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)

Twenty-five patients with primary biliary cirrhosis (PBC) in different stages were investigated with respect to pulmonary function abnormalities. The results were compared with a reference sample of 17 sex- and age-matched healthy subjects. A high prevalence of lung function impairment was found in the PBC patients (14/25 [56%]). Bronchial asthma was present in three patients, and severe lung emphysema in one. These four patients had an abnormal lung function, mainly of obstructive type. There was a statistically significant difference between the remaining 21 PBC patients without chronic obstructive lung disease and the reference subjects with respect to diffusion capacity. Almost all abnormal lung function data were found in the symptomatic PBC patients (i.e. symptoms of pruritus, xanthoma, xanthelasmata, jaundice, hyperpigmentation, hepatosplenomegaly), 13 out of 18 (72%), whereas only one out of seven asymptomatic patients was affected. Nine patients (36%) had reduced diffusion capacity compared with none of the reference subjects. The lung function abnormalities in PBC patients are similar to those found in sarcoidosis, another granulomatous disease.
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PMID:Lung function abnormalities in patients with primary biliary cirrhosis. 338 7

A survey of all patients in whom liver biopsy showed epithelioid granulomas was undertaken at two major teaching hospitals in Glasgow for the period 1970-1979. Seventy-seven patients with hepatic granulomas were studied retrospectively. In 53 cases (69 per cent) a clear-cut clinical diagnosis was established, which included sarcoidosis (eight cases), tuberculosis (eight), extrahepatic biliary obstruction (seven), primary liver diseases (11), neoplasm (six), bacterial infection (five) and miscellaneous (eight). In 24 patients (31 percent) no cause was found. Seventeen patients from this idiopathic group were studied prospectively and single examples of the following conditions were subsequently diagnosed; pulmonary tuberculosis, primary biliary cirrhosis, ulcerative colitis, adenocarcinoma of rectum, primary hepatocellular carcinoma, alpha-one antitrypsin deficiency and pulmonary fibrosis, sarcoidosis, pulmonary fibrosis alone, gallstones, rheumatic heart disease, unexplained hepatosplenomegaly and one death from mesenteric artery thrombosis. Only six cases remained truly idiopathic. Three of these patients recovered and in two liver biopsy became normal. The other three have persistent granulomas associated with continuing illness.
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PMID:Hepatic granulomas: experience over a 10-year period in the West of Scotland. 711 78

Sarcoidosis frequently involves the abdomen, although imaging studies often fail to demonstrate disease. The most common computed tomography (CT) findings in abdominal sarcoidosis are hepatosplenomegaly and retroperitoneal adenopathy, followed by focal low-attenuation lesions of the liver and spleen. Other abdominal viscera are involved infrequently. We present four cases of abdominal sarcoidosis demonstrating a range of CT findings.
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PMID:Abdominal manifestations of sarcoidosis. CT appearances. 789 93

Childhood sarcoidosis is a rare disorder with protean manifestations. The case of a child with prolonged fever, hepatosplenomegaly, pancytopenia, and systemic necrotizing vasculitis manifesting as fever, rash and skin infarctions, digital pregangrene, and foot drop is reported. This is the first case of systemic necrotizing vasculitis reported in sarcoidosis. The fulminant course of the disease required treatment with intravenous pulsed cyclophosphamide and high doses of corticosteroids. The spectrum of vasculitis in childhood and adult sarcoidosis is reviewed.
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PMID:Systemic necrotizing vasculitis associated with childhood sarcoidosis. 793 24

In adult acquired hypogammaglobulinaemia multi focal granulomas have often been described and have regularly led to the hypothesis of an association with sarcoidosis. We present a case of this type in a man aged 29 who was a smoker with a hypoglobulinaemia involving IgG, IgA and IgM and which was discovered following pneumococcal pneumonias. He presented with a significant hepatosplenomegaly and absent cutaneous reactions to T dependent antigens with an elevated ACE activity. Histological examination of the splenectomy specimen and of the liver biopsy showed an infiltration by epithelioid follicles and confluent giant cells without necrosis. The pulmonary studies showed a normal chest radiograph but the bronchial biopsy again found a granulomatous infiltration. The broncho-alveolar lavage was cytologically normal and a very slight and paradoxical reduction of the alveolar immunoglobulins was noted implying either an active intra-alveolar concentration of immunoglobulins or a local synthesis. In the light of the few reported cases it seems that the diagnosis of sarcoidosis should be dismissed here in favour of multi focal granulomatosis with hypogammaglobulinaemia. In hypogammaglobulinaemia there is no clinical or biological method (IDR tuberculin, ACE, Kveim, histology) to confirm a superadded diagnosis of sarcoidosis.
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PMID:[Acquired hypogammaglobulinemia and multifocal granulomatosis]. 825 39

The clinical picture of twelve patients with sarcoidosis commencing under 4 years of age is presented. Follow-up ranged from 5 to 30 years with a mean of 11.5 years. Two patients were mother and daughter, the other cases were sporadic. All children developed the typical symptom triad of rash, polyarthritis and uveitis with onset before their 4th birthday. Further symptoms comprised fever, hepatosplenomegaly, parotid swelling and hypertension. Eight patients suffered from cardiac and four from cerebral involvement; one girl died from cerebral involvement at the age of 12. Two developed severe lung changes. The prognosis was poor with sequelae in all patients. Several children seem to have improved on immunosuppressive therapy. Early onset sarcoidosis is a multisystemic granulomatous disease; since polyarthritis is a dominant manifestation, many patients are diagnosed as having juvenile chronic arthritis. The early onset form must be distinguished from later onset sarcoidosis, which mainly affects children over 8 years of age or young adults. The disease types differ in their presentation, symptoms, course and prognosis; some of the clinical features and the histology, however, are identical.
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PMID:Sarcoidosis of early onset. A challenge for the pediatric rheumatologist. 829 66

Isolated extrathoracic sarcoidosis is a rare entity. We present a 25-year old woman with recurrent episodes of fever of unknown origin in the 10 years previously, with a new episode of fever with hepatosplenomegaly, abdominal adenopathies and cranial osteolysis without pulmonary or mediastinal affectation. With the suspicion of linfoproliferative disease we have resected the spleen with a weight of 1500 g. and multiple non caseous granulomata also in the liver and adenopathies. The clinical presentation is compatible with an atypical sarcoidosis without thoracic affectation and with two rare associated clinical manifestation namely giant splenomegaly and sarcoidosis of the skull.
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PMID:[Extrathoracic sarcoidosis with giant splenomegaly and cranial osteolysis]. 906 37

Diffuse parenchymal infiltrates in lower lung fields, right pleural effusion, multiple bronchial stenoses and hepatosplenomegaly were observed in a 30-year-old woman with iritis. Pleural effusion was a yellow exudate which was predominantly lymphocytic. A pulmonary function test showed an obstructive pattern, and multiple bronchial stenoses were observed on bronchoscopy. Both transbronchial lung biopsy (TBLB) and bronchial mucosal biopsy failed in demonstrating non-caseating granulomas, but liver biopsy revealed granulomas consistent with sarcoidosis. This is a very rare case, in which pleural effusion and bronchial stenoses occurred in the same patient.
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PMID:Sarcoidosis accompanied by pleural effusion and multiple bronchial stenoses. 931 8

Sarcoidosis is a granulomatous multisystem disorder that may uncommonly involve muscle. Muscular sarcoid may be nodular, atrophic myopathic, or acute myositic. We illustrate a case of the myopathic type of muscular sarcoid that is unusual because the abdominal wall muscles, rather than the extremity muscles, were involved. Muscular involvement by sarcoid should be considered in the differential diagnosis of focal muscle disease, especially in a patient with a known history of sarcoid. The presence of typical bilateral hilar adenopathy on a chest radiograph as well as the presence of abdominal findings (hepatosplenomegaly and retroperitoneal adenopathy) may help establish the diagnosis. Otherwise, sonographically guided biopsy may be necessary for definitive diagnosis.
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PMID:Sarcoid myopathy: imaging findings. 935 May 74

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascularization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, and immunologic aberrations in idiopathic reticulosis. Initially, AILD was thought to be a disease of B-cell origin that represented reactive immune response to unknown stimulus and high potential for malignant transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if complete response with chemotherapy is not achieved. Immunologic and molecular studies have demonstrated that the majority of AILD cases are T-cell clonal disorders. Despite the numerous reports on the role of Epstein-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that accompanies AILD, or whether it is a pathogenetic factor. In contrast to non-Hodgkin's lymphomas, a stage is not usually assigned to the patient since the disease is systemic in nature, subsequently, parameters such as extent of disease and tumor bulk used to identify high-risk patients with non-Hodgkin's lymphomas, do not appear to correlate with disease activity or prognosis in AILD. Treatment of AILD has been unsatisfactory, with approximately 25% of patients achieving complete and sustained remission when combined chemotherapy agents are used. This article is devoted to a discussion of the different manifestations, suggested pathogenesis, and treatment of AILD.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. 985 98


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