Iodine supplementation in children receiving long-term parenteral nutrition.

In 18 children receiving long-term total parenteral nutrition (TPN) without iodide supplements, thyroid function test results were normal but serum iodide levels were greater than in control subjects (p less than 0.01). Iodine contamination of TPN solutions and fat emulsions accounted for only half of the recommended parenteral intake. Skin absorption of topical iodinated disinfectant may explain the adequate, if not excessive, iodine intake. We conclude that iodine is an unnecessary supplement in TPN solutions.     

Serum trace elements in children receiving long-term parenteral nutrition

The trace elements iron (Fe), copper (Cu), zinc (Zn), selenium (Se), chromium (Cr), manganese (Mn), nickel (Ni), rubidium (Rb), and bromine (Br) were determined in serum from 18 children, ages 4 to 65 months, who received long-term total parenteral nutrition (TPN). Nine patients (group 1) received TPN as their only source of nutrition; the other nine (group 2) ingested 30% to 70% of their total calorie requirement and received the remainder intravenously. The reference group consisted of 19 healthy children of similar age and sex distribution. Groups 1 and 2 had received parenteral nutrition for 28.4 +/- 13.7 (SD) and 23.1 +/- 17.1 (SD) months, respectively. All patients in group 1 and four in group 2 had short bowel syndrome; four in group 2 had pseudo-obstruction syndrome. Patients in group 1 had normal serum levels of Fe, Cu, and Mn, but increased levels of Zn; they had decreased serum concentrations of Se, Cr, Ni, Rb, and Br. Patients in group 2 had decreased serum levels of Mn, Cr, Ni, Rb, and Br. Those trace elements not supplemented in TPN solutions (Se, Cr, Mn, Ni, Rb, and Br) were lower in the children receiving TPN than in the control children, indicating inadequate intake of these trace elements from food and TPN solutions. However, no obvious clinical signs or symptoms of trace element deficiencies were observed.     

Plasma amino acid concentrations in children receiving long-term parenteral nutrition

Plasma amino acid concentrations were measured in 15 children aged 4-65 months who received home parenteral nutrition (PN) and in 10 normal controls. In seven patients (Group I), PN was the only source of nutrition. Eight patients (Group II) ingested 30%-70% of their total energy requirements and received the remainder intravenously. Group I and Group II patients received PN for 25.6 +/- 14.4 (SD) and 25.7 +/- 19.1 months, respectively. In comparison to controls, Group I had significantly decreased plasma concentrations of total essential amino acids, the three branched chain amino acids, cystine, tyrosine, and the three major urea cycle amino acids. Group II children displayed decreased plasma concentrations of two branched chain amino acids, leucine and valine, as well as cystine and tyrosine. Taurine was decreased in both groups. The presence of liver disease did not have a major effect on amino acid levels. These data indicate that children receiving long-term parenteral nutrition will develop abnormalities in their plasma amino acid concentrations even though they appear to receive adequate amounts of amino acids in their intravenous solutions. Further studies will be necessary to assess whether modifying the intravenous intake of amino acids will normalize plasma amino acid concentrations in these children.     

Low blood and plasma carnitine levels in children receiving long-term parenteral nutrition

Total and free carnitine and acylcarnitine concentrations were analyzed in whole blood and plasma in 12 children with a mean age of 68.4 +/- 42.9 months who had received carnitine-free total parenteral nutrition (TPN) for an average of 4 years. The purpose of the study was to see if the children had become carnitine deficient and, if so, whether this correlated with poor lipid clearance. Compared to controls, the TPN-dependent children had significantly decreased concentrations of total and free carnitine in blood (26.6 +/- 9.4 (SD) mumols/L vs. 43.3 +/- 9.1 mumols/L, p less than 0.001, and 17.1 +/- 7.7 mumols/L vs. 35.2 +/- 8.1 mumols/L, p less than 0.001, respectively). Similar results were found in plasma (total carnitine of 19.0 +/- 8.0 mumols/L vs. 41.9 +/- 5.2 mumols/L, p less than 0.001, and free carnitine of 15.7 +/- 7.3 mumols/L vs. 36.1 +/- 5.2 mumols/L, p less than 0.001, respectively). The acylcarnitine concentration in plasma was decreased in the TPN children (3.3 +/- 1.5 mumols/L vs. 5.8 +/- 3.0 mumols/L, p less than 0.01) compared to controls. Despite the low carnitine concentrations, serum triglyceride levels and serum free fatty acid levels were within the normal range. There was no correlation between carnitine concentrations in plasma and serum triglyceride and free fatty acid levels. Our data show that children receiving carnitine-free TPN for many years developed markedly decreased concentrations of carnitine in blood and plasma. However, no adverse effects of the low carnitine levels were found on triglyceride and free fatty acid metabolism under stable conditions.     

Nutritional status in children receiving home parenteral nutrition

Nutrition status was evaluated in 19 children aged 4 to 65 months who received total parenteral nutrition as their only source of nutrition (nine patients, group 1) or who ingested 30% to 70% of their total energy requirements orally and received the remainder intravenously (10 patients, group 2). All patients had received parenteral nutrition for 31.8 +/- 9.8 SD and 33.1 +/- 17.1 months, respectively. All patients in group 1 and four in group 2 had short bowel syndrome; five in group 2 had pseudo-obstruction syndrome. The children in group 1 had normal height, weight, midarm circumference (MAC), midarm muscle circumference (MAMC), and triceps skinfold thickness (TSF). In group 2 patients, height, MAC, and TSF, were significantly below normal, and their weight, MAMC, and TSF, were lower than in group 1. Hemoglobin, total lymphocyte count, and serum albumin concentration were below normal in group 1; serum total protein concentration was low in both groups. Serum bilirubin concentration was higher in group 1; serum glutamic pyruvic transaminase activity was elevated in both groups. These data indicate that children receiving long-term total parenteral nutrition can obtain and maintain normal height, weight, and other anthropometric measurements of nutritional status. Group 1 patients had better nutritional status than those in group 2, perhaps because of differences in the underlying medical disorders between the two groups, or possibly because of a propensity to underestimate the amount of parenteral nutrition needed by patients who ingest some nutrients.     

Whole-blood manganese levels and brain manganese accumulation in children receiving long-term home parenteral nutrition

Recent reports attribute neurological and cerebral disorders to the accumulation of manganese (Mn) in the brain in patients receiving home parenteral nutrition (HPN). It is desirable to control the amount of Mn delivered to these patients, but a suitable method for monitoring an individual's Mn status and assessing Mn accumulation remains debatable. The aim of this study was to evaluate whether whole-blood manganese levels (WB-Mn) correlate with the accumulation of Mn in the brains of children who receive long-term HPN, using magnetic resonance imaging (MRI) of the brain. Six patients who had received HPN (duration of HPN, 18-137 months) were included in this study. The daily parenteral doses of Mn were calculated while on HPN. WB-Mn was measured and T1-weighted MRI of the brain was obtained for each patient with a 1.5-T MR imager. Twelve months after the withdrawal of Mn from HPN, measurements of WB-Mn and brain MRI were repeated in all patients except for one who was lost after initial examination. The same examinations were performed on an additional patient who had been successfully weaned off a 179 month course of HPN 20 months prior to the initial examination. The parenteral dose of Mn while receiving HPN ranged from 15.7 to 91.5 micro g/kg/day. Initially, MRI showed hyperintensity in the globus pallidus in all patients and in the anterior pituitary in one patient. WB-Mn was elevated in four patients, but was in the normal range in the remaining three. Following subsequent measurements 12 months later, WB-Mn was normal in all patients and MRI hyperintensity remained in the globus pallidus in one patient. One patient was lost after the initial examinations. WB-Mn does not necessarily correlate with the accumulation of Mn in the brain. Periodic MRI should be performed in patients receiving long-term NPN to monitor for excessive Mn accumulation in the brain.     

Lipid tolerance in children receiving long-term parenteral nutrition: a biochemical and immunologic study

The effect of intravenously administered lipids (intralipid) on immunologic function, complement, and coagulation was prospectively studied over 1 year in 15 children. The mean age of the children was 52.4 +/- 37.9 months; they had received total parenteral nutrition for an average of 3 years. Immunoglobulins (IgA, IgM, IgG), coagulation studies (platelets, prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrinogen degradation products, factor V) and components of complement (C3, C4, and CH100) were analyzed. Activation of monocytes by opsonized zymosan was measured by chemiluminescence and compared with that of normal control subjects. The clinically stable children had normal monocyte activation and normal complement levels. The PT and PTT values were significantly increased but improved with increased intralipid dose; other coagulation factors were normal. Acutely sick children, however, had decreased fat tolerance with significantly increased serum triglyceride levels and PT and PTT values; their monocyte activation and complement factors remained normal. These data indicate that the dose of intralipid should be lowered during acute illnesses; we suggest close monitoring of PT and PTT values and of serum triglyceride and cholesterol levels to avoid the fat overload syndrome.     

Carnitine status of children receiving long-term total parenteral nutrition: a longitudinal prospective study.

Nine children receiving carnitine-free total parenteral nutrition for 7.2 +/- 2.6 years since birth were prospectively studied for 3 years. Plasma values of total and free carnitine were 50% lower than those of age-matched healthy control subjects (p less than 0.02) but did not decrease further during the 3-year period. No significant abnormalities in free fatty acids, triglycerides, or cholesterol were found. The mean levels of alanine and aspartate aminotransferases and of alkaline phosphatase were slightly increased (p less than 0.02) at the initiation of the study but remained in the same range 3 years later. The low plasma carnitine values appeared to be without clinical consequence after 10 years of carnitine-free total parenteral nutrition.     

长期肠外营养新生儿血浆肉碱水平分析

目的 了解长期全肠外营养的足月新生儿血浆肉碱浓度变化和相关的代谢改变.方法 需全肠外营养支持的新生儿12例(男4例,女8例),采用同一肠外营养方案,于肠外营养支持第1天和最后1天测定血浆肉碱、肝功能和血脂水平.结果 最后1天与第1天比较血浆肉碱的浓度明显下降(29.3μmol/L对32.2 μmol/L,P=0.021);肝功能中AIJT、AKP显著上升(ALT:22.8 IU/L对12.3 IU/L,P:0.020:AKP:191.5 μmol/L对125.5μmol/L,P=0.043);血甘油三酯明显上升(1.17 mmol/L对0.65mmol/L,P=0.035).结论 长期接受不含肉碱的全肠外营养将影响血浆肉碱的浓度,导致血脂和肝功能变化.     

Growth of children under long-term total parenteral nutrition

The modalities of growth were determined with anthropometric measurements in 7 children under total parenteral nutrition for more than 2 years. Their speed of height growth is normal and correlated with their increase in lean body mass. Their weight growth is normal, however with a relative excess in fat mass explained by its high energy cost; on the contrary, the cost of maintenance activity is reduced. In order to limit the energy cost and, overall the risk of an excessive load of lipid deposits, the authors emphasize the importance of regularly determining the lean body mass and the composition of weight gain to manage properly the intake in prolonged total parenteral feeding.     

Aluminum contamination of parenteral nutrition and aluminum loading in children on long-term parenteral nutrition

BACKGROUND: Children who are receiving parenteral nutrition are at risk of aluminum overload, which may contribute to such side effects as osteopenic bone disease. The aim of the present study is to determine the aluminum contamination of parenteral nutrition solutions and their components, and to assess the aluminum status of children on long-term parenteral nutrition. METHODS: Aluminum concentrations were determined by graphite furnace absorption spectroscopy in components and in final parenteral nutrition solutions. The urinary aluminum excretion and plasma aluminum concentration were determined in 10 children on long-term parenteral nutrition. RESULTS: The mean aluminum concentration in the administered parenteral nutrition solutions was 1.6 +/- 0.9 micromol x l(-1)(mean +/- standard deviation (SD)). The resulting mean aluminum daily intake of the 10 patients was 0.08 +/- 0.03 micromol x kg(-1) x day(-1). CONCLUSIONS: Compared to two previous studies performed in 1990 and in 1995 in our hospital, the aluminum contamination of parenteral nutrition solutions and the daily aluminum intake of the children seemed to decrease. However, the plasma aluminum concentration and daily urinary aluminum excretion of the children still remain above normal standards. The children had no clinical symptoms of bone disease but aluminum accumulation in tissue can not be excluded. To prevent this iatrogenic toxicity, the aluminum contamination of parenteral nutrition should be assessed regularly.     

Increased risk of gallstones in children receiving total parenteral nutrition

Twenty-one children receiving long-term total parenteral nutrition were prospectively evaluated for the presence of gallstones. Using ultrasonography, nine children (43%) were found to have cholelithiasis, and five have since undergone cholecystectomy. Only children with ileal disorders or previous resection developed stones. In the select group of patients with ileal disorders or previous resection, the prevalence of stones was 64%, nearly twice that which has been observed in similarly defined adults not receiving total parenteral nutrition. Data from this study suggest that the prolonged administration of parenteral nutrition significantly enhances the risk of gallstone formation already imposed by a previous ileal resection or disorder. Periodic ultrasonograms provide a safe and accurate means of monitoring high-risk patients during and after prolonged total parenteral nutrition therapy.     

Nutritional assessment of children with short-bowel syndrome receiving home parenteral nutrition

Serial nutritional assessments using arm anthropometry, computed tomography of the thigh, and serum biochemical indexes during an eight-month period were performed on nine children with short-bowel syndrome receiving home parenteral nutrition. The mean patient age at the beginning of the study was 3.0 years. In anthropometric measurements, the mean body weight of our test population did not deviate from that of the normal population. Most patients were below the normal median for height. The mean midarm muscle area was 114% of the normal median, and the mean midarm fat area was 98% of the normal median. The mean weight and height velocities were 148% and 122% of the standard, respectively. Retinol-binding protein values, albumin levels, and total lymphocyte counts of the patients were low, while levels of aspartate aminotransferase and alanine aminotransferase were slightly elevated. Midarm muscle and fat compartment sizes were highly correlated with thigh muscle and fat compartment sizes, as demonstrated by computed tomography. Our results demonstrate that children with short-bowel syndrome receiving home parenteral nutrition can maintain normal growth characteristics and extremity compartment sizes.     

Enhanced lipid utilization in infants receiving oral L-carnitine during long-term parenteral nutrition

Fourteen infants requiring long-term total parenteral nutrition but able to tolerate small quantities of enteral feedings were randomized into carnitine treatment and placebo control groups. All infants had received nutritional support devoid of carnitine. Plasma carnitine levels and observed plasma lipid indices were not different before supplementation. Under standardized, steady-state conditions, 0.5 g/kg fat emulsion (intralipid) was administered intravenously over 2 hours both before and after infants received 7 days of continuous nasogastric or gastric tube L-carnitine (50 mumol/kg/day) or placebo. Plasma triglyceride, free fatty acid, acetoacetate, beta-hydroxybutyrate, and carnitine concentrations were observed at 0 (start of lipid infusion), 2, and 4 hours for pre- and post-treatment periods, and in addition at 6 and 8 hours after carnitine supplementation. Infants receiving carnitine had significantly greater beta-hydroxybutyrate plasma concentrations (P less than 0.05) and carnitine (P less than 0.001) at 0, 2, 4, 6, and 8 hours, and greater plasma acetoacetate concentrations (P less than 0.05) at 2, 4, 6, and 8 hours, compared with controls. Twenty-four-hour urinary carnitine excretion was very low for both groups before supplementation; after supplementation, excretion was higher (P less than 0.05) in the carnitine group. No significant differences were found between groups for plasma triglyceride or free fatty acid concentrations at any observation period. This study demonstrated enhanced fatty acid oxidation, as evidenced by increased ketogenesis, with L-carnitine supplementation in infants receiving long-term total parenteral nutrition.     

A home program of long-term total parenteral nutrition in children.

Three children--ages 4 months, 5 months, and 14 years--have been on a program of total parenteral nutrition at home for ten, 23 and 44 months respectively, as of January, 1978. Using a specially designed silicone rubber catheter, placed in the right atrium, total nutritional needs of these children were delivered nightly by family members; the children carried out normal activity during the day with the catheter line maintained by a heparin lock. Normal skeletal development and weight gain have been achieved while allowing these children normal social and psychlogic development outside the hospital. During the course of the therapy the patients had multiple metabolic abnormalities which were successfully treated by replacement therapy. The duration of catheter patency ranged from three to 22 months. Catheter sepsis or mechanical failure occasionally required catheter removal and replacement.