A patient with congenital plasminogen deficiency manifesting primary pulmonary hypertension.
A 25-year-old Japanese man was diagnosed as having primary pulmonary hypertension, on the basis of the findings of cardiac catheterization and ventilation-perfusion scintiscans. The plasma level of plasminogen in this patient was found to be reduced to 43% by a functional assay and 61 mg/l by an antigenic assay. Based on the family study, the patient was considered to have a heterozygous congenital plasminogen deficiency. Accordingly, it is suggested that the defective fibrinolysis in this patient may have played an important role in the development of primary pulmonary hypertension through microthrombosis.
Okamura
T
T
First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Tsuda
Y
Y
Murakawa
M
M
Makizumi
K
K
Suehiro
K
K
Shibuya
T
T
Harada
M
M
Niho
Y
Y
D000328:Adult; D001777:Blood Coagulation / Q000502:physiology; D006579:Heterozygote; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology*; D008297:Male; D010375:Pedigree; D010958:Plasminogen / Q000172:deficiency* / Q000235:genetics
D010958:Plasminogen