1/4. Acquired factor viii autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.The development of an autoantibody to human factor viii is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold standard of treatment or widely accepted guidelines existing. This has prompted us to examine all cases of this condition presenting at Fremantle Hospital over the last decade. We describe four cases which demonstrate the heterogeneity of this condition and its treatment and review the recent literature on the subject.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/4. Late cardiovascular and pulmonary complications of therapy in Hodgkin's disease: report of three unusual cases, with a review of relevant literature.With the advent of modern therapeutic approaches, even patients with advanced Hodgkin's disease have high cure rates today. Therefore, more attention is gradually being focused upon the late complications of chemotherapy and irradiation, appearing long after the patient is in remission and thought to be cured. In this report, we review the incidence and presentation of some of the cardiovascular and pulmonary complications which may appear later in the course of the disease. Cardiovascular mishaps reviewed include pericardial manifestations, conduction abnormalities, cardiomyopathy, and premature coronary artery disease. Pulmonary complications discussed are lung fibrosis, spontaneous pneumothorax, pulmonary veno-occlusive disease, and hyperlucent lung. Three instructive cases from our recent experience, are also presented. One fatal case was due to cardiac failure because of radiation-induced pericarditis and coronary artery disease. Another patient with an almost fatal complication required lung transplantation because of severe bilateral radiation fibrosis of the lung and pulmonary veno-occlusive disease. The third instance was also life-threatening in nature, with radiation-induced arterial changes in the major arteries of the chest and neck, resulting in recurrent cerebral and ophthalmic thromboembolic disease. It is suggested that potentially severe cardiopulmonary complications be considered during the planning of the initial and subsequent management of patients with Hodgkin's disease, particularly in an era employing autologous and allogeneic bone marrow transplantation as part of therapy in some cases.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
3/4. In-stent restenosis and remote coronary lesion progression are coupled in cardiac transplant vasculopathy but not in native coronary artery disease.OBJECTIVES: The purpose of this study was to describe the clinical, angiographic, and histological features of concomitant in-stent restenosis (ISR) and cardiac allograft vasculopathy (CAV) progression. BACKGROUND: Cardiac allograft vasculopathy is a major challenge to long-term success of heart transplantation. Coronary stenting for CAV is hampered by ISR. methods: Quantitative coronary angiography compared late lumen loss (LL) at stented and reference, non-stented segments during 1-year follow-up in post-heart transplant and control atherosclerosis patients. Stented and non-stented arteries with CAV were also obtained post-mortem for immunohistochemical analysis. RESULTS: In 37 stented lesions (25 patients), 1-year binary restenosis occurred in 37.8%. patients with ISR had higher long-term cardiac death/myocardial infarction rates than patients without ISR (53.8% vs. 9.1%, p = 0.03). In the same 25 patients, 34 CAV lesions with non-significant obstructions were identified as reference controls. After 1 year, patients who developed ISR also had more control lesion LL (0.78 /- 0.38 mm vs. 0.39 /- 0.27 mm, p < 0.006) compared to patients without ISR. In the post-transplant patients, in-stent LL was closely coupled to control segment LL (R(2) = 0.63, p < 0.05). Conversely, in native atherosclerosis patients, ISR and remote disease progression were not correlated. Histological staining of stented and control arteries from CAV patients revealed similar pathologies common to ISR and non-intervened CAV segments. CONCLUSIONS: Progression of CAV at non-intervened segments and ISR correlate strongly and share common histopathology. Optimized treatment for patients with aggressive CAV needs to address the widespread nature of this disease, even when it presents as an initially focal lesion.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
4/4. Evaluation of a new method for cardiovascular reasoning.OBJECTIVE: Evaluate the accuracy of the detailed diagnostic reasoning of the heart failure Program incorporating a new mechanism to handle temporal relationships and severity constraints. DESIGN: Tools were developed to summarize diagnoses and automatically generate evaluation forms. Five expert cardiologists were asked to review the reasoning of the program, with two analyzing each case. Cases were gathered retrospectively for diversity and difficulty and 26 randomly selected cases were evaluated. The underlying issues were identified and classified. RESULTS: Both reviewers rated the first diagnosis correct in 25% of the cases and at least one rated it wrong in 10%. Analyzing the detailed reasoning, 137 issues were raised, about 5.3 per case. Of these, 53% were possible concerns raised by one reviewer. Of the 5.3 issues per case, 2.5 were attributable to controversies, misunderstandings, or mistakes; 1 was due to the overly simplistic representation of the summaries; and 1.8 were issues related to the program. CONCLUSION: overall, the program is capable of providing high-quality detailed diagnostic hypotheses for complex cardiovascular cases. The results highlight several issues: 1) the difficulty of effectively summarizing hypotheses, 2) the nature of a physician's causal explanation, and 3) some problems in evaluating detailed diagnostic reasoning. The mistakes the program made imply that some additional refinement is needed but that the reasoning mechanisms developed can support the appropriate reasoning. The appropriate next step is a prospective evaluation addressing the program's usefulness.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |