The Budd-Chiari syndrome.
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Biomedical subjects
Publications and source records attributed to G M Edington.
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There is a high prevalence of primary liver cell carcinoma in the Guinea Savanna country of Nigeria, one of the highest frequencies in the world. The highest frequency of the tumour was among males aged 20 to 49 years. The clinical and laboratory findings are similar to those found in other series in Africa. Alpha1-foeto-protein occurred in 85% of cases while HBsAg was noted in 49% of cases.
Evidence for the hypertensive origin of Nigerian 'heart muscle disease' is presented. Epidemiologically, clinically and pathologically it is similar to hypertensive failure and it is associated with hypertension. It is more severe than hypertensive failure as judged by its prognosis and the myocardial damage at necropsy. The radiographic aortic width and renal histology, but not the retinal arteries have evidence of chronic hypertension in some. Using renal histology and scores on a discriminant analysis it was estimated that 34/62 cases in the necropsy series had been hypertensive . Up to 28 cases had not suffered from chronic hypertension but this figure (3%) of cardiac necropsies does not justify the identification of a highly prevalent myocardial disease in Nigeria. The arguments could apply to South African cryptogenic 'heart disease' and 'Jamaican cardiomyopathy'. The study presents data on the natural history of hypertensive failure.
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Seventy five patients with large spleens were investigated in order to establish the causes of splenomegaly in Northern Nigeria, to define further the diagnostic criteria of tropical splenomegaly syndrome (TSS), and to study its pathogenesis. Investigations included examination of liver biopsy, bone marrow cytology, lymphocyte response to phytohaemagglutinin (PHA), serum immunoglobulins and complement, and the presence of immunoglobulin and complement fixed in Kupffer cells. Thirty patients had TSS, five chronic lymphatic leukaemia (CLL), four a syndrome of gross lymphoid hyperplasia (GLH) distinct from TSS, CLL and the lymphomas, and twenty three miscellaneous conventional diseases. In thirteen cases no definite diagnosis could be established. TSS was found to be predominantly a disease of female Fulani cattle herders. Its essential characteristics were splenomegaly in the presence of acquired immunity to malaria, a grossly raised serum IgM, a lowered serum complement, and the presence of IgM fixed in Kupffer cells. There was lymphoid hyperplasia in bone marrow, hepatic sinusoids and often blood which may be indistinguishable from that in CLL. Lymphocytes undergo normal blastogenesis to PHA. There was clinical and haematological response to proguanil therapy. Reticuloendothelial phagocytosis of IgM, probably as a complex, seems to be the essential feature of the condition. As it was impossible to identify early cases of TSS it is unclear whether IgM overproduction or phagocytosis of IgM complexes is the first stage of the disease. The precise nature of the association with malaria remains obscure. The diagnosis of CLL demanded the demonstration of an abnormally low immunoglobulin level and impaired lymphocyte responsiveness to PHA by blast transformation or 3H-thymidine incorporation, in addition to the usual haematological findings. The syndrome GLH occurred in multiparous Hausa women. It was characterised by intense lymphocytosis with active, PHA-responsive cells, and normal immunoglobulin levels. Patients responded to proguanil therapy. It is suggested that these patients have a depressed immune response to malaria, perhaps through repeated pregnancies, and to a leukaemogenic agent, both of which stimulate lymphocytosis. Antimalarial treatment at this stage may prevent the development of frank leukaemia or lymphoma. The usefulness of the various investigative procedures and the problem of managing the large number of undiagnosed cases are discussed.
The findings in 147 unselected consecutive post mortem coronary arteriograms carried out at the University College Hospital, Ibadan, are analysed. The so-called third primary division of the left coronary artery was noted in 35% of cases which is much less than the 74% quoted for the South African Bantu. In atrial blood supply, there was a definite predominance of one coronary artery. The predominant artery to both atria originated in one-third of cases from the right coronary, in another third from the left coronary and in the remaining third from both coronary artery systems respectively. Only in eight instances was atherosclerotic disease noted and even then, this was of the mildest degree with only minimal and slight intimal irregularity. This further explains the known rarity of myocardial infarction as a cause of death in the Nigerian. The study forms a useful basis for the baseline anatomy of the coronary arteries in Nigerians as a prelude to in vivo studies in the cardiomyopathies.
Ultrastructural changes appeared in liver biopsy specimens from eight patients with glucose-6-phosphate dehydrogenase deficiency who developed jaundice during the course of lobar pneumonia. The observed changed indicate that the hyperbilirubinemia and jaundice that develop in patients with glucose-6-phosphate dehydrogenase deficiency with lobar pneumonia are caused partly by hemolysis and partly by intrahepatic cholestasis. The cholestasis may be caused by a genetic predisposition in enzyme-deficient individuals; the hemolysis may be induced by pulmonary infection in individuals with an enzyme deficiency.
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