Objective In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short-and long-term follow-up. Methods MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively. Results A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p = 0.005, p < 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/ hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short-and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization. Conclusion MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points• In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.
BackgroundThis study aimed to investigate the characteristics of patients with preadolescent‐ and adolescent‐onset immunoglobulin A vasculitis (IgAV) and to determine whether age affects IgAV outcomes in adolescents.MethodsDemographic, clinical, and laboratory data of 333 patients diagnosed with IgAV at the Department of Pediatric Rheumatology, University of Health Sciences, Ankara City Hospital, were evaluated retrospectively. The patients were classified into two groups: preadolescents (<10 years) and adolescents (10–19 years). Subgroup analyses were also performed by grouping the adolescent patients into early, middle, and late adolescent groups.ResultsOf the 333 patients, 219 (65.8%) and 114 (34.2%) were preadolescents and adolescents. Palpable purpura, renal, joint, and gastrointestinal (GI) tract involvement were detected in 333 (100%), 78 (23.4%), 79 (23.7%), and 124 (37.2%) patients, respectively; testicular involvement was observed in 25 (13.3%) of 187 male patients. The frequency of renal involvement was significantly higher in the adolescent group than in the preadolescent group at the time of diagnosis (p = 0.030). Notably, joint involvement was significantly higher in the adolescent group (p = 0.001). The need for aggressive therapy was significantly higher in the adolescent group than in the preadolescent group (p = 0.003). There was no significant difference in clinical data, demographic characteristics, and laboratory findings between the adolescent subgroups (p > 0.05).ConclusionsImmunoglobulin A vasculitis can occur at any age but the disease prognosis appears to worsen with age. The present study reported that joint involvement, kidney involvement, and the need for more aggressive treatment were higher in the adolescent group than in the preadolescent group.
Objective: Juvenile idiopathic arthritis (JIA) is a childhood rheumatic disease that causes joint inflammation and tissue damage. Non-infectious uveitis is the most common extra-articular manifestation of JIA.The aim of this study is to evaluate the risk factors that play a role in occurrence and recurrence of uveitis and, to determine the relationship between arthritis and uveitis activity in patients with JIA. Material and Methods: This retrospective, cross sectional study included JIA patients with/without uveitis from a referral center in Turkey. The Juvenile Arthritis Disease Activity Score was used to evaluate the disease activity and calculated for arthritis and uveitis separately. Results: Uveitis was seen in 26 (13.3%) of 195 JIA patients. Of 26 JIA associated uveitis (JIA-U) patients, 19 (73%) had an oligoarticular subtype. The median age at diagnosis of JIA with uveitis was younger than without uveitis (p=0.015). Oligoarticular JIA was found to be associated with recurrence of uveitis (p=0.021). The occurrence age of arthritis and uveitis was significantly younger in patients with recurrent uveitis (p=0.041, p=0.002, respectively). The median JADAS27 score at the onset of uveitis was lower in the recurrent group (p=0.038). Conclusion: Early age is a significant risk factor for occurrence and recurrence of uveitis. It is important to remember that, during the disease course, patients with low disease activity may also develop uveitis.
Çocukluk çağının en sık görülen vasküliti olan immünglobulin A vasküliti (IgAV) küçük damarlarda immünglobulin A ve immün kompleks birikimi ile karakterize, genellikle kendi kendini sınırlayan sistemik bir vaskülittir. Spesifik bir laboratuvar testi olmayan IgAV’ne klinik ve histopatolojik bulgular ile tanı konulmaktadır. Kesin prognostik parametreler henüz net olarak tanımlanmamakla beraber nüksler yaygındır. Bu çalışmada, IgAV’nin demografik, klinik özellikleri ve laboratuvar belirteçleri değerlendirilerek nüksleri öngörebilecek risk faktörlerinin belirlenmesi amaçlanmıştır. Ankara Şehir Hastanesi Çocuk Romatolojisi Kliniği’nde Ocak 2012 ile Haziran 2021 tarihleri arasında takip edilen IgAV tanılı 318 hastanın klinik ve laboratuvar verileri geriye dönük olarak değerlendirildi. Çalışmada yer alan 318 olgunun 17’sinde (%5,3) nüks gelişti. Nüks gelişen ve gelişmeyen hastaların medyan başlangıç yaşı, cinsiyet, hastalık başlangıç mevsimi arasında istatistiksel olarak anlamlı fark saptanmadı. Enfeksiyonun tetiklediği IgAV’nde daha az nüks geliştiği saptandı (p=0,048). Her iki grup arasında başvuruda bakılan; beyaz küre, hemoglobin, platelet, eritrosit sedimentasyon hızı, C-reaktif protein, immünglobulin A, anti nükleer antikor, kompleman 3 ve 4 düzeyleri arasında fark yoktu. Tanı anında gastrointestinal, renal, eklem ve testis tutulum oranları arasında fark saptanmadı. Kortikosteroid kullanımı ve tedavi süresi açısından 2 grup arasında anlamlı fark yoktu (p=0,512). Bu çalışmada IgAV’nin nüks oranı %5,3 olarak bulundu. Literatürde cilt tutulumuna renal tutulumun eşlik etmesinin ve 10 günden uzun süreli kullanılan kortikosteroid tedavisinin nüks için risk faktörü olduğu gösterilmiştir. Çalışmamızda nüks ile ilişkili herhangi bir risk faktörü saptanmamakla beraber semptomlar başlamadan önce enfeksiyon öyküsü olan hastalarda daha az nüks geliştiği gösterildi. IgAV tanılı hastalarda nüksleri öngörmemize katkıda bulunan diğer olası faktörleri belirlemek için ileriye yönelik takip edilen geniş hasta serilerine ihtiyaç vardır
ObjectiveThe present study aimed to compare the demographic, clinical, and laboratory characteristics of patients with pediatric Behçet disease (BD) with and without thrombosis to elucidate the factors that may contribute to the development of thrombosis.MethodsThis observational, descriptive, medical records review study included patients with BD (n = 85) who were diagnosed at age younger than 16 years at our clinic between 2010 and 2022. The demographic, clinical, and available laboratory data of patients with and without thrombosis were compared. The potential risk factors for the development of thrombosis were evaluated with multivariable logistic regression analysis.ResultsCentral venous sinus thrombosis was the most common type of thrombosis. Thrombosis was significantly more common in male patients (p = 0.002), and regression analysis revealed that being male was a risk factor for developing thrombosis. Genital ulcers were less common in patients with thromboses. Patients with thrombosis had higher erythrocyte sedimentation rates, C-reactive protein, leukocyte, and neutrophil counts, as well as antinuclear antibody positivity. In contrast, mean platelet volume and lymphocyte counts were significantly lower in patients with thrombosis. According to the logistic regression analysis, erythrocyte sedimentation rate value >17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1–1.8; p = 0.012).ConclusionsMale sex has been associated with an increased risk of thrombosis in children with BD. Inflammatory parameters may serve as predictive factors for thrombosis in pediatric BD.
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