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    Hvidoere Smiley Faces: International diabetes quality of life assessment tool for young children.
    Pediatr Diabetes 2017 Nov 22. Epub 2017 Nov 22.
    Clinique Pediatrique/CHL, Diabetes & Endocrine Care CP, Luxembourg.
    Background: Few diabetes-specific quality of life (QOL) tools are available for young children.

    Objectives: To design and evaluate, a new age-specific QOL questionnaire and its associations with treatment regimens and metabolic control.

    Methods: Clinical, demographic data and centrally analyzed HbA1c were collected on 1133 children <11 years (girls 48%; mean ± SD age 8. Read More

    International Consensus on Use of Continuous Glucose Monitoring.
    Diabetes Care 2017 Dec;40(12):1631-1640
    The Myrtle and Henry Hirsch National Center for Childhood Diabetes, The Jesse and Sara Lea Shafer Institute of Endocrinology and Diabetes, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
    Measurement of glycated hemoglobin (HbA1c) has been the traditional method for assessing glycemic control. However, it does not reflect intra- and interday glycemic excursions that may lead to acute events (such as hypoglycemia) or postprandial hyperglycemia, which have been linked to both microvascular and macrovascular complications. Continuous glucose monitoring (CGM), either from real-time use (rtCGM) or intermittently viewed (iCGM), addresses many of the limitations inherent in HbA1c testing and self-monitoring of blood glucose. Read More

    Formal Neurocognitive Testing in 60 Patients with Congenital Hyperinsulinism.
    Horm Res Paediatr 2017 Nov 17. Epub 2017 Nov 17.
    Department of Pediatrics, Otto von Guericke University Magdeburg, Magdeburg, Germany.
    Background: Congenital hyperinsulinism (CHI) is hallmarked by persistent hypoketotic hypoglycemia in infancy. In the majority of all patients, CHI is caused by mutations in the KATP channel genes ABCC8 and KCNJ11, but other genes in the insulin-regulatory pathway have also been described. Repeated episodes of hypoglycemia include an increased risk of seizures and intellectual disability. Read More

    A fatal case of mitochondrial DNA depletion syndrome with novel compound heterozygous variants in the deoxyguanosine kinase gene.
    Oncotarget 2017 Oct 15;8(48):84309-84319. Epub 2017 Sep 15.
    The Center for Pediatric Liver Disease, Children's Hospital of Fudan University, Shanghai 201102, China.
    The deoxyguanosine kinase (DGUOK) gene controls mitochondrial DNA (mtDNA) maintenance, and variation in the gene can alter or abolish the anabolism of mitochondrial deoxyribonucleotides. A Chinese female infant, whose symptoms included weight stagnation, jaundice, hypoglycemia, coagulation disorders, abnormal liver function, and multiple abnormal signals in the brain, died at about 10 months old. Genetic testing revealed a compound heterozygote of alleles c. Read More

    Intrapartum glycaemic control and neonatal hypoglycaemia in pregnancies complicated by diabetes mellitus: a systematic review.
    Diabet Med 2017 Nov 8. Epub 2017 Nov 8.
    Departments of Obstetrics and Gynecology, University of Calgary, Calgary, Alberta, Canada.
    Aims: To examine whether, in neonates of mothers with Type 1, Type 2 and gestational diabetes mellitus, in-target intrapartum glycaemic control was associated with a lower risk of neonatal hypoglycaemia compared with out-of-target glycaemic control.

    Methods: We searched PubMed and EMBASE for all available publications, regardless of year, based on a published protocol (PROSPERO CRD42016052439). Studies were excluded if they did not report original data or were animal studies. Read More

    18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT scans as diagnostic tools in focal congenital hyperinsulinism: a blinded evaluation.
    Eur J Nucl Med Mol Imaging 2017 Nov 8. Epub 2017 Nov 8.
    Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark.
    Purpose: Focal congenital hyperinsulinism (CHI) is curable by surgery, which is why identification of the focal lesion is crucial. We aimed to determine the use of 18F-fluoro-dihydroxyphenylalanine (18F-DOPA) PET/CT vs. 68Ga-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic-acid-1-Nal3-octreotide (68Ga-DOTANOC) PET/CT as diagnostic tools in focal CHI. Read More

    Occult Massive Visceral Fat Necrosis Following Therapeutic Hypothermia for Neonatal Encephalopathy.
    Pediatr Dev Pathol 2017 Jan 1:1093526617737881. Epub 2017 Jan 1.
    1 Department of Pathology, 22209 Women and Infants Hospital, Providence, Rhode Island.
    Therapeutic hypothermia (head or whole-body cooling) improves survival and neurodevelopmental outcome in term newborns with moderate-to-severe encephalopathy. Hypothermia treatment is well tolerated; the most common side effect is thrombocytopenia. In about 1% of infants, focal subcutaneous fat necrosis has been reported. Read More

    Clues for early detection of autoimmune Addison's disease - myths and realities.
    J Intern Med 2017 Nov 3. Epub 2017 Nov 3.
    Department of Clinical Medicine, University of Bergen, Bergen, Norway.
    Background: Early detection of autoimmune Addison's disease (AAD) is important as delay in diagnosis may result in a life-threatening adrenal crisis and death. The classical clinical picture of untreated AAD is well-described, but methodical investigations are scarce.

    Objective: Perform a retrospective audit of patient records with the aim of identifying biochemical markers for early diagnosis of AAD. Read More

    A New Familial Form of a Late-onset, Persistent Hyperinsulinemic Hypoglycemia of Infancy caused by a Novel Mutation in KCNJ11.
    Channels (Austin) 2017 Oct 31. Epub 2017 Oct 31.
    a Institute of Biomedical Sciences , Academia Sinica, Taipei , Taiwan.
    The ATP-sensitive potassium channel (KATP) functions as a metabo-electric transducer in regulating insulin secretion from pancreatic β-cells. The pancreatic KATP channel is composed of a pore-forming inwardly-rectifying potassium channel, Kir6.2, and a regulatory subunit, sulphonylurea receptor 1 (SUR1). Read More

    Potential Clinical Benefits of a Two-bag System for Fluid Management in Pediatric Intensive Care Unit Patients with Diabetic Ketoacidosis.
    Pediatr Endocrinol Diabetes Metab 2017 ;23(1):6-13
    Nassau University Medical Center, Department of Pediatrics, East Meadow, NY, USA.
    Introduction: Diabetic ketoacidosis (DKA) is a life-threatening complication of diabetes mellitus (DM) that requires appropriate treatment with insulin and intravenous fluids. Both one-bag and two-bag systems of fluid management are used to treat pediatric diabetic ketoacidosis.

    Aim Of The Study: We compare the one-bag and two-bag systems of fluid managementwith regard to incidence of hypoglycemia, serum bicarbonate correction, pH correction and discharge from the pediatric intensive care unit (PICU). Read More

    Reducing Time to Antibiotics in Children With Intestinal Failure, Central Venous Line, and Fever.
    Pediatrics 2017 Nov;140(5)
    Division of Emergency Medicine and.
    Background: Children with intestinal failure (IF) on parenteral nutrition (PN) are at high risk for bacteremia, and delays in antibiotic administration have been associated with increased morbidity and mortality. We designed an emergency department (ED) quality improvement (QI) initiative to reduce time to administration of intravenous antibiotics in febrile children with IF on PN.

    Methods: Our aim was to decrease the mean time for febrile children with IF on PN to receive intravenous antibiotics by 50% to <60 minutes over a 12-month period. Read More

    A randomized controlled trial of one bag vs. two bag system of fluid delivery in children with diabetic ketoacidosis: Experience from a developing country.
    J Crit Care 2017 Sep 22. Epub 2017 Sep 22.
    Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India.
    Purpose: To compare one vs. two bag system with respect to blood glucose variability (BGV), time for resolution of acidosis and incidence of hypoglycemia, hypokalemia, and cerebral edema in children with diabetic ketoacidosis (DKA).

    Material And Methods: In an open labelled randomized controlled trial, thirty consecutive patients ≤12years with DKA were randomized to either one (n=15) or two bag (n=15) system of intravenous fluid delivery. Read More

    Sustained endocrine profiles of a girl with WAGR syndrome.
    BMC Med Genet 2017 Oct 23;18(1):117. Epub 2017 Oct 23.
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, 812-8582, Japan.
    Background: Wilms tumor, aniridia, genitourinary anomalies and mental retardation (WAGR) syndrome is a rare genetic disorder caused by heterozygous deletions of WT1 and PAX6 at chromosome 11p13. Deletion of BDNF is known eto be associated with hyperphagia and obesity in both humans and animal models; however, neuroendocrine and epigenetic profiles of individuals with WAGR syndrome remain to be determined.

    Case Presentation: We report a 5-year-old girl with the typical phenotype of WAGR syndrome. Read More

    Continuous glucose monitoring in neonates: a review.
    Matern Health Neonatol Perinatol 2017 17;3:18. Epub 2017 Oct 17.
    Liggins Institute, University of Auckland, Private Bag 92019, Victoria St West, Auckland, 1142 New Zealand.
    Continuous glucose monitoring (CGM) is well established in the management of diabetes mellitus, but its role in neonatal glycaemic control is less clear. CGM has provided important insights about neonatal glucose metabolism, and there is increasing interest in its clinical use, particularly in preterm neonates and in those in whom glucose control is difficult. Neonatal glucose instability, including hypoglycaemia and hyperglycaemia, has been associated with poorer neurodevelopment, and CGM offers the possibility of adjusting treatment in real time to account for individual metabolic requirements while reducing the number of blood tests required, potentially improving long-term outcomes. Read More

    Association of Insulin Pump Therapy vs Insulin Injection Therapy With Severe Hypoglycemia, Ketoacidosis, and Glycemic Control Among Children, Adolescents, and Young Adults With Type 1 Diabetes.
    JAMA 2017 10;318(14):1358-1366
    Institute of Epidemiology and Medical Biometry, ZIBMT, University of Ulm, Ulm, Germany.
    Importance: Insulin pump therapy may improve metabolic control in young patients with type 1 diabetes, but the association with short-term diabetes complications is unclear.

    Objective: To determine whether rates of severe hypoglycemia and diabetic ketoacidosis are lower with insulin pump therapy compared with insulin injection therapy in children, adolescents, and young adults with type 1 diabetes.

    Design, Setting, And Participants: Population-based cohort study conducted between January 2011 and December 2015 in 446 diabetes centers participating in the Diabetes Prospective Follow-up Initiative in Germany, Austria, and Luxembourg. Read More

    Nutrient sensing in pancreatic islets: lessons from congenital hyperinsulinism and monogenic diabetes.
    Ann N Y Acad Sci 2017 Oct 16. Epub 2017 Oct 16.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
    Pancreatic beta cells sense changes in nutrients during the cycles of fasting and feeding and release insulin accordingly to maintain glucose homeostasis. Abnormal beta cell nutrient sensing resulting from gene mutations leads to hypoglycemia or diabetes. Glucokinase (GCK) plays a key role in beta cell glucose sensing. Read More

    Effect of Metformin on vascular function in children with type 1 diabetes: A 12 month randomized controlled trial.
    J Clin Endocrinol Metab 2017 Oct 9. Epub 2017 Oct 9.
    Discipline of Paediatrics, Robinson Research Institute, University of Adelaide, Australia.
    Context: Children with type 1 diabetes have vascular dysfunction preceding atherosclerosis. Early interventions are needed to reduce cardiovascular disease.

    Objective: To evaluate the effect of metformin on vascular function in type 1 diabetes children. Read More

    The value of real-time continuous glucose monitoring in premature infants of diabetic mothers.
    PLoS One 2017 16;12(10):e0186486. Epub 2017 Oct 16.
    Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan.
    To determine the feasibility of using a real-time continuous glucose monitoring system (RTGMS) in intensive care units, our study focus on preterm infants with diabetic mothers owing to their high risk of blood sugar abnormalities. Thirty preterm babies (M = 15 and F = 15; ≤ 36 week gestation age) were studied from within 72 hours of delivery. These babies were admitted to the newborn intensive care and were further categorized into groups based on whether their mothers with or without diabetic mellitus. Read More

    Clinical characteristics of septo-optic dysplasia accompanied by congenital central hypothyroidism in Japan.
    Clin Pediatr Endocrinol 2017 28;26(4):207-213. Epub 2017 Sep 28.
    Department of Pediatrics, Jichi Children's Medical Center Tochigi, Tochigi, Japan.
    Septo-optic dysplasia (SOD) is a congenital anomaly in which agenesis of the septum pellucidum and optic nerve hypoplasia are accompanied by hypopituitarism. Typically, the symptoms develop in 3 organs, the brain, eyes, and pituitary, and approximately one third of the patients present with all of the three cardinal features. The diagnostic criteria for SOD were established in Japan in 2015. Read More

    Compound heterozygosity for two GHR missense mutations in a patient affected by Laron Syndrome: a case report.
    Ital J Pediatr 2017 Oct 12;43(1):94. Epub 2017 Oct 12.
    Division of Pediatrics, Department of Health Sciences, University of Piemonte Orientale, Novara, Italy.
    Background: Mutations localized in the Growth Hormone Receptor (GHR) gene are often associated with the pathogenesis of Laron Syndrome, an autosomal recessive hereditary disorder characterized by severe growth retardation. Biochemically, patients present normal to high circulating GH levels, in presence of very low or undetectable IGF-I levels, which do not rise after rhGH treatment.

    Case Presentation: We describe the case of a 3. Read More

    Prophylactic intravenous calcium therapy for exchange blood transfusion in the newborn.
    Cochrane Database Syst Rev 2017 10 12;10:CD011048. Epub 2017 Oct 12.
    Department of Paediatrics (Neonatal Unit), Obafemi Awolowo College of Health Sciences, Olabisi Onabanjo University, Sagamu, Ogun State, Nigeria, 121001NG.
    Background: Exchange blood transfusion (EBT) is a form of whole blood transfusion in which the total blood volume is replaced within a few hours. In perinatal and neonatal medicine, EBT is most often used in the management of severe anaemia or severe hyperbilirubinaemia in the first week of life. Hypocalcaemia, one of the common morbidities associated with EBT, is thought to arise from the chelating effects of the citrate commonly used as an anticoagulant in the donor's blood. Read More

    Early neonatal hypoglycemia: incidence of and risk factors. A cohort study using universal point of care screening.
    J Matern Fetal Neonatal Med 2017 Oct 26:1-7. Epub 2017 Oct 26.
    b Hebrew University of Jerusalem , Israel.
    Objective: The objective of this study is to determine the true incidence of early neonatal hypoglycemia and to confirm potential risk factors.

    Study Design: The study was conducted at tertiary Medical Center in Israel, between June and September 2014. First blood glucose concentrations of all infants admitted to the nursery were measured using a "point of care" analyzer (Accu-Chek). Read More

    Dissociation Between Hormonal Counterregulatory Responses and Cerebral Glucose Metabolism During Hypoglycemia.
    Diabetes 2017 Dec 2;66(12):2964-2972. Epub 2017 Oct 2.
    Department of Pediatrics, Washington University School of Medicine in St. Louis, St. Louis, MO
    Hypoglycemia is the most common complication of diabetes, causing morbidity and death. Recurrent hypoglycemia alters the cascade of physiological and behavioral responses that maintain euglycemia. The extent to which these responses are normally triggered by decreased whole-brain cerebral glucose metabolism (CMRglc) has not been resolved by previous studies. Read More

    Type 1 diabetes during adolescence: International comparison between Germany, Austria, and Sweden.
    Pediatr Diabetes 2017 Sep 22. Epub 2017 Sep 22.
    Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.
    Objectives: By using pediatric diabetes quality registries in Austria, Germany, and Sweden treatment of type 1 diabetes and the outcome of care during the vulnerable adolescence period were compared.

    Methods: Data in DPV, broadly used in Austria and Germany, and Swediabkids used in Sweden, from clinical visits in the year 2013 on 14 383 patients aged 11 to 16 years regarding hemoglobin A1c (HbA1c), insulin regimen, body mass index (BMI)-SD score (SDS), blood pressure, hypoglycemia, ketoacidosis, and smoking habits were analyzed.

    Results: Patients in Sweden had fewer clinical visits per year (P < . Read More

    Effect of Exercise-Induced Lactate Elevation on Brain Lactate Levels During Hypoglycemia in Patients With Type 1 Diabetes and Impaired Awareness of Hypoglycemia.
    Diabetes 2017 Dec 21;66(12):3105-3110. Epub 2017 Sep 21.
    Department of Radiology and Nuclear Medicine, Radboud University Medical Center, Nijmegen, the Netherlands.
    Since altered brain lactate handling has been implicated in the development of impaired awareness of hypoglycemia (IAH) in type 1 diabetes, the capacity to transport lactate into the brain during hypoglycemia may be relevant in its pathogenesis. High-intensity interval training (HIIT) increases plasma lactate levels. We compared the effect of HIIT-induced hyperlacticacidemia on brain lactate during hypoglycemia between 1) patients with type 1 diabetes and IAH, 2) patients with type 1 diabetes and normal awareness of hypoglycemia, and 3) healthy participants without diabetes (n = 6 per group). Read More

    Continuous longitudinal infusion of rhIGF-1/rhIGFBP-3 in extremely preterm infants: Evaluation of feasibility in a phase II study.
    Growth Horm IGF Res 2017 Oct 31;36:44-51. Epub 2017 Aug 31.
    Department of Neonatology, CLINTEC, Karolinska Institutet and Karolinska University Hospital, Karolinska vägen 8, 171 76 Stockholm, Sweden. Electronic address:
    Objective: To evaluate the feasibility of continuous longitudinal intravenous infusion of recombinant human insulin-like growth factor-1/recombinant human insulin-like growth factor binding protein-3 (rhIGF-1/rhIGFBP-3) for prevention of retinopathy of prematurity and other complications in extremely preterm infants (<28weeks' gestational age), based on initial sections of a phase II randomized controlled trial.

    Design: The phase II trial was designed in four sections (A-D); we report pharmacokinetic and adverse events (AEs) data pooled for Sections B and C. Infants in these study sections received rhIGF-1/rhIGFBP-3 or standard neonatal care up to postmenstrual age (weeks+days) 28+6 (Section B) or 29+6 (Section C). Read More

    Continuous Glucose Monitoring in Very Preterm Infants: A Randomized Controlled Trial.
    Pediatrics 2017 Oct 15;140(4). Epub 2017 Sep 15.
    NICU, Departments of Women's and Child's Health and.
    Background And Objectives: Impaired glucose control in very preterm infants is associated with increased morbidity, mortality, and poor neurologic outcome. Strategies based on insulin titration have been unsuccessful in achieving euglycemia in absence of an increase in hypoglycemia and mortality. We sought to assess whether glucose administration guided by continuous glucose monitoring (CGM) is more effective than standard of care blood glucose monitoring in maintaining euglycemia in very preterm infants. Read More

    Deficient Endogenous Glucose Production During Exercise After Total Pancreatectomy/Islet Autotransplantation.
    J Clin Endocrinol Metab 2017 Sep;102(9):3288-3295
    Pacific Northwest Diabetes Research Institute, Seattle, Washington 98122.
    Context: Total pancreatectomy followed by intrahepatic islet autotransplantation (TP/IAT) is performed to alleviate severe, unrelenting abdominal pain caused by chronic pancreatitis, to improve quality of life, and to prevent diabetes.

    Objective: To determine the cause of exercise-induced hypoglycemia that is a common complaint in TP/IAT recipients.

    Design: Participants completed 1 hour of steady-state exercise. Read More

    Correlation of cord blood telomere length with birth weight.
    BMC Res Notes 2017 Sep 8;10(1):469. Epub 2017 Sep 8.
    Research Laboratory, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore, Singapore.
    Background: Intrauterine growth restriction affects 3% of newborns; and the lightest 10% of whom are classified as small for gestational age (SGA). These low-birth weight newborns are at increased risk of neonatal morbidity such as hypoxia and hypoglycaemia. In later life, they are at higher risk of several age-related diseases such as cardiovascular and metabolic disorders and dementia. Read More

    Global motion perception is associated with motor function in 2-year-old children.
    Neurosci Lett 2017 Sep 31;658:177-181. Epub 2017 Aug 31.
    Liggins Institute, University of Auckland, Auckland, New Zealand.
    The dorsal visual processing stream that includes V1, motion sensitive area V5 and the posterior parietal lobe, supports visually guided motor function. Two recent studies have reported associations between global motion perception, a behavioural measure of processing in V5, and motor function in pre-school and school aged children. This indicates a relationship between visual and motor development and also supports the use of global motion perception to assess overall dorsal stream function in studies of human neurodevelopment. Read More

    Closed-Loop Control During Intense Prolonged Outdoor Exercise in Adolescents With Type 1 Diabetes: The Artificial Pancreas Ski Study.
    Diabetes Care 2017 Dec 30;40(12):1644-1650. Epub 2017 Aug 30.
    Barbara Davis Center for Childhood Diabetes, University of Colorado Denver, Aurora, CO.
    Objective: Intense exercise is a major challenge to the management of type 1 diabetes (T1D). Closed-loop control (CLC) systems (artificial pancreas) improve glycemic control during limited intensity and short duration of physical activity (PA). However, CLC has not been tested during extended vigorous outdoor exercise common among adolescents. Read More

    Dilemma of diagnosing sulphonylurea overdose in children: deliberations and considerations before reaching a diagnosis.
    BMJ Case Rep 2017 Aug 28;2017. Epub 2017 Aug 28.
    Department of Paediatrics, Doncaster and Bassetlaw Hospitals NHS Foundation Trust, Doncaster, UK.
    A 15-year-old non-diabetic Caucasian girl presented with sudden onset of seizures, unrecordable blood glucose readings and acute renal failure. She denied any medication ingestion and no other precipitating factors were encountered for this acute presentation. She was treated with intravenous glucose infusion and hydrocortisone injection. Read More

    Functional and molecular studies in primary carnitine deficiency.
    Hum Mutat 2017 Dec 14;38(12):1684-1699. Epub 2017 Sep 14.
    Division of Medical Genetics/Pediatrics, University of Utah, Salt Lake City, Utah.
    Primary carnitine deficiency is caused by a defect in the OCTN2 carnitine transporter encoded by the SLC22A5 gene. It can cause hypoketotic hypoglycemia or cardiomyopathy in children, and sudden death in children and adults. Fibroblasts from affected patients have reduced carnitine transport. Read More

    Closed-loop glucose control in young people with type 1 diabetes during and after unannounced physical activity: a randomised controlled crossover trial.
    Diabetologia 2017 Nov 24;60(11):2157-2167. Epub 2017 Aug 24.
    Department of Paediatric Endocrinology, Diabetes and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, SI-1000, Ljubljana, Slovenia.
    Aims/hypothesis: Hypoglycaemia during and after exercise remains a challenge. The present study evaluated the safety and efficacy of closed-loop insulin delivery during unannounced (to the closed-loop algorithm) afternoon physical activity and during the following night in young people with type 1 diabetes.

    Methods: A randomised, two-arm, open-label, in-hospital, crossover clinical trial was performed at a single site in Slovenia. Read More

    A study on the safety and efficacy of reveglucosidase alfa in patients with late-onset Pompe disease.
    Orphanet J Rare Dis 2017 Aug 24;12(1):144. Epub 2017 Aug 24.
    BioMarin Pharmaceutical, 105 Digital Drive, Novato, CA, 94949, USA.
    Background: Late-onset Pompe disease is a rare genetic neuromuscular disorder caused by lysosomal acid alpha-glucosidase (GAA) deficiency that ultimately results in mobility loss and respiratory failure. Current enzyme replacement therapy with recombinant human (rh)GAA has demonstrated efficacy in subjects with late-onset Pompe disease. However, long-term effects of rhGAA on pulmonary function have not been observed, likely related to inefficient delivery of rhGAA to skeletal muscle lysosomes and associated deficits in the central nervous system. Read More

    Nutrition from the Inside Out.
    Annu Rev Nutr 2017 Aug;37:1-31
    USDA/ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030; email:
    Nearly 50 years ago, I set out to investigate the clinical problem of hypoglycemia in children with illnesses that limited their food intake. My goal was to gather accurate and precise measurable data. At the time, I wasn't interested in nutrition as a discipline defined in its more general or popular sense. Read More

    Insulin delivery and nocturnal glucose control in children and adolescents with type 1 diabetes.
    Expert Opin Drug Deliv 2017 Dec 18;14(12):1367-1377. Epub 2017 Aug 18.
    a Wellcome Trust-MRC Institute of Metabolic Science , University of Cambridge , Cambridge , UK.
    Introduction: Nocturnal glucose control remains challenging in children and adolescents with type 1 diabetes due to highly variable overnight insulin requirements. The issue may be addressed by glucose responsive insulin delivery based on real-time continuous glucose measurements. Areas covered: This review outlines recent developments of glucose responsive insulin delivery systems from a paediatric perspective. Read More

    Clinical practice guidelines for congenital hyperinsulinism.
    Clin Pediatr Endocrinol 2017 27;26(3):127-152. Epub 2017 Jul 27.
    Department of Pediatric Surgery, Tohoku University, Miyagi, Japan.
    Congenital hyperinsulinism is a rare condition, and following recent advances in diagnosis and treatment, it was considered necessary to formulate evidence-based clinical practice guidelines reflecting the most recent progress, to guide the practice of neonatologists, pediatric endocrinologists, general pediatricians, and pediatric surgeons. These guidelines cover a range of aspects, including general features of congenital hyperinsulinism, diagnostic criteria and tools for diagnosis, first- and second-line medical treatment, criteria for and details of surgical treatment, and future perspectives. These guidelines were generated as a collaborative effort between The Japanese Society for Pediatric Endocrinology and The Japanese Society of Pediatric Surgeons, and followed the official procedures of guideline generation to identify important clinical questions, perform a systematic literature review (April 2016), assess the evidence level of each paper, formulate the guidelines, and obtain public comments. Read More

    Association of Neonatal Glycemia With Neurodevelopmental Outcomes at 4.5 Years.
    JAMA Pediatr 2017 Oct;171(10):972-983
    Liggins Institute, The University of Auckland, Auckland, New Zealand.
    Importance: Hypoglycemia is common during neonatal transition and may cause permanent neurological impairment, but optimal intervention thresholds are unknown.

    Objective: To test the hypothesis that neurodevelopment at 4.5 years is related to the severity and frequency of neonatal hypoglycemia. Read More

    [Identification of a novel mutation of AGL gene in two siblings affected with glycogen storage disease type IIIa].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Aug;34(4):499-503
    Department of Pediatrics, the First Affiliated Hospital of Jinan University, Guangzhou, Guangdong 510630, China.
    Objective: To detect potential mutation of the AGL gene in two siblings affected with glycogen storage disease type IIIa.

    Methods: Clinical data of the two siblings was collected and analyzed. Genomic DNA was extracted from peripheral venous blood samples from the patients and their parents. Read More

    Closed-Loop Control Without Meal Announcement in Type 1 Diabetes.
    Diabetes Technol Ther 2017 Sep 2;19(9):527-532. Epub 2017 Aug 2.
    1 Department of Chemical and Biological Engineering, Rensselaer Polytechnic Institute , Troy, New York.
    Objective: A fully closed-loop insulin-only system was developed to provide glucose control in patients with type 1 diabetes without requiring announcement of meals or activity. Our goal was to assess initial safety and efficacy of this system.

    Research Design And Methods: The multiple model probabilistic controller (MMPPC) anticipates meals when the patient is awake. Read More

    Incidence of severe hypoglycemia in children with type 1 diabetes in the Nordic countries in the period 2008-2012: association with hemoglobin A 1c and treatment modality.
    BMJ Open Diabetes Res Care 2017 29;5(1):e000377. Epub 2017 May 29.
    Copenhagen Diabetes Research Center (CPH-DIRECT), Department of Children and Adolescents, Copenhagen University Hospital, Herlev, Denmark.
    Objective: Treatment of type 1 diabetes has been intensified aiming at normalizing blood glucose, which may increase the risk of severe hypoglycemia (SH). We aimed to compare the incidence of SH events in the four Nordic countries Denmark, Iceland, Norway and Sweden, and to assess the influence of hemoglobin A1c (HbA1c) and treatment modalities on the frequency of SH; particularly, to explore if a HbA1c target ≤6.7% (50 mmol/mol) is feasible. Read More

    Successful treatment of a newborn with congenital hyperinsulinism having a novel heterozygous mutation in the ABCC8 gene using subtotal pancreatectomy.
    Ci Ji Yi Xue Za Zhi 2016 Oct-Dec;28(4):162-165. Epub 2016 May 27.
    Department of Pediatrics, MacKay Children Hospital, Taipei, Taiwan.
    Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in newborns and infants. CHI is characterized by unregulated secretion of insulin from pancreatic β: cells. Here, we reported the case of a large-for-gestational-age, full-term newborn that suffered from CHI and developed severe and persistent hypoglycemia at an early stage of life. Read More

    Hereditary Tyrosinemia Type 1 in Turkey.
    Adv Exp Med Biol 2017 ;959:157-172
    Cerrahpasa Medical Faculty, Central Laboratories, Metabolic Diseases Unit, University of Istanbul, 34098, Kocamustafapasa Fatih, Istanbul, Turkey.
    Hereditary tyrosinemia type 1 (HT1, OMIM 276700) is a rare autosomal recessively inherited inborn error of metabolism in the tyrosine catabolic pathway due to deficiency of the enzyme fumarylacetoacetate hydrolase. The clinical features of HT1 are widely heterogenous even within the same family members. Clinical features includes acute or chronic liver disease with increased risk of hepatocellular carcinoma, hypophosphatemic rickets due to renal tubular dysfunction, glomerulosclerosis, failure to thrive, neurological porphyria-like crisis, hypertrophic cardiomyopathy and hypoglycemia due to hyperinsulinism. Read More

    Neurodevelopmental Outcome of Asymptomatic Hypoglycemia Compared With Symptomatic Hypoglycemia and Euglycemia in High-Risk Neonates.
    Pediatr Neurol 2017 Sep 7;74:74-79. Epub 2017 Jun 7.
    Neonatal Unit, Department of Pediatrics, PGIMER, Chandigarh, India.
    Aims: We assessed the neurodevelopmental outcome at one year of age of children with asymptomatic neonatal hypoglycemia and compared their outcome with that of symptomatic hypoglycemic and euglycemic neonates.

    Method: Seventy two hypoglycemic (plasma glucose less than 50 mg/dL) neonates, both symptomatic (n = 27) and asymptomatic (n = 45), and 70 weight- and gestation-matched euglycemic neonates of gestational age greater than 32 weeks were enrolled during the first week of life then assessed for neurodevelopmental outcome at corrected age six and 12 months (n = 67 and 62 in hypoglycemia group and 63 and 54 in euglycemia group, with the rest lost to follow-up, and death = 1).

    Results: At one year, 8% (five of 62, four in symptomatic and one in asymptomatic group) of hypoglycemic neonates developed cerebral palsy. Read More

    Pilot study of propranolol premedication to reduce FDG uptake in brown adipose tissue on PET scans of adolescent and young adult oncology patients.
    Pediatr Hematol Oncol 2017 Apr 20;34(3):149-156. Epub 2017 Jul 20.
    a Department of Pediatrics , University of Kentucky , Lexington , Kentucky , USA.
    Objective: Physiologic uptake of (18)F-fluorodeoxyglucose (FDG) in brown adipose tissue (adipose tissue) of cancer patients may confound interpretation of positron emission tomography (PET) scans. Uptake in adipose tissue occurs in up to half of pediatric oncology patients undergoing PET scans, and is especially common in adolescents. adipose tissue is innervated by the sympathetic nervous system, and beta blockers such as propranolol have shown efficacy in reducing adipose tissue uptake on PET scans done in older adult oncology patients. Read More

    Population Pharmacokinetics of Diazoxide in Children with Hyperinsulinemic Hypoglycemia.
    Horm Res Paediatr 2017 14;88(5):316-323. Epub 2017 Jul 14.
    Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
    Background: Diazoxide is the first-line treatment for pediatric hyperinsulinemic hypoglycemia (HI). This study aimed to elucidate the pharmacokinetics of diazoxide in children with HI.

    Methods: We obtained 81 blood samples from 22 children with HI. Read More

    Assessing the effectiveness of a 3-month day-and-night home closed-loop control combined with pump suspend feature compared with sensor-augmented pump therapy in youths and adults with suboptimally controlled type 1 diabetes: a randomised parallel study protocol.
    BMJ Open 2017 Jul 13;7(7):e016738. Epub 2017 Jul 13.
    Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge, Cambridge, UK.
    Introduction: Despite therapeutic advances, many individuals with type 1 diabetes are unable to achieve tight glycaemic target without increasing the risk of hypoglycaemia. The objective of this study is to determine the effectiveness of a 3-month day-and-night home closed-loop glucose control combined with a pump suspend feature, compared with sensor-augmented insulin pump therapy in youths and adults with suboptimally controlled type 1 diabetes.

    Methods And Analysis: The study adopts an open-label, multi-centre, multi-national (UK and USA), randomised, single-period, parallel design and aims for 84 randomised patients. Read More

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