Presentation of a nationwide multicenter registry of intestinal failure and intestinal transplantation.

BACKGROUND & AIMS: Exact data on Dutch patients with chronic intestinal failure (CIF) and after intestinal transplantation (ITx) have been lacking. To improve standard care of these patients, a nationwide collaboration has been established. Objectives of this study were obtaining an up-to-date prevalence of CIF and characterizing these patients using the specially developed multicenter web-based Dutch Registry of Intestinal Failure and Intestinal Transplantation (DRIFT). METHODS: Cross-sectional study. CIF was defined as type 3 intestinal failure in which >75% of nutritional requirements were given as home parenteral nutrition (HPN) for ≥ 4 weeks in children and >50% for ≥3 months in adults. All patients with CIF receiving HPN care by the three Dutch specialized centers on January 1, 2013 and all ITx patients were registered in DRIFT (https://drift.darmfalen.nl). RESULTS: In total, 195 patients with CIF (158 adults, 37 children) were identified, of whom 184 were registered in DRIFT. The Dutch point prevalence of CIF was 11.62 per million (12.24 for adults, 9.56 for children) on January 1, 2013. Fifty-seven patients (31%) had one or more indications for ITx, while 12 patients actually underwent ITx since its Dutch introduction. Four patients required transplantectomy of their intestinal graft and 3 intestinal transplant patients died. CONCLUSION: The multicenter registry DRIFT revealed an up-to-date prevalence of CIF and provided nationwide insight into the patients with CIF during HPN and after ITx in the Netherlands. DRIFT will facilitate the multicenter monitoring of individual patients, thereby supporting multidisciplinary care and decision-making.

Prophylactic anticoagulation decreases catheter-related thrombosis and occlusion in children with home parenteral nutrition.

BACKGROUND: Home parenteral nutrition (PN) has improved the survival of children with intestinal failure. Important complications include catheter-related thrombosis, occlusion, and infection. This study evaluated the efficacy and safety of prophylactic anticoagulation in the prevention of these complications. METHODS: Medical records were retrospectively reviewed of all children (0-18 years) with PN between January 1994 and March 2007 in 1 tertiary center. After introduction of prophylactic low molecular weight heparin or vitamin K antagonists in March 2007, all patients were prospectively followed until March 2010. RESULTS: In sum, 14 patients did not receive prophylaxis; 13 switched from no prophylaxis to prophylaxis in March 2007; and 5 directly received prophylaxis. Median age of PN onset was 4 months (range, 0.1-202) in the nonprophylaxis group (n = 27) and 25 (range, 2-167) in the prophylaxis group (n = 18); 16 children received low molecular weight heparin and 2, vitamin K antagonists. Catheter-related thrombosis developed in 9 patients with no prophylaxis (33%) and 1 with prophylaxis (6%) (P = .034). Cumulative 5-year thrombosis-free survival was 48% and 93% in the nonprophylaxis and prophylaxis groups, respectively (P = .047). Per 1,000 PN days, the nonprophylaxis and prophylaxis groups had 2.6 and 0.1 occlusions (P = .04) and 4.6 and 2.1 infections (P = .06), respectively. Cumulative infection-free survival after 3 years was 19% and 46% in the nonprophylaxis and prophylaxis groups, respectively (P = .03). Bleeding complications did not occur. CONCLUSION: Thromboprophylaxis significantly decreased catheter-related thrombosis and occlusion in children with PN without complications.

The 48-hour tetrahydrobiopterin loading test in patients with phenylketonuria: evaluation of protocol and influence of baseline phenylalanine concentration.

BACKGROUND: The 24- and 48-hour tetrahydrobiopterin (BH4) loading test (BLT) performed at a minimum baseline phenylalanine concentration of 400 µmol/l is commonly used to test phenylketonuria patients for BH4 responsiveness. This study aimed to analyze differences between the 24- and 48-hour BLT and the necessity of the 400 µmol/l minimum baseline phenylalanine concentration. METHODS: Data on 186 phenylketonuria patients were collected. Patients were supplemented with phenylalanine if phenylalanine was <400 µmol/l. BH4 20mg/kg was administered at T = 0 and T = 24. Blood samples were taken at T=0, 8, 16, 24 and 48 h. Responsiveness was defined as ≥ 30% reduction in phenylalanine concentration at ≥ 1 time point. RESULTS: Eighty-six (46.2%) patients were responsive. Among responders 84% showed a ≥ 30% response at T = 48. Fifty-three percent had their maximal decrease at T = 48. Fourteen patients had ≥ 30% phenylalanine decrease not before T = 48. A ≥ 30% decrease was also seen in patients with phenylalanine concentrations <400 µmol/l. CONCLUSION: In the 48-hour BLT, T = 48 seems more informative than T = 24. Sampling at T = 32, and T = 40 may have additional value. BH4 responsiveness can also be predicted with baseline blood phenylalanine <400 µmol/l, when the BLT is positive. Therefore, if these results are confirmed by data on long-term BH4 responsiveness, we advise to first perform a BLT without phenylalanine loading and re-test at higher phenylalanine concentrations when no response is seen. Most likely, the 48-hour BLT is a good indicator for BH4 responsiveness, but comparison with long term responsiveness is necessary.

Towards implementation of optimum nutrition and better clinical nutrition support.

Clinical Nutrition Support--defined as nutrition for hospitalized patients suffering from metabolic stress--plays a limited role in the therapeutic routine of the physician. This is not surprising as most research in the field of clinical nutrition is disappointing with regard to the objective outcomes: morbidity and mortality. These reflections advocate a more 'pharmaceutical approach' to nutrition in order to perform more proper studies on the potential effectiveness of this treatment modality. To provide all patients in the Academic Medical Centre (AMC) in Amsterdam, The Netherlands, with optimum clinical nutrition support, a Nutrition Support Team (NST) was established in 1996. This NST is coaching the dieticians and physicians in the AMC regarding clinical nutrition support. In practice this coaching consists of providing clear guidelines on what is supposed to be optimum nutrition, a basic course in parenteral nutrition and further continuous education. The concept of optimum nutrition is spread by the NST through various ways of education, both nationally and internationally. For adults, optimum nutrition is defined as the amount of protein, that stimulates whole body protein synthesis maximally (1.7 g/kg actual body weight) and covers anabolic energy need (35 kcal/kg actual body weight). The dietician is considered to be the expert in the field of optimum nutrition by oral, enteral or parenteral route. The Dietetic Department has increased its influence in the care of the patient by placing nutritional status and care on the chart of the patient's treatment. To provide optimal Nutrition Support for children and severe ill patients (Intensive care department) specialized teams were started which were co-ordinated by the central NST. The central NST has a co-ordinating and educating role, while the Specialized Nutrition Support Teams (Specialized NST) construct guidelines, undertake research and provide continuous optimum nutrition care.

Total energy expenditure in human immunodeficiency virus-infected men and healthy controls.

Total daily energy expenditure (TEE) has been reported to be slightly decreased in weight-stable acquired immune deficiency syndrome (AIDS) patients. This conclusion is based on a comparison of TEE measurements to the data reported by others. We measured TEE in nine weight-stable human immunodeficiency virus (HIV)-infected homosexual men (Centers for Disease Control [CDC]-II to -IV) without active opportunistic disease and nine age-, sex-, and height-matched healthy controls using the doubly labeled water technique for 2 weeks, and resting energy expenditure (REE) using the ventilated-hood technique. TEE in HIV-Infected patients was not significantly different from that in healthy controls (221 +/- 12.5 and 210 +/- 9 kJ.kg lean body mass [LBM]-1.d-1, respectively, NS). REE was approximately 10% higher in HIV patients than in healthy controls (134 +/- 4 and 125 +/- 4 kJ.kg LBM-1.d-1, respectively, P = .02). The energy spent in relation to physical activity was not different between HIV-Infected patients and the controls (66 +/- 10 and 64 +/- 5 kJ.kg LBM-1.d-1, respectively, NS). In conclusion, REE is increased by about 10% in weight-stable HIV-infected men without active opportunistic disease. TEE and the energy spent during physical activity are not different in this group of patients versus healthy controls. This is in contrast to the previously reported decrease of TEE in weight-losing AIDS patients. Therefore, the energy requirements of stable HIV-infected patients are not decreased compared with those of healthy subjects.