Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
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Four adults with an illness similar to the systemic variant of juvenile rheumatoid arthritis seen in children (Still's disease) are described. All four had fever, an erythematous maculopapular rash, splenomegaly and arthritis. The arthritis was asymmetric and involved only a few joints simultaneously. Erosive arthritis developed in one patient. Three patients had a sore throat, two had pleurisy and pericarditis, and one had transient abnormalities of liver function. The laboratory features included anemia, leukocytosis and high leukocyte counts in the synovial fluid. High titres of rubella hemagglutination-inhibiting antibody were detected in two patients, one of whom was found to have rubella virus in the urine. Only one patient responded well to therapy with acetylsalicylic acid; the other three were given prednisone therapy, and two continue to require it.
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PMID:Adult-onset Still's disease. 45 6

Adult onset Still's disease seems to be the adult form of Still's disease in children. The key symptoms of the disease are high spiking fever, arthritis and a macular or maculopapular, salmon-pink evanescent rash, almost always accompanied by neutrophilic leukocytosis and frequently by sore throat, intense myalgias, lymphadenopathy, splenomegaly and signs of serositis. Tests for IgM rheumatoid factor and antinuclear antibody are characteristically negative. With respect to haematologic abnormalities, the disease may give rise to several problems. First, there is a neutrophilic leukocytosis, which currently is unexplained, and often a normocytic normochromic anaemia, that may be profound. The anaemia has the characteristics of anaemia of chronic inflammatory disease. Both abnormalities disappear after effective treatment of the disease or at spontaneous remission. Secondly, there might be a problem to differentiate AOSD from malignant haematological disorders, including malignant lymphoma and leukaemia, especially when the picture is dominated by lymphadenopathy, splenomegaly, fever and leukocytosis. Although in rare cases the differential diagnosis is extremely difficult, diagnosis can mostly be made or excluded by peripheral blood smear staining, bone marrow biopsies and occasionally lymph node biopsy. Finally, like the juvenile counterpart, AOSD is occasionally complicated by sometimes life-threatening diffuse intravascular coagulation. Factors that might be important in the development of this complication include severe disease activity, liver abnormalities and particular drugs including salicylates, other NSAIDs and some slow-acting antirheumatic drugs. Prompt therapy, including withdrawal of the drug, corticosteroids and sometimes anticoagulant therapy have been successfully applied to most patients.
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PMID:Adult-onset Still's disease. 175 84

We reported a case of adult Still's disease accompanied by pericarditis, pleuritis and extensive pneumonitis with respiratory failure. A 59-year-old woman was admitted to our hospital because of high grade fever and sore throat. She had a spiking fever between 38 degrees C and 40 degrees C. Surface lymph nodes were palpable in the neck and inguinal lesions and hepatosplenomegaly was recognized. Laboratory data showed a marked increase in peripheral leukocytes, erythrocyte sedimentation rate, liver dysfunction and anemia. Serologic tests were negative for various autoantibodies and rheumatoid factor. She received various antibiotics, but there was no improvement. Later, a rheumatoid rash which suggested adult Still's disease developed. The diagnosis of adult Still's disease was made by skin biopsy and clinical course. Although pericarditis, pleuritis and extensive pneumonitis were accompanied with severe respiratory failure, her symptoms improved with steroid pulse-therapy. A transbronchial lung biopsy revealed moderate fibrosis and cell infiltration in alveoli.
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PMID:[A case of adult Still's disease with severe pulmonary complications]. 258 6

Four new cases of adult Still's disease have been observed in the past three years. Clinical and biological features are high fever, polyarthralgia, macular rash on forearm, pericarditis, splenomegaly, lymphadenopathy, alopecia, anemia, hyper-leucocytosis, raised ESR. Transient neurological findings may also be observed with abnormal reflexes, cranial nerve paralysis. High dose steroids treatment can be used in case of systemic involvement. The long term prognosis is usually good with treatment. In conclusion, the diagnosis of Still's disease is essentially clinical and should not be mistaken for septicemia.
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PMID:[Still's disease in the adult (author's transl)]. 627 24

Still's disease is characterized by arthritis, fever, rash, lymphoid hypertrophy, leukocytosis, and anemia, often in association with thrombocytosis. We describe a patient with Still's disease and thrombocytopenia secondary to disseminated intravascular coagulation (DIC). Fifteen definite cases of DIC complicating Still's disease have been reported in the English literature. Most developed this complication while receiving high doses of salicylates in association with hepatic dysfunction. In a few, the coagulopathy has been associated with gold therapy. Our patient is only the second reported to develop DIC in the absence of drug therapy and the fourth reported to die from this complication. The clinical features of these 16 patients are summarized and proposed mechanisms of pathogenesis are reviewed.
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PMID:Disseminated intravascular coagulation in Still's disease. 789 78

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

A 37-year-old man presented with symptoms consistent with adult-onset Still's disease. Fever and leukocytosis were prominent, and the patient was started on high-dose aspirin for possible acute rheumatic fever. He developed severe anemia as a result of glucose-6-phosphate dehydrogenase deficiency. His treatment was changed to naproxen, and he recovered with restoration of his hematologic parameters. Although Still's disease is frequently accompanied by mild-to-moderate anemia, the development of severe anemia should raise the possibilities of hemolysis secondary to glucose-6-phosphate dehydrogenase deficiency.
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PMID:Adult-onset Still's disease associated with G6PD deficiency: a case report and literature review. 974 60

Adult-onset Still's disease is a systemic inflammatory disorder with a highly variable clinical course. Mild hepatitis and anemia are common manifestations. We describe a patient with adult Still's disease who developed a severe hepatitis and a life-threatening pure red cell aplasia. The hepatitis developed after treatment with NSAIDs was started. The patient was successfully treated with a combination of prednisone, cyclosporin, and methotrexate. Physicians should be aware that severe hepatitis and pure red cell aplasia can occur in adult Still's disease. We recommend a careful monitoring of liver functions in patients with adult Still's disease who are being treated with NSAIDs.
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PMID:Severe hepatitis and pure red cell aplasia in adult Still's disease: good response to immunosuppressive therapy. 1049 46

In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common.
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PMID:Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. 1274 Jun 70

Pure red cell aplasia (PRCA) associated with adult-onset Still's disease (AOSD) is very rare. In this report a 28-year-old woman was admitted with fever, skin rash, jaundice and anemia. She was diagnosed as having AOSD with PRCA by bone marrow examination. Treatment with high-dose prednisolone and intravenous immunoglobulin resulted in remission of the PRCA and a good response of the AOSD.
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PMID:Pure red cell aplasia and adult-onset Still's disease. 1529 4


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