Inflammatory Lung Disease in Rett Syndrome
المؤلفون المشاركون
Rossi, Marcello
Chisci, Glauco
Lonetti, Giuseppina
Vannuccini, Laura
Spina, Donatella
Ginori, Alessandro
Iacona, Ingrid
Hayek, Joussef
Pecorelli, Alessandra
Pizzorusso, Tommaso
Leoncini, Silvia
Cortelazzo, Alessio
Signorini, Cinzia
Ciccoli, Lucia
De Felice, Claudio
Giuseppe, Valacchi
المصدر
العدد
المجلد 2014، العدد 2014 (31 ديسمبر/كانون الأول 2014)، ص ص. 1-15، 15ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2014-03-17
دولة النشر
مصر
عدد الصفحات
15
التخصصات الرئيسية
الملخص EN
Rett syndrome (RTT) is a pervasive neurodevelopmental disorder mainly linked to mutations in the gene encoding the methyl-CpG-binding protein 2 (MeCP2).
Respiratory dysfunction, historically credited to brainstem immaturity, represents a major challenge in RTT.
Our aim was to characterize the relationships between pulmonary gas exchange abnormality (GEA), upper airway obstruction, and redox status in patients with typical RTT (n = 228) and to examine lung histology in a Mecp2-null mouse model of the disease.
GEA was detectable in ~80% (184/228) of patients versus ~18% of healthy controls, with “high” (39.8%) and “low” (34.8%) patterns dominating over “mixed” (19.6%) and “simple mismatch” (5.9%) types.
Increased plasma levels of non-protein-bound iron (NPBI), F2-isoprostanes (F2-IsoPs), intraerythrocyte NPBI (IE-NPBI), and reduced and oxidized glutathione (i.e., GSH and GSSG) were evidenced in RTT with consequently decreased GSH/GSSG ratios.
Apnea frequency/severity was positively correlated with IE-NPBI, F2-IsoPs, and GSSG and negatively with GSH/GSSG ratio.
A diffuse inflammatory infiltrate of the terminal bronchioles and alveoli was evidenced in half of the examined Mecp2-mutant mice, well fitting with the radiological findings previously observed in RTT patients.
Our findings indicate that GEA is a key feature of RTT and that terminal bronchioles are a likely major target of the disease.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
De Felice, Claudio& Rossi, Marcello& Leoncini, Silvia& Chisci, Glauco& Signorini, Cinzia& Lonetti, Giuseppina…[et al.]. 2014. Inflammatory Lung Disease in Rett Syndrome. Mediators of Inflammation،Vol. 2014, no. 2014, pp.1-15.
https://search.emarefa.net/detail/BIM-1043638
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
De Felice, Claudio…[et al.]. Inflammatory Lung Disease in Rett Syndrome. Mediators of Inflammation No. 2014 (2014), pp.1-15.
https://search.emarefa.net/detail/BIM-1043638
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
De Felice, Claudio& Rossi, Marcello& Leoncini, Silvia& Chisci, Glauco& Signorini, Cinzia& Lonetti, Giuseppina…[et al.]. Inflammatory Lung Disease in Rett Syndrome. Mediators of Inflammation. 2014. Vol. 2014, no. 2014, pp.1-15.
https://search.emarefa.net/detail/BIM-1043638
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1043638
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر