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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Hematin and propranolol in acute intermittent porphyria. Full recovery from quadriplegic coma and respiratory failure.

The authors present a case of acute intermittent porphyria (AIP) in an almost fatal relapse with quadriplegia, bulbar paralysis and coma. Intravenous hematin produced an immediate arousal from coma and allowed a gradual resumption of bulbar and autonomic functions. Persistent tachycardia and hypertension necessitated huge doses of intravenous propranolol. Both hematin and propranolol administrations were followed by a remarkable decrease in urinary amino-levulinic acid and porphobilinogen excretion. Nevertheless, after the acute stage, the patient was left with a severe generalized muscle wasting. After 7 months of intensive physical therapy, complete recovery of all neuromuscular functions was achieved. The modern aspects of the management of AIP are presented; the efficacy and the limits of hematin and propranolol therapy are discussed.[1]

References

  1. Hematin and propranolol in acute intermittent porphyria. Full recovery from quadriplegic coma and respiratory failure. Brezis, M., Ghanem, J., Weiler-Ravell, D., Epstein, O., Morris, D. Eur. Neurol. (1979) [Pubmed]
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