Acceptability of antenatal diagnosis for sickle-cell disease among Jamaican mothers and female patients

A questionnaire on the need and acceptability of antenatal diagnosis (AND) for sickle-cell disease was given to 112 women with homozgous sickle-cell (SS) disease and 117 AS mothers of patients with SS disease, all aged 15-45 years. AND was favoured by 57 percent of patients and 76 percent of mothers, the option for termination of an affected pregnancy being the reason in 43 percent of patients and in 51 percent of mothers among this group. Fear of the procedure was the commonest reason for not selecting AND. Of the two common methods, chorinonic villus sampling (first trimister) and amniocentesis (second trimester), 74 percent of all women selected the first trimester method, and 93 percent considered these test should be available in Jamaica. Faced with the diagnosis of a foetus with SS disease, 30 percent of patients and 46 percent of mothers stated that they would request termination. (AU)

Parvovirus-associated aplastic crisis in homozygous sickle cell disease - abstract

Aplastic crises in patients with homzygous sickle-cell (SS) disease in Jamaica affect predominantly children and occur in epidemics. Of 67 cases in a cohort study of 314 children with SS disease, 62 were attributable to human parvovirus infection. Affected children were aged 0.5 - 12.5 years, and the incidence rose to 28 percent in 10 years. No recurrences were seen. Symptoms and signs on presentation were attributable to the viraemia, and acute anaemia. Asymptomatic thrombocytopaenia was common. Blood transfusion was given in 54 (87 percent) cases. Thirty-eight (61 percent) children were admitted to hospital, 16 of whom were extremely ill on presentation and one died soon after admission to hospital. Twenty-four (39 percent) were managed as out-patients, 16 of whom were transfused. Parvovirus-associated aplastic crisis is a self-limiting condition with excellent prognosis if diagnosed promptly and appropriately managed (AU)

Parvovirus infection and the aplastic crisis of sickle cell disease - abstract

The aplastic crisis is a temporary self-limited erythropietic maturation arrest which, because of the short red cell survival in homozygous sickle-cell (SS) disease, results in rapid fall in haemoglobin level. These aplastic crises occur in epidemics, predominantly affect children, frequently involve siblings simultaneously, and often follow an upper respiratory tract type of infection. Recent evidence suggests that these events may follow human parvovirus (HPV) infection and the role of HPV infection has been examined in the last three epidemics of aplastic crises observed among SS patients in Jamaica. Eighteen patients were affected in the 1973-1975 epidemics, 45 in the 1979-1981 epidemic, and 41 so far in an epidemic in 1984-1985. Serological studies have been possible in 73 patients and in 69 (95 percent) there was evidence consistent with recent HPV infections. This infection appears to confer long-lasting immunity, and recurrent attacks of aplastic crisis have never been described. These data suggest that a parvovirus vaccine may be beneficial in the prevention of aplastic crises (AU)