Keshan disease: An entity or not

Yu, Peiliang

doi:10.1016/s0046-8177(88)80275-2

“…It takes into account the malabsorption commonly seen in untreated CFand pancreatic lipomatosis as well as the chronic starvation seen in Chinese children. In Shandong province,``the main staple of the children was a kind of dry roll made of sweet potato £our which is practically devoid of water-soluble vitamins and lacking in protein'' [15]. The fact that myocardial necrotic changes also have been reported in 350 C. Nezelof et al patients who underwent extensive intestinal resection and were put on total parenteral nutrition [3,18] also tends to support this nutritional de¢ciency hypothesis.…”

Section: Discussionmentioning

confidence: 99%

Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Nézelof¹,

Bouvier

²

,

Dijoud

³

2002

Pediatric Pathology & Molecular Medicine

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Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.

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“…It takes into account the malabsorption commonly seen in untreated CFand pancreatic lipomatosis as well as the chronic starvation seen in Chinese children. In Shandong province,``the main staple of the children was a kind of dry roll made of sweet potato £our which is practically devoid of water-soluble vitamins and lacking in protein'' [15]. The fact that myocardial necrotic changes also have been reported in patients who underwent extensive intestinal resection and were put on total parenteral nutrition [3,18] also tends to support this nutritional de¢ciency hypothesis.…”

Section: Discussionmentioning

confidence: 99%

“…A decrease in the incidence of Keshan disease was indeed observed, but this occurred both in areas in which the selenium supplement was given as well as in the control areas. The concomitant introduction of ¢sh soup and a less monotonous diet [15] could provide a possible explanation for these con£icting and inconclusive results. Moreover, the fact that selenium supplements were not able to reverse the ultrastructural changes in the myocardia of latent Keshan disease cases [15] renders even more uncertain the exclusive role of selenium de¢ciency in the development of myocardial changes.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Nézelof¹,

Bouvier

²

,

Dijoud

³

2002

Pediatric Pathology & Molecular Medicine

11

1

0

View full text Add to dashboard Cite

Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.

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“…Individuals living in those areas had low dietary intakes of Se that were reflected in low serum and hair levels of Se. Populations living in areas of China with Se-rich soil did not develop KD [ 25 – 28 ].…”

Section: Discussionmentioning

confidence: 99%

Selenium in the Prevention of Anthracycline-Induced Cardiac Toxicity in Children with Cancer

Taçyıldız

¹

,

Özyörük

²

,

Kavas

³

et al. 2012

Journal of Oncology

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High cumulative doses of anthracyclines (300–500 mg/m2) used in the treatment of children with cancer may result in cardiotoxicity, a major long-term adverse effect that limits clinical usefulness of this class of chemotherapeutic agents. We assessed anthracycline-induced cardiotoxicity by measuring Pro-BNP levels and echocardiographic (ECHO) findings and investigated potential protective effect of selenium (Se) supplementation in a group of pediatric cancer patients. Plasma level of Pro-BNP was measured, and ECHO was performed in 67 patients (45 boys, 22 girls; ages 2–18 years; median age 12 years) after they completed anthracycline-containing chemotherapy. Serum Se level was measured in 37 patients. Eleven patients had high Pro-BNP levels and/or cardiac failure with Pro-BNP levels of 10–8,022 pg/mL (median 226.3 pg/mL; laboratory normal level is less than 120 pg/mL). Serum Se levels were low (20–129 mcg/L, median 62 mcg/L) in ten of these eleven patients. Eight of 10 patients with low Se and high Pro-BNP levels were supplemented with Se 100 mcg/day for a period of 4–33 months (median 6 months) which resulted in improvement in Pro-BNP and/or ECHO findings. These results suggest that Se supplementation may have a role in protection against anthracycline-induced cardiac toxicity.

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Keshan disease: An entity or not

Cited by 6 publications

References 1 publication

Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Selenium in the Prevention of Anthracycline-Induced Cardiac Toxicity in Children with Cancer

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