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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intolerance of dietary fat, massive hepatomegaly and sensation of right upper Quadrant oppression were clinical symptoms of a bioptically verified rare hepatic amyloidosis. There was no other evidence for amyloid deposition in other organs. Hepatomegaly was slightly progressive over a period of 2 years, accompanied by slight edema of lower legs and ascites detected by sonography.
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PMID:[Fat intolerance, pressure in the right upper quadrant, hepatomegaly]. 234 20

We report an unusual case of lambda light chain deposits without overt plasma cell dyscrasia. The clinical presentation was hepatomegaly without biochemical sign of renal involvement. Portal hypertension, spontaneous rupture of the spleen and fracture of the 12th thoracic vertebra occurred during the course of the disease. Ultrastructural studies showed that lambda light chain deposits were associated with amyloid-like deposits. This case suggests that light chain deposition disease and amyloidosis could be two expressions of the same disease.
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PMID:Light chain deposition disease presenting with hepatomegaly: an association with amyloid-like fibrils. 315 78

Amyloidosis is classified according to the distribution pattern of amyloid deposition sites and associated diseases. Hepatic amyloidosis is not infrequent, although rarely causes clinical liver disease. We report two cases of amyloidosis diagnosed by liver biopsy. One presented with symptoms related almost to the liver disease, such as jaundice, hepatomegaly and indigestion. Echocardiogram revealed hypertrophic cardiomyopathy, suggesting cardiac involvement of the amyloidosis. The patient died of hepatic failure. The other case was found in a patient with an end stage renal disease. Features of congestive heart failure in this case may reflect cardiac involvement. The pattern of hepatic amyloid deposition in both of these cases was diffuse perisinusoidal. The predominant intralobular deposition suggests that these are amyloidosis of the secondary type.
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PMID:Hepatic amyloidosis--two cases report. 326 63

Recurrent hereditary polyserositis (RHP) or familial Mediterranean fever (FMF) is a chronic inherited illness of obscure aetiology. The disease is characterised by paroxysmal attacks of fever, peritonitis, pleuritis or arthritis, and predominantly affects Sephardic Jews, Arabs, Turks and Armenians. In this study, we report our 11-year experience of 175 Arab patients with this disease. As with other ethnic groups, the most common manifestation (93.7 per cent) was peritonitis. Arthritis (33.7 per cent) and pleurisy (32 per cent) were next in frequency. Adult patients in this series unlike those in other ethnic groups, rarely presented with arthritis. Similarly rare were amyloidosis, rashes, splenomegaly, hepatomegaly or lymphadenopathy. The aetiology of this disease is not clear but we suspect that abnormalities in catecholamine metabolism may be a factor in the pathogenesis.
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PMID:Familial Mediterranean fever (recurrent hereditary polyserositis) in Arabs--a study of 175 patients and review of the literature. 330 55

Necropsy materials from 57 cases of generalized amyloidosis in rhesus monkeys were reviewed. Clinically, animals with the disease were characterized by cachexia with muscle wasting, recurrent diarrhea, and arthritis. Gross lesions included hepatomegaly, splenomegaly, chronic/active colitis, fibrous strictures of the cecocolic junction, osteoarthritis, and generalized muscle atrophy. Histologic examination revealed minimal to severe deposits of amyloid in the small intestine (100%), spleen (93%), large intestine (67%), liver (40%), lymph nodes (71%), stomach and/or adrenal gland (32%). More amyloid was deposited in the spleen, liver, and small intestine than in other organs. Shigella sp. were isolated from feces in 23% of the cases and 84% had histologic evidence of colitis. Other findings indicated that 100% of the animals had lung mites, 25% had strictures of the cecocolic junction, and 40% had osteoarthritis. Thirty percent of the cases occurred in animals 10 months to 5 years of age, 10% in ages 6 to 10 years, and 60% in animals greater than 10 years old.
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PMID:Generalized amyloidosis in rhesus monkeys. 375 Jul 36

The foregoing case is an example of amyloidosis in which biopsy of a hemorrhagic vesicle on the buccal mucosa established the diagnosis. Once the diagnosis was made, the patient's cardiomyopathy, nephrotic syndrome, hepatomegaly, GI hemorrhage, hematuria, and oral lesions readily were explained.
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PMID:Recurrent petechial hemorrhages and hemorrhagic vesicles of the oral mucosa. 387 62

One hundred twenty-eight records of patients with multiple myeloma were reviewed to assess the incidence and manifestations of liver involvement. Histologic study of the liver was available in 21 patients. Diffuse infiltration of the liver by plasma cells was observed in 10 patients, myeloid metaplasia in four, amyloidosis in two, toxic hepatitis in two, and extrahepatic cholestasis secondary to infiltration of the peripancreatic tissue by plasma cells in one. The clinical signs of plasma cell infiltration of the liver consisted of hepatomegaly in seven patients, mild elevation of liver enzymes in five, and portal hypertension in two. Jaundice was only observed in patients with hepatitis or extrahepatic cholestasis. Liver infiltration by plasma cells did not appear to have a major prognostic significance.
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PMID:Liver involvement in multiple myeloma. 402 19

I treated a patient who had amyloidosis with predominantly hepatic involvement and portal hypertension. The main clinical features were hepatomegaly, gross ascites, proteinuria, and elevated alkaline phosphatase levels. Despite permanganate-sensitive AA protein being present in the biopsy specimen, none of the recognized disease entities associated with secondary amyloidosis were found. A review of the literature and the mechanism of portal hypertension in amyloidosis is given. It is suggested that elevated portal pressures may be of greater importance in the pathogenesis of ascites in amyloidosis than has been appreciated.
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PMID:Hepatic amyloidosis. An unusual cause of ascites and portal hypertension. 649 30

The records of 52 patients with amyloidosis admitted to Groote Schuur Hospital, Cape Town, between January 1969 and August 1982 were analysed. The male: female ratio was 1,3:1 and the mean age was 49,3 years. Forty-eight per cent of the patients had secondary amyloidosis, 21% had primary amyloidosis, 11,5% had localized amyloidosis and 11,5% had amyloidosis associated with multiple myeloma. Tuberculosis, chronic pulmonary sepsis and other chronic infections were present in 88% and rheumatoid arthritis in 16% of the patients with secondary amyloidosis. The commonest features at diagnosis were proteinuria (70%), oedema (52%) and hepatomegaly (39%). The diagnosis of amyloidosis was established by renal, liver and rectal biopsy (either singly or in combination) in 82% of cases. The prevalence of amyloidosis at autopsy was 0,28%.
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PMID:Amyloidosis at Groote Schuur Hospital, Cape Town. 674 Apr 24

4 cases of massive hepatic amyloidosis are reported with special reference to their clinical profiles and histologic features. On the basis of these data, two different clinical and histologic courses of the disease can be distinguished. 2 patients showed marked hepatomegaly without cholestasis, whereas in the other 2 the clinical picture was characterized by much less pronounced hepatomegaly, but by severe and progressive intrahepatic cholestasis. The time course of the disease seems to be different in the two forms, the cholestatic form being more rapidly fatal than the other.
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PMID:Different morphologic aspects and clinical features in massive hepatic amyloidosis. 674 5


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