13 resultados para Withhold and withdraw medical treatment
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Background: Rett disorder (RD) is a progressive neurodevelopmental entity caused by mutations in the MECP2 gene. It has been postulated that there are alterations in the levels of certain neurotransmitters and folate in the pathogenesis of this disease. Here we re-evaluated this hypothesis. Patients and Methods: We evaluated CSF folate, biogenic amines and pterines in 25 RD patients. Treatment with oral folinic acid was started in those cases with low folate. Patients were clinically evaluated and videotaped up to 6 months after therapy. Results: CSF folate was below the reference values in 32% of the patients. Six months after treatment no clinical improvement was observed. Three of the four patients with the R294X mutation had increased levels of a dopamine metabolite associated to a particular phenotype. Three patients had low levels of a serotonin metabolite. Two of them were treated with fluoxetine and one showed clinical improvement. No association was observed between CSF folate and these metabolites, after adjusting for the patients age and neopterin levels. Conclusion: Our results support that folinic acid supplementation has no significant effects on the course of the disease. We report discrete and novel neurotransmitter abnormalities that may contribute to the pathogenesis of RD highlighting the need for further studies on CSF neurotransmitters in clinically and genetically well characterized patients.
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OBJECTIVES: To evaluate the short- and medium-term results of prostatic arterial embolisation (PAE) for benign prostatic hyperplasia (BPH). METHODS: This was a prospective non-randomised study including 255 patients diagnosed with BPH and moderate to severe lower urinary tract symptoms after failure of medical treatment for at least 6 months. The patients underwent PAE between March 2009 and April 2012. Technical success is when selective prostatic arterial embolisation is completed in at least one pelvic side. Clinical success was defined as improving symptoms and quality of life. Evaluation was performed before PAE and at 1, 3, 6 and every 6 months thereafter with the International Prostate Symptom Score (IPSS), quality of life (QoL), International Index of Erectile Function (IIEF), uroflowmetry, prostatic specific antigen (PSA) and volume. Non-spherical polyvinyl alcohol particles were used. RESULTS: PAE was technically successful in 250 patients (97.9 %). Mean follow-up, in 238 patients, was 10 months (range 1-36). Cumulative rates of clinical success were 81.9 %, 80.7 %, 77.9 %, 75.2 %, 72.0 %, 72.0 %, 72.0 % and 72.0 % at 1, 3, 6, 12, 18, 24, 30 and 36 months, respectively. There was one major complication. CONCLUSIONS: PAE is a procedure with good results for BPH patients with moderate to severe LUTS after failure of medical therapy. KEY POINTS: • Prostatic artery embolisation offers minimally invasive therapy for benign prostatic hyperplasia. • Prostatic artery embolisation is a challenging procedure because of vascular anatomical variations. • PAE is a promising new technique that has shown good results.
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OBJECTIVE/BACKGROUND: The association between socioeconomic status (SES), presentation, and outcome after vascular surgery is largely unknown. This study aimed to determine the influence of SES on post-operative survival and severity of disease at presentation among vascular surgery patients in the Dutch setting of equal access to and provision of care. METHODS: Patients undergoing surgical treatment for peripheral artery disease (PAD), abdominal aortic aneurysm (AAA), or carotid artery stenosis between January 2003 and December 2011 were retrospectively included. The association between SES, quantified by household income, disease severity at presentation, and survival was studied using logistic and Cox regression analysis adjusted for demographics, and medical and behavioral risk factors. RESULTS: A total of 1,178 patients were included. Low income was associated with worse post-operative survival in the PAD cohort (n = 324, hazard ratio 1.05, 95% confidence interval [CI] 1.00-1.10, per 5,000 Euro decrease) and the AAA cohort (n = 440, quadratic relation, p = .01). AAA patients in the lowest income quartile were more likely to present with a ruptured aneurysm (odds ratio [OR] 2.12, 95% CI 1.08-4.17). Lowest income quartile PAD patients presented more frequently with symptoms of critical limb ischemia, although no significant association could be established (OR 2.02, 95% CI 0.96-4.26). CONCLUSIONS: The increased health hazards observed in this study are caused by patient related factors rather than differences in medical care, considering the equality of care provided by the study setting. Although the exact mechanism driving the association between SES and worse outcome remains elusive, consideration of SES as a risk factor in pre-operative decision making and focus on treatment of known SES related behavioral and psychosocial risk factors may improve the outcome of patients with vascular disease.
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Diabetic macular oedema (DMO) is a leading cause of vision loss in the working-age population worldwide. Corticosteroid drugs have been demonstrated to inhibit the expression of both the vascular endothelial growth factor (VEGF) gene and other anti-inflammatory mediators, such as prostaglandins. Triamcinolone, fluocinolone and dexamethasone are the main steroids that have been studied for the treatment of macular oedema. Over the last few years, several studies have suggested an important role for dexamethasone in the management of DMO. The dexamethasone intravitreal implant (DEX implant) (Ozurdex®; Allergan, Inc., Irvine, CA) is a novel approach approved by the US Food and Drug Administration (FDA) and by the EU for the intravitreal treatment of macular oedema after branch or central retinal vein occlusion, and for the treatment of non-infectious uveitis affecting the posterior segment of the eye. We reviewed manuscripts that had investigated the pharmacokinetics, efficacy and safety of the DEX implant regarding DMO treatment.
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Cardiac hydatid cyst is a rare disease, especially in children. An 11-year-old boy with a previous anaphylactic reaction and episodes of abdominal pain was admitted for workup of an acquired long systolic murmur. Echocardiographic investigation disclosed a tumor of the right ventricular anterior wall, with multiple loculations. Magnetic resonance imaging characterized it as a multilobular tumor with cyst formation and disclosed another cyst in the right pulmonary artery. With a positive ELISA reaction the child was admitted for surgery with the diagnosis of cardiac and pulmonary hydatid cysts. Cardiac surgery was performed with good results, followed by medical treatment with albendazole.
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INTRODUCTION: Chylothorax is a rare but serious postoperative condition in children with congenital heart disease. Conventional medical treatment consists of specific long-term dietary modification, and surgical reintervention, such as lymphatic duct ligation, may be indicated in refractory cases. In recent years, an additional conservative treatment, octreotide, a synthetic analog of somatostatin, has been used in management of congenital and postoperative chylothorax. METHODS: The objective of this work was to analyze the efficacy and safety of this treatment for chylothorax after congenital heart surgery. We reviewed the records of sixteen patients with chylothorax after surgery for congenital heart disease between January 1999 and December 2007, and collected the following data: demographic information; type of surgical procedure; onset, duration and management of chylothorax and treatment; and duration of hospital stay. To analyze efficacy we compared these parameters in children receiving conventional treatment only with those receiving octreotide. To analyze safety we compared the adverse effects of both treatments. Octreotide was administered at a dose of 4 to 10 microg/kg/hour, with monitoring of side effects. RESULTS: The incidence of chylothorax in our population was 1.6%. It occurred more often after Glenn and Fontan procedures (8 patients). Octreotide was begun three days after diagnosis of chylothorax and continued for a median of seventeen days (ranging from 4 to 26 days), until complete resolution. Side effects were frequent (in 3 of the 8 patients) but of no clinical relevance. All patients responded to the therapy and there was no indication for further surgical intervention. DISCUSSION AND CONCLUSIONS: Octreotide is safe and effective in the treatment of postoperative chylothorax in children with congenital heart disease. It is a useful adjunctive therapy to the conventional treatment of this complication.
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This retrospective study was designed to evaluate the outcome of pregnancies in women diagnosed with systemic lupus erythematosus (SLE) followed in a tertiary fetal–maternal center. Data were collected from clinical charts between January 1993 and December 2007, with a total of 136 pregnancies (107 patients). Mean maternal age was 29 years, with the vast majority of patients being Caucasian. Most patients were in remission 6 months prior to pregnancy (93%) and the most frequently affected organs were the skin and joints. Renal lupus accounted for 14% of all cases. Twenty-nine percent of patients were positive for at least one antiphospholid antibody (aPL) and nearly 50% had positive SSa/SSb antibodies. All patients with positive aPL received low-dosage aspirin and low molecular- weight heparin (LMWH). There were no pregnancy complications in more than 50% of cases and hypertensive disease and intrauterine growth restriction were the most common adverse events. There were 125 live births, one neonatal death, eight miscarriages, and three medical terminations of pregnancy. Preterm delivery occurred in 25% of pregnancies. Our results are probably the conjoined result of a multidisciplinary approach together with a systematic management of SLE pregnancies, with most patients keeping their prior SLE medication combined with low-dosage aspirin and LMWH in the presence of aPL.
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Introduction:Women with antiphospholipid syndrome(APS) may suffer from recurrent miscarriage, fetal death, fetal growth restriction (FGR), pre-eclampsia, placental abruption, premature delivery and thrombosis. Treatment with aspirin and low molecular weight heparin (LMWH) combined with close maternal-fetal surveillance can change these outcomes. Objective: To assess maternal and perinatal outcome in a cohort of Portuguese women with primary APS. Patients and Methods: A retrospective analysis of 51 women with primary APS followed in our institution (January 1994 to December 2007). Forty one(80.4%) had past pregnancy morbidity and 35.3%(n=18) suffered previous thrombotic events. In their past they had a total of 116 pregnancies of which only 13.79 % resulted in live births. Forty four patients had positive anticardiolipin antibodies and 33 lupus anticoagulant. All women received treatment with low dose aspirin and LMWH. Results: There were a total of 67 gestations (66 single and one multiple). The live birth rate was 85.1%(57/67) with 10 pregnancy failures: seven in the first and second trimesters, one late fetal death and two medical terminations of pregnancy (one APS related). Mean (± SD) birth weight was 2837 ± 812 g and mean gestational age 37 ± 3.3 weeks. There were nine cases of FGR and 13 hypertensive complications(4 HELLP syndromes). 54.4% of the patients delivered by caesarean section. Conclusions: In our cohort, early treatment with aspirin and LMWH combined with close maternal-fetal surveillance was associated with a very high chance of a live newborn.
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PURPOSE: To evaluate whether prostatic arterial embolization (PAE) might be a feasible procedure to treat lower urinary tract symptoms associated with benign prostatic hyperplasia (BPH). MATERIALS AND METHODS: Fifteen patients (age range, 62-82 years; mean age, 74.1 y) with symptomatic BPH after failure of medical treatment were selected for PAE with nonspherical 200-μm polyvinyl alcohol particles. The procedure was performed by a single femoral approach. Technical success was considered when selective prostatic arterial catheterization and embolization was achieved on at least one pelvic side. RESULTS: PAE was technically successful in 14 of the 15 patients (93.3%). There was a mean follow-up of 7.9 months (range, 3-12 months). International Prostate Symptom Score decreased a mean of 6.5 points (P = .005), quality of life improved 1.14 points (P = .065), International Index of Erectile Function increased 1.7 points (P = .063), and peak urinary flow increased 3.85 mL/sec (P = .015). There was a mean prostate-specific antigen reduction of 2.27 ng/mL (P = .072) and a mean prostate volume decrease of 26.5 mL (P = .0001) by ultrasound and 28.9 mL (P = .008) by magnetic resonance imaging. There was one major complication (a 1.5-cm(2) ischemic area of the bladder wall) and four clinical failures (28.6%). CONCLUSIONS: In this small group of patients, PAE was a feasible procedure, with preliminary results and short-term follow-up suggesting good symptom control without sexual dysfunction in suitable candidates, associated with a reduction in prostate volume.
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INTRODUCTION: We describe our center's initial experience with alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy. The procedure, its indications, results and clinical outcomes will be addressed, as will its current position compared to surgical myectomy. OBJECTIVE: To assess the results of ASA in all patients treated in the first four years of activity at our center. METHODS: We retrospectively studied all consecutive and unselected patients treated by ASA between January 2009 and February 2013. RESULTS: In the first four years of experience 40 patients were treated in our center. In three patients (7.5%) the intervention was repeated. Procedural success was 84%. Minor complications occurred in 7.5%. Two patients received a permanent pacemaker for atrioventricular block (6% of those without previous pacemaker). The major complication rate was 5%. There were no in-hospital deaths; during clinical follow-up (22 ± 14 months) cardiovascular mortality was 2.5% and overall mortality was 5%. DISCUSSION AND CONCLUSION: The results presented reflect the initial experience of our center with ASA. The success rate was high and in line with published results, but with room to improve with better patient selection. ASA was shown to be safe, with a low complication rate and no procedure-related mortality. Our experience confirms ASA as a percutaneous alternative to myectomy for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy refractory to medical treatment.
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Nexplanon (Schering-Plough Limited/Merck Sharp & Dohme Limited (MSD)) is a long active reversible contraceptive method that provides effective contraception for 3 years. It consists of a single, flexible, rod-shaped implant, containing 68 mg etonogestrel. It is 4 cm long, consists of an ethylene vinyl acetate copolymer, a non-absorbable material, and also contains 15 mg of barium sulfate, which makes it visible by X-ray. We describe a case of a 39-year-old woman who experienced a local reaction to the barium sulfate in Nexplanon. She was given medical treatment, but only the removal of the implant resolved the symptoms. After removal there was gradual improvement and 72 h later the patient was asymptomatic. Allergic reaction to barium sulfate is extremely rare: until now, there have only been two cases associated with Nexplanon described in the literature.
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Adult B-cell acute lymphoblastic leukemia remains a major therapeutic challenge, requiring a better characterization of the molecular determinants underlying disease progression and resistance to treatment. Here, using a phospho-flow cytometry approach we show that adult diagnostic B-cell acute lymphoblastic leukemia specimens display PI3K/Akt pathway hyperactivation, irrespective of their BCR-ABL status and despite paradoxically high basal expression of PTEN, the major negative regulator of the pathway. Protein kinase CK2 is known to phosphorylate PTEN thereby driving PTEN protein stabilization and concomitant PTEN functional inactivation. In agreement, we found that adult B-cell acute lymphoblastic leukemia samples show significantly higher CK2 kinase activity and lower PTEN lipid phosphatase activity than healthy controls. Moreover, the clinical-grade CK2 inhibitor CX-4945 (Silmitasertib) reversed PTEN levels in leukemia cells to those observed in healthy controls, and promoted leukemia cell death without significantly affecting normal bone marrow cells. Our studies indicate that CK2-mediated PTEN posttranslational inactivation, associated with PI3K/Akt pathway hyperactivation, are a common event in adult B-cell acute lymphoblastic leukemia and suggest that CK2 inhibition may constitute a valid, novel therapeutic tool in this malignancy.
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We describe the case of a 22-year-old black female with type 1 diabetes mellitus diagnosed when she was 12 years old. She first presented (March 1994) with pustules and ulcerations on the upper and lower limbs, trunk and scalp at the age 17. The diagnosis of pyoderma gangrenosum was made. Since presentation, changes in liver function were detected and subsequent study led to the diagnosis of sclerosing cholangitis. The diagnosis of ulcerative colitis was made after colonoscopy. Partial response was obtained with minocycline and clofazimine, but treatment with 5-aminosalicylic acid achieved no improvement of the ulcerations. Liver transplantation, followed by immunosuppressive therapy led to complete regression of the cutaneous lesions.