title: The non-surgical management of a patient with Kostmann syndrome-associated periodontitis: a case report.
abstract: Kostmann syndrome is a rare, congenital immunological disorder caused by a mutation of the hematopoietic cell-specific LYN substrate 1-associated protein X1. These patients pose a unique challenge to the dental practitioner due to the severe oral infections that are often seen in this population. The patient described in this report is a 16-year-old female with Kostmann syndrome-associated periodontitis. The treatment consisted of scaling and root planing performed in conjunction with subgingival irrigation with povidone-iodine solution. This report details how Kostmann syndrome-associated periodontitis can be successfully treated and maintained long-term, using non-surgical treatment modalities and local antimicrobial therapy.
authors.affiliation: Dental Science Center, Herman Ostrow School of Dentistry of the University of Southern California.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Gonzalez
authors.forename: Alon
authors.initials: A
authors.lastname: Frydman
mesh_terms: D000293:Adolescent; D000891:Anti-Infective Agents, Local / Q000627:therapeutic use; D000080984:Congenital Bone Marrow Failure Syndromes; D012534:Dental Scaling / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D017823:Furcation Defects / Q000628:therapy; D005889:Gingival Recession / Q000628:therapy; D005891:Gingivitis / Q000628:therapy; D006801:Humans; D009503:Neutropenia / Q000150:complications / Q000151:congenital*; D057747:Periodontal Debridement / Q000379:methods*; D010514:Periodontal Pocket / Q000628:therapy; D010518:Periodontitis / Q000209:etiology / Q000628:therapy*; D011206:Povidone-Iodine / Q000627:therapeutic use; D016745:Root Planing / Q000379:methods; D007507:Therapeutic Irrigation / Q000295:instrumentation / Q000379:methods
chemical_list: D000891:Anti-Infective Agents, Local; D011206:Povidone-Iodine