Sickle cell anemia, a genetic disease caused by a mutation in the beta-globin gene, can present oral manifestations such as delayed tooth eruption and hypomineralized enamel and dentin. The aim of the present study was to evaluate the prevalence and severity of developmental defects of enamel (DDE) and delayed tooth eruption in children with sickle cell anemia. The sample comprised 56 male and female children with sickle cell anemia aged 6 to 12 years and treated at the Hematology and Hemotherapy Center of Pernambuco, Brazil. The data were collected according to the WHO criteria for DDE and tooth eruption. The prevalence of DDE was 58.2% and increased with age, affecting 43.8% of children aged 6 to 8 years and 66.7% of those aged 10 to 12 years (p>0.05; Pearson's chi-square test). There was no significant association between DDE and sex; the most prevalent type of DDE was diffuse opacity (6.2%). Tooth eruption was delayed in 18 children (32.1%). The delay increased with age and was detected in 11.8% of children aged 6 to 8 years, in 20.0% of those aged 8 to 10 years and in 54.2% of those aged 10 to 12 years (p<0.05; Pearson's chi-square test). Delayed tooth eruption was higher in males (36.7%, p>0.05). The prevalence of DDE was high, increased with age and was similar between sexes, while delayed eruption was higher in males and showed a significant association with age.

To assess the health-related quality of life of children and adolescents with sickle cell disease (SCD). This is a cross-sectional study carried out with 97 male and female patients aged 5 to 18 years with clinical and laboratory diagnosis of SCD, seen at the Hematology and Hemotherapy Hospital of Pernambuco. The Pediatric Quality of Life InventoryTM questionnaires, version 4.0 – child/adolescent report – were applied. Most patients were female (76.3%), had sickle cell anemia (89.7%) and mixed race (59.8%). There was a significant association in the psychosocial domain between age groups 5 to 7 (72.10) and 8 to 12 years (p≤0.05), the latter of which had the worst quality of life score (65.36). There were significant differences between sexes only in patients aged 13 to 18 years for physical functioning and for the overall mean of the domains. The means in both cases were higher in female patients than in male ones (70.89 x 56.88 for physical functioning and 71.72 x 59.77 for the overall mean of the domains). Poor quality of life was observed among children and adolescents with SCD, with negative impacts according to age and sex.

Objective: To identify the association of occlusal disorders in patients with sickle cell disease (SCD). Study design: A literature review was conducted, and articles published between 2010 and 2019 were searched on Bireme and PubMed websites and in MEDLINE and LILACS databases, in English, Portuguese, and Spanish, using the keywords “malocclusion,” “sickle cell disease,” and “cephalometry,” combined by Boolean operators AND and OR. One of the criteria for the selection of articles was the presence of adolescents in the sample. This methodology followed the PRISMA recommendations. Seventy-nine articles were found, seven of which were included in the review as they met the inclusion criteria and the study goals. Results: The prevalence of malocclusion in SCD patients ranged from 62.9% to 100%, which was considered very severe in 30.1% to 80.6%. The most common occlusal changes were Angle’s class II malocclusion, increased maxillary overjet, and anterior open bite. In addition, class II skeletal pattern was the most prevalent due to mandibular retrusion. Conclusion: Malocclusion prevalence in SCD patients is high and considered to be a risk factor, with a significant rate of very severe malocclusion when compared to healthy patients.

Avicennia schaueriana is found in Brazilian mangrove coast. the cytotoxicity in vitro of the Aqueous extract of leaves of Avicennia schaueriana (AelAs) and the healing activity of the plant in cream on mice skin wounds were evaluated in this study. the cytotoxic evaluation was performed on Vero cells. the healing activity was evaluated on mice treated during 5, 10 and 15 days with cream at 5%, solution of sodium chloride at 0.9% and dexpanthenol in cream at 5%. the extract did not show cytotoxicity, but showed mitogenic activity (100μg/ml). In morphometric analysis, the percentage of wound contraction after 10 days was higher in dexpanthenol group (93.41%). In 15 days, the lowest percentage of contraction was observed in the dexpanthenol group (94.41%) and the highest in the AelAs cream group (98.50%). In histomorphometry the dexpanthenol showed the lowest length of re-epithelialization in 10 days. In 15 days, the AELAs cream group showed 100% of re-epithelialization. The number of fibroblasts found in AelAs cream group was higher than the saline solution in 10 days. In 15 days, AelAs cream group maintained a higher amount of fibroblasts when compared to the others. A. schaueriana did not show cytotoxicity. Furthermore, topical application of AelAs cream decreased the wound area, stimulated the re-epithelialization and increased the number of fibroblasts. The species A. schaueriana could become a topical treatment in tissue repair process.

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To analyze the profile of pain, its triggering factors, and the relationship with the use of hydroxyurea in children and adolescents with sickle cell disease. This was an analytical cross-sectional study of 80 patients with sickle cell disease, both male and female, aged 6 to 18 years, seen at the Center for Hematology and Hemotherapy of Pernambuco, Brazil. To assess the pain profile, forms with the adapted visual scale from the “Fear of Dental Pain Questionnaire – Short Form” were used. Fisher's exact test was used to evaluate the association between pain manifestations and the use of hydroxyurea. Pain was reported by 68.7% of the patients and 52.7% of them reported severe pain, which eventually affected their daily routines. Physical triggers presented the highest rate (78.2%). The most frequent sites of pain were the trunk (80%) and lower extremities (54.5%), and constricting (40%) and deep (40%) pain were the most frequent types. Thirty percent of the patients reported being treated with hydroxyurea for prevention of painful events. It is concluded that the patients with sickle cell disease showed a high rate of painful events and physical factors, such as cold temperature, trauma and physical effort, were considered to be the most prevalent. There was no association between the use of hydroxyurea and pain improvement.

BackgroundSickle cell disease (SCD) is the most important hemoglobinopathy in terms of frequency and social impact and can affect the stomatognathic system.AimTo assess and compare the developmental defects of the enamel (DDE) in children and adolescents with and without SCD.DesignThis was a cross‐sectional, analytical, and comparative study of 210 children and adolescents aged 5–18 years, who visited the Hematology and Hemotherapy Hospital of Pernambuco.ResultsDevelopmental defects of the enamel were observed in 55.2% of the SCD patients and 35.2% of the non‐SCD patients (healthy group; p < .05). In the SCD group, DDE were more common in females than in males (69.1% vs. 40.0%; p < .05). The incidence of DDE in the permanent teeth was higher in the upper arch than in the lower arch (SCD group, 13.1% vs. 4.6%; healthy group, 8.9% vs. 3.6%; p < .05).ConclusionCompared with the healthy group, the SCD patients were almost twice as likely to develop DDE, mostly affecting females and the permanent teeth. These findings suggest that individuals with SCD need early dental care to avoid future oral problems.

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