Skip to main content

Main menu

  • Home
  • COVID-19
    • Articles & podcasts
    • Blog posts
    • Collection
    • News
  • Content
    • Current issue
    • Past issues
    • Early releases
    • Collections
    • Sections
    • Blog
    • Infographics & illustrations
    • Podcasts
  • Authors
    • Overview for authors
    • Submission guidelines
    • Submit a manuscript
    • Forms
    • Editorial process
    • Editorial policies
    • Peer review process
    • Publication fees
    • Reprint requests
  • CMA Members
    • Overview for members
    • Earn CPD Credits
    • Print copies of CMAJ
    • Career Ad Discount
  • Subscribers
    • General information
    • View prices
  • Alerts
    • Email alerts
    • RSS
  • JAMC
    • À propos
    • Numéro en cours
    • Archives
    • Sections
    • Abonnement
    • Alertes
    • Trousse média 2021
  • CMAJ JOURNALS
    • CMAJ Open
    • CJS
    • JAMC
    • JPN

User menu

Search

  • Advanced search
CMAJ
  • CMAJ JOURNALS
    • CMAJ Open
    • CJS
    • JAMC
    • JPN
CMAJ

Advanced Search

  • Home
  • COVID-19
    • Articles & podcasts
    • Blog posts
    • Collection
    • News
  • Content
    • Current issue
    • Past issues
    • Early releases
    • Collections
    • Sections
    • Blog
    • Infographics & illustrations
    • Podcasts
  • Authors
    • Overview for authors
    • Submission guidelines
    • Submit a manuscript
    • Forms
    • Editorial process
    • Editorial policies
    • Peer review process
    • Publication fees
    • Reprint requests
  • CMA Members
    • Overview for members
    • Earn CPD Credits
    • Print copies of CMAJ
    • Career Ad Discount
  • Subscribers
    • General information
    • View prices
  • Alerts
    • Email alerts
    • RSS
  • JAMC
    • À propos
    • Numéro en cours
    • Archives
    • Sections
    • Abonnement
    • Alertes
    • Trousse média 2021
  • Visit CMAJ on Facebook
  • Follow CMAJ on Twitter
  • Follow CMAJ on Pinterest
  • Follow CMAJ on Youtube
  • Follow CMAJ on Instagram
SynopsisC

Alkaptonuria and photography: A patient's urine tells the story

David Maxwell
CMAJ April 12, 2005 172 (8) 1002-1003; DOI: https://doi.org/10.1503/cmaj.045188
David Maxwell
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Tables
  • Responses
  • Metrics
  • PDF
Loading

This picture of a patient who had alkaptonuria (Fig. 1) was taken by my father, Dr. Ian Maxwell, in 1957 and was developed using the patient's own urine.

Figure1
  • Download figure
  • Open in new tab
  • Download powerpoint

Figure 1.

Alkaptonuria was the first described “inborn error of metabolism.” This rare (< 1 per 250 000 births) hereditary recessive disorder is characterized by a triad of excretion of homogentisic acid in the urine, ochronosis (dark pigmentation of the connective tissues) and early-onset arthritis. It results from the absence of an enzyme, homogentisic acid oxidase. Homogentisic acid is produced during the metabolism of phenylalanine and tyramine and, in the absence of the enzyme, accumulates in large quantities in the plasma and urine. In patients with alkaptonuria, a second enzyme, homogentisic acid polyphenoloxidase, present in mamallian tissue, catalyzes the oxidation of the accumulated homogentisic acid in vivo to an ochronotic pigment that has a high affinity for cartilage and connective tissues, forming fragile complexes. The clinical manifestations of the disease — dense, coal-black pigmentation of costal, tracheal and laryngeal cartilages, blue-grey pigmentation of the ears and sclera, and extensive early degenerative arthritis of the spine and large joints (due to the breakdown of the cartilage–pigment complexes) — are the result of this pigment deposition. The excess homogentisic acid also appears in the urine, where it may undergo oxidation on exposure to atmospheric oxygen, particularly in an alkaline environment. This oxidation results in a characteristic gradual darkening of the urine downward from the surface on standing.1

To appreciate the relation of this to photography, we need a (somewhat simplified) explanation of the processes involved in the creation of a photographic image. The photographic emulsion consists of microscopic crystals of silver bromide embedded in a gelatin matrix. Photons striking the crystals dislodge electrons, resulting in the formation of metallic silver atoms and bromine. The latter is trapped by the gelatin matrix, leaving the silver atoms. At this stage the number of silver atoms is relatively small, creating what is termed a latent image. If this image is now “developed,” by placing it in a solution containing a relatively strong reducing substance, the crystals in which the process of conversion to metallic silver has been initiated rapidly seize the electrons donated by the reducing substance, multiplying the original generation of metallic silver atoms by a factor of roughly 10 000. The developed image is now formed of crystals of metallic silver wherever light struck it, interspersed with unaltered crystals of silver bromide. The latter are then rendered soluble and washed out of the emulsion, leaving only the areas of dark metallic silver to form the photographic image.2

Figure2
  • Download figure
  • Open in new tab
  • Download powerpoint

Figure 2.

The connection between this process and alkaptonuria becomes clearer when one examines the molecular structures of hydroquinone (a widely used developer present, for example, in Kodak D11 and D76) and homogentisic acid (Fig. 2).

As a developer, the paired hydroxyl (OH) groups on the benzene ring serve to reduce the silver ions. The effectiveness of homogentisic acid in this process was noted as early as 1942 by Fishberg, who proposed its use as a specific test for “the instantaneous diagnosis of alkaptonuria on a single drop of urine.” 3 It should be noted that, although the fundamental requirement for a developer is that it be a reducing substance, the process is rendered more efficient in the usual commercial developers by the addition of a number of other chemicals. These obviously are lacking in urine, which probably accounts for the restricted range of contrast evident in Fig. 1. An intriguing speculation is the role played by the pigment formed as the homogentisic acid oxidizes, either in contributing to the overall cast evident in the photograph or to the image itself.

My father never published this experiment, but the same concept was applied by Scott Williams and his students in the 1995 Technical Photographic class at the Rochester Institute of Technology.4 They used coffee as a developer, affording a new use for the cold dregs at the end of a long day. One of Williams' students subsequently extended the concept, using an infusion of mint, which proved to be an even more effective developer, in addition to producing olfactorily enhanced prints,5 perhaps an advantage over the urine of ochronotic patients.

References

  1. 1.↵
    La Du BN. Alkaptonuria. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. 8th ed. New York: McGraw-Hill; 2001. p. 2109-23.
  2. 2.↵
    Glafkidès P. Chimie et physique photographiques. 4e éd. Paris: Publications Photo-Cinéma, Paul Montel; 1976. p. 68-80.
  3. 3.↵
    Fishberg EH. The instantaneous diagnosis of alkaptonuria on a single drop of urine. JAMA 1942;119:882.
    OpenUrlCrossRef
  4. 4.↵
    Williams S. That last cup of coffee: photographic developers of last resort for the weary, the bleary, and the unrested. Darkroom & Creative Camera Techniques 1995;16(5):35-7.
  5. 5.↵
    Iverglynne D. A biodegradeable development solution and method of use [international patent application no PCT/US/2002/005653]. PCT Gazette 2003 Dec 31. Publ no WO 2004/001504].
PreviousNext
Back to top

In this issue

Canadian Medical Association Journal: 172 (8)
CMAJ
Vol. 172, Issue 8
12 Apr 2005
  • Table of Contents
  • Index by author
  • Canadian Adverse Reaction Newsletter (1133 - 1140)

Article tools

Print
Download PDF
Article Alerts
To sign up for email alerts or to access your current email alerts, enter your email address below:
Email Article

Thank you for your interest in spreading the word on CMAJ.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Alkaptonuria and photography: A patient's urine tells the story
(Your Name) has sent you a message from CMAJ
(Your Name) thought you would like to see the CMAJ web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Alkaptonuria and photography: A patient's urine tells the story
David Maxwell
CMAJ Apr 2005, 172 (8) 1002-1003; DOI: 10.1503/cmaj.045188

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
‍ Request Permissions
Share
Alkaptonuria and photography: A patient's urine tells the story
David Maxwell
CMAJ Apr 2005, 172 (8) 1002-1003; DOI: 10.1503/cmaj.045188
Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • References
  • Figures & Tables
  • Responses
  • Metrics
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • Benzene rings
  • Benzene rings
  • Google Scholar

More in this TOC Section

  • Applying the 2005 Canadian Hypertension Education Program recommendations: 4. Managing uncomplicated hypertension
  • A newborn requiring selective bronchial intubation
  • Does β-blocker prophylaxis improve survival after major noncardiac surgery?
Show more Synopsis

Similar Articles

Collections

  • Topics
    • Genetics

Content

  • Current issue
  • Past issues
  • Collections
  • Sections
  • Blog
  • Podcasts
  • Alerts
  • RSS
  • Early releases

Information for

  • Advertisers
  • Authors
  • Reviewers
  • CMA Members
  • Media
  • Reprint requests
  • Subscribers

About

  • General Information
  • Journal staff
  • Editorial Board
  • Governance Council
  • Journal Oversight
  • Careers
  • Contact
  • Copyright and Permissions

Copyright 2021, Joule Inc. or its licensors. All rights reserved. ISSN 1488-2329 (e) 0820-3946 (p)

All editorial matter in CMAJ represents the opinions of the authors and not necessarily those of the Canadian Medical Association or its subsidiaries.

To receive any of the resources on this site in an accessible format, please contact us at cmajgroup@cmaj.ca.

Powered by HighWire