Congenital neutropenia is characterized by a decrease in the absolute number of circulating neutrophils and an increased susceptibility to infections. This paper describes the case history of a child with severe congenital neutropenia who had aggressive periodontitis associated with generalized carious lesions. She had Kostmann syndrome, which is associated with recessive mutation of the HAX-1 gene. She had extensive dental caries, which is not common in patients with Kostmann syndrome. The caries caused oral pain and loss of weight and could have been avoided if the parents had received early oral hygiene instructions, and if the child had received regular, professional dental care.
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