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

11:28 EDT 17th April 2014 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 17,000+

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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.

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.

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...

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.

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...

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.

Transcatheter aortic valve replacement in patients with aortic stenosis and left ventricular dysfunction: patient selection counts more than ever!

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...

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.

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...

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...

Anesthetic management of a patient with Marfan syndrome and severe aortic root dilatation undergoing cholecystectomy and partial hepatic resection.

Due to high mortality associated with aortic dissection, anesthetic management of patients with Marfan syndrome with severe aortic root dilation is a challenging situation. We describe the anesthetic management of a patient with Marfan syndrome with severe aortic root dilation, who required major surgery like cholecystectomy with partial liver resection under general anesthesia. A 47-year-old female presented to pre-anesthetic clinic for cholecystectomy with partial hepatic resection for gall bladder carcin...

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...

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...

Surgical Treatment and Thoracic Endovascular Aortic Repair in Type A Aortic Aortic Dissection in a Pregnant Patient with Marfan Syndrome.

We report about an acute aortic dissection type Stanford A extending down to both iliac arteries affecting a 32 year old woman during the 37(th)+1 week of gravidity suspected to have Marfan syndrome. In a multidisciplinary approach emergent Cesarean section was performed followed by an abdominal hysterectomy and a valve sparing aortic root replacement in reimplantation technique. The aorta was replaced up to the hemi arch. Due to high suspicion of visceral ischemia as confirmed 'ex juvantibus' an endovascul...

Rapidly progressing mycotic aortic aneurysm masquerading as acute coronary syndrome.

Mycotic aortic aneurysms are rare. The most common cause of a mycotic aortic aneurysm is bacterial seeding in a diseased or injured aortic intima with subsequent arteritis. Because the clinical presentation of mycotic aortic aneurysms can be quite variable, the diagnosis hence can often be quite challenging. We herewith report an interesting case study in which the patient with a mycotic aortic aneurysm presented with the clinical picture masquerading as an acute coronary syndrome. The scenario reiterates t...

Surgical treatment of valvular and supravalvular aortic stenosis in homozygous familial hypercholesterolemia.

A 61-year-old male with homozygous familial hypercholesterolemia presented with dyspnea and syncope. He had been treated with low-density lipoprotein apheresis for 26 years. Echocardiography and computed tomography showed severe valvular and supravalvular aortic stenosis. Computed tomography and cardiac catheterization revealed a severely calcified narrowed aortic root and an occlusion in the proximal right coronary artery. During surgery, the ascending aorta was replaced under deep hypothermic circulatory...

Loeys-Dietz Syndrome.

Loeys-Dietz syndrome is an autosomal dominant aortic aneurysm syndrome characterized by multisystemic involvement. The most typical clinical triad includes hypertelorism, bifid uvula or cleft palate and aortic aneurysm with tortuosity. Natural history is significant for aortic dissection at smaller aortic diameter and arterial aneurysms throughout the arterial tree. The genetic cause is heterogeneous and includes mutations in genes encoding for components of the transforming growth factor beta (TGFβ) signa...

Functional mitral regurgitation in patients with aortic stenosis: prevalence, clinical correlates and pathophysiological determinants: a quantitative prospective study.

In patients with aortic stenosis (AS) functional mitral regurgitation (FMR) is frequent and is attributed to left ventricular (LV) remodelling and to aortic gradient. However, the association of these variables with mitral effective regurgitant orifice (ERO) is still unknown.

Anesthetic management of a patient with obstructive prosthetic aortic valve dysfunction: a case report.

We present a 55-year-old female patient who underwent burr-hole drainage due to chronic subdural hematoma, with obstructive prosthetic aortic valve dysfunction. Anesthetic management of a patient with severe obstructive prosthetic aortic valve dysfunction can be challenging. Similar considerations should be given to patients with aortic stenosis with an additional emphasis on thrombotic complication due to discontinuation of anticoagulation, which may further jeopardize the valve dysfunction. This case emph...

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