Mild peripheral neuropathy but biochemical chromium sufficiency during 16 months of "chromium-free" total parenteral nutrition.

A 6-yr, 4-month-old boy was started on total parenteral nutrition (TPN) because of chronic diarrhea. The TPN regimen (3 liter/day) initially included supplemented Cr (3 micrograms/day) in addition to standard components (including FreAmine III). At age 8 yr, 8 months, the serum Cr level was elevated: 3.7 ng/ml (normal 0.03-0.85). A repeat level at the same time by another commercial laboratory was also high (7.0). Cr supplementation was stopped. At age 10 yr, he was noted to have mild peripheral neuropathy although glucose tolerance was excellent (alpha-linolenic acid was undetectable in the plasma). Cr status was reevaluated in a research lab. The serum level was 1.4 ng/ml (normal 0.05-0.4). The urine chromium excretion was 1.27 micrograms/day (normal 0.22). The TPN regimen (unsupplemented with Cr) provided 4 micrograms/day. Normal Cr intake is about 60 micrograms/day with 0.4% absorption (net 0.24 microgram/day). We conclude that Cr contamination of standard PN fluid may prevent biochemical evidence of low Cr status. In addition, alpha-linolenic acid-free parenteral nutrition for 46 months was not associated with clinically significant neurological dysfunction.

Manifestations of chronic selenium deficiency in a child receiving total parenteral nutrition.

A child receiving total parenteral nutrition for about 1 1/2 yr developed intermittent leg muscle pain and tenderness and elevation in serum activities of glutamic oxaloacetic transaminase, glutamic pyruvic transaminase, and creatine kinase. Approximately 6 months later he developed white fingernail beds. Detailed cardiological evaluation revealed no evidence of cardiac muscle cell damage despite markedly elevated serum activities of the MB isoenzyme of creatine kinase. Retrospective analyses of serum and 24 hr urine samples collected during this period demonstrated very low serum Se concentration (0.003 to 0.007 micrograms/g) and urine excretion (0.0 to 8.9 micrograms/day) Se. Intravenous Se supplementation (42 micrograms/day elemental Se as H2SeO3) and a prospective study of biochemical Se status were begun 33.5 months after initiation of total parenteral nutrition. Serum Se concentration and whole blood glutathione peroxidase activity and Se concentration were respectively 0.020 mu/g, 3.5 EU/g Hb, and 0.018 microgram/g, 1 month after intravenous Se therapy was started. These very low values increased after further Se therapy. Serum enzyme activities markedly improved and the fingernail bed abnormalities resolved after therapy.

Rickets in premature infants receiving parenteral nutrition: a case report and review of the literature.

There is a multifactorial etiology for rickets in premature infants receiving total parenteral nutrition. As an example of this, we describe a premature infant fed almost exclusively parenterally for the first 8 months of life who developed biochemical and radiologic evidence of rickets despite receiving vitamin D, calcium, and phosphorus at presently recommended levels. From our experience and a review of previous literature, there appears to be considerable uncertainty regarding the intravenous requirements of calcium, phosphorus, and vitamin D, particularly in low-birth weight infants. Therefore, measurements of serum 25-hydroxyvitamin D levels, vitamin D, and mineral intakes, and calcium and phosphorus excretion might be clinically useful for monitoring premature infants receiving total parenteral nutrition. Further research delineating more precisely the vitamin D, calcium, and phosphorus requirements of such infants is also suggested.

Biotin-responsive in vivo carboxylase deficiency in two siblings with secretory diarrhea receiving total parenteral nutrition.

Two siblings with a congenital syndrome of secretory diarrhea and seizures developed progressive skin rash, alopecia, and mucocutaneous candidiasis while receiving biotin-free total parenteral nutrition. Abnormally low urinary biotin excretion was associated with these clinical findings, but the serum concentration of biotin was within the normal range. There was also increased urinary excretion of lactic acid, 3-hydroxyisovaleric acid, 3-hydroxypropionic acid, and 3-methylcrotonylglycine. The younger of the two children subsequently died with severe metabolic acidosis. In the oder sibling, intravenous treatment with biotin (200 micrograms/day) resulted in resolution of the organic aciduria. A larger dose (10 mg/day) appeared to be required for rapid improvement in the skin lesions. These cases suggest that clinically significant biotin deficiency can occur in patients with chronic diarrhea receiving biotin-free total parenteral nutrition.

Amino acid utilization and urine protein excretion in children treated with succinylated Acinetobacter glutaminase-asparaginase.

Amino acid utilization was evaluated in seven children with acute lymphocytic leukemia treated with succinylated Acinetobacter glutaminase-asparaginase. All patients received food p.o. ad libitum and glucose-electrolyte solutions i.v.; four patients received an i.v. amino acid supplement (1.5 g/kg/day). Although all patients were in negative energy balance, there was a significant linear regression between nitrogen balance and nitrogen intake during Days 1 to 7 and Days 8 to 14 of the study. The slope of the regression line, reflecting exogenous nitrogen utilization, was not significantly different from that found in healthy young men ingesting adequate or subadequate energy intakes. The Y-intercept (-210 mg/kg/day) indicated an obligatory nitrogen loss that was much greater than normal. Most of the nitrogen loss was due to urinary excretion. Ammonia and urea accounted for 77 to 91% of the urine nitrogen. Urinary glutamate accounted for 4 to 10% of this loss. Urine protein excretion was abnormally high in each of the patients, ranging from 987 to 3440 mg/day. Urine excretion of N-acetyl-beta-glucosaminidase and beta 2-microglobulin was also abnormally high, despite normal blood urea nitrogen and serum creatinine, suggesting that these children had renal tubular dysfunction. The antileukemic effect of succinylated Acinetobacter glutaminase-asparaginase did not appear to be altered by amino acid supplementation. These data indicate that amino acid supplementation can improve nutritional status in patients treated with succinylated Acinetobacter glutaminase-asparaginase.