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Clinical experience with a pasteurised human plasma concentrate in factor XIII deficiency.
1Department of Haematology, Bristol Royal Infirmary, UK.
Thrombosis and Haemostasis
|April 8, 1988
Summary
Infants with factor XIII deficiency can be safely treated with prophylactic factor XIII concentrate, preventing bleeding episodes. This treatment achieved satisfactory factor XIII levels without transmitting infections like hepatitis or HIV.
Area of Science:
- Hematology
- Pediatrics
- Immunology
Background:
- Factor XIII deficiency is a rare inherited bleeding disorder.
- Umbilical hemorrhage in neonates can indicate underlying coagulation defects.
- Early diagnosis and treatment are crucial for managing bleeding disorders in infants.
Observation:
- A neonate presented with umbilical bleeding, leading to a diagnosis of factor XIII deficiency.
- The infant received prophylactic pasteurized factor XIII concentrate from one month of age.
- A sibling diagnosed with the same condition also received prophylactic treatment.
Findings:
- Prophylactic factor XIII concentrate injections over 2.5 years prevented hemorrhagic episodes in the infant.
- Treatment resulted in satisfactory post-infusion factor XIII levels.
- No evidence of hepatitis or HIV transmission was observed, though three transient elevations in aspartate transaminase occurred.
- The factor XIII concentrate was well-tolerated by both infants.
Implications:
- Prophylactic factor XIII concentrate is an effective and safe treatment for infants with factor XIII deficiency.
- This approach can significantly improve the quality of life and prevent complications in affected children.
- Further investigation into the transient aspartate transaminase elevations is warranted.