Haemophilia in northern Nigeria.

Thirteen patients from 11 families were diagnosed as cases of haemophilia A or B during 1977 to 1984. The majority presented with post circumcisional bleeding. Cryoprecipitate and packed red cells were used in the management. Haemophilia and other inherited bleeding disorders are not as rare as otherwise assumed. This is the first report from Northern Nigeria.

Usefulness of the bleeding time to predict the risk of clinical bleeding in patients with uraemia.

In a prospective study of haemostatic status of 15 uraemic Nigerians a highly significant prolongation of bleeding time (p less than 0.001) and significantly lower haematocrit levels (p less than 0.001) were detected in comparison to healthy controls. A highly significant positive correlation (r = +0.778) between prolongation of bleeding time and blood urea was present. Six patients had overt clinical bleeding with significantly prolonged bleeding time (p less than 0.01) compared to uraemics without overt bleeding. Blood urea was significantly different in both groups (p greater than 0.1). Platelet count, prothrombin time, partial thromboplastin time and Hess tourniquet test did not differ between the uraemic patients and controls. Haemostatic dysfunction has been noted in Nigerian uraemics we studied. Bleeding time is a useful but simple means of assessing such dysfunction.

Hereditary haemorrhagic telangiectasia in Nigeria: case report.

A case of hereditary haemorrhagic telangiectasia is described. This is the second case report of this disorder in Nigerians.

Antithrombin III in Echis carinatus envenomation in northern Nigeria.

Antithrombin III was evaluated in 21 patients admitted with Echis carinatus envenomation. Although there was evidence of incoagulability in all patients and elevated fibrin degradation products, mean antithrombin III levels were found to be significantly greater than in controls. This result suggests that Echis carinatus venom (echarin) may bypass the consumption of antithrombin III molecules.

Human immunodeficiency virus antibody screening in blood donors from India, Nigeria and Thailand.

Screening for human immunodeficiency virus (HIV) (LAV/HTLV-III) antibodies in 3 blood donor populations from India (n = 1,000), Nigeria (n = 500) and Thailand (n = 650; sampling in 1982) with a sensitive enzyme immunoassay (EIA; Abbott) yielded seropositivity rates of 0.5, 2.2 and 1.7%, respectively. Two EIAs with control antigens prepared from uninfected cell cultures ('ELAVIA', VIRGO'), a recombinant Escherichia coli DNA EIA ('ENV/CORE'), Western blot, an immunofluorescence assay and a radio-immunoprecipitation assay confirmed none of the EIA-reactive specimens as truly positive. The lack of specificity of the screening test was also attributable to monochromatic evaluation of the test trays at 492 nm only, and to reactivities against determinants of H9 cells used to grow HIV (HLA antibodies).

Polyarticular pneumococcal pyarthrosis in a young haemophiliac.

A case of pneumococcal pyarthrosis involving multiple joints in a moderate haemophiliac is reported. To our knowledge this is the second report of this rare complication in the English literature. Infection should be considered in an acutely inflamed joint in a haemophiliac with hyperpyrexia and unresponsive to replacement therapy. A review of the literature and our observation in this case highlight some criteria that could be used to diagnose this rare but debilitating complication early.

Haemoglobin GPhiladelphia and its interaction with haemoglobin S and alpha-thalassaemia in Nigerians.

The diagnosis of Hb SS/GPhiladelphia disease was made in four young Nigerians from separate families. Their Hb electrophoretic patterns on cellulose acetate membrane at alkaline pH were similar to those obtained in sickle-cell haemoglobin C (HbSC) disease, but their clinical features and haematological data were consistent with the diagnosis of homozygous sickle-cell disease. Family studies also revealed that they had inherited an additional alpha-chain mutant haemoglobin. In one of the families, fingerprints of the globin peptides and amino acid analysis confirmed that the mutant haemoglobin was Hb GPhiladelphia (alpha 2 68 Asn----Lys beta 2 A). The results of the whole blood solubility test for sickle-haemoglobin provided firm support for the diagnosis of homozygous sickle-cell disease and distinguished clearly Hb SS/GPhiladelphia disease from Hb SC disease and Hb AS from Hb AGPhiladelphia heterozygotes. Restriction endonuclease mapping of the globin genes of the propositus and some relatives of one of the families revealed also that they were carriers of the alpha-thalassaemia-2 gene (deletion-type). The globin gene-analysis data indicate also that the alpha GPhiladelphia and alpha-thalassaemia genes are linked closely in Nigerians.

Glycosylated haemoglobin (HbA1) for diabetic control in Africans; preliminary findings with the microcolumn technique.

Glycosylated haemoglobin (HbA1) was determined by a microcolumn technique using Biorex 70 resin in normal Nigerians and in patients with diabetes, iron deficiency anaemia, sickle cell disease and renal failure. Mean %HbA1 was 10.0 +/- 1.9% in controls which is higher than levels reported in Caucasians. HbAS but not HbSS had a reducing effect on the %HbA1 which was also found elevated in iron deficiency anaemia and diabetes mellitus. Factors peculiar to tropical medical practice affecting glycosylated haemoglobin must be appreciated and further investigated to assess the usefulness of HbA1 determination in the long term control of diabetes mellitus.

Endotoxaemia in Vibrio El Tor cholera.

Endotoxaemia and endotoxin-induced changes were sought in Nigerian patients presenting with cholera/diarrhoea. The organism was Vibrio cholerae, bio-type El Tor, serotype Hikojima. The limulus amoebocyte lysate gelation test was used qualitatively by the clot method, whilst a spectrophotometric method was used quantitatively to measure endotoxin levels. 25 acutely ill patients tested had detectable endotoxaemia by the Escherichia coli endotoxin standard. The highest endotoxin level was found in a patient with sub-conjunctival haemorrhage. Changes in platelet counts, the detection of complement breakdown product C3d in plasma, the elevation of fibrin degradation products, the finding of elevated, normal or depressed C3 levels and the absence of circulating immune complexes, suggest a pathogenic role for endotoxin in Vibrio cholerae El Tor diarrhoea.