Taurine supplementation in infants receiving long-term total parenteral nutrition

Twenty-one children and 23 adults receiving long-term total parenteral nutrition (TPN) for 27 +/- 23 (SD) months were investigated to determine if they were taurine-deficient because the TPN solutions were taurine-free. The fasting plasma taurine level was reduced in the children to 26 + 13 mumol/liter vs the control 57 +/- 16 mumol/liter (P greater than 0.001). The plasma taurine level was significantly reduced in those adults who absorbed less than 25% of their nutritional needs from their diet. Electroretinograms were abnormal in each of eight children who were examined; isolated cone and rod implicit times were both significantly delayed. Electroretinograms were not abnormal in those adults with low plasma taurine levels. Taurine was added to the TPN solutions of four children, and the plasma taurine level became normal in each of them. Electroretinograms of three of these children became normal. One year after discontinuing intervenous taurine supplementation, the plasma taurine level became abnormal in two of three children. These observations indicate that children, and possibly adults, receiving long-term TPN have a nutritional requirement for taurine.     

Taurine supplementation in infants receiving long-term total parenteral nutrition.

Twenty-one children and 23 adults receiving long-term total parenteral nutrition (TPN) for 27 +/? 23 (SD) months were investigated to determine if they were taurine-deficient because the TPN solutions were taurine-free. The fasting plasma taurine level was reduced in the children to 26 + 13 mumol/liter vs the control 57 +/? 16 mumol/liter (P greater than 0.001). The plasma taurine level was significantly reduced in those adults who absorbed less than 25% of their nutritional needs from their diet. Electroretinograms were abnormal in each of eight children who were examined; isolated cone and rod implicit times were both significantly delayed. Electroretinograms were not abnormal in those adults with low plasma taurine levels. Taurine was added to the TPN solutions of four children, and the plasma taurine level became normal in each of them. Electroretinograms of three of these children became normal. One year after discontinuing intervenous taurine supplementation, the plasma taurine level became abnormal in two of three children. These observations indicate that children, and possibly adults, receiving long-term TPN have a nutritional requirement for taurine.     

Blood and urinary amino acids in children receiving total parenteral nutrition

Plasma concentrations and urinary outputs of amino acids were estimated in nineteen patients receiving intravenous hyperalimentation to evaluate the adequacy of dosage and composition of the infusates for the maintenance of normal blood concentrations of essential amino acids. The use of high concentrations of branched chain amino acids seems to be appropriate for valine and isoleucine but not for leucine. The high concentration of cysteine in the infusates used induces a very high urinary excretion of cysteine and cystine and are ineffective to bring the decreased plasma cystine levels back to normal.     

Zinc and copper balance studies in infants receiving total parenteral nutrition

To determine the adequacy of zinc and copper supplementation for infants receiving total parenteral nutrition (TPN), we performed 24-h balance studies in infants with diarrhea and infants who had recently undergone surgery. Measurements were made at base line, 1, and 2 wk. Mean serum Zn and Cu levels of the diarrhea group remained normal and were low in the postoperative group but normalized over the study period. Mean 24-h Zn and Cu balances were positive in infants with diarrhea and negative in postoperative infants. The high Zn and Cu content in the gastrointestinal fluid loss associated with surgery may have accounted in part for this finding. Normal serum levels of Zn and Cu did not guarantee positive balance. No significant changes were found in serum albumin, alkaline phosphatase, or ceruloplasmin. The current Zn and Cu recommendations may be appropriate only for hospitalized infants who have no excessive gastrointestinal fluid losses.     

Renal function and urinary excretion of electrolytes in patients receiving cyclic parenteral nutrition

BACKGROUND: Long-term parenteral nutrition (LTPN) has been shown to induce renal impairment and bone demineralization. However, the mechanism of both injuries has not been clarified. METHODS: This prospective study was performed in 16 patients with short bowel syndrome, aged 28 to 63 years, who had received LTPN for 31 +/- 7 months. Urinary excretion of electrolytes were measured before (diurnal, 12 hours) and during (nocturnal, 12 hours) parenteral nutrition. Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were measured in the morning after the nutritional bag supply. RESULTS: Mean GFR was 86 +/- 7 mL/min/1.73 m2 and ERPF was 412 +/- 31 mL/min/1.73 m2. Decreased GFR was present in 9 patients. There was no relation between renal function and age or the duration of LTPN. Urine volume and excretion of urea, creatinine, sodium, magnesium, and phosphate but not potassium increased significantly in nocturnal urine compared with diurnal urine. On the basis on 24-hour calciuria, 7 patients were normocalciuric (NCa) whereas 9 were hypercalciuric (HCa). Both had excessive nocturnal calciuria, but only the HCa group had diurnal hypercalciuria, the calcium supply being identical. Bone mineral density (BMD) was slightly, although not significantly, higher in NCa group, but in all patients BMD correlated significantly with calciuria. Serum parathyroid hormone and vitamin D were not different in the two groups. CONCLUSIONS: In patients receiving LTPN, renal function is frequently impaired, by a mechanism which remains unclear. In nocturnal cyclic mode of nutrition, urinary volume and electrolyte excretion occurred predominantly during the infusion, but some patients have diurnal hypercalciuria. In these patients a defect in renal calcium reabsorption or more likely the inability of bone to retain the infused calcium may be responsible for bone demineralization.     

Plasma amino acid concentrations in newborn infants during parenteral nutrition

Amino acid substance concentrations in plasma have been measured during total and partial parenteral nutrition with Vamin in 12 seriously ill newborn infants. When plasma amino acid concentrations were compared to the levels in reference infants, 11 of 21 were low, among the 11 were seven essential amino acids (Arg, Cys, Ile, Leu, Lys, Thr, Tyr), while concentrations above the reference median and range occurred for seven amino acids. For three (Asp, Glu, Phe) levels were exceptionally high. From these findings, from studies by others on amino acid levels during parenteral nutrition with Vamin, and from amino acid needs judged from venous-arterial differences in newborns, an amino acid preparation for parenteral administration to newborn infants is suggested.     

Fatty acid composition of adipocyte membrane phospholipids and stored triglycerides in infants receiving total parenteral nutrition

Fatty acid (FA) composition of membrane phospholipids (PL) and stored triglycerides (TG) from adipose tissue was studied in eight infants aged 1 to 4 months receiving total parenteral nutrition (TPN) since birth. During this period, essential fatty acid (EFA) intake consisted exclusively of soybean oil emulsion administered by intravenous route (Intralipid 20%) representing 301 +/- 88 mg/kg/24 hr of linoleic acid and 58 +/- 18 mg/kg/24 hr of alpha-linolenic acid, or 2.3 +/- 0.6% and 0.4 +/- 0.1%, respectively, of total energy intake. The results were compared with those of eight control infants of the same age receiving orally a normal milk diet with an intake of 660 +/- 260 mg/kg/24 hr of linoleic acid and 101 +/- 35 mg/kg/24 hr of alpha-linolenic acid, or 4.5 +/- 0.7% and 0.7 +/- 0.3%, respectively, of total energy intake. Although their EFA intake was significantly lower (p less than 0.01) and administered only parenterally, after 1 to 4 months the infants receiving TPN still had a membrane phospholipid FA pattern of adipose tissue which was not significantly different from that of normal children of the same age. In stored adipocyte TG, the percentage of linoleic acid was significantly lower (p less than 0.01) in infants receiving TPN. This is probably of nutritional importance as at this stage of life the child builds up its stores of EFA. The proportion of the other fatty acids in adipocyte TG was not significantly modified.     

Hypermanganesemia in patients receiving total parenteral nutrition

BACKGROUND: Manganese is one of the trace elements that is routinely administered to total parenteral nutrition (TPN) patients. The recommended daily IV dosage ranges from 100 to 800 MICROg. We have used 500 microg daily. Recent reports have suggested neurologic symptoms seen in some patients receiving home parenteral nutrition (HPN) may be due to hypermanganesemia. Therefore, HPN patients and some short-term inpatients receiving TPN were studied to ascertain the relationship between dose and blood levels. METHODS: Red blood cell manganese levels were obtained by atomic absorptiometry. RESULTS: The levels in 36 hospitalized, short-term patients obtained within 48 hours of initiating TPN were all normal. The 30 patients receiving TPN from 3 to 30 days had levels that ranged from 4.8 to 28 microg/L (normal, 11 to 23 microg/L). Two patients had abnormal levels, at days 14 and 18. Fifteen of the 21 patients receiving inpatient TPN or HPN for 36 to 5075 days had elevated Mn levels. Only one patient with hypermanganesemia, an inpatient, had abnormal biochemical liver tests (bilirubin and alkaline phosphatase). One of the patients with a high level had some vestibular symptoms attributed to aminoglycoside use and had increased signal density in the globus pallidus on T1-weighted images on magnetic resonance imaging (MRI). A second patient with Mn levels twice normal had no neurologic symptoms, but had similar MRI findings. A third had some basal ganglia symptoms, confirmed by a neurologic evaluation, seizures, and very high Mn levels. The MRI showed no signal enhancement, but motion artifacts limited the study technically. CONCLUSIONS: Hypermanganesemia is seen in HPN patients receiving 500 microg manganese daily and may have resulted in some neurologic damage in three patients. Hypermanganesemia is sometimes seen after a short course of TPN in inpatients, as early as 14 days. Patients should be monitored for hypermanganesemia if they receive Mn in their TPN for >30 days. A 500 microg/d dose of Mn is probably excessive, and 100 microg/d should probably never be exceeded. Mn should be eliminated from the solution if the Mn level is elevated and should not be readministered unless the level returns to normal or subnormal. Mn should not be supplemented if the patient has liver disease with an elevated bilirubin.     

Hypocupremia in patients receiving total parenteral nutrition

Although hypocupremia is a well-known consequence of long-term total parenteral nutrition (TPN), its incidence as well as the duration of TPN necessary to induce it are still unsettled. The purpose of this study is to review the changes in serum copper level in 25 patients receiving TPN for a period longer than 2 wk (mean duration 6 wk) at the Istituto Nazionale Tumori of Milan and to evaluate the possible relationship of cupremia with the basic disease. Main indications for TPN included enterocutaneous fistulas (11 patients), cancer cachexia (10 patients), radiation enteropathy (two patients), and severe postoperative stricture following esophagogastric resection (two patients). Mean value of serum copper at the beginning of the study was 143 micrograms/100 ml (normal value 65-165 micrograms/100 ml), and the regression analysis showed a mean fall of 5.64 micrograms/100 ml/wk. Hypocupremia occurred in four patients (three with intestinal fistulas and one with radiation obstructive enteritis) at 5th, 6th, 9th, and 6th wk of TPN, respectively. No patient with cancer cachexia developed hypocupremia. No patient with hypocupremia had clinical evidence of a copper deficiency syndrome. We conclude that 1) hypocupremia does not occur within the first month of TPN; 2) its incidence is about 16% in patients intravenously fed for period longer than 2 wk; 3) it is more frequent in patients with enterocutaneous fistulas, whereas it never occurs in patients with cancer cachexia, and 4) it is not necessarily associated to a clinicometabolic syndrome of copper deficiency. Finally, the "nutritional" meaning of serum copper should be questioned in cancer patients since it could represent a "tumor marker."     

Fecapentaene excretion: aspects of excretion in newborn infants, children, and adult normal subjects and in adults maintained on total parenteral nutrition

People in developed nations such as the United States and Canada have an increased risk of colon cancer. Fecal mutagens have been detected in the feces of individuals at high risk for colon cancer. We describe a rapid, sensitive, reliable, reproducible high-pressure liquid chromatography (HPLC) method for detecting fecapentaenes, the most active and chief mutagen found in human stool. We found fecapentaene in all the stool samples of adults on typical high-fat, low-fiber Western diets. These fecapentaene concentrations remained largely constant when subjects consumed constant diets. Fecapentaene concentrations were reduced for total-parenteral-nutrition (TPN) patients with severe intestinal malabsorption. This finding with TPN patients may reflect changes in important variables of gut microflora in fecapentaene production. Studies with newborns and children showed that fecapentaenes appeared very early in life but are not present in stool at birth.     

Concurrent administration of albumin with total parenteral nutrition in sick newborn infants.

The effects of concurrent administration of albumin with total parenteral nutrition were studied in 12 premature newborns (birth weight 1.26 +/- 0.1 kg [mean +/- SEM] and gestational age 30 +/- 0.8 weeks [mean +/- SEM]) compared with a control group of 12 premature newborns (birth weight 1.17 +/- 0.2 kg and gestational age 29 +/- 0.1 weeks) who received total parenteral nutrition. All newborns had a plasma albumin level below 3 g/dL and were in cardiorespiratory distress requiring assisted ventilation. Albumin supplementation of total parenteral nutrition resulted in a sustained increase in serum albumin concentration as well as increased mean arterial blood pressures in the study group. Slow albumin infusion had no observed effect on the severity of respiratory distress. Study group infants regained birth weight earlier than control group infants. These data suggest that the concurrent administration of albumin may be clinically beneficial in critically ill newborn infants.     

Protein deficiency in premature infants receiving parenteral nutrition

A classical finding of protein deficiency is hair depigmentation, or the flag sign. To determine the clinical conditions that predispose ill premature infants to the development of protein deficiency, we compared the clinical courses and nutritional histories of premature infants receiving parenteral nutrition who developed the flag sign (group F), with those of a matched control group (Group C). Occurrences of necrotizing enterocolitis (NEC) and consequent surgery were significantly higher in group F than in group C (p less than 0.002 and p less than 0.005, respectively). No differences were found in the mean (+/- SD) administration of amino acids (group F, 2.5 +/- 0.2 g X kg X day vs group C, 2.4 +/- 0.4) and total energy (83.4 +/- 13.4 kcal X kg X day vs 83.6 +/- 11.1, respectively). Mean serum albumin levels (2.6 +/- 0.4 g/dL vs 2.6 +/- 0.5, respectively) also were similar. Because infants in both groups received recommended amounts of protein, results suggest that infants who have NEC, and surgery as a consequence of NEC, require more protein than is presently recommended.     

Vitamin D requirement in infants receiving parenteral nutrition

The adequacy of low dose vitamin D (25 IU/dl) parenteral nutrition (PN) solution was studied in 18 infants. All infants had surgical indications for PN. The birth weights were 2810 +/- 135 g and gestational ages 37.4 +/- 0.5 wk (mean +/- SEM). Duration of study ranged from 5 to 175 days. Thirteen infants were studied for up to 6 weeks and five infants for 71 to 175 days. Results showed that studied infants maintained growth along normal percentiles for weight, length, and head circumference. Vitamin D status as indicated by serum 25 hydroxyvitamin D (25 OHD) rose from 15 +/- 1.9 ng/ml to 26 +/- 2.8 ng/ml, mean +/- SEM (p less than 0.001) after 9 days, and remained normal up to 6 months. Five infants with biochemical liver dysfunction also had normal serum 25 OHD concentrations, indicating the hepatic 25 hydroxylation process was not severely impaired. Serum total and ionized calcium, phosphorus, and vitamin D-binding protein concentrations were normal. Serum magnesium was mildly elevated in five infants (2.6 to 3 mg/dl) on one occasion and resolved spontaneously. Serum alkaline phosphatase (AP) concentrations rose above baseline values in 12 of 17 infants, but remained within normal range (less than 400 IU/liter at 30 degrees C). Another infant with markedly elevated AP values died from liver dysfunction. Radiographs of the forearms were normal except for marked demineralization in one infant in spite of normal 25 OHD concentrations. We conclude that 25 IU vitamin D/dl of nutrient infusate is adequate to maintain normal vitamin D status, as indicated by normal serum 25 OHD concentrations in infants receiving PN for as long as 6 months.     

Dispensable and indispensable amino acid requirements in depleted patients receiving total parenteral nutrition

Fifty depleted patients (15% ideal body weight loss) were treated at two different non protein kcal (35 and 50) levels with three different amino acid solutions, while receiving a standard daily nitrogen (N) intake of 0.19 gN/kg. Six groups were studied including one in which 50 kcal were administered with a mixed lipid and glucose system. Daily N balance and amino acid profile, were determined for 5 days in low kcal groups and 10 days in the high kcal groups. Positive N balance was obtained at only low kcal intake with two of the three solutions. Judging from pooled data, N balance was related to phenilalanine (Phe) and Methionine (Met) intake and a minimum intake of 91 mg/kg Phe and 70 mg/kg Met seemed necessary to obtain positive N balance. Increasing kcal reduced Phe and Met needs allowing positive N balance with lower intake. A lower then normal baseline amino acid level was observed for nearly all amino acid [except Isoleucine (Ile) Phe, Met, Lysine (Lys) and Ornitine (Orn)]. During treatment plasma concentration of administered amino acids increased in relation to the administered amount for Valine (Val), Ile, The, Met, Lys, Threonine (Thr), Arginine (Arg), Glycine (Gly), (p < 0.001) Alanine (Ala) (p < 0.025) and Proline (Pro) (P < 0.05). A significant negative correlation with administered kcal was found for Ile, Met, Lys and Arg. Amino acids not administered did not show an homogeneous pattern and, using multiple regression, the variables affecting single amino acid behaviour were identified.     

Relationship between the sulfur content of total parenteral nutrition and sulfoester excretion in low-birthweight infants

Inorganic sulfate is an end product of sulfur amino acid metabolism but it is also the cosubstrate for the biosynthesis of a wide array of complex sulfoesters. In vitro studies have shown that SO4 availability may be the primary determinant of sulfoconjugation rates for specific substrates but the relationship between S intake and sulfoester formation in vivo is not known. By substituting MgCl2 for MgSO4 in an amino acid infusate for parenteral nutrition, we were able to examine prospectively the effect of an altered SO4 load on S metabolism. In comparing 21 low-birthweight infants on the experimental MgC2 infusate with 14 subjects on the control MgSO4 infusate, we observed a 40% decrease in urinary excretion of free SO4 and a 31% decrease in excretion of total acid-labile sulfoesters. There was a significant correlation (r = 0.44; p less than 0.02) between total S intake and sulfoester excretion, suggesting that S intake influences sulfoconjugation in the low-birthweight infant requiring total parenteral nutrition.     

Folic acid and total parenteral nutrition

The stability of folic acid in a variety of solutions used for parenteral nutrition has been determined over a 2-wk period. Provided that the acidity of the solution remains above pH 5.0 the folate, in the concentrations usually used for parenteral nutrition, will remain stable in solution, and all of the folate added to the solution will be delivered to the patient. The applicability of this in vitro work to a group of patients requiring parenteral nutrition was assessed, in order to determine a suitable dose. A dose of 0.2 mg of folic acid daily was inadequate to meet the requirements of these patients. In contrast a dose of 1.2 mg daily for 7 days was sufficient to cause an increase in the serum folate concentration.     

Clinical zinc deficiency in total parenteral nutrition: zinc supplementation

The incidence of clinical zinc (Zn) deficiency was rare when solutions used for total parenteral nutrition (TPN) contained amino acids derived from hydrolyzed casein or fibrin, inasmuch as the Zn content of these solutions was high. Between 1978 and 1979 at The University of Iowa Hospitals, the incidence of clinical Zn deficiency increased significantly and was noted in eight patients (3%). During this time, the solution used for TPN contained crystalline amino acids and contained lower levels of Zn. The incidence of clinical Zn deficiency apparently decreased in 1980 and 1981, when the TPN solutions were supplemented with Zn intermittently. Only three patients (1%) developed clinical Zn deficiency. The clinical course of these three patients is reported. All three were in a poor nutritional state and had diseases of the gastrointestinal tract or of the pancreas which are known to be associated with decreased absorption and/or excessive loss of Zn from the body. The signs and symptoms of Zn deficiency developed at a time when the nutritional status of the patients was improving. Zinc serum levels were low (15-40 micrograms per deciliter); but none of the three patients had essential fatty acid deficiency. Treatment with intravenous ZnCl2 or oral ZnSO4 caused a rapid and dramatic improvement in the signs and symptoms. Skin lesions disappeared within 8 days after initiation of therapy. It is suggested that in similar patients Zn supplementation should be on a daily basis. Urine, stool, and serum Zn levels should be monitored closely, especially when the nutritional status of the patient is improving.     

Plasma manganese concentrations in infants and children receiving parenteral nutrition

Manganese is excreted primarily via the bile. The objective of this study was to determine if plasma Mn concentrations were elevated in infants and children fed intravenously who developed cholestatic liver disease. An additional aim was to determine the Mn content of intravenous solutions. The study included nine subjects with normal liver function (group A) and five subjects who developed cholestatic liver disease while fed intravenously (group B). Serum total bilirubin in group B had been elevated for 1 wk to 5 months prior to entry into this study. Mean plasma Mn for group A was 0.79 +/- 0.18 ng/ml, which was similar to that for adult controls. The mean for group B was 3.06 +/- 2.07 ng Mn/ml which was significantly higher (p less than 0.005). Plasma Mn was correlated positively with serum total bilirubin (r = 0.874, p less than 0.001). In four group B subjects studied longitudinally, plasma Mn declined. This decline was associated with a decline in serum total bilirubin in one subject and both a decline in serum total bilirubin and a reduction in, or a cessation of, supplemental intravenous Mn in two subjects. In the fourth subject, hepatic dysfunction remained severe, but plasma Mn declined to a near normal value after discontinuing supplemental intravenous Mn. The mean Mn contamination in the final intravenous infusate was 5.1 +/- 2.1 micrograms Mn/1. In conclusion, it is recommended that plasma Mn concentrations be monitored in patients fed intravenously who have evidence of cholestatic liver disease. If monitoring is not feasible, Mn supplements should be withheld.