title: Remission of congenital hyperinsulinism following conservative treatment: an exploratory study in patients with KATP channel mutations.
abstract: BACKGROUND
  During conservative treatment, congenital hyperinsulinism (CHI) can resolve spontaneously. This study describes the hormonal and metabolic profiles in three patients with ABCC8/KCNJ11 mutations in clinical remission.
  
  
  METHODS
  An age-adapted fasting and oral glucose tolerance test (OGTT) were performed.
  
  
  RESULTS
  All patients (aged 6-9 years) tolerated age-adapted fasting durations (20, respectively 24 h), without reaching glucose concentrations ≤2.5 mmol/L, nor developing hypoglycemia-related symptoms. Nevertheless, insulin concentrations from all patients exceeded the 90th reference percentile at the end of the fasting test (range: 4.2-15.8 mU/L). During the OGTT, one patient (patient 2; BMI: 23.4 kg/m2; age: 7 years) reached a glucose concentration of 11.4 mmol/L after 2 h (concomitant insulin concentration: 148.3 mU/L).
  
  
  CONCLUSIONS
  The insulin concentration profiles in CHI patients in apparent clinical remission range from almost complete normalization to persistent, yet attenuated, hypersecretion. The hyperglycemia, detected during the OGTT, must be further monitored.
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Lachmann
authors.forename: Burak
authors.initials: B
authors.lastname: Salgin
authors.forename: Sebastian
authors.initials: S
authors.lastname: Kummer
authors.forename: Alena
authors.initials: A
authors.lastname: Welters
authors.forename: Carsten
authors.initials: C
authors.lastname: Döing
authors.forename: Martin
authors.initials: M
authors.lastname: Zenker
authors.forename: Ilse
authors.initials: I
authors.lastname: Wieland
authors.forename: Ertan
authors.initials: E
authors.lastname: Mayatepek
authors.forename: Thomas
authors.initials: T
authors.lastname: Meissner
mesh_terms: D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D044903:Congenital Hyperinsulinism / Q000188:drug therapy* / Q000235:genetics*; D000072700:Conservative Treatment; D005215:Fasting; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D005951:Glucose Tolerance Test; D006801:Humans; D007328:Insulin / Q000378:metabolism; D000078790:Insulin Secretion; D008297:Male; D009154:Mutation / Q000235:genetics*; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics*; D011379:Prognosis; D012074:Remission Induction; D064233:Sulfonylurea Receptors / Q000235:genetics*; D000099161:Kcnj11 Channel
chemical_list: D001786:Blood Glucose; D007328:Insulin; D024661:Potassium Channels, Inwardly Rectifying; D064233:Sulfonylurea Receptors; D000099161:Kcnj11 Channel; C577507:ABCC8 protein, human