title: Perioperative management of a patient with Fechtner syndrome.
abstract: Fechtner syndrome is a rare type of familial thrombocytopenia associated with large platelets, leukocyte inclusions, and features of Alport's syndrome. The bleeding tendency is usually mild, but severe hemorrhages have been reported. This is the case of a patient with Fechtner syndrome who was scheduled to undergo tonsillectomy. The patient had a history of easy bruising in childhood and a markedly prolonged bleeding time. Administration of DDAVP led to normalization of the bleeding time, and the patient underwent surgery without complications. With this approach the use of platelet concentrates could be avoided.
authors.affiliation: Department of Haematology/Oncology, Center for Internal Medicine, Justus-Liebig-University, Giessen, Germany. axel.matzdorff@innere.med.uni-giessen.de
authors.forename: A C
authors.initials: AC
authors.lastname: Matzdorff
authors.forename: J G
authors.initials: JG
authors.lastname: White
authors.forename: K
authors.initials: K
authors.lastname: Malzahn
authors.forename: A
authors.initials: A
authors.lastname: Greinacher
mesh_terms: D000328:Adult; D001760:Bleeding Time; D001777:Blood Coagulation / Q000187:drug effects; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D009394:Nephritis, Hereditary / Q000150:complications*; D011182:Postoperative Care; D011300:Preoperative Care; D013921:Thrombocytopenia / Q000150:complications* / Q000235:genetics*; D014068:Tonsillectomy
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin