Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and pathological features of four liver biopsies and 12 autopsies from 1981-1990 with hepatic amyloidosis were reviewed. All of the patients had a history of both intravenous and subcutaneous cocaine and heroin use with chronic suppurative skin ulcers. Five patients were proven to have the acquired immunodeficiency syndrome at autopsy. Systemic amyloidosis was diagnosed in only five out of the 16 patients prior to death. Hepatomegaly was present in 12 patients. The amyloid protein was AA in 14 and AL in one case. Definitive characterization of the amyloid substance was not possible in one case. There was no evidence of multiple myeloma or a plasma cell dyscrasia in the one patient with AL amyloid. The amyloid distribution in the liver was both parenchymal and vascular in 12 cases. Cholestasis, which appeared to occur preterminally, was the only histological finding that correlated with the patient's clinical condition. The histological pattern of amyloid in the liver could not predict the type of amyloid protein that was identified. Amyloidosis should be considered in the differential diagnosis of unexplained hepatomegaly in the acquired immunodeficiency syndrome with chronic suppurative infections.
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PMID:Hepatic amyloidosis in intravenous drug abusers and AIDS patients. 830 Oct 46

Systemic amyloidosis presenting with jaundice is rare. A case of primary amyloidosis presenting with severe intrahepatic cholestasis is reported. The patient had hepatomegaly, ascites, and a markedly elevated serum alkaline phosphatase level. He had a rapid downhill course resulting in death. Autopsy showed evidence of amyloidosis involving multiple organs, including the liver, kidney, and heart.
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PMID:Hepatic amyloidosis presenting with severe intrahepatic cholestasis. 925 70

Systemic amyloidosis results from the deposition of insoluble, fibrous amyloid proteins. It occurs mainly in the extracellular spaces of multiple organs and tissues including the kidney, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinically apparent liver disease is relatively rare. Indeed, most patients with systemic amyloidosis manifest only minimal to moderate hepatomegaly and trivial abnormalities in liver function tests. Recently, we experienced two cases of patients who presented with abnormalities in liver function tests and hepatomegaly as manifestations of systemic amyloidosis. We report these cases with a review of the relevant literatures.
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PMID:[Two cases of systemic amyloidosis presenting with abnormalities in liver function tests]. 1463 55

Systemic amyloidosis with hepatic involvement is a rare disorder, which is characterized by the deposits of amyloid fibrils in the liver. The prognosis is poor and the median survival is 13 months. Bleeding problems resulting from coagulopathy frequently complicates systemic amyloidosis. We present two patients with a severe factor X deficiency and hepatomegaly as the presenting abnormalities of systemic amyloidosis. One of the patients was treated with high dose melphalan chemotherapy and autologous stem cell reinfusion, resulting in a normalization of the liver enzyme tests and the factor X level. The diagnosis and treatment of systemic amyloidosis with hepatic involvement and the management of the multifactorial coagulopathy in these cases is discussed.
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PMID:Successful treatment of systemic amyloidosis with hepatic involvement and factor X deficiency by high dose melphalan chemotherapy and autologous stem cell reinfusion. 1496 36

Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man.
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PMID:Primary Hepatic Amyloidosis Presenting as Acute-on-Chronic Liver Failure. 2828 88

Systemic amyloidosis is a rare disease involving multiple organs. It is difficult to establish diagnosis as the symptoms is diverse and non-specific. And without specific therapy the prognosis is very poor. We analyzed detailed clinical and laboratorial data of a 53-year-old male patient. The characteristic features included refractory pleural effusion, extraordinary hepatomegaly and cardiac failure. The illness lasted 9 months and therapy period spanned 4 months. Fine needle biopsy of liver, lung, heart, pancreas and kidney was performed. Immunohistochemistry, immunofluorescence, Congo staining and hematoxylin and eosin staining were performed. All specimens were stained pink with haematoxylin and eosin staining. Amorphous deposits of eosinophilic material were visible within the Congo red dye stained liver tissue whereas under cross-polarized light pathognomonic apple-green birefringence of amyloid deposits was visible. At last systemic AL amyloidosis diagnosis was confirmed. The report showed an unusual AL amyloidosis case in detail which would be helpful for physician in clinical work.
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PMID:Clinical characteristics and diagnosis of a rare case of systemic AL amyloidosis: a descriptive study. 2984 40