Cases reported "Porphyrias"

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1/6. Acute intermittent porphyria and caesarean delivery.

    A 29-yr-old patient was diagnosed with acute intermittent porphyria (AIP) during pregnancy. She had a Caesarean section under lidocaine/fentanyl epidural blockade. Because of inadequate analgesia, general anaesthesia was induced with propofol. Postoperatively urinary porphobilinogen excretion (625 mumol.day-1) exceeded the upper limit of normal but no symptoms of porphyria developed. In anecdotal clinical reports and in a previously described rat model of porphyria, propofol was found to be safe. This is the first reported use of propofol in a pregnant porphyric patient. Anaesthetic drug safety in porphyria is reviewed and the choice of induction agent discussed. Data on which to base these decisions is limited but we conclude that propofol may be suitable for use in patients with porphyria.
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ranking = 1
keywords = anaesthesia
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2/6. Use of regional anaesthesia in a patient with acute porphyria.

    The porphyrias are inherited disorders of haem metabolism, acute attacks of which may be precipitated by anaesthesia, surgery and pregnancy. The principal clinical feature of the disease is an acute neuropathy. A patient with acute intermittent porphyria was given bupivacaine as part of a regional anaesthetic for Caesarean section. The course of anaesthesia was uneventful.
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ranking = 6
keywords = anaesthesia
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3/6. Is propofol a safe agent in porphyria?

    A case is presented of the use of a propofol infusion for anaesthesia in a patient with known variegate porphyria. Urinary screening for porphyrins demonstrated a marked increase after this anaesthetic, but there were no clinical symptoms.
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ranking = 1
keywords = anaesthesia
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4/6. Induction of anaesthesia with ketamine during an acute crisis of hereditary coproporphyria.

    An appendectomy operation was undertaken in a 29-year-old patient with signs of an acute crisis of hereditary coproporphyria. Anaesthetic induction with ketamine 75 mg IV was uneventful. The safety of ketamine in patients with coproporphyria is discussed.
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ranking = 4
keywords = anaesthesia
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5/6. Induction of anaesthesia with etomidate in a patient with acute intermittent porphyria.

    This is a case report of a 35-year-old female with acute intermittent porphyria who presented for elective vagotomy and pyloroplasty. The diagnosis of porphyria was made two years previously when she developed acute abdominal pain and lower motor neurone paralysis following ingestion of a barbiturate. The urine porphobilinogen test was positive. The patient had no other acute attack of porphyria and had not had a previous anaesthetic. Anaesthesia was induced with etomidate 0.3 mg X kg-1 IV. muscle relaxation was obtained with pancuronium 6 mg IV and ventilation was mechanically controlled. Intraoperative analgesia was with 66 per cent nitrous oxide in oxygen and intermittent doses of fentanyl. The patient was stable during anaesthesia and surgery. The postoperative period was uneventful and patient did not have an acute attack of porphyria. This experience suggests that etomidate is safe for intravenous induction of anaesthesia in acute intermittent porphyria. However, reports of its use in more patients with this disease will be necessary before a final conclusion can be made.
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ranking = 6
keywords = anaesthesia
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6/6. A previous history of acute intermittent porphyria as a complication of obstetric anaesthesia.

    The administration of general anaesthesia for Caesarean section is described in a patient with an established biochemical diagnosis of acute intermittent porphyria. The technique used did not precipitate an acute exacerbation of the disease. Drugs associated with obstetric care are considered in the context of their use in porphyria.
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ranking = 5
keywords = anaesthesia
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