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Testing for Passovoy defect in children with prolonged activated partial thromboplastin time (APTT)
A Hayani1, C R Suarez, J E Godwin
1Department of Pediatrics, Loyola University Medical Center, Maywood, Illinois, USA.
Journal of Pediatric Hematology/Oncology
|August 1, 1996
Summary
Testing for Passovoy defect using Passovoy trait plasma (PTP) may not be a reliable diagnostic method in children with prolonged activated partial thromboplastin time (APTT). Further research is needed to confirm if Passovoy defect is a distinct bleeding disorder.
Area of Science:
- Pediatric Hematology
- Coagulation Disorders
Background:
- Prolonged activated partial thromboplastin time (APTT) in children necessitates investigation into potential bleeding disorders.
- The Passovoy defect is a suspected inherited coagulation disorder, but its diagnostic criteria remain unclear.
Purpose of the Study:
- To evaluate the utility of Passovoy trait plasma (PTP) in diagnosing Passovoy defect in pediatric patients presenting with prolonged APTT.
- To explore the clinical significance and diagnostic challenges associated with prolonged APTT in children.
Main Methods:
- A cohort of 13 children with prolonged APTT was studied.
- Mixing studies were performed using normal human plasma and PTP.
- Comprehensive laboratory testing of intrinsic pathway factors and von Willebrand factor was conducted.
Main Results:
- All patients showed correction of prolonged APTT with normal plasma but not with PTP.
- Eight patients had abnormal coagulation factor levels, including low von Willebrand activity (6 patients), low factor XII (1 patient), and lupus anticoagulant (1 patient).
- Bleeding manifestations were present in 5 patients, while 8 were asymptomatic.
Conclusions:
- The use of PTP in mixing studies appears insufficient for diagnosing Passovoy defect.
- The findings question the existence of Passovoy defect as a distinct clinical entity.
- Further investigation is warranted to clarify the diagnostic approach for prolonged APTT in children and the nature of suspected coagulation abnormalities.