Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003864 (arthritis)
 69,039 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic-onset juvenile chronic arthritis (SoJCA) is associated with high levels of circulating interleukin-6 (IL-6) and is frequently complicated by severe microcytic anemia whose pathogenesis is unclear. Therefore, we studied 20 consecutive SoJCA patients with hemoglobin (Hb) levels <12 g/dL, evaluating erythroid progenitor proliferation, endogenous erythropoietin production, body iron status, and iron supply for erythropoiesis. Hb concentrations ranged from 6.5 to 11.9 g/dL. Hb level was directly related to mean corpuscular volume (r = .82, P < .001) and inversely related to circulating transferrin receptor (r = -.81, P < .001) suggesting that the severity of anemia was directly proportional to the degree of iron-deficient erythropoiesis. Serum ferritin ranged from 18 to 1,660 microgram/L and was unrelated to Hb level. Bone marrow iron stores wore markedly reduced in the three children investigated, and they also showed increased serum transferrin receptor and normal-to-high serum ferritin. All 20 patients had elevated IL-6 levels and normal in vitro growth of erythroid progenitors. Endogenous erythropoietin (epo) production was appropriate for the degree of anemia as judged by both the observed to predicted log (serum epo) ratio 10.95 +/- 0.12) and a comparison of the serum epo-Hb regression found in these subjects with that of thalassemia patients. Multiple regression analysis showed that serum transferrin receptor was the parameter most closely related to hemoglobin concentration: variation in circulating transferrin receptor explained 61% of the variation in Hb level (P < .001). In 10 severely anemic patients, amelioration of anemia following intravenous iron administration resulted in normalization of serum transferrin receptor. Defective iron supply to the erythron rather than blunted epo production is the major cause of the microcytic anemia associated with SoJCA. A true body-iron deficiency caused by decreased iron absorption likely complicates long-lasting inflammation in the most anemic children, and this can be recognized by high serum transferrin receptor levels. Although oral iron is of no benefit, intravenous iron saccharate is a safe and effective means for improving iron availability for erythropoiesis and correcting this anemia. Thus, while chronically high endogenous IL-6 levels do not appear to blunt epo production, they are probably responsible for the observed abnormalities in iron metabolism. Anemia of chronic disease encompasses a variety of anemic conditions whose peculiar features may specifically correlate with the type of cytokine(s) predominantly released.
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PMID:Defective iron supply for erythropoiesis and adequate endogenous erythropoietin production in the anemia associated with systemic-onset juvenile chronic arthritis. 863 55

Nutritional status and nutrient intake were assessed in 17 children with active juvenile chronic arthritis (JCA) who never received steroids and in 17 controls matched for age and sex. Five patients had systemic, seven polyarticular and five oligoarticular JCA. Values significantly below those of the controls were found in systemic patients for height (p<0.05), upper arm circumference (p<0.05) and arm muscle area (p<0.01), and in polyarticular subjects for arm muscle area (p<0.01). All patients had unremarkable anthropometric fat measurements. All anthropometric measurements were normal in oligoarticular patients. Twelve JCA patients had reduced serum iron (Fe), 6 reduced serum zinc (SZn), 14 reduced intra-erythrocytic zinc (EZn) and 2 reduced serum copper (SCu). SZn was inversely correlated with erythrocyte sedimentation rate (ESR) (p=0.023). EZn was inversely related to lymphocyte count (p=0.022). SCu was related to ESR (p=0.037) and to lymphocyte count (p=0.016). No significant difference in nutrient intake was found between patients and controls. Active JCA was associated with reduced muscular mass, Fe, SZn, EZn. These alterations did not depend on reduced nutrient intake.
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PMID:Nutritional status in active juvenile chronic arthritis not treated with steroids. 865 51

Hemochromatosis is characterized by excessive absorption and subsequent deposition of iron in various organs and is prevalent in 1 out of 20,000 hospitalized patients. Most patients with hereditary hemochromatosis (HHC) become symptomatic between the ages of 50 and 60 years. Distinct forms of arthritis have been associated with HHC and may be the initial clinical manifestation in some patients. This is a case of a patient who had chronic hip and back pain and painless swelling over the knuckles. Radiographs revealed classical signs of HHC. Early recognition and prompt institution of phlebotomy can improve the outcome of patients with HHC.
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PMID:Case report: arthropathy as the presenting symptom in hereditary hemochromatosis. 896 22

Leukocyte scintigraphy (LS) was performed in 20 pediatric patients with inflammatory bowel disease (IBD: 10 with ulcerative colitis, 2 with indeterminate colitis, and 8 with Crohn disease) in different stages of clinical activity. Leukocytes were separated from 15 to 60 ml venous blood and were labeled in vitro with [99mTc]HM-PAO. The segmental extent (small intestine; ascending, transverse, and descending colon; and recto-sigmoideum) of the process was determined by LS. The uptake of each bowel segment was scored in relation to the bone marrow uptake. The scintigraphic activity, calculated by summing the segment scores, was compared with laboratory parameters. The mean labeling efficacy was 76% (60-86%). The segmental extent of the process determined by LS was compared with the results of barium enema or colonoscopy with regard to 32 bowel segments. The sensitivity, specificity, and accuracy of LS were 93, 88, and 91%, respectively. Two extraintestinal manifestations (abdominal abscess and joint involvement) were also detected by LS. These lesions were verified by computed tomography (CT) (abscess) and on the basis of the clinical outcome (arthritis). The scintigraphic activity correlated with the C-reactive protein (CRP) level (r = 0.82, p < 0.001), the alpha 2-globulin level (r = 0.63, p < 0.02), the sedimentation rate (r = 0.51, p < 0.05), and the fS iron level (r = -0.66, p < 0.005). LS is applicable in pediatric patients. The method is an excellent technique for assessment of the extent of IBD in children. Extraintestinal manifestations of IBD can also be investigated by LS. The scintigraphic activity is a useful parameter for determination of the activity of IBD in children.
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PMID:HM-PAO-labeled leukocyte scintigraphy in pediatric patients with inflammatory bowel disease. 898 43

Approximately 1.5 million persons in the United States are affected by iron overload diseases, which are primarily caused by hereditary hemochromatosis--the most common genetic disorder in the United States. Hereditary hemochromatosis is characterized by increased iron absorption in the gastrointestinal tract, which may cause lifelong excessive iron absorption and accumulation and serious health effects, including arthritis, cirrhosis, diabetes, impotence, heart failure, and death. Hereditary hemochromatosis is an autosomal recessive disease; the estimated prevalence of the homozygous genotype is 1:200 - 1:250 persons, and 10% of persons are carriers. Although the disease was previously believed to affect primarily white males of northern European descent, recent data indicate hereditary hemochromatosis also occurs among blacks. Moreover, iron overload diseases are underdiagnosed among whites and may not be considered in other racial/ethnic groups (e.g., Hispanics) even when compatible symptoms and clinical findings are present. As part of a joint demonstration project during August-October 1995 to determine the overall prevalence of iron overload, CDC reviewed data from a health-maintenance organization (HMO) in San Diego, California; the prevalence among Hispanics appeared similar to that for non-Hispanic whites. This report presents the preliminary findings of an analysis of the prevalence of iron overload among Hispanics and compares these findings with nationally representative data from the Third National Health and Nutrition Examination Survey (NHANES III). These findings indicate that the prevalence of possible iron overload among Hispanic clients of the HMO based on initial screening was consistent with the nationwide prevalence of possible iron overload based on a single screening test for Hispanics of Mexican descent and non-Hispanic whites.
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PMID:Iron overload disorders among Hispanics--San Diego, California, 1995. 900 7

A thalassaemic girl presented with agranulocytosis, arthritis of both ankles and clinical and laboratory features consistent with the diagnosis of systemic vasculitis, during oral iron chelator L1 (deferiprone) treatment. Changes in the humoral and cell-mediated immune function. including antinuclear antibodies (ANA), anti-DNA and extractable-nuclear antigens (ENA) antibodies positivity, increased immunoglobulin values, decreased T suppressor and the presence of circulating immune complexes, suggest a cause-and-effect relationship with the observed clinical manifestations. A careful monitoring of the immune function is recommended in patients who are receiving the oral iron chelator L1.
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PMID:Agranulocytosis, arthritis and systemic vasculitis in a patient receiving the oral iron chelator L1 (deferiprone). 902 8

Iron overload caused by lifelong transfusion-dependent anaemias, such as beta-thalassaemia major, usually results in lethal cardiac toxicity in the second decade of life if not treated by iron chelation. There is no physiological mechanism for excreting the excess iron accumulated from blood transfusions and, unlike hereditary haemochromatosis, venesection is not an option. Therefore, chelation therapy is the only way to remove excess iron. This must be removed while not depriving cells of the essential iron needed for normal metabolism. Additionally, the iron chelator must prevent iron from participating in the generation of harmful free radicals. Parenteral chelation therapy with deferoxamine (desferrioxamine) is well established as promoting negative iron balance, reversing cardiac toxicity, and prolonging life expectancy well into the fourth decade of life and, most likely, beyond. Unfortunately, poor compliance with the rigours of parenteral treatment in a minority of patients limits its regular use, resulting in reduced life expectancy in these patients. Use of deferoxamine in excessive dosages may result in growth retardation, sensorineural ototoxicity and ocular toxicity, as well as bone deformities. These effects can be largely avoided if the dosage is adjusted to take account of the degree of iron overload (using the therapeutic index) and if the mean daily dose does not exceed 40 mg/kg. Nevertheless, it is recommended that patients be regularly monitored for such adverse effects. Deferiprone (L1; CP20) is an orally absorbed bidentate hydroxypyridinone iron chelator that can induce urinary iron excretion, promote negative iron balance and reduce hepatic iron levels in some transfusion-dependent patients, particularly in those who are markedly iron overloaded and have not received regular deferoxamine therapy. The long term efficacy and toxicity of deferiprone are the subjects of some controversy, and the published results of randomised controlled trials are awaited. Preliminary results suggest that when currently recommended dosages of deferiprone (75 mg/kg/day) are used, hepatic iron settles at levels that still put most patients at an increased risk from iron overload. A number of adverse effects may occur, and require cessation of therapy in up to 30% of patients. These effects include arthritis, nausea and (most seriously) agranulocytosis in 0.6 to 4% of patients. The risk of the latter complication means that frequent white blood cell counts are mandatory for patients taking this drug. There remains an urgent need to identify an orally active chelator regimen that is as effective as deferoxamine and has an acceptable degree of tolerability.
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PMID:A risk-benefit assessment of iron-chelation therapy. 942 39

MRL-lpr/lpr mice spontaneously develop manifestations of autoimmunity including arthritis, vasculitis, and glomerulonephritis. The paramagnetic molecule nitric oxide has been implicated as an effector molecule in initiation and propagation of these inflammatory conditions. In this study, we utilized electron paramagnetic resonance spectroscopy to directly detect nitrosylated protein complexes as products of nitric oxide in whole blood and in kidneys of MRL-lpr/lpr mice. Electron paramagnetic resonance spectra of blood samples from MRL-lpr/lpr mice showed nitrosyl hemoglobin species. Amounts of blood nitrosyl hemoglobin in MRL-lpr/lpr mice were significantly increased as compared to age-matched control mice. Electron paramagnetic resonance spectra of MRL-lpr/lpr kidney tissue exhibited a signal characteristic of a dinitrosyl-iron-dithiolate complex at g approximately 2.04. Formation of nitrosylated nonheme protein in diseased kidneys is associated with development of glomerulonephritis in the autoimmune mice. The presence of nitrosylated nonheme protein indicates the formation of nitric oxide within the kidneys of the diseased mice signifying in situ renal nitric oxide formation.
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PMID:Nitrosylation of blood hemoglobin and renal nonheme proteins in autoimmune MRL-lpr/lpr mice. 943 30

Jehovah's Witness who require operation represent a challenge to the physician because of the patients' refusal to accept blood transfusion. We report an 8-year-old male of Jehovah's Witness who underwent a surgical treatment of infective endocarditis. He was transferred to our hospital because of high fever and heart murmur. Echocardiogram revealed a developing vegetation of aortic cusps and an aneurysmal change of the non-coronary sinus Valsalva. On admission he was complicated by anemia, purulent meningitis and suppurative arthritis of left knee. There were no signs of cardiac failure. Erythropoietin (6000 U thrice weekly) and iron (60 mg daily) were given for 11 weeks prior to surgery, raising the hemoglobin level from 9.2 g/dl to 18.4 g/dl. Aortic valve replacement and plasty of the sinus Valsalva were then performed. Intraoperatively hemoglobin concentration dropped to 10.3 g/dl and it raised to 15 g/dl postoperatively. We also used Cell-Saver to reduce blood loss. The patient made an uncomplicated recovery. Erythropoietin therapy contributed substantially to the successful outcome of this case.
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PMID:[Open heart surgery in a Jehovah's Witness boy--a case report of successful management of aortic regurgatation and aneurysm of sinus Valsalva due to infective endocarditis]. 945 16

Genetic hemochromatosis is a disorder of iron metabolism that results in deposition of massive amounts of iron in the tissues. Arthropathy is one of numerous clinical manifestations associated with this disease. Characteristic radiographic features have been reported in the hand and wrist, and the hip; however, there is no mention in the literature of joint manifestations in the foot and ankle. In this report, the authors present three patients with hemochromatosis arthropathy of the foot and ankle. Two patients presented primarily with foot pain and were treated initially with orthoses. One of these patients went on to have to midfoot arthrodesis performed. The third patient presented with ankle joint symptoms, and was treated successfully with an ankle foot orthosis. The arthritis of hemochromatosis has classic radiographic findings. However, the arthritis of hemochromatosis may be difficult to differentiate from several other joint diseases. Characteristic features of this disease in the foot and ankle are discussed.
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PMID:Hemochromatosis of the foot and ankle. Report of three cases and review of the literature. 958 73


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