Kostmann syndrome: oral aspects and 10-year follow-up case report

Kostmann syndrome (KS) is an autosomal recessive disorder characterized by a low neutrophil count and recurrent bacterial infections, including periodontal disease. This report describes the case of a 5-year-old female KS patient treated with regular infusions of granulocyte stimulating factor (granulokine), with primary complaint of gingival bleeding and tooth mobility, and followed up for 10 years. Conventional periodontal treatment and some extractions were performed, after neutrophil function tests using flow cytometry. The invasive procedures were carried out at an outpatient clinic under antibiotic prophylaxis due to a low neutrophil count and an impaired neutrophil function presented. During the 10-year follow-up period, despite episodes of recurrent gingivitis and periodontitis the present report describes the positive outcome of dental treatment of a patient with KS.