Cases reported "Mucopolysaccharidoses"

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1/3. 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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2/3. Anaesthetic problems in Hurler-Scheie syndrome. Report of two cases.

    Hurler-Scheie syndrome is an intermediate form of mucopolysaccharidosis. Affected patients characteristically present in infancy with serious abnormalities including the upper airways and the lungs. We present two patients with Hurler-Scheie syndrome and describe their anaesthetic management. One case was complicated by difficult endotracheal intubation and postoperative pneumonia. The second case was successfully managed using spinal anaesthesia and mild sedation.
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ranking = 0.16666666666667
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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ranking = 0.16666666666667
keywords = anaesthesia
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