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CSTE POSITION STATEMENT 1999-ID 4 COMMITTEE: Infectious DiseasesTITLE: Intensifying
surveillance and updating of Congenital Rubella Syndrome (CRS)
case definition. ISSUE: Rubella vaccines were licensed in the United States in 1969.
At that time, an expert panel on rubella and CRS met to establish case
definitions and classification for a national CRS registry.
Many changes in rubella vaccination policies and epidemiology have
occurred since 1969, including high coverage with MMR among children,
implementation of a routine second dose schedule of MMR for prevention of
measles, record low levels of reported rubella and CRS cases, and development of
new laboratory methods for rubella. As
part of Healthy People 2000, a goal for the elimination of indigenous CRS was
established for the year 2000. POSITION
TO BE ADOPTED: States and territories should be provided with additional resources
for vaccine-preventable disease surveillance in order to carry out intensified
surveillance for congenital rubella surveillance activities. States and
territories should intensify surveillance for CRS. Efforts should be focused on providing educational
information to health care providers who are responsible for the diagnosis and
care of these infants (e.g. cardiologists, audiologists, ophthamologists and
neonatologists). Because hearing
impairment is one of the most common manifestations of CRS, states that have
newborn hearing screening programs in place may wish to develop partnerships
with these programs to enhance ascertainment of infants with CRS. CDC will provide
a package with educational materials that can be distributed.
This package would include information on newborn hearing testing,
signs/symptoms associated with CRS and the appropriate laboratory work-up. The following
case definition for CRS is adopted: Clinical
case definition
Laboratory
criteria for diagnosis
Clinical
description Presence
of any defect(s) or laboratory data consistent with congenital rubella
infection. Infants with CRS usually present with more than one sign or
symptom consistent with congenital rubella infection. However, infants may present with a single defect.
Deafness is most common single defect. Case
classification
Note: In
probable cases, either or both of the eye-related findings (cataracts and
congenital glaucoma) count as a single complication. In cases classified as infection only, if any compatible
signs or symptoms (e.g., hearing impairment) are identified later, the case is
reclassified as confirmed. The
elimination of indigenous rubella and CRS in the United States has been targeted
for the year 2000. To document that
elimination has been achieved, improved surveillance is needed.
Moreover, extensive evaluation of reported cases and new methods of case
diagnosis support revisions of the case definition. In a meeting of experts on May 11, an analysis of the
prospective and retrospective studies evaluating the clinical manifestations of
CRS was presented and an update of the CRS case definition was proposed.
The revisions in the CRS case definition are included in the clinical
description and clinical case definition to more clearly describe and update the
spectrum of CRS clinical presentation. Laboratory
criteria for diagnosis was expanded to include PCR. With
record low levels of CRS cases, intensifying surveillance is critical to
document the elimination of CRS. In
a retrospective chart review of infants hospitalized between 1994-1996 in a
hospital at the U.S.-Mexico border, 9 infants were identified in having
signs/symptoms that met the probable or confirmed case classification for CRS
(unpublished data, CDC). Of
those 9, two infants had confirmed CRS and only one CRS case had been reported
to the state health dept. None of
the 7 other cases had appropriate laboratory evaluation for CRS.
The incidence of CRS in that hospital was >400 times that of the
national incidence of CRS. These
findings emphasize the need to educate health care providers about the
signs/symptoms and diagnostic evaluation of infants with CRS and reporting
requirements. As
noted in the previous example, infants with CRS are being missed.
Identifying mechanisms to identify possible cases is important. In 1999, the American Academy of Pediatrics (AAP) recommended
universal newborn hearing screening. With
hearing impairment being one of the most common defects in CRS, this
recommendation provides a new opportunity to improve ascertainment of less
severely affected cases. COORDINATION
WITH OTHER ORGANIZATIONS: Agency for Response: Centers for Disease Control and Prevention
(CDC) Institute of Medicine Immunization Committee Agency for Information: Association of State and Territorial Health Officials, ASTHO CONTACTS: Stephen Waterman, MD, MPH
David Fleming, MD
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