174 Frequency of diabetic keto-acidosis in children with cystic fibrosis related diabetes

Abstract

Objectives To evaluate the frequency of diabetic keto-acidosis (DKA) in cystic fibrosis related diabetes (CFRD) population and to identify associated risk factors. Methods A retrospective database review of all CF patients with diabetes mellitus (DM) from 1992 to 2014. Data collected included demographics, sweat chloride, sputum culture, evidence of compliance, DM diagnosis parameters, insulin dose and weight profile prior to the diagnosis of DM. CFRD was diagnosed as per American Diabetes Association guidelines. Results Of 392 children with CF, 47 (12%) had DM: CFRD (n = 44), type 1(n = 2, 1 had DKA) and type 2 (n = 1). Only 2/44 patients with CFRD had DKA. Each patient had evidence of poor compliance with CF and DM managements and presented with pulmonary exacerbation and DKA. Both patients had higher HbA1c and insulin dose when compared to the CFRD group (Table). Pseudomonas (56%) and Aspergillus (23%) isolation was higher in CFRD patients when compared to non-CFRD patients. 47% CFRD patients had significant weight loss in 3 months preceding diagnosis – mean 7.4% (range 2–22). TableComparison of the 2 DKA patients with CFRD groupData are expressed as mean(SD) (range).CFRD group (n = 42)CFRD DKA Case 1CFRD DKA Case 2Age at CFRD diagnosis (years)13.8(2) (10–17)1311Sweat Chloride (mmol/L)107(17) (74–165)10385OGTT results (mmol/L) at 0 hrs6.4(2.5) (4–17)Presented in DKA6.5 at 2 hrs12.5(4.8) (4–26)11.8Pulmonary exacerbation at diagnosisNoneYesYesHbA1c (%)7.1(2.5) (5–16.8)10.614Insulin dose (IU/kg/day)0.6(0.8) (0–2.2)3.83.4Number of episodes of DKA03 in total, 1st at diagnosis2 since diagnosisFactors associatedN/APoor compliance high insulin dose & HbA1c (both) Conclusion DKA in CFRD is uncommon (5%) when reviewed over 22 years. Poor compliance with CF therapy and poor diabetic control (high insulin dosage and HbA1c) were common factors. An index of suspicion for keto-acidosis is indicated when those patients present with pulmonary exacerbations. Oral glucose tolerance test is indicated in patients with persistent weight loss and/or new sputum microbiology.