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https://www.readbyqxmd.com/read/29679622/pharmacotherapy-for-hyperglycemia-in-pregnancy-do-oral-agents-have-a-place
#1
REVIEW
Rosa Corcoy, Montserrat Balsells, Apolonia García-Patterson, Anat Shmueli, Eran Hadar
Diabetes is a frequent condition in pregnancy and achieving adequate glycemic control is of paramount importance. Insulin treatment is the gold standard, oral agents are more attractive, but their safety and efficiency should be a prerequisite for their use. We have more information regarding treatment of women with gestational diabetes mellitus where glyburide can induce a picture of fetal hyperinsulinism (higher birthweight and more neonatal hypoglycemia) whereas metformin requires supplemental insulin in a larger proportion of women but achieves satisfactory perinatal outcomes with the exception of preterm birth...
April 18, 2018: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/29599801/continuous-flash-glucose-monitoring-in-children-with-congenital-hyperinsulinism-first-report-on-accuracy-and-patient-experience
#2
Hussain Alsaffar, Lucy Turner, Zoe Yung, Mohammed Didi, Senthil Senniappan
Background: The factory calibrated FreeStyle Libre (FSL) flash glucose monitoring system has been recently introduced for use in patients with diabetes mellitus. There are no reports available regarding its use in patients with congenital hyperinsulinism (CHI). We have assessed the accuracy of FSL compared to the finger prick capillary blood glucose (CBG) over 2 weeks period in patients with CHI and evaluated the parents' experience of using FSL. Methods: Four hundred sixty-seven episodes of CBG along with corresponding swipe FSL readings were available from 11 children with CHI (0...
2018: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/29589989/a-unique-allosteric-insulin-receptor-monoclonal-antibody-that-prevents-hypoglycemia-in-the-sur-1-mouse-model-of-katp-hyperinsulinism
#3
Puja Patela, Lawrenshey Charles, John Corbin, Ira D Goldfine, Kirk Johnson, Paul Rubin, Diva D De León
Loss-of-function mutations of the ß-cell ATP-sensitive potassium channels (KATP ) cause the most common and severe form of congenital hyperinsulinism (KATP HI), a disorder of ß-cell function characterized by severe hypoglycemia. Children with KATP HI are typically unresponsive to medical therapy and require pancreatectomy for intractable hypoglycemia. We tested the hypothesis that inhibition of insulin receptor signaling may prevent hypoglycemia in KATP HI. To test this hypothesis, we examined the effect of an antibody allosteric inhibitor of the insulin receptor, XMetD, on fasting plasma glucose in a mouse model of KATP HI (SUR-1- / - mice)...
March 28, 2018: MAbs
https://www.readbyqxmd.com/read/29589453/-multidisciplinary-approach-to-surgical-disorders-of-the-pancreas-in-children
#4
J Šnajdauf, M Rygl, O Petrů, B Frýbová, J Náhlovský, V Mixa, R Keil, J Bronský, M Kynčl, R Kodet
INTRODUCTION: Surgical diseases of the pancreas in children are not common and may be associated with significant morbidity and potential mortality. A multidisciplinary approach is essential for correct diagnosis, surgical strategy and postoperative as well as follow-up care. METHOD: Retrospective analysis of patients operated on due to a pathological lesion of the pancreas focused on diagnostics, operating procedures, postoperative complications, and long-term results...
2018: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/29558846/focal-congenital-hyperinsulinism-as-a-cause-for-sudden-infant-death
#5
Amish Chinoy, Indraneel Banerjee, Sarah E Flanagan, Sian Ellard, Bing Han, Zainab Mohamed, Mark J Dunne, Stefania Bitetti
Congenital hyperinsulinism (CHI) is the commonest cause of persistent and severe hypoglycemia in infancy due to unregulated insulin secretion from pancreatic β-cells. Prompt early diagnosis is important, as insulin reduces glucose supply to the brain, resulting in significant brain injury and risk of death. Histologically, CHI has focal and diffuse forms; in focal CHI, an inappropriate level of insulin is secreted from localized β-cell hyperplasia. We report a 4-month-old male infant, who presented with sudden illness and collapse without a recognized cause and died...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29538671/corrigendum-for-novel-hypoglycemia-phenotype-in-congenital-hyperinsulinism-due-to-dominant-mutations-of-uncoupling-protein-2
#6
(no author information available yet)
No abstract text is available yet for this article.
March 9, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29503277/ketotic-hypoglycemia-in-children-with-previous-transient-congenital-hyperinsulinism
#7
Prashant Patil, Dinesh Giri, Mohammed Didi, Senthil Senniappan
Congenital Hyperinsulinism (CHI) is a major cause of neonatal hypoglycemia characterised by non-ketotic hypoglycemia. We describe the occurrence and higher prevalence of ketotic hypoglycemia (KH) in 5 children with transient CHI. Four children had required diazoxide to control the persistent hypoglycemia that was discontinued at a mean age of 11.25 (+5.25) months. KH developed after an average time period of 6.7 months following the resolution of CHI. Children with transient CHI may be at risk of subsequently developing KH at a variable age period...
February 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/29502919/complex-phenotypes-in-inborn-errors-of-metabolism-overlapping-presentations-in-congenital-disorders-of-glycosylation-and-mitochondrial-disorders
#8
REVIEW
Thatjana Gardeitchik, Jeroen Wyckmans, Eva Morava
Congenital disorders of glycosylation (CDG) and mitochondrial disorders have overlapping clinical features, including central nervous system, cardiac, gastrointestinal, hepatic, muscular, endocrine, and psychiatric disease. Specific abnormalities orienting the clinician toward the right diagnostic approach include abnormal fat distribution, coagulation abnormalities, together with anticoagulation abnormalities, hyperinsulinism, and congenital malformations in CDG. Diabetes, sensorineural deafness, and depression are very rare in CDG but common in mitochondrial disease...
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502912/inborn-errors-of-metabolism-with-hypoglycemia-glycogen-storage-diseases-and-inherited-disorders-of-gluconeogenesis
#9
REVIEW
David A Weinstein, Ulrike Steuerwald, Carolina F M De Souza, Terry G J Derks
Although hyperinsulinism is the predominant inherited cause of hypoglycemia in the newborn period, inborn errors of metabolism are the primary etiologies after 1 month of age. Disorders of carbohydrate metabolism often present with hypoglycemia when fasting occurs. The presentation, diagnosis, and management of the hepatic glycogen storage diseases and disorders of gluconeogenesis are reviewed.
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29495350/the-role-of-insulin-resistance-hyperinsulinism-on-the-rising-trend-of-thyroid-and-adrenal-nodular-disease-in-the-current-environment
#10
REVIEW
Agathocles Tsatsoulis
Thyroid follicular cells, as well as adrenocortical cells, are endowed by an intrinsic heterogeneity regarding their growth potential, in response to various stimuli. This heterogeneity appears to constitute the underlying cause for the focal cell hyperplasia and eventually the formation of thyroid and adrenal nodules, under the influence of growth stimulatory factors. Among the main stimulatory factors are the pituitary tropic hormones, thyroid-stimulating hormone (TSH) or thyrotropin and adrenocorticotropic hormone (ACTH), which regulate the growth and function of their respective target cells, and the insulin/insulin-like growth factor system, that, through its mitogenic effects, can stimulate the proliferation of these cells...
February 26, 2018: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/29493090/clinical-heterogeneity-of-hyperinsulinism-due-to-hnf1a-and-hnf4a-mutations
#11
Joanna Yuet-Ling Tung, Kara Boodhansingh, Charles A Stanley, Diva D De León
BACKGROUND: Dominant inactivating mutations in HNF1A and HNF4A have been described to cause hyperinsulinism (HI) before evolving to diabetes. However, information available in the literature regarding the clinical phenotype is limited. OBJECTIVE: To report the prevalence of HNF1A and HNF4A mutations in a large cohort of children with HI, and to describe their genotypes and phenotypes. DESIGN: Retrospective descriptive study. METHODS: Medical records were reviewed to extract clinical information...
March 1, 2018: Pediatric Diabetes
https://www.readbyqxmd.com/read/29470101/ebselen-reversibly-inhibits-human-glutamate-dehydrogenase-at-the-catalytic-site
#12
Yanhong Jin, Di Li, Shiying Lu, Han Zhao, Zhao Chen, Wei Hou, Benfang Helen Ruan
Human glutamate dehydrogenase (GDH) plays an important role in neurological diseases, tumor metabolism, and hyperinsulinism-hyperammonemia syndrome (HHS). However, there are very few inhibitors known for human GDH. Recently, Ebselen was reported to crosslink with Escherichia coli GDH at the active site cysteine residue (Cys321), but the sequence alignment showed that the corresponding residue is Ala329 in human GDH. To investigate whether Ebselen could be an inhibitor for human GDH, we cloned and expressed an N-terminal His-tagged human GDH in E...
February 22, 2018: Assay and Drug Development Technologies
https://www.readbyqxmd.com/read/29425029/mechanistic-origins-of-enzyme-activation-in-human-glucokinase-variants-associated-with-congenital-hyperinsulinism
#13
Shawn Sternisha, Peilu Liu, Alan G Marshall, Brian G Miller
Human glucokinase (GCK) acts as the body's primary glucose sensor and plays a critical role in glucose homeostatic maintenance. Gain-of-function mutations in gck produce hyperactive enzyme variants that cause congenital hyperinsulinism. Prior biochemical and biophysical studies suggest that activated disease variants can be segregated into two mechanistically distinct classes, termed α-type and β-type. Steady-state viscosity variation studies indicate that the kcat values of wild-type GCK and an α-type variant are partially diffusion-limited, whereas the kcat value of a β-type variant is viscosity-independent...
February 9, 2018: Biochemistry
https://www.readbyqxmd.com/read/29417725/whole-exome-sequencing-for-mutation-detection-in-pediatric-disorders-of-insulin-secretion-maturity-onset-diabetes-of-the-young-and-congenital-hyperinsulinism
#14
S R Johnson, P J Leo, A M McInerney-Leo, L K Anderson, M Marshall, I McGown, F Newell, M A Brown, L S Conwell, M Harris, E L Duncan
BACKGROUND: To assess the utility of whole-exome sequencing (WES) for mutation detection in maturity-onset diabetes of the young (MODY) and congenital hyperinsulinism (CHI). MODY and CHI are the two commonest monogenic disorders of glucose-regulated insulin secretion in childhood, with 13 causative genes known for MODY and 10 causative genes identified for CHI. The large number of potential genes makes comprehensive screening using traditional methods expensive and time-consuming. METHODS: Ten subjects with MODY and five with CHI with known mutations underwent WES using two different exome capture kits (Nimblegen SeqCap EZ Human v3...
February 8, 2018: Pediatric Diabetes
https://www.readbyqxmd.com/read/29415555/stable-liquid-glucagon-beyond-emergency-hypoglycemia-rescue
#15
Leah M Wilson, Jessica R Castle
Glycemic control is the mainstay of preventing diabetes complications at the expense of increased risk of hypoglycemia. Severe hypoglycemia negatively impacts the quality of life of patients with type 1 diabetes and can lead to morbidity and mortality. Currently available glucagon emergency kits are effective at treating hypoglycemia when correctly used, however use is complicated especially by untrained persons. Better formulations and devices for glucagon treatment of hypoglycemia are needed, specifically stable liquid glucagon...
February 1, 2018: Journal of Diabetes Science and Technology
https://www.readbyqxmd.com/read/29406007/hyperinsulinism-in-the-neonate
#16
REVIEW
Katherine Lord, Diva D De León
Hyperinsulinism (HI) is the leading cause of persistent hypoglycemia in infants. Prompt recognition and treatment, independent of whether infants have transient or permanent HI, are essential to decrease risk of neurologic damage. The most common form of congenital HI is due to inactivating mutations of the β-cell ATP-sensitive potassium (KATP ) channel (KATP -HI) and is typically diazoxide unresponsive. KATP -HI occurs in diffuse and focal forms. Distinguishing between the 2 forms is crucial, because pancreatectomy is curative in the focal form but palliative in the diffuse form...
March 2018: Clinics in Perinatology
https://www.readbyqxmd.com/read/29355062/effects-of-gestational-and-pregestational-diabetes-mellitus-on-the-foetal-heart-a-cross-sectional-study
#17
Pinar Dervisoglu, Mustafa Kosecik, Serkan Kumbasar
We examined the foetal cardiac structural and functional characteristics in diabetic pregnancies versus non-diabetic, healthy pregnancies. Between August 2015 and April 2016, 32 pregnant women with pregestational diabetes, 36 pregnant women with gestational diabetes, and 42 healthy pregnant women were scheduled to have foetal echocardiograms to assess cardiac structure and function. In the diabetic groups, the foetal interventricular septum (IVS) thickness was significantly greater than in non-diabetics (p < ...
April 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29334830/cardiopulmonary-bypass-for-a-patient-with-congenital-hyperinsulinemia
#18
Richard Crook, Richard Issitt
Congenital hyperinsulinism is a clinical syndrome of pancreatic β-cell dysfunction characterized by failure to suppress insulin secretion in the presence of hypoglycemia. Here, we describe the concerns, the techniques used to ameliorate these potential problems, and the outcomes for a child with this condition undergoing cardiopulmonary bypass for correction of an atrial septal defect.
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29329447/congenital-hyperinsulinism-and-hypopituitarism-attributable-to-a-mutation-in-foxa2
#19
Mary Ellen Vajravelu, Jinghua Chai, Bryan Krock, Samuel Baker, David Langdon, Craig Alter, Diva D De León
Context: Persistent hypoglycemia in the newborn period most commonly occurs as a result of hyperinsulinism. The phenotype of hypoketotic hypoglycemia can also result from pituitary hormone deficiencies, including growth hormone and adrenocorticotropic hormone deficiency. Forkhead box A2 (Foxa2) is a transcription factor shown in mouse models to influence insulin secretion by pancreatic β cells. In addition, Foxa2 is involved in regulation of pituitary development, and deletions of FOXA2 have been linked to panhypopituitarism...
March 1, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29301189/congenital-hyperinsulinism-diagnostic-and-management-challenges-in-a-developing-country-case-report
#20
Cheri Mathews John, Prakash Agarwal, Suriyakumar Govindarajulu, Sandhya Sundaram, Senthil Senniappan
Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography (PET/CT) scan (68 Ga-labeled [1,4,7,10-tetraazacyclododecane- N,N',N'',N''' -tetraacetic acid]-1-NaI3-octreotide) demonstrated focal disease in the body of the pancreas. Genetic studies indicated paternal inheritance, making focal disease likely. She was started on diazoxide therapy with partial improvement in blood glucose levels...
December 2017: Annals of Pediatric Endocrinology & Metabolism
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