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Diabetic Ketoacidosis in Cystic Fibrosis
Arthur B. Atlas, MD;
David N. Finegold, MD;
Dorothy Becker, MB, BCh;
Massimo Trucco, MD;
Geoffrey Kurland, MD
Am J Dis Child. 1992;146(12):1457-1458.
Abstract
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• 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)
Author Affiliations
From the Department of Pediatrics, Children's Hospital of Pittsburgh, Pa.
Footnotes
Accepted for publication August 27, 1992.
Reprint requests to Division of Pediatric Pulmonology, Morristown Memorial Hospital, 100 Madison Ave, Morristown, NJ 07960 (Dr Atlas).
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