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https://www.readbyqxmd.com/read/28701683/efficacy-and-safety-of-octreotide-for-the-treatment-of-congenital-hyperinsulinism-a-prospective-open-label-clinical-trial-and-an-observational-study-in-japan-using-a-nationwide-registry
#1
Yuki Hosokawa, Rie Kawakita, Susumu Yokoya, Tsutomu Ogata, Keiichi Ozono, Osamu Arisaka, Yukihiro Hasegawa, Satoshi Kusuda, Michiya Masue, Hironori Nishibori, Toshimi Sairenchi, Tohru Yorifuji
Octreotide, a long-acting somatostatin analog, has been used for treating hypoglycemia caused by congenital hyperinsulinism (CHI). However, octreotide has not been evaluated in clinical trials and has not been approved in any developed country. We aimed to test the efficacy and safety of octreotide for diazoxide-unresponsive CHI through a combination of a single-arm, open-label clinical trial (SCORCH study) and an observational study to collect data on the clinical course of patients treated off-label in Japan (SCORCH registry)...
July 11, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28663158/an-abcc8-nonsense-mutation-causing-neonatal-diabetes-through-altered-transcript-expression
#2
Sarah Flanagan, Vũ Chí Dũng, Jayne Houghton, Elisa De Franco, Can Thi Bich Ngoc, Annet Damhuis, Frances Ashcroft, Lorna Harries, Sian Ellard
OBJECTIVE: The pancreatic ATP-sensitive K+ (K-ATP) channel is a key regulator of insulin secretion. Gain-of-function mutations in the genes encoding the Kir6.2 (KCNJ11) and SUR1 (ABCC8) subunits of the channel cause neonatal diabetes whilst loss-of-function mutations in these genes result in congenital hyperinsulinism. We report two patients with neonatal diabetes in whom we unexpectedly identified recessively inherited loss-of-function mutations. The aim of this study was to investigate how a homozygous nonsense mutation in ABCC8 could result in neonatal diabetes...
June 30, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28650702/a-case-report-of-methadone-associated-hypoglycemia-in-an-11-month-old-male
#3
Michael S Toce, Margaret A Stefater, David T Breault, Michele M Burns
BACKGROUND: Methadone is a synthetic μ-opioid receptor agonist that is used in the management of pain, neonatal abstinence withdrawal syndrome, and opioid dependence. Overdose can cause miosis, respiratory depression, and central nervous system depression. Rarely, hypoglycemia has been reported. We present the case of an 11-month-old male who developed hypoketotic, hyperinsulinemic, hypoglycemia after an acute, unintentional methadone exposure. CASE DETAILS: The patient was a previously healthy 11-month-old male who presented in respiratory failure...
June 26, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28642988/development-of-pulmonary-hypertension-during-treatment-with-diazoxide-a-case-series-and-literature-review
#4
Matthew R Timlin, Alexander B Black, Heather M Delaney, Renée I Matos, Candace S Percival
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy. The mainstay of medical management for CHI is diazoxide. Diazoxide inhibits insulin release from the pancreas, but also causes smooth muscle relaxation and fluid retention so it is typically given with chlorothiazide. In July 2015, the FDA issued a drug safety communication warning that pulmonary hypertension (PH) had been reported in 11 infants being treated with diazoxide and that the PH resolved with withdrawal of diazoxide...
June 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28605545/atypical-forms-of-congenital-hyperinsulinism-in-infancy-are-associated-with-mosaic-patterns-of-immature-islet-cells
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
Logan Bond, Zaria Murrell, Tiffany Wright, Sheldon Bond
No abstract text is available yet for this article.
August 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28549478/gladiator-a-global-approach-for-elucidating-disease-modules
#13
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
#14
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
#15
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
#16
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
#17
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%C3%A2-on-clinical-metabolic-and-genetic-parameters-in-polycystic-ovary-syndrome-a-randomized-controlled-clinical-trial
#18
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...
August 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28458900/congenital-hyperinsulinism-and-poland-syndrome-in-association-with-10p13-14-duplication
#19
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
#20
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
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