Homozygous haemoglobin O Arab disease and conjugated hyperbilirubinaemia.
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Biomedical subjects
Publications and source records attributed to S E Heard.
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High oxygen affinity haemoglobins result in polycythaemia and cardiovascular adaptation to maintain tissue oxygenation. The polycythaemia can cause symptoms of hyperviscosity and vaso-occlusive disease. We report a kindred with a high affinity haemoglobin (Haemoglobin Yakima) one of whose members gave birth to two infants with intra-uterine growth retardation and who suffered with symptoms of hyperviscosity which settled on reduction of the PCV by venesection.
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Two important haematological problems were found in an otherwise healthy 78 year old man: chronic myelomonocytic leukaemia; and a complex, acquired, hyperfibrinolytic bleeding disorder characterized by prolonged coagulation times, deficiency of coagulation factors V, X, and XI, anti-thrombin III and proteins C and S, with high concentrations of circulating tissue plasminogen activator, and low concentrations of plasminogen activator inhibitor. There may be a causal relation between the two conditions, with the peripheral blood monocytes mediating the hyperfibrinolytic process by the abnormal production of tissue plasminogen activator, though no previous description of a similar association has been reported.
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Bone marrow smears from 15 patients with multiple myeloma, 15 patients with monoclonal gammopathy of undetermined significance (MGUS), and 15 control subjects were examined for the presence of cytoplasmic 5'nucleotidase (c5NT) in plasma cells. Plasma cell positivity for c5NT (mean and 95% confidence interval) in patients with multiple myeloma numbered 46.4% (38.0-54.8%), in those with MGUS it was 15.3% (11.1-19.6%), and in control subjects it was 1.2% (0.3-2.1%). These findings indicate that c5NT can be used to differentiate benign from malignant monoclonal gammopathies.
Acquired immunodeficiency syndrome (AIDS)-related services offered by San Francisco General Hospital (SFGH); programs to protect employees who are at occupational risk for infection with human immunodeficiency virus (HIV); and the legal, ethical, and economic implications of such infection are discussed. Support from public officials, health-care professionals, and the community has enabled SFGH to develop an extensive program of AIDS-related services. The program consumes 10% of SFGH's budget and was responsible for treating 1693 patients between January 1981 and June 1988. The hospital has two internationally recognized units dedicated to the care of AIDS patients. Many of the medical and support departments at SFGH contribute expertise directly; clinical and basic research are also conducted. Other services sponsored by the hospital include HIV testing and counseling; special training for physicians and nurses; assisting patients with finances, housing, and home care; emotional support; and community education. Expenses per patient are lower at SFGH than nationally because of contributions from the private and public sectors, but costs are not fully recovered. Hospital employees are protected by a body substance precautions program, a comprehensive needle-stick program, mandatory reporting and evaluation of all exposures, and enrollment in a study to document seroconversions. Confidentiality and compensation remain major concerns. San Francisco General Hospital has a model program of caring for patients with AIDS and protecting its workers, but the issue of compensating those employees who do become infected with HIV has not yet been resolved.