By Arata Azuma, Michael S. Schechter
This quantity describes the pathogenesis and pathophysiology of a number of pulmonary illnesses in addition to their therapy. It additionally discusses the underlying genetic and molecular organic foundation, which opens the way in which for brand new remedies for those conditions.
It specializes in the therapy of cystic fibrosis together with CFTR (cystic fibrosis transmembrane-conductance regulator) modulator remedies, drug cures that increase airway floor liquid in addition to anti inflammatory and anti-infective cures. extra subject matters comprise long term, low-dose macrolide treatment for diffuse panbronchiolitis; novel brokers for formerly untreatable idiopathic pulmonary fibrosis; attainable new remedies for pulmonary alveolar proteinosis (PAP); and a number of novel healing pursuits for treating lymphangiomyomatosis. learn into those stipulations has resulted in significant advances in our realizing of the underlying genetic and molecular foundation of this affliction, and to dramatic advancements in survival and caliber of existence for affected individuals.
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Additional info for Treatment of Cystic Fibrosis and Other Rare Lung Diseases
Am J Respir Crit Care Med 162:1277–1284 Pignatti PF, Bombieri C, Benetazzo M et al (1996) CFTR gene variant IVS8-5T in disseminated bronchiectasis. Am J Hum Genet 58:889–892 Rose MC, Voynow JA (2006) Respiratory tract mucin genes and mucin glycoproteins in health and disease. Physiol Rev 86:245–278 Rubin BK, Druce H, Ramirez OE et al (1997) Effect of clarithromycin on nasal mucus properties in healthy subjects and in patients with purulent rhinitis. Am J Respir Crit Care Med 155:2018–2023 Sato A, Chida K, Iwata M et al (1992) Study of bronchus-associated lymphoid tissue in patients with diffuse panbronchiolitis.
2011; Raghu et al. 2011). IPF is more frequent in men (approx. 2:1) compared to women (Raghu et al. 2006) and typically occurs in middle-aged and elderly adults (median age 66 years). It is very uncommon to see IPF under the age of 50 (Raghu et al. 2011; King et al. 2011). 2 Pathology The main pathological features of UIP are heterogeneous degrees of fibrosis throughout the lungs with subpleural and paraseptal fibrosis, excessive and disorganized deposition of ECM (mainly collagen), honeycomb cysts, and destruction of lung architecture (Raghu et al.
2011). CD151 is a tetraspanin expressed at the basolateral surface of AECs and is important to maintain epithelial integrity.
Treatment of Cystic Fibrosis and Other Rare Lung Diseases by Arata Azuma, Michael S. Schechter