With enzyme replacement therapy now available to treat hypophosphatasia, a rare skeletal disease but with a relatively higher prevalence among rare bone disorders, Japanese researchers say their findings will help dentists identify suspected cases earlier.
Published in Scientific Reports, researchers from Osaka University analyzed the dental issues faced by Japanese patients in their largest survey to date.
“The trends that we observed in this study will be helpful for dentists who might come across patients with hypophosphatasia in their clinics,” says Kazuhiko Nakano, senior author. “Improved knowledge of the various ways this disease presents will lead to better diagnostic rates and earlier treatments for affected patients.”
Since 2013, researchers have conducted nationwide surveys of dental clinics every five years. In their most recent survey, they collected data from 103 cases across 30 clinics, a significant increase from the 19 and 52 cases recorded in the previous two surveys.
“We had enough cases that we could break them down into two groups—those whose symptoms involved the teeth only, known as odonto-type disease, and those who had other symptoms, which we termed non-odonto-type disease,” explains Rena Okawa, lead author of the study. “We were surprised to see big differences in oral manifestations between the two groups.”
Two groups
Patients with odonto-type hypophosphatasia were more likely to lose their first teeth early, whereas those with non-odonto-type disease were more likely to experience tooth misalignment and tooth hypomineralization, a condition in which the enamel softens.
Non-odonto-type disease was also linked to poor oral habits, such as finger sucking and tongue thrusting, as well as difficulties with swallowing.
In Canada, severe hypophosphatasia is estimated to occur in approximately 1 in 100,000 live births. However, it is particularly frequent in a Mennonite population in Manitoba, Canada, where about 1 in 2,500 infants is born with severe features of the condition.
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