The following syndrome is associated with bronchiectasis –
Correct Answer: Kaagener's syndrome
Description: Ref:Textbook of pathology (Harsh mohan) 6th edition,page no. 484 ETIOPATHOGENESIS of BRONCHIECTASIS: . The origin of inflammatory destructive process of bronchial walls is nearly always a result of two basic mechanisms: endobronchial obstruction and infection. Endobronchial obstruction by foreign body, neoplastic growth or enlarged lymph nodes causes resorption of air distal to the obstruction with consequent atelectasis and retention of ecretions. Infection may be secondary to local obstruction and impaired systemic defense mechanism promoting bacterial growth, or infection may be a primary event i.e. bronchiectasis developing in suppurative necrotising pneumonia. These 2 mechanisms--endobronchial obstruction and infection, are seen in a number of clinical settings as under: 1. Hereditary and congenital factors. Several hereditary and congenital factors may result secondarily in diffuse bronchiectasis: i) Congenital bronchiectasis caused by developmental defect of the bronchial system. ii) Cystic fibrosis, a generalised defect of exocrine gland secretions, results in obstruction, infection and bronchiectasis iii) Hereditary immune deficiency diseases are often associated with high incidence of bronchiectasis. iv) Immotile cilia syndrome that includes Kaagener's syndrome (bronchiectasis, situs inversus and sinusitis) is characterised by ultrastructural changes in the microtubules causing immotility of cilia of the respiratory tract epithelium, sperms and other cells. Males in this syndrome are often infeile . v) Atopic bronchial asthma patients have often positive family history of allergic diseases and may rarely develop diffuse bronchiectasis. 2. Obstruction. Post-obstructive bronchiectasis, unlike the congenital-hereditary forms, is of the localised variety, usually confined to one pa of the bronchial system. The causes of endobronchial obstruction include foreign bodies, endobronchial tumours, compression by enlarged hilar lymph nodes and post-inflammatory scarring (e.g. in healed tuberculosis) all of which our the development of postobstructive bronchiectasis. 3. As secondary complication Necrotising pneumonias such as in staphylococcal suppurative pneumonia and tuberculosis may develop bronchiectasis as a complication.
Category:
Pathology
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