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.
D000208:Acute Disease; D000293:Adolescent; D003128:Coma / Q000188:drug therapy*; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007263:Infusions, Parenteral; D008297:Male; D011164:Porphyrias / Q000150:complications / Q000188:drug therapy*; D011433:Propranolol / Q000008:administration & dosage / Q000627:therapeutic use*; D011782:Quadriplegia / Q000188:drug therapy; D012131:Respiratory Insufficiency / Q000188:drug therapy*
D006418:Heme; D006427:Hemin; D011433:Propranolol