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)

This report describes nine cases of endomyocardial fibrosis occurring in four families. All patients came from Rwanda or South-western Uganda, and five had tropical splenomegaly syndrome as well. It seems likely that genetic as well as environmental factors are operative in the aetiology of endomyocardial fibrosis.
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PMID:Familial cases of endomyocardial fibrosis in Uganda. 512 10

The prognosis of the hypereosinophilic syndrome (HS) depends mainly on the development of endomyocardial fibrosis (EMF). This complication may be overlooked at an early stage, although its presence is an indication for steroid or antimitotic therapy of the HS. Even at an advanced EMF and associated intracardiac thrombi may not be visualised by angiography. This study was undertaken to assess the diagnostic value of 2D echocardiography in 12 patients. The patients were all men (12 of them) aged 22 to 64 years with unexplained eosinophilia 1 500/mm3 for over 6 months, and visceral lesions. The patients were divided into 3 clinical groups. Group A comprised 4 "allergic" patients with chronic asthma and a significant elevation of IgE; Group B comprised 5 "myeloproliferative" patients with splenomegaly and/or hepatomegaly and a significant elevation of serum B12 levels. The 3 remaining patients who could not be allocated to either Group A or B formed the third group (Group C). 2D echocardiography was carried out on average 30 months after diagnosis of the HS and six planes of examination were used systematically (two parasternal, two apical, one extreme apical and one subcostal). Right and left ventriculography was performed in 6 patients (less than one month before or after 2D-echo). Anatomical studies were obtained in 4 cases (2 operations, 3 autopsies). Echocardiographic signs of EMF were observed in 8 cases. Four patients had a restrictive cardiopathy associated to a large LV thrombus in 2 cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cardiac manifestations of the hypereosinophilic syndrome. The value of 2-dimensional echography (12 cases)]. 643 27

Heart disease characterized by endomyocardial fibrosis is one of the major causes of morbidity and mortality in the idiopathic hypereosinophilic syndrome. From our series of 50 patients with idiopathic hypereosinophilia, we defined the noncardiovascular characteristics that distinguish patients at risk of developing endomyocardial fibrosis from those who remain free of heart disease. These groups did not differ with respect to the extent of eosinophilia or the duration of disease. Patients with clinically overt heart disease were more likely (p less than 0.05) to be male and HLA-Bw44 positive and have splenomegaly, thrombocytopenia, elevated serum levels of vitamin B12, and hypogranular or vacuolated eosinophils and abnormal early myeloid precursors in the peripheral blood. These idiopathic hypereosinophilic patients with heart disease were also more likely to have fibrosis and decreased megakaryocytes in the bone marrow. In contrast, those who remained free of heart disease tended to be female and have angioedema, hypergammaglobulinemia, elevated serum levels of immunoglobulin E (IgE), and circulating immune complexes. Therefore, in the idiopathic hypereosinophilic syndrome, male patients with a myeloproliferative type disorder and the HLA-Bw44 haplotype were at a much increased risk for the development of endomyocardial fibrosis. However, those patients with a hypersensitivity-like illness and angioedema who were female did not develop heart disease. Appreciation of this relative degree of risk for the major complication of the idiopathic hypereosinophilic syndrome should prove useful in the early identification and appropriate treatment of patients in whom endomyocardial fibrosis might develop.
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PMID:Noncardiovascular findings associated with heart disease in the idiopathic hypereosinophilic syndrome. 686 80

A 32-year-old man presented with left hypochondrial pain and myalgia. On examination he was found to have widespread lymphadenopathy and splenomegaly, and lymph node biopsy revealed diffuse lymphocytic lymphoma of the small cleaved cell type. There was hypereosinophilia (20 x 10(9)/1). The patient was initially treated with chlorambucil and prednisone, which controlled the lymphoma for 9 months but did not affect the eosinophilia. He then developed thrombocytopenia with further lymph node enlargement which was managed with a combination of bleomycin, vincristine and prednisone; this again controlled both spleen and lymph node size without affecting the eosinophilia. Six months after presentation the patient developed refractory cardiorespiratory failure due to endomyocardial fibrosis and fibrosing alveolitis. The cardiac failure was thought to be related to the hypereosinophilia resulting from the lymphoma, while the respiratory insufficiency was attributed to the bleomycin. This case illustrates the rare association between lymphocytic lymphoma and eosinophilia and shows that cardiac damage can be associated with these cells.
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PMID:Lymphocytic lymphoma, hypereosinophilia and endomyocardial fibrosis. 689 45

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

The aetiology of endomyocardial fibrosis (EMF) and tropical splenomegaly syndrome (TSS) though speculative, was considered by the author to be the same or related since the two diseases may occur in the same individual and locality. Accordingly, when attempting to prove a hypothesis for the causation of EMF that prolonged ingestion of tuber (cassava/tapioca) associated with extreme deprivation of protein causes EMF; one group of three Cercopithecus aethiops was fed on uncooked cassava while a second group was fed with uncooked bananas and in addition to harvesting the hearts whenever the animal health deteriorated, livers were also harvested for histological changes. While hearts from the animals on cassava revealed changes seen in human EMF the livers from the same animals exhibited Kupffer cell hyperplasia and hypertrophy as well as sinusoidal lymphocytosis, features seen in human TSS thereby confirming that the aetiology of these two diseases is the same. However, the banana diet did not produce such changes.
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PMID:Effects of cassava diet on Cercopithecus aethiops livers: a case for cassava as the cause of both tropical splenomegaly syndrome (TSS) and endomyocardial fibrosis (EMF). 875 24

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