Этот адрес видимо тоже подходит.

Dear all,

I have a patient 2-yr-old boy with permanent abnormal Urine Color since birth(photo - see attachment , please). Color changes only become apparent after the urine stands for some time.
Porphobilinogen is normal.
He has no other problems.

What is your opinion?

I will be grateful for your input.

Sincerely Yours,
Mikhail Kagan, M.D.,
Pediatric Nephrologist,
Orenburg regional children's hospital,
Ribakovskaya street 3, 460006,
Orenburg, Russia.
e-mail :mkaganorenburg@yahoo.com

I would bet on Alkaptonuria.

See article N Engl Journal of Medicine, Vol 347. No 26 page 2111.

Robert Haws, M.D.
Pediatric Nephrologist
Marshfield Clinic

(715) 387-5092

Alkaptonuria urine turns really black not reddish in an alkalotic
environment with time. Can you get urine and do this experiment, add
acid or alkali to see the pH dependence?
Urine from Hartnup patients (indicanuria) can also turn dark.
Best, Robert

I have two brothers with alkaptonuria. The urine typically turns a shade of red after several hours but does not turn black unless it is allowed to sit for more than a day. I assume this is a pH phenomenon. In light of the fact that Dr Kagan's patient is developmentally normal Hartnup disorder is less likely but should also be considered.

Robert Haws, M.D.
Pediatric Nephrologist
Marshfield Clinic

(715) 387-5092