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Three cases of unilateral right-sided pulmonary venous atresia were evaluated over an 18-year period. These bring the total number of cases to 25 in the literature. The clinical presentation of all these patients was similar and consisted of recurrent pulmonary infections, asthma-like symptoms, and exercise intolerance. The patients presented in 1982 (patient 1, a 12-year-old boy), 1994 (patient 2, a 9-year-old girl), and 1999 (patient 3, a 13-year-old boy). All patients were evaluated with a chest roentgenogram, and patients 1 and 2 had a ventilation and perfusion scan. Patients 1 and 3 also had cardiac catheterization and pulmonary angiography. Patient 2 had a magnetic resonance imaging study of the chest. Only patient 3 had wedge pulmonary angiography. Although a rare congenital defect, this diagnosis should be strongly suspected based on the typical clinical presentation and the preliminary studies, such as the chest roentgenogram and ventilation and perfusion scan. However, for definitive diagnosis, cardiac catheterization with wedge pulmonary angiography is necessary. Anastomosis of the atretic pulmonary veins to the left atrium is a theoretical consideration. However, this may not be feasible due to pulmonary venous anatomy or significant pulmonary dysfunction with pulmonary vascular changes. In these circumstances, we recommend performing pneumonectomy to remove the nidus for repeated bouts of pulmonary infections, to eliminate the left-to-right shunt, and to eliminate the dead space contributing to exercise intolerance.

Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.

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We describe the development and psychometric properties of a survey tool designed to evaluate children's level of independence in their cystic fibrosis (CF) treatment. Children's self-care autonomy is important to measure and may have a direct effect on children's active involvement in their treatment and their adherence. Existing instruments that evaluate children's independence in their CF treatment fail to yield practical information that can help research and patient management. The Self-Care Independence Scale (SCIS) is a 44-item questionnaire that is completed by parents. The SCIS was completed by the parents of 76 patients with CF (ages 4-17 years). Youths completed two structured interviews, which screened patients for average or higher cognitive functioning (an inclusion criterion) and measured their CF treatment knowledge. Parents completed two other self-report questionnaires. Results indicated that the SCIS has acceptable internal consistency and good test-retest reliability. The construct validity of the SCIS was supported by positive correlations between patient age, number of years since diagnosis, and SCIS total scores. The concurrent validity of the SCIS was supported by correlations between a measure of nonillness-specific dependence and a measure of patient self-care knowledge. Children's SCIS total scores per age group are presented. This study supports the SCIS as a psychometrically sound measure of self-care independence in CF. This measure has several uses. For example, the SCIS may be a screening tool for adolescents who are preparing to transition to adult CF centers and who will need to assume more independence in their self-care. The SCIS may also be used to evaluate educational programs that promote self-care knowledge and skill in children with CF.