Cases reported "Joint Diseases"

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1/35. Peripheral joint scanning with technetium pertechnetate. Application in clinical practice.

    Results of a prospective study to evaluate the clinical usefulness of radioisotope joint scanning in the practice of rheumatology are reported. The radiopharmaceutical used was technetium pertechnetate, increased synovial blood flow being the main contributory factor to abnormal joint images. Strict adherence to a procedural protocol was necessary to assure reproducible images. Joint scintigraphy was performed on 56 control subjects without joint disease and 51 patients with different rheumatic disorders. There was excellent agreement between clinical examination and joint scintigraphy in all groups studied. In cases of disagreement, the joint scintigram generally was more sensitive. This high sensitivity for the detection of synovitis was accompanied by a lack of specificity, because it failed to diagnose the nature of the joint involvement. The joint scan was helpful for diagnosing early or steroid-suppressed synovitis and also for suspected nonarticular rheumatism. It aided in delineating the pattern of joint involvement. Joint scintigraphy is the only objective nontraumatic permanent record documenting joint inflammation.
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2/35. Atraumatic hemarthrosis caused by a large mediopatellar plica.

    hemarthrosis of the knee has various etiologies and is classified into atraumatic or post-traumatic. Among atraumatic factors, hemarthrosis due to synovial plica is extremely rare. We report a case of atraumatic hemarthrosis caused by the mediopatellar plica. A 21-year-old male truck driver was referred to our hospital, because of swelling and pain of the right knee without history of trauma. Bloody synovial fluid was aspirated by arthrocentesis. However, his symptoms recurred and persisted. The range of motion was normal, but the patient complained of anteromedial knee pain during maximum flexion. Routine biochemical analyses were within normal limits. Plain radiographs were normal. magnetic resonance imaging (MRI) of the knee showed the hypertrophic mediopatellar plica and an irregular signal of the infrapatellar fat pad. arthroscopy revealed a voluminous mediopatellar plica trapped between the patella and the medial femoral condyle. It attached to the center of the infrapatellar fat pad, the so-called tongue. When the tourniquet was released, fresh bleeding was observed from the region between the mediopatellar plica and the tongue. Because that region was considered to be the cause of the hemarthrosis, the mediopatellar plica and the tongue were excised. A histologic examination of the tongue showed evidence of bleeding with nonspecific synovitis. After the procedure, the patient was asymptomatic and there were no clinical signs of recurrence.
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3/35. Investigation of sacroiliac disease: Comparative evaluation of radiological and radionuclide techniques.

    An attempt has been made to improve diagnostic precision in a group of diseases associated with inflammation of the sacroiliac joints, by using a 99mTechnetium stannous pyrophosphate bone scan. inflammation of these joints is associated with osteoblastic activity and is reflected by an increase in the uptake of radionuclide, which can be precisely quantitated. The uptake was markedly above the range of normal in patients with active ankylosing spondylitis (AS), and also in a number of patients with possible AS, psoriasis, and Reiter's syndrome. However, patients with Grade 4 radiological changes of the sacroiliac joints frequently had normal scans. This technique may be useful in the early diagnosis of sacroiliitis, and the nosological implications of the changes in patients with Reiter's syndrome and psoriasis are of interest.
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4/35. sarcoidosis in a preschooler with only skin and joint involvement.

    sarcoidosis is a multisystemic granulomatous disease of unknown etiology. It is relatively rare in children less than 15 years of age and especially in those less than 5-6 years of age. sarcoidosis characteristically involves the skin, eyes, and synovial tissues in patients less than 5-6 years of age. We report a 3-year-old boy with sarcoidosis who had cutaneous findings with joint symptoms. Dermatologic examination revealed lichenoid, erythematous, 2-3 mm papules, some of them grouped, all over his extremities and trunk. There were symmetric swellings on his ankles and wrists without erythema or pain which did not interfere with function. However, until now, no eye involvement had been detected in the patient.
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5/35. alkaptonuria, ochronosis, and ochronotic arthropathy.

    OBJECTIVES: To describe the clinical presentation and course of a relatively large group of Italian adult patients screened for mutation of the homogentisate dioxygenase gene causing alkaptonuria (AKU) and ochronosis, and to review typical and atypical facets of this condition. methods: We reviewed the medical records of 9 patients affected by ochronotic arthropathy who were observed in our institutions between 1979 and 2001. All patients were diagnosed as having AKU through a rapid urine test with alkali. mutation screening was performed by single-strand conformation analysis of all homogentisate dioxygenase exons, followed by sequencing of altered conformers. RESULTS: Our 9 cases had similar clinical features and they reflected those described in the literature: a progressive degenerative arthropathy mainly affecting axial and weight-bearing joints associated with extraarticular manifestation. Musculoskeletal symptoms began in most of our patients around the age of 30 years with back pain and stiffness: involvement of the large peripheral joints usually occurred several years after spinal changes. Ochronotic peripheral arthropathy generally was degenerative, but joint inflammation was observed in some cases; this could be attributed to an inflammatory reaction of the ochronotic shard in the synovial membrane. CONCLUSIONS: ochronosis is a model of arthropathy with known etiologic factors. Over time, AKU, the genetically determined metabolic defect, leads to the accumulation of pigment and the development of this crippling condition. Most of the clinical findings may be explained by inhibition of collagen crosslinks, but some require additional interpretation. For example, inflammatory features of the ochronotic joint only occur in a minority of cases, and may be attributable to ochronotic shards. Further studies are needed to establish the genotype-phenotype correlation to identify mutations that are predictive of severe disease. For this purpose, the Italian Study Group on alkaptonuria (www.dfc.unifi.it/aku) is enrolling affected patients in an on-line database to characterize the molecular defects and their relationship to clinical data.
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6/35. Early loss of hip containment in a child with dysplasia epiphysealis hemimelica.

    Progressive loss of hip containment attributable to dysplasia epiphysealis hemimelica of the right proximal femur and macrodactyly of the right second toe was diagnosed and monitored by radiographs and magnetic resonance imaging in a 7-year-old boy. The patient had early surgical correction including shortening osteotomy of the hypertrophic toe and partial resection of the involved superolateral femoral head-neck junction combined with a Pemberton-like acetabuloplasty. This treatment restored containment and function. At 4 years followup, the femoral head remained contained and the patient was participating fully in the activities of daily life for his age-group. A review of the literature suggests monitoring the hip with magnetic resonance imaging may allow early identification of a hip at risk for loss of containment. This treatment may save the hip from severe deformity and early secondary osteoarthritis.
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7/35. Jaccoud arthropathy: a rarity in the spectrum of HIV-associated arthropathy.

    In studies comparing HIV-infected patients with HIV-negative patients, rheumatic manifestations, including reactive arthritis, psoriatic arthritis, myalgia, tendinitis, fibromyalgia, necrotizing vasculitis, polymyositis, Sjogren syndrome, were shown to occur more frequently in the HIV-positive group. Uncommon clinical features and the demonstration of hiv antigens in the synovial membranes suggest a direct role of HIV in the pathogenesis of HIV-associated rheumatic disease. Jaccoud arthropathy is a nonerosive deforming arthropathy reported to occur in cases of chronic rheumatic fever and systemic lupus erythematosus. Only two cases of HIV-associated Jaccoud arthropathy has been reported in the literature thus far, both in patients with features of reactive arthritis. We report a case of HIV-associated Jaccoud arthropathy in a patient without features of reactive arthropathy.
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8/35. Hypophosphataemic rickets and pyrophosphate arthropathy.

    Pyrophosphate arthropathy (PA) has been reported in association with a number of diseases, usually occurring in the older age group. We report a 40-year-old female with untreated X-linked hypophosphataemic rickets who presented with PA.
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9/35. mycobacterium fortuitum group periprosthetic joint infection.

    The mycobacterium fortuitum group of rapidly growing mycobacteria is recognized infrequently as a cause of periprosthetic joint infection. This report describes a case of periprosthetic joint infection due to a member of the M. fortuitum group where failure to consider this pathogen caused months of diagnostic delay. Aggressive surgery and 6 months of combination antimicrobial therapy eradicated the infection.
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10/35. Arthropathy of the ankle in hemophilia.

    Seventy-five patients who had hemophilia were followed clinically and roentgenographically to assess the prevalence of hemarthrosis and the prevalence and severity of arthropathy of the ankle. The mean age of the patients at the time of follow-up was twenty-two years and seven months. The patients were divided into four age-groups: less than ten years (eleven patients), ten to nineteen years (twenty-one patients), twenty to thirty years (twenty-four patients), and more than thirty years (nineteen patients). Intra-articular bleeding occurred more frequently in the joints of the lower extremities than in the joints of the upper extremities. During the second decade of life, hemarthroses occurred more often in the ankle than in the knee. A history of recurrent bleeding into the ankle joint, chronic synovitis, and overgrowth of the medial portion of the distal tibial epiphysis was associated with an early onset of arthropathy. In older patients, compression arthrodesis of the ankle joint was helpful in eliminating pain, recurrent bleeding, and equinus deformity.
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