PLATELET-FUNCTION DEFICIENCY IN DUCHENNE MUSCULAR-DYSTROPHY

Platelet function and plasmatic coagulation of 51 patients with Duchen ne muscular dystrophy (DMD) were studied in order to find an explanati on for the known substantial blood loss in spinal surgery of these ver y patients. A normal plasmatic coagulation and a slight but not signif icant increase of bleeding time was found. However, a significant defi ciency of platelet adhesion and ristocetin induced aggregation (P < 0. 001) as well as a marked reduction of expression of glycoprotein IV (s ynonyms: GPIV, GPIIIb, CD36) to 50% were detected. We conclude from ou r study, that this finding of deficiency of platelet function in DMD p atients is of no importance in everyday life and minor operations, e.g . lower limb surgery. In major spinal surgery, however, the platelet f unction deficiency occurs. A decompensation of platelet adhesion as we ll as aggregation capacity can be assumed due to the unavoidable intra operative dilution effect by the inevitable volume replacement. (C) 19 98 Elsevier Science B.V.