Polish experience in nutrition support in children.

Organization of enteral nutrition programme in Poland has developed rapidly in the last years, however, the underdiagnosis and late diagnosis of malnutrition are still the major challenges. For those children who are unable to tolerate enteral diet, intravenous support is required. Main achievements in our parenteral nutrition programme (PN) consist in decreasing septic complication rate and introducting fish oil based emulsions for prevention of PN-related liver disease. The challenge to combat in the future comprises development of a network of nutritional centres covering the whole country, which will take care of patients requiring nutrition support. The Children's Memorial Health Institute in Warsaw will remain the reference centre for the management of the most complicated cases. The organization of postgraduate courses should lead to continuous reduction of nutrition related complications rate. The important problem remains the relatively low number of patients weaned off PN. The widespread introduction of intestine rehabilitation programme is essential to improve this issue.

Long-term follow-up of patients on home parenteral nutrition in Europe: implications for intestinal transplantation.

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) (p<0.001). The primary cause of death on HPN was underlying disease-related in patients with HPN duration ≤2 years, and HPN-related in those on HPN duration >2 years (p=0.006). In candidates, the death HRs were increased in those with desmoids (7.1; 95% CI 2.5 to 20.5; p=0.003) or liver failure (3.4; 95% CI 1.6 to 7.3; p=0.002) 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 (p=0.041). 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 (p=0.767). 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.

Survival of patients identified as candidates for intestinal transplantation: a 3-year prospective follow-up.

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.

Candidates for intestinal transplantation: a multicenter survey in Europe.

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.

Hydrolyzed versus nonhydrolyzed protein diet in short bowel syndrome in children.

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.