What question did this study set out to answer?

To describe the clinical spectrum, laboratory characteristics, and therapeutic management of severe congenital protein C deficiency presenting in adult patients from two families.

June 1, 1989British Journal of Haematology

Congenital severe protein C deficiency in adults

View Full Paper

Ask AI

Bookmark

Authors

EME MelissariOnassis Cardiac Surgery Center VKV V KakkarUniversity of London

Discussion

Member takes

Implication

Case series demonstrates severe adult thrombotic complications in congenital protein C deficiency, highlighting successful management with targeted anticoagulation therapy.

Key Points

To describe the clinical spectrum, laboratory characteristics, and therapeutic management of severe congenital protein C deficiency presenting in adult patients from two families.
Evaluated clinical histories, pedigree data, and coagulation profiles in four adult patients with severe protein C deficiency (plasma level ≤ 5% of normal) from two unrelated families.
Measured markers of disseminated intravascular coagulation, including plasma D-dimer, platelet count, and fibrinogen levels, during thrombotic crises and baseline periods.
Monitored treatment response to long-term anticoagulation regimens consisting of low molecular weight heparin alone or combined with low-dose warfarin.
Thrombotic manifestations emerged primarily in patients' early twenties, featuring recurrent deep iliofemoral vein thromboses, pulmonary emboli, mesenteric vein thrombosis leading to peritonitis, cavernous sinus thrombosis, renal vein thrombosis, and priapism.
Acute thromboembolic episodes were accompanied by compensated disseminated intravascular coagulation, marked by elevated mean D-dimer levels of 5000 ng/ml alongside reduced platelet and fibrinogen counts, with noncrisis D-dimer levels remaining elevated at a mean of 400 ng/ml.
Five family members in the second pedigree died of intravascular abdominal thrombosis at approximately 40 years of age, while surviving patients achieved satisfactory thrombosis control on long-term low molecular weight heparin alone or with low-dose warfarin.

Cite This Study

Melissari et al. (1989) studied this question.

synapsesocial.com/papers/6a9464fd5cc4cfd2f72dda03 https://doi.org/10.1111/j.1365-2141.1989.tb07686.x

View Full Paper

Ask AI

Bookmark

Implication

Case series demonstrates severe adult thrombotic complications in congenital protein C deficiency, highlighting successful management with targeted anticoagulation therapy.

Congenital severe protein C deficiency in adults

Discussion

Member takes

Implication

Key Points

Cite This Study

Congenital severe protein C deficiency in adults

Discussion

Member takes

Implication

Key Points

Cite This Study

Also Consider

Also Consider