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Coagulation disorder as a presentation of cystic fibrosis

Gary Lewis McPhail1

  • 1Division of Pulmonary Medicine, University of Cincinnati, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA.

The Journal of Emergency Medicine
|April 4, 2008
PubMed

Insights

Cystic fibrosis (CF), a common inherited disease, can rarely present in infants with bleeding disorders due to vitamin K deficiency. This case highlights an infant with CF experiencing bruising and anemia from this rare presentation.

Area of Science:

  • Pediatrics
  • Genetics
  • Hematology

Background:

  • Cystic fibrosis (CF) is a prevalent inherited disorder affecting multiple organs, primarily the lungs and digestive system.
  • Coagulation abnormalities in infancy are uncommon manifestations of CF.
  • Vitamin K deficiency is a known complication of CF, impacting clotting factors.

Observation:

  • An infant presented with significant bruising and occult gastrointestinal bleeding.
  • Laboratory findings revealed anemia and evidence of vitamin K deficiency.
  • Diagnostic workup confirmed cystic fibrosis as the underlying cause.

Findings:

  • The infant's clinical presentation was directly linked to severe vitamin K deficiency.
  • This deficiency resulted in a rare coagulopathy, manifesting as bleeding and anemia.
  • Early diagnosis of CF is crucial to prevent such complications.

Implications:

  • This case underscores the importance of considering CF in infants with unexplained bleeding tendencies.
  • Timely vitamin K supplementation and CF management can prevent severe hematological complications.
  • Increased awareness among clinicians can improve early detection and outcomes for CF patients.

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