Quality of life in patients with anorectal malformation or Hirschsprung's disease: development of a disease-specific questionnaire.

PURPOSE: Hirschsprung's disease and anorectal malformation are congenital diseases of the digestive tract with sequelae into adulthood. The quality of life of patients with these diseases is largely unknown. The aim of the study was 1) to construct a self-report disease-specific instrument to assess the quality of life in these patients and 2) to evaluate its psychometric performance. METHODS: An age-specific (6 and 7 years, 8-11 years, 12-16 years, and >17 years) questionnaire called the Hirschsprung's disease/anorectal malformation quality-of-life instrument was constructed. This questionnaire consists of 39 to 42 items, grouped into 10 to 11 scales that cover physical, emotional, and social functions as well as disease-related symptoms. Generic quality-of-life data were obtained in addition. A national sample of 715 patients aged six years and older completed the questionnaire (response rate, 61.9 percent). RESULTS: Multitrait scaling analyses confirmed the hypothesized scale structure with exception of the scales related to diet for the two youngest groups. Cronbach's alpha ranged (with exception of the diet scales) from 0.62 to 0.91 for children (8-11 years), from 0.69 to 0.82 for adolescents (12-16 years) and from 0.57 to 0.91 for adults. Selective scales were able to discriminate between subgroups of adult patients known to differ in disease and disease severity. Relevant scales of the adult version showed substantial correlations (> 0.40) with comparable scales of the SF-36. In the two youngest age groups the differences between subgroups of patients were less significant, but in the expected direction. CONCLUSIONS: With the exception of the scales related to diet, the Hirschsprung's disease/anorectal malformation quality-of-life instrument is an instrument with promising reliability and validity, to measure the disease-specific quality of life of patients with anorectal malformation or Hirschsprung's disease.

The geographical distribution of tick bites and erythema migrans in general practice in The Netherlands.

BACKGROUND: Lyme disease is caused by Borrelia burgdorferi which is transmitted in Europe by the tick ixodes ricinus. Erythema migrans is a skin lesion which is pathognomonic of Lyme disease. A retrospective study was carried out to determine the geographical distribution of the occurrence of tick bites and erythema migrans in the Netherlands and to identify ecological risk factors. METHODS: In April 1995, all general practitioners (GPs) in the Netherlands were asked to complete a postal questionnaire on the number of tick bites and erythema migrans case-patients seen in 1994 and the size of the practice. Reminders were sent to non-responders. Information on ecological risk factors by local government area was obtained from a geographical information system. RESULTS: The response rate was 79.9%. In 1994, GPs reported seeing approximately 33,000 patients with tick bites and 6500 with erythema migrans. The incidence rate of erythema migrans was estimated at 4.3 per 10,000 population. Ecological risk factors for both tick bites and erythema migrans were the proportion of the area covered by woods, sandy soil, dry uncultivated land, the number of tourist-nights per inhabitant and sheep population density. The cattle population density was a risk factor for erythema migrans. CONCLUSIONS: Using simple methods, a crude estimate of the incidence rate of erythema migrans was obtained rapidly, and high risk areas were identified. Lyme disease appears to be an important problem in the Netherlands.