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    2702 results match your criteria Pediatrics Hypoglycemia

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    Poor Sleep Quality is Associated with Nocturnal Glycemic Variability and Fear of Hypoglycemia in Adults with Type 1 Diabetes.
    J Adv Nurs 2018 Jun 19. Epub 2018 Jun 19.
    University of Illinois at Chicago, College of Nursing, Department of Biobehavioral Health Science, Chicago, IL.
    Aims: To examine sleep quality and its associations with glycemic control, glycemic variability and fear of hypoglycemia in adults with type 1 diabetes.

    Background: Poor sleep quality has negative health consequences and is a frequent complaint among adults with type 1 diabetes. Sleep quality in adults with type 1 diabetes is likely affected by glucose levels as well as stressors associated with managing a chronic condition. Read More

    Congenital hyperinsulinism as the presenting feature of Kabuki syndrome: clinical and molecular characterization of 10 affected individuals.
    Genet Med 2018 Jun 15. Epub 2018 Jun 15.
    Department of Human Genetics, University of Chicago Genetic Services Laboratory, The University of Chicago, Chicago, Illinois, USA.
    Purpose: Describe the clinical and molecular findings of patients with Kabuki syndrome (KS) who present with hypoglycemia due to congenital hyperinsulinism (HI), and assess the incidence of KS in patients with HI.

    Methods: We documented the clinical features and molecular diagnoses of 10 infants with persistent HI and KS via a combination of sequencing and copy-number profiling methodologies. Subsequently, we retrospectively evaluated 100 infants with HI lacking a genetic diagnosis, for causative variants in KS genes. Read More

    Congenital Hyperinsulinism in Infants with Turner Syndrome: Possible Association with Monosomy X and KDM6A Haploinsufficiency.
    Horm Res Paediatr 2018 Jun 14:1-10. Epub 2018 Jun 14.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
    Background: Previous case reports have suggested a possible association of congenital hyperinsulinism with Turner syndrome.

    Objective: We examined the clinical and molecular features in girls with both congenital hyperinsulinism and Turner syndrome seen at The Children's Hospital of Philadelphia (CHOP) between 1974 and 2017.

    Methods: Records of girls with hyperinsulinism and Turner syndrome were reviewed. Read More

    Effect of 6 months of hybrid closed-loop insulin delivery in adults with type 1 diabetes: a randomised controlled trial protocol.
    BMJ Open 2018 Jun 9;8(6):e020274. Epub 2018 Jun 9.
    Department of Medicine, University of Melbourne, Melbourne, Victoria, Australia.
    Introduction: Manual determination of insulin dosing largely fails to optimise glucose control in type 1 diabetes. Automated insulin delivery via closed-loop systems has improved glucose control in short-term studies. The objective of the present study is to determine the effectiveness of 6 months' closed-loop compared with manually determined insulin dosing on time-in-target glucose range in adults with type 1 diabetes. Read More

    Mortality in children with classic congenital adrenal hyperplasia and 21-hydroxylase deficiency (CAH) in Germany.
    BMC Endocr Disord 2018 Jun 8;18(1):37. Epub 2018 Jun 8.
    Paediatric Endocrinology, Department Paediatrics, University Hospital of Bonn, Bonn, Germany.
    Background: Adrenal crises in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) are life-threatening and have the potential to death.

    Methods: A survey was performed among Paediatric Endocrinologists in Germany to report on deceased children with CAH. Our survey covered the whole of Germany. Read More

    Polymicrogyria in association with hypoglycemia points to mutation in the mTOR pathway.
    Eur J Med Genet 2018 Jun 5. Epub 2018 Jun 5.
    Department of Neurology, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia.
    We report a 16-month-old male with congenital megalencephaly, polymicrogyria and persistent hypoglycemia caused by a mosaic PIK3CA pathogenic variant. Hypoinsulinaemic, hypoketotic hypoglycaemia is a rare complication of pathogenic variants in the PI3K-AKT-mTOR pathway genes including AKT2, AKT3, CCND2, PIK3R2 and PIK3CA, and has been identified in a PIK3CA mutant mouse model. Our case highlights the importance of considering PI3K-AKT-mTOR pathway variants as a cause for megalencephaly and cortical malformation when the phenotype includes hypoglycaemia. Read More

    Effectiveness and safety of long-term treatment with sulfonylureas in patients with neonatal diabetes due to KCNJ11 mutations: an international cohort study.
    Lancet Diabetes Endocrinol 2018 Jun 4. Epub 2018 Jun 4.
    KG Jebsen Centre for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway; Department of Paediatrics and Adolescents, Haukeland University Hospital, Bergen, Norway.
    Background: KCNJ11 mutations cause permanent neonatal diabetes through pancreatic ATP-sensitive potassium channel activation. 90% of patients successfully transfer from insulin to oral sulfonylureas with excellent initial glycaemic control; however, whether this control is maintained in the long term is unclear. Sulfonylurea failure is seen in about 44% of people with type 2 diabetes after 5 years of treatment. Read More

    Assessment and management of hypoglycemia in children and adolescents with diabetes.
    Pediatr Diabetes 2018 Jun 5. Epub 2018 Jun 5.
    Division of Endocrinology, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA.
    The ISPAD Hypoglycemia guidelines have been harmonized with the International Hypoglycemia Study Group (IHSG). This article is protected by copyright. All rights reserved. Read More

    Protocol of a randomised controlled trial of real-time continuous glucose monitoring in neonatal intensive care 'REACT'.
    BMJ Open 2018 Jun 4;8(6):e020816. Epub 2018 Jun 4.
    Department of Paediatrics, University of Cambridge, Cambridge, UK.
    Introduction: Hyperglycaemia is common in the very preterm infant and has been associated with adverse outcomes. Preventing hyperglycaemia without increasing the risk of hypoglycaemia has proved challenging. The development of real-time continuous glucose monitors (CGM) to inform treatment decisions provides an opportunity to reduce this risk. Read More

    A rare cause of neonatal hypoglycemia in two siblings: TBX19 gene mutation.
    Hormones (Athens) 2018 May 3. Epub 2018 May 3.
    Department of Pediatric Endocrinology, Dicle University Faculty of Medicine, Sur, Diyarbakir, Turkey.
    Congenital isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) is a rarely seen disease characterized by low serum ACTH and cortisol levels accompanied by normal levels of the other anterior pituitary hormones. In these patients, severe hypoglycemia, convulsions, and prolonged cholestatic jaundice are expected findings in the neonatal period. In this paper, we present two siblings with TBX19 gene mutation. Read More

    A phase I window, dose escalating and safety trial of metformin in combination with induction chemotherapy in relapsed refractory acute lymphoblastic leukemia: Metformin with induction chemotherapy of vincristine, dexamethasone, PEG-asparaginase, and doxorubicin.
    Pediatr Blood Cancer 2018 Jun 1:e27224. Epub 2018 Jun 1.
    Johns Hopkins All Children's Hospital, St. Petersburg, FL.
    Background: Acute lymphoblastic leukemia (ALL) remains a major cause of death in children. AMP-activated protein kinase (AMPK) affects the unfolded protein response (UPR), leading to increased vulnerability to endoplasmic reticulum (ER) stress in ALL cells. In vitro, metformin causes ALL cell death via AMPK-mediated inhibition of the UPR. Read More

    Total Bile Acid Concentration in duodenal fluid Is a Useful Preoperative Screening Marker to Rule Out Biliary Atresia.
    J Pediatr Gastroenterol Nutr 2018 May 30. Epub 2018 May 30.
    Department of Pediatrics, Osaka University Graduate School of Medicine.
    Objectives: Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concentration in duodenal fluid. Read More

    Primum non nocere: earlier cessation of glucose monitoring is possible.
    Eur J Pediatr 2018 May 30. Epub 2018 May 30.
    Division of Neonatology, Department of Paediatrics, Radboudumc, Amalia Children's Hospital, Geert Grooteplein Zuid 10, 6525 GA, Nijmegen, the Netherlands.
    Newborns are at relatively high risk for developing hypoglycaemia in the first 24 h after birth. Well-known risk factors are prematurity, small for gestational age (SGA) or large for gestational age (LGA), and maternal pre-existent or gestational diabetes mellitus. Prolonged hypoglycaemia is associated with poor neurodevelopmental outcomes; hence, prevention through proper monitoring and treatment is important. Read More

    Safety of Oral Propranolol for Infantile Hemangioma.
    Pediatrics 2018 Jun;141(6)
    University of Rennes, Rennes, France.
    Objectives: The safety of oral propranolol for infantile hemangioma has not yet been studied at population level since the pediatric use marketing authorization was obtained in Europe.

    Methods: A survey of a nationwide, claim-based observational cohort of children <3 years old, with at least 1 delivery of oral propranolol between July 2014 and June 2016, was performed by using the database of the French National Health Insurance system. Standardized morbidity ratios (SMRs) were calculated by using, from the same database, a representative random sample of nonexposed subjects. Read More

    Decreasing NICU admissions of asymptomatic infants of women with pregestational diabetes mellitus improves breastfeeding initiation rates.
    J Neonatal Perinatal Med 2018 May 23. Epub 2018 May 23.
    Department of Pediatrics, The Ohio State University, Columbus, OH, USA.
    Background: Asymptomatic infants born to women with pregestational diabetes mellitus (PGDM) are usually admitted to the well baby nursery (WBN) while those who are symptomatic or in need of specialized care are admitted to the neonatal intensive care unit (NICU).

    Objective: To determine if changes in the NICU admission rate of asymptomatic infants born to women with PGDM during two different epochs affected breastfeeding (BF) initiation rates.

    Design/methods: Retrospective cohort investigation of 386 women with PGDM and their infants who delivered in 2008-11 (epoch 1) and 457 who delivered in 2013-16 (epoch 2) at a single institution. Read More

    A novel ZC4H2 gene mutation, K209N, in Japanese siblings with arthrogryposis multiplex congenita and intellectual disability: characterization of the K209N mutation and clinical findings.
    Brain Dev 2018 May 24. Epub 2018 May 24.
    Department of Pediatrics, Akita University Graduate School of Medicine, Akita, Akita, Japan. Electronic address:
    Objective: To reveal a molecular lesion in the ZC4H2 gene in a Japanese family with arthrogryposis multiplex congenita (AMC) and intellectual disability (ID), and to characterize clinical features of patients with ZC4H2 gene mutations through a literature review.

    Patients: The probands are male siblings. The elder brother is an 11-year-old boy who showed AMC and ID and frequent postprandial hypoglycemia since 3 years of age. Read More

    Role of continuous glucose monitoring in the management of glycogen storage disorders.
    J Inherit Metab Dis 2018 May 25. Epub 2018 May 25.
    Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, 905 South LaSalle Street, GSRB1, Durham, NC, 27710, USA.
    Management of liver glycogen storage diseases (GSDs) primarily involves maintaining normoglycemia through dietary modifications and regular glucose monitoring. Self-monitoring of blood glucose is typically done 3-6 times per day, and may not sufficiently capture periods of asymptomatic hypoglycemia, particularly during sleep. Continuous glucose monitoring systems (CGMS) provide 24-h continuous glucose data and have been used effectively in diabetes mellitus to monitor metabolic control and optimize treatment. Read More

    Diabetes symptoms predictors of health-related quality of life in adolescents and young adults with type 1 or type 2 diabetes.
    Qual Life Res 2018 May 21. Epub 2018 May 21.
    Cook Children's Medical Center, Fort Worth, TX, USA.
    Objectives: The objective was to investigate the patient-reported diabetes symptoms predictors of generic health-related quality of life (HRQOL) in adolescents and young adults (AYA) with type 1 or type 2 diabetes.

    Methods: The 15-item PedsQL™ 3.2 Diabetes Module Diabetes Symptoms Summary Score and PedsQL™ 4. Read More

    Body mass index standard deviation score and obesity in children with type 1 diabetes in the Nordic countries. HbA and other predictors of increasing BMISDS.
    Pediatr Diabetes 2018 May 21. Epub 2018 May 21.
    Department of Paediatrics, Herlev Hospital, University of Copenhagen, Copenhagen, Denmark.
    Background: Intensified insulin therapy may increase body weight and cause obesity. This study compared body mass index standard deviation score (BMISDS) and obesity rate in children with type 1 diabetes (T1D) in Denmark, Iceland, Norway and Sweden, and uncovered predictors for increasing BMISDS.

    Methods: Data registered in the Nordic national childhood diabetes databases during the period 2008-2012 on children below 15 years with T1D for more than 3 months were compiled, including information on gender, age, diabetes duration, hemoglobin A (HbA ), insulin dose, severe hypoglycemia (SH), treatment modality, height and weight. Read More

    Disruption of the Responsible Gene in a Phosphoglucomutase 1 Deficiency Patient by Homozygous Chromosomal Inversion.
    JIMD Rep 2018 May 12. Epub 2018 May 12.
    Division of Molecular Genetics, Institute for Comprehensive Medical Science, Fujita Health University, Toyoake, Japan.
    Phosphoglucomutase 1 (PGM1) deficiency is a recently defined disease characterized by glycogenosis and a congenital glycosylation disorder caused by recessive mutations in the PGM1 gene. We report a case of a 12-year-old boy with first-cousin parents who was diagnosed with a PGM1 deficiency due to significantly decreased PGM1 activity in his muscle. However, Sanger sequencing revealed no pathogenic mutation in the PGM1 gene in this patient. Read More

    Genetic characteristics of patients with congenital hyperinsulinism.
    Curr Opin Pediatr 2018 May 9. Epub 2018 May 9.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia.
    Purpose Of Review: Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in infants and children. Early and appropriate recognition and treatment of hypoglycemia is vital to minimize neurocognitive impairment.

    Recent Findings: There are at least 11 known monogenic forms of hyperinsulinism and several associated syndromes. Read More

    Effect of lactate administration on brain lactate levels during hypoglycemia in patients with type 1 diabetes.
    J Cereb Blood Flow Metab 2018 Jan 1:271678X18775884. Epub 2018 Jan 1.
    2 Department of Internal Medicine, Radboud university medical center, Nijmegen, The Netherlands.
    Administration of lactate during hypoglycemia suppresses symptoms and counterregulatory responses, as seen in patients with type 1 diabetes and impaired awareness of hypoglycemia (IAH), presumably because lactate can substitute for glucose as a brain fuel. Here, we examined whether lactate administration, in a dose sufficient to impair awareness of hypoglycemia, affects brain lactate levels in patients with normal awareness of hypoglycemia (NAH). Patients with NAH ( n = 6) underwent two euglycemic-hypoglycemic clamps (2. Read More

    The glycaemic benefits of a very-low-carbohydrate ketogenic diet in adults with Type 1 diabetes mellitus may be opposed by increased hypoglycaemia risk and dyslipidaemia.
    Diabet Med 2018 May 8. Epub 2018 May 8.
    School of Human Sciences, University of Western Australia, Perth, Australia.
    Aims: To investigate whether very-low-carbohydrate high-fat diets, typical of ketogenic diets, can improve glycaemic control without causing any ill health effects in adults with Type 1 diabetes.

    Methods: In this observational study, 11 adults with Type 1 diabetes (seven men, four women, mean ± sd age 36.1± 6. Read More

    Management of Type 1 Diabetes With a Very Low-Carbohydrate Diet.
    Pediatrics 2018 Jun 7;141(6). Epub 2018 May 7.
    Division of Endocrinology, and
    Objectives: To evaluate glycemic control among children and adults with type 1 diabetes mellitus (T1DM) who consume a very low-carbohydrate diet (VLCD).

    Methods: We conducted an online survey of an international social media group for people with T1DM who follow a VLCD. Respondents included adults and parents of children with T1DM. Read More

    Cerebral glucose deficiency versus oxygen deficiency in neonatal encephalopathy.
    J Neonatal Perinatal Med 2018 Apr 24. Epub 2018 Apr 24.
    Department of Pediatrics, University of California, San Francisco, CA, USA.
    Hypoxic-ischemic encephalopathy (HIE) in newborn infants is generally considered to result from decreased arterial oxygen content or cerebral blood flow. Cerebral injury similar to that of HIE has been noted with hypoglycemia. Studies in fetal lambs have shown that ventilation with 3% oxygen did not change cerebral blood flow, but ventilation with 100% oxygen resulted in marked reduction in cerebral blood flow, glucose delivery and glucose consumption. Read More

    Short-term metabolic control and sleep in children and adolescents with type 1 diabetes mellitus.
    J Diabetes Complications 2018 Jun 22;32(6):580-585. Epub 2018 Mar 22.
    Department of Pediatrics, Comenius University Bratislava, Jessenius Faculty of Medicine and University Hospital, 036 01 Martin, Slovakia. Electronic address:
    Aims: The aim of this study was to examine sleep in T1D children and in healthy controls by polysomnographic (PSG) examination and to determine the influence of short-term metabolic compensation on sleep quality and sleep disordered breathing (SDB).

    Methods: The prospective cross-sectional study included 44 T1D subjects and 60 healthy controls, aged 10-19 years. Subjects underwent anthropometric measurements, laboratory testing and standard overnight in-laboratory video polysomnography with continuous glucose monitoring (CGM). Read More

    Lower Glucose Target Is Associated With Improved 30-Day Mortality in Cardiac and Cardiothoracic Patients.
    Chest 2018 Apr 26. Epub 2018 Apr 26.
    Division of Pulmonary and Critical Care, Intermountain Medical Center, Murray, UT; Division of Pulmonary and Critical Care Medicine, University of Utah, Salt Lake City, UT.
    Background: Practice guidelines recommend against intensive insulin therapy in patients who are critically ill based on trials that had high rates of severe hypoglycemia. Intermountain Healthcare uses a computerized IV insulin protocol that allows choice of blood glucose (BG) targets (80-110 vs 90-140 mg/dL) and has low rates of severe hypoglycemia. We sought to study the effects of BG target on mortality in adult patients in cardiac ICUs that have very low rates of severe hypoglycemia. Read More

    Vanishing 17-Hydroxyprogesterone Concentrations in 21-Hydroxylase Deficiency.
    Horm Res Paediatr 2018 Apr 25:1-7. Epub 2018 Apr 25.
    Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, University Hospital of Schleswig-Holstein, UKSH, Campus Kiel/Christian Albrecht University of Kiel, CAU, Kiel, Germany.
    We present a boy with a genetically proven congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. While massively elevated 17-hydroxyprogesterone (17-OHP) concentrations after birth led to the diagnosis, 17-OHP concentrations became immeasurable starting with the second year of life even though the dose of hydrocortisone was continuously decreased to ∼7 mg/m2/day. Furthermore, 17-OHP levels were immeasurable during the ACTH test and after withdrawing hydrocortisone medication. Read More

    Continuous Glucose Monitoring in Resource-Constrained Settings for Hypoglycaemia Detection: Looking at the Problem from the Other Side of the Coin.
    Biosensors (Basel) 2018 Apr 25;8(2). Epub 2018 Apr 25.
    Centro de Investigação em Saúde de Manhiça (CISM), CP1929 Maputo, Mozambique.
    The appearance, over a decade ago, of continuous glucose monitoring (CGM) devices has triggered a patient-centred revolution in the control and management of diabetes mellitus and other metabolic conditions, improving the patient’s glycaemic control and quality of life. Such devices, the use of which remains typically restricted to high-income countries on account of their elevated costs, at present show very limited implantation in resource-constrained settings, where many other urgent health priorities beyond diabetes prevention and management still need to be resolved. In this commentary, we argue that such devices could have an additional utility in low-income settings, whereby they could be selectively used among severely ill children admitted to hospital for closer monitoring of paediatric hypoglycaemia, a life-threatening condition often complicating severe cases of malaria, malnutrition, and other common paediatric conditions. Read More

    Transition to adult diabetes care in Germany-High risk for acute complications and declining metabolic control during the transition phase.
    Pediatr Diabetes 2018 Apr 25. Epub 2018 Apr 25.
    Institute of Epidemiology and Medical Biometry, ZIBMT, University of Ulm, Ulm, Germany.
    Objective: There is evidence that transition from pediatric to adult health care is frequently associated with deterioration of health in youths with type 1 diabetes (T1D). The aim of this study was to compare metabolic control, acute complications and microvascular complications in adolescents and young adults before and after transfer to an adult treatment center with respect to the time between first visit in the adult center and last visit in pediatric treatment.

    Methods: All data were collected during routine care and retrieved from the German/Austrian DPV database. Read More

    Forebrain cellular bioenergetics in neonatal mice.
    J Neonatal Perinatal Med 2018 ;11(1):79-86
    Department of Pediatrics, College of Medicine and Health Sciences, United Arab Emirates University, United Arab Emirates.
    Background: Hypoglycemia occurs frequently in the neonate and may result in neurologic dysfunction. Its impact on the kinetics of cellular respiration and bioenergetics in the neonatal brain remains to be explored.

    Aims: Develop murine model to investigate the effects of hypoglycemia on neonatal brain bioenergetics. Read More

    Young people's experiences of managing Type 1 diabetes at university: a national study of UK university students.
    Diabet Med 2018 Apr 23. Epub 2018 Apr 23.
    Diabetes and Endocrinology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
    Aim: Little is known about the challenges of transitioning from school to university for young people with Type 1 diabetes. In a national survey, we investigated the impact of entering and attending university on diabetes self-care in students with Type 1 diabetes in all UK universities.

    Methods: Some 1865 current UK university students aged 18-24 years with Type 1 diabetes, were invited to complete a structured questionnaire. Read More

    Giant umbilical cord and hypoglycemia in an infant with Proteus syndrome.
    Am J Med Genet A 2018 May;176(5):1222-1224
    Department of Pediatrics, University of Yamanashi, Yamanashi, Japan.
    Proteus syndrome (PS) is characterized by the progressive, segmental, or patchy overgrowth of the skin, and other tissues. This is the first case report of recurrent severe insulin-independent hypoglycemia in an infant with PS. Somatic p. Read More

    Stigma and Its Association With Glycemic Control and Hypoglycemia in Adolescents and Young Adults With Type 1 Diabetes: Cross-Sectional Study.
    J Med Internet Res 2018 Apr 20;20(4):e151. Epub 2018 Apr 20.
    Centre for Outcomes Research and Evaluation, Research Institute of the McGill University Health Centre, Department of Medicine, McGill University, Montreal, QC, Canada.
    Background: Qualitative studies in type 1 diabetes indicate that visibility of diabetes supplies, self-care, and hypoglycemia symptoms are associated with stigma and suboptimal management. This may be particularly salient in youth who face concurrent challenges such as establishing autonomy and making vocational choices.

    Objective: The aim of the study was to estimate stigma prevalence in youth (aged 14-24 years) with type 1 diabetes and its associations with glycemic control. Read More

    Longitudinal assessment of hippocampus structure in children with type 1 diabetes.
    Pediatr Diabetes 2018 Apr 19. Epub 2018 Apr 19.
    Department of Pediatrics, Washington University in St. Louis and the St. Louis Children's Hospital, St. Louis, Missouri.
    The extant literature finds that children with type 1 diabetes mellitus (T1D) experience mild cognitive alterations compared to healthy age-matched controls. The neural basis of these cognitive differences is unclear but may relate in part to the effects of dysglycemia on the developing brain. We investigated longitudinal changes in hippocampus volume in young children with early-onset T1D. Read More

    Attention deficit/hyperactivity disorder as an associated feature in OCTN2 deficiency with novel deletion (p.T440-Y449).
    Clin Case Rep 2018 Apr 9;6(4):585-591. Epub 2018 Feb 9.
    Division of NeurologyDepartment of Pediatrics, and Genetics and Genome Biology ProgramThe Research InstituteThe Hospital for Sick ChildrenUniversity of TorontoTorontoOntarioM5G 1X8Canada.
    This boy presented with ADHD at 3 years and at 8 years was hyperactive with no documented hypoglycemia and had myopathy, cardiomyopathy, and very low serum carnitine. L-carnitine improved his exercise intolerance, cardiomyopathy, and behavior. Analysis of revealed a premature stop codon (p. Read More

    Hypocarnitinemia Observed in an Infant Treated with Short-Term Administration of Antibiotic Containing Pivalic Acid.
    Tohoku J Exp Med 2018 04;244(4):279-282
    Department of Pediatrics and Child Health, Nihon University School of Medicine.
    Carnitine is a water-soluble amino acid derivative required for β-oxidation of long-chain fatty acids. In carnitine cycle abnormalities and low carnitine states, fatty acid β-oxidation is inhibited during fasting, resulting in hypoglycemia. Pivalic acid is a substance used in prodrugs to increase absorption of parent drugs, and antibiotics containing pivalic acid are frequently used as wide spectrum antibiotics for pediatric patients in Japan. Read More

    Efficacy and Safety of Rapid-Acting Insulin Analogs in Special Populations with Type 1 Diabetes or Gestational Diabetes: Systematic Review and Meta-Analysis.
    Diabetes Ther 2018 Jun 5;9(3):891-917. Epub 2018 Apr 5.
    Diabetes, Endocrinology and Metabolism, Division of Health Sciences, Warwick Medical School, University of Warwick, Coventry, CV4 7AL, UK.
    Introduction: To assess the efficacy and safety of three available rapid-acting insulin analogs (insulins lispro, aspart and glulisine, respectively) in pregnant women, children/adolescents and people using continuous subcutaneous insulin infusion (CSII) with type 1 diabetes.

    Methods: PubMed, EMBASE and Cochrane Reviews were searched electronically, and their bibliographies examined to identify suitable studies for review and inclusion in a meta-analysis. Eligible studies were randomized controlled trials that reported data on relevant clinical outcomes. Read More

    Blue Diaper Syndrome and Mutations.
    Pediatrics 2018 Apr;141(Suppl 5):S501-S505
    Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.
    Blue diaper syndrome (BDS) (Online Mendelian Inheritance in Man number 211000) is an extremely rare disorder that was first described in 1964. The characteristic finding is a bluish discoloration of urine spots in the diapers of affected infants. Additional clinical features of the first described patients included diarrhea, inadequate weight gain, hypercalcemia, and nephrocalcinosis. Read More

    Mental health visits and low socio-economic status in adolescence are associated with complications of Type 1 diabetes in early adulthood: a population-based cohort study.
    Diabet Med 2018 Jul 19;35(7):920-928. Epub 2018 Apr 19.
    Institute for Clinical Evaluative Sciences, Toronto, Ontario, Canada.
    Aim: To determine the association of mental health visits and socio-economic status in late adolescence with the risk of mortality and acute and chronic diabetes complications in early adulthood.

    Methods: We conducted a population-based cohort study of individuals in Ontario, Canada, who had their 20th birthday between January 1999 and March 2015 and a diagnosis of diabetes prior to their 15 birthday, using linked administrative databases (n=8491). The main outcome was death; other outcomes were hypoglycaemia or hyperglycaemia-related hospitalizations and emergency department visits and chronic diabetes complications (dialysis, ophthalmological and macrovascular complications). Read More

    Agraphia with reversible splenial corpus callosum lesion caused by hypoglycemia.
    Brain Dev 2018 Mar 29. Epub 2018 Mar 29.
    Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan.
    Background: Neurological manifestations caused by hypoglycemia range from reversible focal deficits and transient encephalopathy to irreversible coma or death. Recently, high signal intensity lesions in the splenium of the corpus callosum on diffusion-weighted magnetic resonance imaging were reported in adults experiencing hypoglycemia. However, patients presenting with agraphia are rare. Read More

    Prophylactic Dextrose Gel Does Not Prevent Neonatal Hypoglycemia: A Quasi-Experimental Pilot Study.
    J Pediatr 2018 Mar 28. Epub 2018 Mar 28.
    Department of Pediatrics, Section of Neonatology, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX.
    Objective: To test the hypothesis that prophylactic dextrose gel administered to newborn infants at risk for hypoglycemia will increase the initial blood glucose concentration after the first feeding and decrease neonatal intensive care unit (NICU) admissions for treatment of asymptomatic neonatal hypoglycemia compared with feedings alone.

    Study Design: This quasi-experimental study allocated asymptomatic at-risk newborn infants (late preterm, birth weight <2500 or >4000 g, and infants of mothers with diabetes) to receive prophylactic dextrose gel (Insta-Glucose; Valeant Pharmaceuticals North America LLC, Bridgewater, New Jersey); other at-risk infants formed the control group. After the initial feeding, the prophylactic group received dextrose gel (0. Read More

    Neonatal hyperinsulinemic hypoglycemia in a patient with 9p deletion syndrome.
    Eur J Med Genet 2018 Mar 27. Epub 2018 Mar 27.
    Department of Pediatric Dentistry and Clinical Genetics, School of Dentistry, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark; 3D Craniofacial Image Research Laboratory, School of Dentistry, Copenhagen University Hospital Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
    We report the clinical and neuroradiological findings in a young boy harboring the 9p deletion syndrome including the novel findings of thalamic infarction and germinal matrix haemorrhage and neonatal hyperinsulinemic hypoglycemia. Both the hypoglycemic events and the ventriculomegaly found in this patient have previously only been reported in two patients, while the thalamic infarction and germinal matrix haemorrhage are novel features. Read More

    Continuous Flash Glucose Monitoring in children with Congenital Hyperinsulinism; first report on accuracy and patient experience.
    Int J Pediatr Endocrinol 2018 27;2018. Epub 2018 Mar 27.
    2Department of Paediatric Endocrinology, Alder Hey Children's Hospital, L14 5AB, Liverpool, UK.
    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. Read More

    A unique allosteric insulin receptor monoclonal antibody that prevents hypoglycemia in the SUR-1 mouse model of KATP hyperinsulinism.
    MAbs 2018 Mar 28:1-7. Epub 2018 Mar 28.
    a Division of Endocrinology, Department of Pediatrics , The Children's Hospital of Philadelphia , Philadelphia , Pennsylvania.
    Loss-of-function mutations of the ß-cell ATP-sensitive potassium channels (K) cause the most common and severe form of congenital hyperinsulinism (KHI), a disorder of ß-cell function characterized by severe hypoglycemia. Children with KHI 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 KHI. Read More

    Metabolic control of type 1 diabetes in youth with autism spectrum disorder: A multicenter Diabetes-Patienten-Verlaufsdokumentation analysis based on 61 749 patients up to 20 years of age.
    Pediatr Diabetes 2018 Mar 26. Epub 2018 Mar 26.
    University of Ulm, Institute of Epidemiology and Medical Biometry, Central Institute for Biomedical Technology, Ulm, Germany.
    Background: A paucity of reports in the literature exists concerning the co-existence between autism spectrum disorder (ASD) and type 1 diabetes (T1D).

    Objective: To compare clinical characteristics, diabetes management and metabolic control in youth with T1D and ASD (T1D-ASD) with youth without ASD (T1D-non ASD).

    Methods: Using the German/Austrian diabetes patient follow-up registry, this study analyzed aggregated data from the last available year of observation for each patient with T1D, ages 1-20 with consistent data on insulin regimen and glycated hemoglobin (A1C), between January, 2005 and March, 2017. Read More

    Continuous glucose monitoring abnormalities in cystic fibrosis youth correlate with pulmonary function decline.
    J Cyst Fibros 2018 Mar 23. Epub 2018 Mar 23.
    Department of Pediatrics, Division of Pediatric Endocrinology, Children's Hospital Colorado and University of Colorado Anschutz Medical Campus, Aurora, CO 80045, United States.
    Background: To characterize glucose patterns with continuous glucose monitoring (CGM) in cystic fibrosis (CF) and assess relationships between CGM and clinical outcomes.

    Methods: 110 CF youth and healthy controls (HC), 10-18 years, wore CGM up to 7 days. Correlations between CGM and lung function and BMI z-score change over the prior year were determined. Read More

    Glucose Gel in Infants at Risk for Transitional Neonatal Hypoglycemia.
    Am J Perinatol 2018 Mar 26. Epub 2018 Mar 26.
    Division of Neonatology, Department of Pediatrics, University of Florida College of Medicine-Jacksonville, Jacksonville, Florida.
    Objective:  To evaluate whether glucose gel as a supplement to feedings in infants admitted to the newborn nursery at risk for neonatal hypoglycemia (NH) reduces the frequency of transfer to a higher level of care for intravenous dextrose treatment.

    Study Design:  We revised our newborn nursery protocol for management of infants at risk for NH to include use of 40% glucose gel (200 mg/kg). Study population included late preterm, small and large for gestational age infants, and infants of diabetic mothers. Read More

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