TY - JOUR
T1 - Increased operative bleeding during orthopaedic surgery in patients with type I gaucher disease and bone involvement
AU - Katz, Kalman
AU - Tamary, Hanna
AU - Lahav, Judith
AU - Soudry, Michael
AU - Cohen, Ian J.
PY - 1999
Y1 - 1999
N2 - To aid clinicians in identifying patients with type I Gaucher disease who are at risk of excessive bleeding, we reviewed the coagulation parameters of six affected patients with bone involvement who underwent orthopaedic surgery at two centers, and of 22 patients under treatment at another, seven of whom had total splenectomy. All patients were of Jewish Ashkenazi origin. Among the latter group, prolonged prothrombin time was noted in 81%. Incidence of clotting factor deficiency were as follows: factor XI, 36.3%; V, 31.8%; VIII, 27.2%; IX, 13.6%; and XII, 27.2%. Most of the abnormalities occurred in the non-splenectomized patients. Two of the six orthopaedic surgery patients had excessive intraoperative and postoperative bleeding. One, who underwent spinal decompression had prolonged prothrombin time, and the other, who had total hip replacement, showed a deficiency of factor XI. The second patient's hemoglobin level was maintained with transfusion of fresh frozen plasma during contralateral hip arthroplasty five months later. We suggest that preoperative evaluation of clotting factors and replacement therapy may prevent excessive bleeding in patients with type I Gaucher disease.
AB - To aid clinicians in identifying patients with type I Gaucher disease who are at risk of excessive bleeding, we reviewed the coagulation parameters of six affected patients with bone involvement who underwent orthopaedic surgery at two centers, and of 22 patients under treatment at another, seven of whom had total splenectomy. All patients were of Jewish Ashkenazi origin. Among the latter group, prolonged prothrombin time was noted in 81%. Incidence of clotting factor deficiency were as follows: factor XI, 36.3%; V, 31.8%; VIII, 27.2%; IX, 13.6%; and XII, 27.2%. Most of the abnormalities occurred in the non-splenectomized patients. Two of the six orthopaedic surgery patients had excessive intraoperative and postoperative bleeding. One, who underwent spinal decompression had prolonged prothrombin time, and the other, who had total hip replacement, showed a deficiency of factor XI. The second patient's hemoglobin level was maintained with transfusion of fresh frozen plasma during contralateral hip arthroplasty five months later. We suggest that preoperative evaluation of clotting factors and replacement therapy may prevent excessive bleeding in patients with type I Gaucher disease.
KW - Gaucher disease
KW - Intraoperative bleeding
KW - Orthopaedic surgery
UR - http://www.scopus.com/inward/record.url?scp=0033492764&partnerID=8YFLogxK
M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???
AN - SCOPUS:0033492764
SN - 0018-5647
VL - 58
SP - 188
EP - 190
JO - Bulletin: Hospital for Joint Diseases
JF - Bulletin: Hospital for Joint Diseases
IS - 4
ER -