The answer is b Fauci 14e p 1446 Kartagener syndrome is the

inheritable disorder of dextrocardia, chronic sinusitis (with the formation of nasal polyps), and bronchiectasis. Patients may also present with situs inversus. The disorder is due to a defect that causes the cilia within the respiratory tract epithelium to become immotile. Cilia of the sperm are also affected.

115. The answer is d. (Fauci, 14/e, pp 1466-1468.) Primary pulmonary hypertension (PPH) is of unknown etiology and primarily affects women in their thirties or forties. The underlying problem in the disorder is a fixed increased resistance to pulmonary blood flow. Pulmonary function in PPH is usually normal, but the elevation in pulmonary artery pressure causes a decrease in cardiac output and eventually right ventricular failure. Patients become dyspneic and hypoxemic due to the mismatch of pulmonary ventilation and perfusion and the reduced cardiac output. Physical examination reveals signs of right ventricular hypertrophy, right- and left-sided heart failure, and tricuspid and pulmonic regurgitation. The mean survival for this disease is 2-3 years from the time of diagnosis.

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