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PubMed Journals Articles About "Aortic Stenosis Patient With Hurler Syndrome After Bone" RSS

23:11 EDT 22nd July 2014 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 24,000+

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Transcatheter Aortic Valve Replacement Options for Severe Aortic Stenosis in High-Risk Patients.

Calcific aortic stenosis has now become the most common valvular disease in Western countries. It is a disease of the old and very old. Senile calcific aortic stenosis affects 5% of the population 70 years or older in the United States. Traditionally, the gold standard for treating aortic stenosis has been surgical aortic valve replacement. Unfortunately, surgical aortic valve replacement is, in many cases, not a viable option for this patient population.

Transcatheter aortic valve implantation in a 54-year-old patient with aggressive HIV.

We report a case of a 54-year-old patient who was denied surgical replacement for severe aortic stenosis because of complicated acquired immunodeficiency syndrome and who successfully underwent transcatheter aortic valve implantation at our institution.

A patient with Marfan's syndrome presented with severe rheumatic mitral stenosis and successfully treated with percutaneous transmitral balloon commissurotomy - Report of first case.

Marfan's syndrome (MFS) is an inherited autosomal dominant disorder of connective tissue with mutation on the fibrillin-1 gene encoding for fibrillin. This frequently involves the cardiovascular system with prevalence is 1:5000-10,000. The clinical major criteria involve the skeletal and ocular apparatus and the cardiovascular and central nervous system. In Marfan's syndrome mitral valve prolapse and aortic dilation are the main cardiovascular manifestations. According to the literature database the prevale...

Combined severe valvular aortic and valvular pulmonary stenosis and its management - A case report and literature review.

Isolated congenital valvular stenosis of either aortic or pulmonary valve is commonly seen yet the presence of both these lesions in the same patient is rare. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves. We present here a case report of a three and half year old boy diagnosed as a combined congenital severe valvular aortic stenosis with valvular pulmonary stenos...

Delayed aortic dissection after transcatheter aortic valve implantation.

In selected high-risk patients with aortic stenosis, transcatheter aortic valve implantation (TAVI) can provide comparable functional improvement and early survival after surgical aortic valve replacement. However, the long-term outcome after TAVI is still to be determined and the occurrence of aortic dissection has not been systematically reported. Herein, a case is presented of delayed aortic dissection and rupture several months after an uneventful TAVI in a patient with bicuspid aortic valve stenosis.

Doppler-Detected Valve Movement in Aortic Stenosis: A Predictor of Adverse Outcome.

The absence of auscultatory aortic valve closure sound is associated with severe aortic stenosis. The absence of Doppler-derived aortic opening (Aop ) or closing (Acl ) may be a sign of advanced severe aortic stenosis.

Successful transcatheter aortic valve implantation in a Hodgkin lymphoma patient with severe aortic stenosis.

Transcatheter aortic valve implantation (TAVI) has emerged as an acceptable treatment modality for patients with severe aortic stenosis (AS) who are deemed unsuitable for conventional surgical aortic valve replacement. TAVI not only provides the treatment of AS, but also makes some other diseases treatable by relieving hemodynamic distress resulting from AS. In this case report, we presented a 74-year-old patient with Hodgkin's lymphoma (HL) that had been left untreated due to the development of acute pulmo...

Endovascular Treatment for Primary Aortic Angiosarcoma to Relieve Thoracic Aortic Stenosis.

Aortic aneurysms and stenosis are widely treated with endovascular procedures in the current era. In this report, we present endovascular stent grafting for symptomatic treatment of severe thoracic aortic stenosis caused by an aortic sarcomatoid carcinoma in a 73-year-old female. The peri- and post-operative courses were complicated with tumor embolisms that were managed both endovascular and surgical measures.

The impact of the metabolic syndrome on the outcome after aortic valve replacement.

The aim of this study was to examine the influence of the metabolic syndrome on the left ventricular geometry as well as on the early and mid-time outcome in patients with aortic stenosis who underwent aortic valve replacement.

Percutaneous balloon aortic valvuloplasty in different age groups.

Aortic stenosis is a congenital or acquired reduction in the area of the aortic valve, resulting in obstruction of the blood flow from the left ventricle to the aorta. Aortic stenosis accounts for 2-5% of all congenital heart defects and is a potentially life-threatening disorder. In adults aortic stenosis represents 34% of all valvular heart diseases. Degenerative etiology is present in 80% of cases. Patients with mild aortic stenosis are usually asymptomatic. Symptoms of the disease occur along with the d...

Effect of recombinant human growth hormone on changes in height, bone mineral density, and body composition over 1-2years in children with Hurler or Hunter syndrome.

Patients with Hurler or Hunter syndrome typically have moderate to severe growth deficiencies despite therapy with allogeneic hematopoietic stem cell transplantation and/or enzyme replacement therapy. It is unknown whether treatment with recombinant human growth hormone (hGH) can improve growth in these children. The objectives of this study were to determine the effects of hGH on growth, bone mineral density (BMD), and body composition in children with Hurler or Hunter syndrome enrolled in a longitudinal o...

Postcardiotomy mechanical circulatory support in two infants with williams' syndrome.

Supravalvar aortic stenosis (SVAS) in patients with Williams' syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use...

Treatment of aortic stenosis with a self-expanding transcatheter valve: the International Multi-centre ADVANCE Study.

Transcatheter aortic valve implantation has become an alternative to surgery in higher risk patients with symptomatic aortic stenosis. The aim of the ADVANCE study was to evaluate outcomes following implantation of a self-expanding transcatheter aortic valve system in a fully monitored, multi-centre 'real-world' patient population in highly experienced centres.

97 Value of Cardiopulmonary Exercise and Non-invasive Haemodynamic assessment during Exercise in Patients with Asymptomatic Severe Aortic Stenosis.

Timing of aortic valve replacement in patients with severe asymptomatic aortic stenosis (VD) is controversial. Exercise testing may uncover symptoms and early cardiac dysfunction. However there is little information on the value of cardiopulmonary exercise (CPX) testing combined with haemodynamic assessment during exercise in this patient group. We report preliminary results of CPX and haemodynamic data in this patient cohort.

18F-FDG Uptake and Calcifications on Positron Emission Tomography/Computed Tomography in octogenarians with severe aortic stenosis.

The degree of inflammation within the atherosclerotic plaque can be detect non--invasively by positron emission tomography (PET) with 18F--fluorodeoxyglucose (18F--FDG). The incidence of aortic plaques with 18F--FDG increased uptake in octogenarians with aortic stenosis is unknown. Aim of this study was to evaluate the frequency of inflamed aortic atherosclerotic plaques in octogenarians with or without severe aortic stenosis and their correlations with calcifications.

Acute coronary syndrome as a result of left main coronary artery stenosis after aortic valve replacement. A report of three cases.

Acute coronary syndrome (ACS) as a result of iatrogenic coronary ostial stenosis (ICOS) is a rare but potentially life-threatening complication of aortic valve replacement (AVR). We present three cases of patients with ACS shortly after AVR, in whom ICOS were revealed. They refused an operation and thus they were treated with percutaneous coronary intervention. The potential pathomechanisms of ICOS and treatment options are discussed.

Aortic aneurysm and craniosynostosis in a family with Cantu syndrome.

Cantu syndrome is an autosomal dominant overgrowth syndrome associated with facial dysmorphism, congenital hypertrichosis, and cardiomegaly. Some affected individuals show bone undermodeling of variable severity. Recent investigations revealed that the disorder is caused by a mutation in ABCC9, encoding a regulatory SUR2 subunit of an ATP-sensitive potassium channel mainly expressed in cardiac and skeletal muscle as well as vascular smooth muscle. We report here on a Japanese family with this syndrome. An a...

The aortic valve: structure, complications and implications for transcatheter aortic valve replacement.

The aortic valve operates in a complex haemodynamic environment, opening and closing over 100,000 times a day. When complications arise, such as aortic stenosis, prognosis can be very poor, leading to death within the first few years. Surgical valve replacement is currently the standard treatment for aortic stenosis. A thorough understanding of the anatomy and function of the native valve is imperative when developing a prosthetic replacement that can withstand the complex demands of the heart. This review...

Similar morphology, but different function: acute improvement of myocardial longitudinal strain after percutaneous transcatheter aortic valve implantation therapy in a severe aortic stenosis patient.

Anatomical factors associated with left innominate vein stenosis in hemodialysis patients.

Central venous stenosis remains a challenge in hemodialysis patients. Venograms have shown that left innominate vein (LIV) stenosis often occurs in front of the trachea, where it crosses the aortic arch, suggesting that there may be an anatomical factor involved, such as iliac vein compression syndrome. The goal of this study was to determine whether LIV stenosis can be attributed to compression. From September 2008 to December 2011, 19 hemodialysis patients (ten women, nine men) with symptomatic venous hyp...

Novel SMAD3 Mutation in a Patient with Hypoplastic Left Heart Syndrome with Significant Aortic Aneurysm.

Aneurysms-osteoarthritis syndrome (AOS) caused by haploinsufficiency of SMAD3 is a recently described cause of syndromic familial thoracic aortic aneurysm and dissection (TAAD). We identified a novel SMAD3 mutation in a patient with hypoplastic left heart syndrome (HLHS) who developed progressive aortic aneurysm requiring surgical replacement of the neoaortic root, ascending aorta, and proximal aortic arch. Family screening for the mutation revealed that his father, who has vascular and skeletal features of...

Prevalence and factors associated with false positive suspicion of acute aortic syndrome: experience in a patient population transferred to a specialized aortic treatment center.

Acute aortic syndrome (AAS) is a medical emergency that requires prompt diagnosis and treatment at specialized centers. We sought to determine the frequency and etiology of false positive activation of a regional AAS network in a patient population emergently transferred for suspected AAS.

Expression of uPA, tPA, and PAI-1 in Calcified Aortic Valves.

Purpose. Our physiopathological assumption is that u-PA, t-PA, and PAI-1 are released by calcified aortic valves and play a role in the calcification of these valves. Methods. Sixty-five calcified aortic valves were collected from patients suffering from aortic stenosis. Each valve was incubated for 24 hours in culture medium. The supernatants were used to measure u-PA, t-PA, and PAI-1 concentrations; the valve calcification was evaluated using biphotonic absorptiometry. Results. Aortic stenosis valves expr...

High-sensitivity troponin I concentrations are a marker of an advanced hypertrophic response and adverse outcomes in patients with aortic stenosis.

High-sensitivity cardiac troponin I (cTnI) assays hold promise in detecting the transition from hypertrophy to heart failure in aortic stenosis. We sought to investigate the mechanism for troponin release in patients with aortic stenosis and whether plasma cTnI concentrations are associated with long-term outcome.

Symptomatic Obstruction of the Brachiocephalic and Left Subclavian Arteries Obscured by Aortic Stenosis.

Stenosis or occlusion of the brachiocephalic artery represents an uncommon cause of cerebrovascular insufficiency. We report a patient with combined brachiocephalic and left subclavian obstruction with clinical manifestations of lightheadedness, syncope, and left-sided weakness who remained misdiagnosed essentially because of symmetrical pressures in the upper extremities. Aortic valve replacement for aortic stenosis failed to provide symptomatic relief. Eventual stenting of the brachiocephalic trunk resolv...


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