This NPAW, thought I’d share bits of history uncovered as I review, sort and file zillions (ok, not zillions, but a lot) of porphyria-related articles.
Orange–hued urine…acute porphyria attacks associated with severe heme depletion in the liver….
U.S. Dr. Cecil J. Watson (1901-1983)’s medical fellowship was completed in the Munich laboratory of the world’s leading expert in the chemistry of hemoglobin, bile pigments, and porphyrins, Nobel laureate Dr. Hans Fischer. Dr. Watson had developed a keen interest “in the formation and structure of the various heme derivatives which are formed in the intestine from bilirubin and are in part reabsorbed and re-excreted in urine or bile.” He is credited with proving that excreted porphyrins represent intermediates formed in the course of heme synthesis, whereas the tetrapyrroles of the urobilin group are degradation products of heme formed by stepwise microbial reduction of bilirubin in the intestine. One of his early findings showed that when bilirubin has reached the intestine, it is almost quantitatively converted to colorless derivatives by the intestinal microflora. On oxidation, these chromogens turn into bright orange pigments, several of which [he] succeeded in crystallizing.”
It was Dr. Watson who recognized that acute porphyric attacks are associated with severe heme depletion in the liver—hmmm, exactly what Jill and I have observed ourselves for years. In 1941, he and his coworker Schwartz developed the classic Watson-Schwartz test for qualitative detection of urinary PBG .
Retirement didn’t stop this Energizer bunny-scientist—“ he had the unique intellectual satisfaction of discovering a highly effective and often life-saving therapy for a disease which had preoccupied him for over forty years—the successful treatment of acute porphyric attacks with the infusion of hematin (heme replacement). Yet another “proof” Jill and I have observed for years.
In reading a review of this exceptional yet humble doctor who wasn’t above “riding the ambulance which, at that time, was a duty assigned to assistant residents” and lament that that era of U.S. porphyria scientist is long gone.
Source available by leaving a message at www.purplecanaries.com.