Congenital Rubella Syndrome Surveillance in South Africa Using a Sentinel Site Approach: A Cross-sectional Study.

BACKGROUND: Congenital rubella syndrome (CRS) includes disorders associated with intrauterine rubella infection. Incidence of CRS is higher in countries with no rubella-containing vaccines (RCV) in their immunization schedules. In the World Health Organization African region, RCVs are being introduced as part of the 2012-2020 global measles and rubella strategic plan. This study aimed to describe the epidemiology of confirmed CRS in South Africa prior to introduction of RCVs in the immunization schedule. METHODS: This was a descriptive study with 28 sentinel sites reporting laboratory-confirmed CRS cases in all 9 provinces of South Africa. In the retrospective phase (2010 to 2014), CRS cases were retrieved from medical records, and in the prospective phase (2015 to 2017) clinicians at study sites reported CRS cases monthly. RESULTS: There were 42 confirmed CRS cases in the retrospective phase and 53 confirmed CRS cases in the prospective phase. Most frequently reported birth defects were congenital heart disease and cataracts. The median age of mothers of CRS cases was 21 years in the retrospective phase (range: 11 to 38 years) and 22 years in the prospective phase (range: 15 to 38 years). CONCLUSION: Baseline data on laboratory-confirmed CRS will enable planning and monitoring of RCV implementation in the South African Expanded Programme on Immunization program. Ninety-eight percent of mothers of infants with CRS were young women 14-30 years old, indicating a potential immunity gap in this age group for consideration during introduction of RCV.

Peripartum HIV infection in very low birth weight infants fed 'raw' mother's own milk.

BACKGROUND: HIV-exposed very low birth weight (VLBW) infants (≤ 1500 g) are considered at high risk of peripartum mother-to-child HIV transmission (MTCT). In the past, they received formula to prevent breast milk related HIV transmission. This denied them the benefits of breast milk, thus exposing the infant to the risk of necrotising enterocolitis (NEC). From 2010, 'raw' mother's own milk (rMOM) has been recommended for term infants whose mothers' received antenatal antiretroviral therapy (ART). At the same time, the infant received antiretroviral (ARV) prophylaxis as per the National Prevention of MTCT programme. OBJECTIVES: To determine the cumulative incidence of peripartum HIV infection by 4-6 weeks of age in HIV-exposed VLBW infants, who received rMOM and infant ARV prophylaxis. METHOD: A retrospective, observational audit over 3 years at a single institution was undertaken. The study population comprised HIV-exposed VLBW infants who received both nevirapine prophylaxis and rMOM from birth until discharge. A positive HIV-PCR by 4-6 weeks of life was used to confirm maternal to infant HIV transmission. RESULTS: Of the 80 eligible infants admitted between 2010 and 2013, 63 (79%) were exposed to antenatal ART. Seventy-eight (97.5%) tested HIV-PCR negative at 4-6 weeks. Of the two infants who tested positive, both presented with features of an acute HIV infection. The absence of MTCT in the remaining 78 infants given ARV prophylaxis and rMOM suggests that rMOM is an unlikely source of infection in the two infected infants. CONCLUSION: rMOM, in the presence of infant prophylaxis, was a safe feeding option for HIV-exposed VLBW infants. It should be strongly considered for these infants, as rMOM likely provides additional maternal and child benefits.

Pena-Shokeir syndrome: current management strategies and palliative care.

Pena-Shokeir syndrome (PSS) type 1, also known as fetal akinesia deformation sequence, is a rare genetic syndrome that almost always results in intrauterine or early neonatal death. It is characterized by markedly decreased fetal movements, intrauterine growth restriction, joint contractures, short umbilical cord, and features of pulmonary hypoplasia. Antenatal diagnosis can be difficult. Ultrasound features are varied and may overlap with those of Trisomy 18. The poor prognosis of PSS is due to pulmonary hypoplasia, which is an important feature that distinguishes PSS from arthrogryposis multiplex congenital without pulmonary hypoplasia, which has a better prognosis. If diagnosed in the antenatal period, a late termination of pregnancy can be considered following ethical discussion (if the law allows). In most cases, a diagnosis is only made in the neonatal period. Parents of a baby affected with PSS require detailed counseling that includes information on the imprecise recurrence risks and a plan for subsequent pregnancies.