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Periodontal Disease in Patients From the Original Kostmann Family With Severe Congenital Neutropenia
Author(s) -
Carlsson Göran,
Wahlin YlvaBritt,
Johansson Anders,
Olsson Anders,
Eriksson Torbjörn,
Claesson Rolf,
Hänström Lennart,
Henter JanInge
Publication year - 2006
Publication title -
journal of periodontology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.036
H-Index - 156
eISSN - 1943-3670
pISSN - 0022-3492
DOI - 10.1902/jop.2006.050191
Subject(s) - congenital neutropenia , medicine , neutropenia , periodontitis , gingivitis , actinobacillus , chronic granulomatous disease , immunology , absolute neutrophil count , oral microbiology , dentistry , biology , chemotherapy , bacteria , genetics
Background: Patients with Kostmann syndrome (severe congenital neutropenia [SCN]) typically normalize their absolute neutrophil count (ANC) upon granulocyte colony‐stimulating factor (G‐CSF) therapy. However, although they no longer experience life‐threatening bacterial infections, they frequently still have recurrent gingivitis and even severe periodontitis, often starting in early childhood. Methods: We studied the periodontal disease in the four surviving patients belonging to the family originally described by Kostmann. Their odontological records, x‐rays, color photos, bacterial cultures, serum antibodies to oral bacteria, and histopathological examinations were reviewed. The data were also correlated to previous investigations on their antibacterial peptides and molecular biology. Results: Three patients had periodontal disease, despite normal ANC and professional dental care, and had neutrophils deficient in antibacterial peptides. One of these patients also had a heterozygous mutation in the neutrophil elastase gene, had severe periodontal disease and overgrowth of the periodontal pathogen Actinobacillus actinomycetemcomitans in the dental flora, and 15 permanent teeth had been extracted by the age of 27. One bone marrow–transplanted patient had no periodontal disease. Conclusions: Normalized ANC levels are not sufficient to maintain normal oral health in SCN patients, and because neutrophils are important for first‐line defense and innate immunity, the deficiency of the antibacterial peptide LL‐37 probably explains their chronic periodontal disease. Professional dental care is still important for SCN patients, despite treatment with G‐CSF and normal ANC levels. Whether antibacterial peptides play a role in the pathogenesis of periodontitis in other patients remains to be elucidated.