1/17. Ochronotic arthropathy: case report and review of the literature.Alkoptonuria is an inherited metabolic disorder which is associated with various systemic abnormalities and related to the deposition of homogentisic acid pigment in connective tissues. These pigmentary changes are termed "ochronosis". We describe two patients with ochronotic arthropathy who presented with progressive and advanced degenerative changes in the lumbo-sacral spine. The literature, differential diagnosis and management of this rare condition are reviewed in this article. Management is usually conservative, but replacement surgery may be offered for severely affected major joints.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
2/17. chiropractic care and ochronotic arthropathy.OBJECTIVE: To discuss the case of a patient with ochronotic arthropathy whose symptoms were treated with chiropractic care. An emphasis is placed on this condition's radiographic features. CLINICAL FEATURES: A 59-year-old woman with pain in her low back, right knee, and left ankle sought chiropractic evaluation. Black pigmentation was found in the sclera of both eyes, and homogentisic acid was present in the urine. Orthopedic evaluation revealed uncomplicated, nonspecific joint pain, and radiographs demonstrated characteristic spinal changes. INTERVENTION AND OUTCOME: The patient was treated with chiropractic manipulation, physiotherapy modalities, bracing, and exercises. This type of therapy was successful in reducing the symptoms and helped decrease the severity and frequency of acute exacerbations. CONCLUSION: Ochronotic arthropathY is a rare metabolic disorder that can be diagnosed from spinal radiographs. chiropractic care is an appropriate tool for reducing its symptomatology.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
3/17. Bilateral hip arthroplasty for ochronotic arthropathy.ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare hereditary metabolic disorder characterised by the absence of the enzyme homogentisic acid oxidase and associated with various systemic abnormalities related to the deposition of homogentisic acid pigment (ochronotic pigment). In this report, we describe a 53-year-old, HLA-B27( ) woman with ochronotic arthropathy. In addition to the typical clinical features of the disorder, she had bilateral hip involvement, which was improved by cementless total hip prosthesis.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
4/17. Cementless total knee arthroplasty in ochronotic arthropathy: a case report with a 4-year follow-up.alkaptonuria is an inherited metabolic disorder characterized by the absence of the enzyme homogentisic acid oxidase, which leads to the accumulation of homogentisic acid, produced during normal metabolism of phenylalanine and tyrosine. ochronosis, which is the dark pigmentation of connective tissues in patients with long-lasting alkaptonuria, can cause severe cartilage destruction in large joints and the vertebral column. knee joint involvement, which occurs at relatively early ages, can be quite restrictive. arthroplasty may be the treatment of choice in these patients because of limited mobility and diffuse involvement of the joint. We report a 48-year-old man who had been treated with cementless total knee arthroplasty. Theoretically, there are no bone ingrowth deficits that might be detrimental for the stabilization of cementless prostheses in ochronotic arthropathy because the bone tissue is not primarily affected by the disease. The 4-year follow-up of cementless total knee arthroplasty was satisfactory without any evidence of loosening.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
5/17. alkaptonuria: a case report.alkaptonuria is a rare, autosomally recessive, metabolic disorder caused by a deficiency in homogentisic acid oxidase. It results in accumulation and deposition of homogentisic acid in cartilage, eyelids, forehead, cheeks, axillae, genital regions, nail beds, buccal mucosa, larynx, tympanic eardrum, and the tendons. We report a 33-year-old woman who presented with alkaptonuria and ochronotic pigment deposited in articular cartilage and cartilage of the ear and sclera.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
6/17. Ochronotic arthropathy.Ochronotic arthropathy is the musculoskeletal manifestation of alkaptonuria. an uncommon inherited metabolic disorder associated with various clinical and radiologic abnormalities due to the deposition of homogentisic acid. We report on a 35-year-old man with ochronotic arthropathy who presented with the main characteristic radiological findings. The differential diagnosis of this rare condition is discussed with a review of the literature.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
7/17. Vaginal hyperpigmentation due to ochronosis.BACKGROUND: ochronosis is a manifestation of alkaptonuria, a rare metabolic disorder. It results in the accumulation of pigment in connective tissues. After several years, ochronosis may produce a distinctive form of degenerative arthritis.CASE: Vaginal ochronosis was diagnosed in an asymptomatic elderly woman with vaginal hyperpigmentation and severe degenerative arthritis.CONCLUSION: Vaginal hyperpigmentation is a rare clinical finding that necessitates biopsy.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
8/17. Ochronotic arthropathy: arthroscopic findings in the shoulder and the knee.Ochronotic arthropathy is a disorder resulting from the deposition of homogentisic acid derivatives in the articular cartilage and the menisci. Large joints of the appendicular skeleton are preferentially affected. The clinical picture resembles that of degenerative joint disease. We present the arthroscopic findings in the shoulder and the knee in a 40-year-old man with ochronotic arthropathy and discuss the role of arthroscopy in the diagnosis and management of this rare metabolic disorder.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
9/17. Ochronotic arthritis: case reports and review of the literature.Alkoptunuria is an inherited autosomal recessive metabolic disorder which is caused by the lack of homogentisic acid-oxidase enzyme. It is associated with various systemic abnormalities and related to the deposition of homogentisic acid pigment in connective tissues. These pigmentary changes are termed "ochronosis". We describe two patients with ochronotic arthritis who presented with advanced degenerative changes in the lumbo-sacral spine, knee and hip. The literature, differential diagnosis and management of this rare condition are reviewed in this article.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
10/17. Alkaptonuric ochronosis: a case report.Alkaptonuric ochronosis is a rare autosomal recessive metabolic disorder resulting in a deficiency of homogentisic acid oxidase (alkaptonuria). Ultimately, this enzyme deficiency enables homogentisic acid to accumulate, become polymerized, and be systemically deposited within various tissues of the body (ochronosis). As the disease progresses, tissue deposition of polymerized homogentisic acid eventually will lead to the progressive degeneration of all affected body systems. There is no definitive cure for alkaptonuric ochronosis, and treatment is aimed at controlling and ameliorating symptoms. Multiple systemic complications occur as a result of alkaptonuric ochronosis. In the skeletal system, cervical, thoracic and lumbosacral degenerative disk disease develops, as do widespread arthritic changes in peripheral and weight-bearing joints. In the respiratory system, dyspnea can develop owing to limited chest excursion as a result of stiffening of cartilage in the chest wall. In the cardiovascular system, coronary and valvular calcification frequently occurs. In the genitourinary system, calculi formation and urine discoloration are chief manifestations. This case report describes a 63-year-old man with alkaptonuric ochronosis who sustained a stress fracture of the left femoral neck, necessitating surgical repair, which was done without complications. An overview of alkaptonuric ochronosis is presented, and anesthetic implications are discussed.- - - - - - - - - - ranking = 1keywords = metabolic disorder (Clic here for more details about this article) |
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