149 research outputs found

    O kysto hydatico do cerebro na criança

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    Noticias médicas: organización del vii congreso de la unión internacional sobre protección a la infancia

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    El doctor A. Lesage, Presidente del Comité que organiza el VIICongreso de la Unión Internacional de Protección a la Infancia, arealizarse en París del 5 al 9 de julio de 1933, me pide quiera interesarme por hacer formar los Comités Nacionales que en los países de América organicen la propaganda a favor de dicho Congreso, para obtener adhesiones y trabajos

    Spinal involvement in mucopolysaccharidosis IVA (Morquio-Brailsford or Morquio A syndrome): presentation, diagnosis and management.

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    Mucopolysaccharidosis IVA (MPS IVA), also known as Morquio-Brailsford or Morquio A syndrome, is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase (GALNS). MPS IVA is multisystemic but manifests primarily as a progressive skeletal dysplasia. Spinal involvement is a major cause of morbidity and mortality in MPS IVA. Early diagnosis and timely treatment of problems involving the spine are critical in preventing or arresting neurological deterioration and loss of function. This review details the spinal manifestations of MPS IVA and describes the tools used to diagnose and monitor spinal involvement. The relative utility of radiography, computed tomography (CT) and magnetic resonance imaging (MRI) for the evaluation of cervical spine instability, stenosis, and cord compression is discussed. Surgical interventions, anaesthetic considerations, and the use of neurophysiological monitoring during procedures performed under general anaesthesia are reviewed. Recommendations for regular radiological imaging and neurologic assessments are presented, and the need for a more standardized approach for evaluating and managing spinal involvement in MPS IVA is addressed

    Scintigraphic features of Morquio's syndrome: a case report

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    <p>Abstract</p> <p>Introduction</p> <p>To the best of our knowledge, we present for the first time the bone scintigraphy findings of a patient with Morquio's syndrome.</p> <p>Case presentation</p> <p>A 46-year-old Caucasian man with Morquio's syndrome presented with lower back pain six weeks after a left total hip replacement. A whole body bone scan demonstrated an anthropomorphic skeletal pattern consistent with a mucopolysaccharide storage disease, thereby showing the cause of the patient's pain.</p> <p>Conclusions</p> <p>To the best of our knowledge, the bone scintigraphy findings of a case of Morquio's syndrome have never before been published. We present our case report to add to the knowledge we have of this rare disease.</p

    Complicaciones de las incisiones quirúrgicas

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    Presented at session 18th August 1948Presentado en sesión de 18 de agosto de 194

    The puzzle of TRPV4 channelopathies

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    Anaesthetic considerations of adults with Morquio's syndrome - a case report

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    <p>Abstract</p> <p>Background</p> <p>The anaesthetic management of patients with Morquio syndrome is complicated by a number of factors including odontoid hypoplasia, atlantoaxial instability, thoracic kyphosis, and deposition of mucopolysaccharides in the soft tissue of the oropharnyx.</p> <p>Case presentation</p> <p>Herein we describe the anaesthetic considerations and management of a 26 year old adult with Morquio syndrome, who presented for an elective hip replacement.</p> <p>Conclusion</p> <p>This report details an awake fiberoptic intubation in an adult with Morquio syndrome. We recommend that this approach be considered in patients with Morquio syndrome undergoing general anaesthesia.</p
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