Patton Minkin1, Richard Bertetti2, Sean Lindsey3, Brian Bovino4. 1. Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY. Electronic address: pminkin@chpnet.org. 2. Chief Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY. 3. Junior Resident, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY. 4. Residency Program Director, Department of Oral and Maxillofacial Surgery, Mt Sinai-St Luke's/Mt Sinai-Roosevelt Hospitals, New York, NY.
Abstract
PURPOSE: 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. MATERIALS AND 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. CONCLUSION: Surgical and routine extractions appear to be safe procedures in patients with Hermansky-Pudlak syndrome when appropriate local and systemic hemostatic measures are used.
PURPOSE: 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. MATERIALS AND 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. CONCLUSION: 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: Marjan Huizing; May C V Malicdan; Jennifer A Wang; Hadass Pri-Chen; Richard A Hess; Roxanne Fischer; Kevin J O'Brien; Melissa A Merideth; William A Gahl; Bernadette R Gochuico Journal: Hum Mutat Date: 2020-01-23 Impact factor: 4.700