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• Failure of the right side of the heart with cardiac dilation and fluid retention occurred in 55 of 170 patients who died of cystic fibrosis; six patients survive. All had severe hypoxia, but 24% had normal Paco2. Cardiac catheterization showed high mean pulmonary artery pressure and resistance. Pulmonary artery wedge pressure was greater than 12 mm Hg in 40% of the patients. Mean survival was eight months. Male survival was significantly better than female survival. Digitalis treatment was of no clear benefit. Tolazoline hydrochloride was also ineffective. Recent medical advances have not substantially affected prognosis.(Am J Dis Child 134:267-272, 1980)

• Nasal polyposis complicated the course of cystic fibrosis in 157 (26%) of 605 patients. Onset before age 5 years or after age 20 years was rare. Polyposis was the initial symptom of cystic fibrosis in 13 patients. Common symptoms included obstruction to nasal air flow, mouth breathing, epistaxis, and rhinorrhea. Intranasal and oral corticosteroids and antihistamines were ineffective in preventing recurrences but did occasionally afford symptomatic relief of obstruction. Nineteen (31%) of 62 patients who never had surgery had spontaneous and permanent disappearance of polyps. Simple polypectomy was an adequate procedure for patients with substantial nasal symptoms. There were no visual complications. Other surgical complications were rare. Children and adolescents with nasal polyps should have sweat tests by pilocarpine iontophoresis to rule out cystic fibrosis.(Am J Dis Child 1982;136:1067-1070)

• Data on 16 sibling pairs with cystic fibrosis were analyzed to test the hypothesis that early treatment of this condition improves prognosis. Younger siblings' conditions were diagnosed before 1 year of age, usually before the onset of pulmonary disease. Older siblings' conditions were diagnosed after 1 year of age and after the onset of pulmonary disease. Although the sibling pairs received similar treatment, comparison at 7 years of age showed that the younger siblings had significantly better chest roentgenogram scores, total clinical scores, residual lung volumes, and ratios of residual volume to total lung volume. Younger siblings also required fewer hospital admissions to control their lung disease. The results suggest that, in general, early initiation of therapy is beneficial for patients with cystic fibrosis.(Am J Dis Child 131:973-975, 1977)