Al-Otaibi, Basmah; (2024) Dental Management of Children with X-Linked Hypophosphatemia (XLH): A scoping review and exploration of patient-reported outcome measure (PROM). Doctoral thesis (D.Dent), UCL (University College London).

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Abstract

Introduction: X-Linked Hypophosphatemia (XLH) is a genetic multisystem disorder, characterised by low levels of phosphate in the blood causing distinctive skeletal and dental manifestations.// Aims and objectives: The aim was to review medical and dental management of paediatric patients with XLH in two parts; (i) the medical approach of XLH and its impact on dental manifestations such as recurrent spontaneous abscess (ii) the availability of dental guidelines, for instance whether endodontic treatment is superior to dental extraction for abscessed teeth.// The third part aimed to explore how paediatric patients with XLH seen at the Eastman Dental Hospital (EDH) feel about their dental health, using Patient-related outcome measure (PROM) questionnaire.// Methods: The eligibility for both scoping reviews was based on Population, Concept, and Context (PCC) framework. Population of interest in the medical and dental reviews were children under the age of 16 with XLH. The concept covered all available research in both fields of study, for medical review the timeframe was publications between 2018 and 2023, whereas dental review covered a wider duration from 2000 to 2022. The third part was a service evaluation based in the paediatric department at EDH. The questionnaire was tailored according to clinician and patient input, as well as piloting the PROM with two XLH patients and their parents. The finalised questionnaire was then distributed to all eligible patients between July and December 2023.// Results: The first scoping review yielded 14 articles from 21 countries researching the dental implications of medical therapies of XLH, 4 studies (28.5%) were multicentred covering a wider range of patients. 7 studies (50%) were in favour of burosumab for children with XLH. The second review included 37 articles covering dental aspect of XLH. The most mentioned dental manifestation was recurrent spontaneous abscess with 31 articles (83.7%) followed by malocclusion and dental caries with 8 articles (21.6%). Endodontic treatment was preferred for abscessed permanent teeth, while extraction was the choice for primary abscessed teeth. For the service evaluation, 13 children (aged 7 to 16 years) participated. Three patients (23%) reported feeling unhappy with their teeth, with 9 patients (69%) undergoing dental extractions, and 4 (31%) underwent endodontic treatment.// Conclusion: The scoping review did not conclude one medical treatment was superior in preventing dental manifestations, due to limited evidence and novelty of burosumab. Also, there are no clinical guidelines on the dental management of XLH, it remains subjective. Finally, the PROM demonstrated a wide range of complications these patients face, allowing clinicians to monitor their individual progress to ensure the provided treatment addresses their concerns and meets their dental needs.

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