[Hematin treatment of acute intermittent porphyria. A case report (author's transl)].
A young woman with acute intermittent porphyria in profound relapse and severe nervous and respiratory involvement was treated by intravenous infusions of hematin, followed by improvement of symptoms. The diet with high carbohydrate and protein content and a B-adrenergic blocking agent, not showed any beneficial effect on acute attack. The infusion of hematin was followed by a lowering effect on urine porphybilinogen and delta-aminolevulinic acid and clinical improvement. The return to normal of porphyrin precursors in the urine was accompanied by almost complete clinical remission. The relationship of remission and repression of delta-aminolevulinic acid, decrease of urinary levels of porphyrin precursors, pulmonaries tests and electromyograms, were discussed.
D000293:Adolescent; D000622:Aminolevulinic Acid / Q000652:urine; D000959:Antihypertensive Agents / Q000627:therapeutic use; D004576:Electromyography; D005260:Female; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007263:Infusions, Parenteral; D008168:Lung / Q000503:physiopathology; D011162:Porphobilinogen / Q000652:urine; D011164:Porphyrias / Q000178:diet therapy / Q000188:drug therapy*
D000959:Antihypertensive Agents; D006418:Heme; D006427:Hemin; D011162:Porphobilinogen; D000622:Aminolevulinic Acid
Tratamento da porfiria aguda intermitente com hematina. Registro de caso.