5 resultados para Metapragmatic awareness
Resumo:
INTRODUCTION: Clopidogrel is frequently associated with thrombotic thrombocytopenic purpura, however this drug is rarely related to severe isolated thrombocytopenia. Pentoxifylline has previously been associated with thrombocytopenia only once. To the best of our knowledge, this is the first report of severe isolated thrombocytopenia after therapy with both clopidogrel and pentoxyfilline. CASE PRESENTATION: We report the case of a 79-year-old Caucasian man who presented to our facility with intermittent claudication. He had obliterative arterial disease and started therapy with clopidogrel and pentoxifylline. His basal platelet count was 194 × 109 cells/L. At three days after the start of treatment, our patient had lower limb petechia and stopped taking clopidogrel and pentoxifylline. His platelet count lowered to 4 × 109 cells/L and our patient was admitted to hospital. Our patient had purpura with no other hemorrhages or splenomegaly. Results of a blood smear were normal, and a bone marrow study showed dysmegakaryopoiesis. Antiplatelet antibody test results were negative, as were all viral serology tests. Imaging study results were normal. Our patient was given immunoglobulin but there was no sustained platelet increase, so corticotherapy was started as the next treatment step. At five months after clopidogrel and pentoxifylline were discontinued, his platelet count continued increasing even after prednisolone was tapered. CONCLUSIONS: Severe isolated thrombocytopenia may appear as a side effect when using clopidogrel and pentoxifylline. These drugs are widely used by general physicians, internists, cardiologists and vascular surgeons. We hope this report will raise awareness of the need to monitor the platelet count in patients taking these drugs.
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INTRODUCTION: Synovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma. CASE PRESENTATION: We describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented. CONCLUSION: Awareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.
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Neurologic disease is believed to be an unusual complication during the course of chronic lymphocytic leukemia. Nevertheless, it has already been proven in autopsy series that the incidence of occult nervous system infiltration is much higher than was previously expected. The advent of more potent drugs to treat this lymphoproliferative disorder has brought a new hope for a possible cure in the future. However, an appropriate systemic treatment for central nervous system infiltration of this disease is still lacking. Also, due to the potent immunosuppressive properties of the agents used in the up-front treatment, for example, the purine nucleoside analogues, we have witnessed an increase in the incidence of opportunistic infections, with progressive multifocal leukoencephalopathy being one of the most serious. The goal of this review is to summarize the spectrum of neurologic derangements linked to chronic lymphocytic leukemia and to raise clinicians’ awareness to recognize the possibility of such associations.
Resumo:
Hereditary neuropathy with liability to pressure palsy (HNPP) results from the deletion of the PMP22 gene in chromosome 17p11.2. Clinically, it presents with painless pressure palsies, typically in the 2nd and 3rd decades of life, being a rare entity in childhood. We present the case study of a six-year-old male child who presented with left hand drop that he kept for over four weeks. Electrophysiological studies suggested HNPP and genetic studies confirmed it. With this paper, we pretend to create awareness to this entity as a diagnosis to be considered in a child with painless monoparesis and to emphasize the importance of electrophysiological studies in the diagnosis.
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OBJECTIVE: A familial predisposition to abdominal aortic aneurysms (AAAs) is present in approximately one-fifth of patients. Nevertheless, the clinical implications of a positive family history are not known. We investigated the risk of aneurysm-related complications after endovascular aneurysm repair (EVAR) for patients with and without a positive family history of AAA. METHODS: Patients treated with EVAR for intact AAAs in the Erasmus University Medical Center between 2000 and 2012 were included in the study. Family history was obtained by written questionnaire. Familial AAA (fAAA) was defined as patients having at least one first-degree relative affected with aortic aneurysm. The remaining patients were considered sporadic AAA. Cardiovascular risk factors, aneurysm morphology (aneurysm neck, aneurysm sac, and iliac measurements), and follow-up were obtained prospectively. The primary end point was complications after EVAR, a composite of endoleaks, need for secondary interventions, aneurysm sac growth, acute limb ischemia, and postimplantation rupture. Secondary end points were specific components of the primary end point (presence of endoleak, need for secondary intervention, and aneurysm sac growth), aneurysm neck growth, and overall survival. Kaplan-Meier estimates for the primary end point were calculated and compared using log-rank (Mantel-Cox) test of equality. A Cox-regression model was used to calculate the independent risk of complications associated with fAAA. RESULTS: A total of 255 patients were included in the study (88.6% men; age 72 ± 7 years, median follow-up 3.3 years; interquartile range, 2.2-6.1). A total of 51 patients (20.0%) were classified as fAAA. Patients with fAAA were younger (69 vs 72 years; P = .015) and were less likely to have ever smoked (58.8% vs 73.5%; P = .039). Preoperative aneurysm morphology was similar in both groups. Patients with fAAA had significantly more complications after EVAR (35.3% vs 19.1%; P = .013), with a twofold increased risk (adjusted hazard ratio, 2.1; 95% confidence interval, 1.2-3.7). Secondary interventions (39.2% vs 20.1%; P = .004) and aneurysm sac growth (20.8% vs 9.5%; P = .030) were the most important elements accounting for the difference. Furthermore, a trend toward more type I endoleaks during follow-up was observed (15.6% vs 7.4%; P = .063) and no difference in overall survival. CONCLUSIONS: The current study shows that patients with a familial form of AAA develop more aneurysm-related complications after EVAR, despite similar AAA morphology at baseline. These findings suggest that patients with fAAA form a specific subpopulation and create awareness for a possible increase in the risk of complications after EVAR.