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Gregg's congenital rubella patients 60 years later
Jill M Forrest1, Fiona M Turnbull, Gary F Sholler
1Royal Alexandra Hospital for Children, Locked Bag 4001, Westmead, NSW. jillf@chw.edu.au
Background:
In 1941, a Sydney ophthalmologist, Norman McAlister Gregg, correctly identified the link between congenital cataracts in infants and maternal rubella early in pregnancy. Fifty of Gregg's subjects with congenital rubella, born in 1939-1944, were reviewed in 1967 and again in 1991. We reviewed this cohort in 2000-2001, 60 years after their intrauterine infection.
Methods:
The subjects underwent full clinical assessment, plus pathology tests, an ophthalmological and cardiological review (including electrocardiography and echocardiography) and HLA histocompatibility testing.
Results:
Since they were first seen in 1967, 10 have died (cardiovascular causes [4], malignant disease [4], AIDS [1], and hepatitis C-related cirrhosis [1]). All surviving men came for review (19) and 13 women (eight women declined). Echocardiography showed mild aortic valve sclerosis in 68%. The prevalence of diabetes (22%), thyroid disorders (19%), early menopause (73%) and osteoporosis (12.5%) was increased compared with the Australian population; 41% had undetectable levels of rubella antibodies. The frequency of HLA-A1 (44%) and HLA-B8 (34%) antigens was increased, and the haplotype HLA-A1, B8, DR3, said to be highly associated with many autoimmune conditions, was present in 25%.
Conclusions:
This cohort of people with congenital rubella has illuminated our understanding of viral teratogenesis.