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  • Article
    Citation - Scopus: 2
    Clinical and Demographic Characteristics of Cutaneous Mastocytosis in Childhood: Single-Center Experience
    (Lippincott Williams and Wilkins, 2022) Erdem,A.Y.; Özyörük,D.; Emir,S.; Çakmakçı,S.; Ceylan,G.G.; Toyran,M.; Mısırlıoğlu,E.D.
    Introduction: Mastocytosis is a rare and heterogenous disease, and in children it is generally limited to the skin and tends to regress spontaneously in adolescence. Aim: In this study, demographic, clinical, and laboratory characteristics of pediatric patients with mastocytosis, and also coexisting diseases were investigated. Results: A total of 61 pediatric patients were included in the study. The male-to-female ratio was 2.2, the median age was 2 years (range, 0.25 to 19 y), and the median follow-up period was 2.0 years (range, 0.25 to 19 y). Types of clinical presentation at diagnosis consisted of mainly urticaria pigmentosa (45.9%). Seven patients were further investigated with suspicion of systemic mastocytosis, they were followed up, median of 9 years (range, 2.5 to 16 y), and none of them developed systemic disease. Coexisting allergic diseases were recorded in total 5 patients (8.2%). Three patients had immunoglobulin A deficiency, 1 patient had elevated immunoglobulin E level. A patient developed mature B-cell lymphoma with a heterozygous mutation in c-KIT exon 11. Discussion: Cutaneous mastocytosis in children may present as a complex disease with different clinical signs and symptoms. Standardized clinical criteria and guidelines for the follow-up of children with mastocytosis are required. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
  • Article
    Development and Psychometric Evaluation of the Treatment Management Adherence Scale for Children With Multiple Sclerosis
    (Elsevier Sci Ltd, 2024) Yuksel, Didem; Yardimci, Figen
    Background: Pediatric multiple sclerosis (pMS) is a chronic inflammatory, demyelinating, and neurodegenerative disease affecting the central nervous system in children and adolescents The aim of this correlational, comparative study was to develop an assessment scale for adherence to treatment management in pMS. Methods: Two measurement tools were used to develop a scientifically sound tool to assess adherence in pediatric patients (12-18 years) diagnosed with multiple sclerosis (MS). Cases of pMS (n = 120) in 7 hospitals in Turkey were included between August 2021-February 2022. The tools were a "Sociodemographic and Disease-Related Information" and a newly developed "Treatment Management Adherence Scale for Children with Multiple Sclerosis". The form and questionnaire were completed by the children through online using the Zoom platform in approximately 10 min. The questionnaire on adherence contains 16 items related to the disease and treatment, scored in a 5-point Likert type. Face validity was established by pretesting with 20 children, and construct validity was established using the statistical methods of exploratory factor analysis and confirmatory factor analysis. For the reliability of the scale, Cronbach's Alpha and omega coefficients, item test correlation values, split-half, test-retest techniques were used. Results: There were 120 eligible patients, 71.2 % girls, with mean age (fSD) 13,6 f 2,2 years at disease onset and 15,7 f 1,5 at the time of the study, all under disease-modifying therapy. The sample size and items were sufficient to conduct a factor analysis. The Cronbach's Alpha and Omega value was 0.75, indicating participants' opinions were consistent across items. The mean content validity index was 0.93, showing the scale represented the measured data, and the exploratory factor analysis showed the scale measures adherence in 55 % of patients (desired figures: >0.80 and 40-60 % respectively). The 16 items of the questionnaire were grouped into 4 dimensions. These dimensions were termed 'physiological', 'self-concept', 'role function' and 'interdependence', in line with different styles of adaptation. The total score can be between 16 and 80, with higher scores indicating strong adherence to treatment. The mean total score of 54,3 f 9,53 (min=31, max= 75) in this study was in the "moderate adherence" range. Conclusions: This new scale is the first to assess adherence in pMS. The study supports its validity, reliability, and likelihood to address adjustment issues in children and adolescents with MS accurately and can be recommended for clinical use.
  • Article
    Citation - WoS: 3
    Citation - Scopus: 4
    Levels of Physical Activity and Physical Fitness in Pediatric Pacemaker Patients: a Cross-Sectional Study
    (Springer, 2020) Teberl, Sena; Saglam, Melda; Ertugrul, Ilker; Vardar-Yagli, Naciye; Cakmak, Aslihan; Calik-Kutukcu, Ebru; Karagoz, Tevfik
    Children with heart diseases have reduced physical activity (PA) levels relative to their peers, which in turn increases cardiovascular risk. To the best of our knowledge, physical fitness and objectively measured PA levels have not been previously studied in children with pacemakers. We evaluated PA levels and physical fitness in pediatric pacemaker patients compared to their healthy peers. Twenty-eight pediatric patients with pacemakers (15 female, 13 male; mean age 13.43 +/- 3.68 years) and 24 healthy subjects (14 female, 10 male; mean age 13.08 +/- 3.67 years) were included. Physical fitness was assessed using the Munich Fitness Test (MFT). SenseWear Armband metabolic Holter device was used to record the PA for 7 consecutive days. MFT total and sub-parameter scores were significantly lower in the patient group (p < 0.05). Patients' total and active energy expenditure, PA level, total distance, number of steps, and vigorous PA were significantly lower than those of healthy children (p < 0.05). Sedentary activity and light, moderate, and very vigorous PA durations were similar in both groups (p > 0.05). Duration of mean moderate to vigorous PA was higher than 60 min/day recommended in PA guidelines in both patients and healthy subjects. These results provide initial data on PA and fitness in children with pacemakers and suggest that physical fitness and activity levels in children with pacemakers are lower than in healthy peers. Appropriate exercise programs may improve PA levels in pediatric pacemaker patients. Awareness of the importance of PA should be raised among the parents and families of these children.