1918 Archives of Internal Medicine  
The occurrence of alkaptonuria is sufficiently rare to justify the publication of the following case. Only nine of the seventy or so recorded cases of alkaptonuria have been reported occurring in the states. As the exact cause of this condition is still unknown and as relatively few cases have been reported, it seemed well to add this record to those which have been published, with the findings and result of treatment. Fromherz has recently expressed the opinion that many alkaptonurics remained
more » ... unnoticed, and he calls attention to the fact that forty-five of the seventy cases have been reported within the last fifteen years. The term alkaptone (from alkali and k\ l =a' \ \ g=p\ TELV, "to absorb greedily") was first ascribed by B\l=o"\dekerin 1857 to a substance found in the urine of a patient, which possessed two chief characteristics : first, the power to reduce alkaline copper solutions, and secondly, the property of absorbing oxygen from the atmosphere in the presence of alkali, and as a result turning to a brown-black color. The urine may be clear and of normal color when passed, but later becomes of a dark brown and finally black color on exposure to the air. This condition may be present in cases of ochronosis, but is not a necessary accompaniment of this affection. Gouget has found a number of references in the literature of the past to cases of melanuria which were probably instances of alkaptonuria. Thus, Scribonius (1584), Zacutus and Lusitances (1649) describe cases of young chil¬ dren in apparent health who passed black urine; and Scheneck (1609) reports the case of a Monk who presented the same urinary anomaly during his whole life. In 1875 Ebstein and Müller obtained a substance from the urine of an alkaptonuric patient by treating the urine with alcohol-ether, which formed the reactions given by pyrocatechin. This observation was of interest because it was the first case in which the opinion had been expressed as to what the reactions in alkaptonuria were. Fleischer next observed a urine which gave similar reactions with those of Fürbinger in which he was able to demonstrate small quantities of pyrocatechin. Bödeker in a communication to Fürbinger expresses the view that his alkaptone bodies might be similar to pyrocatechin.
doi:10.1001/archinte.1918.00090120087008 fatcat:te7j7unmdzbh7jzz47pzv37fwm