Selenium and chromium deficiency during long-term home total parenteral nutrition in chronic idiopathic intestinal pseudoobstruction.

Abstract

A 35-y-old man was admitted for muscle weakness of the limbs, especially the proximal lower limbs, and a progressive rise in serum creatine phosphokinase (CPK). A diagnosis of chronic idiopathic intestinal pseudoobstruction (CIIP) had been made when the patient was 22 y old. Since then, he had received total parenteral nutrition (TPN) repeatedly. Because of recurrent abdominal symptoms such as ileus for many years, a subcutaneous port for TPN was implanted 2 y ago for home TPN. The patient received 2.4 L of TPN solution daily. The solution was composed of 500 g glucose, 10% amino acids, and standard maintenance electrolytes. In addition, a multivitamin solution and essential elements, 35 mmol Fe, 20 mmol Mn, 60 mmol Zn, 5 mmol Cu, and 1 mmol I, were administered; fat emulsions were administered every other day. Thirteen years after the start of home TPN, the patient complained of muscle weakness in both the upper and lower limbs, especially the proximal lower limbs. But he did not complain of muscle pain. Blood chemistry results showed a progressive rise in serum CPK. The patient was admitted to the hospital for evaluation and therapy of muscle weakness and the increased CPK. On physical examination at admission, the patient’s vital signs were within normal limits. Neurologic findings revealed proximal dominant muscle weakness, but the cranial nerve, sensory nerve, coordination, and autonomic nerve were intact. Deep tendon reflexes were diminished. The other physical findings were unremarkable. Laboratory data on admission are summarized in Table I. Isozymes of CPK in serum were shown to be mainly of the MM type. Aldolase and lactate dehydrogenase were also significantly elevated in the serum. Antinuclear antibody and anti–Jo-1 antibody were negative, and thyroid function was within normal limits. Results of the chest X-ray, electrocardiogram, and cardioechogram were within normal limits. A peripheral muscle electromyographic study revealed a myogenic pattern of short duration and low amplitude. Muscle biopsy revealed myopathic changes with mild variation in size and regeneration of muscle fibers and muscle cell necrosis. Infiltration of inflammatory cells was not observed. Selenium deficiency was suspected to be the cause of the patient’s symptoms because of the patient’s long period of TPN dependency and because the serum concentration of selenium was found to be very low (0.1 mg/dL versus normal 9.7–16.0 mg/dL). Therefore, supplemental selenium of 100 mg