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.

How strict is galactose restriction in adults with galactosaemia? International practice.

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides ± fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.

Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach.

BACKGROUND: There appears little consensus concerning protein requirements in phenylketonuria (PKU). METHODS: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. RESULTS: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). CONCLUSIONS: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population.

[Doctors focus on the needs of the elderly]. / Artsen stellen behoeften van ouderen centraal.

The Dutch National Care for the Elderly Programme started in 2008. Eight regional networks were formed, in which more than 75 projects were carried out to improve care and wellbeing for frail elderly persons. The programme will come to an end in 2017. The results and lessons learned are being spread via the knowledge platform 'BeterOud'. In this article we describe some lessons from the programme for clinicians. The programme shows the value and the difficulties of clinicians taking the needs and wishes of frail elderly persons as the guiding principle of their care. Most frail elderly persons cannot be treated through generic interventions but need multidisciplinary and personalised care and support. In addition, standard research methods and outcome measures often do not coincide with the perceptions of elderly persons. This might explain the limited results of many projects in terms of cost-effectiveness. Benefits from the perspective of both professionals and elderly persons should be established in advance.

The prognosis of incurable cachectic cancer patients on home parenteral nutrition: a multi-centre observational study with prospective follow-up of 414 patients.

BACKGROUND: The role of home parenteral nutrition (HPN) in incurable cachectic cancer patients unable to eat is extremely controversial. The aim of this study is to analyse which factors can influence the outcome. PATIENTS AND METHODS: We studied prospectively 414 incurable cachectic (sub)obstructed cancer patients receiving HPN and analysed the association between patient or clinical characteristics and surviving status. RESULTS: Median weight loss, versus pre-disease and last 6-month period, was 24% and 16%, respectively. Median body mass index was 19.5, median KPS was 60, median life expectancy was 3 months. Mean/median survival was 4.7/3.0 months; 50.0% and 22.9% of patients survived 3 and 6 months, respectively. At the multivariable analysis, the variables significantly associated with 3- and 6-month survival were Glasgow Prognostic Score (GPS) and KPS, and GPS, KPS and tumour spread, respectively. By the aggregation of the significant variables, it was possible to dissect several classes of patients with different survival probabilities. CONCLUSIONS: The outcome of cachectic incurable cancer patients on HPN is not homogeneous. It is possible to identify groups of patients with a ≥6-month survival (possibly longer than that allowed in starvation). The indications for HPN can be modulated on these clinical/biochemical indices.

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.

Decreased food intake is a risk factor for mortality in hospitalised patients: the NutritionDay survey 2006.

BACKGROUND & AIMS: Malnutrition is a known risk factor for the development of complications in hospitalised patients. We determined whether eating only fractions of the meals served is an independent risk factor for mortality. METHODS: The NutritionDay is a multinational one-day cross-sectional survey of nutritional factors and food intake in 16,290 adult hospitalised patients on January 19th 2006. The effect of food intake and nutritional factors on death in hospital within 30 days was assessed in a competing risk analysis. RESULTS: More than half of the patients did not eat their full meal provided by the hospital. Decreased food intake on NutritionDay or during the previous week was associated with an increased risk of dying, even after adjustment for various patient and disease related factors. Adjusted hazard ratio for dying when eating about a quarter of the meal on NutritionDay was 2.10 (1.53-2.89); when eating nothing 3.02 (2.11-4.32). More than half of the patients who ate less than a quarter of their meal did not receive artificial nutrition support. Only 25% patients eating nothing at lunch receive artificial nutrition support. CONCLUSION: Many hospitalised patients in European hospitals eat less food than provided as regular meal. This decreased food intake represents an independent risk factor for hospital mortality.

Effectiveness and cost-effectiveness of a minimal psychological intervention to reduce non-severe depression in chronically ill elderly patients: the design of a randomised controlled trial [ISRCTN92331982].

BACKGROUND: Depression is a prevalent disorder in chronically ill elderly persons. It may decrease quality of life, and increase functional disability, medical costs, and healthcare utilisation. Because patients may slip into a downward spiral, early recognition and treatment of depression is important. Depression can be treated with antidepressants or psychological interventions; the latter can also be applied by trained paraprofessionals. In this paper, we describe the design of the DELTA study (Depression in Elderly with Long-Term Afflictions). The first objective of the DELTA study is to evaluate the effectiveness and cost-effectiveness of a minimal psychological intervention (MPI) to reduce depression in chronically ill elderly patients. The second objective is to evaluate whether a potential effect of the MPI may differ between types of chronic illnesses. The tailor-made intervention is administered by nurses, who are trained in the principles of cognitive behavioural therapy and self-management. METHODS/DESIGN: DELTA is a two-armed randomised controlled trial, comparing MPI to usual care. A total number of 180 patients with diabetes mellitus type II (DM) and 180 patients with chronic obstructive pulmonary disease (COPD), who in addition suffer from non-severe depression, will be included in the study. In our study, non-severe depression is defined as having minor depression, mild major depression or moderate major depression. The primary outcome measure is depression using the Beck Depression Inventory. Secondary outcome measures include quality of life, daily functioning, self-efficacy, autonomy, and participation. In the economic evaluation, cost-effectiveness and cost-utility ratios will be calculated. Furthermore, a process evaluation will be carried out. Analyses will include both univariate and multivariate techniques and according to the intention to treat principle. The economic evaluation will be done from a societal perspective and data of the process evaluation will be analysed using descriptive techniques. DISCUSSION: A total number of 361 patients has been included in the study. All interventions have been administered and follow-up data will be complete in September 2006. Preliminary results from the process evaluation indicate that patients' satisfaction with the intervention is high. If this intervention proves to be effective, implementation of the DELTA intervention is considered and anticipated.

[Treatment of intestinal failure by total parenteral nutrition at home in children and adults]. / De behandeling van darmfalen met parenterale thuisvoeding bij kinderen en volwassenen.

Intestinal failure is characterised by inability of the intestine to absorb sufficient nutrients to maintain the integrity and function of the body. This can be caused by malabsorption due to too short an intestine or an abnormality of the mucosa, or by a severe motility disorder. In addition to dietary measures, the prescription of total parental nutrition (TPN) at home is sometimes necessary. This treatment is a burden on the patient and the risk of complications must be reduced to a minimum. The risks of long-term parenteral nutrition can be limited and the quality of the provision of services can be increased if the co-ordination is in the hands of a centre for home parenteral nutrition. In the Netherlands there are two centres for home-TPN: the St Radboud University Medical Centre in Nijmegen and the University Medical Centre (AMC) in Amsterdam. In both children and adults, the most common indications are the short bowel syndrome and motility disorders. However, the syndromes that cause this are clearly different in the different age groups. Parenteral nutrition can be given for long periods of time. A large variety of complications can occur, related especially to the equipment or the nutrients. When the nutrition is given via a central venous catheter, then sepsis is a serious and possibly life-threatening complication. In case of administration via an arteriovenous shunt, thrombosis of the shunt is the most frequent problem. If the treatment by means of home-TPN fails, then transplantation of the small intestine should be considered.

Go or no go for gel entrapped nitrifiers? A Belgian case study.

In the coming years, as stricter environmental requirements are imposed, many European Union wastewater treatment plants (WWTP) need to be expanded and/or upgraded. This requires considerable investments. Optimising the renovation recourses can lead to significant savings. The use of entrapped nitrifying bacteria for upgrading of WWTP towards nutrient removal may be benificial. Long term pilot tests were performed to evaluate a so-called pellet reactor. Differences in performance and microbiological composition of classical activated sludge and the pellet reactor were investigated. FISH analyses showed (i) absence of Nitrobacter cells and (ii) high abundance of Nitrospira in the pilot reactors. Two Belgian WWTP make use of fine bubble aeration and could--theoretically--easily be renovated towards nitrogen removal using encapsulated nitrifiers. Financial aspects are commented on.

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.

Membranebioreactor with external membranes.

The recent development of less expensive and more performant ultrafiltration membranes has generated a new concept in biological treatment known as membrane bioreactors (MBR). In the field of wastewater treatment, the MBR process is used as a modification of the conventional activated sludge (CAS) process, where the clarifier is replaced by a membrane module for the separation of the solid and the liquid phase. Membrane technology can, amongst others, be applied for the fast retrofit of relatively high loaded activated sludge systems to nutrient removal. In the last year Aquafin has built up extensive experience with submerged membrane systems. The research presented here was aimed at evaluating a particular external tubular membrane unit for MBR. More specifically it was evaluated whether higher stable flux levels could compensate the higher energy consumption, typical for external membrane systems. Both (1) treatment performance and (2) possible operational drawbacks were tested on a 5 m3/h activated sludge pilot-scale. The membrane bioreactor was tested in parallel with a conventional activated sludge system.

Inter-laboratory comparison of liquid chromatographic techniques and enzyme-linked immunosorbent assay for the determination of surfactants in wastewaters.

Seven laboratories participated in an inter-laboratory comparison exercise within the framework of the PRISTINE, SANDRINE and INEXsPORT European Union Projects. Solid-phase extraction (SPE) methodologies were used for the extraction of target analytes from wastewaters. The analytical strategies were based on liquid chromatography (LC) coupled to mass spectrometric (MS) or to fluorescent (FL) detection in all cases with the exception of one laboratory using a test-tube enzyme-linked immunosorbent assay kit. Samples were spiked with the surfactants nonylphenolpolyglycol ether, coconut diethanolamide, linear alkylbenzene sulfonate, nonylphenolpolyglycol ether sulfate, alkylpolyglycol ether and secondary alkane sulfonate. After enrichment on previously conditioned SPE cartridges, the SPE cartridges were distributed among the participating laboratories without the information about the amount of spiked surfactants. In addition, SPE cartridges loaded with a real-world environmental sample containing a tannery wastewater were also analyzed. The results of the programme showed that SPE followed by LC-MS techniques are reliable for the surfactants determination at submicrogram to microgram per liter levels in wastewaters. Inter-laboratory precision values were calculated as the reproducibility relative standard deviation (RSD(R)) which was determined from the reproducibility standard deviation (sR) and the average concentration at a particular concentration level. When data from all laboratories were pooled, the RSD(R) values ranged from 5.1 to 28.3% for the determination of target analytes. The most accurate result corresponded to that given for linear alkylbenzene sulfonates. Taking into account that different methodologies were used (including non-chromatographic techniques) and the complexity of the samples analyzed, it can be considered that acceptable reproducibility values were obtained in this inter-laboratory study.

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.