Cases Of Congenital Rubella Syndrome Research Articles

The seroepidemiology of rubella in western Europe.

Most of the countries in western Europe have now implemented mass infant rubella immunization programmes, instead of or in addition to selective vaccination in order to achieve the elimination of congenital rubella syndrome. The European countries Denmark, England and Wales, Finland, France, Germany, Italy and the Netherlands undertook large, national serological surveys collecting several thousand serum specimens during 1994-8. Antibodies against rubella virus were detected by a variety of enzyme immuno-assays. Comparability of the assay results was achieved by a standardized methodology. The age- and sex-stratified serological results were related to the schedules, coverage of rubella vaccination and the incidence in these countries. The results show widely differing levels of immunity to rubella both in the general population and in the specific age groups of males and females. A low rate (< 5%) of susceptibles in childhood and adolescents of both sexes was obtained only in Finland and the Netherlands. Countries such as Italy with only moderate coverage for the infant immunization programme currently have both high susceptibility levels in the general population and in the at-risk population. The likelihood is of continued epidemics of rubella with cases of congenital rubella syndrome. The continued implementation of selective vaccination will help to offset the impact of this ongoing transmission and to protect women on reaching childbearing age.

RUBELLA IMMUNITY IN YOUNG WOMEN

To The Editors: The articles on rubella in the July issue of the Pediatric Infectious Disease Journal® by Plotkin1 and Aksit et al.2 were of considerable interest to us. Last year we carried out a retrospective study of the prevalence of rubella IgG antibody in our patient population as measured by an enzyme-linked immunosorbent assay (Sigma). Overall 7848 specimens (7636 female and 210 male) were evaluable, and 1033 specimens (13.1%) lacked detectable antibody. A substantial portion of women in their childbearing years, 14.2% of those between 15 and 35 years of age, did not have rubella antibodies. These percentages are much higher than the estimate of 8% for rubella susceptibility in the United States cited by Aksit et al. The lack of detectable antibody suggests to us that many opportunities for immunization have been missed and that the potential for cases of congenital rubella syndrome will remain until the percentage of seronegative individuals is reduced. William T. Walsh Ph.D. Loma Payton M.P.A., M.T. (ASCP) Hurley Medical Center Flint, MI Accepted for publication Nov. 19, 1999.

Modelling the incidence of congenital rubella syndrome in developing countries.

As of 1997, less than one-third of developing countries included rubella vaccine in their national immunization programme. In countries that have achieved high coverage of measles vaccine, an ideal opportunity exists to include control of rubella and congenital rubella syndrome (CRS) in enhanced measles control activities. Data on the burden of congenital rubella syndrome are important to guide rubella vaccination policies. We reviewed the literature to identify studies of rubella antibody prevalence in developing countries that were conducted on populations with no major selection bias, prior to wide-scale rubella vaccination in the country. We used a simple catalytic model to describe the age-specific prevalence of susceptibility to rubella virus infection in given populations. Estimates of the incidence of infection among pregnant women were calculated using expressions for the average prevalence of susceptibility to infection and the incidence of infection during gestation. To estimate the number of cases of CRS, we assumed an overall risk of 65% after infection in the first 16 weeks of pregnancy and zero risk thereafter. These estimates were derived for each country for which data were available, then for each World Health Organization region, excluding Europe. The estimated mean incidence of CRS per 100,000 live births was lowest in the Eastern Mediterranean region (77.4, range 0-212) and highest in the Americas (175, range 0-598). The mean of the estimates of the total number of cases of CRS in developing countries in 1996 was approximately 110,000. The range was, however, very wide, from as few as 14,000 to as many as 308,000 cases. Congenital rubella syndrome is an under-recognized public health problem in many developing countries. There is an urgent need for collection of appropriate data to estimate the cost-effectiveness of a potential global rubella control programme.

Prevalence of antibodies against rubella virus in The Netherlands 9 years after changing from selective to mass vaccination.

A two-dose mass vaccination programme with a combined vaccine against measles, mumps and rubella (MMR) was adopted in the Netherlands in 1987, replacing the selective schoolgirl vaccination strategy introduced in 1974. To obtain insight into the effect of mass vaccination and the population's immunity, the antibody levels against rubella were studied in the general Dutch population and in religious groups refusing vaccination. In the national sample, we observed a high prevalence (96.5%) for rubella antibodies in vaccinated cohorts as well as in the older unvaccinated cohorts. No indications of rapidly waning immunity after vaccination were found. There are indications of low virus circulation in the last few years. The very high seroprevalence in women at childbearing age is consistent with the few reported cases of congenital rubella syndrome (CRS) at present. However, individuals in the age group of 1-9 years who are not vaccinated for religious or other reasons have a considerably lower seroprevalence and thus there is a potential risk of a CRS outbreak in the future.

Changing Epidemiology of Congenital Rubella Syndrome in the United States

To describe clinical presentation and epidemiology of US infants with congenital rubella syndrome (CRS) and to identify missed opportunities for maternal vaccination, data from CRS cases reported to the National Congenital Rubella Syndrome Registry (NCRSR) from 1985 through 1996 were analyzed. Missed opportunities for maternal vaccination were defined as missed postpartum, premarital, and occupational opportunities, that is, times when rubella vaccination is recommended but was not given. From 1985 through 1996, 122 CRS cases were reported to the NCRSR. The most frequent CRS-related defect was congenital heart disease. Of the reported infants with CRS, 44% were Hispanic. Of 121 known missed opportunities for rubella vaccination among 94 mothers of infants with indigenous CRS, 98 (81%) were missed postpartum opportunities. CRS continues to occur in the United States. Hispanic infants have an increased risk of CRS. Missed opportunities for postpartum rubella vaccination were identified for 52% of indigenous CRS cases.

A model-based evaluation of the national immunization programme against rubella infection and congenital rubella syndrome in The Netherlands.

In order to improve the prevention of cases of congenital rubella syndrome in The Netherlands, in 1987 the selective vaccination strategy against rubella infection in girls was replaced by mass vaccination. This decision was supported by mathematical model analyses carried out by Van Druten and De Boo. In order to compare the predicted impact of the rubella vaccination programme with the current available data in more detail, a similar model was built. Although the model predicts elimination of the rubella virus, data show that virus circulation is still present at a higher level than expected by the model. Simulation studies indicate that import of infection and a lower vaccine effectiveness, related to possible asymptomatic reinfection of vaccinated people, could be sources contributing to the present virus circulation. Even though the number of infections is much higher than the number of reported cases of disease, limited serosurveillance data and case notification data show that females of childbearing age are well protected by immunization.

Changing epidemiology of congenital rubella syndrome in the United States.

To describe clinical presentation and epidemiology of US infants with congenital rubella syndrome (CRS) and to identify missed opportunities for maternal vaccination, data from CRS cases reported to the National Congenital Rubella Syndrome Registry (NCRSR) from 1985 through 1996 were analyzed. Missed opportunities for maternal vaccination were defined as missed postpartum, premarital, and occupational opportunities, that is, times when rubella vaccination is recommended but was not given. From 1985 through 1996, 122 CRS cases were reported to the NCRSR. The most frequent CRS-related defect was congenital heart disease. Of the reported infants with CRS, 44% were Hispanic. Of 121 known missed opportunities for rubella vaccination among 94 mothers of infants with indigenous CRS, 98 (81%) were missed postpartum opportunities. CRS continues to occur in the United States. Hispanic infants have an increased risk of CRS. Missed opportunities for postpartum rubella vaccination were identified for 52% of indigenous CRS cases.

The impact of rubella immunisation on the incidence of rubella, congenital rubella syndrome and rubella-related terminations of pregnancy in South Australia.

To describe the impact of rubella immunisation on the incidence of rubella, congenital rubella syndrome and rubella-related terminations of pregnancy in South Australia, and to identify factors associated with a re-emerging problem. A population-based descriptive study using data from South Australian notifications of disease, births and terminations of pregnancy, the rubella immunisation programme, antenatal rubella antibody screening and paediatric hospital case records. South Australia (population 1.48 million people; 20,000 births per year). Incidence of rubella (age-sex specific), congenital rubella syndrome and rubella-related terminations of pregnancy; antenatal rubella sero-positive rates; rubella immunisation uptake rates. Rubella notification rates in 1990-1996 were significantly higher for males than females for ages 15-34 years. There were five cases of congenital rubella syndrome notified in 1980-1996 compared with at least 20 confirmed or compatible cases in 1965-1979. Rubella-related terminations of pregnancy are now rare, with the last termination for maternal rubella being in 1993. The antenatal rubella sero-positive rate in 1995 was 96.7%, but was significantly lower among Asian women born overseas (78.6% among those 30 years or older). Vaccination uptake rates in schoolgirls decreased between 1990 and 1994 (91.2% to 86.9%). Since the introduction of rubella immunisation, the incidence of rubella infection among women of reproductive age, and of rubella-related terminations, has fallen. Congenital rubella syndrome has not been notified since 1990 but its risk persists with a recent increase in rubella notifications, a fall in school immunisation rates, a relatively low antenatal sero-positive rate among older Asian women born overseas and the trend towards giving birth at older ages. Effective immunisation programmes must be maintained, particularly in schools and for young children and migrant women.

Chronic Arthropathy After Rubella Vaccination in Women

The last large epidemic of rubella in the United States was in 1964 when almost half a million cases were reported. The National Communicable Disease Center, now the Centers for Disease Control and Prevention, estimated the true size of the 1964 epidemic as 12.5 million cases, and at least 20 000 clinically evident cases of the congenital rubella syndrome (CRS) were subsequently reported.¹The first rubella vaccine was licensed in the United States in 1969, and from that year on rubella and CRS incidence began to decline. In 1994, there were 227 reported cases of rubella and 7 cases of CRS²; in 1995, there were 128 cases of rubella—the lowest number in history—and 6 cases of CRS³; and in 1996, there were 210 cases of rubella (CRS data are not yet available).⁴Through June 1997, only 64 cases of rubella had been reported in the United

A case of congenital rubella syndrome with diffuse white matter lesion on MRI

A case of congenital rubella syndrome with diffuse white matter lesion on MRI

Ocular manifestations of congenital rubella.

Ocular disease is a major component of the congenital rubella syndrome (CRS). Interest in this condition has been renewed after identification of a spectrum of delayed manifestations and the realization that sporadic cases of CRS will continue to occur despite vaccination programs due to reinfection in the immune patient. Work is continuing on strategies and planning of vaccination programs for disease prevention and control, refinements of diagnosis with particular emphasis on the prenatal diagnosis of fetal infection, and fundamental research into the pathogenic and immunologic mechanisms underlying CRS and its late manifestations.

Assessing the efficacy of a mixed vaccination strategy against rubella in São Paulo, Brazil.

In 1992 a major vaccination strategy against measles-mumps-rubella was introduced in the State of Saão Paulo, Brazil. This strategy was based on mathematical models and comprised a pulse vaccination covering all children aged 1-10 years, followed by the inclusion of this vaccine in the routine calendar at 15 months of age. The present work reports the evaluation of the efficacy of this mixed vaccination strategy. A rubella seroprevalence survey was carried out immediately and one year after the campaign, comprising 4953 children aged 1-15 years. We show that average rubella seroprevalence increased from 0.40 to 0.97 and that the reported number of congenital rubella syndrome (CRS) cases dropped dramatically. The mixed vaccination strategy adopted against rubella has proved to be very effective in reducing the number of CRS cases in São Paulo.

Distribuição por sexos das cardiopatias congénitas.

For 17 years, 4150 infants and children under 13 years of age with final diagnosis of well defined congenital heart disease were studied and the pattern of sex differences are reported. In general, the sex distribution was equal: 2108 males (50.8%) and 2042 females (49.2%) with a male to female ratio of 1.03. Male sex predominance was marked for the following specific lesions: aortic stenosis (valvar and subvalvular) (70%), coarctation of the aorta (66%), transposition of the great arteries (60%), univentricular heart (76%), tricuspid atresia (63%), Ebstein anomaly (76%), hypoplastic left heart syndrome (85%), vascular rings (77%) and right midventricular stenosis (70%). Female sex predominance was marked for the following specific lesions: Persistent ductus arteriosus (72%), atrioventricular septal defects (62%) supravalvular aortic stenosis (71%) and isolated infundibular pulmonic stenosis (80%). A more balanced sex distribution was confirmed for cases of congenital rubella syndrome with persistent ductus arteriosus as isolated cardiovascular malformation (56%). The Importance to establish the sex dominance is emphasized as predictive of recurrence risk of congenital heart disease.

Rubella Vaccination and Congenital Rubella Syndrome in Western Australia

Objective To review the incidence of congenital rubella syndrome (CRS) and the acceptance of schoolgirl rubella vaccination in Western Australia (WA), and to determine whether any groups in the community are at increased risk of having a child affected by CRS. Design Review of records of the WA Birth Defects Registry for cases of CRS; survey of obstetricians for terminations of pregnancy for maternal rubella infection; survey of schoolgirls eligible for the 1991 annual rubella vaccination campaign; review of Perth Immunisation Clinic and rural community and child health records; review of the rubella immune status of women tested antenatally. Main outcome measures The incidence rate of CRS per 10,000 live births in WA 1980-1990; the incidence of rubella-associated terminations of pregnancy during 1990 and 1991; trends in rubella vaccine acceptance among WA schoolgirls between 1971 and 1991; and the proportion of women attending antenatal clinics who showed satisfactory immunity against rubella, stratified by country of birth. Results The incidence rate of CRS in WA remains below 2 cases per 10,000 live births. Approximately 86% of Perth schoolgirls have been vaccinated against rubella. Rubella vaccine acceptance fell in some country areas during the late 1980s, but is now returning to acceptable levels. Women born in Asia are at greater risk of having a baby affected by CRS than women born in Australia. Babies with CRS may be born to women who have previously been vaccinated against rubella. Conclusions The incidence of CRS has fallen dramatically since the introduction of the schoolgirl vaccination program, but there is still a need for mechanisms to identify and vaccinate non-immune women.

Giving Pediatric Immunizations the Priority They Deserve

In the first 6 months of 1991, a rubella outbreak struck a rural community in Pennsylvania, resulting in the births of at least 27 infants with congenital rubella syndrome. Until that time, the entire state had witnessed only two documented cases of congenital rubella syndrome in the previous 20 years—one in 1972 and one in 1975.¹Unfortunately, when it comes to preventable childhood infectious diseases in the United States, this story is not unique. Today, between 37% and 56% of the 7.8 million 2-year-olds in America—about 4 million children— are not fully immunized (unpublished data, Centers for Disease Control and Prevention, 1991). These figures are alarming. Although the vaccines necessary to guard children against infectious diseases such as mumps, measles, diphtheria, hepatitis B, and polio are available, safe, and effective, we as a nation have failed to provide these immunizations on time. Clearly, protecting our See also p 1817.

Rubella vaccination and congenital rubella syndrome in Western Australia.

To review the incidence of congenital rubella syndrome (CRS) and the acceptance of schoolgirl rubella vaccination in Western Australia (WA), and to determine whether any groups in the community are at increased risk of having a child affected by CRS. Review of records of the WA Birth Defects Registry for cases of CRS; survey of obstetricians for terminations of pregnancy for maternal rubella infection; survey of schoolgirls eligible for the 1991 annual rubella vaccination campaign; review of Perth Immunisation Clinic and rural community and child health records; review of the rubella immune status of women tested antenatally. The incidence rate of CRS per 10,000 live births in WA 1980-1990; the incidence of rubella-associated terminations of pregnancy during 1990 and 1991; trends in rubella vaccine acceptance among WA schoolgirls between 1971 and 1991; and the proportion of women attending antenatal clinics who showed satisfactory immunity against rubella, stratified by country of birth. The incidence rate of CRS in WA remains below 2 cases per 10,000 live births. Approximately 86% of Perth schoolgirls have been vaccinated against rubella. Rubella vaccine acceptance fell in some country areas during the late 1980s, but is now returning to acceptable levels. Women born in Asia are at greater risk of having a baby affected by CRS than women born in Australia. Babies with CRS may be born to women who have previously been vaccinated against rubella. The incidence of CRS has fallen dramatically since the introduction of the schoolgirl vaccination program, but there is still a need for mechanisms to identify and vaccinate non-immune women.

Rubella in the developing world

Before Gregg's historic observation [1] rubella was not considered to have clinical or epidemiological importance in any country. In the western world epidemics occurred at varying intervals but with little morbidity and apparently only minor complications. Despite confirmation of Gregg's findings from many quarters, it was not until the worldwide outbreaks in the 1960s that the aftermath of rubella infection in pregnancy was fully realized. As a result of the 1964 outbreak in New York City, more than 1000 children were born with congenital rubella syndrome (CRS) and over 300 pregnancies either aborted spontaneously or were terminated for rubella infection [2]. The number of children affected represented 1% of births in the city; if extrapolated to the whole country this gave an estimated total of 30000 cases of CRS. No such disasters have so far been reported from the developing world.

A Profile of Mothers Giving Birth to Infants With Congenital Rubella Syndrome

To formulate strategies for elimination of congenital rubella syndrome, it is important to identify risk factors for delivering an infant affected by it. We analyzed cases of congenital rubella syndrome in infants born from 1970 to 1985 and reported to either one of two independent Centers for Disease Control surveillance systems. Mothers of infants with congenital rubella syndrome identified in both surveillance systems were disproportionately younger than mothers giving birth in the United States. The risk for delivering an infant with congenital rubella syndrome was approximately 2.5 times higher for blacks compared with whites for both reporting systems. A total of 18% of infants with congenital rubella syndrome born since 1979 were Hispanic (national population average, 7%). Both surveillance systems showed that, although primiparous mothers were at highest risk, 39% of women delivering infants affected by congenital rubella syndrome had had at least one previous live birth, suggesting that postpartum immunization could have prevented these congenital rubella syndrome cases. Young, black, and Hispanic primiparous women represent populations at elevated risk for delivering a congenital rubella syndrome-affected infant and should be specifically targeted for immunization.

Incidence of congenital rubella syndrome in Japan (1965-1985). A nationwide survey of the number of deaf children with history of maternal rubella attending special schools for the deaf in Japan.

Accurate congenital rubella syndrome surveillance in Japan is of great interest because of the hypothesis that the Japanese rubella virus was less virulent than the strains prevalent in Europe and the Americas 20 years ago. A nationwide survey of deaf children with a history of maternal rubella in special schools for the deaf in Japan yielded a total of 365 deaf school children with congenital rubella syndrome born between 1963 and 1982. The overall average prevalence was 0.31 case per 100,000 population, and the incidence was 0.1-6.1 cases per 100,000 annual live births in Japan. If one considers the decrease in the number of deaf children attending special schools for the deaf because of the recent adoption of the policy of integrating deaf children into ordinary schools, the number of cases of congenital rubella syndrome might actually be much greater than that revealed by this survey and much greater than the 88 cases previously reported in Japan. Among the 365 cases who had deafness, 8.2% were found to have cataract (with and without congenital heart disease), and 11.0% were found to be complicated by congenital heart disease (but without cataract).

Two Unusual Cases of Congenital Rubella Syndrome Associated with Acute Myelocytic Leukemia or chilaiditi's Syndrome

AbstractIn the Ryukyu Islands of Japan, an extensive epidemic of rubella occurred from the end of 1964 until the beginning of 1965. Approximately 400 children with congenital rubella syndrome were born in 1965 [1]. Two boys with unusual associated problems are reported in this paper. One case was complicated with leukemia and the other with Chilaiditi's syndrome.