Cases reported "Mucopolysaccharidosis II"

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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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2/3. Delayed awakening from general anaesthesia in a patient with Hunter syndrome.

    Hunter syndrome is one of a heterogeneous group of recessively inherited mucopolysaccharide storage diseases (MPS) with similar biochemical defects manifested by impairments in mucopolysaccharide catabolism with variable but progressive clinical courses. Abnormal accumulation and deposition of mucopolysaccharides in the tissue of several organs to numerous anatomical, musculoskeletal and neurological abnormalities which are known to complicate anaesthetic and airway management. Hunter syndrome has a wide variance of clinical phenotypes ranging from mild to severe. We present a patient having physical and neurological features consistent with a severe clinical presentation of Hunter syndrome (MPS, Type II). Following a seemingly uneventful intraoperative anaesthetic course including isoflurane, nitrous oxide and fentanyl (0.93 microgram.kg-1), resumption of spontaneous ventilation and return to consciousness were delayed until intravenous naloxone (200 micrograms) was administered 110 min after the opioid administration. The cause of delayed recovery from anaesthesia in this patient is unknown.
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keywords = anaesthesia
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3/3. Failure to control the airway in a patient with Hunter's syndrome.

    Hunter's syndrome is due to recessively inherited mucopolysaccharide storage diseases. The anaesthetic complications in this syndrome are related to the organs involved in the disease, but pertain especially to upper airway obstruction problems. We report a patient with Hunter's syndrome who, while under general anaesthesia for a simple procedure (the introduction of bilateral grommets), suffered a fatal outcome.
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keywords = anaesthesia
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