Careful examination of the oral cavity may reveal findings indicative of an underlying systemic condition, and allow for early diagnosis and treatment Examination should include evaluation for mucosal changes, periodontal inflammation and bleeding, and general condition of the teeth Oral findings of anemia may include mucosal pallor, atrophic glossitis, and candidiasis Oral ulceration may be found in patients with lupus erythematosus, pemphigus vulgaris, or Crohn disease Additional oral manifestations of lupus erythematosus may include honeycomb plaques (silvery white, scarred plaques); raised keratotic plaques (verrucous lupus erythematosus); and nonspecific erythema, purpura, petechiae, and cheilitis Additional oral findings in patients with Crohn disease may include diffuse mucosal swelling, cobblestone mucosa, and localized mucogingivitis Diffuse melanin pigmentation may be an early manifestation of Addison disease Severe periodontal inflammation or bleeding should prompt investigation of conditions such as diabetes mellitus, human immunodeficiency virus infection, thrombocytopenia, and leukemia In patients with gastroesophageal reflux disease, bulimia, or anorexia, exposure of tooth enamel to acidic gastric contents may cause irreversible dental erosion Severe erosion may require dental restorative treatment In patients with pemphigus vulgaris, thrombocytopenia, or Crohn disease, oral changes may be the first sign of disease

Oral manifestations of systemic disease

Beta-thalassemia and particularly its transfusion-dependent form (TDT) is a demanding clinical condition, requiring life-long care and follow-up, ideally in specialized centers and by multidisciplinary teams of experts. Despite the significant progress in TDT diagnosis and treatment over the past decades that has dramatically improved patients’ prognosis, its management remains challenging. On one hand, diagnostic and therapeutic advances are not equally applied to all patients across the world, particularly in several high-prevalence eastern regions. On the other, healthcare systems in low-prevalence western countries that have recently received large numbers of migrant thalassemia patients, were not ready to address patients’ special needs. Thalassaemia International Federation (TIF), a global patient-driven umbrella federation with 232 member-associations in 62 countries, strives for equal access to quality care for all patients suffering from thalassemia or other hemoglobinopathies in every part of the world by promoting education, research, awareness, and advocacy. One of TIF’s main actions is the development and dissemination of clinical practice guidelines for the management of these patients. In 2021, the fourth edition of TIF’s guidelines for the management of TDT was published. The full text provides detailed information on the management of TDT patients and the clinical presentation, pathophysiology, diagnostic approach, and treatment of disease complications or other clinical entities that may occur in these patients, while also covering relevant psychosocial and organizational issues. The present document is a summary of the 2021 TIF guidelines for TDT that focuses mainly on clinical practice issues and recommendations. 

2021 Thalassaemia International Federation Guidelines for the Management of Transfusion-dependent Thalassemia

https://link.springer.com/content/pdf/10.1186%2Fs40510-016-0120-6.pdf

Prevalence of malocclusion and assessment of treatment needs in β-thalassemia major children

Objectives: To provide a description of mesiodistal crown diameters (MD) and tooth-size discrepancy (TSD) of the permanent dentition in patients with thalassemia major (TM) and to compare the results with those of unaffected control group. Study design: The sample consisted of 46 thalassemic patients, 25 males and 21 females aged 7.3 to 23.7 years (mean ± SD = 11.2 ± 3.9 years) and control group matched by age and sex. Dental casts of the participants were measured for MD, using a digital sliding caliper. Descriptive statistics were computed for each individual tooth. Student t-test was used for comparison of mean values between males and females as well as between thalassemic and control groups. The differences between sets of data were evaluated by analysis of variance (ANOVA). Results: All means for MD of thalassemic males and females were smaller than their controls, with 20 of the 24 comparisons being statistically significant (ranged from P<0.05 to P<0.001). In both thalassemic and control groups, males exhibited significantly larger MD than females in most instances. Canines displayed the most sexual dimorphic teeth in the dentition. Lateral incisors showed the greatest variability indicated by the coefficient of variation, while the first molars were the least variable teeth (7.6% vs. 5.3%). There were no statistically significant differences in the anterior and overall tooth-size discrepancy ratios between sexes or between thalassemic and control groups. The mean anterior ratio (79.5%) and overall ratio (92.4%) of the control group were significantly larger than of Bolton ratios; P< 0.001 and P<0.05, respectively. Conclusions: The present study demonstrated that thalassemic males and females exhibited significantly smaller MD than the control group. The TSD ratios in both thalassemic and control groups were significantly larger than those of Bolton sample. These findings should be taken into account when planning orthodontic treatment for thalassemic patients. 

Key words:Permanent dentition, tooth size, thalassemia major.

/pdf/mesiodistal-crown-diameters-and-tooth-size-discrepancy-of-51wb8bysa5.pdf

Mesiodistal crown diameters and tooth size discrepancy of permanent dentition in thalassemic patients.

Association of thalassemia major and gingival inflammation: A pilot study

Objective: To assess the prevalence of periodontal disease, orofacial changes and craniofacial abnormalities in patients with thalassemia major (TM). Dental management is discussed. Study design: The sample consisted of 54 patients with TM, 31 males and 23 females aged 5.5 to 18.3 years, with the mean age (± SD) of 11.6 ± 3.2 years. The sample was divided into two subgroups according to age. A similar number of unaffected control group matched by age and sex served as a control. Clinical and radiographic examinations were carried out to assess the prevalence of changes caused by this disorder. Student’s t-test was used to compare the means between thalassemic group and the control group. The Chi-square test was employed to determine statistical differences in frequencies between the two groups. Results: Poor oral hygiene and gingivitis were observed in 61.1% and 43.0% of the thalassemic patients, respectively. The overall mean plaque score was 1.66 ± 0.51 and gingival score 1.43 ± 0.59. In all tested peri...

Periodontal condition and orofacial changes in patients with thalassemia major: a clinical and radiographic overview.

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Periodontal condition and orofacial changes in patients with thalassemia major: a clinical and radiographic overview.