Assessment of Factor Affecting the Quality of Life in Children with Juvenile Idiopathic Arthritis

Abstract Introduction and Objectives: Juvenile idiopathic arthritis (JIA) is a frequently seen chronic rheumatoid disease in childhood, which may cause disability and severely affect quality of life (QoL). The aim of present study was to assess relationships between disease activation and socio-cultural status of family, QoL, anxiety level, and depression level in patients with JIA and their parents. Methods: The study included 100 patients with JIA. The socio-demographic data were obtained from all patients. Child- and parent-reported PedsQL, Beck depression inventory (BDI), Kovacs' Child Depression Inventory (CDI), SCARED child version, CHAQ discomfort and disability scales were applied and JADAS-27 score was calculated in a cross-sectional manner. Then, we compared the characteristics of patients with the scales’ results. Results: JADAS-27, BDI, and CHAQ discomfort scores were higher and child- and parent-reported PedsQL scores were lower in patients with active disease than patients on remission (p<0.05). The SCARED score was higher in girls than boys. The CHAQ disability score was high in children aged 8-12 years (p<0.05). JADAS-27 and CHAQ disability scores were significantly low in patients with better compliance to treatment. Parental statements about changes in mental health after diagnosis were consistent with results of depression and anxiety scales of children. Conclusions: Quality of life is adversely affected in children with JIA, which may result in depression and anxiety. In management of JIA, one of our goals should be maintaining QoL. Further comprehensive studies in relationships between QoL and depression, anxiety, socio-demographic parameters, disease activation and social circle of patient are needed

Antioxidant and Antimicrobial Properties of Cistus Ladanifer

Different parts of Cistus species are traditionally used in folk medicine as a remedy for several microbial disorders and infections. At the beginning of the study, crude extract of the Cistus leaves was obtained to determine its antioxidant and antimicrobial activities. Then volatile compounds were extracted using hydrodistillation and hexane. The Cistus oil was obtained by hydrodistillation of fresh material, using leaves in an altered Clevenger-type device. Aqueous extract of Cistus leaves was neutralized to precipitate gums. The yield of gum was determined as 1%. Resinoid was obtained after ethanol extraction of gum. Antibacterial activities of extract were determined using disc diffusion and micro-dilution assays against gram-positive and gram-negative bacteria. The extract of Cistus leaves tested and exhibited antibacterial activities by inhibiting one or more microorganisms. The tested plant extract was more active against gram-positive bacteria compared with gram-negative bacteria. Total phenol content of the extract was determined with Folin-ciocalteu method. Total phenolic content of Cistus extract was 520 ± 15 mg Gallic Acid Equivalent (GAE)/ g extract. The water soluble (ACW) and lipid soluble (ACL) antioxidant capacities of the extract were also determined. ACW and ACL antioxidant capacities of Cistus extract were found as 650±80 μg Ascorbic acid/ mg extract and 540 ±30 μg Trolox Equivalent/ mg extract, respectively. In this study, there is a clear relationship between the total antioxidant and phenol content analysis results and antibacterial activities. Hereby, bioactive natural compounds present in Cistus species can be used as natural raw material in some related industrial applications

Evaluation of Baseline Characteristics and Prognostic Factors in Multisystemic Inflammatory Syndrome in Children: Is It Possible to Foresee the Prognosis in the First Step?

Background: Two years after the first cases, critical gaps remain in identifying prognostic factors in multisystem inflammatory syndrome in children (MIS-C). Methods: This retrospective study included 99 patients with MIS-C hospitalized between August 2020 and March 2022 in a pediatric tertiary center. The patients were divided into two groups according to clinical severity (low- and high-risk). Prognostic values of baseline clinical and laboratory characteristics were evaluated with advanced statistical analysis, including machine learning. Results: Sixty-three patients were male, and the median age was 83 (3–205) months. Fifty-nine patients (59.6%) were low-risk cases. Patients aged six years and over tended to be at higher risk. Involvement of aortic or tricuspid valve or >1 valve was more frequent in the high-risk group. Mortality in previously healthy children was 3.2%. Intensive care unit admission and mortality rate in the high-risk group were 37.5% and 7.5%, respectively. At admission, high-risk patients were more likely to have reduced lymphocyte count and total protein level and increased brain natriuretic peptide (BNP), ferritin, D-dimer, and troponin concentrations. The multiple logistic regression model showed that BNP, total protein, and troponin were associated with higher risk. When the laboratory parameters were used together, BNP, total protein, ferritin, and D-dimer provided the highest contribution to the discrimination of the risk groups (100%, 89.6%, 85.6%, and 55.8%, respectively). Conclusions: Our study widely evaluates and points to some clinical and laboratory parameters that, at admission, may indicate a more severe course. Modeling studies with larger sample groups are strongly needed

Comparison of refugee patients with cystic fibrosis and their counterpart children from Turkey during the war

Since the outbreak of the Syrian civil war in 2011, the population of Arab refugees in Turkey has rapidly increased. While cystic fibrosis (CF) is believed to be rare among Arabs, recent studies suggest it is underdiagnosed. This study aims to present the demographic, clinical, and genetic characteristics of CF patients among Arab refugees in Turkey. Additionally, a comparison is made between the findings in the National CF Registry 2021 in Turkey (NCFRT) and the refugee CF patient group. The study included refugee patients between the ages of 0 and 18 years who were diagnosed with CF and received ongoing care at pediatric pulmonology centers from March 2011 to March 2021. The study examined demographic information, age at diagnosis, age of diagnosis of patients through CF newborn screening (NBS), presenting symptoms, CF transmembrane conductance regulator (CFTR) mutation test results, sputum culture results, weight, height, and body mass index (BMI) z score. Their results were compared with the NCFRT results. The study included 14 pediatric pulmonology centers and 87 patients, consisting of 46 (52.9%) boys and 41 (47.1%) girls. All of the patients were Arab refugees, with 80 (92%) being Syrian. All the patients were diagnosed in Turkey. The median age at diagnosis of patients was 22.33 (interquartile range, 1–258) months. The median age of diagnosis of patients through NBS was 4.2 (interquartile range, 1–12) months. The median age of older patients, who were unable to be included in the NBS program, was 32.3 (interquartile range, 3–258) months. Parental consanguinity was observed in 52 (59.7%) patients. The mutation that was most frequently found was F508del, which accounted for 22.2% of the cases. It was present in 20 patients, constituting 32 out of the total 144 alleles. There was a large number of genetic variations. CFTR genotyping could not be conducted for 12 patients. These patients had high sweat tests, and their genetic mutations could not be determined due to a lack of data. Compared to NCFRT, refugee patients were diagnosed later, and long-term follow-up of refugee CF patients had significantly worse nutritional status and pseudomonas colonization. Conclusion: Although refugee CF patients have equal access to NBS programs and CF medications as well as Turkish patients, the median age at diagnosis of patients, the median age of diagnosis of patients through NBS, their nutritional status, and Pseudomonas colonization were significantly worse than Turkish patients, which may be related to the difficulties of living in another country and poor living conditions. The high genetic heterogeneity and rare mutations detected in the refugee patient group compared to Turkish patients. Well-programmed NBS programs, thorough genetic studies, and the enhancement of living conditions for refugee patients in the countries they relocate to can have several advantages such as early detection and improved prognosis. What is Known: • Children who have chronic diseases are the group that is most affected by wars. • The outcome gets better with early diagnosis and treatment in patients with Cystic Fibrosis (CF). What is New: • Through the implementation of a newborn screening program, which has never been done in Syria previously, refugee patients, the majority of whom are Syrians were diagnosed with cystic fibrosis within a duration of 4 months. • Despite equal access to the newborn screening program and CF medications for both Turkish patients and refugee patients, the challenges of living in a foreign country have an impact on refugees

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration.Conclusions: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. What is Known:center dot During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring.What is New:center dot There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment.center dot This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests