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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Recommended Dental Care
Due to the sensitivity of your teeth, you should take extra care of your dental hygiene.
- Choose a soft-bristled toothbrush and an age-appropriate fluoride toothpaste.
- Monitor and reduce your sugar consumption and brush your teeth after sugary snacks.
- Have regular dental check-ups for prevention and early treatment of any problems.
- Adults should have their teeth cleaned by a hygienist every 6 months.
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.
If you have just eaten, avoid brushing your teeth for at least 30 minutes afterwards. Brushing too soon after eating may damage the enamel, therefore, if possible, brushing should be done BEFORE breakfast.
X-linked hypophosphatemia (XLH) causes a number of dental development issues. The most common issues are delayed eruption of teeth (teeth may take longer to come in or may not come in at all) and enamel hypoplasia. This means that the enamel (the hard, protective coating on the outside of the teeth) is thinner or more porous than normal, making the teeth more susceptible to cavities and erosion. Other issues can be dental crowding and malocclusion (the teeth may not be properly aligned, which can cause overbite or underbite).
Visit your dentist or doctor for advice to improve calcification of both the teeth and periodontium and ask your dentist about night-time retainers and sealants for the grooves in your teeth. Your dentist, in agreement with your doctor, may provide you with orthodontic treatment.
Reference: Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia. Haffner D, et al. Nat Rev Nephrol 2019;15:435-455.
Anatomy of a Tooth
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.
It is thinner.