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)

Cardiac amyloidosis may occur in any type of systemic amyloidosis. The clinical picture is often characterized by restrictive cardiomyopathy. We report the case of a 41-year-old female patient admitted to the Department of Cardiology with clinical signs of right heart failure: congested jugular veins, hepatomegaly, peripheral edema, ascites associated with atrial fibrillation, low values of arterial blood pressure and oliguria. Echocardiographic findings were helpful for the diagnosis of cardiac amyloidosis: enlarged atrial cavities, normal size ventricles, thickened ventricular septum and posterior left ventricle wall with normal left ventricular ejection fraction, mitral and tricuspid regurgitation. Two-dimensional echocardiography revealed additional features: thickened papillary muscles and a specific "granular sparkling" appearance of the thickened cardiac walls - probably due to the amyloid deposit. Gingival biopsy showing amorphous eosinophilic material located in the vessel walls and the specific dichroism and "apple-green" birefringence under polarized light on Congo red stained slides completed the diagnosis of systemic amyloidosis. We recommend cardiologists to take into account a possible cardiac amyloidosis in a patient with unexplained refractory heart failure and a typical pattern of restrictive cardiomyopathy revealed by echocardiographic examination. We also emphasize the fact that the complete diagnosis cannot be set without a biopsy that should reveal the presence of amyloid. Although endomyocardial biopsy, completed with histochemical and immunohistochemical stains, is a valuable diagnostic method, in cases with advanced cardiac failure, the best site for this biopsy may be the gingiva.
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PMID:Amyloidosis - a rare cause of refractory heart failure in a young female. 2852 19

Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.
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PMID:Diagnosis of Budd-Chiari syndrome. 2928 98

Budd-Chiari syndrome (BCS) is a disorder with numerous causes that is a result of hepatic outflow obstruction, in the absence of right heart failure or constrictive pericarditis. Acute Budd-Chiari syndrome is uncommon and clinically characterized by ascites, hepatomegaly, and hepatic insufficiency. In the majority of cases, patients present with chronic BCS, showing a dysmorphic liver disease with variable fibrosis deposition. In chronic Budd-Chiari syndrome, hepatocellular carcinoma (HCC) and benign regenerative nodules (called large regenerative nodules or FNH-like lesions) have been described in the literature. Very few studies have reported magnetic resonance imaging (MRI) findings about these nodules, using hepatobiliary contrast medium. The aim of our review is to describe the magnetic resonance imaging findings of hepatic regenerative nodules in BCS, with emphasis on the hepatobiliary phase, and to compare the imaging features of benign nodules with those of HCC.
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PMID:Budd-Chiari Syndrome and hepatic regenerative nodules: Magnetic resonance findings with emphasis of hepatobiliary phase. 3130 41


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