Impending paradoxical embolism: echocardiographic diagnosis of an intracardiac thrombus crossing a patent foramen ovale.
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Biomedical subjects
Publications and source records attributed to M Barnett.
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The impact of plasma exchange (PE) on the treatment of severe Guillain-Barré syndrome (GBS) was studied by comparing all the 16 patients treated with PE in a London teaching hospital between January 1985 and August 1987, with 64 historical controls drawn from a series of patients observed during a prospective study in South East England in 1983 and 1984. There were no GBS-related deaths in the PE treated group but seven in the historical controls (2P = 0.39). The median duration of ventilation was only 20 days (range 7-64) in the PE group compared with 36 days (range 14-365) in the surviving patients in the control group (2P = 0.06, 95% confidence interval of difference in medians -36 to 0 days). The PE group walked earlier without aid (median 55.5 compared with 86 days, 2P = 0.04, 95% confidence interval of difference in medians -88 to -2 days). Three months after the onset of neuropathy all PE treated patients were able to walk with support or better, whereas 19 of the surviving historical controls were unable to walk even with support (2P = 0.009). The costs of PE were offset by savings in intensive care unit and hospital expenditure.
Guillain-Barré syndrome is a predominantly motor polyradiculoneuropathy in which neurological deficit reaches a peak within 1 month of onset. Seventy per cent of patients recover within 1 year, but 10% die and 20% have residual disability. Plasma exchange is advocated for severe cases early in the course of the disease but the role of steroids has yet to be determined.
One hundred fifty-eight hemophilia A, B, and von Willebrand disease (VWD) patients treated with clotting factor concentrates from a single manufacturer were tested for antibody to the human T-lymphotropic virus type III (HTLV-III). Antibody was detected in 63% and 40% of those with severe hemophilia A and B, respectively, 12% and 0% of those with mild hemophilia A and B, and two patients with recessive VWD. Forty-two antibody-positive and 20 antibody-negative patients were studied for clinical and laboratory features of infection. Eleven seropositive patients had clinical signs of infection including Pneumocystis carinii pneumonia, lymphadenopathy, splenomegaly or diarrhea; however, only one patient had developed acquired immune deficiency syndrome (AIDS), and only two had significant impairment of their performance status. Thirty-one patients remained totally asymptomatic. Eight patients had a history suggestive of acute HTLV-III infection. Thrombocytopenia was observed in 18% of seropositive patients, lymphopenia in 60%, depressed T-helper cells in 43%, reduced T-helper:T-suppressor ratios (TH:TS) in 33%, and elevated platelet-bound immunoglobulin in 53%. The antibody-negative group had normal T-helper cell levels (except one patient) and TH:TS ratios, and normal platelet immunoglobulin levels. Both groups demonstrated a significant elevation of immunoglobulin levels and a high prevalence of antinuclear factor and antismooth muscle antibodies. The mean level of IgG was significantly higher in the antibody-positive group. This study confirms the correlation between HTLV-III infection and reduced T-helper cells in hemophiliacs but demonstrates a low incidence of clinical symptomatology. There was evidence of polyclonal B-cell hyperactivity in the antibody-negative group as well as the seropositive group.
The Philadelphia (Ph1) chromosome, the cytogenetic hallmark of chronic myeloid leukemia (CML), has also been detected in a significant number of acute lymphoblastic leukemias (ALL). Using in situ hybridization, we demonstrate that in accordance with observations in CML the Ph1 chromosome in ALL patients is the result of a consistent translocation of the c-abl oncogene to the Ph1 chromosome. Southern blot analysis using bcr probes, however, suggests that Ph1-positive ALL includes heterogeneous leukemic subtypes: six ALL patients showed bcr rearrangements as observed in CML; in three other patients recombination involving 5' bcr sequences could be demonstrated, but the corresponding translocated 3' bcr sequences were not detectable. A third group of five patients did not show any bcr rearrangements at all. Northern blot analysis using RNA from three Ph1-positive ALL patients revealed that in the leukemic cells of two patients larger c-abl mRNA transcripts were present, as in CML. In the RNA of one patient without a detectable bcr rearrangement, only the normal c-abl mRNA transcripts are present. The observed heterogeneity in bcr rearrangements of this group of Ph1-positive ALL patients is in contrast with the consistent results obtained in more than 50 Ph1-positive CML patients investigated in chronic and acute states.
Eight renal transplant recipients with neurogenic bladders or lower urinary tract dysfunction were managed with clean intermittent self-catheterization after transplantation instead of urinary diversion. A total of 85 treatment months was reviewed. Of the patients 5 continue to do well after 10 to 17 months of intermittent catheterization and 3 suffered immunological graft failures. In selected renal transplant recipients with lower urinary tract dysfunction clean intermittent catheterization is a reasonable alternative to urinary diversion.
The thoroughness of intraoperative evaluation of the extent of disease in 291 women with primary ovarian cancer was investigated. Notable differences among physician specialties but not types of hospitals where initial surgery was performed were observed. A review of medical record documentation revealed that 97% of the cases operated on by gynecologic oncologists had complete staging evaluations performed intraoperatively, but only 52 and 35% of cases operated on by obstetricians/gynecologists and general surgeons, respectfully, were adequately evaluated. Roughly one-half of the cases diagnosed in community hospitals and in hospitals with teaching affiliations were found to be completely studied, and 66% of those operated on in university hospitals received complete intraoperative evaluations.
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During an ongoing survey of 161 patients with ankylosing spondylitis (AS), 25 females were identified and retrospectively studied in detail. For each, a case-control male subject with similar age of onset of illness (mean, 23.0 years for women, 22.2 years for men) and duration of followup (mean, 20.7 years for women, 17.0 years for men) was selected. The diagnosis of AS was delayed an average of 12.8 years for females and 10.3 years for males. Extraspinal arthropathy occurred in 18 women (72%) and 8 men (32%) (p less than 0.05). Cervical spine disease and shoulder arthritis each occurred in 10 women (40%) and 4 men (16%) (NS). Hip disease affected 10 females and 6 males (NS). Peripheral joint disease occurred in 12 women (48%) and 9 men (36%); the knees were most commonly involved. Recurrent uveitis affected 10 females and 1 male (p less than 0.01). Anemia was detected in 8 women and 2 men (p less than 0.05). Sixty percent of the women had relatives with spondylarthritis, compared to 35% of the men (p less than 0.05). This last finding suggests that, in addition to possible hormonal and anatomic influences on the expression of disease, there may be some sex-linked, genetic factors.
23 descendents of a 74--year-old Englishman who had beta-thalassaemia trait, and died of hepatoma, were studied to discover whether thalassaemia minor alone could predispose to iron overload. Serum ferritin and HLA antigens were assessed in all members, and adults underwent radioiron investigations and liver biopsy. 2 members of the second generation and 1 of the third generation, all of whom had thalassaemia trait, had elevated liver iron concentration, indicating preclinical iron overload. This was not associated with any HLA type. None of the subjects had been treated with exogenous iron. The one member of the second generation who had thalassaemia minor but not iron overload was female, and the 5 members of the third generation with the trait, but with normal serum ferritin levels, were all under the age of 15 years. Members of the family without beta-thalassaemia minor had normal iron metabolism. It is possible that the development of iron overload in 4 members of this family was related to the presence of thalassaemia minor, and not to the inheritance of another abnormal gene causing idiopathic haemochromatosis.
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Iron overload was found in 3 patients who had undergone partial gastrectomy: a 61-year-old woman developed iron overload because she may have had idiopathic haemochromatosis and had also been given parenteral iron; in a 62-year-old man with thalassaemia minor, iron overload may have developed because of increased oral iron ingestion, low serum folate, increased, albeit ineffective, erythropoiesis and sideroblastic anaemia; a 74-year-old man with thalassaemia minor developed iron overload without exogenous therapy and died from a hepatoma. These cases illustrate that partial gastrectomy fails to protect patients from developing iron overload, particularly if given uncontrolled iron therapy.
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