Terry Chin

By Terry W. Chin

Since the basic defect in cystic fibrosis (CF) involves a defective cell surface protein controlling chloride channel transport across cell membranes, medications which are developed to enhance the cystic fibrosis transmembrane conductance regulator (CFTR) protein should result in improvement in patients with CF. The presence of over 2000 genetic mutations have made these efforts difficult. However, a classification scheme of these mutations has allowed three basic approaches: to bypass missense mutations by having the cellular translation machinery read through the premature stop codon, to enhance the “gating” function of the CFTR protein on the cell surface, and to correct a defective CFTR protein “trafficking” though the cytoplasm to be inserted properly in the cell membrane. This chapter will review clinical trials using drugs which are designed to enhance CFTR protein activity.

Part of the book: Cystic Fibrosis in the Light of New Research

By Terry Chin

There will be a discussion of the manifestation of acute bronchitis in children and note differences with that seen in the adult population. In particular, the need for identifying the specific cause of coughing such as inhalation of a foreign body or diagnosing the newly recognized protracted bacterial bronchitis (PBB) in children is emphasized. Understanding the differing pathophysiology of afferent hypersensitivity and inflammatory infiltrates in the bronchial epithelium enables for different therapeutic approaches. Therefore, the chapter concludes with a discussion on the role for anti-inflammatory and antimicrobial therapies in children, as well as possible intervention to the neuronal hypersensitivity. Anti-tussive and mucolytic modes of treatment are also reviewed.

Part of the book: Bronchitis in Children