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Background & Aims: The US Medicare indications for intestinal transplantation are based on failure of home parenteral nutrition. The American Society of Transplantation also includes patients at high risk of death from their primary disease or with high morbidity intestinal failure. A 3-year prospective study evaluated the appropriateness of these indications. Methods: Survival on home parenteral nutrition or after transplantation was analyzed in 153 (97 adult, 56 pediatric) candidates for transplantation and 320 (262 adult, 58 pediatric) noncandidates, enrolled through a European multicenter cross-sectional survey performed in 2004. Kaplan–Meier and chi-square test statistics were used. Results: The 3-year survival was 94% (95% CI, 92%–97%) in noncandidates and 87% (95% CI, 81%–93%) in candidates not receiving transplants (P = .007). Survival was 80% (95% CI, 70%–89%), 93% (95% CI, 86%–100%), and 100% in parenteral nutrition failure, high-risk primary disease, and high-morbidity intestinal failure, respectively (P = .034). Fifteen candidates underwent transplantation. Six died, including all 3 of those who were in hospital, and 25% of those who were at home at time of transplantation (P = .086). Survival in the 10 patients receiving a first isolated small bowel transplant was 89% (95% CI, 70%–100%), compared with 85% (95% CI, 74%–96%) in the candidates with parenteral nutrition failure not receiving transplants because of central venous catheter complications, or 70% (95% CI, 53%–88%) in those with parenteral nutrition–related liver failure (P = .364). Conclusions: The results confirm home parenteral nutrition as the primary therapeutic option for intestinal failure and support the appropriateness and potential life-saving role of timely intestinal transplantation for patients with parenteral nutrition failure.

Background: Home parenteral nutrition has become routine for management of intestinal failure in patients. In Poland the main obstacle to widespread use of home parenteral nutrition is the lack of interest of commercial companies in delivering feedings and ancillaries to patients. Methods: Twenty-five home parenteral nutrition patients aged from 4 months to more than 13 years were reviewed. The mother or both parents were trained in home parenteral nutrition techniques for 4 to 6 weeks and compounded the nutrients themselves at home. Results: The mean duration of home parenteral nutrition was 10,117 patient days. Hospital stays of patients receiving parenteral feedings were significantly shorter than the duration of administration of home parenteral nutrition (p < 0.001), Eleven children are continuing the home parenteral nutrition program. Eighty-three catheters were used in these patients. The rate of catheter occlusion decreased within the observation period, and in 1997 not one case of occlusion was observed. In 1997 only three catheters were removed during 7.8 patient years, and the overall incidence of catheter-related complications was 0.38 per patient year. The overall occurrence of septicemia was one case in 516 days and of catheter infection was one in 459 days. In 1997 a catheter was infected on average of once every 1419 days. There was significant improvement in the z score for weight during therapy. The average monthly cost of nutrients and ancillary items was approximately $1200 (4200 Polish zlotys [PLN]). These costs are 1.6 to 3 times lower than those recorded in other studies. Conclusion: Home parenteral nutrition in children with nutrients mixed by caregivers in the home setting is a safe and appropriate method of treatment that can be used in countries where home parenteral nutrition solutions are not manufactured or where commercial home parenteral nutrition is not economically feasible.

The aim of our study was to assess the metabolic consequences of short-term administration of growth hormone in children after gut resection and influence on polyamine production in red blood cells (RBC). Twelve children aged 4-60 months were studied. All children remained on parenteral nutrition and 11 also received oral feeding. Total non-protein energy intake was 429 +/- 86 kJ/kg body weight (BW)/day. Recombinant growth hormone (GH) was administered subcutaneously at a dose of 0.3 IU/kg BW/day for 10 days. Resting energy expenditure (REE; kJ/kg BW/day) was: 316.07 +/- 54.08 before and 346.04 +/- 54.40 during GH administration (P < 0.02), but daily weight gain before GH administration was significantly lower than during treatment. A significant increase of polyamine concentrations was observed in the RBC (spermidine: 30.1 +/- 15.1 and 43.8 +/- 24.9 nmol/ml packed RBC, P < 0.003; spermine: 15.6 +/- 5.1 and 19.6 +/- 10.6 nmol/ml packed RBC, P < 0.02) and in jejunal mucosa (spermidine: 172.10 +/- 142.35 nmol/g tissue and 193.92 +/- 108.15 nmol/g tissue). The authors concluded that increased polyamine concentrations in the RBC and jejunal mucosa reflect a cellular response to GH administration. The anabolic effect of GH results in higher weight gain, although increased REE may indicate increased energy requirements during GH treatment.

This study was designed to assess resting energy expenditure (REE) and nutritional status in children with hepatic and prehepatic portal hypertension in comparison with healthy controls. Twenty-five patients with portal hypertension (PHT) and a history of variceal bleeding were compared with 14 healthy volunteers selected after matching for age and sex. PHT patients were allocated to one of two groups: 11 children with liver cirrhosis and/or chronic hepatitis, aged 14.0 +/- 3.3 y (means +/- SD) or 14 children with extrahepatic portal vein obstruction, aged 12.3 +/- 2.8 y. The control group consisted of 14 healthy children, aged 14.0 +/- 1.8 y. REE (indirect calorimetry) assessed after an overnight fast was significantly higher in PHT patients than in controls when related to body mass (143.7 +/- 29.5 and 116.1 +/- 5.9 kJ/kg, respectively; p < 0.004), lean body mass (168.0 +/- 28.9 and 146.4 +/- 14.1 kJ/kg, respectively; p < 0.02), and body surface area (7480 +/- 736 and 6590 +/- 567 kJ/1.73 m2, respectively; p < 0.001). The ratios of measured REE to basal energy expenditure calculated from standard equations (Schofield equations) indicated higher REE in PHT patients (102.24 +/- 6.90% and 93.54 +/- 4.47%, respectively; p < 0.001). Fat was the predominant source of energy in both PHT patients and controls; the percentage of nonprotein energy derived from carbohydrate oxidation was equaled: 36.04 +/- 18.84% and 37.15 +/- 15.71%, respectively. Analysis of percentage of undernutrition in PHT patients and controls revealed significant differences (44% and 21%, respectively; p < 0.001). Children with PHT are susceptible to malnutrition and have elevated REE compared with healthy controls. Fat is the principal basal state oxidative substrate for patients with PHT and healthy children.

Background The indications for intestinal transplantation (ITx) are still debated. Knowing survival rates and causes of death on home parenteral nutrition (HPN) will improve decisions. Methods A prospective 5-year study compared 389 non-candidates (no indication, no contraindication) and 156 candidates (indication, no contraindication) for ITx. Indications were: HPN failure (liver failure; multiple episodes of catheter-related venous thrombosis or sepsis; severe dehydration), high-risk underlying disease (intra-abdominal desmoids; congenital mucosal disorders; ultra-short bowel), high morbidity intestinal failure. Causes of death were defined as: HPN-related, underlying disease, or other cause. Results The survival rate was 87% in non-candidates, 73% in candidates with HPN failure, 84% in those with high-risk underlying disease, 100% in those with high morbidity intestinal failure and 54%, in ITx recipients (one non-candidate and 21 candidates) (p2 years (p¼0.006). In candidates, the death HRs were increased in those with desmoids (7.1; 95% CI 2.5 to 20.5; p¼0.003) or liver failure (3.4; 95% CI 1.6 to 7.3; p¼0.002) compared to non-candidates. In deceased candidates, the indications for ITx were the causes of death in 92% of those with desmoids or liver failure, and in 38% of those with other indications (p¼0.041). In candidates with catheter-related complications or ultra-short bowel, the survival rate was 83% in those who remained on HPN and 78% after ITx (p¼0.767). Conclusions HPN is confirmed as the primary treatment for intestinal failure. Desmoids and HPN-related liver failure constitute indications for life-saving ITx. Catheter-related complications and ultra-short bowel might be indications for pre-emptive/rehabilitative ITx. In the early years after commencing HPN a life-saving ITx could be required for some patients at higher risk of death from their underlying disease.

OBJECTIVES: Epidemiology of candidacy for intestinal transplantation (ITx) and timing for referral for ITx are unknown. Patient candidacy and physician attitudes toward ITx were investigated among centers that participated in previous European surveys on home parenteral nutrition (HPN). METHODS: Patients on HPN for benign intestinal failure (IF) were evaluated by a structured questionnaire. Candidacy was assessed by USA Medicare and American Transplantation Society criteria, categorized as: (1) life-threatening HPN complications; (2) high risk of death because of the gastrointestinal disease; (3) IF with high morbidity or patient HPN refusal. Physicians judged candidacy as immediate or potential. RESULTS: Forty-one centers from nine countries enrolled 688 adults (> 18 yr) and 166 pediatric patients; 70% of patients were from five countries which collected 60-100% of their HPN patients. Candidacy was 15.7% in adults and 34.3% in pediatrics (HPN failure, 62.1% and 28.1%; gastrointestinal disease, 25.9% and 59.6%; high morbidity IF or HPN refusal, 12.0% and 12.3%, respectively). Immediate candidacy was required for 14.8% of adult and 15.8% of pediatric candidates (< 50% of candidates because of HPN-related liver failure). Among centers, the candidacy rate ranged 0-100% and was negatively associated with the number of patients enrolled in the survey (R = -0.463, p = 0.002). Among the major contributing countries, candidacy ranged 0.3-0.8/million inhabitants for adults and 0.9-2/million inhabitants < or = 18 yr for pediatric candidates. CONCLUSIONS: The rate of candidacy and the indications for ITx candidacy differed greatly among age groups and HPN centers; within countries candidacy was more homogeneous; physicians had a generally reserved attitude toward ITx.

Background: There is no consensus regarding the optimal enteral formula in patients with neonatal short bowel syndrome. The common practice in many centers is to give a semielemental diet. Methods: To test the hypothesis that hydrolyzed protein is not superior to standard formula in promoting growth and development of children with short bowel syndrome, 10 children aged 4.08 ± 2.45 months (mean ± SD) underwent a prospective, randomized, crossover, double-blind study lasting 60 days (with crossover on day 31). Two enteral formulas, which differed only with respect to the nitrogen form-hydrolyzed and nonhydrolyzed whey protein-were used. The endpoints of the study were nitrogen balance and intestinal permeability measured by the sugar absorption test (lactulose/mannitol excretion ratio). Results: Energy intake from enteral formula in patients fed hydrolyzed and nonhydrolyzed formula was the same and amounted to about 31% of total intake. The ratio of total energy intake (enteral and parenteral) to resting energy expenditure was 1.7 ± 0.5 and 1.5 ± 0.3 in patients fed hydrolyzed and non hydrolyzed formula respectively. Nitrogen balance was 0.28 ± 0.05 g/kg/d and 0.29 ± 0.05 g/kg/day, respectively. Lactulose/mannitol ratio before the study was 0.85 ± 0.85 and after hydrolyzed and nonhydrolyzed formula was 0.59% ± 0.51% and 0.69% ± 0.72%, respectively. Conclusion: Intestinal permeability, energy, and nitrogen balance in short bowel syndrome were not influenced in the short term by hydrolysis of the enteral nitrogen source. Infant formula-Intestinal permeability.

Contamination of parenteral nutrition solutions with aluminum may result in accumulation of this element in bones and, in premature infants, may inhibit bone calcium uptake and induce cholestasis. We measured the aluminum concentration of small-volume parenterals, amino acid solutions, lipid emulsions, and special solutions containing glucose, amino acids, electrolytes, and trace elements (standard I for children with a body weight of 3-5 kg, standard II for children with a body weight of 5-10 kg). The method used was graphite furnace atomic absorption spectrometry GTA-AAS (SpectrAA-400 Plus, Varian, PtY Ltd., Mulgrave, Australia). Quality control was run with the use of control serum (Seronorm, Nycomed, Oslo, Norway). The aluminum contents of parenterally administered solutions were: pediatric trace elements, 130 micrograms/L, and pediatric trace elements, 3000 micrograms/L; phosphorus salts: K-phosphates, 9800 micrograms/L, and Na/K phosphates, 13,000 micrograms/L; 10% calcium gluconate, 4400 micrograms/L; 6.5% amino acids, 30 micrograms/L; 10% amino acids, 120 micrograms/L; 12.5% amino acids, 121 micrograms/L; 20% lipid emulsion, 30 micrograms/L; 20% lipid emulsion, 180 micrograms/L; water-soluble vitamins, 12 micrograms/L; lipid soluble vitamins, 360 micrograms/L; standard I, 55 micrograms/L; standard II, 90 micrograms/L; The aluminum intake from parenteral nutrition was 6.6-10.8 micrograms.kg-1.d-1--a dose exceeding the safety limit of 2 micrograms.kg-1.d-1. The possible association of aluminum not only with metabolic bone disease, but also with encephalopathy, dictates caution when dealing with the pediatric population on long-term parenteral nutrition. In the absence of reliable label information, it seems proper to monitor the aluminum concentration in parenteral nutrition products and to report it in professional journals.