Clinical and Laboratory Studies of Severe Periodontal Disease in an Adolescent Associated With Hypophosphatasia. A Case Report

Abstract

Hypophosphatasia is a congenital disease characterized by defective bone mineralization, deficiency of alkaline phosphatase (ALP) activity, increased excretion of the phosphoethanolamine (PEA) in the urine, and premature loss of the deciduous teeth. A male hypophosphatasia patient (aged 15 years 6 months) with premature exfoliation of the deciduous teeth was referred to our hospital because of severe periodontal destruction in the permanent dentition. Blood and urine tests as well as oral and periodontal examinations were performed. Serum antibody titers against 7 periodontopathic bacteria by the enzyme-linked immunosorbent assay (ELISA), monocyte and neutrophil chemotaxis measurements, and cellular immunity tests were also performed. Low levels of ALP in serum and PEA in the urine were found. Monocyte and neutrophil chemotaxis exhibited normal values. Slightly depressed CD2+, CD3+, and CD4+ and slightly elevated activity of NK cells were found. An elevated level of serum antibody to Porphyromonas gingivalis was observed. Oral radiographic examination showed a mirror pattern of alveolar bone loss which is similar to that seen in localized juvenile periodontitis. Periodontal treatment of this patient was carried out for 4 years. The severely affected sites, the lower right and left first molars and the upper right first molar, had to be extracted. However, the other sites were well maintained. The serum IgG level against P. gingivalis was decreased after 4 years of periodontal treatment. Infection with P. gingivalis was suspected to be associated with the destruction of this patient's hypophosphatasia, but other dental abnormalities such as abnormal enamel, dentin, and cementum formation may also have contributed to the periodontal pathology.

Langerhans' Cell Histiocytosis in a 5‐Year‐Old Girl: Evidence of Periodontal Pathogens

“…Severe periodontitis is uncommon in children 13,14 . It is usually associated with systemic diseases such as hypophosphatasia, 15,16 Papillon‐Lefèvre syndrome, 17,18 Langerhans' cell histiocytosis, 7,8 neutropenia, 19 or leukocyte adhesion deficiency 20 . However, it has also been observed in systemically healthy children 8,14,21‐25 .…”

“…Other species also likely to be involved included Fusobacterium nucleatum , Eikenella corrodens , and P . gingivalis 8,15,21,22,25 . Among these, A .…”

Analysis of the periodontal microbiota in childhood-type hypophosphatasia

“…Baab et al (1985Baab et al ( , 1986 reported the presence of putative periodontal pathogens in the subgingival microflora and elevated levels of serum antibodies to the periodontal pathogen Porphyromonas gingivalis in HP patients. Watanabe et al (1993) observed a severe periodontal destruction in the permanent dentition associated with serum antibodies to Porphyromonas gingivalis in a subject suffering from HP.…”

“…Periodontitis can be viewed as a disturbance of the microbial ecology resulting in an increase in the number of Gram-negative and anaerobic bacteria. Case reports have described an association between HP and periodontitis (Baab et al, , 1986Page and Baab, 1985;Watanabe et al, 1993). Baab et al (1985Baab et al ( , 1986 reported the presence of putative periodontal pathogens in the subgingival microflora and elevated levels of serum antibodies to the periodontal pathogen Porphyromonas gingivalis in HP patients.…”

Rare case of generalised aggressive periodontitis in the primary dentition

“…Severe destruction of periodontal tissue in young children is mostly associated with systemic diseases such as Papillon-Lefèvre syndrome, hypophosphatasia, neutropenia or histiocytosis X (Shaw and Glenwright 1988;Watanabe et al 1993;Ishikawa et al 1994;Kamma et al 1998;Califano 2003). Only a few reports of aggressive periodontitis cases exist in the literature affecting the primary dentition without evidence of an underlying systemic disease (Suzuki et al 2003;Portaro et al 2008).…”