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Objectives This descriptive study aimed to compare the clinical and laboratory features of the children with the multisystem inflammatory syndrome in children (MIS-C), requiring pediatric intensive care unit (PICU), admission with the MIS-C patients who did not require PICU admission. Patients and methods This study was conducted between March 2020 and February 2021 at the University of Health Sciences Dr. Behçet Uz Children’s Hospital, a referral center for pediatric infectious diseases in the Aegean Region of Turkey. All hospitalized patients aged 18 years old or less with MIS-C according to the definition of the universal guidelines were included in the study. Data of the patients with the diagnosis of MIS-C were recorded and collected from the electronic medical records of the hospital. The data included demographic characteristics, presenting signs and symptoms, laboratory findings and clinical data. Results A total of 58 patients with MIS-C were included in this study. Thirty-eight (65.5%) patients were male. The median age was 6 years (2 months–16 years). The patients admitted to PICU were 15 (25.9%). The rate of pulmonary involvement was 81.3% (n = 13) in the PICU group. The median procalcitonin, C-reactive protein, erythrocyte sedimentation rate, D-Dimer and ferritin values were significantly higher in the PICU group compared to non-PICU group (p < 0.001, p = 0.02, p < 0.001, p = 0.006 and p = 0.031). Conclusions Besides the depressing cardiac functions reported before, the pulmonary involvement and signs of shock are important factors for PICU admission in children with MIS-C.

WOS: 000463722800005, PubMed ID: 31365342, Objective: Familial Mediterranean fever (FMF) is the most common inherited monogenic autoinflammatory disease worldwide. It is caused by loss-of-function mutations in the MEFV gene, mostly affecting Eastern Mediterranean population. It is discussed if it should be considered an autosomal-dominant disease with variable penetrance, because heterozygosis mutations are associated with clinical autoinflammatory manifestations. Colchicine constitutes that the mainstay of FMF treatment should be preventing acute attacks and amyloidosis, and decreasing the chronic inflammation. In colchicine-resistant or intolerant patients, recent insights into the pathogenesis of FMF have made the anti-IL1 treatments important. We aimed to search for the retrospective results of canakinumab treatment in patients with FMF who are unresponsive to colchicine. Methods: In this study, 22 (13 males and nine females) patients with FMF with colchicine resistance/intolerance, age ranging from 6 to 18 years, were included in Ege University Department of Pediatric Rheumatology. After clinical and genetic diagnosis, colchicine treatment with standard doses was started. After treatment with canakinumab, complete response to treatment was determined as no acute episodes and normal level of acute phase reactants. Results: After canakinumab treatment, 22 patients with FMF who were colchicine-resistant were evaluated. After the treatment, no attack was observed in 19 patients, and the values of acute phase reactants were normal in 22 patients. In three patients, disease attack was observed 16 months after the first dose treatment. In all patients, the values of acute phase reactants were found at normal level during treatment. No drug-related side effects were observed in any patient. Conclusion: Canakinumab is an effective and safe anti-IL1 agent to reduce attacks in patients with FMF with no response to colchicine and to reduce the level of high-level laboratory findings associated with FMF.

Bronkojenik kistler embriyonik dönemde anormal tomurcuklanma sonucu gelişen iyi huylu lezyonlardır. Sıklıkla karinanın arka bölgesinde yerleşirler ve mediastinal kitlelerin yaklaşık %50’sini oluştururlar. Asemptomatik olabilecekleri gibi çocukluk çağında solunum yollarına bası yaparak bulgu verebilirler. Semptomların süresi ve sıklığı kronik akciğer hastalığı gelişimi açısından risk oluşturmaktadır. Bu yazıda tekrarlayan hışıltı nedeniyle başvuran ve bronkojenik kist saptanan bir olgu sunulmuştur., Bronchogenic cysts are benign congenital anomalies caused by aberrant development of the foregut in the embryonic period. These anomalies are located in the posterior part of the carina and constitute about 50% of all mediastinal lesions. In childhood, bronchogenic cysts may be asymptomatic or cause symptoms due to pressure on the respiratory tract. The duration and severity of the symptoms are main determinants for the developments of chronic lung diseases. In this paper, we present an infant case with bronchogenic cyst admitted to our clinic with recurrent wheezing.

Bronkojenik kistler embriyonik dönemde anormal tomurcuklanma sonucu gelişen iyi huylu lezyonlardır. Sıklıkla karinanın arka bölgesinde yerleşirler ve mediastinal kitlelerin yaklaşık %50’sini oluştururlar. Asemptomatik olabilecekleri gibi çocukluk çağında solunum yollarına bası yaparak bulgu verebilirler. Semptomların süresi ve sıklığı kronik akciğer hastalığı gelişimi açısından risk oluşturmaktadır. Bu yazıda tekrarlayan hışıltı nedeniyle başvuran ve bronkojenik kist saptanan bir olgu sunulmuştur. Pam Tıp Derg 2013;6(3):168-173, Bronchogenic cysts are benign congenital anomalies caused by aberrant development of the foregut in the embryonic period. These anomalies are located in the posterior part of the carina and constitute about 50% of all mediastinal lesions. In childhood, bronchogenic cysts may be asymptomatic or cause symptoms due to pressure on the respiratory tract. The duration and severity of the symptoms are main determinants for the developments of chronic lung diseases. In this paper, we presented an infant case with bronchogenic cyst admitted to our clinic with recurrent wheezing.