Nutrition management of acute intermittent porphyria.
A patient with acute intermittent porphyria is presented to emphasize the relevance of nutrition in its management. Clinical manifestations, diagnosis of acute intermittent porphyria, simplified heme biosynthetic pathway, the glucose effect, and the rationale for giving nutritional therapy for acute intermittent porphyria are discussed.
Robert
T L
TL
Department of Surgery, University Hospital, SUNY Health Science Center 13210.
Varella
L
L
Meguid
M M
MM
D000328:Adult; D002405:Catheterization, Central Venous; D002408:Catheters, Indwelling; D003131:Combined Modality Therapy; D004040:Dietary Carbohydrates / Q000627:therapeutic use*; D005260:Female; D005947:Glucose / Q000494:pharmacology; D006418:Heme / Q000096:biosynthesis; D006427:Hemin / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007262:Infusions, Intravenous; D010289:Parenteral Nutrition, Total; D017118:Porphyria, Acute Intermittent / Q000178:diet therapy* / Q000188:drug therapy / Q000628:therapy
D004040:Dietary Carbohydrates; D006418:Heme; D006427:Hemin; D005947:Glucose