Etiology, diagnosis, and treatment in childhood atelectasis

Introduction: Atelectasis is the loss of lung volume secondary to collapse. Narrow and collapsible airways and underlying chronic diseases facilitate the development of atelectasis in children. Since atelectasis is an important cause of morbidity and mortality in children, early diagnosis and treatment are of great importance. Methods: Thirty-six patients who were followed up in the pediatric service and pediatric intensive care unit of our clinic be - tween December 1, 2018, and June 1, 2019, and were diagnosed radiologically with atelectasis were evaluated retrospectively. Results: The median age was 1.85 years (1.0–7.37). The most common cause for hospitalization was pneumonia (n=30, 83%). Except for two patients, all patients had an underlying disease that increased the risk of atelectasis. Neurological diseases were the most common diseases among the underlying diseases (n=12, 36%). For the treatment, 4 (11.1%) patients received chest physiotherapy, 19 (52.7%) patients received nebulized medications, and chest physiotherapy, and 13 (36.1%) patients received positive end-expiratory pressure support in addition to these treatments. The frequency of atelectasis in more than one localization was higher in children with the neurological disease than in other patients (n=7, [54%] vs. n=3, [13%]; p=0.018). In patients with atelectasis in more than one localization, the duration of hospitalization was longer (median 12.5 days [9.5–16.75] vs. 19 days [13–22.75]; p=0.034). Discussion and Conclusion: Atelectasis is common in hospitalized children with an underlying disease. In the presence of pathological respiratory symptoms and signs, atelectasis should be kept in mind, and treatment should be started early

Blau Syndrome and Early-Onset Sarcoidosis: A Six Case Series and Review of the Literature.

Objectives: This study aims to discuss the clinical, laboratory and genetic findings, and treatment options for six patients who were diagnosed with Blau syndrome (BS)/early-onset sarcoidosis (EOS). Patients and methods: The study included four patients (2 males,2 females; mean age 7 years; range 4 to 10 years) with EOS and two siblings (1 male, 1 female; mean age 10 years; range, 9 to 11 years) with BS. Age, age of initial symptoms, age of diagnosis; articular involvement, presence of uveitis, dermatitis, or fever, other organ involvement, laboratory findings, results of metabolic tests for mucopolysaccharidosis and mucolipidosis, results of genetic, pathologic, and immunologic tests, radiologic findings to evaluate skeletal dysplasia, and treatment options were collected. Results: The median age at diagnosis of all patients was 6 years (range, 1 to 10 years). Five patients had camptodactyly and bilateral boggy synovitis in the wrists and ankles, one had granulomatous inflammatory changes in the liver and kidney biopsy, and one had attacks of fever and granulomatous dermatitis. None had uveitis. The detected mutations in nucleotide-binding oligomerization domain containing 2 (NOD2) were P268S (rs2066842), M513T (rs104895473), R702W (rs2066844), V955I (rs5743291), H343Y (rs199858111), and M491L (16:50745293). The treatments of patients included corticosteroids, non-steroid anti-inflammatory drugs, methotrexate, infliximab, adalimumab, anakinra, and canacinumab. Conclusion: Camptodactyly and boggy synovitis are important signs of BS/EOS. Methotrexate and tumor necrosis factor blockers are more effective in patients with predominantly articular symptoms. In patients 5 and 6 and their mother, we determined a novel M491L mutation in the NOD2 gene. Currently, this work is in progress towards identifying the pathogenesis and treatment options for this disease

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