title: Successful treatment of young infants presenting neonatal diabetes mellitus with continuous subcutaneous insulin infusion before genetic diagnosis.
abstract: OBJECTIVE
  Neonatal diabetes mellitus (NDM) is defined as hyperglycemia and impaired insulin secretion with onset within 6 months of birth. While rare, NDM presents complex challenges regarding the management of glycemic control. The availability of continuous subcutaneous insulin infusion pumps (CSII) in combination with continuous glucose monitoring systems (CGM) provides an opportunity to monitor glucose levels more closely and deliver insulin more safely.
  
  
  METHODS
  We report four cases of young infants with NDM successfully treated with CSII and CGM. Moreover, in two cases with Kir 6.2 mutation, we describe the use of CSII in switching therapy from insulin to sulfonylurea treatment.
  
  
  RESULTS
  Insulin pump requirement for the 4 neonatal diabetes cases was the same regardless of disease pathogenesis and c-peptide levels. No dilution of insulin was needed. The use of an integrated CGM system helped in a more precise control of BG levels with the possibility of several modifications of insulin basal rates. Moreover, as showed in the first two case-reports, when the treatment was switched from insulin to glibenclamide, according to identification of Kir 6.2 mutation and diagnosis of NPDM, the CSII therapy demonstrated to be helpful in allowing gradual insulin suspension and progressive introduction of sulfonylurea.
  
  
  CONCLUSIONS
  During the neonatal period, the use of CSII therapy is safe, more physiological, accurate and easier for the insulin administration management. Furthermore, CSII therapy is safe during the switch of therapy from insulin to glibenclamide for infants with permanent neonatal diabetes mellitus.
authors.affiliation: Department of Pediatrics, Regina Margherita Hospital, University of Turin, Piazza Polonia 94, Turin, Italy. ivana.rabbone@unito.it.
authors.forename: Ivana
authors.initials: I
authors.lastname: Rabbone
authors.affiliation: Department of Experimental Medicine and Surgery, University of Tor Vergata, Rome, Italy.|Bambino Gesù Children Hospital, IRCCS, Rome, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Barbetti
authors.affiliation: Regional Center for Pediatric Diabetes, Pediatric Diabetes and Metabolic Disorders Unit, University of Verona, Verona, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Marigliano
authors.affiliation: Department of Pediatrics, Ospedale San Raffaele Scientific Institute, Milan, Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Bonfanti
authors.affiliation: Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children's Hospital, Bari, Italy.
authors.forename: Elvira
authors.initials: E
authors.lastname: Piccinno
authors.affiliation: Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children's Hospital, Bari, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Ortolani
authors.affiliation: Department of Pediatrics, Regina Margherita Hospital, University of Turin, Piazza Polonia 94, Turin, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Ignaccolo
authors.affiliation: Regional Center for Pediatric Diabetes, Pediatric Diabetes and Metabolic Disorders Unit, University of Verona, Verona, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Maffeis
authors.affiliation: Regional Center for Pediatric Diabetes "G.Stoppoloni", Department of Pediatrics, Second University of Naples, Naples, Italy.
authors.forename: Santino
authors.initials: S
authors.lastname: Confetto
authors.affiliation: Department of Pediatrics, Regina Margherita Hospital, University of Turin, Piazza Polonia 94, Turin, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Cerutti
authors.affiliation: Regional Center for Pediatric Diabetes "G.Stoppoloni", Department of Pediatrics, Second University of Naples, Naples, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Zanfardino
authors.affiliation: Regional Center for Pediatric Diabetes "G.Stoppoloni", Department of Pediatrics, Second University of Naples, Naples, Italy.
authors.forename: Dario
authors.initials: D
authors.lastname: Iafusco
mesh_terms: D001786:Blood Glucose / Q000378:metabolism; D002096:C-Peptide / Q000097:blood; D003920:Diabetes Mellitus / Q000097:blood / Q000188:drug therapy* / Q000235:genetics; D003922:Diabetes Mellitus, Type 1 / Q000097:blood / Q000188:drug therapy* / Q000235:genetics; D005260:Female; D005905:Glyburide / Q000627:therapeutic use; D006801:Humans; D006943:Hyperglycemia / Q000188:drug therapy; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000008:administration & dosage*; D007332:Insulin Infusion Systems; D008297:Male; D008991:Monitoring, Physiologic; D013453:Sulfonylurea Compounds
chemical_list: D001786:Blood Glucose; D002096:C-Peptide; D007328:Insulin; D013453:Sulfonylurea Compounds; D005905:Glyburide
keywords: Continuous glucose monitoring systems (CGMS); Continuous subcutaneous insulin infusion pumps (CSII); Hyperglycemia; Neonatal diabetes mellitus