Infantile genetic agranulocytosis (Kostmann type). A case report.
Severe periodontitis in a patient with infantile genetic agranulocytosis (Kostmann syndrome) is presented. This rare syndrome is inherited as an autosomal recessive pattern and characterized by severe neutropenia. The periodontal status and treatment of the patient is described. It is concluded that periodontal therapy including scaling, root planning, soft tissue curettage, and the use of selected antimicrobial agents can be successful in this particular syndrome.
D000328:Adult; D000380:Agranulocytosis / Q000235:genetics*; D000891:Anti-Infective Agents, Local / Q000627:therapeutic use; D002710:Chlorhexidine / Q000031:analogs & derivatives / Q000627:therapeutic use; D003131:Combined Modality Therapy; D012534:Dental Scaling; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D009503:Neutropenia / Q000235:genetics; D010518:Periodontitis / Q000209:etiology* / Q000628:therapy; D016745:Root Planing; D013357:Subgingival Curettage
D000891:Anti-Infective Agents, Local; C010882:chlorhexidine gluconate; D002710:Chlorhexidine