[Protein C deficiency with recurrent infarct of the small intestine].
Protein C and antithrombin III represent main inhibitors of the plasmatic coagulation system. Due to the lack of practicable assays the clinical importance of protein C was only established during the last six years. In familial protein C deficiency 77% of patients present with recurrent venous thromboses, half of them below the age of 30. In addition to recurrent superficial thrombophlebitis more serious manifestations like deep vein thrombosis and pulmonary embolism have been described. Mesenteric vein thrombosis has been reported in only 5 cases all of which could be controlled by conservative treatment. In our patient protein C deficiency was discovered 10 years after the angiographic diagnosis of portal and mesenteric vein thrombosis. Thereafter, the patient complained of recurrent abdominal discomfort. Intestinal ischaemia due to mesenteric vein thrombosis required segmental resection twice. Postoperatively the patient was heparinized. After excluding a secondary protein C deficiency due to a lack in vitamin K, hepatic disease, or disseminated intravascular coagulation, long-term anticoagulation by dicumarol was implemented as therapy of first choice.
Klar
E
E
Chirurgische Klinik, Universitätsklinikum Heidelberg.
Buhr
H
H
Zimmermann
R
R
D005260:Female; D006801:Humans; D007238:Infarction / Q000601:surgery*; D007583:Jejunum / Q000098:blood supply*; D008641:Mesenteric Vascular Occlusion / Q000601:surgery*; D008642:Mesenteric Veins / Q000601:surgery; D008875:Middle Aged; D011183:Postoperative Complications / Q000601:surgery*; D020151:Protein C Deficiency; D012008:Recurrence; D012086:Reoperation; D013927:Thrombosis / Q000601:surgery*
Protein C-Mangel mit rezidivierender Dünndarminfarzierung.