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Updated: May 23, 2025

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Diabetic Ketoacidosis in a Pediatric Patient with Cystic Fibrosis-related Diabetes

Shyam Harinarayanan1, Taylor Merritt1, Perrin C White1

  • 1Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX 75390-9063, USA.

JCEM Case Reports
|May 22, 2025
PubMed

Insights

Cystic fibrosis-related diabetes (CFRD) can rarely lead to diabetic ketoacidosis (DKA) during pulmonary exacerbations. Early recognition of DKA in pediatric CF patients is crucial for timely management.

Area of Science:

  • Medical Genetics
  • Endocrinology
  • Pulmonology

Background:

  • Cystic fibrosis (CF) is a genetic disorder caused by CFTR gene mutations.
  • Cystic fibrosis-related diabetes (CFRD) is a distinct diabetic complication in CF patients.
  • Diabetic ketoacidosis (DKA) is a severe complication of diabetes.

Observation:

  • A pediatric patient with CFRD experienced DKA during a severe pulmonary exacerbation.
  • The patient presented with respiratory distress, a common CF symptom.
  • Typical DKA symptoms like polyuria and polydipsia were absent.

Findings:

  • DKA management included fluid/electrolyte correction, nutritional support, and insulin therapy.
  • The case highlights a rare presentation of DKA in CFRD.
  • Pulmonary exacerbation was a trigger for DKA in this CFRD patient.

Implications:

  • Clinicians should consider DKA in CF patients with respiratory distress, irrespective of typical diabetic symptoms.
  • This case underscores the importance of a broad differential diagnosis in managing CF complications.
  • Prompt recognition and management of DKA are vital for improving outcomes in pediatric CFRD.

Keywords:
cystic fibrosis–related diabetesdiabetic ketoacidosisivacaftor/tezacaftor/elexacaftor

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