words)

  
 Octreotide in hyperinsulinism -- Barrons 31 (2): 239 -- The Annals of Pharmacotherapy
Octreotide in hyperinsulinism -- Barrons 31 (2): 239 -- The Annals of Pharmacotherapy
Octreotide may worsen existing hypoglycemia as result of suppressing glucagon and growth hormone in the presence of unresponsive pancreatic hyperinsulinism.
Octreotide may be useful in management of hypoglycemic symptoms in adult patients requiring medical treatment for insulinoma who are refractory or intolerant of diazoxide.
www.theannals.com /cgi/content/abstract/31/2/239   (312 words)

  
 Preoperative Evaluation of Infants with Focal or Diffuse Congenital Hyperinsulinism by Intravenous Acute Insulin ...
hypoglycemia in 86% of the infants with focal hyperinsulinism.
Not shown is one case with a presumed lesion in the head of the pancreas and 10 focal cases with large lesions.
hyperinsulinism after surgery in cases of focal disease.
jcem.endojournals.org /cgi/content/full/89/1/288   (5090 words)

  
 New Syndrome of Hyperinsulinism and Hyperammonemia
Although there are many causes of hypoglycemia, a new syndrome associating hyperinsulinism with hyperammonemia was recently described (Zammarachi, et al.
The incidence of this type of hypoglycemia is significant.
The authors conclude that ammonia concentrations should be measured in every patient investigated for hyperinsulinism and that, conversely, hypoglycemia should be looked for in all patients with unexplained hyperammonemia.
www.gghjournal.com /volume18/18-2/articles/new.htm   (346 words)

  
 Hyperinsulinism and Hyperammonemia Syndrome: Report of Twelve Unrelated Patients -- DE LONLAY et al. 50 (3): 353 -- ...   (Site not responding. Last check: )
Hyperinsulinism is a common cause of recurrent hypoglycemia
Stanley C 1997 Hyperinsulinism in infants and children.
Bruining GJ 1990 Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus.
www.pedresearch.org /cgi/content/full/50/3/353   (2918 words)

  
 Children's Mercy Hospitals and Clinics
Hyperinsulinism occurs when a body becomes resistant to insulin.
Hyperinsulinism does not mean your child has diabetes.
You should always understand how the medication works, what the side effects are and any special precautions that need to be taken while taking any medication.
www.childrens-mercy.org /CareCard/Card/View.asp?lang=e&id=CMH-04-269   (294 words)

  
 Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1 -- Huopio et al. 106 (7): ...
Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1 -- Huopio et al.
Mutations in the sulonylurea receptor gene are associated with familial hyperinsulinism in Ashkenazi Jews.
Hyperinsulinism caused by paternal-specific inheritance of a recessive mutation in the sulfonylurea-receptor gene.
www.jci.org /cgi/content/full/106/7/897   (6323 words)

  
 Hyperinsulinism Reduction Associated with Icodextrin Treatment in Continuous Ambulatory Peritoneal Dialysis Patients
In these patients, this condition can lead to stable elevated plasma insulin levels and, consequently, to reduced tissue insulin sensitivity owing to downregulation of tissue receptor expression (7).
This condition is generally known as hyperinsulinism, or insulin resistance.
The reduction or elimination of free glucose load through the use of alternative solutions is a promising strategy for reducing hyperinsulinism and the associated cardiovascular risk in CAPD patients.
www.advancesinpd.com /adv01/17Gianpaolo.htm   (1830 words)

  
 Hyperinsulinism and Cerebral Microangiopathy -- Zunker et al. 27 (2): 219 -- Stroke   (Site not responding. Last check: )
Hyperinsulinism and Cerebral Microangiopathy -- Zunker et al.
The prevalence of arterial hypertension in patients with hyperinsulinism
Experimental atherosclerosis-like lesions induced by hyperinsulinism in Wistar rats.
stroke.ahajournals.org /cgi/content/full/27/2/219   (2979 words)

  
 Medical Dictionary: Hyperinsulinism - WrongDiagnosis.com
Hyperinsulinism: A syndrome with excessively high INSULIN levels in the BLOOD.
Etiology of hyperinsulinism varies, including hypersecretion of a beta cell tumor (INSULINOMA); autoantibodies against insulin (INSULIN ANTIBODIES); defective insulin receptor (INSULIN RESISTANCE); or overuse of exogenous insulin or HYPOGLYCEMIC AGENTS.
Hyperinsulinism : syndrome with excessively high insulin levels in the blood; it may cause hypoglycemia; etiology of hyperinsulinism varies, including hypersecretion of a beta cell tumor, autoantibodies against insulin, defective insulin receptor, or overuse of exogeneous insulin or hypoglycemic agents.
www.wrongdiagnosis.com /medical/hyperinsulinism.htm   (212 words)

  
 Earliest Cooking? / Mismatch of Grains/Dairy w/Human Physiology
Lastly, and most importantly, significant amounts of grain consumption considerably increase the carbohydrate intake of the diet, and excessive carbohydrate consumption is the primary factor driving what has come to be known as the hyperinsulinism syndrome.
Hyperinsulinism and its associated constellation of resulting symptoms, collectively known as "Syndrome X," is not yet well-
If the relationship is causative, then by extension hyperinsulinism would also presumably be causative of the health problems that these symptoms themselves lead to (i.e., heart disease, etc.).
www.beyondveg.com /nicholson-w/hb/hb-interview2g.shtml   (2078 words)

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