title: Management of tooth extraction in a patient with a rare bleeding disorder associated with Hermansky-Pudlak syndrome: a case report.
abstract: OBJECTIVE
  This report describes the case of a 27-year-old man who had been diagnosed with Hermansky-Pudlak syndrome shortly after birth. Because the patient had a major bleeding disorder associated with his syndrome, local and systemic hemostatic protection recommendations had to be considered before tooth extraction.
  
  
  METHODS
  Synthetic vasopressin (1-deamino-8-d-arginine vasopressin [DDAVP]) was transfused intravenously before surgery. During surgery the patient was transfused with 1 U of human leukocyte antigen (HLA)-matched apheresis platelets. A hemostatic packing of Avitene and Gelfoam was adapted to the extraction site.
  
  
  RESULTS
  Treatment with DDAVP, HLA-matched platelets, and local application of a packing with Avitene and Gelfoam resulted in sustained hemostasis and an excellent healing response.
  
  
  CONCLUSIONS
  Surgical and routine extractions appear to be safe procedures in patients with Hermansky-Pudlak syndrome when appropriate local and systemic hemostatic measures are used.
authors.affiliation: Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY. Electronic address: pminkin@chpnet.org.
authors.forename: Patton
authors.initials: P
authors.lastname: Minkin
authors.affiliation: Chief Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY.
authors.forename: Richard
authors.initials: R
authors.lastname: Bertetti
authors.affiliation: Junior Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY.
authors.forename: Sean
authors.initials: S
authors.lastname: Lindsey
authors.affiliation: Residency Program Director, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY.
authors.forename: Brian
authors.initials: B
authors.lastname: Bovino
mesh_terms: D000328:Adult; D001778:Blood Coagulation Disorders / Q000503:physiopathology*; D022861:Hermanski-Pudlak Syndrome / Q000503:physiopathology*; D006801:Humans; D008297:Male; D014081:Tooth Extraction