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Keshan disease — Clinical Guidelines

Overview

Keshan disease is a cardiomyopathy primarily associated with severe selenium deficiency, often exacerbated by Coxsackievirus B3 infection and other environmental factors, leading to myocardial damage and necrosis 4 6.

Diagnosis

Serum/Hair Selenium Levels: Measure serum selenium levels (ideally >1.0 μmol/L) and hair selenium levels (ideally >0.34 mg/kg) to identify deficiency 2 3.

Coxsackievirus B3 Detection: Detect Coxsackievirus B3 RNA in myocardial tissue via in situ hybridization and RT-PCR to assess viral involvement 4.

Myocardial Biopsy: Evaluate myocardial ultrastructural changes and enzyme activities (e.g., glutathione peroxidase, alpha-GPD) to confirm myocardial damage 6 10.

Management

Selenium Supplementation: Administer selenium supplements to correct deficiency; specific doses not detailed but supplementation shown to alleviate some symptoms 6 10.

Vitamin E and Other Antioxidants: Consider vitamin E supplementation to protect against oxidative stress and mitigate myocardial damage 7 8.

Nitrite Reduction: Minimize exposure to sodium nitrite, which can exacerbate myocardial glutathione peroxidase activity reduction 8.

Special Populations

Pregnancy: Specific management guidelines not provided; selenium deficiency risks may be heightened, warranting close monitoring 10.

Pediatrics: Selenium deficiency impacts are noted in endemic areas; supplementation may be crucial for prevention 10.

Elderly: Increased susceptibility to myocardial damage; selenium and antioxidant support may be particularly beneficial 6 7.

Key Recommendations

Screen for Selenium Deficiency: Regularly measure serum and hair selenium levels in endemic populations to identify at-risk individuals (Evidence: Moderate 2 3).

Supplement with Selenium: Implement selenium supplementation to correct deficiency and potentially reduce disease incidence and severity (Evidence: Moderate 6 10).

Consider Antioxidant Support: Include vitamin E supplementation alongside selenium to protect against oxidative stress and myocardial damage (Evidence: Moderate 7 8).

References

1 Wang Q, Zhan S, Han F, Liu Y, Wu H, Huang Z. The Possible Mechanism of Physiological Adaptation to the Low-Se Diet and Its Health Risk in the Traditional Endemic Areas of Keshan Diseases. Biological trace element research 2022. link 2 Liu X, Wang Y, Han S, Zhang Y, Zou Y, Su S et al.. A Spatial Ecological Study on Serum Selenium and Keshan Disease in Heilongjiang Province, China. Biological trace element research 2021. link 3 Zhang X, Wang T, Li S, Ye C, Hou J, Li Q et al.. A Spatial Ecology Study of Keshan Disease and Hair Selenium. Biological trace element research 2019. link 4 Ren LQ, Li XJ, Li GS, Zhao ZT, Sun B, Sun F. Coxsackievirus B3 infection and its mutation in Keshan disease. World journal of gastroenterology 2004. link 5 Wu HY, Xia YM, Ha PC, Chen XS. Changes in myocardial thyroid hormone metabolism and alpha-glycerophosphate dehydrogenase activity in rats deficient in iodine and selenium. The British journal of nutrition 1997. link 6 Xu GL, Wang SC, Gu BQ, Yang YX, Song HB, Xue WL et al.. Further investigation on the role of selenium deficiency in the aetiology and pathogenesis of Keshan disease. Biomedical and environmental sciences : BES 1997. link 7 Liu SY, Wang F. Damage to hepatic thyroxine 5'-deiodination induced by pathogenic factors of Keshan disease and the preventive effects of selenium and vitamin E. Biomedical and environmental sciences : BES 1991. link 8 Yang Q, Wang F. Effect of sodium nitrite on myocardial glutathione peroxidase and protective action of vitamin E and selenium. Biomedical and environmental sciences : BES 1991. link 9 Li C, Wang F, Wang LJ, Li CK. Increased sarcolemmal membrane permeability of cardiac muscle cells in rats fed grains cultivated in a Keshan disease endemic area: a study with horseradish peroxidase as a tracer. Experimental and molecular pathology 1989. link90041-5) 10 Xia YM, Hill KE, Burk RF. Biochemical studies of a selenium-deficient population in China: measurement of selenium, glutathione peroxidase and other oxidant defense indices in blood. The Journal of nutrition 1989. link

Keshan disease