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

The liver is frequently involved by amyloidosis, but hyperbilirubinemia and liver failure are uncommon features. A mild elevation of the serum alkaline phosphatase value and, less frequently, hepatomegaly are the most common findings. Usually the patients have no symptoms related with the liver involvement; the clinical manifestation and the long term prognosis depends on the renal and cardiac disease. We report an unusual clinical presentation of primary amyloidosis in a previously asymptomatic 65 years old woman who was admitted to the hospital because of ictericia and ascitis mimicking a drug induced acute hepatic failure.
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PMID:[Hepatic amyloidosis as cause of severe intrahepatic cholestasis]. 1266 5

We present the case of a 70-year-old woman who had been suffering from constitutional syndrome for several months, abdominal distension, and yellowish coloration of the skin for the previous few days with a rapidly fatal course. Examination revealed hepatomegaly and ascites. Laboratory investigations revealed hyperbilirubinemia with cholestasis. The remaining investigations (abdominal ultrasound, barium transit evaluation, bone marrow study, analysis of ascitic fluid and laparoscopy) did not establish the diagnosis. This was established by liver and subcutaneous fatty tissue biopsies, which revealed type AL amyloid deposits. Autopsy confirmed that the patient had primary systemic amyloidosis. This infrequent form of presentation of systemic amyloidosis and its poor prognosis are discussed.
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PMID:[Cholestatic jaundice and constitutional syndrome as early manifestations of primary systemic amyloidosis]. 1288 57

Casuistry of a 59-year-old woman is presented. The patient was examined for nephrotic syndrome with relapsing stenosis of vena cava inferior treated with stent implantation and with severe protein-caloric malnutrition. She had macroglosy, mild hepatomegaly, severe impairment of gastrointestinal motility, and had in her history an operation of carpal tunnel. It took two years to establish the diagnosis, which was amyloidosis of AL.
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PMID:[A less common cause of malabsorption syndrome]. 1292 Jul 96

The liver is a common site of amyloid deposition in primary systemic amyloidosis. We reviewed the clinical features and natural history of patients with primary systemic amyloidosis and biopsy-proven hepatic involvement who were evaluated at Mayo Clinic from January 1, 1975, to December 31, 1997. The median age of the study group (68 men; 30 women) was 58.5 years. Seventy-one patients (72%) had involuntary weight loss. Hepatomegaly was found in 79 patients (81%). Eighty-two patients (89%) had proteinuria, and 81 patients (86%) had elevated serum alkaline phosphatase levels. Seventy-six patients (83%) had either a serum or urine monoclonal protein. Before liver biopsy, clinicians considered amyloidosis in the differential diagnosis for only 14 patients (26%). None of our patients experienced hepatic rupture or death due to liver biopsy, and only 4 (4%) bled after liver biopsy. The median survival of the 98 patients was 8.5 months. Predictors of a poor prognosis were congestive heart failure, elevated concentrations of bilirubin, and a platelet count greater than 500 x 109/L. In conclusion, clinicians should consider the diagnosis of primary hepatic amyloidosis in patients who present with involuntary weight loss or hepatomegaly. Other clues to the diagnosis include an unexplained elevated serum alkaline phosphatase level, proteinuria, and evidence for hyposplenism (for example, Howell-Jolly bodies on peripheral blood smear). Liver biopsy was safe. Some patients benefit from systemic chemotherapy.
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PMID:Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. 1453 Jul 78

Although the liver and spleen are frequently involved in primary systemic amyloidosis, the clinical manifestations of hepatic and splenic involvement are usually mild and a dominant symptomatic hepatic presentation is uncommon. We report a case of a 51-year-old woman with giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, and ascites, in whom the findings of dual-phase spiral computed tomography suggested liver and splenic amyloidosis.
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PMID:Hepatic and splenic amyloidosis: dual-phase spiral CT findings. 1462 77

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

This is the case of a patient who presented with severe right-sided heart failure due to diastolic dysfunction that caused a dilemma of differential diagnosis between restrictive cardiomyopathy and constrictive pericarditis. Restrictive cardiomyopathy was diagnosed based on noninvasive and invasive hemodynamic testing. However, the patient did not respond to therapy and succumbed to worsening heart failure and multiple comorbidities. Clinical features of right heart failure with edema, ascites, jugular venous distention, and tender hepatomegaly are commonly seen in clinical practice. When systolic function is determined to be normal, diastolic causes of heart failure must be ruled out. These include myocardial disorders with a broad range of pathologies leading to restrictive physiology, of which amyloidosis is a prototype. Pericardial disorders leading to diastolic heart failure are usually in the form of constrictive physiology, when pericardial tamponade is ruled out. Differentiation between restrictive and constrictive pathologies is often difficult and requires careful attention to hemodynamic and Doppler echocardiographic features. We report a case of severe right heart failure illustrating some of the complexities in decision-making and the importance of meticulous hemodynamic and ancillary testing in the diagnosis and treatment of this often fatal condition.
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PMID:Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management. 1547 69

Amyloidosis is the term for specific pathological peptide deposits in various tissues. Amyloid substances may be the manifestation of the following nosological units: AL-amyloidosis, AA-amyloidosis, ATTR-amyloidosis, beta2-microglobulin amyloidosis and possibly othr familiar forms of amyloidosis. The most frequent symptoms of amyloidosis are: 1. proteinuria with nephrotic syndrome, 2. cardiac failure with restrictive cardiomyopathy, 3. unexplicable hepatomegaly, 4. idiopathic peripheral polyneuropathy, 5. haemorrhagic manifestations and symptoms of increased fragility of the capillaries while the number of thrombocytes is normal, 6. periorbital haematomas and 7. macroglossia. Diagnostic and therapeutic procedures differ according to the type of amyloidosis and are analyzed in detail in the text.
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PMID:[Clinical manifestations of AL-amyloidosis and some other types of amyloidosis]. 1563 67

We here report a case of a 50-year-old man who showed histologically evident resolution of primary amyloidosis by melphalan and prednisolone. The patient was admitted to our hospital for further evaluation of nephrotic syndrome and remarkable hepatomegaly with refractory ascites, on September 11, 1998. Laboratory tests at presentation showed nephrotic syndrome with slight renal impairment and elevation of the enzymes of the biliary system. Monoclonal light chains were not detected in the serum or urine by immunoelectrophoresis. A renal biopsy revealed global deposition of amyloid in all glomeruli, interstitium and blood vessels. Immunofluorescence staining was positive for kappa light chains. Liver biopsy specimens showed extensive deposition of amyloid along sinusoid walls. Bone marrow aspiration contained 7% plasma cells but no clusters or abnormal cells. Based on these findings, systemic AL- (amyloid light chain) amyloidosis was diagnosed, and the treatment with combinations of melphalan and prednisolone was started from October 1998 at intervals of 4-6 weeks. Renal impairment progressed, resulting in the initiation of maintenance hemodialysis in February 1999. Reinfusion of ascitic fluid into the hemodialysis circuit had been performed from March 1999 for refractory ascites, and ascites disappeared in July 1999. Furthermore, urinary output increased after 14 courses of chemotherapy. Renal function gradually ameliorated with a concomitant reduction in the enzymes of biliary system, and finally hemodialysis was discontinued in April 2001. Sixteen courses of chemotherapy were administered by April 2001. Proteinuria was negative in August 2001. A second renal biopsy was performed on November 20, 2001, which showed markedly decreased amyloid deposition and a proliferation of mesangial cells and increase in matrix in various degrees. We report a case of a patient with primary amyloidosis who was successfully treated by melphalan and prednisolone, resulting in marked resolution of renal amyloidosis.
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PMID:Resolution of primary amyloidosis by melphalan and prednisolone: a case report. 1578 23


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