Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The patient was a 29-year-old woman. She was well until autumn 1983, when she presented with polyarthralgia, fever above 39 degrees C, hepatosplenomegaly, swelling of lymphnode and salmon pink rash. Laboratory tests revealed marked leucocytosis with shift to the left, elevated ESR, strong positivity of CRP and abnormal liver function tests. However, anti-nuclear antibody and RA factor were negative. She was diagnosed as adult onset Still's disease (AOSD) by characteristic clinical course and laboratory data. During her disease course these abnormal findings could be well controlled neither by nonsteroidal anti-inflammatory drugs, immunosuppressive agents nor corticosteroids. Two and half years after the first admission, she began to complain of dry cough, dyspnea on efforts. Auscultation revealed an increased pulmonic sound and systolic murmur of cardiac apex. Chest X-Rays showed enlarged main pulmonary arteries. The lung fields were normal. Pulmonary function tests gave no evidence of a significant obstructive or restrictive defect but showed the low DLco and hypoxemia. Ventilation-perfusion lung scanning failed to reveal pulmonary embolism. Finally, right heart catheterization confirmed the pulmonary precapillary hypertension. Her pulmonary hypertension has progressed rapidly, strongly suggesting poor prognosis. Her pulmonary hypertension associated with no apparent parenchymal involvement was thought to be caused by a pulmonary vascular change probably related to AOSD. This case is a first case of AOSD with pulmonary hypertension.
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PMID:[A case of adult Still's disease with pulmonary hypertension]. 237 40

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

A 23-yr-old woman in her sixth month of pregnancy presented with the systemic variant of juvenile rheumatoid arthritis (Still's disease). The symptoms included high fever, erythematous evanescent maculopapular rash, hepatosplenomegaly and asymmetric arthritis. During a follow-up period of 1 yr, anti-inflammatory drugs (both steroidal and nonsteroidal) were administered, leading to the eventual disappearance of the patient's complaints. Adult-onset Still's disease reported in the literature is reviewed, and the association of Still's disease with pregnancy is discussed.
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PMID:Adult Still's disease associated with pregnancy. 717 71

This report describes the fatal outcome of a case of adult onset Still's disease in a 46-year old man. The diagnosis was made according to the 1992 criteria, proposed by Yamaguchi. Nine months after the initial disease manifestations a rapid deterioration with progressive hepatosplenomegaly developed. In parallel, pancytopenia and marked hyperferritinemia could be detected. Transjugular liver biopsy revealed the presence of a hemophagocytic syndrome. The course of the disease was refractory to any form of treatment and the patient died from disseminated intravascular coagulation, hepatic and pulmonary failure. Pathogenetic mechanisms and possible associations between Still's disease and reactive hemophagocytic syndrome are discussed.
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PMID:[Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome]. 934 Apr 75

We report the case of a 50-year-old man with a 4-year history of high spiking fever accompanied by a widespread, urticarial, non-pruritic or only sometimes mildly pruritic eruption and arthralgia. He also had generalized lymphadenopathy, hepatosplenomegaly, and hyperosteoses of the lower lumbar spine. Laboratory examination revealed an elevated erythrocyte sedimentation rate, elevated white blood cell and platelet counts, hypoalbuminemia, and elevated serum IgM with IgM kappa monoclonal immunoglobulin. We diagnosed his condition as Schnitzler's syndrome, in contrast to the diagnosis of adult onset Still's disease, for which he had been initially followed up by his internist. We compare clinical and histopathological findings for both diseases and, as this patient meets two of the six existing diagnostic criteria for adult onset Still's disease, we propose that Schnitzler's syndrome is an important entity to be added to the list of differential diagnoses for adult onset Still's disease.
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PMID:Schnitzler's syndrome versus adult onset Still's disease. 964 52

A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud's phenomena for 2 months. He was initially diagnosed with adult-onset Still's disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman's disease. The patient's symptoms disappeared soon after excision of the lymph nodes. evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman's disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman's disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla.
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PMID:Localised hyaline vascular type of Castleman's disease mimicking adult-onset Still's disease. 1063 75

We describe a 21-year old man who was diagnosed as having adult onset Still's disease (AOSD) in association with isotretinoin treatment for acne conglobata. The patient was febrile, with a macular salmon pink rash, arthritis, hepatosplenomegaly, and axial lymphadenopathy. Laboratory results showed leukocytosis, mild liver dysfunction and negative rheumatoid factor and antinuclear antibodies. Isotretinoin, an orally active derivative of vitamin A, has been associated with various rheumatologic conditions such as arthralgia, myalgia, vasculitis and arthritis. The etiology of rheumatic disorders associated with retinoids is still obscure; however, it is presumed that immunomodulation by several mechanisms (such as an alteration of the cytokine balance) is probably ascribable to this interesting association.
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PMID:Isotretinoin-induced adult onset Still's disease. 1107 5

We report the case of a woman with a characteristic transient skin rash, fever, severe polyarthritis, hepatosplenomegaly, lymphadenopathy and myalgia. The clinical and laboratory data led to a diagnosis of adult-onset Still's disease. The elevated levels of serum ferritin and caeruloplasmin could be important as diagnostic indicators.
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PMID:Elevated serum caeruloplasmin level in a patient with adult Still's disease. 1195 55

A 31-year-old man with Crohn's disease developed arthritis, spiking fever, and skin rash indistinguishable from that of adult-onset Still's disease. He was admitted to our hospital because of a periumbilical intestinal skin fistula. Crohn's disease had been diagnosed in 1991, and had required intestinal resection twice, and schizophrenia had been diagnosed in 1993. He developed polyarthritis and spiking fever, accompanied by a macular skin rash on both forearms. Marked hepatosplenomegaly and bilateral pleural effusion were detected on computed tomography examination. These findings are indistinguishable from those of adult-onset Still's disease. Because his mental status had deteriorated following high-dose prednisolone on a previous admission, he was treated with an immunosuppressive agent on this occasion, with the treatment being successful. This is the first report of adult-onset Still's disease complicating Crohn's disease. In patients with Crohn's disease, polyarthritis and skin rash can easily be misdiagnosed as enteropathic arthritis with erythema nodosum associated with the Crohn's disease. Although adult-onset Still's disease may not be fatal, early diagnosis is important because it can, in rare cases, result in life-threatening complications.
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PMID:Crohn's disease complicated by adult-onset Still's disease. 1456 35

Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.
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PMID:Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. 1496 57


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