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)

Fever of unknown origin (FUO) in adults is a commonly encountered clinical problem. Treatable causes of FUO in the adult should be the primary focus of the diagnostic workup. Neoplasms have replaced infectious diseases as being the most common cause of FUO in adults, and collagen vascular diseases are now relatively rare. The most important collagen vascular diseases presenting as an FUO include Takayasu's arteritis, Kikuchi's disease, polymyalgia rheumatica, and adult juvenile rheumatoid arthritis (JRA) (adult Still's disease). There are no specific diagnostic tests for these disorders, which commonly present as prolonged fevers that are not easily diagnosed (i.e., FUO). Adult JRA is a rare but important cause of FUO in adults. Typically, patients with adult Still's disease present with liver/spleen involvement, posi-articular arthritis, ocular involvement, and evanescent salmon-colored truncal rash. An important diagnostic finding in adult JRA is the presence of a double quotidian fever, which occurs in few other disorders. Only visceral leishmaniasis and adult JRA are causes of FUO in adults associated with double quotidian fevers. Highly elevated serum ferritin levels are the most important nonspecific diagnostic finding associated with adult JRA. We present a case of FUO caused by adult JRA presenting with diffuse polyarticular migrating arthritis, evanescent rash, and splenomegaly. The diagnosis of adult JRA was suggested by these findings in association with a double quotidian fever and a highly elevated serum ferritin level. Clinicians should appreciate the diagnostic significance of fever patterns and the diagnostic significance of elevated serum ferritin levels in patients with FUO.
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PMID:Fever of unknown origin caused by adult juvenile rheumatoid arthritis: the diagnostic significance of double quotidian fevers and elevated serum ferritin levels. 1559 97

Reactive hemophagocytic syndrome (HPS) is characterized by hemophagocytosis by activated histiocytes, resulting in pancytopenia and liver dysfunction. We describe a patient with adult onset Still's disease (AOSD) in whom HPS developed. An 80-year-old Japanese woman with high fever, arthralgia, skin rash, and pleuritis was admitted to our hospital for further examination. She was diagnosed with AOSD and steroid therapy was initiated. During the course of steroid therapy, a re-elevation of serum ferritin levels and a marked increase in serum transaminase were observed. Bone marrow aspiration revealed an increase in the number of histiocytes with hemophagocytosis and cytomegalovirus (CMV)-positive leukocytes were detected. At this time we diagnosed the patient as having virus-associated hemophagocytic syndrome (VAHS) and elevated levels of trasaminase and ferritin were normalized by ganciclovir treatment. Reactive HPS occurs in cases of active AOSD. However, it should be noted that HPS may be accompanied by opportunistic infections during immunosuppressive therapy requiring prompt antibiotic therapy.
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PMID:Cytomegalovirus-associated hemophagocytic syndrome in a patient with adult onset Still's disease. 1578 96

Heme oxygenase-1 (HO-1), an inducible heme-degrading enzyme, is expressed by macrophages and endothelial cells in response to various stresses. Because ferritin synthesis is stimulated by Fe2+, which is a product of heme degradation, we examined the relation between HO-1 and ferritin levels in the serum of patients with hemophagocytic syndrome (HPS), adult-onset Still's disease (ASD), and other diseases that may cause hyperferritinemia. Seven patients with HPS, 10 with ASD, 73 with other rheumatic diseases, 20 with liver diseases, 10 recipients of repeated blood transfusion because of hematological disorders, and 22 healthy volunteers were enrolled. Serum HO-1 and ferritin levels were determined by ELISA. Expression of HO-1 mRNA and protein by peripheral blood mononuclear cells (PBMCs) was determined by real-time PCR and immunocytochemical techniques, respectively. Serum levels of HO-1 were significantly higher in patients with active HPS and ASD than in the other groups (P < 0.01). HO-1 levels were not elevated in patients with other causes of hyperferritinemia but were moderately elevated in patients with dermatomyositis/polymyositis. Among patients with HPS and ASD, serum HO-1 levels correlated closely with serum ferritin levels, and the levels of both returned to normal after therapy had induced remission. Increased expression of HO-1 mRNA was confirmed in PBMCs from some patients with HPS and ASD. Hyperferritinemia correlated closely with increased serum HO-1 in patients with HPS and ASD but not other conditions, indicating that measurement of serum HO-1 and ferritin levels would be useful in the differential diagnosis of hyperferritinemia and perhaps also in monitoring disease activity in HPS and ASD.
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PMID:Increased serum HO-1 in hemophagocytic syndrome and adult-onset Still's disease: use in the differential diagnosis of hyperferritinemia. 1589 48

The authors report three cases of adult-onset Still's disease with severe hypoxemic pulmonary involvement, mimicking severe pulmonary sepsis. Clinicians must be aware of this rare form of such disease. Low (<20%) glycosylated ferritin level in the presence of unexplained prolonged fever with leukocytosis can help in the diagnosis.
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PMID:Acute respiratory failure revealing adult-onset Still's disease: diagnostic value of low glycosylated ferritin level. 1628 19

The subject was a 22-year-old woman who developed high fever and arthralgias and eruptions in the extremities around June 2005. She sought medical advice at a nearby dermatology clinic, where hepatic dysfunction was noted on blood testing. The patient was thus hospitalized the next day. Although CRP levels were significantly high, no sign of infection was observed and bone marrow cell differentiation was normal. Adult onset Still's disease was diagnosed based on the observation of persistent high fever >39 degrees C, eruptions, increased leukocytes, pharyngeal pain, splenomegaly, hepatic dysfunction, negative autoantibody results from blood testing, and high serum ferritin levels. Administration of prednisolone 30 mg/day was initiated, but proved ineffective. Steroid pulse therapy was conducted, and the subject was transferred to our medical facility for continued treatment. Attempts were made to control the disease using combined steroid and cyclosporine administration; but exacerbation of high serum ferritin levels and hepatic dysfunctions were observed, so a second course of steroid pulse therapy was conducted. Symptoms improved temporarily, but steroid levels were difficult to reduce. Cyclosporine was therefore replaced by methotrexate, and administration of infliximab was initiated. In the course of treatment, administration of a sulfamethoxazole/trimethoprim combination was initiated, but was discontinued due to suspicion of drug-induced hepatic injury. A second administration of infliximab was conducted in late August, and rapid improvements in clinical symptoms and abnormal test values was observed. However, high fever and headache developed suddenly in early September. Based on the results of spinal fluid testing, blood and spinal fluid cultures and MRI of the head, Listeria meningoencephalitis was diagnosed. Diplopia and impaired consciousness occurred during the disease course, and formation of a brain abscess was observed on imaging. However, symptoms were controlled by long-term combination administration of ampicillin and gentamicin. Administration of infliximab was discontinued for treatment of adult onset Still's disease, and steroid levels were reduced following double-membrane filtration plasma exchange. On follow-up, no relapse of symptoms or abnormalities in blood test values were observed, so the subject was discharged from our medical facility in December 2005. In treatment for rheumatic diseases, a dramatic improvement in treatment results for pathologies displaying tolerance against conventional treatments has been acquired with the development of biological drugs. However, opportunistic infections represent a serious problem, and appropriate preventative measures are required. The present report describes a case in which the subject was affected by Listeria meningoencephalitis during administration of infliximab for steroid-dependent adult Still's disease. Since listeriosis is one of the complications, along with tuberculosis, that warrants precautionary measures, this case is reported and discussed.
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PMID:[A case in which the subject was affected by Listeia meningoencephalitis during administration of infliximab for steroid-dependent adult onset Still's disease]. 1681 65

Fever of unknown origin (FUO) is a common clinical diagnostic dilemma. In the elderly, causes of FUO most commonly include malignancy or infection, and less commonly include collagen vascular diseases. Among the collagen vascular diseases causing FUO in the elderly, polymyalgia rheumatica/temporal arteritis, and adult Still's disease (adult juvenile rheumatoid arthritis) are difficult diagnoses to prove. Among the infectious causes of FUO in the elderly are subacute bacterial endocarditis, intra-abdominal abscesses, and extrapulmonary tuberculosis. In the elderly, neoplastic causes of FUO include lymphomas, hepatomas, renal cell carcinomas, and hepatic or central nervous system metastases. Acute leukemias, particularly during "blast" transformation, may present as acute fevers in the absence of infection, but are rare causes of FUO. Preleukemia/myelodysplastic syndromes are exceedingly rare causes of FUO. We present a case of an elderly man who presented with findings that initially suggested adult Still's disease. Prolonged and profound monocytosis provided the key clue to his subsequent diagnosis of preleukemia/myelodysplastic syndrome. In this patient, a positive Naprosyn test result also suggested a neoplastic cause for his FUO. After months of prolonged fevers, myelocytes/metamyelocytes were eventually demonstrated in his peripheral smear during hospital evaluation. These findings, in concert with the persistent monocytosis, highly elevated ferritin levels, polyclonal gammopathy on serum protein electrophoresis, and eventual presence of myelocytes/metamyelocytes on peripheral smear, prompted a bone marrow test that demonstrated blast cells confirming the diagnosis of preleukemia myelodysplastic syndrome as the cause of this patient's FUO.
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PMID:Fever of unknown origin due to preleukemia/myelodysplastic syndrome: the diagnostic importance of monocytosis with elevated serum ferritin levels. 1686

This study aims to report on the clinical and laboratory picture and the disease course and outcome in patients having adult onset Still's disease (AOSD), to briefly review existing literature on the subject, and to compare our findings with those previously reported. Results are reported for 28 patients with AOSD satisfying the preliminary criteria of Yamaguchi et al. seen in a teaching hospital over the last 10 years. A high percent of the patients with AOSD were women. The mean (+SD) age at disease onset was 27.8 (+8.4) years. We found fever in 100%, rash in 85%, arthritis in 64%, lymphadenopathy in 60%, splenomegaly in 57%, hepatomegaly in 35%, pleural effusion in 17.9%, and pericardial effusion in 3.6% of our patients. Leukocytosis was present in 96% of the patients, a normochromic, normocytic anemia in 54%, and an elevated erythrocyte sedimentation rate (ESR) in all. Serum ferritin levels were raised in 89% of the patients. The mean follow-up of the patients was 3.72 + 2.46 years. The mean delay in diagnosis was 7.32 + 18.0 months. The mean time to enter remission was 9.7 months. Self-limited, intermittent, and chronic disease course was seen in 14.3, 57.1, and 28.6% of patients, respectively. The outcome was good in about 89% of patients, and mortality was nil. No particular clinical or laboratory variable was found to predict the subsequent disease course and outcome in our patients. On comparing our data with important previous series, we found a higher percentage of women and of patients presenting in the age group 16-35 years, a lower frequency of arthritis and pericardial effusion, and some other notable differences. Importantly, the disease course was benign, probably as an outcome of heightened awareness and less diagnostic delay than in the past, allowing for early, aggressive, and appropriate treatment. It is concluded that AOSD is now a relatively benign disease if diagnosed early and treated appropriately.
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PMID:Ten years of clinical experience with adult onset Still's disease: is the outcome improving? 1708 84

A 49-year-old woman was admitted to our hospital because of fever of unknown origin. The patient had long-lasting spiking fever, hepatosplenomegaly, pleural effusion, and skin rash. Laboratory tests showed marked leukocytosis and an extremely high serum ferritin level (240 000 ng/ml) accompanied by disseminated intravascular coagulation and hemophagocytic syndrome. Most of the patient's features were compatible with a diagnosis of adult-onset Still's disease (AOSD), the rash, however, was not a typical rheumatoid rash but multiforme erythema. Biopsy of a breast nodule revealed breast cancer, leading us to a diagnosis of paraneoplastic syndrome mimicking AOSD. Although this is a rare disorder, cases resembling the present one have been reported, indicating the importance of including paraneoplastic syndrome in the differential diagnosis of AOSD.
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PMID:A case of paraneoplastic syndrome mimicking adult-onset Still's disease. 1714 3

We report on an 83-year-old Japanese woman with adult-onset Still's disease (AOSD), with marked hypercytokinemia (serum levels of ferritin (Fer) and interleukin (IL)-18 were markedly high). On seeing older patients with fever of unknown origin (FUO), particularly Asians, AOSD should be considered. Reduced doses of oral prednisolone following intravenous methylprednisolone (mPSL) therapy caused a flare-up of AOSD and led to Pneumocystis carinii (jeroveci) pneumonia. Low-dose methotrexate (MTX) therapy was administered as a steroid-sparing agent with good response. Our case suggests that in very elderly people, as in younger patients, MTX is useful for controlling AOSD with marked hypercytokinemia, and avoiding corticosteroid-induced adverse effects.
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PMID:Adult-onset Still's disease in a patient over 80 years old successfully treated with low-dose methotrexate therapy. 1715 15

We report the case of a 60-year-old woman who had recently been examined for fever of unknown origin and who presented to our hospital with high fever and myalgia, weakness, sore throat, and rash. The patient had a markedly elevated serum ferritin concentration of 40,000 ng/mL and positive antinuclear antibodies (ANA) with a titer of 1/200. Despite the presence of positive ANA, the patient was diagnosed as having adult Still's disease (ASD). High-dose steroid therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as sepsis with a positive ANA test, has not been reported previously, at least not in the last 15 years.
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PMID:Adult Still's disease despite the presence of positive antinuclear antibodies. 1733 72


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