Cases reported "Mucopolysaccharidosis I"

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1/3. Failed epidural anaesthesia in a patient with Hurler's disease.

    Failure of epidural anaesthesia in a patient with Hurler's disease is presented. The possibility that epidural anaesthesia may fail due to deposition of mucopolysaccharide in the meninges, or in the epidural space is discussed. A granuloma in the upper trachea and severe choanal stenosis complicated the already difficult airway management.
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keywords = anaesthesia
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2/3. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses.

    We present case reports of five patients with severe forms of mucopolysaccharidoses who developed postobstructive pulmonary oedema during anaesthesia. The difficulties of anaesthesia in these patients and the particular predisposition that these patients exhibit for the development of postobstructive pulmonary oedema is discussed.
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keywords = anaesthesia
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3/3. Anaesthetic implications of the mucopolysaccharidoses: a fifteen-year experience in a children's hospital.

    patients suffering from the mucopolysaccharidoses, most commonly Hurler's syndrome, present special problems in anaesthesia. A retrospective review of the anaesthetic management of such patients over fifteen years revealed a high incidence of airway problems. Two case histories illustrating these difficulties are presented.
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keywords = anaesthesia
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