Severe Selenium Deficiency Secondary to Chylous Loss 
From the * Division of Gastroenterology,
Hepatology and Nutrition, Correspondence: L. Michaud, Unité de Gastro-entérologie, Hépatologie et Nutrition, Clinique de Pédiatrie, Hôpital Jeanne de Flandre, 59037 Lille cedex, France. Electronic mail may be sent to l-michaud{at}chru-lille.fr. Selenium deficiency is observed in patients with poor intake. We report the first case of a child with lymphangiomatosis who presented a myopathy associated with severe cardiomyopathy secondary to selenium deficiency due to selenium loss in chylous fluid. Selenium is an essential trace element. It is an integral part of the enzyme glutathione peroxidase (GSH-Px), which catalyzes the reduction of organic hydroperoxides and hydrogen peroxides by glutathione, and protects against membrane oxidative damage.1 In some parts of the world, like China, with a poor soil selenium content, various endemic affections are common: Keshan's disease, a dilated cardiomyopathy, and Kashin-Beck's disease, an osteoarthropathy, both related to selenium deficiency. Except for these specific geographical conditions, selenium deficiency has only been observed in patients with low intake (parenteral nutrition), low-protein diet (phenylketonuria), or severe malabsorption.1–3 We report a case of selenium deficiency due to excessive selenium loss by chylous fluid.
An 11-year-old boy was admitted to our hospital for disseminated lymphangiomatosis. Despite a surgical ablation performed elsewhere 6 years earlier, cystic lymphangioma initially in the pelvis recurred on the right thigh, extending secondarily to the kidney, urethra, and bladder, and a bilateral chylothorax appeared 7 years after diagnosis. During this period, nutrition had to be adapted to the clinical evolution: he received a normocaloric diet with 5% long-chain fatty acid and 15% medium-chain triglycerides. When decompensated, parenteral nutrition (PN) was started according to recommendation and including IV trace elements cocktail, 40 mL/d, devoid of selenium (Heptan, Aguettant, Lyon, France), iron, vitamin B12, and IV lipid emulsion (Intralipid, Kabi, Sèvres, France; 1 g/kg/d), whereas albumin loss was compensated by IV 4% albumin (100 mL/L of lymph). At the age of 14 years (weight: 40 kg, –1 SD), evolution led to the constitution of a scrotal fistula with chylous fluid flight, associated with chyloperitoneum and ileus. Despite PN, IV  -interferon
for 3 months, scrotal fistula continued to produce lymph with a mean high
output of 4 L/d (maximal output: 8 L/d). All-in-one PN was supplemented with
electrolytes, vitamins, and trace elements, including selenium (5µg/kg),
according to the recommended
intake.4 The patient
developed an acute respiratory failure and was transferred to the intensive
care unit (ICU). One and a half months later, while still in the ICU for an
unexplained respiratory failure, he developed hypotonia with weakness in the
legs. Electromyography demonstrated the absence of neuropathy but signs in
favor of a myopathy. A cardiomyopathy was found at ultrasound examination:
left ventricular ejection fraction was reduced to 0.45 of normal (N: 0.66) and
cardiac index to 3.85 (N: 3.1–3.8 L/min/m2), without
associated pulmonary hypertension. At that time, laboratory data showed a
profound decrease of serum selenium concentration at 9.5 µg/L (normal
range: 55–150 µg/L; the blood sample was put in dried tube, then
centrifuged, and selenium was estimated by spectrophotometry of atomic
electrothermics absorption). After increase of IV supplementation of selenium
to 400 µg/d, the serum selenium concentration increased to normal level
(110 µg/L) within 8 days, in parallel with improvement of clinical muscle
testing and functional recovery, but the child could not walk, and did not
return to normal cardiac function. Dosage of selenium in the chylous flight
reached values of 6.3–18.7 µg/L, corresponding to a mean selenium
loss of 45–50 µg/d and maximal loss of 100–150 µg/d. Other biologic data at the time of deficiency in the ICU were all normal: serum and erythrocytic magnesium, carnitine, folate, vitamin B12, and amino acids (valine, leucine, histidine). The patient died 2 years later at the age of 16 from progression of lymphangiomatosis involving the intestine and the lungs, with hypovolemic and cardiogenic shock. Just before death, while receiving high-dose supplements of selenium in the PN, serum selenium concentration (65 µg/L) and erythrocyte glutathione peroxidase activity (30 U/g Hb; normal: 13–25 U/g Hb) were within normal ranges.
In our patient, selenium deficiency was not related to low selenium intake during prolonged PN because selenium supplementation was made according to the recommendation.4 The responsibility of prolonged PN without selenium supplementation has already been reported.5,6 PN solutions have negligible levels of selenium as protein source is crystalline amino acids and that they are not routinely supplemented with selenium.5,7 This patient received a dose of 200 µg of selenium daily in PN by means of a pediatric trace element solution (selenium 5 µg/mL, Oligo-éléments Pédiatriques, Aguettant, Lyon, France) that was superior to maintenance needs in children (1.5 µg/kg/d).8 In patients receiving long-term PN without selenium supplementation, selenium deficiency may cause cardiomyopathy.4,7,9–11 In some cases, outcome was fatal, whereas in other cases, there was an improvement with selenium supplementation.6 Other factors may be associated with selenium deficiency in the physiopathology of cardiomyopathy, such as vitamin E deficiency, stress, and concomitant infection (Coxsackie B virus).2 Skeletal muscle disorders, including muscle pain, fatigue, proximal weakness, and creatine kinase elevation, have also been reported in patients with selenium deficiency. All symptoms resolve in a majority of cases after selenium supplementation in PN.11 In our case, we could demonstrate that selenium deficiency was due to chylous loss secondary to lymphangiomatosis. To our knowledge, selenium deficiency due to lymph loss has not been previously described, and we could not find literature data on the selenium composition of lymph. Our case suggests that lymph loss due to exudative enteropathy and lymphangiomatosis should be considered as an important risk factor of selenium deficiency. Because lymph is rich in selenium, selenium concentration should be closely monitored and selenium intake should be increased in case of selenium deficiency. Received for publication November 16, 2004. Accepted for publication December 7, 2005.
Journal of Parenteral and Enteral Nutrition, Vol. 30, No. 2,
173-174 (2006)
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CASE REPORT
DISCUSSION