The Dental Aspects of X-Linked Hypophosphataemia

“Spontaneous” abscesses are a common dental consequence associated with XLH

Patients with XLH have an increased risk of developing periodontal disease with more severe consequences, such as premature tooth loss, even in young adults.

You may develop infections in the bone around the teeth, without any clinical or X-ray changes (no cavities or injury), contrary to what is conventionally observed. The barrier function of the enamel and dentine is compromised and bacteria can easily reach the pulp.

Once the pulp is infected, an ABSCESS (collection of pus) or a FISTULA (hollow tunnel that allows pus to escape) may develop.

Infection can sometimes spread rapidly to the facial tissue causing CELLULITIS, a swelling of the face with an alteration in the overall condition, but without any obvious clinical dental signs.

In case of an ABSCESS or FISTULA, you should URGENTLY see a dentist.
In case of CELLULITIS, you should see your dentist IMMEDIATELY, or go to the nearest emergency unit, to start antibiotics

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Clinical recommendations and prevention

It is recommended that adults and children should have dental visits every 6 months with sealing pits and fissures with flowable resin composite for children on both temporary and permanent teeth as soon and as frequently as possible.

Adults should have their teeth cleaned by a hygienist every 6 months. In addition to brushing (with high fluoride toothpaste) for 2 minutes twice a day, adults should rinse with fluoride mouthwash twice daily, along with flossing.

Top tips

    • Adults should not rinse or use mouthwash immediately after brushing as this washes away the fluoride which assists in protecting your teeth.
    • If possible, use mouthwash in the middle of the day AFTER lunch.
    • When shopping for mouthwash, look for some that contain fluoride.
    • If you have eaten breakfast or dinner, you should avoid brushing your teeth for at least 30 minutes afterwards. When you eat something, your mouth is often acidic and brushing while it is acidic can can soften tooth enamel for a time, and brushing too soon after eating them may damage the enamel while it’s in its weakened state.
    • If possible, brushing should always be done BEFORE breakfast.
    • Where possible, avoid sugary drinks and snacks inbetween meals.

NHS recommends

    • Children under 3 years old should brush twice daily, with a smear of toothpaste containing at least 1,000ppm fluoride.
    • Children between 3 and 6 years old should brush at least twice daily with a pea-sized amount of toothpaste containing more than 1,000ppm fluoride.
    • Adults should brush at least twice daily with a toothpaste containing 1,350-1,500ppm fluoride.

Anatomy of a normal tooth

Covers the crown of the tooth and acts as a protective barrier; it is a highly mineralised semi-transparent layer.

A less dense layer of hard (calcified) tissue beneath the enamel; the composition is similar to bone. The cohesion with enamel helps to cushion pressure on the tooth, especially when chewing, and it serves as a protective barrier for the pulp.

Tissue that is located in the tooth core, it is not calcified and contains the vessels and nerves of the tooth.

A calcified layer of tissue that covers the root of the tooth and anchors it in the jaw bone.

The specialised tissues that surround and support the teeth, maintaining them in the jaw bones.

Anatomy of an XLH affected tooth with dental abscess

The enamel is thinner and more prone to being worn down. Microscopic cracks are often present and allow bacteria to enter the pulp without the presence of cavities.

Dentine is less mineralised and may have structural defects allowing bacteria to enter the pulp; the barrier function is not very effective.

The pulp is generally enlarged and closer to the external surface of the tooth. Therefore, it is more vulnerable and susceptible to infections.

The pulp horns are more prominent.

It is thinner.

There may be abnormalities in the jaw bone, i.e. the support bones of the teeth.