Bronchial cartilage deficiency leading to bronchom Essays - Bacteria

Bronchial cartilage deficiency leading to bronchom Essays - Bacteria bronchial cartilage deficiency leading to bronchomalacia. Mounier - Kuhn syndrome: characterised by congenital deficiency of bronchial cartilage associated ~th tracheobronchomegaly. Enlargement of the airways and deep corrugations produced by the redundant musculo -membranous tissues be~een the cartilaginous rings gives a roentgenographic appearance of multiple diverticulae on CT(lO). Brock's syndrome: characterized by right middle 10 be collapse ~th bronchiectasis caused by a foreign body or enlarged lymph nodeusually tuberculous. Chandra- Khetarpal syndrome: characterized by levocardia, bronchiectasis and frontal sinusitis. Ciliary dysfunction is not a feature of this syndrome (3). MacLeod's (Swyer-James)syndrome: in which the disease is associated ~th a unilateral hyperluscent lung and characterized by brochiolitis obliterans (4). Yellow nail syndrome: associated ~th lymphedema and pleural effusion due to hypoplastic lymphatics. Whooping cough, measles, mumps, influenza, and primary complex are considered to be childhood infections that predispose to bronchiectasis in adulthood(childhood pentad of bronchiectasis). Clinical Features Persons ~th bronchiectasis bring out copious foul smelling khakicoloured(S) sputum containing abundance of neutrophils and have postural variation in sputum production. The foul smell is due to the presence of . anaerobic organisms in the sputum. Excessive ~ounts of protein are lost in the sputum causing hypo albuminaemia. This condition is called protein- losing pulmonopathy. Halitosis may occur. Recurrent pleurisy in the same site may also be a feature (6) . The organisms commonly cultured from the sputum in bronchiectasis are Pseudomonas species and the following. Anaero bic organisms - Bacteroides fragilis etc. and Aspergillus fumigatus Klebsiella pneu!7Wniae Haemophilus influenzae Streptococcus pneumoniae Staphylococcus aureus . If mucoid strains of Pseudomonas aeruginosa or Pseudo~nas cepaciae, Escherichia coli, H. influenzae or S. aureus are cultured the patient should be investigated for cystic fibrosis, an autosomal recessive disorder involving mutations in the CFTR gene on chromosome 7, which can be confirmed by estimating the chloride content in sweat (sweat chloride test). In cystic fibrosis the chloride in sweat will b~ above 60 meq/ L in children and above 80 meq/ L in adults. The sweat is 0 btained by pilocarpine iontophoresis. A test for mucociliary clearance is by nasal saccharin clearance. A 1 mm cube is placed on the inferior turbinate and the time taken for it to be tasted is normally less than 30 minutes(S). It is prolonged in impaired ciliary function. 24