Permanent teeth in hypophosphatasia: light and electron microscopic study

Abstract

The changes in a permanent central incisor of a patient suffering from hypophosphatasia, were investigated light microscopically and compared with those of two of his primary teeth. In addition his other central incisor was studied with transmission and scanning EM. The changes in permanent teeth were similar to those of the primary dentition, including loss of cementum and the presence of deep resorption areas in dentin. Afibrillar cementum was detected with EM, and was attached to dentin surface. The presence of a thick layer of bacterial plaque was a prominent feature in all the teeth examined. A large number of bacteria was also found in resorption bays. It is suggested that bacteria may play an important role in the destruction of soft and hard tissues leading to loss of teeth in hypophosphatasia.

Periodontal manifestation of hypophosphatasia. A family case report

“…Apically areas of calcified tissue with cell inclusions at the side of the pulp chamber are visible (Fig. 5), similar to the "osteodentin" described by other authors (Brittain et al 1976, Bruckner et al 1962, El-Labban et al 1991.…”

Enzyme replacement prevents enamel defects in hypophosphatasia mice

“…In childhood HPP, premature loss of deciduous teeth (i.e., before age five years) is often the earliest sign of the disorder. (21–23, 39) In nearly all HPP patients, incisors are shed first; occasionally, almost the entire primary dentition is exfoliated prematurely. Dental radiographs sometimes show the enlarged pulp chambers and root canals characteristic of the “shell teeth” of rickets.…”

“…(15–20) Dysplasia or aplasia of cementum has been well documented histologically in HPP, and this abnormality explains the early exfoliation of deciduous teeth. (21–23) Irregular calcification of dentin and enlarged pulp chambers have also been documented. (17, 23–25) However, while one might suspect that formation of enamel, another highly mineralized tissue, would also depend on the local regulation of P i /PP i metabolism, there have been no conclusive reports of enamel defects in HPP patients, although some papers have alluded to enamel hypoplasia in this inborn error of metabolism.…”

Observations of root surfaces from patients with early‐onset periodontitis and leukocyte adhesion deficiency

“…In both the severe and miid forms of the disease, the deciduous teeth are primarily involved. The childhood form is characterized by premature exfoliation of the primary teeth and the adult form by loss of the permanent teeth (Primestone et al 1966, Jardon et al 1970, Whyte et al 1979, El-Labban et al 1991. In both types, serum alkaiine phosphatase levels are low and phosphoethanoiamine can be detected in urine.…”

“…Proceedings Worid Workshop in Ciinicai Periodontics 1989. Periodontitis occurring in the primary dentition may be associated with systemic disease (Vann & Oldenburg 1976, Jeddrychowski & Duperon 1979, Arnold & Hoffman 1979, Carrao et al 1979, Baehni et al 1983, Hathway 1983, Prichard et al 1984, El-Labban et al 1991 or immunodeficiency (Crowiey et al 1980, The views expressed in this article are those of the authors and do not refelct the official pohcy of the Department of Defense or orther Departments of the US Goverment. Arnout et al 1982, Bowen et al 1982, Anderson et ai.…”