Oral health status of patients with inherited bone marrow failure syndromes

Introduction/aim

Inherited bone marrow failure syndromes (IBMFS), including Fanconi anemia (FA), Diamond-Blackfan anemia (DBA) and congenital neutropenia (CN), are rare diseases related to impaired hematopoiesis in different hematopoietic cell lineages in the bone marrow. Oral health may be impaired in these patients related to the disease itself, due to cytopenia, or related to the drugs the patients use, such as glucocorticoids. The present research was aimed to present the oral health status of patients with IBMFS.

Methods

This descriptive study was conducted with 27 children and adolescents (11 FA,9 DBA,7 CN). The data was collected via a structured and pre-tested questionnaire and by oral-dental examination clinically and, if needed, radiographically. Dental caries was evaluated with “Decayed, Missing, Filled Tooth/Surface indices (dmft/s, DMFT/S)” and “International-Caries-Detection-and-Evaluation-System-(ICDAS-II)”. The patients' oral hygiene and gingival health status were recorded with “plaque and gingival index”, respectively. Statistical analysis was carried out by using Statistical-Package-for-the-Social-Sciences-Version-20.0.

Results

Of all groups, 54% were found to have no regular teeth brushing habits; 21 patients (77.8%) had initial or cavitated dental caries according to ICDAS–II–index-system. There are various oral mucosal lesions (aphthous lesions, dry chipped lips, angular cheilitis, leukoplakia, hairy and geographic tongue) and dental abnormalities (microdontia, taurodontism, rotation, ankylosis, hypoplastic teeth, germ deficiency, cingulum hypertropia, dens invaginatus, pulp stones) were detected.

Conclusions

Extensive dental caries, gingival inflammation and inadequate oral hygiene status, showed the necessity of early dental visits. Adopting regular dental counseling as part of the multi-disciplinary team approach for patients with IBMFS is necessary to ensure better general and oral health.