In this study, an on-line stacking capillary electrophoresis (CE) method, reverse-mode field amplified sample injection equipped with sweeping micellar electrokinetic chromatography (RMFASI-sweeping MEKC), was established for direct determination of uroporphyrin and coproporphyrin in human urine. Porphyrins playing a very important role in the biosynthesis of heme, chlorophyll and other importa...

High-dose chloroquine therapy for porphyria cutanea tarda is rarely used now because of its hepatic side-effects. The mechanisms of the effects and side-effects are poorly understood. We describe here effects, side-effects and long-term follow-up in 57 patients with a first-time diagnosis of porphyria cutanea tarda treated with 1-3 phlebotomies followed by 250 mg chloroquine phosphate daily for...

Hepta-, hexa-, and pentacarboxylic porphyrins have been detected in porphyric as well as normal urine samples. From the former they have been isolated and converted into pure methyl esters (1). For a better understanding of the chemistry of these porphyrins, pure uroporphyrin I and uroporphyrin III were separately subjected to decarboxylation under controlled conditions. The decarboxylation pro...

Free uroporphyrin is decarboxylated by heating in the air to 150--200 degrees C. Complete decarboxylation occurs after heating to 200 degrees C for 5 min, with a yield of 66--75%. Heptacarboxylic porphyrin can be prepared with a yield of 40% by heating uroporphyrin to 150 degrees C for 60 min, and hexacarboxylic porphyrin with a yield of about 20% by heating to 150 degrees C for 2h.

Patients with porphyria usually discharge large amounts of uroporphyrins, mainly as a mixture of the isomers I and III, as well as other porphyrins. Knowledge concerning their quantitative distribution in the disease has long been sought. The chromatographic separation of methyl esters of uroporphyrin I and “Waldenstrom porphyrin” (1, 2), then believed to be uroporphyrin III, was described by F...

patients with AIP and all four patients with VP. Because our three patients were excreting high concentrations of urine PBG at the time of fecal collection, their serum PBG was probably increased. If this is the source of fecal uroporphyrin, it probably would also be seen during acute attacks of the other acute porphyrias, as has been reported for VP (12). HCP was excluded from the differential...

The studies reported here were conducted on a patient with congenital porphyria who excretes large amounts of uroporphyrin I and coproporphyrin I. They were directed to the following problems: (a) to determine whether glycine is specifically utilized in the biologic synthesis of porphyrins related to the etioporphyrin I configuration as it is utilized in the synthesis of protoporphyrin which is...

9 girl of 4 years, exhibiting photosensitivity, anemia, and splenomegaly, excretes large amounts of coproporphyrin I, uroporphyrin I, and stercobilinogen. She was given 15 gm. of glycine containing 32.2 atom per cent excess Nls in divided doses over a 3 day period. Serial determinations! were then made of the NIK in the hemoglobin protoporphyrin, coproporphyrin I and uroporphyrin I of the urine...

An improved method for separation and fluorescent analysis of coproporphyrin and uroporphyrin in urine was presented by Sobel, Cano, and Thiers [Clin. Chem. 20, 1397 (1974)]. Accurate determination of porphyrin concentrations by this method is impeded by the fluorescent background in urine samples, which varies from one specimen to another. By use of a straightforward procedure, zinc may be inc...

It has been reported (1) and confirmed (2) that the production of uroporphyrin III from porphobilinogen, either by heating in acid solution or enzymatically by hemolyzed avian erythrocytes, is accompanied by the formation of formaldehyde from the aminomethyl group of the pyrrole. These observations have been cited as evidence in support of two hypotheses on the mechanism of uroporphyrin III for...