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Respiratory Medicine
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Colin Wallis, Helen Spencer, Sam Sonnappa
Childhood interstitial lung disease (ILD) is a spectrum of a heterogeneous group of rare lung diseases resulting from varied pathogenic processes that include genetic factors, associated systemic diseases and pathological inflammatory responses to stimuli.
Diagnosing and managing bronchiolitis obliterans in children
Published in Expert Review of Respiratory Medicine, 2019
The treatment of PIBO is empirical. Inhaled and systemic corticosteroids are used to combat the inflammatory component although their effect upon clinical outcomes is unclear. If used, corticosteroids should be given early while the disease process is in the developing phase before airway fibrosis is complete [24]. The most common approach is pulse steroid therapy with intravenous methylprednisolone 10–30 mg/kg for 3 consecutive days and repeated monthly for 3–6 months as used for childhood interstitial lung disease. A study from University of São Paulo [64] retrospectively examined 40 patients with a diagnosis of PIBO who were treated with methylprednisolone pulse therapy in monthly cycles (at least six) 30mg/kg per day for three days. All received inhaled corticosteroids in addition for the follow-up period (for an average of 51.6 months). The frequency of wheezing exacerbations after 24 months of pulse therapy and frequency of hospitalizations after 18 months of pulse therapy were significantly reduced and oxygen saturations improved. New prospective controlled studies are required to confirm the effectiveness of pulsed methylprednisolone courses for the treatment of PIBO.
Advances in synthetic lung surfactant protein technology
Published in Expert Review of Respiratory Medicine, 2019
Frans J. Walther, Larry M. Gordon, Alan J. Waring
Both SP-B and SP-C have been sequenced and genetic studies have shown that hereditary SP-B deficiency causes fatal neonatal RDS [2] and that SP-C mutations can lead to childhood interstitial lung disease [3]. Discovery of the structures of SP-B and SP-C 30 years ago and advances in peptide synthesis promised a quick development of functional peptide mimics and synthetic lung surfactant for clinical use. However, the complex 3-D structure of native SP-B and the propensity of SP-C for amyloid formation of its transmembrane region have been quite a challenge in designing and producing highly functional, stable peptide mimics of SP-B and SP-C.