Document Detail

Investigations on the formation of urinary coproporphyrin isomers I-IV in 5-aminolevulinic acid dehydratase deficiency porphyria, acute lead intoxication and after oral 5-aminolevulinic acid loading.
MedLine Citation:
PMID:  10211628     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: Investigation of the metabolism of the four urinary coproporphyrin isomers I-IV in the extremely rare 5-aminolevulinic acid dehydratase (ALAD) deficiency porphyria (syn.: Doss porphyria), in acute lead intoxication, and after oral 5-aminolevulinic acid (ALA) loading. DESIGN AND METHODS: We analyzed the excretion of total urinary coproporphyrins and the composition of the respective isomers I-IV with ion-pair HPLC methods in these conditions. RESULTS: The concentration of total coproporphyrins was about 30-fold increased in patients with ALAD deficiency porphyria and acute lead intoxication as compared with controls. In addition, the proportion of coproporphyrin III as well as that of the atypical isomers II and IV were significantly elevated at the expense of isomer I. After oral ALA administration to normal volunteers, a 10- to 15-fold increase in the maximal concentration of total urinary coproporphyrins was observed within 12 to 24 h. Urinary levels were back to normal after another 24 h. The excretion pattern of the individual urinary coproporphyrin isomers I-IV after ALA ingestion revealed a dynamic process: initially isomer III was preferentially formed, followed by a 3-fold increase of isomers II and IV via non-enzymatic rearrangement of isomer III, and finally normalization of all four isomers occurred within 48 h. CONCLUSIONS: These results demonstrate that oral ALA loading can be used as an in vivo model to study the metabolism of the four urinary coproporphyrin isomers I-IV especially in ALAD deficiency porphyria and in acute lead poisoning.
K Jacob; E Egeler; U Gross; M O Doss
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Publication Detail:
Type:  Clinical Trial; Comparative Study; Controlled Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical biochemistry     Volume:  32     ISSN:  0009-9120     ISO Abbreviation:  Clin. Biochem.     Publication Date:  1999 Mar 
Date Detail:
Created Date:  1999-05-25     Completed Date:  1999-05-25     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0133660     Medline TA:  Clin Biochem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  119-23     Citation Subset:  IM    
Institute of Clinical Chemistry, University Hospital Grosshadern, Munich, Germany.
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MeSH Terms
Administration, Oral
Aminolevulinic Acid / administration & dosage*
Coproporphyrins / chemistry,  urine*
Lead Poisoning / urine*
Porphobilinogen Synthase / deficiency*
Porphyria, Acute Intermittent / urine*
Reg. No./Substance:
0/Coproporphyrins; 106-60-5/Aminolevulinic Acid; 14643-66-4/coproporphyrin III; 18372-11-7/coproporphyrin IV; 3082-03-9/coproporphyrin II; 531-14-6/coproporphyrin I; EC Synthase

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