Acute variegate porphyria following a Scarsdale Gourmet Diet.
Symptoms of porphyria developed in a 34-year-old woman following 3 weeks of a low carbohydrate diet. Reversal of a comatose state was obtained following multiple infusions of hematin. The diagnosis of variegate porphyria was made from the pattern of quantitative urine and stool porphyrins and a plasma fluorescence that was maximal at 626 nm. This fluorescence wavelength maxima also was found in the plasma of two family members screened for latent porphyria.
D000328:Adult; D003128:Coma / Q000209:etiology; D004038:Diet, Reducing / Q000009:adverse effects*; D005260:Female; D006418:Heme / Q000031:analogs & derivatives* / Q000378:metabolism; D006427:Hemin / Q000008:administration & dosage* / Q000378:metabolism; D006801:Humans; D007263:Infusions, Parenteral; D011164:Porphyrias / Q000150:complications / Q000188:drug therapy / Q000209:etiology*
D006418:Heme; D006427:Hemin