Endomyocardial Fibrosis: Still a Mystery after 60 Years

Division of Social Medicine and Health Inequalities, Brigham and Women's Hospital, Boston, Massachusetts, USA.
PLoS Neglected Tropical Diseases (Impact Factor: 4.49). 02/2008; 2(2):e97. DOI: 10.1371/journal.pntd.0000097
Source: PubMed

ABSTRACT The pathologist Jack N. P. Davies identified endomyocardial fibrosis in Uganda in 1947. Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America. In Kampala, the disease accounts for 20% of heart disease patients referred for echocardiography. We conducted a systematic review of research on the epidemiology and etiology of endomyocardial fibrosis. We relied primarily on articles in the MEDLINE database with either "endomyocardial fibrosis" or "endomyocardial sclerosis" in the title. The volume of publications on endomyocardial fibrosis has declined since the 1980s. Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.


Available from: Gene Bukhman, Dec 11, 2014
1 Follower
  • [Show abstract] [Hide abstract]
    ABSTRACT: To explore the toxic effects of rare earth elements (REEs) accumulated in human body, adopting the inductively coupled plasma mass spectrometry (ICP-MS) method, the present study measured REEs and the contents of eight other elements (Ca, Fe, Cu, Na, K, Zn, Mg, and P) in the hair of eight persons exposed to soil containing REEs for a long time as well as in the control group. In addition, proteomic analysis of serum of the two groups was conducted by isobaric tags for relative and absolute quantitation (iTRAQ) technique. Experimental results show that in the hair of the two groups, 15 REEs and eight other elements were detected, in which the content of La, Ce, Pr, Nd, Tb, Ho, Tm, Yb, and Fe in the exposure group is significantly higher than that of the control group, but the content of Ca in the exposure group is significantly lower than that of the control group; analysis yields out 29 differentially expressed proteins, in which 16 proteins are upregulated and 13 proteins are downregulated. Bioinformatics analysis of differentially expressed proteins demonstrates that they participate in various biological processes and five Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways, forming an interaction network. Besides, some differentially expressed proteins may be related to neurovirulence, hepatotoxicity, pathological fibrosis, osteoporosis, and anticoagulation caused by REEs. The present experiment investigated the toxic effects of REEs accumulated in human body at the molecular level, which may lay a foundation for the future research of biological effect, threshold limit values, protection from exposure, and reasonable application of REEs.
    Biological trace element research 03/2015; DOI:10.1007/s12011-015-0312-9 · 1.61 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Abstract: Loeffler’s endocarditis and cardiac manifestations of the hypereosinophilic syndrome (HES) are rare and difficult to diagnose. We report a case of in a 36 year-old female with a history of rheumatoid arthritis with disabling dyspnea. The transthoracic echocardiogram demonstrated normal systolic cardiac functions and a left ventricular apical thrombus. However, using cardiovascular magnetic resonance (CMR) with inversion-recovery (IR) delayed enhancement, and cine steady-state free precession (SSFP) sequences, we were able to clearly demonstrate endocardial fibrosis, tissue inflammation, apical ventricular hypertrophy, and LV thrombus that correlate with clinical findings. We believe cardiac MRI is more useful than transthoracic echocardiography in the diagnosis and management of HES and ultimately it obviated the need for biopsy to confirm the diagnosis. Keywords: Loeffler’s, hypereosinophilic syndrome, endocarditis,CMR
    11/2014; 27(2). DOI:10.1016/j.jsha.2014.11.002
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: Endomyocardial fibrosis (EMF) is the most common form of restrictive cardiomyopathy worldwide. It has been linked to poverty and various environmental factors, but-for unknown reasons-only some people who live in similar conditions develop the disease. EMF cases cluster within both families and ethnic groups, suggesting a role for a genetic factor in host susceptibility. The human leukocyte antigen (HLA) system is associated with predisposition to various diseases. This two-center study was designed to investigate variation in the HLA system between EMF patients and unaffected controls. We provide the first genetic investigation of patients with EMF, as well as a comprehensive review of the literature. Methods: HLA class I (HLA-A, -B, -C) and class II (DRB1, DQB1) types were determined in 71 patients with severe EMF and 137 controls from Uganda and Mozambique. Chi Square analysis was used to identify any significant difference in frequency of class I and class II HLA types between cases and controls. Results: Compared to ethnically matched controls, HLA-B*58 occurred more frequently in Mozambique patients with EMF and HLA-A*02:02 occurred more frequently in Ugandan patients with EMF. Conclusions: Ample subjective evidence in the historical literature suggests the importance of a genetically susceptible host in EMF development. In this first formal genetic study, we found HLA alleles associated with cases of EMF in two populations from sub-Saharan Africa, with EMF patients being more likely than controls to have the HLA-B*58 allele in Mozambique (p-0.03) and the HLA-A*02:02 in Uganda (p = 0.005). Further investigations are needed to more fully understand the role of genetics in EMF development.
    12/2014; 2014(4):473-481. DOI:10.5339/gcsp.2014.60