![]() Reconstitution of lyophilized hydroxyheme with albumin enhances stability of lyophilized hemin, decreases the incidence of phlebitis, and may enhance efficacy. Another hemin preparation, heme arginate, is more stable in solution but is not available in the United States. It is recommended that lyophilized hemin be reconstituted with human albumin to enhance stability ( 2). With repeated administration, phlebitis can compromise venous access. Degradation products form rapidly in vitro when this product is reconstituted with sterile water, as recommended in product labeling, and these adhere to endothelial cells, platelets, and coagulation factors and cause a transient anticoagulant effect and often a phlebitis at the site of infusion. Hemin (Panhematin ®), Lundbeck Pharmaceuticals, is available in the United States as lyophilized hydroxyheme (hematin) for reconstitution with sterile water just before infusion, and it is approved by the FDA for amelioration of acute porphyric attacks. The standard regimen is 3–4 mg of hemin per kilogram of body weight, infused intravenously once daily for 4 days. Although product labeling recommends an initial trial of intravenous glucose, hemin is the preferred therapy ( 80– 82). ![]() Hemin therapy should be started early ( 2). It cannot be given orally because it is catabolized by heme oxygenase during intestinal absorption. ![]() ![]() Hemin given intravenously at moderate dose (3–4 mg/kg day for 4 days) is mostly taken up in the liver, and can at least transiently replenish the depleted heme pool that regulates the synthesis of ALAS1. Intravenous hemin addresses the underlying pathophysiology by repressing hepatic ALAS1, hence decreasing the overproduction of ALA and PBG. Anderson, in Emery and Rimoin's Principles and Practice of Medical Genetics (Sixth Edition), 2013 99.4.3.7.2 Hemin Therapy ![]()
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