Diabetic ketoacidosis in cystic fibrosis
A. B. Atlas, D. N. Finegold, D. Becker, M. Trucco and G. Kurland
Department of Pediatrics, Children's Hospital of Pittsburgh, Pa.
OBJECTIVE--To differentiate the insulin-dependent glucose intolerance
associated with cystic fibrosis from type I diabetes mellitus in patients
with cystic fibrosis. DESIGN--Patient report. SETTING--Tertiary care
referral center. PARTICIPANT--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. CONCLUSION--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.