Blood loss in Duchenne muscular dystrophy: vascular smooth muscle dysfunction?
Patients with Duchenne muscular dystrophy (DMD) tend to bleed more during surgery than do patients with other conditions. A retrospective analysis of blood loss after spinal surgery for scoliosis was therefore undertaken in 102 patients undergoing surgery in the senior author's unit. These included 48 patients with DMD, 26 patients with spinal muscular atrophy, and a miscellaneous group of 28 other patients most of whom had idiopathic scoliosis. For each patient the age at surgery, estimated blood volume, duration of operation, Cobb angle, and number of vertebrae fused were recorded and compared. Expression of dystrophin in skeletal muscle and the underlying gene mutation were also determined. The estimated blood loss in patients with DMD was significantly higher than that in patients with spinal muscular atrophy undergoing the same or similar procedure (P < 0.005) and was also significantly greater than that of the third group, which consisted mostly of patients with idiopathic scoliosis (P < 0.0005). Blood loss in the patient group with DMD showed a significant relationship with duration of surgery (P < 0.05). As most patients expressed no dystrophin, this did not correlate with the estimated blood loss. There was also no correlation between the estimated blood loss and the type of gene mutation found causing DMD. The authors' previous observations confirm the increased blood loss in patients with DMD who undergo scoliosis surgery. Because children with DMD lack dystrophin in all muscle types, including smooth muscle, the excessive blood loss may be because of a poor vascular smooth muscle vaso-constrictive response due to a lack of dystrophin.
Noordeen
M H
MH
Royal National Orthopaedic Hospital, Stanmore, Middlesex, U.K.
Haddad
F S
FS
Muntoni
F
F
Gobbi
P
P
Hollyer
J S
JS
Bentley
G
G
D000293:Adolescent; D001706:Biopsy; D016063:Blood Loss, Surgical / Q000503:physiopathology* / Q000517:prevention & control; D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D016189:Dystrophin / Q000032:analysis* / Q000172:deficiency; D004587:Electrophoresis, Agar Gel; D005260:Female; D006801:Humans; D015994:Incidence; D016014:Linear Models; D008297:Male; D018482:Muscle, Skeletal / Q000737:chemistry / Q000473:pathology; D009131:Muscle, Smooth, Vascular / Q000737:chemistry* / Q000473:pathology; D009136:Muscular Dystrophies / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D016133:Polymerase Chain Reaction; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012307:Risk Factors; D012600:Scoliosis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D014661:Vasoconstriction
D016189:Dystrophin