title: [Pulmonary thromboembolism associated with familial protein C deficiency type I].
abstract: A 66-year-old man was admitted to our hospital because of progressive dyspnea on effort. Arterial blood gas analysis showed severe hypoxemia, and a chest radiograph revealed reticular shadows in both lower lungs and an increase of the cardiothoracic ratio. Echocardiography demonstrated mild indentation of the interventricular septum toward the left ventricle, moderate pericardial effusion and pulmonary hypertension. From these data, we diagnosed pulmonary thromboembolism and started anticoagulation therapy. After the addition of the administration of warfarin and oxygen therapy, his symptoms disappeared. However, we could not obtain more supporting evidence of thromboembolization by methods of ventilation-perfusion scanning, digital subtraction angiography of the pulmonary artery, or venography. Blood coagulation analysis demonstrated that the patient's plasma protein C antigen levels and its activity were depleted. The patient's son had a history of thrombophlebitis and pulmonary embolization, and his data of protein C antigen levels was also decreased. Therefore, this patient was found to have a character of familial protein C deficiency type I. We could not get the conclusive proof of pulmonary thromboembolism, but we considered that the presence of familial protein C deficiency may cause exacerbation of pulmonary hypertension.
authors.affiliation: Division of Cardiovascular and Respiratory Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Japan, 650-0017.
authors.forename: Sho
authors.initials: S
authors.lastname: Yoshimura
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Nishimura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Funada
authors.forename: Kaori
authors.initials: K
authors.lastname: Takenaka
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Urata
authors.forename: Temiko
authors.initials: T
authors.lastname: Shimada
authors.forename: Teruaki
authors.initials: T
authors.lastname: Nishiuma
authors.forename: Miyako
authors.initials: M
authors.lastname: Satouchi
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Yokoyama
mesh_terms: D000368:Aged; D005192:Family Health; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D008297:Male; D020151:Protein C Deficiency / Q000150:complications* / Q000235:genetics*; D011655:Pulmonary Embolism / Q000209:etiology*