Epidemiology of haemophilus influenzae invasive disease in Jamaica, 1990-1993

Haemophilus influenzae (H influenzae) invasive disease was studied retrospectively over a four-year period in children admitted to the Bustamante Hospital for Children in Kingston, Jamaica. A total of 86cases were identified. The mean estimated annual incidence of H influenzae invasive disease in Kingston and St. Andrew was 39 per 100,000 children under 5 years of age. The majority (77percent) of cases were in the under 2-year age group. A distinct seasonal pattern was noted, with a significantly higher proportion of patients (57-73percent) presenting in the cooler months. Meningitis was the most common clinical diagnosis, accounting for 76percent of the cases. Poor outcome was demonstrated in 21.5percent of patients with meningitis. Sensitivity testing of H influenzae isolates revealed a resistance rate of 26percent for ampicillin and 7percent for chloramphenicol. The epidemiological findings in this study provide reasonable guidelines for empiric antibiotic therapy and also support the need to seriously consider vaccine prophylaxis in Jamaican children.

Recurrent infections in sickle cell disease: haematological and immune studies

Some haematological and immunological indices were compared in 19 children with sickle cell disease and a history of recurrent infections and in 16 children with sickle cell disease without any known infections. The recurrent infection group had significantly greater pitted red cell counts and greater absolute monocyte counts. No differences were apparent in routine haematological indices, foetal haemoglobin, immunoglobulin, or complement levels between the groups. The interpretation of these results is discussed (AU)

Non-gouty arthritis in sickle cell disease: report of 37 consecutive cases

Arthritis in association with sickle cell disease was seen in 37 patients in a 21/2-year period. Cases of gout and of avascular necrosis of the femoral head were excluded. In 12 patients a non-inflammatory effusion occurred during the course of a painful crisis, in 12 patients an ankle effusion occurred in association with spontaneous development or deterioration of leg ulceration and in 13 patients there was a group of miscellaneous arthritides. Ankle arthritis with leg ulceration has not been previously recognised and its association with spontaneous ulceration, which is presumed to have a vaso-occlusive origin, is compatible with ischaemic synovial damage. The aetiology may therefore be similar to that believed to account for effusions in association with the painful crisis. (AU)

The immunological profile of babies with homozygous sickle cell disease - abstract

Seventy-two babies with homozygous sickle cell disease (SS) and age-matched controls with a normal haemoglobin were screened for immunoglobulin and complement abnormalities at birth, and again at 6, 12, 18 and 24 months of age. Twenty-seven SS babies had more than one sample taken at different intervals. Abnormalities of IgG, IgA, C3 and C3d were all detected in SS patients at the age of 2 years or before. As a group, elevations of IgA (p<0.05) and of complement breakdown product levels (p<0.002), were apparent from the age of 6 months onwards. As the immunological abnormalities occur at a time when the majority of infections in SS children are observed, it is suggested that they may contribute to the increased susceptibility to infection in SS disease (AU)

Cord blood screening for sickle hemoglobin: evidence against a female preponderance of hemoglobin S

The genes controlling á-chain synthesis in hemoglobins A, S, and C are not sex linked, and sex differences in the prevalence at birth of the various á-chain genotypes would not be expected. However, the results of a recent study in the United States appeared to conflict with this expectation. In a series of 3,976 black infants whose cord blood was examined by hemoglobin electrophoresis, electrophoretic patterns compatible with AS and SS genotypes occurred in significantly higher proportions of females than males. Moreover, the number of SS births was significantly higher than expected on the basis of the observed frequency of S genes. A similar, but much larger screening program is in progress in Jamaica; results for the first 70,000 births are presented below. There were no appreciable sex differences in the proportions with AS and SS genotypes, and the number of SS births was not significantly in excess of its expected value. It seems likely, therefore, that the findings reported previously were the result of stochastic variation. (AU)

Haemoglobin Spanish Town alpha27 Glu replaced by Val (B8)

A new alpha chain variant Hb Spanish Town, a27 Glutamic acid-Valine, awas detected in the cord blood of a Jamaica Negro infant. In the mother the adult component (a2 Spanish TownB2) has an electrophoretic mobility between haemoglobins S and F at alkaline pH and measures 11.0-12.0 percent of the total haemoglobin. (Summary)