title: Hermansky-Pudlak syndrome: dental management considerations.
abstract: Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by oculocutaneous albinism (OCA), platelet dysfunction, and ceroid storage. It is common among the Puerto Rican population and is expected to spread within the United States and other countries. Due to the platelet deficiency, these patients are of major concern to pediatric dentists. The purpose of this article is to explain in detail the characteristic triad of this syndrome and to propose an adequate approach to perform dental treatment, using appropriate protection recommendations for HPS patients. Recommendations for dental treatment are considered. They include the use of: (1) eyeglasses with 99 UV filter to protect them from the unpleasant dental light stimulus; (2) an extra-soft toothbrush and conservative brushing technique; (3) medication with antifibrinolitic agents; and (4) local measures to achieve hemostasis.
authors.affiliation: Pediatric Dentistry Postgraduate Program, School of Dentistry, University of Puerto Rico School of Dentistry, Bayamûn, Puerto Rico. drfeliciano@hotmail.com
authors.forename: Nilma Z
authors.initials: NZ
authors.lastname: Feliciano
authors.forename: Enid
authors.initials: E
authors.lastname: Rivera
authors.forename: Emille
authors.initials: E
authors.lastname: Agrait
authors.forename: Katherine
authors.initials: K
authors.lastname: Rodriguez
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D002648:Child; D019140:Dental Care for Children; D017742:Dental Care for Chronically Ill; D004867:Equipment Design; D005135:Eye Protective Devices / Q000145:classification; D006489:Hemostatic Techniques; D022861:Hermanski-Pudlak Syndrome / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D014099:Toothbrushing / Q000295:instrumentation; D014466:Ultraviolet Rays
chemical_list: D000933:Antifibrinolytic Agents