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Aortic Stenosis Patient With Hurler Syndrome After Bone PubMed articles on BioPortfolio. Our PubMed references draw on over 21 million records from the medical literature. Here you can see the latest Aortic Stenosis Patient With Hurler Syndrome After Bone articles that have been published worldwide.
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Hurler syndrome is the most severe form of mucopolysaccharidosis (MPS) Type 1. Progressive neurocognitive decline in this condition can be accompanied by macrocephaly, ventriculomegaly, and/or periventricular signal changes on MRI, which often leads to a neurosurgical referral. In this case, the authors describe a 2-year-old boy with ventriculomegaly and periventricular T2 signal changes, both of which decreased following medical management of Hurler syndrome. The authors discuss the possible mechanisms for...
Transcatheter aortic valve implantation (TAVI) has shown favorable outcomes in patients with severe symptomatic aortic valve stenosis who are at high surgical risk or inappropriate for open heart surgery. However, concerns exist over treating patients who have porcelain aorta and familial hypercholesterolemia, due to the potential complications of aortic root and aortic annulus. In this case report, we present a patient with familial hypercholesterolemia, symptomatic severe aortic stenosis, previous coronar...
One common complication of mucopolysaccharidosis I-Hurler (MPS1-H) is corneal clouding, which occurs despite current treatments, including bone marrow transplantation. Human corneas were obtained from a 14 year old subject with MPS1-H and visual disability from progressive corneal clouding despite prior bone marrow transplant at age 2. This was compared to a cornea from 17 year old donated to our eye bank after his accidental death. The corneas were analyzed microscopically after staining with Alcian blue, ...
In approximately one third of patients presenting with suspected severe aortic stenosis, there is a discrepancy between a severely reduced aortic valve opening area (
Congenital bicuspid aortic valve (CBAV) is the main cause of aortic stenosis (AS) in young adults. However, the histopathological features of AS in patients with CBAV have not been fully investigated.
The role of frailty as a prognostic factor in non-selected patients with symptomatic severe aortic stenosis (SAS) is still uncertain. This study aims to examine the association between the frailty syndrome and mortality among very old patients with symptomatic SAS, and to assess whether the association varies with the type of SAS treatment.
An important proportion of patients with aortic stenosis (AS) have a 'low-gradient' AS, i.e. a small aortic valve area (AVA
Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates o...
This study sought to assess the survival benefit associated with aortic valve replacement (AVR) according to different strata of echocardiographic parameters of aortic stenosis (AS) severity, and especially in patients with an aortic valve area (AVA) comprised between 0.8 cm(2) and 1 cm(2).
As the incidence of calcific aortic valve stenosis increases with the aging of the population, improved understanding and novel therapies to reduce its progression and need for aortic valve replacement are urgently needed.
Obstructive sleep apnea syndrome (OSAS) is a risk factor for resistant arterial hypertension and aortic dilatation. We assessed the value of systematic screening for OSAS in patients soon after the onset of acute aortic syndrome (AAS).
In recent years, the use of transcatheter aortic valve replacement (TAVR) has extended beyond the treatment of native aortic valve stenosis in patients with high surgical risk. TAVR is increasingly being performed for bioprosthetic aortic valve failure, i.e., the valve-in-valve (VIV) procedure. Establishing the success of a VIV procedure can be challenging in these cases. Furthermore, the limited availability of prostheses sizes further complicates the management of these patients. We present an unusual cas...
We present a case of a 57-year-old woman patient with a history of mid-aortic syndrome, treated with several vascular procedures, who was referred for investigation of metastatic disease of an undifferentiated sarcoma of unknown origin.
We sought to determine the relationship between aortic valve morphology and left ventricular (LV) systolic function in children with aortic stenosis (AS) prior to balloon aortic valvuloplasty (BAV).
ranscatheter aortic valve implantation is now accepted as a standard mode of treatment for an increasingly large population of patients with severe aortic stenosis. With the availability of this technique, echocardiographers need to be familiar with the imaging characteristics that can help to identify which patients are best suited to conventional surgery or transcatheter aortic valve implantation, and what parameters need to be measured. This article highlights the major features that should be assessed d...
This study was designed to evaluate the evidence base for the 2014 American Heart Association/American College of Cardiology (AHA/ACC) guidelines for severe aortic stenosis (SAS) and preserved left ventricular ejection fraction by comparing the natural history of the disease in subgroups of low-gradient (LG) aortic stenosis patients with high-gradient (HG) patients.
Hypertension is highly prevalent in patients with aortic stenosis (AS) and is associated with worse outcomes. The current prospective study assessed the impact of systolic hypertension (SHPT) on the progression of aortic valve calcification (AVC) measured by multidetector computed tomography (MDCT) in patients with AS.
Transcatheter aortic valve replacement (TAVR) is gaining favor as a treatment for aortic stenosis in patients at high risk for the open surgical approach. The following is a report of a 95-year-old woman with severe aortic stenosis who presented for TAVR with an Edwards SAPIEN valve. Her medical history included pacemaker-dependent complete heart block and a recent episode of congestive heart failure secondary to a non-ST segment elevation myocardial infarction. The TAVR was performed successfully through a...
The aim of this study was to evaluate the choice of treatment for severe aortic valve stenosis in the era of transcatheter aortic valve replacement (TAVR) in eastern Denmark.
This study sought to assess the natural history and optimal timing of surgery in elderly patients with severe asymptomatic aortic stenosis (AS).
There is a sinus P wave in front of each QRS, and the rate is 122 beats/min. The QRS voltage is large and meets many criteria for left ventricular hypertrophy: RI > 13 mm (1.3 mV); RaVF > 19 mm; SaVR > 14 mm; SV1 > 24 mm; SV2 > 30 mm; RV6 > 26 mm; RV6 > RV5; SV1 + RV5 or RV6 > 35 mm; SV2 + RV5 or RV6 > 45 mm; RaVL + SV3 > 20 mm in a woman.1 In addition, there are two non-voltage criteria for LVH: QRS duration = 0.11 s and typical repolarization changes: J-point depression with a downsloping ST segment into ...