Investigation of Dental Health in Children With Neutrophil Defects: A Clinical Study (GOSH)
Primary Immune Deficiency Disorder, Periodontitis
About this trial
This is an interventional treatment trial for Primary Immune Deficiency Disorder focused on measuring primary immunodeficiency, dental health, neutrophil defect, periodontitis
Eligibility Criteria
Inclusion Criteria
Each participant must meet the following inclusion criteria to be enrolled in the study:
- Age 4-16 years
Diagnosis of one of the following neutrophil defects (as confirmed by the treating physician):
Disorders of neutrophil numbers
- Cyclic neutropenia
- Congenital neutropenia
- Severe Congenital Neutropoenia
- X-linked Neutropoenia
Disorders of neutrophil function
- Leucocyte Adhesion Defect s
- Other neutrophil disorders/ undefined neutrophil disorders (functional defects demonstrated on testing but genetic basis not yet known)
Combined immunodeficiency syndromes
- Wiskott Aldrich Syndrome
- Hyper IgM Syndrome
- Chediak Higashi Syndrome
- Undefined combined immunodeficiency (functional defects demonstrated on testing but genetic basis not yet known)
- Participants/parents/guardians willing to sign the informed consent Exclusion Criteria Participants will not be eligible for participation in the study if unwilling to be examined or unable to return for follow-up appointments.
Sites / Locations
- Center for Oral Clinical Research, Queen Mary, University of London
- Dental and Maxillofacial Department, Great Ormond Street Hospital for Children
Arms of the Study
Arm 1
Experimental
Non surgical Periodontal therapy
All children will be offered an oral hygiene and tooth scaling and polishing session. For children affected by periodontal disease, subgingival debridement will also be performed according to needs, with the aim to disrupt the subgingival biofilm responsible for the onset and progression of the periodontal pathology. This can be accompanied, when appropriate, by the use of local anaesthesia and adjunctive antimicrobials (systemic or locally applied subgingivally), according to the clinical presentation and the child's medical conditions. In addition, when required, nitrous oxide sedation (NOS) could be used before treatment. Caries will be treated as necessary (shared care with the general dental practitioners / community dental services). Children with mucosal lesions requiring treatment will be given a letter for their GDP suggesting referral to a collaborating expert in Oral Medicine (Prof. Stephen Porter).