992 resultados para Síndrome de Prader-Willi
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This article contains the conclusions of the November 17-18, 2006 meeting of the Brazilian Study Group of Restless Legs Syndrome (GBE-SPI) about diagnosis and management of restless legs syndrome (RLS). RLS is characterized by abnormal sensations mostly but not exclusively in the legs which worsen in the evening and are improved by motion of the affected body part. Its diagnosis is solely based on clinical findings. Therapeutic agents with efficacy supported by Class I studies are dopamine agonists, levodopa and gabapentine. Class II studies support the use of slow release valproic acid, clonazepan and oxycodone. The GBE-SPI recommendations for management of SPI are sleep hygiene, withdrawal of medications capable of worsening the condition, treatment of comorbidities and pharmacological agents. The first choice agents are dopaminergic drugs, second choice are gabapentine or oxycodone, and the third choice are clonazepan or slow release valproic acid.
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Eisenmenger's syndrome consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular, or aortopulmonary level. Eisenmerger's syndrome in pregnancy is usually associated with high mortality rates (nearly 30-50%). Unfortunately, pulmonary hypertension is aggravated during pregnancy and often leads to an unfavorable outcome. Here, we report a successful pregnancy in a woman with Eisenmenger syndrome.
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Objective: There is strong evidence that methylene blue (MB), an inhibitor of guanylate cyclase, is an excellent therapeutic option for vasoplegic syndrome (VS) treatment in heart surgery. The aim of this article is to review the MB's therapeutic function in the vasoplegic syndrome treatment. Methods: Fifteen years of literature review. Results: 1) Heparin and ACE inhibitors are risk factors; 2) In the recommended doses it is safe (the lethal dose is 40 mg/ kg); 3) The use of MB does not cause endothelial dysfunction; 4) The MB effect appears in cases of nitric oxide (NO) up-regulation; 5) MB is not a vasoconstrictor, by blocking of the GMPc system it releases the AMPc system, facilitating the norepinephrine vasoconstrictor effect; 6) The most used dosage is 2 mg/kg as IV bolus followed by the same continuous infusion because plasmatic concentrations strongly decays in the first 40 minutes; 7) There is a possible window of opportunity for the MB's effectiveness. Conclusions: Although there are no definitive multicentric studies, the MB used to treat heart surgery VS, at the present time, is the best, safest and cheapest option, being a Brazilian contribution for the heart surgery.
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Introduction: The snoring is produced by the vibration of the soft palatum and/or other oropharyngeal tissues, during the air passage, in the breathing function. OSAHS is defined as the closing of 30%, at least, of the nasal/ buccal airway for 10 seconds or more, in spite of existing inspiratory effort, accompanied by oxyhemoglobin de- saturation of 4% or more. Objectives: To evaluate the available scientific evidence about the use of mandibular advancement intraoral appliances in the treatment of the snoring and/or OSAHS. Methodology: Electronic search strategy using predefined key-words and criteria was realized including studies published until October of 2008. It was also used the qualitative evaluation of the articles methodology. Results: Although a significant number of articles has studied this subject, only 7 articles showed methodological quality to be included in this systematic review. Conclusions: The intraoral appliances are widely prescribed for the treatment of snoring and OSAHS as primary therapy and as an alternative to patients who are unable to tolerate the positive airway pressure therapy.
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Objective: The objective of this study was evaluate if the embryos cryopreservation from OHSS patients Intracytoplasmic Sperm Injection (ICSI) cycles could be influence the clinical outcomes when compared to patients who receive oocytes from donors but the endometrium was not prepared and the embryos were cryopreserved. Methods: Fifty eight couples submitted to ICSI cycles in which 26 with OHSS clinical manifestation (OHSS group) and 32 couples who have received oocytes from donors (control group). The embryos were frozen on day+2 or +3of development. All patients included in this study had embryos crypreserved before the transfer, and in the thawing cycle, only the endometrium preparation was performed. The embryo survival, implantation, pregnancy and miscarriage rates were evaluated in the embryo thawing cycle. Results: There was no difference among the groups in relation to fertilization rate (OHSS: 71.89% ± 15.45, Control: 79.75% ± 21.68, p= 0.234), survival embryos rate (OHSS: 68.85 ± 21.10, Control: 59.53 ± 36.79, p= 0.233), high quality embryos rate (OHSS: 25.20 ± 23.90, Control: 27.40 ± 30.30, p= 0.760), implantation rate (OHSS: 17.9 ± 26.9, Control: 12.5 ± 23.7, p= 0.435), pregnancy rate (OHSS: 38.50, Control: 28.60, p= 0.441) and miscarriage rate (OHSS: 40.00, Control: 25.00, p= 0.332). Conclusion: Our findings suggest that clinical outcomes in freeze and thawing cycles were not affected by the presence of ovarian hyperstimulation syndrome clinical manifestation after controlled ovarian stimulation.
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Introduction: Obstructive sleep apnea syndrome is related to cardiopulmonary complications in children. It is important to know its patophysiology and possible complications to help reduce risks in this group. Aims: To report three cases of severe cardiorespiratory complications of obstructive sleep apnea managed in the intensive care unit (ICU). Case report: Two children with no previous diagnosis of obstructive sleep apnea syndrome suffered acute congestive heart failure and acute lung oedema with need of ICU and improved after adenotonsillectomy. In a third case, the patient had acute lung oedema as a complication after adenotonsillectomy. Conclusions: Paediatricians and otolaryngologists must be aware of the clinical manifestations of severe sleep apnea. Early referring to treatment and special attention at pre and post surgical periods are essentials to avoid serious complications.
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PURPOSE: To evaluate the importance of the oral glucose tolerance test for the diagnosis of glucose intolerance (GI) and type 2 diabetes mellitus (DM-2) in women with PCOS. METHODS: A retrospective study was conducted on 247 patients with PCOS selected at random. The diagnosis of GI was obtained from the two-hour oral glucose tolerance test with 75 g of glucose according to the criteria of the World Health Organization (WHO) (GI: 120 minutes for plasma glucose =140 mg/dL and <200 mg/dL), and the diagnosis of DM-2 was obtained by both the oral glucose tolerance test (DM: 120 minutes for plasma glucose =200 mg/dL) and fasting glucose using the criteria of the American Diabetes Association (impaired fasting glucose: fasting plasma glucose =100 and <126 mg/dL; DM: fasting glucose =126 mg/dL). A logistic regression model for repeated measures was applied to compare the oral glucose tolerance test with fasting plasma glucose. ANOVA followed by the Tukey test was used for the analysis of the clinical and biochemical characteristics of patients with and without GI and/or DM-2. A p<0.05 was considered statistically significant. RESULTS: PCOS patients had a mean age of 24.8±6.3, and body mass index (BMI) of 18.3 to 54.9 kg/m2 (32.5±7.6). The percentage of obese patients was 64%, the percentage of overweight patients was 18.6% and 17.4% had healthy weight. The oral glucose tolerance test identified 14 cases of DM-2 (5.7%), while fasting glucose detected only three cases (1.2%), and the frequency of these disorders was higher with increasing age and BMI. CONCLUSIONS: The results of this study demonstrate the superiority of the oral glucose tolerance test in relation to fasting glucose in diagnosing DM-2 in young women with PCOS and should be performed in these patients.
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Background: Fondaparinux is considered an agent with a well-established safety and efficacy profile in the treatment of non-ST segment elevation acute coronary syndromes, but when used alone, is associated to a higher incidence of thrombotic complications during invasive coronary procedures, requiring the supplementation of an anti-IIa agent. This study aimed to evaluate the efficacy and safety of percutaneous coronary intervention (PCI) in patients with non-ST segment elevation acute coronary syndromes previously treated with fondaparinux. Methods: Prospective, controlled registry enrolling 127 consecutive patients submitted to an early invasive stratification during treatment with fondaparinux, with supplementation of intravenous unfractionated heparin at a dose of 85 U/kg at the time of PCI. Results: The rate of the composite primary endpoint including death, acute myocardial infarction, stroke, stent thrombosis or emergency myocardial revascularization was 3.2%. The cumulative incidence of major bleeding and vascular complications was 3.2%. There were no cases of guidecatheter thrombosis or abrupt vessel closure. Conclusions: PCI in patients with acute coronary syndromes receiving fondaparinux is associated with a low rate of major adverse cardiovascular ischemic events and severe hemorrhagic complications. Supplementation of unfractionated heparin during the invasive procedures eliminates the risk of catheter-related thrombosis.
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The patellofemoral pain syndrome (PFPS) is defined as a retropatellar or anterior knee pain, without another disease. It affects until 25% of the population, being more common in women and trained persons. As others pathologies, PFPS have been affected the training of elite and amateurs athletes. Thereby, the general purpose of this study was discuss the occurrence of PFPS as a sports injury, there prevention possibilities and the appropriate recovery training after injury. It had been developed a literature review addressing the specific characteristics of the syndrome, its diagnosis, its target population, its development, how it affects the training and which are their possibilities of prevention and treatment. © FTCD/FIP-MOC.
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Objective: To report the case of a child diagnosed with antiphospholipid syndrome associated with severe thrombocytopenia, and to review the literature on the subject. Case description: Child aged nine years and eight months old with severe thrombocytopenia associated with a positive anticardiolipin antibody. Data were collected by clinical history, physical examination, and laboratorial exams. Diagnosis was confirmed according to criteria established for the antiophospholipid syndrome, associated with the presence of the most common manifestations of the syndrome in children: livedo reticularis and thrombocytopenia. Comments: The antiphospholipid syndrome is an uncommon pediatric disease, and clinical manifestations such as decreased platelet number should be considered.
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PURPOSE: To assess the occurrence of metabolic syndrome (MetS) in postmenopausal breast cancer survivors. METHODS: A total of 158 breast cancer survivors were included in this cross-sectional study. Eligibility criteria were: women with amenorrhea >12 months and age ≥45 years, treated for breast cancer and no metastasis for at least five years. Clinical history and anthropometric indicator data (body mass index (BMI), and waist circumference, (WC) were collected. Biochemical parameters, including total cholesterol, HDL, LDL, triglycerides (TG), glucose and C-reactive protein (CRP), were measured. MetS was diagnosed as the presence of at least three of the following diagnostic criteria: WC>88 cm, blood pressure≥130/85 mmHg, triglycerides≥150 mg/dl, HDL <50 mg/dL,and glucose≥100 mg/dL. The Student's t-test and χ 2 test were used for statistical analysis. RESULTS: The mean age of breast cancer survivors was 63.1±8.6 years, with a mean follow-up of 9.1±4.0 years. MetS was diagnosed in 48.1% (76/158) and the most prevalent diagnostic criterion was abdominal obesity (WC>88 cm), affecting 54.4% (86/158) of the women. The patients without MetS had a longer follow-up compared those with MetS (p<0.05). Regarding the current BMI, PN average, those without MetS were overweight, and those with MetS were obese (p<0.05). Among the latter, comparison of BMI at the time of cancer diagnosis and current BMI (27.8±5.4 versus 33.4±5.4 kg/m2) showed a significant weight gain (p<0.05). Mean CRP values were higher in women with MetS (p<0.05). In the comparison of tumor characteristics and cancer treatments there was no difference between groups (p>0.05). CONCLUSION: Postmenopausal breast cancer survivors had a higher risk of developing metabolic syndrome and central obesity.
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