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

Clinical and pathological findings are reported in a Japanese girl who died of secondary amyloidosis associated with juvenile rheumatoid arthritis two years after the onset of symptoms. The patient had intermittent high fever, rheumatoid rash, polyarthralgia, and hepatosplenomegaly. The joints showed the typical histologic changes of juvenile rheumatoid arthritis. Amyloid deposition was found in various tissues; however, remarkable deposition of amyloid was observed in the gastrointestinal tract, especially in the ileum. The amyloid protein in this patient was identified as protein AA using the methods of potassium permanganate treatment and the peroxidase-antiperoxidase unlabeled antibody technique.
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PMID:Amyloidosis associated with juvenile rheumatoid arthritis. 707 94

A 46-year-old man with primary myelofibrosis developed polyarthralgia. Marked hepatosplenomegaly was noted, and hematological examinations revealed a white cell count of 25,600/microliters with 42% promyelocytes and thrombocytopenia. The promyelocytes were positive for CD4 antigen and nonspecific esterase as well as peroxidase. Cytogenetic analysis of circulating mononuclear cells showed the trisomy of No. 22 chromosome in 3 of 5 cells examined. Four months later, the patient became asymptomatic, and hematological picture and hepatosplenomegaly returned to the original level. This is the first report describing the transient promyelocytic expansion in myeloproliferative disorders.
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PMID:[Transient promyelocytic expansion in primary myelofibrosis]. 756

A rare simultaneous occurrence of multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma was diagnosed in a 70-year-old man who presented with fever, polyarthralgia, weight loss, vascular purpura, anemia, generalized lymphadenopathy, and hepatosplenomegaly. He had no risk of HIV infection and serological tests for HIV were negative twice, but a low number of T-cells and a reversed CD4/CD8 ratio were observed. During hospitalization, he developed Kaposi's sarcoma at the right sole. Lymph node biopsies revealed multicentric Castleman's disease together with a large B-cell lymphoma, which showed monotypic IgM-lambda lymphocytes. To our knowledge, this is the first report in which systemic manifestations of all three diseases occurred simultaneously prior to any specific treatment. The altered immune status and human herpesvirus-8 infection might have played a role in the pathogenesis of this occurrence.
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PMID:Multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma: a rare simultaneous occurrence. 1240 98

A 58-year-old Japanese woman developed spiking fever, polyarthralgia and hepatosplenomegaly, with highly elevated levels of c-creactive protein (CRP) and ferrtin, and elevated erythrocyte sedimentation rate (ESR). The AOSD was diagnosed according to the Yamaguchi-criteria of 1992. She was first treated with a combination of prednisolone (20 mg/day) and oral methotrexate (MTX) (7.5 mg/week). This combination, however, was not effective with tapering the dose of prednisolone. When a high dose of cyclosporin A (CyA) (5.5 mg/kg/day) was then added to MTX (5 mg/week), the patient's fever and polyarthralgia decreased, and her elevated serological parameters such as CRP and ESR also gradually declined. Finally, the dose of prednisolone was tapered to 10 mg/day. Only a few cases of AOSD treated with CyA plus MTX have been reported. Thus, further careful observation will be needed to establish the usefulness of this drug combination as a therapy for AOSD.
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PMID:[Adult onset Still's disease (AOSD) treated with a combination of prednisolone, cyclosporin A, and methotrexate]. 1459 57

We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
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PMID:An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood. 1756 52

We report a patient with acute heart failure due to human parvovirus B19 infection. The patient was a 36-year-old man with polyarthralgia, fatigue and swelling of his upper eyelids and all four limbs. These symptoms disappeared, but 5 days after the first consultation, the patient presented with severe exertional dyspnoea, chest pain and swelling of his whole body. Erythema was observed on the skin of hands, fingers and abdomen. Pleural and pericardial effusion, ascites and hepatosplenomegaly were detected. Laboratory examination showed positive results for anti-human parvovirus B19 IgM and B19 DNA in the serum. A diagnosis of acute heart failure by pericarditis caused by B19 was made. This case report suggests that B19 should be considered as a cause of acute heart failure through acute pericarditis.
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PMID:Acute heart failure associated with human parvovirus B19 infection. 1835 55

The objective of this study is to evaluate the clinical features and prognosis of adult-onset Still's disease (AOSD). One hundred and four AOSD patients who were analyzed retrospectively were enrolled in this study. Medical charts were systematically reviewed for: demographic data, clinical features, laboratory findings, treatments, and outcomes. The major clinical features were: spiking fever 100%, evanescent maculopapular rash 95%, polyarthralgia 90%, sore throat 78%, lymphadenopathy 66%, hepatosplenomegaly 57%, hydrohymenitis 30%, neutrophilia 98%, liver disfunction 62%, increased erythrocyte sedimentation rate (ESR) 96%, and hyperferritinaemia 99%. Reactive hyperplasia was shown in all patients who underwent lymph node biopsy. Ninety-five percent and 63% of the patients were treated with glucocorticoid and immune suppressant, respectively. Those with prednisone or its equivalent dosage of > or =0.8 mg/kg/d achieved quicker remission and less relapse. Persistent fever, evanescent rash, arthritis, and sore throat were the most prevalent symptoms in patients with AOSD, with laboratory findings of leukocytosis, elevated liver enzymes, elevated ESR and serum ferritin. Glucocorticoid and immune suppressive drugs are effective for AOSD; however, the relapsing rate is relatively high. High levels of white blood cells, serum ferritin and ESR, as well as glucocorticoid dosage were related to relapse.
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PMID:Clinical features and prognosis in adult-onset Still's disease: a study of 104 cases. 2054 76

Adult-onset Still's disease (AOSD) is a complicated multi-systemic inflammatory disease, the main features of which are high spiking fever, evanescent rash, polyarthralgia, lymphadenopathy, hepatosplenomegaly and leukocytosis. The pathogenesis and etiology of AOSD are still unknown. We report a case of atypical AOSD presenting with only arthralgia, fever, lymphadenopathy, slightly elevated serum C-reactive protein and ferritin levels and a slightly elevated erythrocyte sedimentation rate; these signs and symptoms did not fulfill the proposed diagnostic criteria of both Cush and Yamaguchi. After exclusion of other likely diagnoses, a diagnosis of atypical AOSD was made, and a low dose of corticosteroids was effective in resolving all of the patient's symptoms. More clinical practice and research are needed to determine pathogenesis and etiology of AOSD and to amend the diagnostic criteria to include such atypical cases.
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PMID:Adult-onset Still's disease: how to make a diagnosis in an atypical case. 2187 45