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)

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

In primary amyloidosis the gastrointestinal tract and the liver are commonly involved, but clinical features and prognosis are mainly determined by the extent of cardiac and renal involvement. We review two cases with primary amyloidosis of the gastrointestinal tract and the liver. The predominant symptoms were malabsorption and hepatomegaly with cholestasis. The clinical aspects, diagnosis, treatment and prognosis of primary amyloidosis of the gastrointestinal tract and the liver are discussed in the context of the current literature.
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PMID:[Primary amyloidosis of the gastrointestinal tract and liver--two case reports]. 916 26

Although the involvement of the liver is common in systemic amyloidosis (AL), clinical features of hepatic dysfunction and liver chemistry abnormalities are often mild or absent. A mild increase in the serum alkaline phosphatase value is the most common finding. Hypertransaminasemia, hyperbilirubinemia, and portal hypertension with ascites and gastroesophageal varices occur late in the course of the disease and predict a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, esophageal varices, and ascites, was rapidly complicated by severe obstructive cholestasis.
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PMID:Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course. 917 38

The symptoms of amyloidosis depend on the type of precursor, the amount of deposits and their location. In systemic amyloidosis almost every organ may be involved. Cardiac involvement is severe, especially in AL amyloidosis, responsible for restrictive cardiomyopathy with right ventricular failure, leading rapidly to death. Renal amyloid deposition causes nephrotic syndrome with hypertension and renal failure. Neurological complications include peripheral neuropathy with dysautonomia cerebral involvement (dementia, cerebral haemorrhages). Arterial deposits are common in systemic senile amyloidosis, and may cause ischaemia. Osteo-articular damage is mainly seen in patients on long-term haemodialysis. Liver enlargement is often the only manifestation of hepatic amyloidosis. Digestive tract involvement includes macroglossia deposits in salivary glands and disturbances in gastrointestinal motility. Pulmonary amyloidosis causes nodular or interstitial infiltrates. Cutaneous lesions are various. Localized amyloidoses include goiter, breast and vesical involvement which can be difficult to differentiate from neoplasm, as well as ocular amyloidosis mimicking posterior uveitis.
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PMID:[Localizations and consequences]. 945

A total of 1300 birds in flock of breeder Pharaoh quail (Coturinix coturnix) experienced a moderate rate of mortality (13%) during a 7-day period. Clinical signs included depression, ruffled feathers, prostration, lameness, inapetence, diarrhea, and periorbital sinus swelling with mucoid discharge and lameness. Gross lesions observed in dead quail were emaciation, carcass congestion, mild hepatomegaly with green discoloration, congested intestinal mucosa, caseous purulent arthritis-osteomyelitis, and thickened crop mucosal epithelium. Histopathologic examination revealed mild hepatic amyloidosis, proliferative parabronchitis, splenic reticular cell hyperplasia, thymic cortical atrophy, subacute bacterial osteomyelitis, periarthritis, and crop mycosis. Pasteurella multocida was isolated from the joints of these birds and the isolates were serotype 3 x 4. These findings suggest that Pharaoh quail are susceptible to P. multocida and are likely to develop subacute to chronic fowl cholera.
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PMID:Subacute to chronic fowl cholera in a flock of Pharaoh breeder quail. 953 3

A 74-year-old man with hepatomegaly, hyperglycemia, and proteinuria was diagnosed with primary amyloidosis with liver involvement, proven by biopsy. Abnormal distribution of tracer in the liver on Tc-99m phytate liver-spleen imaging and abnormal tracer uptake by the liver on Tc-99m pyrophosphate whole body imaging were observed. Scintigraphic imaging studies may be used noninvasively to evaluate the involvement of organs in patients with primary amyloidosis, reducing the risk of bleeding caused by biopsy.
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PMID:Radionuclide imaging in primary amyloidosis with liver involvement. 961 24

We report the case of a middle-aged woman presenting epigastric discomfort, hepatomegaly, biochemical signs of cholestasis, bone marrow plasmocytosis and Bence Jones proteinuria. Percutaneous liver biopsy disclosed kappa light chain deposition disease of the liver and fine needle aspiration of abdominal fat showed amyloid substance. Renal blood chemistries and urinalysis were repeatedly normal. To our knowledge, this is the first reported case of kappa light chain deposition disease of the liver and concomitant amyloidosis without renal involvement as the first manifestation of plasma cell dyscrasia. This condition should be considered in the differential diagnosis of intrahepatic cholestatic liver disease.
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PMID:Kappa light chain deposition disease of the liver. 961 91

We retrospectively investigated the feasibility and the toxicity of autologous stem cell transplantation (ASCT) in 21 cases of systemic amyloidosis (AL). The conditioning regimens consisted of high-dose melphalan (HDM) alone (n = 18) or in combination with 12 Gy total body irradiation (n = 3). Toxic death rate was high: 9/21 patients (43%) died within the first month following ASCT, and 10/12 surviving patients achieved a response. With a median follow-up of 14 months, the OS and the EFS rates at 4 years were 57.1% (+/-10.8) and 29.9% (+/-14.5) respectively for the whole group. The major prognostic factor for both response and survival was the number of clinical manifestations at the time of ASCT, of the following five criteria, i.e. creatinine clearance < 30 ml/min, nephrotic syndrome with urinary protein excretion > 3000 mg/24 h, congestive heart failure, neuropathy, or hepatomegaly associated with alkaline phosphatase level > 200 IU/l. For patients presenting with two or more clinical manifestations the 4-year OS and EFS were both 11.1% compared with 91.7% and 46.3% respectively in patients with fewer than two clinical manifestations at the time of ACST. We conclude that ASCT is feasible in AL in a subset of patients with fewer than two clinical manifestations at the time of ASCT. Given the severe extra-haematological toxicity, ASCT should not be considered in other cases.
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PMID:Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a report on 21 patients. 967 53

Amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), idiopathic peripheral neuropathy, or unexplained hepatomegaly. When a patient has one of these problems, immunoelectrophoresis and immunofixation of the serum and urine should be done for the detection of a monoclonal light chain. If a monoclonal light chain is found, a diagnosis usually can be established by amyloid stains performed on a bone marrow biopsy specimen or a subcutaneous fat aspirate. The presence or absence of cardiac involvement with amyloid is the most important prognostic factor. Treatment can range from observation to oral chemotherapy to hematopoietic stem cell transplantation. A practical understanding of the mechanisms underlying this disease can lead to prompt diagnosis and early therapeutic intervention.
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PMID:Amyloidosis: recognition, confirmation, prognosis, and therapy. 1031 82

A 53-year-old man, who presented with weight loss over a period of 10 months, hepatomegaly, markedly raised cholestatic enzymes and Ca 19-9, was initially suspected of suffering from metastatic cholangio-carcinoma. Liver biopsy revealed depositions of AL-amyloid. Further investigations confirmed a generalized amyloidosis. Biopsies taken from the gastric, colonic, and bronchial mucosa all showed depositions of amyloid. A nephrotic syndrome was interpreted as being secondary to the renal involvement. Echocardiography identified changes which were consistent with cardiac involvement. A plasmacytoma or lymphoma was excluded. At the time of diagnosis the patient was in a good physical condition with normal renal function. Within a few weeks the renal function deteriorated and after 2 months the patient developed ascites and became jaundiced. Four months after initial presentation the patient died from cardiac failure.
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PMID:Hepatomegaly and cholestasis as primary clinical manifestations of an AL-kappa amyloidosis. 1051 29


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