алкаптонурия, охроноз, деформирующий остеоартроз, эндопротезирование коленного сустава, alkaptonuria, ochronosis, osteoarthritis, total knee arthroplasty, Orthopedic surgery, RD701-811

Alkaptonuria is rare disease with deficiency of homogentisate-1,2-dioxygenase enzyme, resulting in excess deposition of homogentisic acid in connective tissue. This deposition leads to ochronosis - brownish-black pigmentation of connective tissue. The result of pigmentation is weakness of connective tissue and, finally, chronic inflammation and osteoarthtritis. Currently specific treatment is absent, only sympothomatic. But total knee arthroplasty has good outcomes in patients of severe ochronotic arthritis similar to osteoarthritic patients without ochronosis. We present a case report of a patient with knee ochronotic arthritis treated with total knee arthroplasty.