Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is autosomal recessiveand clinically characterized by soft extensible skin, laxity ofjoints and kyp...

STUDY OBJECTIVE To analyze predictors of survival for patients receiving home long-term oxygen therapy (LTOT) or prolonged mechanical ventilation (PMV) according to the cause of chronic respiratory insufficiency (CRI) and the patients physiologic data. DESIGN Analysis of a nationwide database (ANTADIR Observatory). SETTING The national nonprofit network for home treatment of patients with C...

The etiology of congenital scoliosis and its development remains unclear and has not yet been fully identified, even there are theories that congenital scoliosis could be derived from the failure of formation or failure of segmentation, which are etiologically heterogeneous with genetic, epigenetic, and environmental factors contributing to their occurrence. We reported a case of long-term foll...

observed nowadays. In affected patients dislocation of inner organs with functional impairment may occur [1,2]. We present the unusual case of a patient with severe kyphoscoliosis who developed extensive esophageal ulceration. An 83-year-old female presentedwith a 3week history of dysphagia, odynophagia, and nocturnal food regurgitation. She reported weight loss of 5 kg during the previous 3 we...

Costal movement was defined on living subjects by determining the spatial vectors along the ribs that are produced during inspiration. The determination of these vectors was achieved with an instrument specially designed for this purpose. Rib movement was studied on 61 ribs in 10 normal subjects and on 35 ribs in six patients suffering from kyphoscoliosis and ankylosing spondylitis. In normal s...

BACKGROUND Research has provided evidence for the safety, feasibility, and efficacy of exercise training in patients with COPD. However, little is known about the impact of exercise training in patients with chronic respiratory failure due to kyphoscoliosis. We evaluated the effect of an exercise training program on exercise capacity, muscle strength, dyspnea, and quality-of-life indices in sub...

BACKGROUND This study investigated the outcomes of preoperative HGT as an adjunct treatment for severe thoracic kyphoscoliosis, its role in radiographic correction, and pulmonary function improvement, together with nursing strategy and incidence of complications. MATERIAL AND METHODS Eleven patients with a mean age of 18.8 years were retrospectively reviewed. Inclusion criteria were: patients w...

AIM The aim of this study is to perform a screening of patients diagnosed with ankylosing spondylitis (AS) in order to evaluate the static spinal disorders and correlate the results with the main clinical and functional parameters that characterize this disease. MATERIAL AND METHODS Ninety-five patients diagnosed with AS according to the 1994 New York criteria were screened, and 68 of them (a...

Kyphoscoliosis is a progressive disease that worsens with age. While applying anesthesia, kyphosis and/or scoliosis can lead to difficulty in both during endotracheal intubation and also performing regional interventional technics. In addition to aging and the direct effects of deformity such as neurological deficities and immobility; deterioration in cardiopulmonary functions can also develop ...