Diabetic ketoacidosis in cystic fibrosis.
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.
Atlas
A B
AB
Department of Pediatrics, Children's Hospital of Pittsburgh, Pa.
Finegold
D N
DN
Becker
D
D
Trucco
M
M
Kurland
G
G
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
D005819:Genetic Markers