Cystic fibrosis mucolytics

Mucous airway obstruction. Mu-colytics, such as acetylcysteine, split disulfide bonds in mucus, hence reduce its viscosity and promote clearing of bronchial mucus. Other expectorants (e.g., hot beverages, potassium iodide, and ipecac) stimulate production of watery mucus. Acetylcysteine is indicated in cystic fibrosis patients and inhaled as an aerosol. Whether mucolytics are indicated in the common cold and whether expectorants like bromohexine or am-broxole effectively lower viscosity of bronchial secretions may be questioned. 

Mucolytic Adjuvant therapy for abnormal, viscid, or inspissated mucus secretions in chronic bronchopulmonary disease (chronic emphysema, emphysema with bronchitis, chronic asthmatic bronchitis, tuberculosis, bronchiectasis, primary amyloidosis of lung) acute bronchopulmonary disease (pneumonia, bronchitis, tracheobronchitis) pulmonary complications of cystic fibrosis tracheostomy care pulmonary complications associated with surgery use during anesthesia posttraumatic chest conditions atelectasis due to mucus obstruction diagnostic bronchial studies (bronchograms, bronchospirometry, bronchial wedge catheterization). 

F. Role in therapy Pulmozyme is a mucolytic enzyme used in the treatment of cystic hbrosis. Although it is not a cure, domase alfa is an effective mucolytic for adjunctive treatment. AU compliant patients with cystic fibrosis, irrespective of disease severity, who produce purulent sputum are potential candidates for Pulmozyme therapy. It is useful for liquefying the thick mucus secreted by cystic fibrosis patients, and causes both objective improvement (as measured by pulmonary function testing) and subjective symptomatic improvement. Pulmozyme reduces the frequency of respiratory infections requiring parenteral antibiotics and improves pulmonary function. 

Another condition where cell debris becomes life threatening is the obstruction of airways by viscous sputum in patients suffering from cystic fibrosis (CF). It has been shown that human plasma gelsolin can rapidly decreased the viscosity of CF sputum samples in vitro (Vasconcellos et al., 1994 Sheils et al., 1996 Davoodian et al., 1997 Ismailov et al., 1997), suggesting that in CF patients gelsolin may have therapeutic potential as a mucolytic agent. 

Mucolytic Agents. Acetylcysteine, which is frequently used as a bronchial mucolytic agent in patients with cystic fibrosis, can be used topically in a weakened concentration for ophthalmic use. It is malodorous and may sting on instillation however, this drug is fairly effective in disrupting mucous strands that are often present in patients with aqueous deficiency dry eye. It is not commercially available in an ophthalmic formulation it must be compounded by a pharmacist. 

Carhocisteine and mecystdne have free sulphydryl groups that open disulphide bonds in mucus and reduce its viscosity. They are given by orally or by inhalation (or instillation) and may be useful chiefly where particularly viscous secretion is a problem (cystic fibrosis, care of tracheostomies). Mucolytics may cause gastrointestinal irritation and allergic reaction. 

Parvolex ) is used a MUCOLYTIC agent, which reduces the viscosity of sputum, so can be used as an expectorant in patients with disorders of the upper respiratory airways, such as chronic asthma and bronchitis. It is also used orally to treat abdominal complications associated with cystic fibrosis, and locally in the eye to increase lacrimation and mucus secretion. It is also used intravenously as an antidote in paracetamol poisoning. 

Respiratory symptoms are obstruction of bronchioles with viscous mucus and recurrent infection. Respiratory infections require intensive antibiotic therapy and together with lung damage are the commonest causes of death in people with cystic fibrosis. Viscous secretion is treated with vigorous physiotherapy and the use of mucolytics. 

Acetylcysteine decreases thickness of mucous secretions in lung. As a mucolytic agent, acetylcysteine splits disulfide linkages between mucoprotein molecular complexes, decreasing their viscosity. It is used as an adjunct therapy in emphysema with bronchitis, chronic asthmatic bronchitis, mberculosis, bronchiectasis, primary amyloidosis of the lung, pneumonia, and tracheobronchitis. In addition, it is used in pulmonary complications of cystic fibrosis and those associated with anesthetics, surgery, or care following tracheostomy. 

Domase alpha, a recombinant human deoxyribonuclease I, a mucolytic enzyme with respiratory stimulant properties, is nsed to improve pulmonary function and reduce the frequency of moderate to severe respiratory infections in patients with cystic fibrosis. 

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