There is wide agreement on the benefits of NBS for CF in terms of lowered disease severity, decreased burden of care, and reduced costs. Risks are mainly associated with disclosure of carrier status and diagnostic uncertainty. When starting a NBS programme for CF it is important to take precautions in order to minimise avoidable risks and maximise benefits. In Europe more than 25 screening programmes have been developed, with quite marked variation in protocol design. However, given the wide geographic, ethnic, and economic variations, complete harmonisation of protocols is not appropriate. There is little evidence to support the use of IRT alone as a second tier, without involving DNA mutation analysis. However, if IRT/DNA testing does not lead to the desired specificity/sensitivity ratio in a population, a screening programme based on IRT/IRT may be used. Sweat chloride concentration remains the gold standard for discriminating between NBS false and true positives, but age-related changes in sweat chloride should be taken into account. CF phenotypes associated with less severe disease often have intermediate or normal sweat chloride concentrations. Programmes should include arrangements for counselling and management of infants where the diagnosis is not clear-cut. All newborns identified by NBS should be managed according to internationally accepted guidelines. CF centre care and the availability of necessary medication are essential prerequisites before the introduction of NBS programmes. Clear explanation to families of the process of screening and of implications of normal and abnormal results is central to the success of CF NBS programmes. Effective communication is especially important when parents are told that their child is affected or is a carrier. When establishing a NBS programme for CF, attention should be given to ensuring timely and appropriate processing of results, to minimise potential stress for families.

We compared the sensitivity of different types of visual analog scales and of descriptive pain terms in healthy volunteers and in postoperative patients. One hundred and seven volunteers marked visual analog scales according to their perception of the descriptive pain terms--little, mild, some, moderate, severe, agonizing. Individual variation in values and preferences between the five following five different visual analog scales were analyzed: 10-cm linear horizontal and vertical scales, a curvilinear scale, and graded horizontal and curvilinear scales. Significantly more volunteers preferred the horizontal scale with gradations. Scores for the vertical linear scale had the greatest coefficient of variation and were least normally distributed. The majority of volunteers considered the phrase "agonizing pain" the best term defining the extreme limit of pain (X2(12) = 41.8, P less than 0.001). There were significant changes in the values of pain intensity measured on visual analog scales by patients using the same descriptive pain term on successive observations. However, the patients' values for pain terms in the preoperative pain-free state were not significantly different from those during postoperative pain. We conclude that graded linear horizontal scales are both more reliable and preferred by participants and that visual analog scales give a more sensitive and accurate representation of pain intensity than do descriptive pain scales.

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Newborn screening was associated with improved growth, reduced morbidity, and reduced therapy, yet generated equivalent pulmonary outcome compared with late clinical diagnosis, suggesting that newborn screening may slow cystic fibrosis lung disease progression.