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    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 Feb 16. 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 Feb 17. Epub 2018 Feb 17.
    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

    Regulation of KChannel Trafficking in Pancreatic β Cells by Protein Histidine Phosphorylation.
    Diabetes 2018 Feb 12. Epub 2018 Feb 12.
    Division of Nephrology,
    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 Kchannels 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 Feb 12. 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 2017 Dec 9. 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

    New Human Combined Immunodeficiency Due to IRF4 Deficiency Inherited by Uniparental Isodisomy.
    J Allergy Clin Immunol 2018 Feb 2. Epub 2018 Feb 2.
    Department of Immunology, Hospital Universitario La Paz, Madrid 28046, Spain; Lymphocyte Pathophysiology Group, La Paz Institute of Biomedical Research, IdiPAZ, Madrid, 28046, Spain.
    Background: Interferon regulatory factor 4 (IRF4) is a fundamental transcription factor in adaptive and innate immunity, due to its key role in the differentiation and functional specialization of lymphoid and myeloid lineage cells. In mouse models, IRF4 participates in bone marrow central tolerance, naïve B cell activation, germinal centre formation, plasma cell differentiation, immunoglobulin secretion, T helper subset differentiation, macrophage polarization, and dendritic cell differentiation, among other processes.

    Objective: To describe the first case of autosomal recessive human IRF4 deficiency. Read More

    Automatic Detection and Estimation of Unannounced Meals for Multivariable Artificial Pancreas System.
    Diabetes Technol Ther 2018 Feb 6. 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

    Impact of late pregnancy haemoglobin Aat 29-30 weeks' gestation on adverse pregnancy outcomes among women with pre-existing diabetes: a retrospective analysis.
    J Obstet Gynaecol 2018 Feb 1:1-5. Epub 2018 Feb 1.
    a Department of Obstetrics and Gynaecology , Universiti Kebangsaan Malaysia , Kuala Lumpur , Malaysia.
    This study was to assess the relationship between late pregnancy haemoglobin A(HbA) at 29-30 weeks of gestation and adverse pregnancy outcomes (APOs) in 272 pregnant women with pre-existing diabetes. HbA≥6.1% was associated with significantly increased risk of preterm delivery, Caesarean section, large for gestational age (LGA), neonatal respiratory distress, neonatal hypoglycaemia, and composite adverse neonatal outcome (p < . Read More

    Acute gastroenteritis: evidence-based management of pediatric patients [digest].
    Pediatr Emerg Med Pract 2018 Feb 1;15(2 Suppl Points & Pearls):1-2. Epub 2018 Feb 1.
    Pediatric Emergency Medicine Ultrasound Fellow, Department of Emergency Medicine, Stanford University School of Medicine, Lucile Packard Children's Hospital, Palo Alto, CA.
    Although most cases of acute gastroenteritis require minimal medical intervention, severe dehydration and hypoglycemia may develop in cases of prolonged vomiting and diarrhea. The mainstay of treatment for mild-to-moderately dehydrated patients with acute gastroenteritis should be oral rehydration solution. Antiemetics allow for improved tolerance of oral rehydration solution, and, when used appropriately, can decrease the need for intravenous fluids and hospitalization. Read More

    Randomised controlled trial of diazoxide for small for gestational age neonates with hyperinsulinaemic hypoglycaemia provided early hypoglycaemic control without adverse effects.
    Acta Paediatr 2018 Jan 31. Epub 2018 Jan 31.
    Neonatal unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
    Aim: Hyperinsulinaemic hypoglycaemia (HH) is a very common cause of hypoglycaemia in small for gestational age (SGA) neonates. We compared using early oral diazoxide or a placebo for this patient group.

    Methods: This was a randomised, double-blind, placebo-controlled trial that focused on SGA neonates born at at least 32 weeks of gestation with HH during the first five days of life. Read More

    Expert consensus document: Clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement.
    Nat Rev Endocrinol 2018 Jan 29. Epub 2018 Jan 29.
    Department of Medical Genetics, University of Cambridge and National Institute for Health Research (NIHR) Cambridge Biomedical Research Centre and Cancer Research UK Cambridge Centre, Cambridge Biomedical Campus, Cambridge, CB2 0QQ, UK.
    Beckwith-Wiedemann syndrome (BWS), a human genomic imprinting disorder, is characterized by phenotypic variability that might include overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycaemia, lateralized overgrowth and predisposition to embryonal tumours. Delineation of the molecular defects within the imprinted 11p15.5 region can predict familial recurrence risks and the risk (and type) of embryonal tumour. Read More

    [TRMU MUTATIONS - REVERSIBLE INFANTILE LIVER FAILURE OR MULTISYSTEM DISORDER?]
    Harefuah 2018 Jan;157(1):52-57
    Pediatrics A, Schneider Children's Medical Center of Israel.
    Introduction: Bi-allelic mutations in the TRMU gene cause reversible infantile liver failure. Little is known about extra-hepatic manifestations in these patients.

    Background: Two infants, aged 4 and 5 months, presented with progressive life threatening liver failure, characterized by lactic acidosis, highly elevated alpha-fetoprotein and recurrent hypoglycemia. Read More

    Late presentation of glycogen storage disease types Ia and III in children with short stature and hepatomegaly.
    J Pediatr Endocrinol Metab 2018 Jan 29. Epub 2018 Jan 29.
    Department of Pediatrics, Naval Medical Center Portsmouth, Portsmouth, VA, USA.
    Background: Glycogen storage diseases (GSDs) are a collection of disorders related to glycogen synthesis or degradation that classically present in infancy with hypoglycemia, failure to thrive and hepatomegaly; however, their phenotype can vary significantly.

    Case Presentation: We present the cases of two children, 5 years old and 3.5 years old, who were referred to endocrinology for short stature. Read More

    International practices in the dietary management of fructose 1-6 biphosphatase deficiency.
    Orphanet J Rare Dis 2018 Jan 25;13(1):21. Epub 2018 Jan 25.
    Birmingham Women's and Children's Hospital, Birmingham, UK.
    Background: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. Read More

    Acute gastroenteritis: evidence-based management of pediatric patients.
    Pediatr Emerg Med Pract 2018 Feb 1;15(2):1-25. Epub 2018 Feb 1.
    Director of Pediatric Emergency Medicine Education, Attending Physician, Department of Emergency Medicine, New York-Presbyterian/Queens; Instructor of Emergency Medicine in Clinical Pediatrics, Weill Cornell Medical College, New York,NY.
    Although most cases of acute gastroenteritis require minimal medical intervention, severe dehydration and hypoglycemia may develop in cases of prolonged vomiting and diarrhea. The mainstay of treatment for mild-to-moderately dehydrated patients with acute gastroenteritis should be oral rehydration solution. Antiemetics allow for improved tolerance of oral rehydration solution, and, when used appropriately, can decrease the need for intravenous fluids and hospitalization. Read More

    Optimizing the management of hypoglycaemia in individuals with type 2 diabetes: A randomized crossover comparison of a weight-based protocol compared with two fixed-dose glucose regimens.
    Diabetes Obes Metab 2018 Jan 23. Epub 2018 Jan 23.
    Centre for Endocrine, Diabetes and Obesity Research (CEDOR), Capital and Coast District Health Board, Wellington, New Zealand.
    Aims: To determine whether an individualized body weight-based glucose treatment in adults with type 2 diabetes (T2DM) is more likely to resolve hypoglycaemia with a single treatment without excessive rebound hyperglycaemia compared to fixed doses of 12 or 30 g of glucose.

    Methods: Adults with T2DM were enrolled in a cross-over study. Each episode of hypoglycaemia (capillary glucose <4. Read More

    A case of CHARGE syndrome associated with hyperinsulinemic hypoglycemia in infancy.
    Eur J Med Genet 2018 Jan 20. Epub 2018 Jan 20.
    Department of Pediatrics, Oita University Faculty of Medicine, Oita, Japan. Electronic address:
    CHARGE syndrome is a rare autosomal dominant disorder involving multiple organs. Chromodomain helicase DNA binding protein-7 (CHD7) is a major causative gene of CHARGE syndrome. We herein report a male infant born at full term with asphyxia who was diagnosed with CHARGE syndrome based on the typical anomalies. Read More

    Deficient glucagon response to hypoglycemia during a mixed meal in total pancreatectomy/islet autotransplantation recipients.
    J Clin Endocrinol Metab 2018 Jan 17. Epub 2018 Jan 17.
    Pacific Northwest Diabetes Research Institute, Seattle, WA.
    Context: Total pancreatectomy and intrahepatic islet autotransplantation (TP/IAT) is performed to alleviate severe abdominal pain, avoid narcotic use, maintain islet function, and avoid diabetes in patients with chronic pancreatitis. However, many TP/IAT recipients complain of post-prandial hypoglycemia.

    Objective: This study was designed to discover mechanisms of this problem. Read More

    Neonatal hypoglycemia: continuous glucose monitoring.
    Curr Opin Pediatr 2018 Jan 17. Epub 2018 Jan 17.
    Liggins Institute.
    Purpose Of Review: Continuous glucose monitoring (CGM) is increasingly used in the management of diabetes in children and adults, but there are few data regarding its use in neonates. The purpose of this article is to discuss the potential benefits and limitations of CGM in neonates.

    Recent Findings: Smaller electrodes in new sensors and real-time monitoring have made CGM devices more approachable for neonatal care. Read More

    Unexpected Management Behaviors in Adolescents With Type 1 Diabetes Using Sensor-Augmented Pump Therapy.
    J Diabetes Sci Technol 2018 Jan 1:1932296817752188. Epub 2018 Jan 1.
    1 Children's Diabetes Centre, Telethon Kids Institute, The University of Western Australia, Perth, Australia.
    Background: Continuous glucose monitoring can improve glycemic outcomes in individuals with type 1 diabetes. However, the constant exposure to real-time glucose levels can sometimes lead the individual to make some risky choices to address the glycemic excursions. Hence, the purpose of this study was to explore the aberrant management behaviors of youth with type 1 diabetes on sensor-augmented pump therapy (SAPT). Read More

    Congenital Hyperinsulinism and Hypopituitarism Attributable to a Novel Mutation in FOXA2.
    J Clin Endocrinol Metab 2018 Jan 10. Epub 2018 Jan 10.
    Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
    Context: Persistent hypoglycemia in the newborn period most commonly occurs due to hyperinsulinism. The phenotype of hypoketotic hypoglycemia can also result from pituitary hormone deficiencies, including growth hormone and adrenocorticotropic hormone deficiency. Forkhead box A2 (Foxa2) is a transcription factor shown in mouse models to influence insulin secretion by pancreatic beta cells. Read More

    Mortality impact of an increased blood glucose cut-off level for hypoglycaemia treatment in severely sick children in Malawi (SugarFACT trial): study protocol for a randomised controlled trial.
    Trials 2018 Jan 11;19(1):33. Epub 2018 Jan 11.
    Global Health - Health System and Policy Research Group, Department of Public Health Sciences, Karolinska Institutet, 171 77, Stockholm, Sweden.
    Background: Mortality in children remains high in sub-Saharan African hospitals. While antimalarial drugs, antibiotics and other definitive treatments are well understood, the role of emergency care with supportive therapies, such as maintaining normal glucose and electrolyte balances, has been given limited attention. Hypoglycaemia is common in children admitted to hospital in low-income settings. Read More

    Loss of the Mitochondrial Fatty Acid β-Oxidation Protein Medium-Chain Acyl-Coenzyme A Dehydrogenase Disrupts Oxidative Phosphorylation Protein Complex Stability and Function.
    Sci Rep 2018 Jan 9;8(1):153. Epub 2018 Jan 9.
    Centre for Genetic Diseases, Hudson Institute of Medical Research, 3168, Melbourne, Australia.
    Medium-chain acyl-Coenzyme A dehydrogenase (MCAD) is involved in the initial step of mitochondrial fatty acid β-oxidation (FAO). Loss of function results in MCAD deficiency, a disorder that usually presents in childhood with hypoketotic hypoglycemia, vomiting and lethargy. While the disruption of mitochondrial fatty acid metabolism is the primary metabolic defect, secondary defects in mitochondrial oxidative phosphorylation (OXPHOS) may also contribute to disease pathogenesis. Read More

    Efficacy of automatic bolus calculator with automatic speech recognition in patients with type 1 diabetes: A randomized cross-over trial.
    J Diabetes 2018 Jan 8. Epub 2018 Jan 8.
    Department of Pediatrics, Institute of Mother and Child, Warsaw, Poland.
    Background: Patients using an insulin pump as part of their diabetes treatment need to calculate insulin bolus doses to compensate for a meal. Some patients do not modify their meal boluses according to changes in the amount and composition of food products in a meal. The lack of correct meal boluses leads to unstable, and therefore harmful, blood glucose levels. Read More

    Recurrent hypoglycaemia in a toddler on β-blocker therapy.
    Cardiol Young 2018 Mar 8;28(3):511-513. Epub 2018 Jan 8.
    Department of Pediatrics,Centre Hospitalier Universitaire de Sherbrooke,Sherbrooke,Quebec,Canada.
    Hypoglycaemia is a well-known side effect of Propranolol. We described the case of a child presenting severe and recurrent Propranolol-induced hypoglycaemia. Those episodes were not related to prolonged fasting and were associated with only mild ketosis. Read More

    Neonatal hypoglycemia, early-onset diabetes and hypopituitarism due to the mutation in EIF2S3 gene causing MEHMO syndrome.
    Physiol Res 2018 Jan 5. Epub 2018 Jan 5.
    Department of Pediatrics, Medical Faculty of Comenius University, Bratislava, Slovakia, DIABGENE & Laboratory of Diabetes and Metabolic Disorders, Institute of Experimental Endocrinology, Biomedical Research Center, Slovak Academy of Sciences, Bratislava, Slovakia. and
    Recently, the genetic cause of several syndromic forms of glycemia dysregulation has been described. One of them, MEHMO syndrome, is a rare X-linked syndrome recently linked to the EIF2S3 gene mutations. MEHMO is characterized by Mental retardation, Epilepsy, Hypogonadism/hypogenitalism, Microcephaly, and Obesity. Read More

    Fifteen-minute consultation: insulin pumps for type 1 diabetes in children and young people.
    Arch Dis Child Educ Pract Ed 2018 Jan 3. Epub 2018 Jan 3.
    Department of Paediatric Endocrinology, Royal Hospital for Sick Children, Edinburgh, UK.
    There is increasing worldwide use of continuous subcutaneous insulin infusions in paediatric type 1 diabetes (T1D), reflecting recent research outcomes and guidance, as well as families' wishes. Children/young people may present acutely with medical or surgical problems, in addition to issues related to T1D. This review provides general paediatricians with an introduction to pump therapy, highlighting common problems, management issues and when to seek specialist advice. Read More

    Weight-based carbohydrate treatment of hypoglycaemia in people with Type 1 diabetes using insulin pump therapy: a randomized crossover clinical trial.
    Diabet Med 2018 Mar 20;35(3):339-346. Epub 2018 Jan 20.
    Endocrine, Diabetes and Research Centre, Capital and Coast District Health Board, Wellington, New Zealand.
    Aim: To test whether weight-based treatment is more effective than usual care in people with Type 1 diabetes receiving continuous subcutaneous insulin infusion therapy with regard to both hypoglycaemia and avoiding excessive rebound hyperglycaemia.

    Methods: Children and adults on continuous subcutaneous insulin infusion were enrolled into a study with a crossover design. Each episode of hypoglycaemia (defined as capillary glucose <4. Read More

    Increased IRS2 mRNA Expression in SGA Neonates: PCR Analysis of Insulin/IGF Signaling in Cord Blood.
    J Endocr Soc 2017 Dec 5;1(12):1408-1416. Epub 2017 Oct 5.
    Division of Pediatrics and Perinatology, Tottori University Faculty of Medicine, Yonago, Japan 683-8504.
    Context: Hypoglycemia is the most common metabolic problem among small-for-gestational-age (SGA) neonates. However, the pathological mechanism and insulin/ insulin-like growth factor (IGF) signaling axis in neonates remain unknown.

    Objective: To determine the insulin/IGF axis in neonates, we analyzed the messenger RNA (mRNA) expression of insulin/IGF signaling in fetal umbilical cord blood. Read More

    Type 1 diabetes does not impair the physical capacity of non-sedentary adolescents.
    Diabetol Metab Syndr 2017 16;9:100. Epub 2017 Dec 16.
    Departamento of Pediatrics, CTIP-Centro de Terapia Intensiva Pediátrica, Hospital Israelita Albert Einstein, Av. Albert Einstein, 627-701, São Paulo, 05651-901 Brazil.
    Background: Type 1 diabetes patients have a higher risk of developing hypoglycemia or hyperglycemia during physical activity, which may compromise their safety during exercise but results regarding the exercise capacity of patients with type 1 DM when compared to control subjects have been contradictory.

    Aim: To evaluate if type 1 diabetes affects the capacity of adolescents to exercise.

    Methods: The study enrolled 37 adolescents in stage 2-4 of the Tanner scale, aged from 10 to 14 years, 21 with type 1 diabetes and 16 without any chronic diseases. Read More

    Glucose-responsive insulin delivery for type 1 diabetes: The artificial pancreas story.
    Int J Pharm 2017 Dec 16. Epub 2017 Dec 16.
    Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge, Cambridge, United Kingdom; Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom.
    Insulin replacement therapy is integral to the management of type 1 diabetes, which is characterised by absolute insulin deficiency. Optimal glycaemic control, as assessed by glycated haemoglobin, and avoidance of hyper- and hypoglycaemic excursions have been shown to prevent diabetes-related complications. Insulin pump use has increased considerably over the past decade with beneficial effects on glycaemic control, quality of life and treatment satisfaction. Read More

    Mice with an Oncogenic HRAS Mutation are Resistant to High-Fat Diet-Induced Obesity and Exhibit Impaired Hepatic Energy Homeostasis.
    EBioMedicine 2018 Jan 6;27:138-150. Epub 2017 Dec 6.
    Department of Medical Genetics, Tohoku University School of Medicine, Sendai, Japan. Electronic address:
    Costello syndrome is a "RASopathy" that is characterized by growth retardation, dysmorphic facial appearance, hypertrophic cardiomyopathy and tumor predisposition. >80% of patients with Costello syndrome harbor a heterozygous germline G12S mutation in HRAS. Altered metabolic regulation has been suspected because patients with Costello syndrome exhibit hypoketotic hypoglycemia and increased resting energy expenditure, and their growth is severely retarded. Read More

    Camp-based multi-component intervention for families of young children with type 1 diabetes: A pilot and feasibility study.
    Pediatr Diabetes 2017 Dec 15. Epub 2017 Dec 15.
    Division of Endocrinology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas.
    Background: Managing type 1 diabetes mellitus (T1DM) in preschool-aged children has unique challenges that can negatively impact glycemic control and parental coping.

    Objective: To evaluate the impact of a camp-based multi-component intervention on glycated hemoglobin A1c (HbA1c) in young children with T1DM and psychosocial measures for their parents.

    Subjects And Methods: Two separate cohorts of 18 children (ages 3-5 years) and their families participated in a camp-based intervention that included didactic and interactive parent education, child-centered education and family-based recreational activities. Read More

    Images fromF-DOPA Scan in Congenital Hyperinsulinism: Not Always a Clue for Diagnosis.
    Nucl Med Mol Imaging 2017 Dec 14;51(4):362-363. Epub 2016 Jul 14.
    Inherited Metabolic Diseases Unit, Department of Paediatrics, Regional Centre for Newborn Screening, Diagnosis and Treatment of Inherited Metabolic Diseases and Congenital Endocrine Diseases, Azienda Ospedaliera Universitaria Integrata, Piazzale Ludovico Antonio Scuro, 10, 37134 Verona, Italy.
    Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in childhood (Horm Res 70:65-72, 2008; J Clin Endocr Metab 93:869-875, 2008).Fluoro-L-dihydroxy-phenylalanine (F-DOPA) positron emission tomography (PET) can detect areas of increased activity in the pancreas and may differentiate focal from diffuse CHI (J Clin Endocr Metab 93:869-875, 2008; Radiology 253:216-222, 2009). We here report the case of a girl who complained of recurrent episodes of severe hypoglycaemia despite previous partial pancreatectomy. Read More

    A Multicenter Experience with Long-Acting Somatostatin Analogues in Patients with Congenital Hyperinsulinism.
    Horm Res Paediatr 2017 Dec 14. Epub 2017 Dec 14.
    Department of Pediatric Endocrinology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
    Background/aims: Congenital hyperinsulinism (CHI) is a rare disease characterized by recurrent severe hypoglycemia. In the diffuse form of CHI, pharmacotherapy is the preferred choice of treatment. Long-acting somatostatin analogues have been used in children as off-label medication. Read More

    Admission of term infants to the neonatal intensive care unit in a Saudi tertiary teaching hospital: cumulative incidence and risk factors.
    Ann Saudi Med 2017 Nov-Dec;37(6):420-424
    Dr. Heidi Al-Wassia, Department of Pediatrics,, King Abdulaziz University,, Jeddah 80215, Saudi Arabia, T: +966-12- 6401000, ext 20208, http://orcid.org/0000-0002-8208-4986.
    Background: An increasing number of term infants of appropriate birthweight receive care in neonatal intensive care units (NICUs).

    Objectives: This study assessed the prevalence, patterns, and risk factors for admission of term infants to a NICU to identify areas for quality improvement.

    Design: Cross-sectional analytical study. Read More

    Asthma in children and adolescents with type 1 diabetes in Germany and Austria: Frequency and metabolic control.
    Pediatr Diabetes 2017 Dec 8. Epub 2017 Dec 8.
    Institute of Epidemiology and Medical Biometry, University of Ulm, ZIBMT, Ulm, Germany.
    Objective: To investigate the prevalence of asthma in young patients with type 1 diabetes mellitus (T1D) from Austria and Germany and its influence on their metabolic control.

    Methods: This prospective, multicenter observational cohort study was based on the DPV-registry (German/Austrian DPV initiative) including 51 926 patients with T1D (<20 years). All clinical data were documented prospectively. Read More

    Risk of recurrent severe hypoglycemia remains associated with a past history of severe hypoglycemia up to 4 years: Results from a large prospective contemporary pediatric cohort of the DPV initiative.
    Pediatr Diabetes 2017 Dec 8. Epub 2017 Dec 8.
    Institute of Epidemiology and Medical Biometry, ZIBMT, University of Ulm, Ulm, Germany.
    Objectives: In a contemporary cohort of youth with type 1 diabetes, we examined the interval between episodes of severe hypoglycemia (SH) as a risk factor for recurrent SH or hypoglycemic coma (HC).

    Methods: This was a large longitudinal observational study. Using the DPV Diabetes Prospective follow-up data, we analyzed frequency and timing of recurrent SH (defined as requiring assistance from another person) and HC (loss of consciousness or seizures) in 14 177 youths with type 1 diabetes aged <20 years and at least 5 years of follow-up. Read More

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