Introduction Among children with haemophilia and their caregivers; problems arising from the teeth and the surrounding tissues have an important role in the treatment of this disease and it affects the quality of life of children and their parents. Aim Aim of this study is to evaluate the oral health‐related quality of life of children with haemophilia from the perspective of their parents. Methods Paediatric oral health‐related quality of life (POQL) instrument was used in this cross‐sectional study for quality of life measurement. The research data collected by the questionnaire form were coded for scale items and personal information questions and transferred to SPSS, a multivariate statistical analysis program for social sciences. SPSS 23.0 (IBM Corp, Armonk, NY) package program was used for statistical analysis of the data. Results Primary dentition dmft scores of patients with haemophilia are higher than the control group; mean value of haemophilic group is 3.5 vs control group are 2.6, respectively (P = 0.034). In spite of higher dmft scores, the haemophilia and control groups have shown no significant difference in oral health‐related quality of life scores; median scores were 63.9 in haemophilic group and 85.3 in control group (P = 0.336), respectively. Conclusion In spite of lower oral health measures, children with haemophilia and their parents reported no difference in oral health‐related quality of life from their healthy counterparts.
Lower incisors moved forward and Incisor Mandibular Plane Angle (IMPA°) increased in both treatment groups. Statistically significant differences between the groups were found when comparing pre-treatment and post-treatment arch dimension and position of mandibular molars. Results were better for lingual arches with extraction on one side than with extraction on both sides Conclusions: A lingual arch seems to be an effective tool for maintaining arch length, and was not found to impair mandibular growth.
Objectives: This study aims to investigate the correlations between clinical features and mouth opening in patients with systemic sclerosis (SSc). Patients and methods: Eighty patients with SSc (13 males, 67 females; mean age 53.4±11.7 years; range, 31 to 76 years) followed in our clinic were enrolled in the study. Skin involvement was assessed using the modified Rodnan skin score (mRSS), joint/tendon involvement with fingertip to palm (FTP) distance and interstitial lung disease (ILD) was evaluated with clinical and radiologic methods. The interincisal distance (ID) measurement was used to assess the maximal mouth opening capacity. We examined associations between the ID and clinical features of SSc. Results: The ID was lower in females compared with males, and in diffuse type compared with the limited type (p<0.001 and p<0.001, respectively). A significant negative correlation was found between the ID with mRSS and FTP distance (p<0.001 and p=0.001, respectively). The ID was lower in patients with ILD than in patients without ILD (p=0.006). A formula was constituted to predict the maximal mouth opening of the patient with regression analysis. According to the formula, being male was associated with an increase of 6.14 mm, the presence of ILD with a decrease of 3.19 mm, every 10 mm increment in mRSS with a decrease of 3.72 mm and FTP distance >0 mm with a decrease of 5.13 mm in mouth opening. Conclusion: Microstomia is associated with low quality of life in patients with SSc. In our study, sex, ILD, mRSS, and FTP distance were observed to be the most important factors that were related with mouth opening in patients with SSc.
Background The causes for intrinsic tooth discoloration can be separated into two categories as systemic and local. Systemic causes are either genetic or drug‐induced effects. The development of dentition can also be affected by a number of systemic factors and metabolic diseases such as porphyria. Congenital erythropoietic porphyria (CEP), also known as Gunther's disease, is a metabolic disease caused by a transformation in the gene that codifies uroporphyrinogen‐3 synthesis, leading to porphyrin aggregation in urine, skin, bone, and dentin. Case Report A 21‐month‐old girl with erythrodontia was referred to Paediatric Dentistry Department in September 2017. A physical examination revealed blisters on her face, nose, hands, and feet. Laboratory findings showed highly elevated urine total uroporphyrin and total coproporphyrin I and III levels. Next‐generation sequencing multigene panel testing for porphyria demonstrated a homozygous c.10C>T (p.L4F) mutation in the UROS gene. For curative therapy, the patient was admitted to the allogeneic bone marrow transplantation program. Conclusion Congenital erythropoietic porphyria most commonly presents in the first few years of life. Manifestations can include reddish‐colored urine, skin blistering, scarring, and erythrodontia. A timely diagnosis can prevent undesirable skin findings of the disease and death due to hematological involvement before a curative allogeneic bone marrow transplantation is performed.
Objectives: This study aims to evaluate the effect of a home-based orofacial exercise program on the oral aperture (OA) of systemic sclerosis (SSc) patients. Patients and methods: This single-blind prospective randomized controlled study performed between March 2017 and January 2019 included 56 SSc patients (3 males, 53 females; mean age 52.9±10.6 years; range, 31 to 70 years) with an OA of <40 mm. Patients were divided into two groups. Group 1 (n=28, mean age 53.8±9.6 years) was given orofacial exercise program twice a day for one month in addition to oral hygiene care advices, followed by no activity for the next month. Group 2 (n=28, mean age 50.0±11 years) received oral hygiene care advices for the first month followed by the same exercise program for the next month. Patients' OA was measured at baseline, and at first and second months. Results: After the first month, OA increased in Group 1 (p<0.001), whereas no change was observed in Group 2 (p=0.579). At the end of two months, there was no additional increase in Group 1 (p=0.352), while there was a significant increase in Group 2 (p<0.001). There was no difference between OAs of the groups at the end of the trial (p=0.564). Conclusion:Our results suggest that home-based orofacial exercise program improves OA of SSc patients.
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