Knee joint replacements in alkaptonuria patients : report of fourteen Jordanian cases and review of literatures

الملخص EN

Objective: Alkaptonuria is a metabolic disease caused by deficiency of the hepatic enzyme es dioxygenase (HGD), consequently the homogentisic acid (HGA) accumulat , 21 homogentisate in the cells and body fluids.

This accumulation causes early degeneration and black discoloration of the knees, spine, sclera, heart endocardium and valves.

Joints arthroplasty is the treatment of choice when the conservative treatment is no longer effective.

Methods: Retrospectively fourteen alkaptonuria patients who have had knee replacement in our center have been analyzed, four females and ten males, six cases had bilateral knees replacements, follow up period ranges between 12- 36 months.

Also, this study includes a review of 22 studies of joints replacement in alkaptonuria patients, these studies have been published between 2000-2016, and have included 36 patients; 12 females and 24 males, 63 joints had been replaced (39 knees, 21 hips and three shoulders).

All these 50 patients have been followed for a period that range between 12-36 months.

Results: Our fourteen patients have had significant improvement after total knee replacements and there were no intra operative complications, during our follow up period no post-operative infections or any significant complaint have been observed.

Also the 22 published studies have had no complication and they had good results.

Conclusion: Few cases of alkaptonuria have been operated by joint replacements and have been reported in the literatures, the follow up periods were not long.

So more cases and longer period follow up are needed to estimate the benefits and the risks of the joint replacement in these patients with alkaptonuria.

But according to our results joint replacement seems to be the treatment of choice to alleviate the pain in severely affected joints in alkaptonuria patients