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    Glycated albumin level during late pregnancy as a predictive factor for neonatal outcomes of women with diabetes.
    J Matern Fetal Neonatal Med 2017 May 22:1-27. Epub 2017 May 22.
    a Department of Pediatrics , Saitama Medical Center Jichi Medical University , 1-847 Amanuma-cho , Saitama 330-8503 , Japan.
    Purpose: To investigate the association between glycated albumin (GA) in diabetic mothers and complications in their children, and determine GA cut-off values for predicting complications in infants.

    Materials And Methods: This hospital-based case-control study involved 71 Japanese diabetic mothers and their children. Mean GA values were compared between mothers of infants with and without complications, and relationship with number of complications was analyzed by Pearson's correlation. Read More

    Change in hemoglobin A1c one year following the 2014 American Diabetes Association guideline update.
    Diabetes Res Clin Pract 2017 May 10;129:169-172. Epub 2017 May 10.
    Department of Pediatrics, University of Colorado School of Medicine, United States; The Barbara Davis Center for Childhood Diabetes, Aurora, CO, United States.
    Aims: In June 2014, the American Diabetes Association lowered recommended hemoglobin A1c (HbA1c) targets from <8.5% (69mmol/mol) for children <6years of age and from <8.0% (64mmol/mol) for children 6-12years of age to <7. Read More

    Effectiveness of SmartGuard Technology in the Prevention of Nocturnal Hypoglycemia After Prolonged Physical Activity.
    Diabetes Technol Ther 2017 May;19(5):299-304
    1 Department of Pediatrics, University Hospital Motol and 2nd Faculty of Medicine, Charles University in Prague , Czech Republic .
    Background: The prevention of postexercise nocturnal hypoglycemia after prolonged physical activity using sensor-augmented pump (SAP) therapy with predictive low-glucose management (PLGM) has not been well studied. We conducted a study at a pediatric diabetes camp to determine whether a SAP with PLGM reduces the frequency of nocturnal hypoglycemia after prolonged physical activity more effectively than a SAP with a carbohydrate intake algorithm.

    Methods: During a 1-week sport camp, 20 children (aged 10-13 years) with type 1 diabetes (T1D) managed by SAP therapy either with (n = 7) or without PLGM (n = 13) were studied. Read More

    Relationship between Glycemic Levels and Treatment Outcome among Critically Ill Children admitted into Emergency Room in Enugu.
    BMC Pediatr 2017 May 16;17(1):126. Epub 2017 May 16.
    Department of Pediatric, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
    Background: Critically ill children are those in need of immediate attention on arrival to an emergency room. The importance of glycemic level measurement as well as maintaining the patency of the airway, effective breathing and circulation cannot be overemphasied. It has been highlighted that the peak hyperglycemia and hypoglycemia predict poor prognosis, longer lengths of hospital stay and higher mortality. Read More

    Real-life experience of patients starting insulin degludec. A multicenter analysis of 1064 subjects from the German/Austrian DPV registry.
    Diabetes Res Clin Pract 2017 May 4;129:52-58. Epub 2017 May 4.
    Institute of Epidemiology and Medical Biometry, ZIBMT, University of Ulm, Ulm, Germany; German Center for Diabetes Research (DZD), Munich-Neuherberg, Germany.
    Background: The long-acting insulin analogue degludec is a therapeutic option for patients with type 1 (T1D) or type 2 diabetes (T2D). Aim of this analysis was to investigate differences in clinical characteristics of patients before and after initiating degludec use in a cohort of German/Austrian patients.

    Methods: 1064 subjects with T1D/T2D and documented degludec use from the Diabetes-Patient-Follow-Up (DPV) registry were included. Read More

    Continuous Subcutaneous Insulin Infusion in Children: A Pilot Study Validating a Protocol to Avoid Hypoglycemia at Initiation.
    Front Endocrinol (Lausanne) 2017 24;8:84. Epub 2017 Apr 24.
    Endocrinology Service and Diabetes Unit, CHU Sainte-Justine, Montreal, QC, Canada.
    Background: The occurrence of hypoglycemia and hyperglycemia during the first days after transition to continuous subcutaneous insulin infusion (CSII) in patients with type 1 diabetes has not been systematically studied in children. The aim of this prospective study was to demonstrate that the protocol applied in our diabetes clinic is safe at CSII initiation in children.

    Methods: We assessed 22 pediatric patients with type 1 diabetes, using continuous glucose monitoring (CGM) before and after CSII initiation (±3 days). Read More

    Dietary intake and risk of non-severe hypoglycemia in adolescents with type 1 diabetes.
    J Diabetes Complications 2017 Apr 20. Epub 2017 Apr 20.
    Department of Nutrition, University of North Carolina, Chapel Hill, NC, USA; Department of Medicine, School of Medicine, University of North Carolina, Chapel Hill, NC, USA. Electronic address:
    Aims: To determine the association between dietary intake and risk of non-severe hypoglycemia in adolescents with type 1 diabetes.

    Methods: Type 1 adolescents from a randomized trial wore a blinded continuous glucose monitoring (CGM) system at baseline for one week in free-living conditions. Dietary intake was calculated as the average from two 24-h dietary recalls. Read More

    A Case Report of Hypoglycemia and Hypogammaglobulinemia: DAVID syndrome in a patient with a novel NFKB2 mutation.
    J Clin Endocrinol Metab 2017 May 3. Epub 2017 May 3.
    Division of Allergy, Immunology & Rheumatology, Departments of Pediatrics, Stanford University School of Medicine, Stanford, California.
    Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID. Read More

    Outpatient Closed-Loop Control with Unannounced Moderate Exercise in Adolescents Using Zone Model Predictive Control.
    Diabetes Technol Ther 2017 May 1. Epub 2017 May 1.
    1 Department of Chemical Engineering, University of California Santa Barbara , Santa Barbara, California.
    Background: The artificial pancreas (AP) has the potential to improve glycemic control in adolescents. This article presents the first evaluation in adolescents of the Zone Model Predictive Control and Health Monitoring System (ZMPC+HMS) AP algorithms, and their first evaluation in a supervised outpatient setting with frequent exercise.

    Materials And Methods: Adolescents with type 1 diabetes underwent 3 days of closed-loop control (CLC) in a hotel setting with the ZMPC+HMS algorithms on the Diabetes Assistant platform. Read More

    Hyperinsulinaemic hypoglycaemia, renal Fanconi syndrome and liver disease due to a mutation in the HNF4A gene.
    Endocrinol Diabetes Metab Case Rep 2017 16;2017. Epub 2017 Mar 16.
    Paediatric Endocrinology Unit, Department of Paediatrics.
    Summary: HNF4A gene mutations have been reported in cases of transient and persistent hyperinsulinaemic hypoglycaemia of infancy (HHI), particularly in families with adulthood diabetes. The case of a patient with HHI, liver impairment and renal tubulopathy due to a mutation in HNF4A is reported.

    Learning Points: Urine specimen study in cases of HHI with diazoxide response is necessary to rule out specific metabolic conditions (l-3-hydroxyacyl-coenzyme A dehydrogenase deficiency) or tubular renal involvement. Read More

    Functional and Metabolomic Consequences of ATP-dependent Potassium Channel Inactivation in Human Islets.
    Diabetes 2017 Apr 25. Epub 2017 Apr 25.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104.
    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. Read More

    Discharge Criteria for the Late Preterm Infant: A Review of the Literature.
    Adv Neonatal Care 2017 Apr 24. Epub 2017 Apr 24.
    Department of Pediatrics, NorthBay Medical Center, Fairfield, California (Ms Quinn); College of Nursing, The University of Arizona, Tucson, Arizona (Ms Sparks and Dr Gephart).
    Background: Standardized late preterm infant (LPI) discharge criteria ensure best practice and help guide the neonatal provider to determine the appropriate level of care following birth. However, the location can vary from the well newborn setting to the neonatal intensive care unit (NICU).

    Purpose: The purpose of this review is to examine differences in LPI discharge criteria between the well newborn setting and the NICU by answering the clinical questions, "What are the recommended discharge criteria for the LPI and do they differ if admitted to the well newborn setting versus the NICU?"

    Search Strategy: Databases searched include CINAHL, TRIP, PubMed, and the Cochrane Library. Read More

    Congenital Hyperinsulinism Caused by a De Novo Mutation in the ABCC8 Gene - A Case Report.
    EJIFCC 2017 Mar 8;28(1):85-91. Epub 2017 Mar 8.
    Department of Laboratory Medicine, University of Debrecen, Debrecen, Hungary.
    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. Read More

    Global motion perception is related to motor function in 4.5-year-old children born at risk of abnormal development.
    Vision Res 2017 Jun 28;135:16-25. Epub 2017 Apr 28.
    School of Optometry and Vision Science, University of Auckland, New Zealand; School of Optometry and Vision Science, University of Waterloo, Canada. Electronic address:
    Global motion perception is often used as an index of dorsal visual stream function in neurodevelopmental studies. However, the relationship between global motion perception and visuomotor control, a primary function of the dorsal stream, is unclear. We measured global motion perception (motion coherence threshold; MCT) and performance on standardized measures of motor function in 606 4. Read More

    An Outpatient Methadone Weaning Program by a Neonatal Intensive Care Unit for Neonatal Abstinence Syndrome.
    Popul Health Manag 2017 Apr 21. Epub 2017 Apr 21.
    2 Department of Pediatrics, Lehigh Valley Health Network , Allentown, Pennsylvania.
    Through retrospective chart review, this study described characteristics and length of stay for a cohort of newborns discharged on methadone following an inpatient weaning for neonatal abstinence syndrome (NAS). Data were assessed for all term infants born between January 1, 2010, and December 31, 2014, admitted to the hospital with a co-diagnosis of NAS at discharge, for gestational age, length of stay, days on treatment protocol before discharge, time to once-daily interval methadone dosing, and hospital charges, as well as for categorical characteristics. The 53 patients were predominantly male (58%), white (71%), and covered by Medicaid insurance (72%). Read More

    Performance of an Artificial Pancreas System for Young Children with Type 1 Diabetes.
    Diabetes Technol Ther 2017 May 20;19(5):293-298. Epub 2017 Apr 20.
    1 Center for Diabetes Technology, University of Virginia , Charlottesville, Virginia.
    Background: Young children 5-8 years old with type 1 diabetes (T1D) exhibit clear needs for improved glycemic control but may be limited in their ability to safely interact with an artificial pancreas system. Our goal was to evaluate the safety and performance of an artificial pancreas (AP) system among young children with T1D.

    Research Design And Methods: In a randomized, crossover trial, children with T1D age 5-8 years were enrolled to receive on separate study periods (in random order) either the UVa AP using the DiAs Control Platform software with child-resistant lock-out screens (followed as an out-patient admission) or their usual insulin pump+continuous glucose monitor (CGM) care at home. Read More

    A Single Bout of High-Intensity Interval Training Reduces Awareness of Subsequent Hypoglycemia in Patients with Type 1 Diabetes.
    Diabetes 2017 Apr 18. Epub 2017 Apr 18.
    Departments of Internal Medicine.
    High-intensity interval training (HIIT) gains increasing popularity in patients with diabetes. HIIT acutely increases plasma lactate levels. This may be important, since administration of lactate during hypoglycemia suppresses symptoms and counterregulation, whilst preserving cognitive function. Read More

    A Rare Cause of Recurrent Acute Pancreatitis in a Child: Isovaleric Acidemia with Novel Mutation.
    Pediatr Gastroenterol Hepatol Nutr 2017 Mar 27;20(1):61-64. Epub 2017 Mar 27.
    Department of Pediatric Gastroenterology Hepatology and Nutrition, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey.
    Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma. Read More

    A Pilot Study Evaluating Therapeutic Response of Different Dosage of Oral Glucocorticoid in Two Children with Familial Glucocorticoid Deficiency Presenting with Diffuse Mucocutaneous Hyperpigmentation.
    Indian J Dermatol 2017 Mar-Apr;62(2):191-194
    Department of Pediatrics, Dr. B. C. Roy Post Graduate Institute of Paediatric Sciences, Kolkata, West Bengal, India.
    Introduction: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive potentially life-threatening condition, characterized by glucocorticoid deficiency, preserved aldosterone/renin secretion, and secondary rise in plasma adrenocorticotropic hormone level. This occurs due to either mutation in adrenocorticotropic receptor (25%, FGD Type-1) or in the MC2 receptor accessory protein (15%-20%). However, in about 50% patients, no identifiable mutations have been identified. Read More

    Flash glucose monitoring system may benefit children and adolescents with type 1 diabetes during fasting at Ramadan.
    Saudi Med J 2017 Apr;38(4):366-371
    Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia. E-mail.
    Objectives: To assess the benefit of using the flash glucose monitoring system (FGMS) in children and adolescents with type 1 diabetes mellitus (T1DM) during Ramadan fasting. Methods: A prospective pilot study of 51 participants visited the pediatric diabetes clinic at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia from between June until and July 2016. The FreeStyle® Libre™ FGMS (Abbott Diabetes Care, Alameda, CA, USA) was used. Read More

    Alcohol and cigarette use among adolescents with type 1 diabetes.
    Eur J Pediatr 2017 Jun 5;176(6):713-722. Epub 2017 Apr 5.
    Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, ul. Sporna 36/50, 91-738, Lodz, Poland.
    In this study, we compare the prevalence of alcohol and cigarette use among Polish adolescents with type 1 diabetes mellitus (T1DM) (n = 209), aged 15-18 years, with that of a large cohort of their healthy peers, using standardized questionnaire used in the European School Survey Project on Alcohol and Drugs (ESPAD). The lifetime, previous year, and past 30-day prevalence of alcohol consumption was high among adolescents with T1DM but lower than in the controls (82.8 vs 92. Read More

    A multicentre study of patients with Timothy syndrome.
    Europace 2017 Mar 24. Epub 2017 Mar 24.
    Department of Cardiology, Royal Hospital for Sick Children, Glasgow, UK.
    Aims: Timothy syndrome (TS) is an extremely rare multisystem disorder characterized by marked QT prolongation, syndactyly, seizures, behavioural abnormalities, immunodeficiency, and hypoglycaemia. The aim of this study was to categorize the phenotypes and examine the outcomes of patients with TS.

    Methods And Results: All patients diagnosed with TS in the United Kingdom over a 24-year period were reviewed. Read More

    Neonatal Hypoglycemia.
    Pediatr Rev 2017 Apr;38(4):147-157
    Division of Neonatology, Children's Hospital at Montefiore, Bronx, NY.
    Lower blood glucose values are common in the healthy neonate immediately after birth as compared to older infants, children, and adults. These transiently lower glucose values improve and reach normal ranges within hours after birth. Such transitional hypoglycemia is common in the healthy newborn. Read More

    Clinical presentation and treatment response to diazoxide in two siblings with congenital hyperinsulinism as a result of a novel compound heterozygous ABCC8 missense mutation.
    J Pediatr Endocrinol Metab 2017 Apr;30(4):471-474
    Genetics and Epigenetics in Health and Disease Genetics and Genomic Medicine Programme, UCL Institute Child Health, London.
    Background: Congenital hyperinsulinism (CHI) can present with considerable clinical heterogeneity which may be due to differences in the underlying genetic etiology. We present two siblings with hyperinsulinaemic hypoglycaemia (HH) and marked clinical heterogeneity caused by compound heterozygosity for the same two novel ABCC8 mutations.

    Case Presentation: The index patient is a 3-year-old boy with hypoglycaemic episodes presenting on the first day of life. Read More

    Sex-Specific Life Course Changes in the Neuro-Metabolic Phenotype of Glut3 Null Heterozygous Mice: Ketogenic Diet Ameliorates Electroencephalographic Seizures and Improves Sociability.
    Endocrinology 2017 Apr;158(4):936-949
    Department of Pediatrics, Division of Neonatology & Developmental Biology, Neonatal Research Center at the UCLA Children's Discovery and Innovation Institute.
    We tested the hypothesis that exposure of glut3+/- mice to a ketogenic diet ameliorates autism-like features, which include aberrant behavior and electrographic seizures. We first investigated the life course sex-specific changes in basal plasma-cerebrospinal fluid (CSF)-brain metabolic profile, brain glucose transport/uptake, glucose and monocarboxylate transporter proteins, and adenosine triphosphate (ATP) in the presence or absence of systemic insulin administration. Glut3+/- male but not female mice (5 months of age) displayed reduced CSF glucose/lactate concentrations with no change in brain Glut1, Mct2, glucose uptake or ATP. Read More

    Hepatic Insulin Resistance and Altered Gluconeogenic Pathway in Premature Baboons.
    Endocrinology 2017 Jan 17. Epub 2017 Jan 17.
    Department of Pediatrics, Neonatology Division, University of Texas Health Science Center at San Antonio, San Antonio, TX, 78229, United States.
    Premature infants have altered glucose regulation early in life and increased risk for diabetes in adulthood. Although prematurity leads to an increased risk of diabetes and metabolic syndrome in adult life, the role of hepatic glucose regulation and adaptation to an early extra-uterine environment in preterm infants remain unknown. The purpose of this study was to investigate developmental differences in glucose metabolism, hepatic protein content and gene expression of key insulin signaling/gluconeogenic molecules. Read More

    Hepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa).
    Sci Rep 2017 Mar 20;7:44408. Epub 2017 Mar 20.
    Cardiovascular and Metabolic Disorders Program, Duke-NUS Medical School Singapore, Singapore.
    Glycogen storage disease type Ia (GSDIa, von Gierke disease) is the most common glycogen storage disorder. It is caused by the deficiency of glucose-6-phosphatase, an enzyme which catalyses the final step of gluconeogenesis and glycogenolysis. Clinically, GSDIa is characterized by fasting hypoglycaemia and hepatic glycogen and triglyceride overaccumulation. Read More

    Carnitine-related hypoglycemia caused by 3 days of pivalate antibiotic therapy in a patient with severe muscular dystrophy: a case report.
    BMC Pediatr 2017 Mar 14;17(1):73. Epub 2017 Mar 14.
    Department of Pediatrics Ehime University Graduate School of Medicine, 454 Shitsukawa, Toon, Ehime, 790-0295, Japan.
    Background: Long-term treatment with antibiotics containing pivalic acid may decrease serum carnitine concentration and can sometimes be associated with severe hypoglycemia and encephalopathy in infants. Little has been reported, however, on severe hypocarnitinemia induced by acute administration in older children.

    Case Presentation: We describe a 6-year-old Japanese girl with Fukuyama-type congenital muscular dystrophy who lost consciousness after 3 days of treatment with an antibiotic containing pivalic acid (cefditoren pivoxil). Read More

    Intensive gestational glycemic management and childhood obesity: a systematic review and meta-analysis.
    Int J Obes (Lond) 2017 Apr 4. Epub 2017 Apr 4.
    Children's Hospital Research Institute of Manitoba, and Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba, Canada.
    Background And Objectives: Hyperglycemia in pregnancy is associated with increased risk of offspring childhood obesity. Treatment reduces macrosomia; however, it is unclear if this effect translates into a reduced risk of childhood obesity. We performed a systematic review and meta-analysis of randomized controlled trials to evaluate the efficacy and safety of intensive glycemic management in pregnancy in preventing childhood obesity. Read More

    Clinical and Molecular Variability in Patients with PHKA2 Variants and Liver Phosphorylase b Kinase Deficiency.
    JIMD Rep 2017 Mar 12. Epub 2017 Mar 12.
    Department of Pediatrics, Duke Medicine, Durham, NC, USA.
    Glycogen storage disease (GSD) type IX is a rare disease of variable clinical severity affecting primarily the liver tissue. Individuals with liver phosphorylase b kinase (PhK) deficiency (GSD IX) can present with hepatomegaly with elevated serum transaminases, ketotic hypoglycemia, hyperlipidemia, and poor growth with considerable variation in clinical severity. PhK is a cAMP-dependent protein kinase that phosphorylates the inactive form of glycogen phosphorylase, phosphorylase b, to produce the active form, phosphorylase a. Read More

    UQCRC2 mutation in a patient with mitochondrial complex III deficiency causing recurrent liver failure, lactic acidosis and hypoglycemia.
    J Hum Genet 2017 Mar 9. Epub 2017 Mar 9.
    APHP-CHU de Bicêtre, Biochemistry Department, 78, rue du Général Leclerc, Le Kremlin Bicêtre, France.
    An isolated mitochondrial complex III (CIII) defect constitutes a rare cause of mitochondrial disorder. Here we present the second case involving UQCRC2 gene, which encodes core protein 2, one of the 11 structural subunits of CIII. The patient has the same mutation (c. Read More

    Computer-determined dosage of insulin in the management of neonatal hyperglycaemia (HINT2): protocol of a randomised controlled trial.
    BMJ Open 2017 Mar 6;7(3):e012982. Epub 2017 Mar 6.
    Liggins Institute, University of Auckland, Auckland, New Zealand.
    Introduction: Neonatal hyperglycaemia is frequently treated with insulin, which may increase the risk of hypoglycaemia. Computer-determined dosage of insulin (CDD) with the STAR-GRYPHON program uses a computer model to predict an effective dose of insulin to treat hyperglycaemia while minimising the risk of hypoglycaemia. However, CDD models can require more frequent blood glucose testing than common clinical protocols. Read More

    Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency.
    Ital J Pediatr 2017 Jan 19;43(1):12. Epub 2017 Jan 19.
    Department of Radiology, Hospital Clínico Universitario, Avda. Blasco Ibáñez 17, 46010, Valencia, Spain.
    Background: 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency is a rare inborn error of metabolism characterized by recurrent metabolic crises caused by fasting, intercurrent illness and excessive physical exercise. Non ketotic hypoglycemia is normally the cause of primary symptoms but without an immediate treatment the illness can evolve into a worsening metabolic state resembling the Reye's syndrome that may cause the patient's death. We report a case with some clinical and therapeutic features not previously described. Read More

    Macrosomic births in abuja: A case-control study of predisposing factors and early neonatal outcome.
    Niger J Clin Pract 2017 Mar;20(3):320-327
    Department of Pediatrics and Child Health, College of Health Sciences, University of Ilorin, Ilorin, Nigeria.
    Background: Although research has shown that having a macrosomic fetus could be predictive of a negative pregnancy outcome, the factors that control its incidence and the outcome of delivery management have been less well characterized in Africa. The aim of this study was to identify specific predispositions and the factors that influence the early neonatal outcome of macrosomic infants in Abuja.

    Methods: Data from 120 mother and macrosomic (weighing ≥4000 g) newborn pairs, and an equal number of mother and normal weight (2500-3999 g) matched controls, delivered over a 5-month period at three large hospitals in Abuja, Nigeria, were analyzed. Read More

    Spectrum and outcome of neonatal emergencies seen in a free health-care program in South-Western Nigeria.
    Niger J Clin Pract 2017 Mar;20(3):283-289
    Department of Paediatrics, Mother and Child Hospital, Akure, Ondo State, Nigeria.
    Background: Neonatal emergencies contribute significantly to under-five morbidity and mortality in developing countries, partly due to poverty and limited access to quality healthcare in rural communities.

    Aims: The aim is to evaluate the spectrum, outcome, case fatality rates (CFRs), and prognostic factors of neonatal emergencies seen in the two free health-care facilities in Ondo State.

    Methods: This was a cross-sectional descriptive study of neonatal emergencies. Read More

    The variable clinical phenotype of three patients with hepatic glycogen synthase deficiency.
    J Pediatr Endocrinol Metab 2017 Apr;30(4):459-462
    Intergen Genetic Center, Ankara.
    Background: Glycogen synthase deficiency, also known as glycogenosis (GSD) type 0 is an inborn error of glycogen metabolism caused by mutations in the GYS2 gene, which is transmitted in an autosomal recessive trait. It is a rare form of hepatic glycogen storage disease with less than 30 cases reported in the literature so far. The disorder is characterized by fasting hyperketotic hypoglycemia without hyperalaninemia or hyperlactacidemia. Read More

    Congenital hyperinsulinism and glycogenosis-like phenotype due to a novel HNF4A mutation.
    Diabetes Res Clin Pract 2017 Apr 16;126:144-150. Epub 2017 Feb 16.
    DIABGENE Laboratory, Institute of Experimental Endocrinology, Biomedical Research Center SAS, Dubravska cesta 9, 845 05 Bratislava, Slovakia. Electronic address:
    Aim: Congenital hyperinsulinism (CHI) and glycogen storage disease (glycogenosis) are both causing hypoglycemia during infancy, but with different additional clinical features and therapeutic approach. We aimed to identify a genetic cause in a child with an ambiguous phenotype.

    Methods And Results: We present a child with hyperinsulinemic hypoglycemia, physiological 3-OH butyrate, increased triglyceride serum levels, increased level of glycogen in erythrocytes, increased liver transaminases, and increased echogenicity on liver ultrasonography. Read More

    Retrospective Evaluation of a National Guideline to Prevent Neonatal Hypoglycemia.
    Pediatr Neonatol 2017 Jan 24. Epub 2017 Jan 24.
    Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark; Institute of Clinical Research, University of Southern Denmark, Odense, Denmark. Electronic address:
    Background: Hypoglycemia is common in neonates and may cause adverse neurological outcomes. Guidelines should aim to prevent repeated hypoglycemic episodes in risk groups, but they are not usually stratified according to the severity of hypoglycemia risk, which may lead to inappropriate and redundant interventions. We evaluated the effect of a national prevention guideline stratified according to mild, moderate, and severe risks of hypoglycemia. Read More

    Whole genome expression profiling associates activation of unfolded protein response with impaired production and release of epinephrine after recurrent hypoglycemia.
    PLoS One 2017 24;12(2):e0172789. Epub 2017 Feb 24.
    Departments of Pediatrics, Biochemistry and Molecular Biology, Division of Newborn Medicine, New York Medical College, Valhalla, New York, United States of America.
    Recurrent hypoglycemia can occur as a major complication of insulin replacement therapy, limiting the long-term health benefits of intense glycemic control in type 1 and advanced type 2 diabetic patients. It impairs the normal counter-regulatory hormonal and behavioral responses to glucose deprivation, a phenomenon known as hypoglycemia associated autonomic failure (HAAF). The molecular mechanisms leading to defective counter-regulation are not completely understood. Read More

    Evaluation of a Predictive Low-Glucose Management System In-Clinic.
    Diabetes Technol Ther 2017 May 16;19(5):288-292. Epub 2017 Feb 16.
    9 Medtronic, Northridge, California.
    Background: Predictions based on continuous glucose monitoring (CGM) data are the basis for automatic suspension and resumption of insulin delivery by a predictive low-glucose management feature termed "suspend before low," which is part of the Medtronic MiniMed(®) 640G combined insulin pump and CGM system. This study assessed the safety and performance characteristics of the system in an in-clinic setting at eight sites.

    Materials And Methods: In-clinic standardized increases in basal insulin delivery rates were used to induce nocturnal hypoglycemia in subjects (14-75 years) with type 1 diabetes wearing the MiniMed 640G system. Read More

    Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction.
    Mol Genet Metab 2017 Apr 6;120(4):337-341. Epub 2017 Feb 6.
    Department of Pediatrics, Centre for Molecular Medicine and Therapeutics, BC Children's Hospital Research Institute, University of British Columbia, Vancouver, Canada; Department of Pediatrics, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
    Clinical and laboratory data were collected from three Finnish patients including a sibling pair and another unrelated child with unexplained childhood hypoglycemia. Transient elevation of alanine transaminase, lactate and tricarboxylic acid cycle intermediates, especially fumarate, were noticed in urine organic acid analysis. Exome sequencing was performed for the patients and their parents. Read More

    Complications During Ketogenic Diet Initiation: Prevalence, Treatment, and Influence on Seizure Outcomes.
    Pediatr Neurol 2017 Mar 16;68:35-39. Epub 2017 Jan 16.
    Department of Pediatrics, The Johns Hopkins Hospital, Baltimore, Maryland; Department of Neurology, The Johns Hopkins Hospital, Baltimore, Maryland. Electronic address:
    Background: Many centers still admit children for several days to start the ketogenic diet. The exact incidence of adverse effects during the admission and their potential later impact on seizure reduction has not been widely studied.

    Methods: We performed a retrospective study of children with intractable epilepsy electively admitted for ketogenic diet initiation at our institution from 2011 to 2016. Read More

    Phase I/II multicenter ketogenic diet study for adult superrefractory status epilepticus.
    Neurology 2017 Mar 8;88(10):938-943. Epub 2017 Feb 8.
    From the Departments of Neurology (M.C.C., E.H.K., A.L.H., J.C.P., D.R.B., A.V., B.R., M.D., F.M.C., P.N.), Pediatrics (E.H.K., A.L.H.), and Anesthesiology-Critical Care Medicine (R.G.G.), Neurosurgery and Medicine, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology (S.H.), Mayo Clinic, Rochester, MN; Neuroscience Institute (M.K., E.C.H., D.D., T.S.), Queen's Medical Center, Honolulu, HI; Department of Neurology (B.B.), Loyola University Health System, Maywood, IL; Institute for Clinical and Translational Research (B.H.-B.), Johns Hopkins University, Baltimore, MD; and Department of Neurology (P.W.K.), Johns Hopkins Bayview Medical Center, Baltimore, MD.
    Objective: To investigate the feasibility, safety, and efficacy of a ketogenic diet (KD) for superrefractory status epilepticus (SRSE) in adults.

    Methods: We performed a prospective multicenter study of patients 18 to 80 years of age with SRSE treated with a KD treatment algorithm. The primary outcome measure was significant urine and serum ketone body production as a biomarker of feasibility. Read More

    Seasonal variation in hospital encounters with hypoglycaemia and hyperglycaemia.
    Diabet Med 2017 Feb 7. Epub 2017 Feb 7.
    Department of Medicine, Division of Nephrology, London, Ontario, Canada.
    Aim: To assess whether rates of hospital encounters with hypoglycaemia and hyperglycaemia display seasonal variation.

    Methods: Time series analyses of the monthly rates of hospital encounters (emergency room visits or inpatient admissions) with hypoglycaemia and hyperglycaemia from 2003 to 2012 using linked healthcare databases in Ontario, Canada.

    Results: Over the study period, there were 129 887 hypoglycaemia and 79 773 hyperglycaemia encounters. Read More

    A severe case of hyperinsulinism due to hemizygous activating mutation of glutamate dehydrogenase.
    Pediatr Diabetes 2017 Feb 6. Epub 2017 Feb 6.
    Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
    Activating mutations in the GLUD1 gene, which encodes glutamate dehydrogenase (GDH), result in the hyperinsulinism-hyperammonemia syndrome. GDH is an allosterically regulated enzyme responsible for amino acid-mediated insulin secretion via the oxidative deamination of glutamate to 2-oxoglutarate, leading to ATP production and insulin release. This study characterizes a novel combination of mutations in GLUD1 found in a neonate who presented on the first day of life with severe hypoglycemia, hyperammonemia, and seizures. Read More

    Systemic primary carnitine deficiency with hypoglycemic encephalopathy.
    Ann Pediatr Endocrinol Metab 2016 Dec 31;21(4):226-229. Epub 2016 Dec 31.
    Department of Pediatrics, Fatima Hospital, Daegu, Korea.
    Acute hypoglycemia in children is not an uncommon disease that can be encountered in the Emergency Department. Most cases of childhood hypoglycemia are caused by ketotic hypoglycemia due to missed meals. Often, hypoketotic hypoglycemia can also occur, which suggests hyperinsulinemia or a defect in fatty acid oxidation. Read More

    Hypogonadotropic Hypogonadism in Males with Glycogen Storage Disease Type 1.
    JIMD Rep 2017 Feb 4. Epub 2017 Feb 4.
    Division of Endocrinology, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada.
    Background: Glycogen storage disease type 1 is an autosomal recessive disorder with an incidence of 1 in 100,000. Long-term complications include chronic blood glucose lability, lactic academia, short stature, osteoporosis, delayed puberty, gout, progressive renal insufficiency, systemic or pulmonary hypertension, hepatic adenomas at risk for malignant transformation, anemia, vitamin D deficiency, hyperuricemic nephrocalcinosis, inflammatory bowel syndrome (type 1b), hypertriglyceridemia, and irregular menstrual cycles. We describe hypogonadotropic hypogonadism as a novel complication in glycogen storage disease (GSD) type 1. Read More

    Pediatric Diabetes Outpatient Center at Rhode Island Hospital: The impact of changing initial diabetes education from inpatient to outpatient.
    R I Med J (2013) 2017 Feb 1;100(2):21-24. Epub 2017 Feb 1.
    Department of Pediatrics, Division of Pediatric Endocrinology at Rhode Island Hospital and Hasbro Children's Hospital/The Warren Alpert Medical School of Brown University, Providence, RI.
    Background: This study compared outcomes and costs for new-onset Type 1 diabetes mellitus (T1DM) patients educated at the outpatient versus inpatient settings.

    Methods/design: Retrospective study examining the following variables: 1) hemoglobin A1c (HbA1c), 2) severe hypoglycemia, 3) admissions for diabetic ketoacidosis (DKA) or ER visits, and 4) healthcare cost.

    Results: 152 patients with new-onset T1DM from September 2007-August 2009. Read More

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