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December 1992

Diabetic Ketoacidosis in Cystic Fibrosis

Author Affiliations

From the Department of Pediatrics, Children's Hospital of Pittsburgh, Pa.

Am J Dis Child. 1992;146(12):1457-1458. doi:10.1001/archpedi.1992.02160240067022

• 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-β 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.(AJDC. 1992;146:1457-1458)

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