Keshan Heart Disease

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Sci China C Life Sci. 2006 Dec;49(6):513-8.
Keshan disease and mitochondrial cardiomyopathy.
Fuyu Y.
National Laboratory of Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, China.

Keshan disease (KD) is a potentially fatal form of cardiomyopathy (disease of the heart muscle) endemic in certain areas of China. From 1984 to 1986, a national comprehensive scientific investigation on KD in Chuxiong region of Yunnan Province in the southwest China was conducted. The investigation team was composed of epidemiologists, clinic doctors, pathologists, biochemists, biophysicists and specialists in ecological environment. Results of pathological, biochemical and biophysical as well as clinical studies showed: an obvious increase of enlarged and swollen mitochondria with distended crista membranes in myocardium from patients with KD; significant reductions in the activity of oxidative phosphorylation (succinate dehydrogenase, cytochrome oxidase, succinate oxidase, H(+)-ATPase) of affected mitochondria; decrease in CoQ, cardiolipin, Se and GSHPx activity, while obvious increase in the Ca2+ content. So, it was suggested that mitochondria are the predominant target of the pathogenic factors of KD. Before Chuxiong KD survey only a few cases of mitochondrial cardiomyopathy were studied. During the multidisciplinary scientific investigation on KD in Chuxiong a large amount of samples from KD cases and the positive controls were examined. On the basis of the results obtained it was suggested that KD might be classified as a "Mitochondrial Cardiomyopathy" endemic in China. This is one of the achievements in the three years' survey in Chuxiong and is valuable not only to the deeper understanding of pathogenic mechanism of KD but also to the study of mitochondrial cardiomyopathy in general. Keshan disease is not a genetic disease, but is closely related to the malnutrition (especially microelement Se deficiency). KD occurs along a low Se belt, and Se supplementation has been effective in prevention of such disease. The incidence of KD has sharply decreased along with the steady raise of living standard and realization of preventive measures. At present, patients of KD are very sparse. In recent years the research on the non-KD mitochondrial cardiomyopathy has progressed rapidly. Given the advances in this aspect a minireview is written to evaluate the classification of KD as a kind of mitochondrial cardiomyopathy.

Postepy Hig Med Dosw (Online). 2006;60:624-31.
Selenium and cardiovascular disease: selected issues
Zagrodzki P, Laszczyk P.
Zaklad Bromatologii Collegium Medicum Uniwersytetu Jagiellonskiego w Krakowie, Kraków, Poland.

The paper presents current knowledge of the role of selenium in the development of cardiovascular system disease (CSD). Already known mechanisms of selenium action in the cardiovascular system are described, whilst underlining the fact that they do not explain all relevant observations and need to be clarified by more studies. Clinical, epidemiological, and experimental studies devoted to the relationship between the progression of CSD and selenium status indices are then reviewed. It could be expected that any explanation of the pathophysiological mechanisms of the influence of tobacco smoking (as one of the classical risk factors for CSD) on selenium status might help to bring about a better understanding of the progression of cardiovascular disorders. Based on studies conducted on animal models, the role of selenium in the antioxidant defense of cardiac muscle is described. Particular attention is paid to a dilated cardiomyopathy known as Keshan disease, for which it has been shown that selenium deficiency is an environmental factor predisposing to the onset of this disease. Similar symptoms of cardiomyopathy are also observed in patients on total parenteral nutrition and patients with AIDS.

Wei Sheng Yan Jiu. 2002 Aug;31(4):261-3.
Coxsackievirus B3 infection and Keshan disease
Huang Z, Xia Y, Jin Q, Wu H.
Institute of Nutrition and Food Hygiene, Chinese Academy of Preventive Medicine, Beijing 100050, China.

To study the relationship between the infection of enteroviruses and the etiology of keshan disease, we examined the 30 blood samples from the latent-chronic KD patients from Mianning, Xider, Derchang county of Sichuan Province with LDE-PCR (long distance enterovirus-specific RT PCR), two sensitive and specific ELISAs (one for CVB1-6 IgM and the other for CVB1-6 IgG), and three-primer RT PCR specific for CVB3. Results show that: 1) The infection rate of enteroviruses in the samples from the latent-chronic KD patients is higher than from the control group (80% VS 0%, P < 0.01); 2) The CVB1-6 antibody positive rate in latent-chronic KD is higher than that of the control group (IgM, 33% VS 0%, P < 0.01; IgG, 23% VS 0%, P < 0.01); 3) 16.6% (4/24) of enteroviruses positive samples or 40% (4/10) of CVB1-6 IgM antibody positive samples can be identified as CVB3. So, the infection of enter viruses maybe involves in the cause and development of latent-chronic keshan disease, and at least we conclude that the mutation of CVB3 is not the only cause of KD.

Med Sci Law. 2002 Jan;42(1):10-3.
Fulminant heart failure due to selenium deficiency cardiomyopathy (Keshan disease).
Burke MP, Opeskin K.
Victorian Institute of Forensic Medicine, Department of Forensic Medicine, Monash University, Southbank, Australia.

Selenium deficiency is a rare cause of cardiomyopathy that may be encountered by the forensic pathologist. Selenium deficiency is associated with a cardiomyopathy, myopathy and osteoarthropathy. In Asia and Africa, dietary selenium deficiency is associated with a cardiomyopathy known as Keshan disease and an osteoarthropathy called Kashin-Beck disease. Chronic selenium deficiency may also occur in individuals with malabsorption and long term selenium-deficient parenteral nutrition. Selenium deficiency causes myopathy as a result of the depletion of selenium-associated enzymes which protect cell membranes from damage by free radicals. We present a case of fulminant heart failure in a middle aged woman with a complex medical and surgical history including documented malabsorption and selenium deficiency. Pathological examination of the heart showed features consistent with Keshan disease.

Nippon Eiseigaku Zasshi. 2002 Jan;56(4):641-8.
Keshan disease--a review from the aspect of history and etiology
Liu Y, Chiba M, Inaba Y, Kondo M.
Department of Epidemiology and Environmental Health, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan.

OBJECTIVE: Keshan disease is an endemic cardiomyopathy found in Keshan, north-east China. The first patient was identified in 1935. This disease is characterized by a blood circulation disorder, endocardium abnormality and myocardium necrosis. Selenium (Se) deficiency is thought to be a major factor by Chinese scientists. However, the exact etiology has not been clarified up to now. The government decided to apply sodium selenite to growing crops, and the incidence of the disease decreased dramatically. However, a few cases still occur as chronic or latent types. This paper reviews Keshan disease from a historical aspect and also the present situation. METHODS: We made a reference survey and summarised the etiology, pathological changes, clinical manifestation, and other views of Keshan disease. RESULT: So far, epidemiological surveys have shown that Keshan disease occurs in large areas in 14 provinces in China, mainly in the countryside. It has been confirmed by clinical and pathological studies that Keshan disease is an independent endemic myocardial disease caused by biological and geological factors. The largest prevalence age rates are boys under 15 years old and women of childbearing age. There are several hypotheses; acute carbon monoxide poisoning, virus infection, malnutrition, or selenium deficiency. The first is not currently believed to be the cause. The following was pointed out; studies on the relationship between diet and the endemic areas of Keshan disease in 1961, where the food custom of the local residents was relatively simple and a so-called "one-sided diet" (eating a limited variety of food) might be related to the incidence of Keshan disease. In 1973, large-scale investigations on the natural environments were performed in the endemic areas of Keshan disease in the whole country. As a result, it was reported that there was a relationship between the incidence of Keshan disease and the special natural environment in the endemic areas and the cause of Keshan disease was strongly supported by nutritional, biological, geological and chemical (selenium deficiency) factors. In 1981, on the other hand, it was found that the levels of antibodies against Coxsackie virus were higher in the serum of Keshan disease patients than of normal subjects. This fact supposed that the cause of Keshan disease was related to a virus infection. However, it is difficult to explain why the clinical and pathological manifestations of Keshan disease are similar to those of other diseases, e.g. idiopathic dilatational myocardial disease. Further research should be performed on Keshan disease to clarify the etiology.

Biomedical and environmental sciences : BES1997 Sep; 10(2-3):316-26.
Further investigation on the role of selenium deficiency in the aetiology and pathogenesis of Keshan disease.
Xu GL, Wang SC, Gu BQ, Yang YX, Song HB, Xue WL, Liang WS, Zhang PY
Research Laboratory of Keshan Disease, Xi' an Medical University, China.

Selenium supplements were not able to restore the ultrastructural changes in the myocardium of latent Keshan disease patients taken by using cardiac catheter endomyocardial biopsy. Observations on the changes of selenium status and the incidence of Keshan disease showed that new latent and naturally-occurring chronic cases were found in the endemic area even after selenium levels had been elevated in the residents to the levels typical in the non-endemic area. These results indicate that although selenium deficiency might be a primary pathogenetic geogen in the occurrence of Keshan disease, it is rather a conditional predisposing factor than a specific or initiative aetiologic factor for the occurrence of Keshan disease. Selenium supplementation could apparently alleviate the higher platelet responsiveness of residents in the endemic area, which might contribute to eliminating the basis for the occurrence of the multifocal perivascular necroses in myocardium of acute and subacute Keshan disease.

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