Cardiomyopathy associated with nonendemic selenium deficiency in a Caucasian adolescent

Abstract

We describe a girl aged 17 y who died after a cardiac arrest secondary to septic shock. At autopsy, the enlarged, soft, and flabby heart showed microscopic evidence of acute myocardial infarction, myocardial edema, myocardiocyte loss, replacement fibrosis in the interventricular septum, and right and left ventricular hypertrophic nucleomegaly. The pathological diagnosis was that of cardiomyopathy due to prolonged selenium deficiency. The patient had been on total parenteral nutrition for 17 mo, following extensive bowel resection for intractable pain, nausea, and vomiting caused by chronic idiopathic intestinal pseudoobstruction. Seven months before death, when severe biochemical selenium deficiency was diagnosed, supplemental selenium was added to the infusion, and plasma selenium concentrations increased. In long-standing selenium deficiency, sepsis may contribute the final insult to a damaged myocardium, triggering symptomatic cardiac failure and sudden death.

Serum and Urine Selenium Concentrations in Patients with Cardiovascular Diseases and Relationship to Other Nutritional Indexes

“…Several researchers found significantly lower plasma Se concentrations in patients with congestive heart failures and other cardiovascular diseases in comparison with those measured in healthy subjects [10,11]. However, the precise mechanism by which low plasma Se levels act in these diseases is not known.…”

The Role of Selenium Deficiency in Dilated Cardiomyopathy in Saudi Arabia

“…2,7,8 In Western countries, a small number of cases of dilated cardiomyopathy complicating selenium deficiency have been reported mainly in patients receiving long-term parenteral nutrition, [9][10][11][12][13][14] or in association with other disease states, such as the chronic uremic syndrome, 15 human immunodeficiency virus infection, 16,17 and autosomal recessive dystrophic epidermolysis bullosa. 18 Selenium deficiency has also been documented in black African women with peripartum cardiomyopathy.…”

Dietary Reference Intakes for Japanese 2010: Microminerals

“…Keshan disease, an endemic form of fatal cardiomyopathy that has been observed in children living in a low-selenium area of China, has been firmly linked to selenium deficiency, with administration of selenium having been found to prevent it (65). Several clinical selenium-responsive syndromes have been observed in patients receiving prolonged TPN, among whom one patient with an extremely low plasma selenium concentration (9 ng/mL) developed muscle pain and tenderness in the thighs, resulting in an inability to walk (66), while another developed a cardiomyopathy and died after a cardiac arrest secondary to septic shock (67).…”