Rapid spectrophotometric quantification of urinary porphyrins and porphobilinogen as screening tool for attacks of acute porphyria.

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  • Additional Information
    • Source:
      Publisher: Published by SPIE--the International Society for Optical Engineering in cooperation with International Biomedical Optics Society Country of Publication: United States NLM ID: 9605853 Publication Model: Print Cited Medium: Internet ISSN: 1560-2281 (Electronic) Linking ISSN: 10833668 NLM ISO Abbreviation: J Biomed Opt Subsets: MEDLINE
    • Publication Information:
      Original Publication: Bellingham, WA : Published by SPIE--the International Society for Optical Engineering in cooperation with International Biomedical Optics Society, c1996-
    • Subject Terms:
      Porphobilinogen/*urine ; Porphyria, Acute Intermittent/*diagnosis ; Porphyrins/*urine ; Spectrometry, Fluorescence/*methods; Algorithms ; Equipment Design ; Humans
    • Abstract:
      Autosomal-dominant acute porphyria, a group of rare diseases, can lead to life-threatening neurovisceral attacks. No efficient screening test is available today. Elevated urinary porphobilinogen in addition to elevated porphyrins is highly specific for an attack of acute porphyria. This study proposes and evaluates a custom-made device, algorithm, and methods for a two-step quantification of urinary porphyrins and porphobilinogen. The first step is oxidation of the nonfluorescent porphyrinogens and subsequent fluorescence-spectroscopic determination of total urinary porphyrins (TUP) using second derivative spectral fitting. Photo-oxidation is compared with chemical oxidation methods. The second step is the quantification of porphobilinogen in case of elevated TUP. Heat-induced conversion products of porphobilinogen, namely uroporphyrin and porphobilin, are quantified by fluorescence and absorption spectroscopy. Results show that the preferred method combination is TUP quantification (lower limit of quantification: 0.2  μmol  /  L) after photo-oxidation with subsequent absorption-spectroscopic determination of porphobilin after heating for indirect quantification of porphobilinogen (quantification range: 0 to 20  mg  /  L). Urinary porphobilinogen and porphyrins of one acute porphyria patient were quantified with <10  %   deviation from an external reference determination. The spectrophotometric approach requires only minimal sample processing and yields a result within 15 min, thus closing the screening gap for acute porphyria.
      ((2018) COPYRIGHT Society of Photo-Optical Instrumentation Engineers (SPIE).)
    • Contributed Indexing:
      Keywords: absorption; acute porphyria; fluorescence; porphobilinogen; porphyrins; spectroscopy; urine
    • Accession Number:
      0 (Porphyrins)
      74KHC72QXK (Porphobilinogen)
    • Publication Date:
      Date Created: 20180602 Date Completed: 20191001 Latest Revision: 20191001
    • Publication Date:
      20240829
    • Accession Number:
      10.1117/1.JBO.23.5.055006
    • Accession Number:
      29855177