Rare disease surveillance: An international perspective.

BACKGROUND: The International Network of Paediatric Surveillance Units (INoPSU) was established in 1998 and met formally for the first time in Ottawa, Ontario in June 2000. OBJECTIVES: To document the methodology and activities of existing national paediatric surveillance units; the formation of INoPSU; the diseases studied by INoPSU members; and the impact of such studies on education, public health and paediatric practice. METHODS: Directors of paediatric surveillance units in Australia, Britain, Canada, Germany, the Netherlands, Latvia, Malaysia, Papua New Guinea, New Zealand and Switzerland were asked to provide information on each unit's affiliations, funding and staffing; the method of case ascertainment, the mailing list and response rates; and diseases studied. Original articles that reported data derived from units were identified by a search of an electronic database (MEDLINE), and additional information was obtained from units' annual reports. RESULTS: Worldwide, 10 units (established from 1986 to 1997), use active national surveillance of more than 8500 clinicians each month to identify cases of rare or uncommon diseases in a childhood population (younger than 15 years of age) of over 47 million (monthly response rate 73% to 98%). By January 1999, units had initiated 147 studies on 103 different conditions, and 63 studies were completed. CONCLUSION: INoPSU enhances collaboration among units from four continents, providing a unique opportunity for simultaneous cross-sectional studies of rare diseases in populations with diverse geographical and ethnic characteristics. It facilitates the sharing of ideas regarding current methodology, ethics, the most appropriate means of evaluating units and their potential application.

The Immunization Monitoring Program Active (IMPACT) prospective five year study of Canadian children hospitalized for chickenpox or an associated complication.

BACKGROUND: Varicella vaccine was approved for use in Canada in 1998. A major goal of universal varicella vaccine programs is to reduce severe infection and associated complications. Baseline data are essential against which to judge the effectiveness of routine childhood immunization. OBJECTIVE: To describe morbidity and mortality among children hospitalized for chickenpox. Methods. From January 1, 1991, to March 31, 1996, chickenpox admissions to 11 pediatric referral centers were actively identified. Patient and illness characteristics were compared for 3 subgroups defined by prior health: healthy; unhealthy but immunocompetent; immunocompromised. RESULTS: Of 861 cases 488 (56.7%) were healthy, 75(8.7%) were unhealthy and 298 (34.6%) were immunocompromised. The immunocompromised children differed from healthy/unhealthy cases in mean age (6.4 vs. 4.0/4.6 years, respectively, P < 0.0001); median interval from rash onset to admission (2 vs. 5/5 days, P < 0.0001); complication rate (20% vs. 90%/79%; P = 0.001); and rate of acyclovir therapy (98% vs. 24%/39%; P = 0.001). Unhealthy vs. healthy cases had a higher frequency (P < 0.01) of intensive care (13.3% vs. 4.7%), ventilation (9.3% vs. 2.0%) and death (4% vs. 0.2%). CONCLUSION: These data provide a baseline for morbidity/mortality resulting from chickenpox before varicella vaccine use in Canada.

Relationship between antenatal group B streptococcal vaginal colonization and premature labour.

OBJECTIVE: To determine whether a population of pregnant women with group B streptococcal (GBS) vaginal colonization had an increased risk of specific epidemiological and intrapartum risk factors for early onset GBS disease. SETTING: Tertiary university centre in Ottawa, Ontario. DESIGN: Hospital-based retrospective cohort study. METHODS: Pregnant women who gave birth during a four-month period in 1994 were included in the study. Potential GBS risk factors were obtained from a review of medical records. The prevalence of each risk factor in colonized and noncolonized women was examined using chi(2) or Fisher's exact test. Multiple logistic regression was performed. RESULTS: A total of 986 women, including 94 (9.5%) women colonized with GBS, were studied. The proportion of women younger than 20 years of age in the colonized group was 2.1% (two of 94) versus 4.6% (41 of 891) in the noncolonized group (P=0.28). Similar rates of multiple births were observed among the colonized and noncolonized groups (2.1% [two of 94] versus 2.5% [22 of 891], respectively) (P=0.94). Likewise, there were no significant differences in either group in the prevalence of a previous pregnancy affected by GBS or diabetes mellitus (P=0.82 and P=0.79, respectively). Multivariable analyses indicated that women who were colonized with GBS were more than twice as likely to deliver prematurely (below 37 weeks' gestational age) (odds ratio [OR] 2.43, 95% CI 1.39 to 4.23). Similarly, colonized women were more likely to be febrile during labour (at least 38 degrees C) (OR 5.05, 95% CI 1.70 to 15.02). CONCLUSION: GBS vaginal colonization was associated with premature labour and intrapartum pyrexia in the population studied. According to Canadian and American guidelines, women with GBS vaginal colonization qualify for intrapartum chemoprophylaxis. The study results suggest that the identification of women at risk of premature labour may be one advantage of early prenatal screening for GBS.