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 clinical presentation and findings in 45 patients with lymphoma of the gastrointestinal tract occurring in an unselected population are described. Nineteen patients presented as emergencies with haemorrhage, perforation, pyloric stenosis or intestinal obstruction. Systemic symptoms, peripheral lymph node enlargement and hepatosplenomegaly were rare, and a preoperative diagnosis of lymphoma was seldom made. Endoscopy was superior to radiology in defining lesions in the stomach and duodenum. Lesions which were multicentric, or of unusual macroscopic appearance, were common in the small bowel, and these should raise the suspicion of lymphoma. Local and systemic recurrence was common, but was not always fatal. Patients with gastric lymphomas survived longer than those with intestinal disease.
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PMID:The presentation of gastrointestinal lymphoma: study of a population. 51 67

Twelve of 43 patients with chronic active hepatitis (CAH) (28%) manifested clinical and laboratory features of cholestasis. The criteria for selection of these patients included at least two of the following: chronic or recurrent pruritus, serum alkaline phosphatase levels of 300 mU./ml. and cholesterol of 300 mg./dl. or more. When compared with 31 control cases these patients were found to have a preponderance of Ashkenazi Jews of Roumanian origin, a higher prevalence of joint and thyroid involvement and higher serum Ig-M Levels. Mortality was similar in both groups but patients with cholestatic features tended to die earlier in the course of the disease. Retrospectively, it was found that they had been treated more intensively, attained complete remissions less frequently and developed cirrhosis more readily. There were no significant differences in the frequency of HBsAg and anti-HBs, the mode of onset, the frequency of hepatosplenomegaly and jaundice, the hematologic findings and the prevalence of autoantibodies. Like acute cholangiolitic viral hepatitis, CAH with cholestatic features emerges as a more serious disease than the classical form of CAH.
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PMID:Chronic active hepatitis with cholestatic features. I. A clinical and immunological study. 53 97

Twenty-one of 30 patients with essential mixed cryoglobulinemia (EMC) had evidence of liver involvement. The liver disease was characterized by the absence of clinical symptoms, hepatosplenomegaly, mild elevation of enzymes, abnormal BSP retention and low albumin levels. Histology, available in 12 patients, showed either chronic persistent or chronic active hepatitis or liver cirrhosis; 44% of the patients had HBsAg or HBsAb in sera and/or cryoglobulins, confirming the high frequency of exposure to hepatitis B virus (HBV) infection in EMC. However, liver lesions were similar in all patients, regardless of HBV exposure. Since other factors usually associated with chronic liver diseases were absent or apparently irrelevant, it is temptative to speculate that a 'cryoglobulinemic hepatitis' may exist as a distinct syndrome. The characteristic complement profile of the patients with EMC (low CH50 and C4, normal C3PA), not related to albumin levels, can help to differentiate this disease from chronic liver disease without cryoglobulins.
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PMID:Liver involvement in essential mixed cryoglobulinemia. 54 44

Regencrative nodular hyperplasia (RHN) is a rare condition, the diagnosis of which is based upon histological findings. It is seen in Felty's syndrome with portal hypertension (PHT), as was the case in the patient reported here. This was a 72-year-old man, with long standing rheumatoid arthritis, hepatosplenomegaly, a neutrophil leucopaenia and oesophageal varices responsible for recurrent haematemeses. Despite a portocaval anastomosis, the patient died from postoperative acute hepatic failure. Histological study revealed changes in the hepatocytes and the reticulin system typical of RNH without cirrhosis. The relationship between Felty's syndrome and RHN, as well as the mechanism of the hypertension, are discussed in the light of cases from the literature.
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PMID:[Anatomo-clinical study of a case of regenerative nodular hyperplasia of the liver with Felty's syndrome and portal hypertension]. 54 55

Malignant histiocytosis, an affection in which there is proliferation of morphologically atypical histiocytes, traditionally associates high fever, deterioration of the general condition, adenopathy, hepatosplenomegaly, and less frequently, cutaneous lesions. Clinical, radiological, and histological signs of bone involvement are rarely observed, which demonstrates the interest of the case reported of a pure medullary form with massive necrosis and successive bone localisations of the osteolytic type. The clinical picture was completed by the progressive development of adenopathy and hepatosplenomegaly. Multiple chemotherapy, according to the A.V.E.C. procedure, controlled the affection for three months before it became totally ineffective. Survival for twelve months after clinical onset demonstrates the extreme malignancy of the affection.
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PMID:[Malignant histiocytosis with bone involvement (author's transl)]. 54 9

Six patients have been observed which exhibited the features of the so called immunoblastic lymphadenopathy. The histological and clinical findings allow to distinguish the disease from both the malignant lymphomas and other forms of benign pseudolymphomas. The most important clinical features are severe general symptoms, generalised lymphadenopathy, hepatosplenomegaly, skin rash and a variety of abnormal reactions in the B-cell system such as hypergammaglobulinaemia, a transient positive Coombs test and the appearance of plasma cells in the peripheral blood. Evidence for a neoplastic nature of the disease is lacking. Nevertheless, the course of the disease may be progressive and fatal, but spontaneous remissions and subsequent relapses have also been essential part of the therapeutic strategy. Immunosupressants such as corticosteroids may have a positive effect in cases with immunological complications.
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PMID:[Immunoblastic lymphadenopathy--malignant neoplasia or hyperimmune reaction? (author's transl)]. 55 3

Report of 2 siblings, aged 12 1/2 and 9 years, with congenital hepatic fibrosis and polycystic disease of the kidneys. Hepatosplenomegaly had been noted in both children at birth. The younger child had suffered from oliguria aged 2 1/2 years. At diagnosis both children had low platelet counts, one also had leucopenia. The cystic disease of the kidneys was verified by angiography. Coeliacography and splenopartography were diagnostically irrelevant. The diagnosis only became apparent from liver biopsy which was performed during splenectomy. After splenectomy there was an increase of platelets, white blood cells and the clotting factors II, V and X. The three years follow-up showed a constancy of renal impairment and of the minor oesophageal varices observed in the one patient who did not have a spontaneous spleno-renal anastomosis. So far no bleeding has been observed. Porto caval anastomosis was omitted in both children. Pros and cons are being discussed.
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PMID:[Congenital hepatic fibrosis and polycystic disease of the kidneys in two siblings (author's transl)]. 55 82

The paper reports on a case of "late onset disease" (congenital German measles). Diagnostically significant for this disease in an infant appearing perfectly healthy when new-born, were mainly the findings of an interstitial pneumonia, hepatosplenomegaly, as well as findings indicating the presence of a meningoencephalitis and retinopathy. The diagnosis of this rare course of congenital German measles was finally established via the identification of specific IgM rubeola antibodies, of a raised hemagglutination inhibition titer, and of the German measles virus itself. An attempt to treat the interstitial pneumonia with cortisone preparations remained unsuccessful. The clinical and diagnostic problem complexes are discussed briefly.
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PMID:["Late onset disease" (congenital german measles) (author's transl)]. 56 31

Platelet volume distribution was examined in 16 patients with hepatosplenomegaly and platelet counts of 45 000 to 90 000/mm; 12 patients with autoimmune thrombocytopenia and randomly matched platelet counts; and 20 normal subjects. Five platelet volume variables of increasing platelet size were defined from the averages of 20 normal curves. Patients with hypersplenism had decreased volume values of 78% to 87% (mean, 83%) of the average normal population (P less than 0.001). Patients with autoimmune thrombocytopenic purpura had values significantly greater than normal by 124% to 149% (mean, 134%) (P less than 0.001). Patients with autoimmune thrombocytopenic purpura, when compared with hypersplenic patients, had significantly greater platelet volume values ranging from 154% to 174% (mean, 161%), P less than 0.001. We concluded that patients with hepatosplenomegaly have smaller platelets in their peripheral blood and a platelet volume distribution that can be distinguished easily from patients with autoimmune thrombocytopenic purpura, despite comparable platelet counts.
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PMID:Hypersplenic thrombocytopenia differentiated from increased peripheral destruction by platelet volume. 56 25

The article reports on a boy of six years of age with hyperlipoproteinaemia of Type I. His case was notable for abducens paralysis and hepatosplenomegaly. Routine diagnosis with plasmatriglyceride and plasma-cholesterol, as well as liproprotein electrophoresis, was supplemented by measurement of the activity of the triglyceride lipases in the post-heparinplasma.
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PMID:[A case of hyperlipoproteinaemia of type I (author's transl)]. 57 52


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