Document Detail

Hematin and propranolol in acute intermittent porphyria. Full recovery from quadriplegic coma and respiratory failure.
MedLine Citation:
PMID:  527604     Owner:  NLM     Status:  MEDLINE    
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.
M Brezis; J Ghanem; D Weiler-Ravell; O Epstein; D Morris
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European neurology     Volume:  18     ISSN:  0014-3022     ISO Abbreviation:  Eur. Neurol.     Publication Date:  1979  
Date Detail:
Created Date:  1980-04-26     Completed Date:  1980-04-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0150760     Medline TA:  Eur Neurol     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  289-94     Citation Subset:  IM    
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MeSH Terms
Acute Disease
Coma / drug therapy*
Heme / analogs & derivatives*
Hemin / administration & dosage,  therapeutic use*
Infusions, Parenteral
Porphyrias / complications,  drug therapy*
Propranolol / administration & dosage,  therapeutic use*
Quadriplegia / drug therapy
Respiratory Insufficiency / drug therapy*
Reg. No./Substance:
14875-96-8/Heme; 16009-13-5/Hemin; 525-66-6/Propranolol

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