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Related Experiment Videos

"Car. factor" deficiency revisited.

D M Komp

    Pediatric Research
    |April 1, 1975
    PubMed
    Summary
    This summary is machine-generated.

    A Virginia family with a bleeding disorder exhibits a novel serum defect in thromboplastin generation, distinct from Car. factor deficiency, impacting platelet function and unresponsive to plasma transfusions.

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    Area of Science:

    • Hematology
    • Human Genetics
    • Molecular Biology

    Background:

    • Bleeding disorders can stem from complex coagulation factor deficiencies.
    • Previous research identified
    • Car. factor
    • deficiency impacting thromboplastin generation.

    Purpose of the Study:

    • To characterize a novel bleeding disorder in a Virginia family.
    • To investigate the underlying serum defect and its relationship to known coagulation disorders.
    • To evaluate platelet function in affected individuals.

    Main Methods:

    • Serum-based thromboplastin generation assays.
    • Cross-correction studies with known factor-deficient sera.
    • Partial thromboplastin time (PTT) measurements.

    Related Experiment Videos

  • Platelet aggregation studies with adenosine diphosphate (ADP).
  • Analysis of patients with Noonan syndrome, albinism, and "Portsmouth" syndrome.

    • Main Results:

    • Affected Virginia family members presented with a serum defect in thromboplastin generation.
    • This defect was not corrected by sera from the original "Car. factor" deficient family.
    • Partial thromboplastin times were normal in both groups.
    • In vitro correction with normal serum did not translate to in vivo correction after fresh frozen plasma infusion.
    • Virginia family members showed significantly impaired platelet aggregation (<30%) with rapid disaggregation.
    • Children with Noonan syndrome, albinism, and "Portsmouth" syndrome exhibited coexisting platelet aggregation defects and nonspecific serum defects.

    Conclusions:

    • The Virginia family exhibits a distinct bleeding disorder characterized by a persistent serum defect and impaired platelet aggregation.
    • This condition is not attributable to the previously described "Car. factor" deficiency.
    • The findings suggest a complex interplay between serum factors and platelet function in certain genetic syndromes.