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Hyperinsulinaemic Hypoglycemia

Zeynep Şıklar, Merih Berberoğlu
Congenital hyperinsulinism (CHI) is a rare disease characterized by dysregulated insulin secretion from pancreatic β-cells. Recurrent hypoglycemia can lead to neurological insult and permanent brain injury. Recently, there are important advances in understanding the genetic mechanisms, histological characteristics, imaging, and surgical techniques of congenital hyperinsulinemic hypoglycemia that could reflect to improvement in the clinical care of infants with this disorder. In Turkey, there is a high rate of consanguinity, thus, the incidence of CHI is expected to be high...
December 1, 2016: Journal of Clinical Research in Pediatric Endocrinology
T Joshi, A Caswell, S Acharya
BACKGROUND: Insulin assays are designed to detect endogenous insulin, however, insulin assays produced by different manufacturers may detect exogenous recombinant insulin, with varying degrees of cross-reactivity between different assays. We report a fascinating and difficult case of recurrent hypoglycaemia, where the final diagnosis was established with the help of insulin assays using different platforms. CASE REPORT: A 24-year-old female presented with recurrent hypoglycaemic episodes on a background of Type 1 diabetes mellitus and a completely resected synovial sarcoma of the right hip several years previously...
December 2016: Diabetic Medicine: a Journal of the British Diabetic Association
Klára Roženková, Maria Güemes, Pratik Shah, Khalid Hussain
Insulin secretion from pancreatic β-cells is tightly regulated to keep fasting blood glucose concentrations within the normal range (3.5-5.5 mmol/L). Hyperinsulinaemic hypoglycaemia (HH) is a heterozygous condition in which insulin secretion becomes unregulated and its production persists despite low blood glucose levels. It is the most common cause of severe and persistent hypoglycaemia in neonates and children. The most severe and permanent forms are due to congenital hyperinsulinism (CHI). Recent advances in genetics have linked CHI to mutations in 9 genes that play a key role in regulating insulin secretion (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, UCP2, HNF4A and HNF1A)...
June 2015: Journal of Clinical Research in Pediatric Endocrinology
Senthil Senniappan, Atefeh Sadeghizadeh, Sarah E Flanagan, Sian Ellard, Mahin Hashemipour, Majid Hosseinzadeh, Mansour Salehi, Khalid Hussain
BACKGROUND: Hyperinsulinaemic hypoglycaemia (HH) is a group of clinically and genetically heterogeneous disorders characterized by unregulated insulin secretion. Abnormalities in nine different genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A, UCP2 and HNF1A) have been reported in HH, the most common being ABCC8 and KCNJ11. We describe the genetic aetiology and phenotype of Iranian patients with HH. METHODS: Retrospective clinical, biochemical and genetic information was collected on 23 patients with biochemically confirmed HH...
August 13, 2015: BMC Research Notes
Anne-Sophie Sejling, Kai H W Lange, Christian S Frandsen, Sarah S Diemar, Lise Tarnow, Jens Faber, Jens J Holst, Bolette Hartmann, Linda Hilsted, Troels W Kjaer, Claus B Juhl, Birger Thorsteinsson, Ulrik Pedersen-Bjergaard
AIMS/HYPOTHESIS: Hypoglycaemia is associated with reduced skin temperature (Ts). We studied whether infrared thermography can detect Ts changes during hypoglycaemia in patients with type 1 diabetes and how the Ts response differs between patients with normal hypoglycaemia awareness and hypoglycaemia unawareness. METHODS: Twenty-four patients with type 1 diabetes (ten aware, 14 unaware) were studied during normoglycaemia (5.0-6.0 mmol/l), hypoglycaemia (2.0-2.5 mmol/l) and during recovery from hypoglycaemia (5...
August 2015: Diabetologia
Siri Malmgren, Bo Ahrén
AIMS/HYPOTHESIS: Glucose-lowering therapy with dipeptidyl peptidase-4 (DPP-4) inhibitors is associated with a low risk of hypoglycaemia. We hypothesise that DPP-4 inhibition prevents hypoglycaemia via increased glucagon counterregulation through the incretin hormone glucose-dependent insulinotropic polypeptide (GIP). METHODS: Using a hyperinsulinaemic-hypoglycaemic clamp that targeted 2.5 mmol/l we examined the effects of the DPP-4 inhibitor vildagliptin and GIP infusion on steady state glucose infusion rate (GIR) and glucagon counterregulation in mice...
May 2015: Diabetologia
Senthil Senniappan, Katherine Pitt, Pratik Shah, Ved Arya, Sanjay Jaiswal, Munther Haddad, Jonathan Hind, Anil Dhawan, Mark Davenport, Khalid Hussain
BACKGROUND: Portosystemic shunts (PSS) are abnormal vascular connections between the portal vein or its tributaries and the systemic vein that allow mesenteric blood to reach the systemic circulation without first passing through the liver. PSS can be associated with various syndromes and can lead to serious complications. We report a rare case of a child with PSS and recurrent hypoglycaemia. CASE: A 20-month-old girl with Down's syndrome presented with recurrent hypoglycaemic episodes...
2015: Hormone Research in Pædiatrics
Erica A Wehrwein, Jacqueline K Limberg, Jennifer L Taylor, Simmi Dube, Ananda Basu, Rita Basu, Robert A Rizza, Timothy B Curry, Michael J Joyner
What is the central question of this study? Hyperoxia blunts hypoglycaemia counterregulation in healthy adults. We hypothesized that this effect is mediated by the carotid bodies and that: (i) hyperoxia would have no effect on hypoglycaemia counterregulation in carotid body-resected patients; and (ii) carotid body-resected patients would exhibit an impaired counterregulatory response to hypoglycaemia. What is the main finding and its importance? Our data indicate that the effect of hyperoxia on hypoglycaemic counterregulation is mediated by the carotid bodies...
January 2015: Experimental Physiology
R W Lash, T J Giordano, A G Moraitis, I Hodish
BACKGROUND: β-cell hyperplasia has been implicated in the aetiology of post Roux-en-Y gastric bypass hyperinsulinaemic hypoglycaemia, but the pathogenesis of this condition is still unclear. CASE REPORT: We report a case of a 52-year-old man with post-Roux-en-Y gastric bypass hyperinsulinaemic hypoglycaemia who underwent distal pancreatectomy to alleviate his symptoms. Pancreatic histopathology showed chronic pancreatitis with a corresponding loss of exocrine tissue and islet retention...
December 2014: Diabetic Medicine: a Journal of the British Diabetic Association
Svetlana Gataullina, Pascale Delonlay, Eric Lemaire, Nathalie Boddaert, Christine Bulteau, Christine Soufflet, Gemma Aznar Laín, Rima Nabbout, Catherine Chiron, Olivier Dulac
AIM: The aim of the study was to characterize seizures and epilepsy related to hypoglycaemia. METHOD: We analyzed the files of 170 consecutive patients referred for hypoglycaemia (onset 1h to 4y) caused by inborn errors of metabolism (glycogen storage disease type I, fatty acid β-oxidation disorders, and hyperinsulinism). RESULTS: Ninety patients (42 males and 48 females; 38 neonates and 52 infants/children) had brief hypoglycaemic seizures (68%) or status epilepticus (32%)...
February 2015: Developmental Medicine and Child Neurology
Prabhakaran Kalaivanan, Ved Bhushan Arya, Pratik Shah, Vipan Datta, Sarah E Flanagan, Deborah J G Mackay, Sian Ellard, Senthil Senniappan, Khalid Hussain
AIM: We describe the novel clinical observation of protein induced hyperinsulinaemic hypoglycaemia following remission of transient neonatal diabetes mellitus (TNDM) in a patient with 6q24 methylation defect. METHODS: A male infant of non-consanguineous Caucasian parents, born at 40 weeks of gestation with a birth weight of 3330 g (-0.55 standard deviation score) presented with hyperglycaemia in the first week of life and was diagnosed with 6q24 TNDM. At 22 months of age, he developed recurrent hypoglycaemic episodes...
November 2014: Journal of Pediatric Endocrinology & Metabolism: JPEM
Seigo Sugiyama, Hideaki Jinnouchi, Kunio Hieshima, Tomio Jinnouchi
A 60-year-old man with uncontrolled type 2 diabetes mellitus (DM) (glycated haemoglobin 11%) had the unusual symptoms of palpitations and sweating after drinking an excessive amount of soft drinks. Three-hour data in the 75-g oral glucose tolerance test (75g-OGTT) repeatedly showed normoinsulinaemic hypoglycaemia. His diabetic disorder was based on a delayed insulin secretory response to hyperglycaemia and daily excessive intake of glucose from a high caloric diet and soft drinks. However, we paradoxically observed increased insulin sensitivity evaluated by a hyperinsulinaemic-euglycaemic clamp (glucose infusion rate: 64...
2014: BMJ Case Reports
Kung-Ting Kao, Peter J Simm, Justin Brown
A 9 year old girl presented with seizures, weight gain and early morning behavioural changes. She had been commenced on anticonvulsants and was subsequently diagnosed with hyperinsulinaemic hypoglycaemia. This case demonstrates the importance of blood glucose monitoring in children presenting with new-onset seizures and/or with early morning or fasting behavioural changes, the challenges in localizing the lesion, as well as the difficulties in achieving normoglycaemia prior to, and immediately following, surgery...
April 2014: Journal of Paediatrics and Child Health
Raymond J Davey, Nirubasini Paramalingam, Adam J Retterath, Ee Mun Lim, Elizabeth A Davis, Timothy W Jones, Paul A Fournier
AIMS/HYPOTHESIS: A 10 s sprint has been reported to provide a means to prevent acute post-exercise hypoglycaemia in young adults with type 1 diabetes because of its glycaemia-raising effect, but it is unclear whether this effect is impaired by antecedent hypoglycaemia. The purpose of this study was to investigate whether antecedent hypoglycaemia impairs the glycaemia-raising effect of a 10 s sprint in individuals with type 1 diabetes. METHODS: Eight individuals underwent a hyperinsulinaemic-hypoglycaemic or hyperinsulinaemic-euglycaemic clamp on two separate mornings...
June 2014: Diabetologia
J Farngren, M Persson, A Schweizer, J E Foley, B Ahrén
AIMS: To determine the effects of dipeptidyl peptidase-4 (DPP-4) inhibition on glucagon dynamics in patients with insulin-treated type 2 diabetes (T2D). METHODS: The study was a single-centre, double-blind, randomized, placebo controlled crossover study in patients with T2D, mean age 59 ± 6 (s.d.) years and mean haemoglobin A1c 7.7 ± 0.8%, treated with exogenous insulin with or without oral antihyperglycaemic agents. Patients received vildagliptin (50 mg BID) or placebo as add-on to insulin for 4 weeks in random order with a 4-week washout in-between...
September 2014: Diabetes, Obesity & Metabolism
Raja Padidela, Miriam Fiest, Ved Arya, Virpi V Smith, Michael Ashworth, Dyanne Rampling, Melanie Newbould, Gauri Batra, Jacqueline James, Neville B Wright, Mark J Dunne, Peter E Clayton, Indraneel Banerjee, Khalid Hussain
BACKGROUND: Insulinomas are a rare cause of hyperinsulinaemic hypoglycaemia (HH) in children. The clinical features, investigations, management and histology of these rare pancreatic tumours in children have not been described in a large cohort of patients. METHODS: We conducted a retrospective review of cases diagnosed between 2000 and 2012, presenting to two referral centres in the United Kingdom. Clinical, biochemical, imaging (magnetic resonance imaging (MRI) and 6-L-¹⁸F-fluorodihydroxyphenylalanine (¹⁸F-DOPA) PET/CT scanning) and histological data were collected...
May 2014: European Journal of Endocrinology
V B Arya, Z Mohammed, O Blankenstein, P De Lonlay, K Hussain
Insulin secretion from pancreatic β-cells is tightly regulated to maintain fasting blood glucose level between 3.5-5.5 mmol/l. In hyperinsulinaemic hypoglycaemia (HH) insulin secretion becomes unregulated so that insulin secretion persists despite low blood glucose levels. HH can be due to a large number of causes and recent advances in genetics have begun to provide novel insights into the molecular mechanisms of HH. Defects in key genes involved in regulating insulin secretion have been linked to HH. The most severe forms of HH are clinically observed in the newborn period whereas in adults an insulinoma is the commonest cause of HH...
March 2014: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
Jacqueline K Limberg, Jennifer L Taylor, Simmi Dube, Rita Basu, Ananda Basu, Michael J Joyner, Erica A Wehrwein
Activation of the carotid body chemoreceptors with hypoxia alters baroreceptor-mediated responses. We aimed to examine whether this relationship can be translated to other chemoreceptor stimuli (i.e. hypoglycaemia) by testing the following hypotheses: (i) activation of the carotid body chemoreceptors with hypoglycaemia would reduce spontaneous cardiac baroreflex sensitivity (sCBRS) in healthy humans; and (ii) desensitization of the carotid chemoreceptors with hyperoxia would restore sCBRS to baseline levels during hypoglycaemia...
April 2014: Experimental Physiology
Jin Ho Chong, Suresh Chandran, Prathibha Agarwal, Victor Samuel Rajadurai
Hyperinsulinaemic hypoglycaemia in small-for-gestational age infants usually presents in the first two postnatal days. We present a preterm, small-for-gestational age infant who had hyperinsulinaemic hypoglycaemia on day 13 of life. A female twin infant weighing 1390 g was born at 32(+6) weeks of gestation. Her glycaemic profile was normal till day 13 of life, after which she was noted to be lethargic and hypoglycaemic and had hyperinsulinism, hypoketonaemia and hypofattyacidaemia, requiring high glucose infusion rate to maintain normoglycaemia, while negative for septic markers and metabolic screen...
2013: BMJ Case Reports
Meidjie Ang, Christian Meyer, Mathias D Brendel, Reinhard G Bretzel, Thomas Linn
AIMS/HYPOTHESIS: Pancreatic islet transplantation stabilises glycaemic control in type 1 diabetes mellitus patients with neuroglycopoenia, despite them not achieving insulin independence because of limited graft function. However, the extent and underlying metabolic pathways of restored glucose counterregulation are unknown. We therefore compared systemic glucose turnover, including lactate gluconeogenesis (GN) and muscle glucose uptake, in individuals with type 1 diabetes who were transplant recipients with partial graft function (T1DM/ITx(+)), matched non-transplanted individuals with type 1 diabetes (T1DM/ITx(-)) and matched healthy non-diabetic individuals...
March 2014: Diabetologia
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