A case of congenital factor V deficiency combined with multiple congenital anomalies: successful management of palatoplasty.
A patient with congenital factor V deficiency combined with mental retardation and several congenital anomalies including cleft palate, dwarfism, microcephaly and right hydrocele testis is described. The levels of factor V activity and factor V antigen of plasma were significantly decreased. The platelet lysate obtained from him also showed a significantly low level of factor V activity. Palatoplasty and tooth extraction were successfully performed under transfusion therapy with fresh-frozen plasma.
Tsuda
H
H
Clinical Laboratory, Kyushu University Hospital, Fukuoka, Japan.
Mizuno
Y
Y
Hara
T
T
Ohtsuki
T
T
Ueda
K
K
Matsuzaki
K
K
Watanabe
T
T
D000015:Abnormalities, Multiple / Q000097:blood / Q000601:surgery*; D001780:Blood Coagulation Tests; D001803:Blood Transfusion; D002648:Child; D002972:Cleft Palate / Q000097:blood / Q000601:surgery*; D005165:Factor V / Q000032:analysis; D005166:Factor V Deficiency / Q000097:blood / Q000601:surgery*; D005884:Gingival Hemorrhage / Q000097:blood / Q000601:surgery; D006801:Humans; D007430:Intraoperative Care; D008297:Male; D010159:Palate / Q000601:surgery*; D010949:Plasma; D011182:Postoperative Care; D014081:Tooth Extraction
D005165:Factor V