title: Diabetic ketoacidosis in cystic fibrosis.
abstract: OBJECTIVE
  To differentiate the insulin-dependent glucose intolerance associated with cystic fibrosis from type I diabetes mellitus in patients with cystic fibrosis.
  
  
  METHODS
  Patient report.
  
  
  METHODS
  Tertiary care referral center.
  
  
  METHODS
  An 11-year-old boy with cystic fibrosis who developed diabetic ketoacidosis. MEASUREMENT/MAIN RESULT: Biochemical, immunologic, and molecular techniques were used to support the sporadic association of type I diabetes mellitus in a patient with cystic fibrosis. Cystic fibrosis was confirmed by sweat test and further supported by the demonstration of a heterozygous deletion of the F508 locus. Evidence for the diagnosis of type I diabetes mellitus was developed from the clinical presentation of diabetic ketoacidosis with hyperglycemia, ketonemia, and ketonuria. Immunologic evidence included the demonstration of anti-insulin antibodies. The demonstration of homozygous absence of aspartic acid at position 57 of the HLA DQ-beta chain placed this child at high risk of type I diabetes mellitus.
  
  
  CONCLUSIONS
  The clinical presentation and the presence of immunologic and genetic markers characteristic of type I diabetes mellitus supports the concordance of cystic fibrosis and type I diabetes mellitus in this patient.
authors.affiliation: Department of Pediatrics, Children's Hospital of Pittsburgh, Pa.
authors.forename: A B
authors.initials: AB
authors.lastname: Atlas
authors.forename: D N
authors.initials: DN
authors.lastname: Finegold
authors.forename: D
authors.initials: D
authors.lastname: Becker
authors.forename: M
authors.initials: M
authors.lastname: Trucco
authors.forename: G
authors.initials: G
authors.lastname: Kurland
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications*; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000235:genetics; D016883:Diabetic Ketoacidosis / Q000209:etiology* / Q000503:physiopathology; D005819:Genetic Markers; D006801:Humans; D007223:Infant; D008297:Male
chemical_list: D005819:Genetic Markers