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Transient Hyperinsulinism

Transient Neonatal Hyperinsulinemic Hypoglycemia And Neurological Outcome: A Case Report.

Transient Neonatal Hyperinsulinemic Hypoglycemia And Neurological Outcome: A Case Report.

Transient neonatal hyperinsulinemic hypoglycemia and neurological outcome: a case report. Department of Child Neuropsychiatry, C. Arrigo Hospital, Alessandria, Italy. [email protected] Transient neonatal hyperinsulinemic hypoglycemia (TNHI) is a form of neonatal-onset hyperinsulinism which usually resolves completely in a few days or months. It is secondary to conditions such as maternal diabetes mellitus or intra-uterine growth retardation. Other rare causes of TNHI are perinatal asphyxia and gestational diabetes. Hyperinsulinemic hypoglycemia (HI) is also observed in association with rare metabolic or genetic conditions. It can also occur in newborns without risk factors. TNHI is usually a transient phenomenon. However, some newborns can have prolonged HI that requires treatment with diazoxide, persists for several months and then resolves spontaneously. Neonatal hyperinsulinemic hypoglycemia must be promptly and correctly diagnosed and treated in order to avoid neurological consequences. We describe a case of transient neonatal hyperinsulinemic hypoglycemia in a full-term born without perinatal complications and appropriate for gestational age with an unfavourable neurological outcome. Continue reading >>

Glucose Metabolism In A Term Infant With Transient Hyperinsulinism

Glucose Metabolism In A Term Infant With Transient Hyperinsulinism

GLUCOSE METABOLISM IN A TERM INFANT WITH TRANSIENT HYPERINSULINISM Pediatric Research volume 32, page 617 (1992) Large amounts of glucose have to be infused in infants with transient hyperinsulinism. The metabolism of the high glucose intake has not been determined. We studied glucose metabolism in an infant with transient hyperinsulinism without maternal diabetes. Gest. age 41 wks, birthweight 3475 g. Hypoglycemia was observed on day 1 (glucose 0,2 mmol/l with convulsions. Insulin levels decreased from 90 mU/l on day 1 to 7 on day 11. Glucose metabolism was measured by indirect caloriuetry (ID) and stable isotope technique (U - 13C glucose) on day 6 and 11. CHO int. = total carbohydrate intake, MR = metabolic rate, RQ = resp. quotient, CHO util. = carbohydrate utilization, measured by ID, Ra = rate of appearance of glucose, Glue. oxid. = glucose oxid. measured by U-13 C glucose, NOD = non-oxidative glue, disposal. Conclusions: 1. Glucose oxidation was not increased despite high glucose intake. 2. Hypoglycemia is not due to increased oxidation but to non-oxidative disposal. 3. There is a high conversion of glucose into fat. Dept. of Peds. Sophia Childrens Hospital, Rotterdam, The Netherlands, Rainbow Babies Hosp. Cleveland, USA. Continue reading >>

Transient Neonatal Hyperinsulinism With Adaptation Disorders: A Report Of Three Cases

Transient Neonatal Hyperinsulinism With Adaptation Disorders: A Report Of Three Cases

Palladino AA, Bennett MJ, Stanley CA. Hyperinsulinism in infancy and childhood: when an insulin level is not always high. Clin Chem 2008;54:25663. Crossref GoogleScholar Collins JE, Leonard JV. Hyperinsulinism in asphyxiated and small-for-dates infants with hypoglycaemia. Lancet 1984;11:3113. Crossref GoogleScholar Mandy GT. Small for gestational age infant. In: Weisman LE, editor. UpToDate, Waltham, MA: UpToDate, Accessed on May 1, 2014. GoogleScholar Watts T, Roberts T. Haematological abnormalities in the growth-restricted infant. Semin Neonatol 1999;4:4154. Crossref GoogleScholar Grosu D, Noel E, Andres E. Life-threatening pancytopenia associated with diazoxide. Eur J Intern Med 2004;15:402. Crossref PubMed GoogleScholar Silvani P, Camporesi A, Mandelli A, Wolfler A, Salvo I. A case of severe diazoxide toxicity. Pediatr Anaesth 2004;14:6079. Crossref GoogleScholar Yoshida K, Kawai M, Marumo C, Kanazawa H, Matsukura T, et al. High prevalence of severe circulatory complications with diazoxide in premature infants. Neonatology 2014;105:16671. Crossref WebofScience PubMed GoogleScholar Corresponding author: Hiroshi Mizumoto, MD, Department of Pediatrics, Kitano Hospital Tazuke Kofukai, Medical Research Institute, 2-4-20 Ohgimachi, Kita-ku, Osaka 530-8480, Japan, Phone: +81-6-6312-1221, Fax: +81-6-6361-0588, E-mail: Continue reading >>

Neonatal Transient Idiopathic Hyperinsulinism: Case Report

Neonatal Transient Idiopathic Hyperinsulinism: Case Report

Neonatal Transient Idiopathic Hyperinsulinism: Case Radouani MA, Azzaoui S, Kabiri M and Amina *Department of Medicine and Neonatal Resuscitation, National Center for Neonatology and Nutrition, Morocco *Corresponding author: Amina Barkat, Department of Medicine and Neonatal Resuscitation, National Center for Neonatology and Nutrition, Childrens Hospital, Ibn Sina hospital, Ibn Rushd bd, 10100 Souissi, Rabat, Morocco, Tel: 0661385108; Email: Received: November 7, 2014 | Published: March 12, 2015 The hyperinsulinism (HI) is a common cause of hypoglycemia in the newborn. Inappropriate insulin secretion by pancreatic beta cells is responsible for severe hypoglycaemia with a high risk of brain damage. It is usually found in hypotrophic newborns and in newborns of diabetic mothers by hyperinsulinemia or secondary to an increase in anti-stress hormones. Syndromic HIs are responsible for permanent hypoglycemia. The diagnosis of idiopathic HI can be done by high insulin and C-peptide in the blood with normal pancreatic and brain imaging and a negative balance metabolic and genetic levels. We report a case of a newborn who has severe idiopathic transient hypoglycemia. This newborn male was two days old admitted for dehydration with hypoglycemia. The mother is 32 years old, primiparous blood type A positive. The pregnancy was without vaginal with immediate cry. Birth weight is 2700g. Clinical examination showed, weight W = 2500g, size S = 47cm, head circumference HC 34 cm, temperature T = 37 , heart rate HR = 160 b / m, Blood pressure BP = 55/30, blood glucose capillary GC: 0.13 g / l. The patient is polypneic, hypotonic with a fold dehydration and sunken eyes. The review noted no facial dysmorphia or micropenis and no hepatomegaly. The remainder The newborn had received a saline Continue reading >>

Congenital Hyperinsulinism

Congenital Hyperinsulinism

General Discussion Congenital hyperinsulinism (HI) is the most frequent cause of severe, persistent hypoglycemia in newborn babies, infants, and children. In most countries it occurs in approximately 1/25,000 to 1/50,000 births. About 60% of babies with HI are diagnosed during the first month of life. An additional 30% will be diagnosed later in the first year and the remainder after that. With early treatment and aggressive prevention of hypoglycemia, brain damage can be prevented. However, brain damage can occur in children with HI if the condition is not recognized or if treatment is ineffective in the prevention of hypoglycemia. Insulin is the most important hormone for controlling the concentration of glucose in the blood. As food is eaten, blood glucose rises and the pancreas secretes insulin to keep blood glucose in the normal range. Insulin acts by driving glucose into the cells of the body. This action of insulin maintains blood glucose levels and stores glucose as glycogen in the liver. Once feeding is completed and glucose levels fall, insulin secretion is turned off, allowing the stores of glucose in glycogen to be released into the bloodstream to keep blood glucose normal. In addition, with the switching off of insulin secretion, protein and fat stores become accessible and can be used instead of glucose as sources of fuel. In this manner, whether one eats or is fasting blood glucose levels remain in the normal range and the body has access to energy at all times. This close regulation of blood glucose and insulin secretion does not occur normally in people who have HI. The beta cells in the pancreas, which are responsible for insulin secretion, are blind to the blood glucose level and secrete insulin regardless of the blood glucose concentration. As a resu Continue reading >>

Fetal Erythroblastosis May Be An Indicator Of Neonatal Transient Hyperinsulinism

Fetal Erythroblastosis May Be An Indicator Of Neonatal Transient Hyperinsulinism

Fetal Erythroblastosis May Be an Indicator of Neonatal Transient Hyperinsulinism Mizumoto H.a Iki Y.a Yamashita S.a Kawai M.b Katayama T.c Hata D.a I have read the Karger Terms and Conditions and agree. I have read the Karger Terms and Conditions and agree. Buy a Karger Article Bundle (KAB) and profit from a discount! If you would like to redeem your KAB credit, please log in . Save over 20% compared to the individual article price. Buy Cloud Access for unlimited viewing via different devices Access to all articles of the subscribed year(s) guaranteed for 5 years Unlimited re-access via Subscriber Login or MyKarger Unrestricted printing, no saving restrictions for personal use * The final prices may differ from the prices shown due to specifics of VAT rules. For additional information: Background: Small for gestational age and birth asphyxia are associated with neonatal transient hyperinsulinism (THI). Some newborns with THI showed marked erythroblastosis on admission to our neonatal intensive care unit. Objective: This study was designed to test our hypothesis that fetal erythroblastosis may be a risk factor for developing THI. Methods: The records of all babies admitted to our neonatal intensive care unit within 24 h of birth between January 2010 and May 2014, and who were born after 34 weeks of gestation, were retrospectively reviewed. Hyperinsulinism was diagnosed as hypoglycemia concomitant with high serum insulin in babies requiring >6 mg/kg/min intravenous glucose and THI as hyperinsulinism without maternal diabetes or genetic disorders. The following three possible risk factors for THI were evaluated: (1) birth weight z-score, (2) 1-min Apgar score and (3) absolute nucleated red blood cell (aNRBC) count on admission. Results: Of 705 infants, 8 were diagnosed wi Continue reading >>

Congenital Hyperinsulinism

Congenital Hyperinsulinism

Topics covered include the following: Congenital Hyperinsulinism Congenital hyperinsulinism (HI) is the most frequent cause of severe, persistent hypoglycemia in newborn babies and children. In most countries it occurs in approximately 1/25,000 to 1/50,000 births. About 60% of babies with HI develop hypoglycemia during the first month of life. An additional 30% will be diagnosed later in the first year and the remainder after that. With early treatment and aggressive prevention of hypoglycemia, brain damage can be prevented. However, brain damage can occur in children with HI if their condition is not recognized or if treatment is ineffective in the prevention of hypoglycemia. The material below explains the different forms of HI, the mechanisms of each type of HI, the genetic defects responsible for HI and their mode of inheritance. There is also information on treatment options and recent advances in diagnosis. Mechanisms of Disease Insulin is the most important hormone for controlling the concentration of glucose in the blood. As food is eaten, blood glucose rises and the pancreas secretes insulin to keep the blood glucose in the normal range. Insulin acts by driving glucose into the cells of the body. This action of insulin has two effects 1) maintaining blood glucose levels and 2) storing glucose particularly as glycogen in the liver. Once feeding is completed and the glucose levels fall, insulin secretion is turned off, allowing the stores of glucose in glycogen to be released into the bloodstream to keep blood glucose normal. In addition, with the switching off of insulin secretion, protein and fat stores become accessible and can be used instead of glucose as sources of fuel. In this manner, whether one eats or is fasting blood glucose levels remain in the norma Continue reading >>

Neonatal Hypoglycemia

Neonatal Hypoglycemia

OBJECTIVES After completing this article, readers should be able to: Describe the most common cause of prolonged neonatal hypoglycemia. List the signs of hypoglycemia. Describe the condition that has been implicated as a mechanism of hypoglycemic brain injury. Case Study A term male infant was born after an uneventful pregnancy to a 28-year-old gravida I woman who had no evidence of hyperglycemia and no chronic diseases. The infant had Apgar scores of 7 and 9 at 1 and 5 minutes, respectively. His growth parameters were in the normal range, with weight at the 60th percentile, head circumference at the 50th percentile, and length at the 50th percentile. The baby was taken to the well baby nursery, examined and bathed, and then taken to the mother for nursing at about 2 hours of age. He appeared slightly jittery at that time and was not very interested in nursing or very aware. A blood glucose concentration of 1.39 mmol/L (25 mg/dL) was obtained using a One Touch® instrument. The baby was fed 25 mL of 5% dextrose in water. The blood glucose concentration obtained 1 hour later was 2.22 mmol/L (40 mg/dL), and the baby nursed for about 5 minutes at each breast with apparent satisfaction. Jitteriness and“ lack of interest” were improved. Normal nursery routine was followed, with no comment in the chart by the nursing staff about the infant’s feeding or behavior until the second day of life when he again appeared jittery and fussy. Glucose concentration at that time was 1.11 mmol/L (20 mg/dL). The infant was fed by breast or bottle (routine 20 kcal/oz house formula) alternating every 2 hours, and clinical signs improved. One Touch® glucose concentrations obtained over the next 24 hours were variable, but overall the concentration increased, with a predischarge, preprand Continue reading >>

Hyperinsulinism - Cancer Therapy Advisor

Hyperinsulinism - Cancer Therapy Advisor

OVERVIEW: What every practitioner needs to know about hyperinsulinemic hypoglycemia in neonates and children Are you sure your patient has hyperinsulinism? What are the typical findings for this disease? Hyperinsulinism is the most common hypoglycemic disorder in infants and neonates. Transient neonatal hyperinsulinism is common due to perinatal stress, including birth asphyxia, preeclampsia, and intrauterine growth retardation. Congenital forms of hyperinsulinism are associated with recessive, dominant, or sporadic genetic defects in insulin regulation--particularly mutations of the beta-cell ATP-dependent potassium channel. Congenital hyperinsulinism can cause focal or diffuse disease and can usually be diagnosed based on genetic testing. Acquired hyperinsulinism due to pancreatic insulinoma or ingestion of antidiabetic drugs should be considered with new onset of hyperinsulinism in older children. Treatment of hyperinsulinism is aimed at maintaining plasma glucose levels at greater than 70 mg/dL. Diazoxide, a potassium channel agonist, is the drug of choice for treatment of hyperinsulinism. Some infants with congenital hyperinsulinism are severely affected and require surgery if they are unresponsive to diazoxide. Surgery can be curative, especially in focal hyperinsulinism, but it requires referral to a center with special expertise. Transient neonatal hyperinsulinism can last for several days to weeks after birth and can be treated with frequent feedings or diazoxide depending on the severity. Clinical features of hyperinsulinism may include large for gestational age (LGA) birth weight, small for gestational age (SGA) birth weight, and perinatal stress (maternal toxemia, birth asphyxia, infant of diabetic mother). Affected infants can present with seizures, lethar Continue reading >>

Ketotic Hypoglycaemia In Children With Transient Congenital Hyperinsulinism Of Infancy

Ketotic Hypoglycaemia In Children With Transient Congenital Hyperinsulinism Of Infancy

Endocrine Abstracts (2016) 45 P48 | DOI: 10.1530/endoabs.45.P48 Ketotic hypoglycaemia in children with transient congenital hyperinsulinism of infancy Dinesh Giri, Prashant Patil, Zoe Yung, Mohammed Didi & Senthil Senniappan Alder Hey Childrens Hospital, Liverpool, UK. Introduction: Congenital hyperinsulinism (CHI) is a rare genetic disorder of unregulated insulin secretion from the pancreatic -cells leading to severe hypoglycaemia & permanent neurological deficit if not managed appropriately. Kenotic hypoglycaemia (KH), a diagnosis of exclusion, is by far the most common form of hypoglycemia in children between 15 years of age characterized by recurrent episodes of hypoglycaemia and ketosis. Aim: To identify the prevalence of kenotic hypoglycaemia in children who had previously been diagnosed with transient CHI. Methods: Retrospective data of 142 patients with persistent/recurrent hypoglycaemia was analysed. Diagnosis of KH was confirmed by documented low levels of insulin and C-peptide with appropriately elevated free fatty acids, 3-betahydroxybutyrate, cortisol and growth hormone during hypoglycaemia. Results: Out of the 53 children with transient CHI, 5 children (9.4%) demonstrated KH after resolution of CHI. All were boys with mean (SD) birth weight of 2.82 kg (0.45). The average age of initial presentation with hypoglycaemia was 46.8 hours. The mean blood glucose concentration was 1.98 mmol/l (0.72). All patients required high glucose infusion rate initially 13.70 mg/kg per min (1.57). 4(80%) children required diazoxide to control the persistent hypoglycaemia [mean dose 7.38 mg/kg per day (1.94)]. Diazoxide therapy was discontinued at a mean age of 11.25 months (5.25). The mean age of presentation of KH was 18 months (2.16). KH developed after an average time per Continue reading >>

Hyperinsulinism (hi)

Hyperinsulinism (hi)

Depending on the degree of involvement of the pancreas the disease has several forms: Diffuse Disease:All the beta cells of the pancreas are affected. Focal Disease:Only some beta cells are affected, usually in the form of a small non-cancerous tumor in a localized area of the pancreas. Transient Disease:The low blood sugar resolves with age. This form can last a few weeks or up to several months. It is associated with prematurity, maternal diabetes during pregnancy (especially if not controlled), heart disease and severe infections in the pregnant mother. Symptoms of HI typically relate to low blood sugar and include: The diagnosis of any form of HI relies on demonstrating inappropriate insulin secretion. Generally testing requires a hospital admission regardless of age to accurately assess the child's insulin secretion and blood sugar control. This involves a number of measurements of the child's blood hormone, sugar and substrates relating to fat metabolism and a glucagon (a hormone that raises the blood sugar) tolerance challenge. In children with an established diagnosis of HI additional genetic testing to identify the mutated gene as well as additional imaging tests are required. Genetic testing can be done on blood or saliva samples from the child. If the child is has one of these genetic disorders, the parents will be tested to identify whether one or both of them are carriers and should have genetic counseling to determine the risk of having a disease in future pregnancies. In addition, children with HI should undergo a special imaging study called a PET-MRI scan using a special tracer that allows doctors to see and evaluate the extent of involvement in the pancreas. It allows doctors to distinguish focal from diffuse disease. This technique is presently avail Continue reading >>

Advances In Diagnosis And Treatment Of Hyperinsulinism In Infants And Children

Advances In Diagnosis And Treatment Of Hyperinsulinism In Infants And Children

Advances in Diagnosis and Treatment of Hyperinsulinism in Infants and Children Division of Endocrinology, Childrens Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 Search for other works by this author on: The Journal of Clinical Endocrinology & Metabolism, Volume 87, Issue 11, 1 November 2002, Pages 48574859, Charles A. Stanley; Advances in Diagnosis and Treatment of Hyperinsulinism in Infants and Children, The Journal of Clinical Endocrinology & Metabolism, Volume 87, Issue 11, 1 November 2002, Pages 48574859, In infants and children, as in adults, the most common cause of persistent hypoglycemia is hyperinsulinism. However, unlike adults, hyperinsulinism in children most often represents a congenital disorder rather than an acquired islet adenoma. Many children are unresponsive to medical therapy, and near total pancreatectomy is often required because of intractable hypoglycemia. Uncontrolled hypoglycemia may lead to seizures or permanent brain damage. Developmental delay or retardation has been reported to occur in 2550% of affected children. In recent years, concepts about hyperinsulinism in infancy have evolved rapidly as reflected in the changing nomenclature for the disorder. When originally described by MacQuarrie as idiopathic hypoglycemia of infancy in 1954, insulin was not considered to be the mechanism of hypoglycemia, because insulinomas were known to be rare in infants and children. One of the first applications of the insulin RIA in the 1960s by Berson and Yallow, however, identified insulin as the underlying problem. By 1970, the disorder had become known as nesidioblastosis through studies of pancreatic pathology by Yakovak et al. ( 1 ). This term implied that hyperinsulinism was due to an anomaly in islet development in which there was a Continue reading >>

Hyperinsulinism In Infancy And Childhood: When An Insulin Level Is Not Always Enough

Hyperinsulinism In Infancy And Childhood: When An Insulin Level Is Not Always Enough

Background: Hypoglycemia in infants and children can lead to seizures, developmental delay, and permanent brain damage. Hyperinsulinism (HI) is the most common cause of both transient and permanent disorders of hypoglycemia. HI is characterized by dysregulated insulin secretion, which results in persistent mild to severe hypoglycemia. The various forms of HI represent a group of clinically, genetically, and morphologically heterogeneous disorders. Content: Congenital hyperinsulinism is associated with mutations of SUR-1 and Kir6.2, glucokinase, glutamate dehydrogenase, short-chain 3-hydroxyacyl-CoA dehydrogenase, and ectopic expression on β-cell plasma membrane of SLC16A1. Hyperinsulinism can be associated with perinatal stress such as birth asphyxia, maternal toxemia, prematurity, or intrauterine growth retardation, resulting in prolonged neonatal hypoglycemia. Mimickers of hyperinsulinism include neonatal panhypopituitarism, drug-induced hypoglycemia, insulinoma, antiinsulin and insulin-receptor stimulating antibodies, Beckwith-Wiedemann Syndrome, and congenital disorders of glycosylation. Laboratory testing for hyperinsulinism may include quantification of blood glucose, plasma insulin, plasma β-hydroxybutyrate, plasma fatty acids, plasma ammonia, plasma acylcarnitine profile, and urine organic acids. Genetic testing is available through commercial laboratories for genes known to be associated with hyperinsulinism. Acute insulin response (AIR) tests are useful in phenotypic characterization. Imaging and histologic tools are also available for diagnosing and classifying hyperinsulinism. The goal of treatment in infants with hyperinsulinism is to prevent brain damage from hypoglycemia by maintaining plasma glucose levels above 700 mg/L (70 mg/dL) through pharmacologi Continue reading >>

Hyperinsulinemic Hypoglycemia

Hyperinsulinemic Hypoglycemia

Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by excessive insulin. Hypoglycemia due to excess insulin is the most common type of serious hypoglycemia. It can be due to endogenous or injected insulin. Causes[edit] Hypoglycemia due to endogenous insulin can be congenital or acquired, apparent in the newborn period, or many years later. The hypoglycemia can be severe and life-threatening or a minor, occasional nuisance. By far the most common type of severe but transient hyperinsulinemic hypoglycemia occurs accidentally in persons with type 1 diabetes who take insulin. Hypoglycemia due to endogenous insulin Congenital hyperinsulinism Transient neonatal hyperinsulinism (mechanism not known) Focal hyperinsulinism (KATP channel disorders) Paternal SUR1 mutation with clonal loss of heterozygosity of 11p15 Paternal Kir6.2 mutation with clonal loss of heterozygosity of 11p15 Diffuse hyperinsulinism KATP channel disorders SUR1 mutations Kir6.2 mutations Glucokinase gain-of-function mutations Hyperammonemic hyperinsulinism (glutamate dehydrogenase gain-of-function mutations) Short chain acyl coenzyme A dehydrogenase deficiency Carbohydrate-deficient glycoprotein syndrome (Jaeken's Disease) Beckwith-Wiedemann syndrome(suspected due to hyperinsulinism but pathophysiology uncertain: 11p15 mutation or IGF2 excess) Acquired forms of hyperinsulinism Insulinomas (insulin-secreting tumors) Islet cell adenoma or adenomatosis Islet cell carcinoma Adult nesidioblastosis Autoimmune insulin syndrome Noninsulinoma pancreatogenous hypoglycemia Reactive hypoglycemia (also see idiopathic postprandial syndrome) Gastric dumping syndrome Drug induced hyperinsulinism Sulfonylurea Aspirin Pentamidine Quinine Disopyramide Bordetella pertussis vaccine or i Continue reading >>

Frontiers | Abnormal Neurodevelopmental Outcomes Are Common In Children With Transient Congenital Hyperinsulinism | Endocrinology

Frontiers | Abnormal Neurodevelopmental Outcomes Are Common In Children With Transient Congenital Hyperinsulinism | Endocrinology

Front. Endocrinol., 20 May 2013 | Abnormal neurodevelopmental outcomes are common in children with transient congenital hyperinsulinism Hima Bindu Avatapalle 1, Indraneel Banerjee 1,2*, Sajni Shah 1, Megan Pryce 1, Jacqueline Nicholson 3, Lindsey Rigby 1, Louise Caine 1, Mohammed Didi4, Mars Skae 1, Sarah Ehtisham 1, Leena Patel 1, Raja Padidela 1, Karen E. Cosgrove 5, Mark J. Dunne 5 and Peter E. Clayton 1,2 1Department of Paediatric Endocrinology, Royal Manchester Childrens Hospital, Manchester, UK 2Manchester Academic Health Sciences Centre, Faculty of Medical and Human Sciences, Royal Manchester Childrens Hospital, University of Manchester, Manchester, UK 3Department of Clinical Psychology, Royal Manchester Childrens Hospital, Manchester, UK 4Department of Paediatric Endocrinology, Alder Hey Childrens Hospital, Liverpool, UK 5Faculty of Life Sciences, University of Manchester, Manchester, UK Introduction: Neuroglycopenia is recognized to be associated with abnormal neurodevelopmental outcomes in 2644% of children with persistent congenital hyperinsulinism (P-CHI). The prevalence of abnormal neurodevelopment in transient CHI (T-CHI) is not known. We have aimed to investigate abnormal neurodevelopment and associated factors in T-CHI and P-CHI. Materials and Methods: A cohort of children with CHI (n = 67, age 2.55 years) was assessed at follow-up review and noted to have normal or abnormal (mild or severe) neurodevelopmental outcomes for the domains of speech and language, motor, and vision. Children were classified as P-CHI (n = 33), if they had undergone surgery or remained on medical therapy, or T-CHI (n = 34), if medical treatment for hypoglycemia was stopped. Results: Overall, abnormal neurodevelopment was present in 26 (39%) children with CHI, of whom 18 (69%) w Continue reading >>

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