Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The detailed features of right sided endomyocardial fibrosis are described in 15 out of 10,000 consecutive patients who all had infection with Schistosoma mansoni and came from rural Egypt. Laboratory investigations, 12 lead electrocardiography, chest radiography, and Doppler echocardiography were performed in all patients. Cardiac catheterisation and angiography were performed in eight. Endomyocardial biopsy specimens were obtained from the right ventricles of two patients and pericardial biopsy specimens from two. Pericardiocentesis was performed in all patients. All patients were infected with S mansoni and had schistosomal hepatic fibrosis and ascites. Eleven had splenomegaly. All patients had raised cervical venous pressure with prominent Y descent and atrial fibrillation. Eosinophilia was notably absent. Echocardiography showed apical fibrosis in the right ventricle, obliteration of the ventricle, and moderate to massive exudative pericardial effusion in all patients. Calcification and fibrosis extended into the right ventricular outflow tracts in two patients. Huge right atrial thrombi occurred in five patients. Tricuspid regurgitation (grades I-II) was detected in 11 patients by Doppler ultrasonography. Haemodynamic and angiographic data confirmed the pure right sided restrictive pathophysiology. Pericardial biopsy specimens showed perivascular inflammatory infiltrates in two patients and a schistosomal granuloma in one. Endocardial biopsy specimens showed dense fibrosis with many fibroblasts. Endomyocardial fibrosis in Egypt is unique in several aspects. It always affected only the right side of the heart. Calcification and fibrosis extended to the right ventricular outflow tract. Pericardial inflammatory reaction was present. The relation to schistosomiasis and the link to periportal hepatic fibrosis in these patients is intriguing.
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PMID:Endomyocardial fibrosis in Egypt: an illustrated review. 915 33

Endomyocardial disease is a form of restrictive cardiomyopathy, of unknown etiology, which occurs most commonly in tropical and subtropical areas. It is characterized by the formation of endomyocardial fibrosis of the apical and subvalvular regions of one or both ventricles. A 29-year-old male patient was admitted with restrictive cardiomyopathy and decompensated heart failure. Telecardiography showed cardiomegaly and right pleural effusion. Transthoracic echocardiography revealed preserved left ventricular systolic functions, biatrial dilatation, apical obliteration of both ventricles, increased endocardial echoreflectivity, and pericardial effusion. The right ventricular outflow tract was dilated. There was no endocardial thickening in this region. Doppler examination showed grade 3 mitral and tricuspid regurgitation. Ventriculograms showed apical obliteration of both ventricles, marked decrease in the size of the right ventricular cavity, significant dilatation of the right ventricular outflow tract and both atria, and severe mitral and tricuspid regurgitation. Laboratory findings showed no hypereosinophilia. Hepatic congestion, splenomegaly, and ascites were noted on abdominal ultrasonography. Following cardiac catheterization, the patient was placed on the waiting list for cardiac transplantation.
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PMID:[Endomyocardial disease: a case report]. 1940 38

Osteopetrosis is a rare genetic disorder of osteoclast dysfunction leading to anatomical and physiological disorders. We present the anesthesia management for the femur fracture of a 4-year-old girl with malignant infantile type of osteopetrosis. She had a ventriculoperitoneal shunt, impaired motion, visual disturbance, growth failure, facial deformity, heart murmur of moderate tricuspid regurgitation, and left ventricular heart failure, with splenomegaly and severe anemia.
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PMID:Anesthesia Management of a Child with Osteopetrosis. 2892 94