The Jamaican Haemophilia Registry: Describing the burden of disease.

INTRODUCTION: Jamaica has an estimated 200 persons with haemophilia (PWH), who face significant constraints in access to specialized haemophilia care, including access to clotting factor concentrates. AIM: The aim of this paper is to establish the current burden of disease in PWH in Jamaica. METHODS: PWH were enrolled through the University Hospital of the West Indies, Jamaica. The impact of haemophilia was assessed using a comprehensive battery of heath outcome measures that included the following: laboratory, clinical information and validated outcome measures of joint structure and function, activity, and health-related quality of life (HRQoL) to provide a health profile of the Jamaican haemophilia population. RESULTS: In all, 45 PWH were registered (mean age: 29, range: 0.17-69 years), including 13 children (<18 years of age) and 32 adults. In this sample, 41 had haemophilia A (30 severe) and 4 had haemophilia B (3 severe); 10 patients with haemophilia A were inhibitor positive. The results indicate that adults with haemophilia in Jamaica have significant joint damage: mean Haemophilia Joint Health Score (HJHS) = 42.1 (SD = 17.3); moderate activity levels - mean Haemophilia Activities List (HAL) score = 64.8 (SD = 17.8); and low HRQoL scores - mean Haemo-QoL-A score = 62.3 (SD = 19.4). Results for children are also reported but should be interpreted with caution due to the small sample size. CONCLUSIONS: There is a very high burden of disease in PWH in Jamaica. The health profiles reported in this paper are an essential first step in advocating for a multidisciplinary Comprehensive Care Program for assessment and care of PWH in Jamaica.

Cost-effectiveness of hepatitis B vaccine in The Gambia.

Hepatitis B vaccine has been progressively introduced into the Gambian programme of immunization since 1986. Other African countries are considering introduction but require evidence of cost-effectiveness to justify such a decision. The cost of the Expanded Programme on Immunization in The Gambia, which includes hepatitis B vaccine, was calculated for 1988. Estimates of the effects of this programme on the incidence of liver cancer were made, based on the national cancer registry and a case control study of primary liver cancer and hepatitis B, regarding hepatitis B vaccine costs as incremental. This gave an estimate of the cost of averting a death from liver cancer in the range US$150-200 (assuming a vaccine cost of US$1 per dose). This indicates that universal hepatitis B immunization is comparable, in terms of cost-effectiveness, to other health interventions in less developed countries.

The Gambia Hepatitis Intervention Study: follow-up of a cohort of children vaccinated against hepatitis B.

Hepatitis B vaccine has been introduced into The Gambia's national Expanded Programme on Immunization, with the aim of evaluating the impact on chronic liver diseases and in particular on hepatocellular carcinoma. As part of the project, a cohort of 1000 children was recruited to assess the long-term protection induced by the vaccine. The first 3 years of follow-up are described. By the age of 3 years, 95% of the children had protective antibody levels; 4 (0.6%) are known to be carriers in contrast to the 90-140 that would be expected in the absence of vaccination. The protective effectiveness of vaccine in preventing chronic carriage in the first 3 years of life is thus estimated as 95%. Since the risk of becoming a carrier is highest in those infected early in life, these results are encouraging.

Antiradiation compounds XVI: N-heterocyclic aminoethyl disulfides and aminoethanethiosulfuric acids.

A series of N-heterocyclic and N-heterocyclic alkyl derivatives of bis(2-aminoethyl) disulfide and aminoethanethiosulfuric acid was synthesized as potential antiradiation and anticancer agents. The compounds were prepared by the reactions of the heterocyclic halides with bis(2-aminoethyl) disulfide and aminoethanethiosulfuric acid. A dithio acid derivative, 3,3-dimercapto-2-cyanoacryloylpyrrolidide, was also prepared. Several compounds, including the dithio acid derivative, provided good radiation protection to mice. None of the compounds screened showed appreciable anticancer activities in two leukemia systems.

Synthesis and antimalarial activity of heterocyclic alkyl disulfides, thiosulfates, and dithio acid derivatives.

Based on the antimalarial activity in mice of bis(4-rho-acetamidobenzenesulfonamidophenyl) disulfide, a series of N-heterocyclic alkyl disulfides and thiosulfates was synthesized and screened for antimalarial activity. Several related dithio acid dianions and S- blocked derivatives were also screened to provide an indication of the possible role that thiol anions might play in malaria chemotherapy. Activity was limited by toxicity with these compounds, and none of those tested, with the exception of bis(4-rho-acetamidobenzenesulfonamidophenyl) disulfide, showed curative activity in either a mouse or chick test.