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Background & aims: Subjects with short bowel syndrome (SBS) have impaired quality of life (QoL). No disease-specific instrument has been available to measure treatment-induced changes in QoL over time. Therefore, the aim was to develop and validate an SBS-specific QoL scale. Methods: Classical test theory and Food and Drug Administration (FDA) guidance were applied for development and validation of the SBS-QoL™. Procedures included item generation and raw scale construction. Factor analysis, construct validity and internal consistency were assessed in a non-interventional observation, test re-test reliability and responsiveness in a randomised clinical study. Results: The SBS-QoL™ comprises 17 items including two subscales. Subjects assessed the scale as easy to handle and comprehensible. Good construct validity was shown by comparison with the Home Parenteral Nutrition-Quality Of Life questionnaire as an external scale, which yielded moderately high correlation (r≥0.7). High internal consistency was demonstrated (Cronbach's alpha: 0.94). Also the test re-test reliability was high (r≥0.95), indicating reliable reproducibility of results. The Responsiveness Index (1.84) indicated the ability of the scale to detect changes in QoL over time. Conclusions: The SBS-QoL™ is an easy to handle and comprehensible SBS-specific subject-reported QoL scale. It is valid, reliable and sensitive with excellent psychometric characteristics to measure treatment-induced changes in QoL over time in subjects with SBS. © 2012 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism.

Summary Background & aims Short bowel syndrome (SBS) is a rare and severe condition where home parenteral nutrition (HPN) dependence can be either permanent or transient. The timing of HPN discontinuation and the survival, according to SBS characteristics, need to be further reported to help plan pre-emptive intestinal transplantation and reconstructive surgery. Methods 268 Non-malignant SBS patients have been followed in our institution since 1980. HPN dependence and survival rate were studied with univariate and multivariate analysis. Results Median follow-up was 4.4 (0.3–24) years. Actuarial HPN dependence probabilities were 74%, 64% and 48% at 1, 2 and 5 years, respectively. In multivariate analysis, HPN dependence was significantly decreased with an early ( 20 μmol/l, a remaining colon >57% (4/7) and a remnant small bowel length >75 cm. Among the 124 patients who became HPN independent, 26.5% did so more than 2 years after SBS constitution. Conclusions This study indicates that long-term HPN is required in 47% of SBS patients started on this therapy. HPN independence is significantly associated with the remnant small bowel length, remaining colon and early plasma citrulline concentration. Noteworthy, HPN dependence could be reversed until 5 years after SBS constitution.

Introduction Management of chronic radiation enteritis is often controversial, particularly due to the risk of short bowel syndrome. Methods One hundred and seven chronic radiation enteritis patients with short bowel syndrome were studied retrospectively between 1980 and 2009. Survival and home parenteral nutrition dependence rates were evaluated with univariate and multivariate analysis. Results The survival probabilities were 93%, 67% and 44.5% at 1, 5 and 10 years, respectively. On multivariate analysis, survival was significantly decreased with residual neoplastic disease (HR = 0.21 [0.11–0.38], p 3 (HR = 0.38 [0.20–0.73], p = 0.004) and an age of chronic radiation enteritis diagnosis >60 years (HR = 0.45 [0.22–0.89], p = 0.02). The actuarial home parenteral nutrition dependence probabilities were 66%, 55% and 43% at 1, 2 and 3 years, respectively. On multivariate analysis, this dependence was significantly decreased when there was a residual small bowel length >100 cm (HR = 0.35 [0.18–0.68], p = 0.002), adaptive hyperphagia (HR = 0.39 [0.17–0.87], p = 0.02) and the absence of a definitive stoma (HR = 0.48 [0.27–0.84], p = 0.01). Conclusion The survival of patients with diffuse chronic radiation enteritis after extensive intestinal resection was good and was mainly influenced by underlying comorbidities. Almost two-thirds of patients were able to be weaned off home parenteral nutrition.

BACKGROUND Segmental reversal of the small bowel (SRSB) is proposed in patients with short-bowel syndrome (SBS) as a rehabilitative therapy, but its effects on absorption have not been studied. OBJECTIVE We aimed to determine intestinal macronutrient absorption and home parenteral nutrition (HPN) dependence in SBS patients with intestinal failure. DESIGN We included in a retrospective study all consecutive patients who had an SRSB between 1985 and 2010 and underwent a study of macronutrient absorption. Patients were matched to SBS controls with the same digestive characteristics. Energy and macronutrient absorption were measured. The dependence on HPN was expressed by the number of infusions per week and by the calories infused daily divided by the basal energy expenditure multiplied by 1.5. RESULTS Seventeen patients who had an SRSB were matched to 17 control patients. Intestinal absorption was higher in the SRSB group for total calories (69.5% compared with 58.0%), fat (48.4% compared with 33.2%), and protein (62.7% compared with 53.4%) (P < 0.05). Median oral autonomy was 100% ± 38.4% in the SRSB group, whereas it was 79% ± 39.6% in the control group (P < 0.05). The number of calories infused was lower in the SRSB group (500 ± 283 compared with 684 ± 541; P < 0.05), as was HPN dependence (33% ± 20% compared with 48% ± 38%; P < 0.05) at the time of the study. CONCLUSION SRSB allows a gain in macronutrient absorption, which is associated with a lower HPN dependence. To our view, SRSB should be integrated in intestinal rehabilitative adult programs.

Teduglutide was discontinued after being tested for ≥ 24 weeks in patients with parenteral nutrition (PN) -dependent short bowel syndrome in a clinical trial for efficacy to reduce PN volume. This study was describes change in body mass index (BMI) and PN volume over 12 months in patients who stopped drug after the clinical trial.Prescribed PN volume, weight, and complications were reported. Patients with stable (NEUT, n = 15) or decreased (DEC, n = 7) PN volume by 12 months after stopping drug (NEUT/DEC, n = 22) were compared to those who had increased PN volume (INC, n = 15). With drug response defined by ≥ 20% reduction from pre-drug PN volume to end of drug therapy, 12 INC and 13 NEUT/DEC patients were drug responders.Eleven of 20 eligible sites reported data for 39 of 53 eligible study participants, with follow-up data for 37. INC patients had shorter colon and less frequently had colon in continuity than NEUT/DEC. BMI was decreased at 3, 6, and 12 months relative to the first off-drug visit in INC patients (P = .001), but not in NEUT/DEC patients. Change in BMI off-drug was predicted by colon and small bowel length, baseline BMI, and on-drug change in PN volume (adjusted R2 = 0.708).Gastrointestinal anatomy, baseline BMI, and PN volume reduction on-drug predicted change in BMI off-drug. Whether this response would be maintained for a longer time or in the context of a challenging clinical situation has not been evaluated.

Background and aims Teduglutide, a GLP-2 analogue, may restore intestinal structural and functional integrity by promoting repair and growth of the mucosa and reducing gastric emptying and secretion, thereby increasing fluid and nutrient absorption in patients with short bowel syndrome (SBS). This 24-week placebo-controlled study evaluated the ability of teduglutide to reduce parenteral support in patients with SBS with intestinal failure. Methods In 83 patients randomised to receive subcutaneous teduglutide 0.10 mg/kg/day (n=32), 0.05 mg/kg/day (n=35) or placebo (n=16) once daily, parenteral fluids were reduced at 4-week intervals if intestinal fluid absorption (48 h urine volumes) increased ≥10%. Responders were subjects who demonstrated reductions of ≥20% in parenteral volumes from baseline at weeks 20 and 24. The primary efficacy end point, a graded response score (GRS), took into account higher levels and earlier onset of response, leading to longer duration of response. The intensity of the response was defined as a reduction from baseline in parenteral volume (from 20% to 100%), and the duration of the response was considered the response at weeks 16, 20 and 24. The results were tested according to a step-down procedure starting with the 0.10 mg/kg/day dose. Results Using the GRS criteria, teduglutide in a dose of 0.10 mg/kg/day did not have a statistically significant effect compared with placebo (8/32 vs 1/16, p=0.16), while teduglutide in a dose of 0.05 mg/kg/day had a significant effect (16/35, p=0.007). Since parenteral volume reductions were equal (353±475 and 354±334 ml/day), the trend towards higher baseline parenteral volume (1816±1008 vs 1374±639 ml/day, p=0.11) in the 0.10 mg/kg/day group compared with the 0.05 mg/kg/day group may have accounted for this discrepancy. Three teduglutide-treated patients were completely weaned off parenteral support. Serious adverse events were distributed similarly between active treatment groups and placebo. Villus height, plasma citrulline concentration and lean body mass were significantly increased with teduglutide compared with placebo. Conclusions Teduglutide was safe, well tolerated, intestinotrophic and suggested pro-absorptive effects facilitating reductions in parenteral support in patients with SBS with intestinal failure. ClinicalTrials.gov number NCT00172185.

Background & Aims Little is known about the magnitude of the risk of anal and rectal cancer in patients with anal and/or perineal Crohn's disease. We aimed to assess the risk of anal and rectal cancer in patients with Crohn's perianal disease followed up in the Cancers Et Surrisque Associe aux Maladies Inflammatoires Intestinales En France (CESAME) cohort. Methods We collected data from 19,486 patients with inflammatory bowel disease (IBD) enrolled in the observational CESAME study in France, from May 2004 through June 2005; 14.9% of participants had past or current anal and/or perianal Crohn's disease. Subjects were followed up for a median time of 35 months (interquartile range, 29–40 mo). To identify risk factors for anal cancer in the total CESAME population, we performed a case-control study in which participants were matched for age and sex. Results Among the total IBD population, 8 patients developed anal cancer and 14 patients developed rectal cancer. In the subgroup of 2911 patients with past or current anal and/or perianal Crohn's lesions at cohort entry, 2 developed anal squamous-cell carcinoma, 3 developed perianal fistula–related adenocarcinoma, and 6 developed rectal cancer. The corresponding incidence rates were 0.26 per 1000 patient-years for anal squamous-cell carcinoma, 0.38 per 1000 patient-years for perianal fistula–related adenocarcinoma, and 0.77 per 1000 patient-years for rectal cancer. Among the 16,575 patients with ulcerative colitis or Crohn's disease without anal or perianal lesions, the incidence rate of anal cancer was 0.08 per 1000 patient-years and of rectal cancer was 0.21 per 1000 patient-years. Among factors tested by univariate conditional regression (IBD subtype, disease duration, exposure to immune-suppressive therapy, presence of past or current anal and/or perianal lesions), the presence of past or current anal and/or perianal lesions at cohort entry was the only factor significantly associated with development of anal cancer (odds ratio, 11.2; 95% CI, 1.18-551.51; P = .03). Conclusions In an analysis of data from the CESAME cohort in France, patients with anal and/or perianal Crohn's disease have a high risk of anal cancer, including perianal fistula–related cancer, and a high risk of rectal cancer.

Optimised Home Parenteral Nutrition is still, after 35 years of progress, the > of benign but chronic Intestinal Failure. A better recognition of chronic Intestinal Failure, in its multiple facets, is warranted for a better approach of associated treatment to Home Parenteral Nutrition, i.e., intestinal trophic factors (growth hormone, Glucagon Like Peptide-2), rehabilitative surgery (reestablishment of colonic continuity, reverse jejunal segment in severe short gut type II) and/or reconstructive surgery (intestinal transplantation for end stage intestinal failure patients). Boundaries of permanent, judged irreversible, intestinal failure will be certainly modified in the following years by combining the various and effective therapies which optimise management by ameliorating absorption of the remnant short gut. The work done on short bowel syndrome in the past 20 years should be done in the next years for chronic-intestinal - pseudo-obstruction patients presenting with intestinal failure on a large European scale because chronic-intestinal - pseudo-obstruction is a group of heterogeneous but rare intestinal diseases. Intestinal transplantation is now a mature therapy with formal indication especially in case of Home Parenteral Nutrition failure (mainly Home Parenteral Nutrition-associated severe liver disease) where combined Liver-intestine transplantation is indicated before end-stage liver failure occurs. For high-risk patients, "preemptive" indication for intestinal transplantation alone will be discussed before home parenteral nutrition complications occur. No doubt that, for improving overall outcome in intestinal failure patients, reference centres should have in expert hands the whole spectrum of medicosurgical therapies for intestinal failure.

Undernutrition as well as specific nutrient deficiencies has been described in patients with Crohn's disease (CD), ulcerative colitis (UC) and short bowel syndrome. In the latter, water and electrolytes disturbances may be a major problem. The present guidelines provide evidence-based recommendations for the indications, application and type of parenteral formula to be used in acute and chronic phases of illness. Parenteral nutrition is not recommended as a primary treatment in CD and UC. The use of parenteral nutrition is however reliable when oral/enteral feeding is not possible. There is a lack of data supporting specific nutrients in these conditions. Parenteral nutrition is mandatory in case of intestinal failure, at least in the acute period. In patients with short bowel, specific attention should be paid to water and electrolyte supplementation. Currently, the use of growth hormone, glutamine and GLP-2 cannot be recommended in patients with short bowel.

In short bowel syndrome (SBS), although a remaining colon improves patient outcome, there is no direct evidence of a mucosal colonic adaptation in humans. This prospective study evaluates morphology, proliferation status, and transporter expression level in the epithelium of the remaining colon of adult patients compared with controls. The targeted transporters were Na+/H+exchangers (NHE2 and 3) and oligopeptide transporter (PepT1). Twelve adult patients with a jejuno-colonic anastomosis were studied at least 2 yr after the last surgery and compared with 11 healthy controls. The depth of crypts and number of epithelial cells per crypt were quantified. The proliferating and apoptotic cell contents were evaluated by revealing Ki67, PCNA, and caspase-3. NHE2, NHE3, PepT1 mRNAs, and PepT1 protein were quantified by quantitative RT-PCR and Western blot, respectively. In patients with SBS compared with controls, 1) hyperphagia and severe malabsorption were documented, 2) crypt depth and number of cells per crypt were 35% and 22% higher, respectively ( P < 0.005), whereas the proliferation and apoptotic levels per crypt were unchanged, and 3) NHE2 mRNA was unmodified; NHE3 mRNA was downregulated near the anastomosis and unmodified distally, and PepT1 mRNA and protein were unmodified. We concluded that, in hyperphagic patients with SBS with severe malabsorption, adaptive colonic changes include an increased absorptive surface with an unchanged proliferative/apoptotic ratio and well-preserved absorptive NHE2, NHE3, and PepT1 transporters. This is the first study showing a controlled nonpharmacological hyperplasia in the colon of patients with SBS.

Chronic intestinal pseudoobstruction (CIPO) is classified into enteric visceral myopathies, neuropathies, and/or mesenchymopathies. Although the histology usually permits to highlight pathologic abnormalities of CIPO, it fails in almost a third of cases. The yield of a systematic immunohistochemistry needs to be evaluating.Twenty-one adult patients with idiopathic CIPO [11 females/10 males, median age 23.1 (0.3 to 57) y] were included and compared with 27 control and 10 with mechanical obstruction patients. Comparison between standard histology (hematoxylin and eosin-stained sections) and systematic immunohistochemistry using muscular (smooth muscle alpha-actin, desmin, and smoothelin-A/B), nervous (Hu C/D, Bcl-2, and S100 protein), and mesenchymal (CD117) markers was carried out.Histology showed neuromuscular abnormalities in 13 out of 21 (62%) patients, consisting of enteric visceral myopathy in 9 (43%) patients, enteric visceral neuropathy in 2 (9.5%), and mixed neuromyopathy in 2 (9.5%). Among the 8 patients who had no histologic structural abnormality, 6 patients (75%) had underlying abnormalities detected with immunohistochemistry: immunostain with Hu C/D detected a hypoganglionosis (50 ganglion cells/cm) in 6 out of 21 (29%) patients, 4 of them undiagnosed on standard histology; CD117 (c-kit) detected a interstitial cells of Cajal defect in 10 out of 21 (48%) patients, 2 of them with no histologic structural abnormality. Smoothelin-A/B and desmin were useless as normally expressed in all patients with no myopathy; although it was not relevant in ileal samples (86% of abnormal expression in control patients), smooth muscle alpha-actin showed an abnormal expression in 2 CIPO patients (2/21).Immunohistochemistry using Hu C/D and CD117 antibodies combined to the standard histology increased the yield of detection of neuromuscular abnormalities in idiopathic CIPO patients.

Background & Aims: Tube feeding, recommended for patients with short bowel syndrome in only the postoperative period, has not been compared with oral feeding for absorption. We studied whether tube feeding increased absorption in patients with short bowel syndrome following the postoperative period. Methods: A randomized crossover study compared absorption between isocaloric tube feeding and oral feeding in 15 short bowel syndrome patients more than 3 months after short bowel constitution. An oral feeding period combined with enriched (1000 kcal ● day 1 ) tube feeding was also tested. We measured the net intestinal absorption rates of proteins, lipids, and total calories using elemental nitrogen, Van de Kamer, and bomb calorimetry methods, respectively. Results: Tube feeding increased the mean (SD) percent absorption (P < .001) of proteins (72% 13% vs 57% 18%), lipids (69% 25% vs 41% 27%), and energy (82% 12% vs 65% 16%) compared with oral feeding. In the group given the combined feedings (n 9), the total enteral intake and net percent absorption increased (P < .001) for proteins (67% 10%), lipids (59% 19%), and total energy (75% 8%) compared with oral feeding. Absorption (kcal ● day 1 ) was greater (P < .001) with tube (2225 457) and combined feedings (2323 491) than with oral feeding (1638 458). Conclusions: In patients with short bowel syndrome, continuous tube feeding (exclusively or in conjunction with oral feeding) following the postoperative period significantly increased net absorption of lipids, proteins, and energy compared with oral feeding.

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.

In human, citrulline (plasma concentration about 40 micromol/L) is an amino acid involved in intermediary metabolism and that is not incorporated in proteins. Circulating citrulline is mainly produced by enterocytes of the small bowel. For this reason plasma or serum citrulline concentration has been proposed as a biomarker of remnant small bowel mass and function. This article reviews this concept and its metabolic basis.Conditions in which there is a significantly reduced small bowel enterocyte mass and function and a plasma or serum citrulline were measured in adults and children. These studies included patients with a short bowel syndrome, villous atrophy states, Crohn's disease, during monitoring of digestive toxicity of chemotherapy and radiotherapy or follow-up of patients after small bowel transplantation.In all these situations, with more than 500 studied patients a decreased level of plasma citrulline correlated with the reduced enterocyte mass independently of nutritional and inflammatory status. A close correlation between small bowel remnant length and citrullinemia was found. In addition, diagnosis of intestinal failure was assessed through plasma citrulline levels in severe small bowel diseases in which there is a marked enterocyte mass reduction.The threshold for establishing a diagnosis of intestinal failure is lower in villous atrophy disease (10mumol/L) than in short bowel syndrome (20mumol/L). Compromised renal function is an important factor when considering plasma citrulline levels as a marker of intestinal failure as this potentially can increase circulating citrulline values.Reduced plasma citrulline levels are an innovative quantitative biomarker of significantly reduced enterocyte mass and function in different disease states in humans.

Resume La pseudo-obstruction intestinale chronique (POIC) est une maladie rare, caracterisee par des symptomes chroniques et/ou recidivants, suggerant une obstruction intestinale sans obstacle organique. Elle est caracterisee par une incapacite intestinale a propulser le bol alimentaire, d’origine neurogene et/ou myogene. Elle se manifeste par des tableaux cliniques varies, associant des signes d’occlusion intestinale et des signes generaux. Chez l’adulte, la POIC est le plus souvent secondaire a une maladie systemique. Les pathologies les plus frequemment incriminees etant une connectivite, certains toxiques, une endocrinopathie, une amylose, une enterite radique ou un syndrome paraneoplasique. Plusieurs cas de POIC, en rapport avec un syndrome paraneoplasique, ont ete rapportes en association a des anticorps anti-Hu. Il s’agit d’anticorps circulants, diriges contre une nucleoproteine neuronale du systeme nerveux central et/ou peripherique (Anna-1). Leur recherche doit etre systematique en cas de POIC, notamment apres 40 ans (association a un cancer bronchopulmonaire a petites cellules dans 75 % des cas). De facon recente et exceptionnelle, l’association anti-Hu et POIC a ete rapportee en l’absence de neoplasie. Nous rapportons, ici, le cas d’un patient atteint de POIC et d’un syndrome des anti-Hu, en l’absence de neoplasie sous-jacente avec un recul de 13 ans.

Resume La flore intestinale peut se reveler deletere dans certaines diarrhees infectieuses ou postantibiotique. Cet ecosysteme complexe est modifie et augmente quantitativement lors des resections etendues de l’intestin grele, pouvant ainsi en modifier le pronostic. Les cliniciens cherchent, par l’utilisation d’outils moleculaires pour l’analyse de la flore intestinale, notamment dans certaines pathologies digestives, de mieux comprendre les interactions flore–intestin.

In addition to non-digested nutrients, human stools contain endogenous substrates, among which bacteria are a major component, whose growth may be stimulated when more dietary nutrients are available for bacterial fermentation, as in patients with malabsorption syndrome. We assessed the energy content and composition of both stools and faecal bacteria in healthy volunteers and patients with a short bowel and colon in continuity (SBC). Our goal was to clarify the magnitude of error introduced by the faecal bacteria in the measurement of the digestibility of ingested energy and nutrients. We studied six healthy volunteers and six patients with a SBC under free oral intake. The bacterial mass of stools was isolated. In the bacterial fractions and fresh stools, calorie, fat, nitrogen and short-chain fatty acid contents were determined. The Wilcoxon signed rank or the Mann–Whitney tests were used for comparison. In healthy volunteers and patients with SBC, faecal bacterial mass accounted for 44 and 35% of faecal dry weight, and contained 50 and 34% of total faecal energy. In healthy volunteers, the apparent digestibilities when corrected by bacterial constituents (88–97% according to nutrients) were significantly higher than the apparent non-corrected digestibilities (84–94%). In patients with SBC, the corrected apparent digestibilities (69–89% according to nutrients) were significantly higher than the apparent non-corrected digestibilities (54–83%). The error introduced by the faecal bacterial fraction when assessing the extent of nutrient digestibility is small in healthy volunteers; it is more pronounced in patients with SBC, reaching 18% for the digestibility of ingested fat.

Chronic intestinal pseudo-obstruction (CIPO) is a disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Pseudo-obstruction is an uncommon condition and can result from primary (40%) or secondary (60%) causes. The most common symptoms are nausea, vomiting, abdominal distension, abdominal pain and constipation or diarrhea. These symptoms are usually present many years before CIPO diagnosis. They can lead to severe electrolyte disorders and malnutrition. Principles for management of patients with CIPO are: to establish a correct clinical diagnosis in excluding mechanical obstruction; to perform a symptomatic and physiologic assessment of the gastrointestinal tract involved; to look for extra-intestinal manifestations, especially for myopathy and neuropathy; to discuss in some cases a surgery for full-thickness intestinal biopsies, and/or a neuromuscular biopsy in case of mitochondrial cytopathy suspicion. The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. Treatment of CIPO includes prokinetic agents which may help to reduce gastrointestinal symptoms Courses of antibiotics may be needed in patients with symptoms suggestive of bacterial overgrowth. When necessary, enteral nutrition is preferred. In carefully selected patients, feeding jejunostomy with or without decompression gastrostomy may be tried. Long term parenteral nutrition should be reserved for patients who can not tolerate enteral nutrition. Intestinal transplantation can be discussed in selected patients.

The development of parenteral nutrition (PN) was an important, life-saving advance in the care of patients with short bowel syndrome (SBS). Nevertheless, its long-term use is often associated with complications. Therefore, it is desirable, when possible, to wean SBS patients to an oral diet. Given the complexity of PN weaning and the lack of published guidelines, the purpose of this article is to describe strategies of PN weaning and illustrate important clinical considerations during the weaning process. Patient education and motivation are key factors in successful PN weaning. The patient should have clearly defined care protocols and understand the importance of each aspect of the weaning program. Other factors likely to influence weaning success include the length and health of the remnant bowel, the presence of a colon, and the degree to which bowel adaptation has occurred. It is imperative that daily oral fluid and caloric intake goals are met prior to initiating PN weaning and that the patient receives an optimized dietary and medication plan. During weaning, the most practical measures for assessing adequate hydration and nutritional status are oral intake, stool and urine output, serum electrolytes and visceral proteins, and body weight. PN reductions can be made by either decreasing the days of PN infusion per week or decreasing the PN infusion volume equally across all days of the week. Use of recombinant human growth hormone, with or without glutamine, may play a role in facilitating the PN weaning process.

Intravenous lipid emulsions (ILE) have demonstrated advantages including prevention of essential fatty acid (EFA) deficiency; however, too much EFA can down regulate fatty acid elongation leading to an imbalance of nutritional compounds in plasma and cell membranes. An olive oil-based ILE containing long-chain triacylglycerols (LCT) with a low content (20 %) of PUFA was administered for home parenteral nutrition (HPN) and compared with a conventional soyabean oil-based ILE (PUFA content, 60 %). Thirteen patients (26–92 years) with stable intestinal failure were randomised after a 1-month run-in period with a medium-chain triacylglycerols–LCT-based ILE, to receive 3 months of HPN with either olive oil- (n6) or soyabean oil-based (n7) ILE. The nutritional impact and safety of HPN, oral intakes and absorption rates, phospholipid fatty acids in plasma and lymphocyte cell membrane were assessed. The only clinical event reported was one case of pneumonia (soya group). In both groups, 20 : 3n-9:20:4n-6 ratios remained within normal ranges (0·03–0·07). There was a significant increase of γ-linolenic acid (γ-LA) in plasma and lymphocyte cell membrane (P=0·02) and of oleic acid in plasma (PP=0·02), but neither with fat intakes nor with fat absorption rates. In conclusion, plasma and lymphocyte EFA pattern remained in normal ranges without EFA deficiency with both lipid emulsions, despite a lower content ofn-3 andn-6 series with the olive oil-based ILE.

We report on four patients with severe polyneuropathy associated with intestinal pseudoobstruction (MNGIE). Three patients presented characteristic supranuclear ophthalmoplegia, and hyperdense signals on T2 weighted cerebral MRI and dystrophic mitochondria in Schwann cells and in endothelial cells in nerve biopsy specimens. Two of these patients had a Charcot-Marie-Tooth (CMT) presentation. All three were heterozygous for a recessively transmitted double substitution in the TP gene: Glu286Lys/Glu289Ala, Asp156Gly/Leu177Pro and Glu289Ala/Gly387Asp. The fourth patient, who was the only patient of this series with an affected sib, had no oculomotor manifestations, nor T2 hyperdense signals on brain MRI, and no TP gene mutation and or morphological abnormalities of mitochondria on electron microscopic examination. He was the only patient of this series with an affected sib. The three patients with the full MNGIE syndrome died before the age of 30 years. Detailed results of nerve pathology show that severe axonal degeneration is associated with segmental abnormalities of the myelin sheath in this syndrome which appears genetically heterogeneous. Our findings suggest that only ophthalmoplegia and hyperdense signals on cerebral MRI are directly related to the mitochondriopathy.

Resume Il faut distinguer les resections intestinales courtes ( a 2 m ; 15 % des cas), qui entrainent un syndrome de grele court (SGC). L’evaluation anatomique du SGC comprend : (a) la longueur et le site, jejunal et/ou ileal, de grele post duodenal restant ; (b) le type d’anastomose intestinale – enterostomie terminale (type I) – anastomose jejuno-colique (type II) ou – anastomose jejuno-ileocolique (type III), et (c) la mesure du pourcentage de colon restant en continuite. Toutes ces donnees conditionnent la clinique du SGC. La resection ileale entraine, si ≥ a 60 cm un deficit en B12 et, si ≥ a 1 m une malabsorption lipidique notable (steatorrhee > a 20 g/J) avec deficit en vitamines liposolubles (D > K > E > A). Leur supplementation – B12 par voie IM et vitamines liposolubles par voie orale – doit etre systematique. Le SGC de type I se traduit par une diarrhee hydroelectrolytique (H20 et Na), profuse en post operatoire, et dont les regles dietetiques hydriques sont strictes. Le SGC entraine un syndrome de malabsorption responsable d’une denutrition proteino-energetique, laquelle doit etre traitee par une hyperalimentation orale libre, hyperlipidique pour le type I, hypergluciqique pour les type II et III. La presence d’un colon en continuite (SGC types II et III)l reduit les pertes hydroelectrolytiques et, par le biais de l’hyperfermentation bacterienne des glucides, augmente la production des acides gras a chaine courte : leur absorption entraine une importante recuperation energetique ; par contre, la lithiase oxalique renale (25 % des cas) doit faire l’objet d’une prevention primaire par regime sans oxalate et calcitherapie orale et une encephalopathie D lactique survient chez 2 % des patients. Le SGC entraine une hypomagnesemie severe quasi constante qui induit une hypocalcemie vitamine D resistante, et une hypokaliemie resistante aux sels de potassium.

Resume Le magnesium a un role majeur dans le metabolisme energetique et proteique et dans l'homeostasie des electrolytes. Un deficit magnesien ou une hypomagnesemie traduit quasi constamment une pathologie sous-jacente d'origine digestive ou renale. Elle est tres frequente en milieu hospitalier notamment en milieu de reanimation. L'efficacite et la tolerance des differents sels de magnesium disponibles sur le marche sont mal connues. Cette mise au point resume les principales causes d'hypomagnesemie ainsi que les differentes methodes de traitement selon les situations cliniques.

Background and aims: Intestinal adaptation after small bowel resection in humans is debated. We have quantified in adult short bowel (remnant small bowel length 1.5×resting energy expenditure), independently and negatively related to fat absorption (p

To assess the dynamics of taurine metabolism in vivo, two sets of studies were carried out in healthy volunteers. First, pilot studies were carried in a single human subject to determine the time course of plasma and whole blood isotope enrichment over the course of an 8-h, unprimed continuous infusion of [1,2-13C2]taurine. Second, five healthy adult males received two tracer infusions on separate days and in randomized order: 1) a 6-h continuous infusion of [1,2-13C2]taurine (3.1 ± 0.2 μmol·kg−1·h−1) and 2) a bolus injection of [13C2]taurine (3.0 ± 0.1 μmol/kg). Isotope enrichments in plasma and whole blood taurine were determined by gas chromatography-mass spectrometry. The pilot experiments allowed us to establish that steady-state isotope enrichment was reached in plasma and whole blood by the 5th h of tracer infusion. The plateau enrichment reached in whole blood was lower than that obtained in plasma taurine ( P < 0.02). In the second set of studies, the appearance rate (Ra) of plasma taurine, determined from continuous infusion studies was 31.8 ± 3.1 μmol·kg−1·h−1. After a bolus injection of tracer, the enrichment decay over the subsequent 2 h was best fitted by a two-exponential curve. Taurine Ra was ≈85% higher when determined using the bolus injection technique compared with continuous infusion of tracer. We conclude that 1) taurine Ra into plasma is very low in healthy postabsorptive humans, and, due to taurine compartmentation between the extra- and intracellular milieus, may represent only interorgan taurine transfer and merely a small fraction of whole body taurine turnover; and 2) the bolus injection technique may overestimate taurine appearance into plasma. Further studies are warranted to determine whether alterations in bile taurine dynamics affect taurine Ra.

Background: Long-term home parenteral nutrition (HPN) may induce liver disorders. Transient elastography (TE) has been proposed as a noninvasive alternative to liver biopsy analysis for assessment of the progression of hepatic fibrosis to cirrhosis. The goal of this study was to compare values from TE measurements to biopsy-determined stages of histologic fibrosis in patients receiving HPN. Methods: In this multicenter prospective study, patients receiving long-term HPN (‰6 months) who required a liver biopsy for clinical reasons were included. TE (FibroScan) values for each patient were compared with the degree of hepatic fibrosis measured from biopsy specimens based on the Brunt classification. TE values were also correlated to biochemical and histologic cholestasis. Two noninvasive indices for predicting liver fibrosis (APRI and FIB-4) were also evaluated. Results: Thirty patients were included in this study (mean age, 42.1 years; 63% male). The mean duration of HPN was 100.7 months; 25 patients had a short bowel and 13 had an intestinal stoma. Biochemical cholestasis was described in 22 patients. Liver histologic features varied among these patients. There was no correlation between the values of TE and the stages of histologic fibrosis, but TE values were significantly correlated to serum bilirubin level and the severity of histologic cholestasis as well as APRI and FIB-4 scores. Conclusions: In patients with long-term HPN, TE failed to assess the degree of hepatic fibrosis. This could be due to the heterogeneity of liver histologic features observed in these patients and the presence of chronic cholestasis.

For the assessment of colonic Crohn's disease (CCD) attacks, no clinicobiological and/or morphological (endoscopic, radiological) criteria of severity have been validated in relation to anatomical criteria of severity (ACS) as a gold standard obtained from colectomy specimen examination. Our objective was to assess the accuracy of colonoscopy in predicting the anatomical severity grading of CCD.Colectomy specimens from 78 consecutive patients operated between 1982 and 1996 for CCD resistant to medical treatment were analyzed and classified into two groups according to the presence (ACS+) or absence (ACS-) of ACS. These were defined as either deep ulcerations eroding the muscle layer, or mucosal detachments or ulcerations limited to the submucosa but extending to more than one third of one defined colonic segment (right, transverse, left colon). Three endoscopic criteria of severity (ECS) were then defined: 1) deep ulcerations eroding the muscle layer (ECS1), 2) deep ulcerations not eroding the muscle layer but involving more than one third of the mucosal area (ECS2), and 3) mucosal detachment on the edge of ulcerations (ECS3).According to colectomy specimen examination, 68 and 10 patients belonged to ACS+ and ACS- groups, respectively. ECS1, ECS2, and/or ECS3 were found in 70 patients. Positive predictive values of ECS1, ECS2, and ECS3 for the presence of ACS were 90%, 98%, and 92%, respectively. Negative predictive values were 43%, 72%, and 23% respectively. However, at least one ECS (ECS1, ECS2, or ECS3) was found in 95% of patients with ACS. The extent of ulcerations at colonoscopy was correlated to the results of colectomy specimen examination (p0.001). Taking into account only patients with ACS, 88% of those with at least one ECS were diagnosed through left side colonoscopy. Usual clinical and biological severity criteria were not different in ACS + and ACS- groups. Two cases of toxic megacolon and toxic shocks were observed after the colonoscopy.In experienced hands, colonoscopy can be useful in severe CCD attacks. When at least one ECS is found, colonoscopy predicts the anatomical severity of the colitis with a high probability. Conversely, when none of the three ECSs is found, colonoscopy can reasonably exclude the diagnosis of severe anatomical CCD.

BACKGROUND Benefits of recombinant human growth hormone (rhGH) alone or combined with glutamine in patients with intestinal failure because of short-bowel syndrome remain controversial. OBJECTIVE We explored effects of rhGH on whole-body protein metabolism in patients with short-bowel syndrome with intestinal failure (SBS-IF) to gain insight into its mechanism of action. DESIGN Eight stable hyperphagic patients with severe SBS-IF received, in a double-blind, randomized crossover study, low-dose rhGH (0.05 mg · kg⁻¹ · d⁻¹) and a placebo for two 3-wk periods. Leucine and glutamine kinetics under fasting and fed conditions, fat-free mass (FFM), and serum insulin were determined on the final day of each treatment. RESULTS rhGH increased FFM and nonoxidative leucine disposal (NOLD; an index of protein synthesis) (P < 0.02), whereas FFM and NOLD were correlated in the fed state (r = 0.81, P = 0.015). With rhGH administration, leucine release from protein breakdown (an index of proteolysis) decreased in the fed compared with fasting states (P = 0.012), which was not observed with the placebo. However, the fast-to-fed difference in leucine release from protein breakdown was not significantly different between rhGH and placebo (P = 0.093). With rhGH, the intestinal absorption of leucine and glutamine increased (P = 0.036) and correlated with serum insulin (r = 0.91, P = 0.002). rhGH increased glutamine de novo synthesis (P < 0.02) and plasma concentrations (P < 0.03) in both fasting and fed states. CONCLUSIONS In SBS-IF patients, feeding fails to decrease proteolysis in contrast to what is physiologically observed in healthy subjects. rhGH enhances FFM through the stimulation of protein synthesis and might decrease proteolysis in response to feeding. Improvements in de novo synthesis and intestinal absorption increase glutamine availability over the physiologic range, suggesting that beneficial effects of rhGH in hyperphagic patients might be achieved without glutamine supplementation.

Background: Home parenteral nutrition (HPN) is a lifesaving treatment in patients with intestinal failure. Dependency of nutritional support becomes permanent for the majority of patients who had received HPN for at least 2 years. The alternative to long term HPN in selected patients is intestinal transplantation. Aims: To study some of the clinical, social and rehabilitation aspects of long-term HPN treatment. Methods: A survey was performed in nine European HPN centres. The questionnaire covered epidemiologic data, underlying diseases, intestinal anatomy, nutritional support and status, marital status, rehospitalization rate, HPN complications, rehabilitation score, drugs use, coexistent diseases and interest in intestinal transplantation. For some items, data were collected within 12 months prior to the evaluation. Results: This survey included 228 patients with a median age of 49 years. The median duration of HPN was 7 years (range 2–24 years). Short bowel length less than 100 cm was reported in 65% of patients with a predominance of end-jejunostomy or jejuno-colonic anastomosis. Global subjective nutritional status was normal in 79% of the patients, who were supplied with a mean number of 5.6 bags of parenteral nutrition weekly. Rehospitalizations within the 12 months prior to evaluation accounted for a mean period of 23 days and were due to HPN complications in half of the cases. Catheter-related sepsis was the most frequent HPN-complication. Bone metabolism disorders, which seemed to be more common than liver diseases, were directly related to HPN duration. One-third of the HPN patients was regularly consuming analgesics or opiates. HPN impair complete rehabilitation status but may improve the status of patients who had a very low rehabilitation score before starting HPN. An interest of intestinal transplantation was noted in only 10% of medical teams and in 8% of HPN patients. Conclusions: This study is the largest European survey on long-term HPN patients with long standing or permanent intestinal failure. Data enlighten clinical, social and rehabilitation aspects of patients who could face the option of intestinal transplantation in the future.

Parenteral nutrition is a risk factor for catheter-related bloodstream infection. Here we reviewed strategies for the prevention of catheter-related infections, which always must begin with the cornerstone of prevention: the strict adherence to aseptic techniques. Most research has been interested in coated catheters. From these results, it may be concluded that antibiotics or antiseptic-impregnated catheters, like those with minocycline-rifampicin or chlorhexidine/silver sulfadiazine, significantly reduce catheter-related blood stream infections. Antibiotics or antiseptic-impregnated central venous catheters may even result in cost saving in intensive care units. Antiseptic or antibiotic-lock techniques would also be of interest to prevent catheter-related sepsis in high-risk patients who are receiving parenteral nutrition.

Background & Aims: No blood marker assessing the functional absorptive bowel length has been identified. Plasma citrulline, a nonprotein amino acid produced by intestinal mucosa, is one candidate. We tested this hypothesis in adult patients with the short-bowel syndrome, whose condition can lead to intestinal failure. Methods: In 57 patients, after a minimal follow-up of 2 years subsequent to final digestive circuit modification, postabsorptive citrulline concentration was measured and parenteral nutrition dependence was used to define permanent (n = 37) and transient (n = 20) intestinal failure. Absorptive function, studied over a 3-day period, was evaluated by net digestive absorption for protein and fat (n = 51). Relations between quantitative values were assessed by linear regression analysis and cutoff citrulline threshold, for a diagnosis of intestinal failure by linear discriminant analysis. Cox model was used to compare citrulline threshold and anatomic variables of the short bowel as indicators of transient as opposed to permanent intestinal failure. Results: In patients with short-bowel syndrome, citrulline levels were lower than in controls (n = 51): 20 ± 13 vs. 40 ± 10 μmol/L (mean ± SD), respectively ( P P r = 0.86) and to net digestive absorption of fat, but to neither body mass index nor creatinine clearance. A 20-μmol/L threshold citrullinemia, (1) classified short bowel patients with permanent intestinal failure with high sensitivity (92%), specificity (90%), positive predictive value (95%), and negative value (86%); and (2) was a more reliable indicator (odds ratio, 20.0; 95% confidence interval, 1.9–206.1) than anatomic variables (odds ratio, 2.9; 95% confidence interval, 0.5–15.8) to separate transient as opposed to permanent intestinal failure. Conclusions: In patients with short-bowel syndrome, postabsorptive plasma citrulline concentration is a marker of functional absorptive bowel length and, past the 2-year adaptive period, a powerful independent indicator allowing distinction of transient from permanent intestinal failure. GASTROENTEROLOGY 2000;119:1496-1505

Resume Les complications hepatiques de la nutrition parenterale sont frequentes et potentiellement graves. Elles se manifestent habituellement par une cholestase chronique biologique et histologique qui, si elle perdure, peut aboutir a la constitution de lesions severes a type de fibrose extensive et de cirrhose et peuvent alors entrainer toutes les complications classiques de l'insuffisance hepatocellulaire et de l'hypertension portale. Les lesions histologiques se rapprochent de celles observees lors des cirrhoses biliaires primitives ou des cholangites sclerosantes mais se caracterisent par la frequence de la surcharge en phospholipides. Les facteurs etiopathogeniques impliques dans ces lesions sont nombreux et souvent intriques. On peut ainsi distinguer des facteurs malade-dependants dont les plus importants sont un grele tres court et la contamination bacterienne chronique du grele, et des facteurs nutrition-dependants : en excluant l'hypernutrition, un apport lipidique superieur a 1 g · kg −1 · j −1 semble etre un des facteurs etiologiques essentiels. Il est fondamental de reduire l'incidence de ces complications qui peuvent conduire a un deces par hepatopathie ou a une double transplantation foie-grele. La prevention de l'hepatopathie est fondee sur la reconnaissance des facteurs malade-dependants et la limitation des apports caloriques et lipidiques. Parmi les moyens therapeutiques, l'acide ursosodesoxycholique et l'utilisation de solutes enrichis en taurine sont certainement prometteurs.

Resume Le but de cette enquete retrospective a ete de rapporter l'activite annuelle d'un centre agree de nutrition parenterale a domicile. La cohorte de 78 patients, dont 19 inclus en 1998, etait composee de patients avec insuffisance intestinale « maligne(14 %) ou « benigne mais chronique(86 %) ayant un syndrome de grele court dans 64 cas (82 %). Au 31 decembre 1998, dix patients (13 %) avaient ete sevres de la nutrition parenterale, 12 etaient decedes (15 %), dont 7 des 11 patients avec insuffisance intestinale maligne, et 56 (72 %) restaient dependants de la nutrition parenterale depuis en moyenne 47 mois (1–248). Aucun deces n'a ete secondaire a la nutrition parenterale. La majorite des patients de la cohorte etait autonome pour la realisation de la nutrition parenterale a domicile (60 %) et recevait une nutrition parenterale par catheter tunnelise a embout externe (72 %), aucun apport lipidique calorique (62 %) et a une alimentation orale non limitee (71 %). L'incidence des complications ( n = 73) reliees au catheter a ete de 1,17 par annee catheter, dont 70 % de complications septiques. Ces dernieres ont ete trois fois moins frequentes ( p p

BACKGROUND Breath tests are currently used to qualitatively assess colonic fermentation; no quantitative estimations are available for healthy subjects. OBJECTIVE This study describes a stable-isotope-dilution method to measure acetate production quantitatively from colonic bacterial fermentation. DESIGN Six volunteers received a primed, constant, intravenous infusion of [1-13C]acetate at a rate of 1.01 +/- 0.04 micromol x kg(-1) x min(-1) for 7 h. They ingested 20 g pure lactulose after 1 h of the tracer infusion. Expired air and arterialized venous blood were sampled every 15 min. RESULTS Before lactulose intake, the breath-hydrogen concentration was 7 +/- 2 ppm and the plasma acetate concentration and isotopic enrichment were 141 +/- 14 micromol/L and 14.8 +/- 1.4 moles percent excess, respectively. Whole-body acetate turnover was 6.0 +/- 0.7 micromol x kg(-1) x min(-1). After lactulose ingestion, maximum breath hydrogen and acetate concentrations reached 63 +/- 15 ppm (P = 0.004) and 313 +/- 25 micromol/L (P = 0.002), respectively, whereas [13C]acetate enrichment decreased to 9.9 +/- 1.3 moles percent excess (P = 0.03). Whole-body acetate turnover increased to 9.8 +/- 1.5 micromol x kg(-1) x min(-1) and later decreased almost to baseline values. Colonic lactulose fermentation yielded 140 +/- 12 mmol acetate over 6 h, representing 86% of the production based on stoichiometric equations. CONCLUSION This new method provides a quantitative estimate of colonic carbohydrate fermentation via evaluation of acetate production.

The Leiden factor V mutation is observed in 20% of unexplained lower limb venous thromboses and involves substitution of the arginine residue at position 506 by glutamine (R506Q). It is known to decrease the anticoagulant activity of activated protein C. This case report describes 4 cases of small bowel infarction (SBI) associated with the presence of this mutation. Two cases of arterial and 2 cases of venous SBI were observed. Extensive assessment excluded the usual causes of SBI and plasma hypercoagulation syndrome (antithrombin III, protein C, and protein S deficiency and myeloproliferative syndrome). An abnormal resistance to activated protein C was observed. Molecular analysis consisting of polymerase chain reaction amplification and digestion with MnlI showed that 2 patients were heterozygous and 2 were homozygous for the R506Q mutation. Despite familial history of thrombosis in only 1 patient, first- and second-degree relatives of 2 patients also had the presence of the mutation. Examination for the presence of abnormal resistance to activated protein C should be part of the etiological assessment of SBI. Its presence may warrant consideration of long-term anticoagulant therapy, especially for patients with shortened small bowel who are treated by home parenteral nutrition with deep venous access.

The vitamin B 12 status of 20 subjects who were on home parenteral nutrition after surgical or functional small bowelresection and were given 1000 μ g cyanocobalamin every 3 mo was studied by comparing their plasma vitamin B 12 , homocysteine (HS), and methylmalonic acid (MMA) concentrations. The plasma vitamin B 12 concentration (median 145 pmol/L, 95% confidence interval: 123–217) was subnormal in four cases and borderline in four others. In the “low B 12 ” group, the concentrations of the markers of vitamin B 12 deficiency were in the normal range: HS 10.7 μ mol/L (8.0–12.3); and MMA, 0.15 μ mol/L (0.09–0.19). References values were HS, 10.0 μ mol/L (9.4–12.6); and MMA, 0.16 μ mol/L (0.10–0.19). Thus, there were no metabolic signs of vitamin B 12 deficiency in these subjects on parenteral nutrition, despite the fact that their plasma vitamin B 12 levels were low. Analysis of individual data showed that the four patients with low circulating B 12 had markers of intracellular vitamin B 12 deficiency in the normal range.

Une enquete retrospective a ete realisee en 1994, incluant 496 patients adultes qui ont debute une nutrition parenterale a domicile (NPAD) au cours de l'annee 1993, au sein de 75 centres repartis dans 13 pays europeens. Pour les pays ayant enregistre plus de 80% des nouveaux cas (423 patients), l'incidence et la prevalence furent estimees dans une echelle de 0,2 a 4,6 patients et de 0,3 a 12,2 patients pour 1 000 000 habitants par an, respectivement. Chez les patients etudies, la maladie sous-jacente etait soit un cancer (42%), une maladie de Crohn (15%), une maladie vasculaire (13%), une enterite radique (8%), un syndrome d'immunodeficience acquise (4%) ou autre (18%), Le syndrome du grele court et l'obstruction intestinale etaient les deux indications majeures pour la NPAD, respectivement dans 31% et 22% des cas. Soixante-trois pour cent des centres avaient une equipe de nutrition. La NPAD etait le plus souvent administree via un catheter central tunnelise (73%) et sous le mode cyclique nocturne (90%), alors qu'elle etait la seule source de nutrition dans 33% des cas. Seulement 44 % de patients prenaient en charge de maniere autonome leur NPAD. La presente etude indique que le cancer est devenu actuellement l'indication majeure pour la NPAD en Europe ; il existe cependant une grande heterogeneite dans les indications parmi les pays europeens ayant participe a l'enquete. La probabilite de survie apres une duree allant de 6 a 12 mois etait faible en cas de SIDA (n = 8; 12%) et de cancer (n = 78; 29%) mais tres satisfaisante dans les autres indications de NPAD (n = 115 ; 92%).

We compared the tolerance of a diet providing 20 g/d lactose and a lactose-free diet in 14 patients with short-bowel syndrome with either the colon in continuity (group A, n = 8) or a terminal jejunostomy (group B, n = 6). Lactose tolerance was studied after a single 20-g lactose load in the fasting state, and during two 3-d periods during which the subjects consumed their usual diet plus either 20 g/d lactose, with no more than 4 g/d as milk, or no lactose. Records and measurements included symptoms, fecal weight, and during the 8 h after the lactose load, breath-hydrogen excretion (group A) or lactose and hexoses flow rates in stomal effluents (group B). Results are expressed as medians with ranges in parentheses. Lactose absorption was 61% (0-90) in group A and 53% (18-84) in group B, and no symptoms of intolerance were noticed. During the lactose-rich diet as compared to the lactose-free diet, no symptoms were noticed nor was there any worsening of diarrhea: 1534 g/d (240-4760) versus 1466 (1590-7030) in group A, and 4122 g/d (1730-6830) versus 3496 (1590-7030) in group B. We conclude that a diet providing 20 g/d lactose with no more than 4 g/d as milk is well tolerated in the majority of patients with short-bowel syndrome, and that a lactose-free diet has usually no benefit in these subjects.

Objective : To evaluate the efficacy of total parenteral nutrition in AIDS patients. Design : A prospective, randomized, controlled, multicentre trial. Methods : Over a period of 2 months, 31 malnourished and severely immunodepressed AIDS patients were assigned to receive either dietary counselling (n = 15) or home total parenteral nutrition (TPN ; n = 16) via a central venous access after an educational program. Results were analysed by intent-to-treat basis. Results : Bodyweight change was +8 kg (+ 13 ± 3%) in the TPN group and - 3 kg (- 6 ± 2%) in the control group (P < 0.0006). Lean body mass increased in the TPN group (+ 9 ± 3%) and decreased in the control group (- 5 ± 3% ; P < 0.004) while body cell mass increased in the former (+ 15 ± 4%) and decreased in the latter (- 12 ± 6% ; P< 0.002). Nutritional subjective global assessment, subjective self-reported health feeling and Karnofsky index were also improved by TPN. Infection line sepsis incidence remained low (0.26 per 100 patient-days). However, no difference in survival rate was exhibited between the two groups by the log-rank test. Conclusion : We conclude that home TPN is an efficient treatment of malnutrition in severely immunodepressed AIDS patients.

Resume La nutrition parenterale a domicile (NPAD) voit croitre sa prevalence, chez l'adulte, essentiellement par une augmentation de sa population ayant une insuffisance intestinale non SIDA, non cancer, et dont la probabilite de survie a 5 ans atteignait 75% a la fin des annees quatre-vingts. Pres de 50% de cette population recoit en effet une NPAD de longue duree, c'est-a-dire superieure a deux ans (NPLD) dont 75% de patients ayant un syndrome de grele court. La NPLD concerne donc les patients ayant une insuffisance intestinale jugee irreversible pour lesquels l'alternative medicale (facteurs trophiques), et/ou chirurgicale (segment de grele antiperistaltique chez l'adulte, transplantation intestinale chez l'enfant et l'adulte) est ici analysee. La mise en evidence d'un marqueur biologique de masse intestinale fonctionnelle serait donc un pas decisif pour definir l'insuffisance intestinale irreversible indiquant ainsi un des traitements alternatifs de la NPLD, avant que ne surviennent les complications severes de celle-ci, dont la thesaurismose lipidique et l'hepatopathie decompensee. La NPLD comporte des besoins en substrats specifiques du fait de la duree de traitement et de la pathologie causale traitee. Afin de demontrer que l'industrie a la necessite de developper de nouveaux produits en ce domaine, la recherche multicentrique, compte tenu d'un nombre limite de patients a travers les centres agrees de NPAD, doit etre poursuivie et renforcee.

Summary Background & aims Short bowel syndrome (SBS)-intestinal failure (IF) patients have impaired quality of life (QoL) and suffer from the burden of malabsorption and parenteral support (PS). A phase III study demonstrated that treatment with teduglutide, a glucagon-like peptide 2 analogue, reduces PS volumes by 32% while maintaining oral fluid intake constant; placebo-treated patients had reduced PS by 21%, but oral fluid intake increased accordingly. As effects of teduglutide on QoL are unknown, they were investigated here. Methods QoL analyses from a double-blind, randomised Phase III study in 86 SBS-IF patients receiving teduglutide (0.05 mg/kg/day s.c.) or placebo over 24 weeks. At baseline and every 4 weeks, QoL was assessed using the validated SBS-QoL™ scale. Results PS reductions were associated with QoL improvements (ANCOVA, p = 0.0194, SBS-QoL per-protocol). Compared to baseline, teduglutide significantly improved the SBS-QoL™ total score and the score of 9 of 17 items at week 24. These changes were not significant compared to placebo. Teduglutide-treated patients with remaining small intestine >100 cm experienced more gastrointestinal adverse events (GI-AE), unfavourably affecting QoL. Conclusions Overall, PS volume reductions were associated with improvements in SBS-QoL™ scores. The short observation period, imbalances in oral fluid intake in relation to PS reductions, large patient and effect heterogeneity and occurrence of GI-AE in a subgroup of teduglutide-treated patients may account for the inability to show statistically significant effects of teduglutide on SBS-QoL™ scores compared to placebo. ClinicalTrials.gov identifier: NCT00798967.

To aid in the understanding of the human requirement for 2-aminoethanesulphonic acid (taurine), a method was developed for the analysis of plasma taurine and used for preliminary studies of the kinetics of plasma taurine in three healthy adult volunteers. A gas chromatographic/electron impact mass spectrometric (GC/EIMS) stable isotope ratio method was developed for the measurement of enrichment of the tracer [1,2-13C2] taurine in plasma. Natural abundance taurine and [1,2-13C2] taurine were analysed as their N-pentafluorobenzoyldi-n-butylamide (PFB-dBA) derivatives by GC/EIMS. With the addition of an internal standard, methyltaurine, the taurine concentration could also be measured. After an overnight fast, three healthy adult human subjects were given an intravenous priming dose of [1,2-13C2] taurine (3 μmol kg−1), which was immediately followed by a continuous infusion of the tracer (3 μmol kg−1 h−1) for 6 h. The mean plasma plateau enrichment was 8.07 ± 0.46 mol% excess (RSD = 5.67%) and a rate of appearance of 34 ± 2.23 μmol kg−1 h−1 was calculated. The plasma taurine concentration was found to be 56 ± 14 μmol l−1. This technique for the assessment of plasma taurine kinetics should enhance the understanding of taurine metabolism as it could be a conditionally essential amino acid in man.

To assess the effect of colonic fermentation on respiratory gas exchanges, six methane-nonproducing healthy volunteers ingested in the postabsorptive state 1 wk apart either 90 mL lactulose syrup containing 60 g lactulose, 4 g lactose, and 7 g galactose or the same solution but without lactulose (control solution). Six patients with short bowel and remnant colon (SBS) also ingested 90 mL lactulose syrup. Carbon dioxide production (VCO 2 ), oxygen consumption (VO 2 ), respiratory quotient (RQ), and hydrogen excreted in breath were measured basally and for 4 h after the ingestion of solutions. In healthy volunteers within 4 h after ingestion of the control solution, VCO 2 and the RQ decreased whereas VO 2 remained unchanged. In contrast, in healthy volunteers and patients with SBS, VCO 2 and the RQ increased after lactulose ingestion, whereas VO 2 did not change. The increase in VCO 2 appeared to be accounted for mainly by bacterial production of carbon dioxide and was significantly related to breath-hydrogen concentration (r = 0.56, P < 0.02 for healthy subjects ; r = 0.59, P < 0.01 for SBS subjects). A breath-hydrogen test should be performed in conjunction with indirect calorimetry to determine whether colonic fermentation is taking place and, if so, to correct appropriately the VCO 2 value in calorimetric xequations.

The outcome and prognosis of home parenteral nutrition (HPN) patients may depend on their age and underlying disease and on HPN techniques. Results in the literature are confounded by mixing child and adult populations, short- and long-term HPN treatments and malignant and non-malignant cases. Only four papers have reported the probability of survival and only one of these used multivariate analysis. The quality of life of HPN patients, focusing on community and social rehabilitation, has been reported. The aspects of treatment differ depending upon the circumstances under which HPN is started (acute vs chronic) and the nature of the disease, facts which have to be taken into account in patient management. Prognosis of the underlying disease obviously has a major influence on how rehabilitation is viewed. Patients should be informed of the objectives of HPN by the nutrition team and family circumstances taken into account, before time-consuming and expensive HPN treatment is started.