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    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, MO.
    The extant literature finds that children with type 1 diabetes (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 Apr 5. 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 Apr 2. Epub 2018 Apr 2.
    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 28:1-36. 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

    Hypoglycaemia incidence and recovery during home use of hybrid closed-loop insulin delivery in adults with type 1 diabetes.
    Diabetes Obes Metab 2018 Mar 25. Epub 2018 Mar 25.
    Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge, Cambridge, UK.
    Glucose excursion was assessed prior to and post hypoglycaemia to increase understanding of hypoglycaemia incidence and recovery during hybrid closed-loop insulin delivery. We retrospectively analysed data from 60 adults with type 1 diabetes who received, in a crossover randomized design, day-and-night hybrid closed-loop insulin delivery and insulin pump therapy, the latter with or without real-time continuous glucose monitoring. Over 4-week study periods, we identified hypoglycaemic episodes, defined as sensor glucose <3. Read More

    Bi-allelic Mutations in the Mitochondrial Ribosomal Protein MRPS2 Cause Sensorineural Hearing Loss, Hypoglycemia, and Multiple OXPHOS Complex Deficiencies.
    Am J Hum Genet 2018 Apr 22;102(4):685-695. Epub 2018 Mar 22.
    Department of Clinical Genomics, Mayo Clinic, Rochester, MN 55905, USA. Electronic address:
    Biogenesis of the mitochondrial oxidative phosphorylation system, which produces the bulk of ATP for almost all eukaryotic cells, depends on the translation of 13 mtDNA-encoded polypeptides by mitochondria-specific ribosomes in the mitochondrial matrix. These mitoribosomes are dual-origin ribonucleoprotein complexes, which contain mtDNA-encoded rRNAs and tRNAs and ∼80 nucleus-encoded proteins. An increasing number of gene mutations that impair mitoribosomal function and result in multiple OXPHOS deficiencies are being linked to human mitochondrial diseases. Read More

    The clinician factor: Personality characteristics of clinicians and their impact upon clinical outcomes in the management of children and adolescents with type 1 diabetes.
    Pediatr Diabetes 2018 Mar 23. Epub 2018 Mar 23.
    Psychological and Clinical Science School, Charles Darwin University, Darwin, Australia.
    Objective: The purpose of this study was to estimate clinician qualities that influence metabolic outcomes in youth with type 1 diabetes.

    Research Design And Methods: Data were gathered over two 3 month periods in a large tertiary diabetes center (1500 patients, 8 clinicians) from patients with type 1 diabetes who received continuous care from each clinician. Data included sex, age, diabetes duration, insulin regimen, body mass index (BMI), insulin dose and episodes of severe hypoglycemia. Read More

    Hypoglycemia in a Patient With a Polyhormonal Pancreatic Neuroendocrine Tumor With Evidence of Endocrine Progenitors.
    J Endocr Soc 2018 Feb 16;2(2):172-177. Epub 2018 Jan 16.
    Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana 46202.
    A 55-year-old woman with a large polyhormonal neuroendocrine tumor with unusual pathology is described. The patient presented with intermittent neuroglycopenic symptoms between more protracted asymptomatic periods occurring over the preceding 4 years. During a diagnostic 72-hour inpatient fast, she exhibited hypoglycemia at 70 hours after initiation. Read More

    Adaptive and Personalized Plasma Insulin Concentration Estimation for Artificial Pancreas Systems.
    J Diabetes Sci Technol 2018 Mar 1:1932296818763959. Epub 2018 Mar 1.
    1 Department of Chemical and Biological Engineering, Illinois Institute of Technology, Chicago, IL, USA.
    Background: The artificial pancreas (AP) system, a technology that automatically administers exogenous insulin in people with type 1 diabetes mellitus (T1DM) to regulate their blood glucose concentrations, necessitates the estimation of the amount of active insulin already present in the body to avoid overdosing.

    Method: An adaptive and personalized plasma insulin concentration (PIC) estimator is designed in this work to accurately quantify the insulin present in the bloodstream. The proposed PIC estimation approach incorporates Hovorka's glucose-insulin model with the unscented Kalman filtering algorithm. Read More

    Impact of adopting the 2013 World Health Organization criteria for diagnosis of gestational diabetes in a multi-ethnic Asian cohort: a prospective study.
    BMC Pregnancy Childbirth 2018 Mar 21;18(1):69. Epub 2018 Mar 21.
    Department of Obstetrics & Gynaecology, National University Hospital, Singapore, 119074, Singapore.
    Background: We assessed the impact of adopting the 2013 World Health Organization (WHO) diagnostic criteria on the rates of gestational diabetes (GDM), pregnancy outcomes and identification of women at future risk of type 2 diabetes.

    Methods: During a period when the 1999 WHO GDM criteria were in effect, pregnant women were universally screened using a one-step 75 g 2-h oral glucose tolerance test at 26-28 weeks' gestation. Women were retrospectively reclassified according to the 2013 criteria, but without the 1-h glycaemia measurement. Read More

    Knowledge Translation to Optimize Adult Inpatient Glycemic Management with Basal Bolus Insulin Therapy and Improve Patient Outcomes.
    Can J Diabetes 2017 Dec 27. Epub 2017 Dec 27.
    Division of Endocrinology and Metabolism, Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada; Diabetes, Obesity, and Nutrition Strategic Clinical Network, Alberta Health Services, Calgary, Alberta, Canada.
    Objectives: To develop and evaluate a Basal Bolus Insulin Therapy (BBIT) Knowledge Translation toolkit to address barriers to adoption of established best practice with BBIT in the care of adult inpatients.

    Methods: This study was conducted in 2 phases and focused on the hospitalist provider group across 4 acute care facilities in Calgary. Phase 1 involved a qualitative evaluation of provider and site specific barriers and facilitators, which were mapped to validated interventions using behaviour change theory. Read More

    Hypoglycaemia was common in acute gastroenteritis in a prospective hospital-based study, but electrolyte imbalances were not.
    Acta Paediatr 2018 Mar 13. Epub 2018 Mar 13.
    Department of Paediatrics, Ostfold Hospital Trust, Grålum, Norway.
    Aim: Using routine blood sampling in a gastroenteritis diagnostic workup is debatable. This study examined the relationship between the severity of acute gastroenteritis and blood test abnormalities.

    Methods: We prospectively enrolled children under five years of age referred for outpatient or inpatient management for gastroenteritis from February 2014 to April 2016. Read More

    Defining Outcomes for β-Cell Replacement Therapy in the Treatment of Diabetes: a Consensus Report on the Igls Criteria from the IPITA/EPITA Opinion Leaders Workshop.
    Transplantation 2018 Mar 9. Epub 2018 Mar 9.
    Institute of Transplantation, The Freeman Hospital and Newcastle University, Newcastle upon Tyne, United Kingdom.
    β-cell replacement therapy, available currently as pancreas or islet transplantation, has developed without a clear definition of graft functional and clinical outcomes. The International Pancreas & Islet Transplant Association (IPITA) and European Pancreas & Islet Transplantation Association (EPITA) held a workshop to develop consensus for an IPITA/EPITA Statement on the definition of function and failure of current and future forms of β-cell replacement therapy. There was consensus that β-cell replacement therapy could be considered as a treatment for β-cell failure, regardless of etiology and without requiring undetectable C-peptide, accompanied by glycemic instability with either problematic hypoglycemia or hyperglycemia. Read More

    Treatment with medium chain fatty acids milk of CD36-deficient preschool children.
    Nutrition 2017 Nov 29;50:45-48. Epub 2017 Nov 29.
    Department of Clinical Laboratory Medicine, Juntendo University School of Medicine, Tokyo, Japan.
    Objective: CD36 deficiency is characterized by limited cellular long chain fatty acid uptake in the skeletal and cardiac muscles and often causes energy crisis in these muscles. However, suitable treatment for CD36 deficiency remains to be established. The aim of this study was to evaluate the clinical and metabolic effects of medium chain triacylglycerols (MCTs) in two CD36-deficient preschool children who often developed fasting hypoglycemia and exercise-induced myalgia. Read More

    Perspectives of Patients with Insulin-Treated Type 1 and Type 2 Diabetes on Hypoglycemia: Results of the HAT Observational Study in Central and Eastern European Countries.
    Diabetes Ther 2018 Apr 9;9(2):727-741. Epub 2018 Mar 9.
    Clinic for Endocrinology Diabetes and Metabolic Diseases, Clinical Center of Serbia (CCS), Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
    Introduction: The aim of this study was to determine the level of awareness of hypoglycemia, the level of fear for hypoglycemia, and the response to hypoglycemic events among insulin-treated diabetes patients from Central and Eastern Europe (CEE). The impact of hypoglycemia on the use of healthcare resources and patient productivity was also assessed.

    Methods: This was a multicenter, non-interventional, two-part, patient self-reported questionnaire study that comprised both a retrospective cross-sectional evaluation and a prospective observational evaluation. Read More

    Adrenaline and cortisol levels are lower during nighttime than daytime hypoglycaemia in children with type 1 diabetes.
    Acta Paediatr 2018 Mar 8. Epub 2018 Mar 8.
    Department of Paediatrics, CPH-Direct, Copenhagen University Hospital, Herlev and Gentofte, Herlev, Denmark.
    Aim: We investigated children's counter regulatory hormone profiles during a hyperinsulinaemic hypoglycaemic clamp procedure at day and night.

    Methods: In 2013, we assessed the counter regulatory response to hypoglycaemia in eight outpatients with type 1 diabetes, recruited from the Herlev Hospital, Denmark, at a mean age of 9.6 ± 2. Read More

    α Cell Function and Gene Expression Are Compromised in Type 1 Diabetes.
    Cell Rep 2018 Mar;22(10):2667-2676
    Department of Medicine, Division of Diabetes, Endocrinology and Metabolism, Vanderbilt University Medical Center, Nashville, TN, USA; Department of Molecular Physiology and Biophysics, Vanderbilt University, Nashville, TN, USA; Department of Veterans Affairs, Tennessee Valley Healthcare System, Nashville, TN, USA. Electronic address:
    Many patients with type 1 diabetes (T1D) have residual β cells producing small amounts of C-peptide long after disease onset but develop an inadequate glucagon response to hypoglycemia following T1D diagnosis. The features of these residual β cells and α cells in the islet endocrine compartment are largely unknown, due to the difficulty of comprehensive investigation. By studying the T1D pancreas and isolated islets, we show that remnant β cells appeared to maintain several aspects of regulated insulin secretion. Read More

    A phase 3 multicenter, open-label, prospective study designed to evaluate the effectiveness and ease of use of nasal glucagon in the treatment of moderate and severe hypoglycemia in children and adolescents with type 1 diabetes in the home or school setting.
    Pediatr Diabetes 2018 Mar 7. Epub 2018 Mar 7.
    Medical Development, Diabetes Business Unit, Eli Lilly Canada Inc., Toronto, Ontario, Canada.
    Objective: This multicenter, open-label study was designed to evaluate real-world effectiveness and ease of use of nasal glucagon (NG) in treating moderate or severe hypoglycemic events in children and adolescents with type 1 diabetes (T1D).

    Methods: Caregivers were trained to administer NG (3 mg) to the child/adolescent with T1D during spontaneous, symptomatic moderate or severe hypoglycemic events, observe treatment response (defined as awakening or returning to normal status within 30 minutes), and measure blood glucose (BG) levels every 15 minutes. Data regarding adverse events and ease of use were solicited using questionnaires. Read More

    Acute liver failure in neonates with undiagnosed hereditary fructose intolerance due to exposure from widely available infant formulas.
    Mol Genet Metab 2018 Apr 27;123(4):428-432. Epub 2018 Feb 27.
    Department of Human Genetics, School of Medicine, Emory University, Atlanta, GA, United States; Department of Pediatrics, School of Medicine, Emory University, Children's Healthcare of Atlanta, Atlanta, GA, United States.
    Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by aldolase B (ALDOB) deficiency resulting in an inability to metabolize fructose. The toxic accumulation of intermediate fructose-1-phosphate causes multiple metabolic disturbances, including postprandial hypoglycemia, lactic acidosis, electrolyte disturbance, and liver/kidney dysfunction. The clinical presentation varies depending on the age of exposure and the load of fructose. Read More

    Newborn Plasma Glucose Concentration Nadirs by Gestational-Age Group.
    Neonatology 2018 Mar 6;113(4):353-359. Epub 2018 Mar 6.
    Department of Pediatrics, Colorado School of Medicine, Aurora, Colorado, USA.
    Background: The glucose concentrations and times to nadir for newborns of all gestational ages when intrapartum glucose-containing solutions are not routinely provided are unknown.

    Objective: To characterize and compare patterns of initial glucose concentration nadirs by gestational-age groups.

    Methods: A cross-sectional cohort study of 1,366 newborns born in 1998 at the University of Arkansas for Medical Sciences, appropriate for gestational age, nonasphyxiated, nonpolycythemic, and not infants of diabetic mothers, were included. Read More

    Complex Phenotypes in Inborn Errors of Metabolism: Overlapping Presentations in Congenital Disorders of Glycosylation and Mitochondrial Disorders.
    Pediatr Clin North Am 2018 Apr;65(2):375-388
    Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium; Hayward Genetics Center, Tulane University Medical School, New Orleans, LA, USA. Electronic address:
    Congenital disorders of glycosylation (CDG) and mitochondrial disorders have overlapping clinical features, including central nervous system, cardiac, gastrointestinal, hepatic, muscular, endocrine, and psychiatric disease. Specific abnormalities orienting the clinician toward the right diagnostic approach include abnormal fat distribution, coagulation abnormalities, together with anticoagulation abnormalities, hyperinsulinism, and congenital malformations in CDG. Diabetes, sensorineural deafness, and depression are very rare in CDG but common in mitochondrial disease. Read More

    Inborn Errors of Metabolism with Myopathy: Defects of Fatty Acid Oxidation and the Carnitine Shuttle System.
    Pediatr Clin North Am 2018 Apr 28;65(2):317-335. Epub 2017 Dec 28.
    Department of Pediatrics, Division of Medical Genetics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, 4401 Penn Avenue, Pittsburgh, PA 15224, USA. Electronic address:
    Fatty acid oxidation disorders (FAODs) and carnitine shuttling defects are inborn errors of energy metabolism with associated mortality and morbidity due to cardiomyopathy, exercise intolerance, rhabdomyolysis, and liver disease with physiologic stress. Hypoglycemia is characteristically hypoketotic. Lactic acidemia and hyperammonemia may occur during decompensation. Read More

    Clinical heterogeneity of hyperinsulinism due to HNF1A and HNF4A mutations.
    Pediatr Diabetes 2018 Mar 1. Epub 2018 Mar 1.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
    Background: Dominant inactivating mutations in HNF1A and HNF4A have been described to cause hyperinsulinism (HI) before evolving to diabetes. However, information available in the literature regarding the clinical phenotype is limited.

    Objective: To report the prevalence of HNF1A and HNF4A mutations in a large cohort of children with HI, and to describe their genotypes and phenotypes. Read More

    A case of Donohue syndrome "Leprechaunism" with a novel mutation in the insulin receptor gene.
    Turk Pediatri Ars 2017 Dec 1;52(4):226-230. Epub 2017 Dec 1.
    Osmangazi University, Faculty of Medicine, Department of Pediatrics, Division of Neonatalogy, Eskişehir, Turkey.
    Donohue syndrome (Leprechaunism) is characterized by severe insulin resistance, hyperinsulinemia, postprandial hyperglycemia, preprandial hypoglycemia, intrauterine and postnatal growth retardation, dysmorphic findings, and clinical and laboratory findings of hyperandrogenemia due to homozygous or compound heterozygous inactivating mutations in the insulin receptor gene. A female newborn presented with lack of subcutaneous fat tissue, bilateral simian creases, hypertrichosis, especially on her face, gingival hypertrophy, cliteromegaly, and prominent nipples. Her laboratory tests revealed hyperandrogenism, postprandial hyperglycemia and preprandial hypoglycemia, and very high concurrent insulin levels. Read More

    Relationships Between Early Nutrition and Blood Glucose Concentrations in Very Preterm Infants.
    J Pediatr Gastroenterol Nutr 2018 Feb 23. Epub 2018 Feb 23.
    Newborn Services, Auckland City Hospital, Auckland, New Zealand.
    Objectives: To determine whether changes to early nutrition are associated with levels of glycaemia in very preterm infants.

    Methods: A retrospective, observational study of infants <1,500 grams or <30 weeks' gestation admitted to Neonatal Intensive Care, National Women's Hospital, NZ, before (Old Protocol) and after (New Protocol) a change in nutritional protocol. Nutritional intakes were calculated and averaged by day for postnatal days 1-7 (Week 1) and 1-28 (Month 1). Read More

    Increasing incidence of optic nerve hypoplasia/septo-optic dysplasia spectrum: Geographic clustering in Northern Canada.
    Paediatr Child Health 2017 Nov 19;22(8):445-453. Epub 2017 Nov 19.
    Section of Pediatric Endocrinology, Children's Hospital Winnipeg, University of Manitoba, Winnipeg, Manitoba.
    Introduction: Owing to the shared embryonic origin, defects in development of optic nerves are often seen in conjunction with defects affecting the surrounding brain and pituitary gland. Optic nerve hypoplasia (ONH) and septo-optic dysplasia (SOD) represent a clinical spectrum associated with visual, pituitary and severe central nervous system structural abnormalities (SODplus). Based on changing clinical patterns, our primary objective was to examine trends in annual incidence of ONH/SOD and geographical clustering in Manitoba. Read More

    Treatment of a case of severe insulin resistance as a result of a PIK3R1 mutation with a sodium-glucose cotransporter 2 inhibitor.
    J Diabetes Investig 2018 Feb 24. Epub 2018 Feb 24.
    Division of Diabetes and Endocrinology, Tokyo, Japan.
    A Japanese woman aged in her late 30s with severe insulin resistance and bodily features including a triangular face, prominent forehead, small chin, large and low-set ears, and ocular depression was investigated. A similar phenotype was not observed in other family members with the exception of her son, suggesting that the condition was caused by a de novo mutation that was transmitted from mother to son. Exome analysis showed the presence in the proband and her son of a c. Read More

    Factitious hypoglycemia in children and adolescents with diabetes.
    Pediatr Diabetes 2018 Feb 21. Epub 2018 Feb 21.
    Section of Pediatric Diabetes and Metabolism, DEOB, NIDDK, NIH, Bethesda, Maryland.
    Background: Factitious hypoglycemia is a condition of self-induced hypoglycemia due to surreptitious administration of insulin or oral hypoglycemic agents. In adults, it is an uncommon, but well known clinical entity observed in individuals with and without diabetes.

    Objectives: To report a case of factitious hypoglycemia highlighting diagnostic pitfalls, to identify common characteristics of children and adolescents with factitious hypoglycemia, and to examine whether the information on long-term outcome exists. Read More

    Patients' and caregivers' experiences of using continuous glucose monitoring to support diabetes self-management: qualitative study.
    BMC Endocr Disord 2018 Feb 20;18(1):12. Epub 2018 Feb 20.
    Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge, Cambridge, UK.
    Background: Continuous glucose monitoring (CGM) enables users to view real-time interstitial glucose readings and provides information on the direction and rate of change of blood glucose levels. Users can also access historical data to inform treatment decisions. While the clinical and psychological benefits of CGM are well established, little is known about how individuals use CGM to inform diabetes self-management. Read More

    Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan.
    Horm Res Paediatr 2018 16;89(3):166-171. Epub 2018 Feb 16.
    The Committee on Mass Screening, Japanese Society for Pediatric Endocrinology, Kyoto, Japan.
    Background/aims: We aimed to evaluate the incidence and characteristics of adrenal crisis in Japanese children with 21-hydroxylase deficiency (21-OHD).

    Methods: We conducted a retrospective nationwide survey for the councilors of the Japanese Society for Pediatric Endocrinology (JSPE) regarding adrenal crisis in children under 7 years with 21-OHD, admitted to hospitals from 2011 through 2016. We defined adrenal crisis as the acute impairment of general health due to glucocorticoid deficiency with at least two of symptoms, signs, or biochemical abnormalities. Read More

    Defining outcomes for β-cell replacement therapy in the treatment of diabetes: a consensus report on the Igls criteria from the IPITA/EPITA opinion leaders workshop.
    Transpl Int 2018 04;31(4):343-352
    Institute of Transplantation, The Freeman Hospital, Newcastle University, Newcastle upon Tyne, UK.
    β-cell replacement therapy, available currently as pancreas or islet transplantation, has developed without a clear definition of graft functional and clinical outcomes. The International Pancreas & Islet Transplant Association (IPITA) and European Pancreas & Islet Transplantation Association (EPITA) held a workshop to develop consensus for an IPITA/EPITA Statement on the definition of function and failure of current and future forms of β-cell replacement therapy. There was consensus that β-cell replacement therapy could be considered as a treatment for β-cell failure, regardless of etiology and without requiring undetectable C-peptide, accompanied by glycemic instability with either problematic hypoglycemia or hyperglycemia. Read More

    Regulation of K Channel Trafficking in Pancreatic β-Cells by Protein Histidine Phosphorylation.
    Diabetes 2018 May 12;67(5):849-860. Epub 2018 Feb 12.
    Division of Nephrology, New York University Langone Medical Center, New York, NY
    Protein histidine phosphatase 1 (PHPT-1) is an evolutionarily conserved 14-kDa protein that dephosphorylates phosphohistidine. mice were generated to gain insight into the role of PHPT-1 and histidine phosphorylation/dephosphorylation in mammalian biology. mice exhibited neonatal hyperinsulinemic hypoglycemia due to impaired trafficking of K channels to the plasma membrane in pancreatic β-cells in response to low glucose and leptin and resembled patients with congenital hyperinsulinism (CHI). Read More

    Well-Appearing Newborn With a Vesiculobullous Rash at Birth.
    Pediatrics 2018 Feb 6. Epub 2018 Feb 6.
    Pediatric Hospital Medicine, and.
    A term, appropriate-for-gestational-age, male infant born via normal spontaneous vaginal delivery presented at birth with a full-body erythematous, vesiculobullous rash. He was well-appearing with normal vital signs and hypoglycemia that quickly resolved. His father had a history of herpes labialis. Read More

    A Quality-Improvement Initiative to Reduce NICU Transfers for Neonates at Risk for Hypoglycemia.
    Pediatrics 2018 Feb 8. Epub 2018 Feb 8.
    General Pediatrics and Adolescent Medicine, Department of Pediatrics, School of Medicine, and.
    Background And Objective: Neonatal hypoglycemia is a common problem, often requiring management in the NICU. Nonpharmacologic interventions, including early breastfeeding and skin-to-skin care (SSC), may prevent hypoglycemia and the need to escalate care. Our objective was to maintain mother-infant dyads in the mother-infant unit by decreasing hypoglycemia resulting in NICU transfer. Read More

    Safety and Feasibility of the OmniPod Hybrid Closed-Loop System in Adult, Adolescent, and Pediatric Patients with Type 1 Diabetes Using a Personalized Model Predictive Control Algorithm.
    Diabetes Technol Ther 2018 Apr 12;20(4):257-262. Epub 2018 Feb 12.
    7 Insulet Corporation , Billerica, Massachusetts.
    Background: The safety and feasibility of the OmniPod personalized model predictive control (MPC) algorithm in adult, adolescent, and pediatric patients with type 1 diabetes were investigated.

    Methods: This multicenter, observational trial included a 1-week outpatient sensor-augmented pump open-loop phase and a 36-h inpatient hybrid closed-loop (HCL) phase with announced meals ranging from 30 to 90 g of carbohydrates and limited physical activity. Patients aged 6-65 years with HbA1c between 6. Read More

    Metabolic control and complications in Italian people with diabetes treated with continuous subcutaneous insulin infusion.
    Nutr Metab Cardiovasc Dis 2018 Apr 9;28(4):335-342. Epub 2017 Dec 9.
    Metabolic Diseases, Department of Medicine-DIMED, University of Padua, Padua, Italy. Electronic address:
    Background And Aim: The objective of this cross-sectional study was to evaluate the degree of glycaemic control and the frequency of diabetic complications in Italian people with diabetes who were treated with continuous subcutaneous insulin infusion (CSII).

    Methods And Results: Questionnaires investigating the organisation of diabetes care centres, individuals' clinical and metabolic features and pump technology and its management were sent to adult and paediatric diabetes centres that use CSII for treatment in Italy. Information on standard clinical variables, demographic data and acute and chronic diabetic complications was derived from local clinical management systems. Read More

    Severe insulin resistance in disguise: A familial case of reactive hypoglycemia associated with a novel heterozygous INSR mutation.
    Pediatr Diabetes 2018 Feb 7. Epub 2018 Feb 7.
    Department of Experimental Medicine and Surgery, University of Rome Tor Vergata, Rome, Italy.
    Aim: Hypoglycemia in childhood is very rare and can be caused by genetic mutations or insulin-secreting neoplasms. Postprandial hypoglycemia has previously been associated with insulin receptor (INSR) gene mutations. We aimed to identify the cause of postprandial hypoglycemia in a 10-year-old boy. Read More

    Automatic Detection and Estimation of Unannounced Meals for Multivariable Artificial Pancreas System.
    Diabetes Technol Ther 2018 Mar 6;20(3):235-246. Epub 2018 Feb 6.
    1 Department of Chemical and Biological Engineering, Illinois Institute of Technology , Chicago, Illinois.
    Background: Automatically attenuating the postprandial rise in the blood glucose concentration without manual meal announcement is a significant challenge for artificial pancreas (AP) systems. In this study, a meal module is proposed to detect the consumption of a meal and to estimate the amount of carbohydrate (CHO) intake.

    Methods: The meals are detected based on qualitative variables describing variation of continuous glucose monitoring (CGM) readings. Read More

    Hyperinsulinism in the Neonate.
    Clin Perinatol 2018 Mar 6;45(1):61-74. Epub 2017 Dec 6.
    The Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA. Electronic address:
    Hyperinsulinism (HI) is the leading cause of persistent hypoglycemia in infants. Prompt recognition and treatment, independent of whether infants have transient or permanent HI, are essential to decrease risk of neurologic damage. The most common form of congenital HI is due to inactivating mutations of the β-cell ATP-sensitive potassium (K) channel (K-HI) and is typically diazoxide unresponsive. Read More

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