Outcomes of universal antenatal screening for haemoglobinopathies.

OBJECTIVE: To evaluate universal antenatal screening for haemoglobinopathies. SETTING: District general hospital serving a London borough with 45% ethnic minorities. METHODS: Retrospective cohort study of 1444 women referred in 1688 pregnancies and 95 tertiary referrals during 101 pregnancies. RESULTS: Unselected women at risk for sickle cell disease booked 2.7 weeks (95% confidence interval (CI) 0.14 to 5.1) later in gestation than those at risk for beta thalassaemia were less likely to attend counselling (83% v 93%, relative risk (RR) 0.89; 95% CI 0.85 to 0.94), their partners were less likely to be tested (77% v 95%, RR 0.81; 0.77 to 0.83), and they were less likely to accept prenatal diagnosis (22% v 90%, RR 0.37; 0.24 to 0.57). Over 99% of tertiary referrals attended counselling and had their partners tested. There were no significant differences in acceptance of prenatal diagnosis between those at risk of sickle cell disease and beta thalassaemia (55% v 67%). Unselected women at risk of sickle cell disease were significantly less likely to have their partner tested or to accept prenatal diagnosis than tertiary referrals, but not those at risk of beta thalassaemia. 80% of beta thalassaemia and 16% of SS births were prevented. CONCLUSIONS: Uptake of prenatal diagnosis among unselected women at risk of beta thalassaemia is similar to that reported by tertiary centres. It is considerably lower for sickle cell disease but could increase considerably if screening occurred earlier in gestation. Acceptance of counselling is universally high, suggesting that informed choices are made, and indicating a need to measure these outcomes for cost effectiveness studies.

Mapping the prevalence of sickle cell and beta thalassaemia in England: estimating and validating ethnic-specific rates.

A range of estimates for sickle cell and beta thalassaemia have been derived for the different ethnic groups living in the U.K., reflecting uncertainty over the true population value in certain countries and the heterogeneity within and between countries of origin comprising the same ethnic group. These were validated against six community screening programmes, with the estimated range correctly predicting the number of affected births observed by the programmes. In England approximately 3000 affected babies (0.47%) carry sickle cell trait and 2800 (0.44%) carry beta thalassaemia trait annually: with approximately 178 (0.28 per 1000 conceptions) affected by sickle cell disease (SCD) and 43 (0.07 per 1000) by beta thalassaemia major/intermedia. Allowing for termination, about 140-175 (0.22-0.28 per 1000) affected infants are born annually with SCD and from 10 to 25 (0.02-0.04 per 1000) with beta thalassaemia major/intermedia. These are the first evidence-based rates for sickle cell and beta thalassaemia for use in the U.K., and should underpin the future planning of services. The long-term solution to monitoring changes in the rates of trait and disease in the population is to introduce a standardized instrument for collecting ethnicity for all community screening programmes.

The state of health and provision of health care in Hong Kong.

In 1997, Hong Kong became a Special Administrative Region of the People's Republic of China. The new government took control of a Western style health-care system and a population whose health is comparable to the rest of the developed world. This article describes the existing health-care system, the state of public health and some of the challenges and opportunities facing the new administration.

Acute lower leg compartment syndrome.

Acute compartment syndromes in the lower leg are well recognized following major trauma. However, although rare, they may occur following seemingly minor sporting injury. A case of acute compartment syndrome, following a football game and affecting the peroneal or lateral compartment, is described, in which prompt diagnosis and treatment led to a satisfactory outcome. The diagnosis and surgical management of acute compartment syndromes are discussed. Early recognition and treatment are important in the prevention of long-term disability.