Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis

BACKGROUND: The Cystic Fibrosis Questionnaire (CFQ) is a disease-specific instrument that measures health-related quality of life (HRQOL) for adolescents and adults with cystic fibrosis (CF) > or = 14 years, consisting of 44 items on 12 generic and disease-specific scales. Versions of the CFQ are also available for children with CF and their parents. This study evaluated the psychometric properties of the CFQ in a national study at 18 CF centers in the United States. PARTICIPANTS: The CFQ-teen/adult was administered to 212 patients with CF ranging in age from 14 to 53 years. Test-retest reliability was assessed in a subset of patients over a 10- to 14-day interval. RESULTS: Multitrait analysis indicated a majority of items (95%) correlated more highly with their intended scale than a competing scale, supporting the conceptual model. Internal consistency coefficients indicated the CFQ scales had good reliability (Cronbach alpha = 0.67 to 0.94), and test-retest stability was acceptable (rs = 0.45 to 0.90). Validity was demonstrated by examining relationships between the CFQ, age, pulmonary function, and body mass index. As expected, the CFQ was inversely correlated with age, with older adults reporting lower CFQ scores than younger adults, better nutritional status was positively correlated with several weight-related scales, and the measure differentiated between individuals with varying levels of disease severity. Strong associations were also found between the CFQ and similar scales on the Short Form-36 Health Questionnaire, a well-known generic HRQOL measure. CONCLUSIONS: The results demonstrated that the CFQ-teen/adult is a reliable and valid measure of HRQOL for individuals with CF. It may be utilized in clinical trials to assess the effects of new therapies, to document the progression of disease, and to inform clinical practice.     

A comparison of bone loss from different skeletal sites during acute calcium deficiency in mice

Severe Ca deficiency was produced by the combined stresses of a Ca-deficient diet and lactation in female mice. Alveolar bone loss was assessed by changes in alveolar crest height and in quantity of supporting trabecular bone. Changes in the femur were measured by changes in mineral content and in the cross-sectional area of the midshaft region, and in vertebrae by changes in mineral content. A similar decrease in bone was observed in all sites during progression of Ca deficiency with a similar increase during recovery. Maximum bone loss amounted to more than 50 per cent. Alveolar bone loss was characterized by a reduction in trabecular bone without loss of alveolar crest height.     

Thymoma in pregnancy

The natural history of slow, localized progression usually seen with thymomas seems to be transformed by pregnancy into one of rapid growth and distant metastatic spread. Five of the six previously reported cases had died of the malignancy within 6 months postpartum. The one previous long-term survivor died of treatment-induced complications. Two important questions remain unresolved: What is the risk of recurrence in the patient who has no residual disease at the time of conception? Approximately 50 per cent of female patients can anticipate having complete resection of their thymoma, and their recurrence risk is about 2 per cent. Potentially, there are many patients who have conceived and delivered after surgical therapy. Documentation of these case histories is needed for an accurate prediction of the true risk. Two of the cases within the present review fall within this category. The clinical course in these two patients is consistent with the natural history of the disease. One exhibited local recurrence and progression. The other patient died from sepsis, probably related to immunosuppression. Whether there is a cause and effect relationship between pregnancy and their recurrences is unknown. What is the risk to the patient who is first diagnosed while pregnant? The only survivor in this category was presented by Goldman. This patient underwent immediate therapeutic abortion following diagnosis. She had radiation therapy for invasive unresectable disease and subsequently delivered two term pregnancies, but died of postcesarean cardiac arrest. She had no evidence of recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)