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)

A 20-year-old woman who had suffered from Still's disease was admitted for fever and progressive pneumonitis after long-term remission. High spiking fever, leukocytosis, splenomegaly and an extremely high serum ferritin concentration strongly suggested a relapse of Still's disease. Intensive therapy with high-dose methylprednisolone, cyclophosphamide and gamma globulin was required for the severe pneumonitis, which was thought to be a rare manifestation in Still's disease.
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PMID:Still's disease relapse with severe pneumonitis after prolonged remission. 820 63

Over a 9-year period, twelve patients (8 boys, 4 girls), from 3 to 14 years old, were diagnosed as having Still's disease. Intermittent spiking high fever, poly- or pauci- articular arthritis, and typical evanescent skin rash were the most prominent clinical features. Hemogram examinations showed that 36% of the patients had anemia, ninety-two percent had neutrophilic leukocytosis and 78% had thrombocytosis. Serologically, none had positive results of rheumatoid factor and anti-nuclear antibody. Serum ferritin level was obtained from six patients and all revealed marked elevation during active disease. C-reactive protein and erythrocyte sedimentation rate were both invariably elevated. Immunologically, elevated serum concentrations of IgG, IgA, and complements (C3, C4) were found in 33%, 20%, and 17%, respectively. Furthermore, eighty percent of patients showed an increased serum level of circulating immune complexes. Aspirin (ASA) was used in all patients, but 92% of them required non-steroid antiinflammatory drugs (NSAIDs) in combination to get a better response. Sixty-seven percent of patients needed corticosteroids to control the acute systemic manifestations. Other disease-modifying agents were also used in 33% of our patients. ASA-induced liver function impairment was found in two cases. In addition, one patient experienced an episode of upper gastrointestinal bleeding. Generally speaking, the overall prognosis was good. One patient (8%) died of internal bleeding after a needle liver biopsy.
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PMID:Still's disease: experience in 12 children. 823 59

Adult Still's disease is a chronic, systemic disease of unknown origin. We describe the case of an otherwise healthy man with an uncommon presentation of Still's disease. A 38-year-old man presented with sore throat, fever, rash and arthritis. Laboratory findings showed that both erythrocyte sedimentation rate and ferritin had increased. Transoesophageal echocardiography revealed a vegetation involving the aortic leaflet. The diagnosis of Still's disease was made after the exclusion of infectious endocarditis, based upon the clinical picture, the high level of ferritin and the follow-up. The patient markedly improved after treatment with prednisone 1 mg. kg-1. This controlled and then progressively reduced the disease; the drug was then withdrawn. This case illustrates that Still's disease can present with endocardial involvement mimicking acute bacterial endocarditis as a first clinical manifestation. The observation suggests that the presence of high ferritinaemia in a patient with some clinical criteria of Still's disease could lead to an early diagnosis.
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PMID:Atypical presentation of adult Still's disease mimicking acute bacterial endocarditis. 874 17

The case of a 75-year-old Japanese woman with adult-onset Still's disease who presented with cerebral haemorrhage is described. She had been in clinical remission for 2 years, after induction therapy including non-steroidal anti-inflammatory drugs, prednisolone, cyclophosphamide and mizoribine followed by auranofin, until her cerebral haemorrhage occurred, although her serum level of ferritin had gradually increased. After the onset of cerebral haemorrhage, the patient's serum level of thrombomodulin was elevated although c-reactive protein and lactate dehydrogenase were not increased. Anti-cardiolipin antibody and lupus anti-coagulant were not detected. Patients with adult-onset Still's disease are rarely reported to develop cerebral vascular disease, possibly because the disease is most frequent in young adults. The cerebral haemorrhage may have been caused by the vasculitis due to Still's disease.
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PMID:Cerebral haemorrhage complicating adult-onset Still's disease: a case report. 895 35

We report here a case of adult-onset Still's disease (AOSD), who finally responded to a combination of cyclophosphamide (CPA) and gold sodium thiomalate (GST) after two years of active disease. A 23-year-old man having continuous high fever with skin rash, polyarthralgia and increased serum ferritin, was diagnosed as AOSD, and oral corticosteroid was initially effective. His symptoms recurred one year later without clinical improvement to increased dosage of steroid. He was admitted to our hospital with pericarditis and pleural effusion but did not respond to either intravenous (i.v.) pulse steroid therapy, methotrexate (MTX) or high dose i.v. gamma-globulin. He was partly responsive to monthly i.v. injection of CPA, but clinical symptoms did not completely subside and hyperferritinemia persisted. GST, initiated in combination with CPA, however, was successful to induce complete remission. MTX has recently been reported to be efficacious to steroid-resistant AOSD, but CPA and gold compounds might be useful to refractory case of AOSD.
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PMID:[A refractory case of adult-onset Still's disease]. 925 12

In April 1988, a 23 year-old woman developed high fever, arthralgia, eruptions and splenomegaly. She was treated with non Steroid anti-inflammatory drugs, and the symptoms disappeared. In June 1991, she was diagnosed as adult Still's disease and treated with prednisolone. In July 1994, she was treated with pulse therapy methylprednisolone due to high fever, eruptions, arthralgia and the high levels of ferritin. However, due to the marked increase of serum transaminase and bilirubin levels, she was referred to University hospital. She developed hepatic failure after admission Bone-marrow puncture revealed hemophagocytosis. She died ten days after admission. She was diagnosed as hemophagocytic syndrome combined with acute hepatic failure.
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PMID:[A case of hemophagocytic syndrome with severe liver injury manifestating adult Still's disease]. 939 9

Crossed affinoimmunoelectrophoresis with Con A as a ligand was used to examine the microheterogeneity of alpha1-acid glycoprotein (AGP) and alpha1-antichymotrypsin (ACT) in sera of patients with child-onset and adult-onset Still's disease. The reactivity of both proteins was increased in sera of adults and decreased in sera of children with active disease, when compared with normal values. We also found statistically significant differences in serum concentration of ACT and ferritin in both diseases. This result suggests different pathogenic mechanisms of Still's disease in children and adults. Serum concentration of ferritin and ACT could be of value as a combined marker for the adult, but not the juvenile form of Still's disease.
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PMID:Still's disease in children and adults: a distinct pattern of acute-phase proteins. 969 67

The authors report 5 cases of Still's disease in adults whose symptoms were mainly characterised by high fever, transient exanthema, polyarthralgia and/or polyarthritis, lymphoadenomegaly, splenomegaly and neutrophil leukocytosis. Assays for leukocytosis were positive, as were those for inflammatory markers and serum ferritin was also high in all 3 patients in which it assayed. On the contrary, serum ferritin latex test, Waaler-Rose reaction and all other tests commonly used to diagnose long-term fevers were all negative. All the subjects examined recovered after prolonged steroid therapy. Only one patient reported severe sequelae in the hip joints and subsequently underwent bilateral hip replacement surgery.
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PMID:[Adult-onset Still's disease. Report of 5 cases]. 1051 58

We report the case of a 55-year-old Japanese woman with adult onset Still's disease in whom hemophagocytic syndrome and severe liver dysfunction developed. High serum levels of ferritin, macrophage colony stimulating factor and interferon-gamma, which imply the presence of hemophagocytic syndrome, were detected. It is known that hemophagocytic syndrome is associated with adult onset Still's disease. In our case, many markedly swollen Kupffer cells with phagocytized red blood cells were found in the liver, as well as macrophages in the bone marrow and spleen. Accordingly, we believe that severe liver dysfunction in this case may have been related to hypercytokinemia due to hemophagocytic syndrome.
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PMID:Adult onset Still's disease with hemophagocytic syndrome and severe liver dysfunction. 1070 7

The clinical manifestations, treatment and course, and articular outcomes of 24 children with juvenile-onset Still's disease (JOSD) and 21 adults with adult-onset Still's disease (AOSD) were compared retrospectively. There was no significant difference in the initial clinical and laboratory manifestations except that more adults presented with a sore throat (81% vs. 46%, p = 0.03). Although serum ferritin was almost always elevated in both diseases, adults had significantly higher serum ferritin concentrations compared with those of children. Steroid treatment was required in 71% of children and 52% of adults, while disease-modifying antirheumatic drugs were used in 42% of children and 24% of adults during the course. Chronic arthritis (>6 months) occurred in comparable proportions of patients with JOSD and AOSD (46% vs 38%, p = 0.82), irrespective of the disease pattern (monocyclic or polycyclic). However, severe deforming arthritis with marked functional limitation occurred only in JOSD, particularly with polyarthritis at disease onset (more than five affected joints). In contrast, AOSD patients with chronic arthritis had a favourable functional outcome at the end of the follow-up. Our study suggested different articular outcomes of Still's disease in Chinese children and adults.
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PMID:Different articular outcomes of Still's disease in Chinese children and adults. 1079 24


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