Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with juvenile rheumatoid arthritis may have an anemia attributable to the chronic disease, to iron deficiency, or to a combination of the two. The contribution of iron deficiency is often difficult to determine by routine laboratory studies. We studied 51 patients with pauciarticular and polyarticular juvenile rheumatoid arthritis with red blood cell counts, indices, free erythrocyte protoporphyrin, and serum ferritin. Fifteen of the 18 who were anemic were restudied after a 3 to 6-month period of iron therapy. Thirteen of the 15 responded by these criteria: a rise in hemoglobin of 1.0 gm/dl or more and an increase in mean corpuscular volume of 3 fl or more; in 11 of these 13, hemoglobin values returned to the normal range for age. These findings indicate that iron deficiency can be a major component of the anemia that is commonly found in patients with active juvenile rheumatoid arthritis.
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PMID:Anemia in patients with juvenile rheumatoid arthritis. 66 Mar 58

Still's disease was reported to be a type of Juvenile Rheumatoid Arthritis (JRA) by Still in 1897. Adult-onset Still's disease is an important clinical entity inducing fever, skin rash and polyarthritis. Spiking fever and rash are characteristic features for early diagnosis. Although chronic polyarthritis is similar to RA, ankylosis of hand joint is characteristic for Still's disease rather than destructive change. Increased ESR, negative autoantibodies, leukocytosis, liver dysfunction and hyperferritinemja are major laboratory findings. A markedly increased level of serum ferritin can be used, not only as an indicator of disease activity, but also as a diagnostic marker of the disease. For therapy, a moderate dose of steroid is the most effective.
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PMID:[Adult Still's disease]. 158 58

Twenty children with systemic juvenile rheumatoid arthritis, aged 0.9-13.7 years, were studied with regard to their serum ferritin concentration at diagnosis and during follow-up, ranging from 2 to 9 years. At diagnosis, during fever, the concentration was extremely high. The median value was 935 micrograms/l. The values were unrelated to other manifestations of the disease or laboratory findings. During glucocorticoid treatment, the serum ferritin concentrations normalized rapidly, usually within a few weeks. The rate of normalization reflected the response of the fever to treatment. Later, subnormal concentrations were found, which were unrelated to the activity of the arthritis. Thus, serum ferritin is a useful guide when tapering glucocorticoid dosage.
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PMID:Ferritinemia as an indicator of systemic disease activity in children with systemic juvenile rheumatoid arthritis. 395 79

Anaemia is a common manifestation of juvenile rheumatoid arthritis (JCA). We have evaluated 26 JCA patients with anaemia and compared their laboratory parameters to those without anaemia. In the patients with anaemia, activation criteria such as erythrocyte sedimentation rate (ESR) and CRP were significantly higher than in those without anaemia. Anaemia was present in all systemic JCA patients and was present in 42% and 78% of the oligoarticular and polyarticular types, respectively. Serum iron levels and transferrin saturations were low in all, whereas serum iron-binding capacities of the patients were normal. Mean ferritin level was 249pg/l (range 8.46-1000pg/l). There was a significant correlation between ferritin levels and CRP and ESR (r = 0.48 and r = 0.55 respectively) (both p < 0.05). Epo levels were normal. Twelve (60%) of the bone marrow aspiration specimens stained positive for iron whereas 40% stained negative; there were also changes suggestive of myelodysplasia. Sideroblasts were also decreased in number. Thus, in these patients iron is not sufficiently transferred to the erythroid series and/or cannot be used by erythroblasts, accompanied by a possible absolute iron deficiency. Thus we suggest that the iron in JCA tends to be stored in the form of ferritin, not in an accessible form and impaired metabolism along with other factors are effective in the anaemia of JCA.
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PMID:Anaemia in juvenile chronic arthritis. 879 53

We encountered seven children with hemophagocytic syndrome complicated with collagen diseases of whom five were JRA and two SLE, HPS was seen as one of initial symptoms in 6 cases excluding one case of JRA. Hematologically, cytopenia in 2 cell lines (RBC and platelets) was seen in most cases. Active hemophagocytosis by matured large histiocytes was observed in all cases. All patients had moderately to extremely high serum ferritin levels and LDH levels. Although both lives did not correlated with prognosis in HPS with collagen disease, the higher serum total cholesterol/serum triglyceride ratio, the worse was the prognosis. Those findings suggested that HPS in collagen diseases may be a different entity from other types of HPS.
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PMID:[Seven cases of hemophagocytic syndrome complicated with childhood collagen diseases]. 891 Oct 81

We have attempted to investigate the dysplastic changes in the hematopoietic system associated with juvenile rheumatoid arthritis (JRA) and its relation to disease activity. The peripheral blood smear and bone marrow aspiration samples of 17 JRA patients were investigated and correlations with laboratory parameters of disease activity sought. The age range was 6-16 years and the duration of disease 1.5-108 months. Abnormal finding of the peripheral smear and bone marrow were scored separately. The score of pathological peripheral blood findings correlated significantly with CRP and ferritin (both P <0.05). In the bone marrow specimens marked changes were noted in the myeloid, erythropoietic, and megakaryopoietic series; however, the score of pathological findings did not correlate with laboratory parameters of disease activity (P > 0.05). We suggest that JRA is associated with marked myelodysplastic changes, also manifested in the peripheral blood smear; these changes may well be the consequence of the inflammatory milieu, including cytokines, during active disease.
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PMID:Myelodysplastic features in juvenile rheumatoid arthritis. 903 94

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperferritinemia and thrombocytosis were noted, in addition to leukocytosis, positive C- reactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.8%) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Non-steroidal anti-inflammatory drugs were used in almost one-third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti-rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.
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PMID:Clinical analysis of 570 cases with juvenile rheumatoid arthritis: results of a nationwide retrospective survey in Japan. 914 Dec 66

Adult onset Stills disease (ASD), an adult variant of systemic onset juvenile rheumatoid arthritis, is a rare disease entity. The diagnosis is solely a clinical one and often difficult. Clinical and laboratory features are not pathognomonic. The diagnosis of ASD has to be considered in patients with high spiking fever, transient rash, arthralgias, oligo- or polyarticular arthritis, leukocytosis, sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction and high serum ferritin levels. We give a brief review of the clinical features, differential diagnosis, treatment and prognosis.
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PMID:[Still disease in adults]. 962 78

Adult-onset Still's disease, the adult variant of the systemic form of juvenile arthritis, is an uncommon systemic inflammatory disorder of unknown etiology characterized by high spiking fevers, neutrophilic leukocytosis, arthritis, and an evanescent rash. There is often a delay in reaching a firm diagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value in establishing the diagnosis. Clinical response to high-dose corticosteroids may be dramatic. We report a case of a 29-year-old woman who had recently been investigated for fever of unknown origin, and who presented to our hospital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and shock. The patient had a markedly elevated serum ferritin concentration of 26,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as septic shock, has not been reported previously.
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PMID:Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease: clinical response with high-dose pulse methylprednisolone therapy. 1037 76

A 26-year-old woman presented with general fatigue, persistent fever, nuchal lymphadenitis, thrombocytopenia, and liver damage. From the bone marrow finding, we diagnosed her condition as hemophagocytic syndrome. Steroid pulse therapy, cyclosporin A treatment, and combined chemotherapy generated no response. The patient showed severe mucosal bleeding, rapidly experienced multiple organ failure, and finally died of a brain hemorrhage on the 13th hospital day. Epstein-Barr virus, cytomegalovirus, human herpes virus type 6, human parvovirus B19, and herpes simplex virus were not detected. Autopsied samples of the spleen, bone marrow, and liver showed extreme proliferation of activated macrophages, so-called histiocytes, without lymphoid malignancy. The interferon gamma level at presentation was prominently high. The continuously elevated levels of ferritin and soluble interleukin 2 receptor were correlated with the catastrophic outcome. The disease in our case mimicked infantile hemophagocytic lymphohistiocytosis. However, there was neither a family history of the disease nor a mutation in the perforin gene. So, it is reasonable to categorize our case as macrophage activation syndrome. Although our patient lacked arthritis or eruption, we cannot deny the possibility that an oligoarthritis type of systemic-onset juvenile rheumatoid arthritis or, considering the patient's age, adult-onset Still disease lies at the base of our case.
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PMID:Fulminant hemophagocytic syndrome with a high interferon gamma level diagnosed as macrophage activation syndrome. 1523 1


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