Hypophosphatasia And Its Dental Manifestations

Hypophosphatasia (HPP) is a common genetic skeletal disorder that affects human bones and teeth, making them more fragile. This condition, in general, is not lethal; still, in some cases, it may decrease a patient's quality of life.

Dental symptoms typically help clinicians diagnose HPP – the condition of the teeth and enamel may indicate the disease even at an early age. Japan has a unique HPP research program led by Osaka University – every 5 years, they survey dental clinics in the country about the number of hypophosphatasia-affected patients. The main goal – is to discover and systematize the disorder's symptoms, expanding the understanding of that specific condition.

Recent follow-up

Last month, researchers published the previous survey's results, which was conducted between 2018 and 2022. First, the research authors highlight that the number of HPP-affected people who visited clinics seeking medical help has increased. These numbers demonstrate that growing awareness about the disorders and new treatment development positively affect national health, allowing people to seek medical help in their local clinics and hospitals:

10 years ago – 19 registered cases
5 years ago – 52 cases
Current research – 103 cases.

Disease details

According to the study authors, modern medicine identifies two main HPP types, both of which are related to dental problems:

Odonto-type disease – patients are more likely to lose teeth. (typically, people with this form of disease lose their first teeth before the age of 4 years)
Non-odonto-type – patients have tooth misalignment and tooth hypomineralization, causing enamel erosion.

Thus, while the first type is linked to losing teeth, the second one typically causes deformation – about 40% of patients with non-odonto-type have malocclusion.

Apparently, non-odonto-type HPP oral manifestation is linked to bad oral health – about 30% of patients had bad oral habits. Additionally, 25% of recipients with this form of the disease had dysphagia – while it's much rarer in odonto-type HPP.

How to treat it?

Severe forms of HPP, despite their type, lead to missing teeth, neurologic problems, and muscular hypotonia (probably due to challenging chewing function). Also, it has significant lethal rates, but, fortunately, 10 years ago, the AstraZeneca Rare Disease group introduced the so-called enzyme replacement therapy that alters the genetic mechanism of HPP, eliminating its symptoms.

Additionally, dentures are crucial for patients with missing teeth to restore proper chewing and, consequently, adequate nutrition. Also, providing patients with chewing/swallowing muscle training is essential in the case of severe disorder forms.

HPP isn't the only disease that dentists can detect during regular check-ups, which involve quizzing patients about their medical histories. Read other news about the role of dentists in general diagnostics on our Newsfeed:

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