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.

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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.

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Amaç: Çalışmamızda pediatrik sistemik lupus eritematozus (SLE) hastalarında, hastalık prezentasyon bulgularının, hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyona ulaşmadaki etkilerinin incelenmesi amaçlanmıştır. Gereç ve Yöntemler: Merkezimizde takipli olan pediatrik SLE hastalarının elektronik tıbbi kayıtları ve hasta dosyalarından demografik, klinik ve laboratuvar verileri kaydedildi. Hastalığın 2. yılındaki aktivite ölçümü SLE Hastalığı Aktivite İndeksi-2000 (SLEDAI-2K) skoru baz alınarak hesaplandı. Prezentasyon bulguları ile düşük hastalık aktivitesi ve remisyon arasında ilişki olup olmadığı istatiksel yöntemlerle incelendi.Sonuçlar: Bu çalışmaya merkezimizde pediatrik SLE tanısı almış ve en az 2 yıl düzenli takibe gelmiş 29 hastayı dahil ettik. Tanıdan sonraki 2. yılda hastalarda SLE aktivite ölçümüne göre 14 hastada (%48.2) HDAS, 15 hastada (%51,7) LDAS-remisyon elde edilmişti. İki grup arasında başlangıçtaki prezentasyon bulguları açısından anlamlı istatiksel farklılık saptanmadı. 5. yılda 15 hastadan 6’sında (%40) LDAS-remisyon, 9’unda (%60) HDAS mevcuttu. 2. yılda HDAS’a sahip hastaların 5. yıl SLEDAI-2K skorları, 2. yılda LDAS-remisyona sahip hastaların 5. yıl SLEDAI-2K skorlarına göre anlamlı olarak yüksekti (p= 0.028). Ayrıca 2. yılda aktif olan 8 hastanın (%80) 5. yılda HDAS’ın devam ettiği gözlendi.Tartışma: Çalışmamızda pediatrik SLE prezentasyon bulgularının hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyon üzerine belirleyici etkilerinin olmadığını gösterildi. Ayrıca 2. yılda elde edilen düşük hastalık aktivitesi ve remisyon 5. yıldaki düşük hastalık aktivitesi ve remisyonun belirleyicisi olabilir.

Aim: To identify the risk factors associated with intussusception in children with immunoglobulin A vasculitis (IgAV)-gastrointestinal (GI) tract involvement and to evaluate the outcomes of medical treatment and surgical intervention and the course of patients with intussusception. Methods: This retrospective study was conducted in 157 patients under 18 years of age who were followed up with the diagnosis of IgAV-GI tract involvement between January 2015 and September 2022. The characteristics of the patients who developed intussusception were evaluated in detail. Results: One hundred and fifty-seven patients with GI tract involvement were included in the study. The mean age of patients with IgAV-GI tract involvement was 8.7 AE 3.7 years. The female-to-male ratio was 1:1.5. Intussusception was detected in 14 patients (8.9%). Two patients (14.3%) underwent surgery, and the remaining 12 patients (85.7%) had their medical therapy intensified. Patients with GI tract involvement were divided into two groups as with (n = 14) and without (n = 143) intussusception. There was a statistically significant difference between the groups in the time from the onset of the first symptom of IgAV to the onset of steroids (P = 0.001). There were no statistically significant differences between the groups in age at onset of IgAV, gender distribution, erythrocyte sedimentation rate and C-reactive protein levels. Conclusions: The time from the onset of the first symptom of IgAV to the start of steroids is a risk factor for the development of intussusception in patients with IgAV-GI tract involvement. In these patients, medical treatment usually reduces intussusception without the need for surgical intervention.

Amaç: Bu çalışmada immünglobulin A vasküliti (IgAV) olan çocuklarda böbrek tutulumunun zamanını etkileyen faktörlerin ortaya konulması amaçlanmıştır. Yöntem: Ocak 2015- Haziran 2022 arasında IgAV tanısı ile en az 1 yıl takip edilen hastaların verileri geriye dönük olarak incelendi. Hastaların demografik, klinik, laboratuar verileri ve uygulanan tedaviler kaydedildi. Bulgular: Çalışmaya 81 IgAV böbrek tutulumu olan hasta dahil edildi. Hastaların 46’sı (%56,8) erkek, 35’i (%43,2) kızdı. Tüm hastalarda cilt, 32’sinde (%39,5) eklem, 44’ünde (%54,3) gastrointestinal sistem (GİS), 2’sinde (%2,5) nörolojik tutulum vardı. 3 hastada tanı anında HT vardı. Böbrek tutulumu, 45 hastada (%55,5) tanı anında, 10 hastada (%12,4) ilk 2 hafta içinde, 12 hastada (%14,8) 2-4. haftalar arasında, 14 hastada (%17,3) 1 ay-3 ay arasında saptandı. 3 aydan sonra böbrek tutulumu olmadı. Tanı anında böbrek tutulumu ile ileri yaş ve düşük albümin düzeyinin olması; 1-3 ayda böbrek tutulumu gelişmesi ile eklem ve GİS tutulumu arasında ilişki bulundu. Böbrek tutulumu olan hastalar içinde iyileşme süresi en uzun olanlar, tanı anında böbrek tutulumu saptananlar ve nefrotik düzeyde proteinüri olan hastalardı. İyileşme süresi en kısa olanlar ise, 1-3 ay arası böbrek tutulumu olan ve hematüri saptananlardı. Sonuç: Çocukluk çağında IgAV tanısı alan hastalarda yaşları büyük olanlar ve düşük albümin düzeyi olanların böbrek tutulumu açısından dikkatli takip edilmesi gereklidir. Özellikle ilk 3 ay böbrek tutulumunun saptanması için riskli dönemdir.

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.