scholarly journals Polyarticular Ochronotic Arthritis: A Case Report

2019 ◽  
Vol 2 (1-3) ◽  
pp. 1-13 ◽  
Author(s):  
Marco Di Marco ◽  
Silvia Elena De Martinis ◽  
Marcello Truzzi ◽  
Roberto Viganò
Keyword(s):  
Functional Limitation ◽  
Homogentisic Acid ◽  
Knee Joints ◽  
Acid Oxidase ◽  
Inherited Disease ◽  
Ochronotic Arthropathy ◽  
Clinical Presentations ◽  
Oxidase Enzyme ◽  
Total Knee

Alkaptonuria is an inherited disease due to the lack of homogentisic acid oxidase enzyme. The most common clinical presentations are a brownish-grey color of the sclera, dark skin, kidney stones, aortic calcifications, and ochronotic arthropathy. Ochronotic arthropathy is characterized by the accumulation of homogentisic acid in the connective tissue determining severe joint degeneration. We present the case of a woman affected by ochronosis since the age of 30 years. The patient showed a severe degeneration of the hip and knee joints bilaterally, associated with an important and painful functional limitation. Conservative care did not achieve good results. We performed bilateral cementless total knee arthroplasty and bilateral cementless total hip arthroplasty. We report satisfactory outcomes in terms of range of motion and pain control in a 6-year follow-up. Our approach to this pathology is the same we adopt with rheumatoid arthritis after our decennial experience. We suggest that prosthetic replacement represents a definitive way to treat ochronotic arthropathy.

scholarly journals Case Report: Ochronotic Arthropathy

2021 ◽  
Author(s):  
Ülkü Dönmez ◽  
Ece Çınar ◽  
Cihat Öztürk ◽  
Simin Hepgüler
Keyword(s):  
Soft Tissues ◽  
Clinical Findings ◽  
Homogentisic Acid ◽  
Inflammatory Back Pain ◽  
Joint Involvement ◽  
Acid Oxidase ◽  
Ochronotic Arthropathy ◽  
Vitamin C Supplementation ◽  
Oxidase Enzyme ◽  
Chronic Arthropathy

Ochronosis is a rare metabolic disease caused by the deficiency of the homogentisic acid oxidase enzyme. It gives clinical findings related to the accumulation of homogentisic acid in soft tissues and excretion in urine. Patients with chronic arthropathy usually have some joint pain and inflammatory back pain. Although axial involvement radiologically resembles ankylosing spondylitis (AS), it is differentiated by the absence of typical syndesmophytes, facet involvement, sacroiliac erosion and fusion.Although there is no effective treatment for ochronosis disease; our patient was given a protein-poor diet, vitamin C supplementation (100 mg/kg/day) and analgesic treatment for symptoms recommended in the literature; and a reduction in joint complaints was observed. In this case, the aim is to diagnose the rare ochronotic arthropathy followed with the diagnosis of AS and to show that both axial and peripheral joint involvement can be together in this disease.

scholarly journals Complications of Patella in Total Knee Arthroplasty (TKA)

2021 ◽  
Vol 6 (5) ◽  
Keyword(s):  
Total Knee Arthroplasty ◽  
Knee Arthroplasty ◽  
Patellar Instability ◽  
Revision Rate ◽  
Functional Limitation ◽  
Patellofemoral Instability ◽  
Mobile Bearing ◽  
Extensor Mechanism ◽  
Total Knee

Background and Methods: Postoperative complications may impair the outcome of total knee arthroplasty (TKA). Patellar instability is a major cause of postoperative pain and functional limitation for which revision surgery may be necessary [1]. It may occur after TKA with or without patellar resurfacing. RESULTS AND CONCLUSIONS: Subluxation is more common than dislocation; the incidence of symptomatic instability leading to revision is low (0.5 to 0.8%) [2, 3]. In a multicentre study of low contact stress mobile bearing TKAs, only 6 of 259 revisions were associated with patellar instability, which accounted for a revision rate of 0.1% after a mean follow-up duration of 5.7 years [4]. A revision rate of 12% was reported secondary to complications of the extensor mechanism [5]. The aetiology of patellofemoral instability can be related to (1) the surgical technique and component positioning, (2) extensor mechanism imbalance, and (3) other causes.

Alkaptonuria and lumbar disc herniation

2003 ◽  
Vol 98 (1) ◽  
pp. 87-89 ◽  
Author(s):  
Akbar Farzannia ◽  
Ghaffar Shokouhi ◽  
Shahram Hadidchi
Keyword(s):  
Lumbar Disc Herniation ◽  
Disc Herniation ◽  
Lumbar Disc ◽  
Homogentisic Acid ◽  
Acid Oxidase ◽  
Content Type ◽  
Discal Herniation ◽  
Black Discoloration ◽  
Fine Print

✓ Alkaptonuria is a rare metabolic disease caused by deficiency of homogentisic acid oxidase and characterized by bluish-black discoloration of cartilages and skin (ochronosis). The authors report the cases of three patients with lumbar disc herniation who underwent discectomy and in whom the nucleus pulposus was discovered to be black. Alkaptonuria was diagnosed after discectomy. Discal herniation requiring surgery is unusual in alkaptonuria, with only a few reports. The symptoms in the three patients disappeared after surgery and no symptoms were demonstrated on follow-up examination.

scholarly journals Skin pigmentation, a window to diagnose Alkaptonuria: a very rare entity

2017 ◽  
Vol 5 (6) ◽  
pp. 2801
Author(s):  
Sandhya Chauhan ◽  
Ashok Garg ◽  
G. R. Tegta ◽  
Kuldeep Thakur
Keyword(s):  
Autosomal Recessive ◽  
Skin Pigmentation ◽  
Clinical Manifestations ◽  
Homogentisic Acid ◽  
Cardiac Complications ◽  
Acid Oxidase ◽  
Urine Examination ◽  
Tyrosine Metabolism ◽  
Oxidase Enzyme ◽  
Rare Inborn Error

Alkaptonuria (AKU) or endogenous ochronosis is a very rare inborn error of tyrosine metabolism inherited by autosomal recessive mode. There is complete absence of homogentisic acid oxidase enzyme which results in accumulation of homogentisic acid in cartilaginous connective tissue thus produces ochronotic clinical manifestations. Here we reported a 36 year old woman with bluish pigmentation of pinnae, index fingers (lateral aspect), nails, teeth and sclera. Detailed clinical and investigative workup was done to diagnose patient. Skin biopsy showed changes of ochronosis and urine examination revealed detectable level of homogentisic acid. Classical ocular findings, ochronosis on clinical and HPE and positive urinary tests for homogentisic acid confirmed the diagnosis of alkaptonuria. The highlight of our case is that an asymptomatic patient was detected early by ochronosis prior to development of musculoskeletal or cardiac complications.

Electron Microscopic and Isozyme Study of a Case of Ochronosis

1972 ◽  
Vol 30 ◽  
pp. 88-89
Author(s):  
Jay W. Cha ◽  
Perry J. Melnick
Keyword(s):  
Benzene Ring ◽  
Enzyme System ◽  
Homogentisic Acid ◽  
Degenerative Changes ◽  
Acid Oxidase ◽  
Electron Microscopic ◽  
Tyrosine Metabolism ◽  
Collagenous Tissues

Hereditary ochronosis in very few cases has been examined electron microscopically or histochemically. In this disease homogentisic acid, a normal intermediary of tyrosine metabolism, forms in excessive amounts. This is believed to be due to absence or defective activity of homogentisic acid oxidase, an enzyme system necessary to break the benzene ring and to further break it down to fumaric and acetoacetic acids. Ochronotic pigment, a polymerized form of homogentisic acid, deposits mainly in mesenchymal tissues. There has been a question whether the pigment originates from the collagenous tissues, or deposits passively, where in contrast to melanin it induces degenerative changes.

Effect of Manipulation under Anesthesia of the First Knee in Staged Bilateral Total Knee Arthroplasty on Clinical Outcome and Satisfaction

2020 ◽  
Author(s):  
Jung-Won Lim ◽  
Yong-Beom Park ◽  
Dong-Hoon Lee ◽  
Han-Jun Lee
Keyword(s):  
Total Knee Arthroplasty ◽  
Patient Satisfaction ◽  
Clinical Outcome ◽  
Clinical Outcomes ◽  
Knee Arthroplasty ◽  
Knee Flexion ◽  
Functional Score ◽  
Manipulation Under Anesthesia ◽  
Total Knee

AbstractThis study aimed to evaluate whether manipulation under anesthesia (MUA) affect clinical outcome including range of motion (ROM) and patient satisfaction after total knee arthroplasty (TKA). It is hypothesized that MUA improves clinical outcomes and patient satisfaction after primary TKA. This retrospective study analyzed 97 patients who underwent staged bilateral primary TKA. MUA of knee flexion more than 120 degrees was performed a week after index surgery just before operation of the opposite site. The first knees with MUA were classified as the MUA group and the second knees without MUA as the control group. ROM, Knee Society Knee Score, Knee Society Functional Score, Western Ontario and McMaster Universities (WOMAC) score, and patient satisfaction were assessed. Postoperative flexion was significantly greater in the MUA group during 6 months follow-up (6 weeks: 111.6 vs. 99.8 degrees, p < 0.001; 3 months: 115.9 vs. 110.2 degrees, p = 0.001; 6 months: 120.2 vs. 117.0 degrees, p = 0.019). Clinical outcomes also showed similar results with knee flexion during 2 years follow-up. Patient satisfaction was significantly high in the MUA group during 12 months (3 months: 80.2 vs. 71.5, p < 0.001; 6 months: 85.8 vs. 79.8, p < 0.001; 12 months: 86.1 vs. 83.9, p < 0.001; 24 months: 86.6 vs. 85.5, p = 0.013). MUA yielded improvement of clinical outcomes including ROM, and patient satisfaction, especially in the early period after TKA. MUA in the first knee could be taken into account to obtain early recovery and to improve patient satisfaction in staged bilateral TKA.

scholarly journals Management and results in periprosthetic tibial fracture after total knee arthroplasty: Two-center 15-case retrospective series at 2 years’ follow-up

2020 ◽  
Vol 106 (3) ◽  
pp. 449-458
Author(s):  
Camille Bauer ◽  
Bogdan Zaharia ◽  
Florent Galliot ◽  
Jauffrey Parot ◽  
Fayçal Houfani ◽  
...  
Keyword(s):  
Total Knee Arthroplasty ◽  
Knee Arthroplasty ◽  
Tibial Fracture ◽  
Total Knee

scholarly journals Outcomes of the rotating hinge knee in revision total knee arthroplasty with a median follow-up of 6.2 years

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Jake von Hintze ◽  
Mika Niemeläinen ◽  
Harri Sintonen ◽  
Jyrki Nieminen ◽  
Antti Eskelinen
Keyword(s):  
Quality Of Life ◽  
Total Knee Arthroplasty ◽  
Survival Rate ◽  
Knee Arthroplasty ◽  
Revision Total Knee Arthroplasty ◽  
Patient Reported ◽  
Total Knee ◽  
Revision Tka

Abstract Background The purpose of this study was to determine the mid-term clinical, radiographic and health-related quality of life (HRQoL) outcomes and define the survival rate in patients who had undergone revision total knee arthroplasty (TKA) using the single rotating hinged knee (RHK) design. Methods Between January 2004 and December 2013, 125 revision TKAs were performed at our institution using the single RHK implant. We conducted both a retrospective analysis of prospectively collected outcome data of these patients and a prospective follow-up study of all 39 living patients (41 knees). The follow-up phase included an optional extra follow-up visit, PROM questionnaires, and plain radiographs. Results The ten-year Kaplan-Meier survival rate of the revision RHK knees was 81.7% (95% CI 71.9–91.6%) with re-revision for any reason as the endpoint. Overall, 15 knees (12% of the total) underwent re-revision surgery during the follow-up. The median follow-up was 6.2 years (range, 0–12.7 years) post-operatively for the baseline group. One mechanical hinge mechanism-related failure occurred without any history of trauma or infection. At the time of the final follow-up, the majority of patients evinced a fairly good clinical outcome measured with patient-reported outcome measures and none of the components were radiographically loose. Conclusion We found that in patients undergoing complex revision TKA, fairly good functional outcome and quality of life can be achieved using an RHK implant. Further, it seems that in this type of patient cohort, revision TKA using an RHK implant relieves pain more than it improves ability to function. The NexGen® RHK design can be regarded as a suitable option in complex revision TKA.

scholarly journals Three-year clinical and radiological results of a cruciate-retaining type of the knee prosthesis with anatomical geometry developed in Japan

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Arata Nakajima ◽  
Manabu Yamada ◽  
Masato Sonobe ◽  
Yorikazu Akatsu ◽  
Masahiko Saito ◽  
...  
Keyword(s):  
Knee Prosthesis ◽  
Cruciate Retaining ◽  
Radiolucent Lines ◽  
Radiographic Outcomes ◽  
Patient Reported ◽  
Long Term Follow Up ◽  
Total Knee ◽  
Clinical And Radiological Results ◽  
Anatomical Geometry

Abstract Background The FINE total knee was developed in Japan and clinical use began in 2001. It has unique design features, including an oblique 3o femorotibial joint line that reproduces anatomical geometry. Although 20 years have passed since the FINE knee was clinically used for the first time in Japan, a formal clinical evaluation including patient-reported and radiographic outcomes has not been undertaken. Methods A total of 175 consecutive primary cruciate-retaining (CR)-FINE total knee arthroplasties (TKAs) at our hospital between February 2015 and March 2017 were included in this study. Three years postoperatively, range of motion (ROM), Knee Society Score (KSS), Knee Injury and Osteoarthritis Outcome Score (KOOS) and Forgotten Joint Score (FJS) were recorded and compared with preoperative scores. Radiographic analyses including mechanical alignment, component alignment, and incidence of radiolucent lines also were undertaken based on the radiographs 3 years postoperatively. Results One-hundred twenty-two knees (70%) were available for 3-year follow-up data using KOOS, except for the sports subscale. Postoperative KOOS-symptom, −pain and -ADL were > 85 points, but KOOS-sports, −QOL and FJS were less satisfactory. ROM, KSS and all the subscales of KOOS were significantly improved compared with preoperative scores. Postoperative mean FJS was 66 and was significantly correlated with all the subscales of KOOS, but not with postoperative ROM. Radiolucent lines ≧1 mm wide were detected in five knees (4.1%). There were no major complications needing revision surgeries. Conclusions Patient-reported outcomes (PROs) for symptoms, pain and ADL after the CR-FINE TKA were generally improved, but those for sports, QOL and FJS were improved less. The incidence of radiolucent lines was rare but detected around the femoral components. With the mid- to long-term follow-up, improvements of surgical technique will be necessary to achieve better PROs from patients receiving the FINE knee.

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