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https://www.readbyqxmd.com/read/28605545/atypical-forms-of-congenital-hyperinsulinism-in-infancy-are-associated-with-mosaic-patterns-of-immature-islet-cells
#1
Bing Han, Zainab Mohamed, Maria Salomon Estebanez, Ross J Craigie, Melanie Newbould, Edmund Cheesman, Raja Padidela, Mars Skae, Matthew Johnson, Sarah Flanagan, Sian Ellard, Karen E Cosgrove, Indraneel Banerjee, Mark J Dunne
Objectives: We aimed to characterise mosaic populations of pancreatic islet cells from patients with atypical CHI (CHI-A) and the expression profile of NKX2.2, a key transcription factor expressed in β-cells but suppressed in δ-cells in the mature pancreas. Patients/Methods: Tissue was isolated from three patients with CHI-A following subtotal pancreatectomy. CHI-A was diagnosed on the basis of islet mosaicism and the absence of histopathological hallmarks of focal- and diffuse CHI (CHI-D)...
June 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28600547/modeling-congenital-hyperinsulinism-with-abcc8-deficient-human-embryonic-stem-cells-generated-by-crispr-cas9
#2
Dongsheng Guo, Haikun Liu, Aynisahan Ruzi, Ge Gao, Abbas Nasir, Yanli Liu, Fan Yang, Feima Wu, Guosheng Xu, Yin-Xiong Li
Congenital hyperinsulinism (CHI) is a rare genetic disorder characterized by excess insulin secretion, which results in hypoglycemia. Mutation of sulfonylurea receptor 1 (SUR1), encoded by the ABCC8 gene, is the main cause of CHI. Here, we captured the phenotype of excess insulin secretion through pancreatic differentiation of ABCC8-deficient stem cells generated by the CRISPR/Cas9 system. ABCC8-deficient insulin-producing cells secreted higher insulin than their wild-type counterparts, and the excess insulin secretion was rescued by nifedipine, octreotide and nicorandil...
June 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28597971/diagnosis-of-congenital-hyperinsulinism-biochemical-profiles-during-hypoglycemia
#3
Azumi Sakakibara, Yukiko Hashimoto, Rie Kawakita, Yuki Hosokawa, Keiko Nagahara, Yukihiro Hasegawa, Shin Hoshino, Hironori Nagasaka, Tohru Yorifuji
OBJECTIVES: To define the ranges of biochemical markers during hypoglycemia for the diagnosis of congenital hyperinsulinism (CHI), using high sensitivity insulin assays. SUBJECTS: A total of 298 patients with CHI and 58 control patients with non-hyperinsulinemic hypoglycemia, who were diagnosed after 2007. METHODS: The levels of biochemical markers (glucose, insulin, β-hydroxybutyrate [BHB], free fatty acids [FFA], lactate, ammonia) at the time of hypoglycemia were analyzed along with the maximal glucose infusion rate (GIR) to maintain euglycemia and clinical outcomes...
June 9, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28594910/testosterone-improves-the-differentiation-efficiency-of-insulin-producing-cells-from-human-induced-pluripotent-stem-cells
#4
Haikun Liu, Dongsheng Guo, Aynisahan Ruzi, Yan Chen, Tingcai Pan, Fan Yang, Jialiang Li, Kecheng Xu, Tiancheng Zhou, Dajiang Qin, Yin-Xiong Li
Human induced pluripotent stem cells (hiPSCs) may provide potential resource for regenerative medicine research, including generation of insulin-producing cells for diabetes research and insulin production. Testosterone (T) is an androgen hormone which promotes protein synthesis and improves the management of type 2 diabetes in clinical studies. Concurrently, co-existed hyperandrogenism and hyperinsulinism is frequently observed in polycystic ovary syndrome, congenital adrenal hyperplasia and some of Wermer's syndrome...
2017: PloS One
https://www.readbyqxmd.com/read/28588306/metabolomics-allow-the-discrimination-of-the-pathophysiological-relevance-of-hyperinsulinism-in-obese-prepubertal-children
#5
G Á Martos-Moreno, A Mastrangelo, V Barrios, A García, J A Chowen, F J Rupérez, C Barbas, J Argente
BACKGROUND/OBJECTIVES: Insulin resistance (IR) is the cornerstone of the obesity-associated metabolic derangements observed in obese children. Targeted metabolomics was employed to explore the pathophysiological relevance of hyperinsulinemia in childhood obesity in order to identify biomarkers of IR with potential clinical application. SUBJECTS/METHODS: One hundred prepubertal obese children (50 girls/50 boys, 50% IR and 50% non-IR in each group), underwent an oral glucose tolerance test for usual carbohydrate and lipid metabolism determinations...
June 7, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28576129/treatment-with-long-acting-lanreotide-autogel-in-early-infancy-in-patients-with-severe-neonatal-hyperinsulinism
#6
Heike Corda, Sebastian Kummer, Alena Welters, Norbert Teig, Dirk Klee, Ertan Mayatepek, Thomas Meissner
BACKGROUND: Treatment of severe diffuse congenital hyperinsulinism (CHI) without sufficient response to diazoxide is complicated by the lack of approved drugs. Therefore, patients are often hospitalized long-term or have to undergo pancreatic surgery if episodes of severe hypoglycaemia cannot be prevented. A long-acting somatostatin analogue, octreotide, has been reported to be an effective treatment option that prevents severe hypoglycaemia in children with CHI, and its off-label use is common in CHI...
June 2, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28567298/recurrent-insulinoma-in-a-10-year-old-boy-with-down-s-syndrome
#7
Noman Ahmad, Abdulmonem Mohammed Almutawa, Mohamed Ziyad Abubacker, Hossam Ahmed Elzeftawy, Osama Abdullah Bawazir
An insulinoma is a rare tumour with an incidence of four cases per million per year in adults. The incidence in children is not established. There is limited literature available in children with insulinoma, and only one case is reported in association with Down's syndrome in adults. Insulinoma diagnosis is frequently missed in adults as well as in children. The Whipple triad is the most striking feature although it has limited application in young children. Hypoglycaemia with elevated insulin, C-peptide and absent ketones is highly suggestive of hyperinsulinism...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28561134/cavitron-ultrasonic-surgical-aspirator-assisted-pancreatic-resection-in-hyperinsulinism
#8
Logan Bond, Zaria Murrell, Tiffany Wright, Sheldon Bond
No abstract text is available yet for this article.
May 23, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28549478/gladiator-a-global-approach-for-elucidating-disease-modules
#9
Yael Silberberg, Martin Kupiec, Roded Sharan
BACKGROUND: Understanding the genetic basis of disease is an important challenge in biology and medicine. The observation that disease-related proteins often interact with one another has motivated numerous network-based approaches for deciphering disease mechanisms. In particular, protein-protein interaction networks were successfully used to illuminate disease modules, i.e., interacting proteins working in concert to drive a disease. The identification of these modules can further our understanding of disease mechanisms...
May 26, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28532504/vineland-adaptive-behavior-scales-to-identify-neurodevelopmental-problems-in-children-with-congenital-hyperinsulinism-chi
#10
Maria Salomon-Estebanez, Zainab Mohamed, Maria Michaelidou, Hannah Collins, Lindsey Rigby, Mars Skae, Raja Padidela, Stewart Rust, Mark Dunne, Karen Cosgrove, Indraneel Banerjee, Jacqueline Nicholson
BACKGROUND: Congenital Hyperinsulinism (CHI) is a disease of severe hypoglycaemia caused by excess insulin secretion and associated with adverse neurodevelopment in a third of children. The Vineland Adaptive Behavior Scales Second Edition (VABS-II) is a parent report measure of adaptive functioning that could be used as a developmental screening tool in patients with CHI. We have investigated the performance of VABS-II as a screening tool to identify developmental delay in a relatively large cohort of children with CHI...
May 22, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28531113/retrospective-evaluation-of-metformin-and-or-metformin-plus-a-new-polysaccharide-complex-in-treating-severe-hyperinsulinism-and-insulin-resistance-in-obese-children-and-adolescents-with-metabolic-syndrome
#11
Stefano Stagi, Franco Ricci, Martina Bianconi, Maria Amina Sammarco, Giovanna Municchi, Sonia Toni, Lorenzo Lenzi, Alberto Verrotti, Maurizio de Martino
Background: Pharmacological treatment of obesity and glucose-insulin metabolism disorders in children may be more difficult than in adults. Thus, we evaluate the effects of metformin in comparison with metformin plus a polysaccharide complex (Policaptil Gel Retard(®), PGR) on body weight and metabolic parameters in obese children and adolescents with metabolic syndrome (MetS). Patients and methods: We retrospectively collected 129 children and adolescents (67 girls, 62 boys; median age 12.6 years) treated for a minimum of two years with metformin and low glycemic index (LGI) diet...
May 20, 2017: Nutrients
https://www.readbyqxmd.com/read/28508606/surgery-in-focal-congenital-hyperinsulinism-chi-the-hyperinsulinism-germany-international-experience-in-30-children
#12
Winfried Barthlen, Emine Varol, Susann Empting, Ilse Wieland, Martin Zenker, Wolfgang Mohnike, Silke Vogelgesang, Klaus Mohnike
OBJECTIVES: Results of surgery for focal CHI in 30 children PATIENTS AND METHODS: All showed an ABCC8 or KCNJ11 mutation. After PET/CT in 29 children and PET/MRT in 1 case, frozen-section guided resection was performed, in left-sided cases by laparoscopy. Mean age at surgery was 11.7 months (2-49). RESULTS: In 28/30 children, the PET/CT or MRT correlated with histopathology. In two cases, a focal lesion was undectable; one of these was cured, one not. In total, 24 children showed lesions with sizes of 5-12 mm...
December 2016: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/28491926/a-novel-intragenic-slc16a1-mutation-associated-with-congenital-hyperinsulinism
#13
Mustafa Tosur, George S Jeha
No abstract text is available yet for this article.
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28485095/comparison-of-myo-inositol-and-metformin-on-clinical-metabolic-and-genetic-parameters-in-polycystic-ovary-syndrome-a-randomized-controlled-clinical-trial
#14
Mehri Jamilian, Pegah Farhat, Fatemeh Foroozanfard, Faraneh Afshar Ebrahimi, Esmat Aghadavod, Fereshteh Bahmani, Bita Badehnoosh, Hamidreza Jamilian, Zatollah Asemi
OBJECTIVE: To our knowledge, data on comparison of myo-inositol and metformin on clinical, metabolic and genetic parameters in subjects with polycystic ovary syndrome (PCOS) are limited. This study was carried out to compare myo-inositol and metformin on clinical, metabolic and genetic parameters in subjects with PCOS. DESIGN, PATIENTS AND MEASUREMENTS: This randomized controlled trial was conducted among 60 subjects with PCOS aged 18-40 years. Subjects were randomly allocated into two groups to receive either myo-inositol (N=30) or metformin (N=30) for 12 weeks...
May 9, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28458900/congenital-hyperinsulinism-and-poland-syndrome-in-association-with-10p13-14-duplication
#15
Dinesh Giri, Prashant Patil, Rachel Hart, Mohammed Didi, Senthil Senniappan
SUMMARY: Poland syndrome (PS) is a rare congenital condition, affecting 1 in 30 000 live births worldwide, characterised by a unilateral absence of the sternal head of the pectoralis major and ipsilateral symbrachydactyly occasionally associated with abnormalities of musculoskeletal structures. A baby girl, born at 40 weeks' gestation with birth weight of 3.33 kg (-0.55 SDS) had typical phenotypical features of PS. She had recurrent hypoglycaemic episodes early in life requiring high concentration of glucose and glucagon infusion...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28458896/octreotide-therapy-and-restricted-fetal-growth-pregnancy-in-familial-hyperinsulinemic-hypoglycemia
#16
Marianne Geilswijk, Lise Lotte Andersen, Morten Frost, Klaus Brusgaard, Henning Beck-Nielsen, Anja Lisbeth Frederiksen, Dorte Møller Jensen
SUMMARY: Hypoglycemia during pregnancy can have serious health implications for both mother and fetus. Although not generally recommended in pregnancy, synthetic somatostatin analogues are used for the management of blood glucose levels in expectant hyperinsulinemic mothers. Recent reports suggest that octreotide treatment in pregnancy, as well as hypoglycemia in itself, may pose a risk of fetal growth restriction. During pregnancy, management of blood glucose levels in familial hyperinsulinemic hypoglycemia thus forms a medical dilemma...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28442472/functional-and-metabolomic-consequences-of-atp-dependent-potassium-channel-inactivation-in-human-islets
#17
Changhong Li, Amanda M Ackermann, Kara E Boodhansingh, Tricia R Bhatti, Chengyang Liu, Jonathan Schug, Nicolai Doliba, Bing Han, Karen E Cosgrove, Indraneel Banerjee, Franz M Matschinsky, Itzhak Nissim, Klaus H Kaestner, Ali Naji, N Scott Adzick, Mark J Dunne, Charles A Stanley, Diva D De León
Loss-of-function mutations of β-cell KATP channels cause the most severe form of congenital hyperinsulinism (KATPHI). KATPHI is characterized by fasting and protein-induced hypoglycemia that is unresponsive to medical therapy. For a better understanding of the pathophysiology of KATPHI, we examined cytosolic calcium, insulin secretion, oxygen consumption, and [U-(13)C]glucose metabolism in islets isolated from the pancreases of children with KATPHI who required pancreatectomy. Basal cytosolic calcium ([Ca(2+)] i ) and insulin secretion were higher in KATPHI islets compared to controls...
April 25, 2017: Diabetes
https://www.readbyqxmd.com/read/28439221/congenital-hyperinsulinism-caused-by-a-de-novo-mutation-in-the-abcc8-gene-a-case-report
#18
Zsuzsanna Molnár, Lfdia Balogh, János Kappelmayer, László Madar, Éva Gombos, István Balogh
Congenital hyperinsulinism (CHI) is a rare genetic disorder characterized by inappropriate insulin secretion and severe hypoglycaemia. There are two histological subtypes: diffuse and focal form. Diffuse form is most common in autosomal recessive mutations in ABCC8/KCNJ11 gene, while focal CHI is caused a paternally inherited mutation and a somatic maternal allele loss. Here we report a case of a term male infant presented with severe hyperinsulinaemic hypoglycaemia. Gene panel testing was performed to give rapid genetic diagnosis...
March 2017: EJIFCC
https://www.readbyqxmd.com/read/28435156/polycystic-kidney-disease-pmm2-mutation-causes-pkd-and-hyperinsulinism
#19
Ellen F Carney
No abstract text is available yet for this article.
June 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28427440/hypoglycemia-identified-by-a-continuous-glucose-monitoring-system-in-a-second-trimester-pregnant-woman-with-insulinoma-a-case-report
#20
Marjeta Tomazic, Andrej Janez, Maja Ravnik Oblak
BACKGROUND: Insulinoma associated with pregnancy is a very rare condition and can be difficult to diagnose. Here, we present an interesting case of insulinoma occurring during pregnancy with special attention paid to the use of a continuous glucose monitoring system to detect hypoglycemia. CASE PRESENTATION: A 36-year-old white woman in the second trimester of pregnancy presented with recurrent episodes of hypoglycemia associated with neuroglycopenic symptoms. The use of a continuous glucose monitoring system confirmed hypoglycemia...
April 21, 2017: Journal of Medical Case Reports
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