Standardized one-day evaluation before urinary reconstructive surgery for neurogenic lower urinary tract dysfunction: Feasibility and impact on surgical strategy and care pathway.

OBJECTIVES: To describe a concept of standardized preoperative one-day evaluation before urinary reconstructive or diversion surgery for the treatment of neurogenic lower urinary tract (LUT) dysfunction, and to evaluate its feasibility and its impact on the care pathway. MATERIALS AND METHODS: All patients who underwent a one-day standardized evaluation before a urinary reconstructive or derivation surgery for the treatment of neurogenic LUT dysfunction between January 2017 and December 2021 in our institution were included. Data were collected retrospectively from standardized reports. The main outcome was the rate of completion of the tests and consultations planned during this evaluation. Secondary outcomes included the findings from the one-day evaluation and changes in the urological surgical strategy at different time points within one year. RESULTS: One hundred and thirty-one patients benefited from this one-day standardized evaluation. The overall completeness rate of the data collected was 77.5%, increasing from 62.3% in 2017 to 89.3% in 2021. The urological surgical plan was modified for 19.1% of patients following this preoperative evaluation. The indication was then confirmed for 114 patients (87.0%) by the multidisciplinary meeting and was carried out unchanged during the following year for 89 patients (67.9%). An associated colostomy procedure was proposed for 18.3% of patients and was finally performed in 11.5%. CONCLUSION: A standardized multidisciplinary preoperative one-day evaluation before performing reconstructive or diversion surgery for the treatment of neurogenic LUT dysfunction seems feasible and makes it possible to optimize the surgical plan and adapt the course of care.

Six-month outcomes of teduglutide treatment in adult patients with short bowel syndrome with chronic intestinal failure: A real-world French observational cohort study.

BACKGROUND & AIMS: Teduglutide, a GLP-2-analog, has proven effective in two placebo-controlled studies in reducing parenteral support (PS) in patients with short bowel syndrome-associated intestinal failure (SBS-IF) after 24 weeks. The aim of this study was to describe in a real-life situation the effects of teduglutide treatment and their predictive factors. METHODS: We included 54 consecutive SBS-IF patients treated with teduglutide in France for at least 6 months from 10 expert centers. Small bowel length was 62 ± 6 cm and 65% had colon in continuity. PS was 4.4 ±0 .2 infusions per week, started 9.8 ± 1.2 years before. Response (PS reduction ≥ 20%) and PS discontinuation rates were assessed at week 24. Adjusted p values of factors associated with response and weaning were calculated using a multivariate logistic regression model. RESULTS: At week 24, 85% of patients were responders and 24% had been weaned off PS, with a 51% reduction of PS needs and 1.5 ± 0.2 days off PS per week. Response to teduglutide was influenced by a higher baseline oral intake (p = 0.02). Weaning off PS was influenced by the presence of colon (p = 0.04), a lower PS volume (p = 0.03) and a higher oral intake (p = 0.01). There were no differences based on age, bowel length or SBS-IF causes. CONCLUSIONS: Our study confirms the effectiveness of teduglutide in reducing PS needs in SBS-IF patients. We associated reduced parenteral support volume with baseline parenteral volume support, bowel anatomy, and oral intake. These findings underline the role of nutritional optimization when starting the treatment.

Compliance to oral nutritional supplementation decreases the risk of hospitalisation in malnourished older adults without extra health care cost: Prospective observational cohort study.

BACKGROUND & AIMS: Malnutrition affects 5-10% of elderly people living in the community. A few studies suggest that nutritional intervention may reduce health care costs. The present study included malnourished elderly patients living at home. It aimed to compare health care costs between patients that were prescribed ONS by their general practitioner and those who were not, and to assess the effect of ONS prescription on the risk of hospitalisation. METHODS: This prospective multicentre observational study included malnourished patients ≥70 years old who lived at home. Patients were defined as malnourished if they presented with one or more of the following criteria: weight loss ≥5% in 1 month, weight loss ≥10% in 6 months, BMI <21 kg/m2, albuminemia <35 g/L or Short-Form MNA ≤ 7. Their general practitioners prescribed an ONS, or not, according to their usual practice. Health care costs were recorded during a 6-month period. Other collected data were diseases, disability, self-perception of current health status, quality of life (QoL), nutritional status, appetite and compliance to ONS. A propensity score method was used to compare costs and risk of hospitalisation to adjust for potential confounding factors and control for selection bias. RESULTS: We analysed 191 patients. At baseline, the 133 patients (70%) who were prescribed ONS were more disabled (p < 0.001) and had poorer perception of their health (p = 0.02), lower QoL (p = 0.04) and lower appetite (p < 0.001) than the 58 patients (30%) who were not prescribed ONS. At 6 months, appetite had improved more in the ONS prescription group (p = 0.001). Weight change was not different between groups. Patients prescribed ONS were more frequently hospitalised (OR 2.518, 95% CI: [1.088; 5.829] hosp; p = 0.03). Analyses of adjusted populations revealed no differences in health care costs between groups. In the ONS prescription group, we identified that health care costs were lower (p = 0.042) in patients with an energy intake from ONS ≥ 500 kcal/d (1389 ± 264 €) vs. < 500 kcal/d (3502 ± 839 €). The risk of hospitalisation was reduced 3 and 5 times when the intake from ONS was ≥30 g of protein/day or ≥500 kcal/d, respectively. CONCLUSIONS: ONS prescription in malnourished elderly patients generated no extra heath care cost. High energy and protein intake from ONS was associated with a reduced risk of hospitalisation and health care costs.

Safety and effectiveness of levodopa-carbidopa intestinal gel for advanced Parkinson's disease: A large single-center study.

BACKGROUND: Treatment with levodopa-carbidopa intestinal gel (LCIG) can effectively relieve motor and non-motor symptoms in advanced Parkinson's disease (PD). However, adverse events (AEs) are frequent. OBJECTIVE: To describe AEs associated with LCIG treatment and the main reasons for treatment discontinuation. We also looked for factors that were potentially predictive of serious AEs and assessed the effectiveness of and satisfaction with LCIG. METHOD: We retrospectively analyzed data on AEs in patients treated with LCIG at a French university medical center. For patients still receiving treatment at last follow-up, effectiveness was assessed according to the Clinical Global Impression (CGI) scale and the Movement Disorders Society - Unified Parkinson's Disease Rating Scale motor score. RESULTS: Of the 63 patients treated with LCIG for a mean (range) of 19 months (8-47), 57 (90%) experienced at least one AE (340 AEs in total). Most of the AEs (in 69.8% of the patients) were related to percutaneous endoscopic gastrostomy with a jejunal tube (PEG-J) or affected the gastrointestinal tract (granuloma, leakage, or a local infection). Device-related AEs (such as PEG-J removal and device occlusion) were frequent (in 63.5% of patients). Forty-three patients (68%) required at least one additional endoscopic procedure. Dopatherapy-related AEs occurred in 30 patients (48%). Most of the AEs occurred long after treatment initiations, and only a small proportion led to discontinuation. On the CGI scale, 53 patients (84.4%) considered that their condition had improved during LCIG treatment. CONCLUSION: Despite the high frequency of AEs, patients with advanced PD gain clinical benefit from treatment with LCIG. This treatment requires a competent, multidisciplinary team on site.

Better early outcome with enteral rather than parenteral nutrition in children undergoing MAC allo-SCT.

There is no consensus on the type of nutritional support to introduce in children undergoing allogeneic stem cell transplantation (allo-SCT) after myeloablative conditioning (MAC). This retrospective, multicenter, observational study compared the early administration of enteral nutrition (EN group, n = 97) versus parenteral nutrition (PN group, n = 97) in such patients with matching for important covariates. The primary endpoint was the study of day 100 overall mortality. The early outcome at day 100 was better in EN group regarding mortality rate (1% vs. 13%; p = 0.0127), non relapse mortality (1% vs. 7%; p = 0.066), acute GVHD grades II-IV (37% vs. 54%; p = 0.0127), III-IV (18% vs. 34%; p = 0.0333) and its gut localization (16% vs. 32%; p = 0.0136). Platelet engraftment was better in EN group than in PN group for the threshold of 20 G/L (97% vs. 80% p < 0.0001) and 50 G/L (92% vs. 78%, p < 0.0001). The length of stay was shorter in EN group (28 vs. 52 days, p < 0.0001). There were no differences between the two groups regarding the polynuclear neutrophil engraftment, infection rate or mucositis occurrence. These results suggest that, in children undergoing MAC allo-SCT, PN should be reserved to the only cases when up-front EN is insufficient or impossible to perform.

Counter-intuitive plasma vitamin D and zinc status in HIV-1-infected adults with persistent low-level viraemia after treatment initiation: a pilot case-control study.

Determinants of persistent low-level viraemia [PLLV, a viral load (VL) of between 50 and 500 copies/mL] have not been elucidated. In a case-control study, we evaluated the influence of micronutrients on PLLV in a population of 454 HIV-1 adults having initiated antiretroviral therapy (ART) between January 2007 and December 2011. Plasma levels of retinol (vitamin A), 25-OH vitamin D2 + D3, vitamin E and zinc were measured at ART initiation in cases (PLLV after 6 months of ART) and in controls (VL <50 copies/mL after 6 months). Cases and controls were matched for the CD4 cell count (±50/mm3) and ethnic origin. Intergroup differences in demographic, biological and treatment parameters and sunshine intensity at ART initiation were adjusted using a propensity score. A receiver operating characteristic (ROC) curve was used to assess intergroup differences in plasma micronutrient levels. Thirty-three of the 454 patients (7.3%) displayed PLLV (median VL: 92 copies/mL). Patients were predominantly male (89%), Caucasian (64%) and CDC stage C (25%). The median age was 38 years, the median initial VL was 5.2 log10 copies/mL and the median CD4 count was 74/mm3. The 22 cases and matched controls were balanced in these respects, and had similar vitamin A/E levels. Two cases (9%) and 9 controls (41%) had a vitamin D level <10.3 ng/mL (p = 0.0015), and 2 cases (9%) and 10 controls (48%) had a zinc level <74.6 µg/dL (p = 0.04). Our results support in vitro studies suggesting that vitamin D favours HIV-1 replication and that HIV-1 is zinc-dependent. Wide-scale, prospective studies are required.

[Mitochondrial Neuro-Gastro-Intestinal Encephalopathy (MNGIE): When and how to suspect it in front of an atypical anorexia nervosa?] / Encéphalopathie mitochondriale neuro-gastro-intestinale (MNGIE) : quand et comment l'évoquer devant une anorexie mentale atypique ?

INTRODUCTION: The Mitochondrial Neurogastrointestinal Encephalopathy (MNGIE) disease is an extremely underrated syndrome beginning around the age of eighteen years. Because of its severity, this diagnosis should be considered when a patient presents an atypical anorexia nervosa. MNGIE disease is inherited in an autosomal recessive manner and related to mutations of the TYMP gene (ch22q13.32-qter), encoding the thymidine phosphorylase. The MNGIE is often misdiagnosed and is associated with a time to diagnostic of about 12 years after first symptoms. Thus this critical review aims to help clinicians better identify symptoms and paraclinical markers of the MNGIE as a differential diagnosis of atypical anorexia nervosa. METHODS: A literature search was performed using PubMed and Google Scholar databases. RESULTS: The clinical diagnosis of the MNGIE disease should be based on the association of severe loss of weight and some additional symptoms: (1) severe gastrointestinal dysmotility (nausea, vomiting, intestinal pseudo-obstruction), (2) ptosis or external ophtalmoplegia and (3) peripheral sensorimotor neuropathy. When MNGIE disease is clinically suspected, paraclinical testing can help to validate the MNGIE diagnostic: (1) Arterial blood test reveals lactic acidemia (e.g. an increased serum concentration of lactate without pH modifications), and (2) Brain MRI indicates leukoencephalopathy, usually asymptomatic. Direct evidence of MNGIE disease is based on specific testing of: (1) the thymidine phopshorylase enzyme activity in leukocytes is less than 10% of the control, (2) the increase of plasmatic thymidine (>3µmol/L) and the increase of plamatic deoxyuridine (>5µmol/L), (3) the evidence of mutations of the TYMP gene by molecular genetic testing. CONCLUSION: The MNGIE disease is a severe trouble with multisystemic complications. The thymidine phopshorylase enzyme activity in leukocytes should be measured as soon as possible when a patient presents atypical anorexia nervosa.

Conservative management of severe caustic injuries during acute phase leads to superior long-term nutritional and quality of life (QoL) outcome.

PURPOSE: Best clinical management of severe caustic injury is still a subject of debate. Most surgical teams consider severe caustic injury as an indication for emergency surgery. But, under certain circumstances, conservative management is feasible, avoids the need for gastrectomy, and has a low mortality rate. Postponed reconstructive surgery is usually performed several months after caustic ingestion to restore intestinal continuity or to treat stricture sequelae. This study aimed to investigate long-term nutritional and quality of life (QoL) outcomes after reconstructive surgery for high-grade esophageal and gastric caustic injury. METHODS: Twenty-one patients were assessed for biological and nutritional parameters, physical findings, and quality of life surveys at least 25 months after reconstructive surgery among patients who have required emergency surgery (n = 10) and patients who did not undergo emergency surgery (n = 11). RESULTS: After median follow-up of 74 months (after caustic ingestion) and 67 months (after reconstructive surgery), patients who avoided emergency surgery experienced faster oral diet resumption (49 vs. 157 days, p = 0.004), less weight loss (0.3 vs. 20 kg, p = 0.002), greater body fat percentage (22 vs. 18 %, p = 0.046), better nutritional and physical status (SF12 PCS 51 vs. 43, p = 0.036), and less trouble eating (EORTC QLQ-OG 25 31 vs. 56, p = 0.01). CONCLUSIONS: Our study is the first to evaluate long-term nutritional and QoL outcomes, following severe caustic injury. It demonstrates better long-term nutritional outcomes and QoL in patients suitable for initial conservative management. These results support full functional recuperation of spared organs, even severely burned. In the absence of clinical or biological signs necessitating immediate surgery, conservative management should be advocated.

[Esophageal atresia]. / Devenir à moyen et long terme des enfants atteints d'atrésie de l'œsophage.

Most of the children operated for esophageal atresia will survive the neonatal period. However, medium-term and late complications are frequent in this population. Gastroesophageal reflux disease is observed in 26 to 75% of the cases and can be responsible for peptic esophagitis, anastomotic stenosis, and Barrett esophagus, which is a risk factor for adenocarcinoma of the esophagus. Dysphagia is frequently observed, sometimes several years after the surgery, affecting up to 45% of children at the age of 5 years. Growth retardation is present in nearly one-third of children at the age of 5 years. Ear, nose, and throat and respiratory complications are also very frequent but tend to improve with time. Tracheomalacia is found in 75% of these children at birth, sometimes responsible for severe complications (malaise, bradycardia). Respiratory symptoms are dominated by chronic cough, wheezing, and infections reported in 29% of the children by the age of 5 years. Restrictive, obstructive syndromes and bronchial hyperactivity can be observed, but usually remain moderate. All these complications can influence the patient's quality of life, which is moderately impaired compared to healthy controls. The high frequency of late sequelae in esophageal atresia justifies regular and multidisciplinary follow-up through adulthood.

Factors associated with recurrence of catheter-related bloodstream infections in home parenteral nutrition patients.

Blood cultures from outpatients receiving home parenteral nutrition (HPN) via long-term central venous access (CVA) were retrospectively analyzed from January 2003 to May 2009. When infection of the CVA was not due to Staphylococcus aureus, Pseudomonas aeruginosa, or Candida, catheter salvage was attempted for a maximum of three consecutive infections on the same CVA. Factors influencing the time-to-next-infection were studied, whether the catheter was changed after the last infection or not. Neither the McCabe score, age, history of cancer, diabetes mellitus nor immunosuppression, curative antibiotic lock, type of bacteria, type or duration of treatment had an influence on the time-to-next-infection. The time-to-next-infection was significantly associated with the status of CVA (saved or changed) and its type (tunneled catheter with or without a cuff, or implanted port catheter).

Enteral nutrition: a first option for nutritional support of children following allo-SCT?

Parenteral nutrition (PN) is the treatment of choice for nutritional support of patients undergoing allo-SCT following myeloablative conditioning (MAC). Here we prospectively assessed the outcomes of early enteral nutrition (EN) in a paediatric cohort. From 2003 to 2010, all 65 consecutive children undergoing MAC allo-SCT at our referral centre began EN the day after transplantation. Post-transplant and nutritional outcomes of patients receiving only EN (EN group, n=50) were compared with those of patients requiring additional PN (EN-PN group, n=15). In the EN group time to platelet recovery (P=0.01) and length of hospitalisation (P<0.001) were shorter, while in the EN-PN group the proportion of unrelated donors (P=0.02) and the frequency of severe acute GVHD (aGVHD; P=0.004) were higher. All patients were alive at day 100. PN was started 14 days after transplant because of poor digestive tolerance to EN or severe gut aGVHD. The body mass index Z-score in the EN-PN group decreased from transplant to discharge (P=0.02). In only 23% of cases was PN required for severely ill patients. Our results suggest that EN might be considered to be an option for nutritional support in children undergoing MAC allo-SCT, while PN should be used only as a rescue option, possibly in combination with EN.

Crohn's disease patients with chronic intestinal failure receiving long-term parenteral nutrition: a cross-national adult study.

BACKGROUND: Chronic intestinal failure (CIF) is a very rare Crohn's disease (CD) complication. AIM: To determine incidence of CIF treated with home parenteral nutrition (HPN) in adult CD patients and to isolate factors associated with severe CIF. METHODS: This retrospective multicentre study included 38 patients with CD-related CIF treated with HPN for at least 12 months in French HPN centres. Severe CIF was defined by a length of remnant small bowel of less than 100 cm or CIF occurrence within the 15 years following CD diagnosis. RESULTS: Median delay between CD diagnosis and CIF was 15 years. CIF incidence did not decrease over time (1.4/year before 1995 vs. 2.2/year after). Median number of small bowel resections per patient was three (range 1-8). Median small bowel resection, remnant and initial lengths were 160, 80 and 260 cm, respectively. Twenty-four per cent of patients developed stenosis within 1 year after CD diagnosis and 76% developed perforative complications within 2 years. In multivariate analysis, severe CIF, defined as CIF onset <15 years after CD diagnosis, was associated with a more recent CD diagnosis (odds ratio, 0.785; 95% confidence interval, 0.623-0.989). CIF occurred despite frequent use of immunosuppressants. Course of CD remained severe during HPN: immunosuppressants prescription occurred in 11 patients, surgery in six. Six patients died from CD (n = 2), HPN complications (n = 2) or other causes (n = 2). CONCLUSIONS: Chronic intestinal failure requiring HPN is rare during CD. Incidence remained stable over time. Surgical procedures play a minor role in the occurrence of severe chronic intestinal failure compared to CD severity.

Pre-operative management is associated with low rate of post-operative morbidity in penetrating Crohn's disease.

BACKGROUND: Ileocaecal resection for penetrating Crohn's disease is still challenging with a high rate of post-operative morbidity and faecal diversion. AIM: To report retrospectively the results of pre-operative management for penetrating Crohn's disease focusing on the rate of post-operative major morbidities and need for faecal diversion. METHODS: Between 1997 and 2007, 78 patients with penetrating Crohn's disease underwent a first ileocaecal resection after a pre-operative management consisting in bowel rest, nutritional therapy, intravenous antibiotics, weaning off steroids and immunosuppressors, and drainage of abscesses when appropriate. RESULTS: Resection was performed for terminal ileitis associated with (n = 41), abscesses (n = 37) or both (n = 5). A pre-operative nutritional therapy was performed in 50 patients (68%) for 23 days (range, 7-69 days) along with a weaning off steroids and immunosuppressors. A diverting stoma was performed for six patients (7.7%). There was no post-operative death. Post-operative complications were classified as minor in 10 patients (12.8%), and major in four patients (5%). Overall, the post-operative course was uneventful in 58 patients (74%). CONCLUSION: Pre-operative management for penetrating Crohn's disease allowed ileocaecal resection with low rates of post-operative morbidity and faecal diversion.

Efficacy and tolerance of gastrostomy feeding in Duchenne muscular dystrophy.

Undernutrition occurs often in individuals with Duchenne muscular dystrophy (DMD). Between 1997 and 2007, a gastrostomy was placed in 25 patients with DMD (median: 23 years old; range, 11-38 years) for weight loss (n=22) and/or swallowing disorders (n=13). We evaluated nutritional status using the weight-for-age (W/A) ratio, comparing the values to the reference curve for DMD patients. During the first 9 months, nutritional status improved: the W/A ratio increased and reached a plateau. The W/A ratio was 69% (range, 45-128%) at the start and increased to 87% (range, 49-164%) at the maximal follow-up of 22 months (P<0.001). However, the W/A ratio did not reach the median value for age. Complications occurred in 21 patients (84%), but caused no mortality. Our data suggest that gastrostomy is well tolerated by, and effective for improving the nutritional status of, individuals with DMD.

[Continuous dopaminergic stimulation by Duodopa in advanced Parkinson's disease: Efficacy and safety]. / Intérêts de la stimulation dopaminergique continue par Duodopa((R)) dans la maladie de Parkinson évoluée : efficacité et tolérance.

INTRODUCTION: When advanced Parkinson's disease (PD) patients experience motor complications (fluctuations and dyskinesias) despite standard oral treatment, two treatment options are available: deep brain stimulation and subcutaneous apomorphine infusion with respects of indications for each strategy. Continuous intraduodenal infusion of levodopa (Duodopa) via a gastrojejunal tube may be proposed at this stage of the disease and the study of indications and clinical results with Duodopa may develop this new therapeutic alternative. PATIENTS AND METHODS: Seven patients with advanced PD (dementia for all and psychiatric disorders for some of them, axial signs) were treated with Duodopa. We evaluated neuropsychological functions, all UPDRS scales, gait and quality-of-life just before Duodopa onset and six months after treatment end. Moreover, we described all adverse events (early and late) and studied daily levodopa doses before and 6 months after treatment. RESULTS: We demonstrated an improvement in motor UPDRS (44%), in axial signs (40% for UPDRS part III axial subscore and 12% for gait) and a reduction of fluctuations (37.5%) and in UPDRS part IV dyskinesia (20%). These significant results are observed without any change in the quality-of-life. Adverse events were due to PEG positioning for four patients, the equipment (pump, connection, inner tube) for all patients and levodopa for four patients. Daily levodopa dose had to be increased 13.5%. CONCLUSION: Duodopa can be considered as a new treatment strategy providing significant improvements in motor fluctuations, dyskinesia and severe axial signs. These results were demonstrated in very advanced PD patients, who had been excluded from previous studies, with cognitive disorders and for some of them dopaminergic psychosis well controlled by medications.

Laparoscopic gastric bypass complicated by portal venous thrombosis and severe neurological complications.

The laparoscopic Roux-en-Y gastric bypass (LRYGBP) is increasingly popular for the treatment of morbid obesity, although its postsurgical complications are often underestimated. We report the case of a 22-year-old morbidly obese woman who underwent a LRYGBP, which was rapidly complicated by portal venous thrombosis and severe neurological complications due to vitamin deficiencies. She presented rapid body weight loss with optic and peripheral neuropathy. Clinical chemistry results showed low transthyretin and micronutrient levels. Intravenous micronutrient infusion and cyclic nocturnal enteral tube feeding were needed to slowly improve gait and visual acuity. We then discuss (1) factors that could have contributed to the nutritional deficiencies and (2) the preventive management of these types of nutritional complications. Gastric bypass procedures can cause multivitamin deficiencies. In the case presented here, complications occurred very soon after surgery. The increasing incidence of obesity and bariatric surgery warrants better patient education concerning strict adherence to vitamin supplementation.

[Perioperative evolution of the nutritional status in head and neck surgical patients. Prospective and descriptive case series]. / Evolution périopératoire du statut nutritionnel en chirurgie carcinologique des voies aérodigestives supérieures. Etude prospective et descriptive.

AIM OF THE STUDY: To assess the perioperative evolution of the nutritional status of head and neck surgical patients. DESIGN: Prospective, descriptive case series. PATIENTS AND METHODS: Fifty-four patients candidates for total or partial laryngectomy for malignancy of the neck tract without a past of neck surgery. The nutritional status of all patients hospitalized for total pharyngolaryngectomy, total or partial laryngectomy was assessed by 1) clinical parameters including weight (W), weight variation (WV, percentage of loss), Body Mass Index (BMI), triceps skin fold measurement (T), midarm circumference (M), and 2) biological parameters such as serum albumin (SA), transthyretin (TTR), lymphocytes (Ly). These parameters were noted at the time of diagnostic laryngoscopy (T1), the day before surgery (T2), and 10 days afterward (T3) when patients were authorized to eat normally. All patients had enteral nutrition (EN) support (35 kcal/kg/day) starting at D1 and for 10 days afterward. Only complete data per patient were analyzed. RESULTS: 24 patients were excluded. During the 21 days [7-53] preoperative period (T1-T2), WV was (6.6% [-8,1-+20.0] [T2] vs. 4.7% [-12,9-+20.0] [T1], p<0.05) without difference in T and M. In the postoperative period (T2-T3), all parameters worsened, except T, with: WV (8.2% [-8,1-+20.0] [T3], p<0.05 vs T2), M (27.4 cm [20.0-37.0] [T3] vs 28.3 cm [20.5-39.0] [T2], p<0.05) et TTR (0.21 mg/l [0.09-0.36] [T3] vs. 0.27 mg/l [0.08-0.45] [T2], p<0.05). BMI was 22.9 [15.2-36.7] (T1) vs 22.9 [15.2-35.3] (T2), NS and 22.1 [15.0-34.9] (T3), p<0.05 vs (T2). CONCLUSION: The nutritional status in malignancy head and neck surgical patients seems to be best assessed by loss weight. It worsened mainly during the postoperative period even if a well-conducted EN was performed as defined by the French consensus conference.

Thymidine phosphorylase gene mutations in patients with mitochondrial neurogastrointestinal encephalomyopathy syndrome.

The mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) syndrome is characterized by the association of gastrointestinal and neurological symptoms. It is a rare autosomal recessive mitochondrial disorder with multiple mitochondrial DNA deletions and/or depletion. It is caused by thymidine phosphorylase (TP) gene mutations resulting in a complete abolition of TP activity. We tested 31 unrelated patients presenting either with a complete MNGIE syndrome (8 patients), a severe intestinal pseudo-obstruction (10 patients), and multiple deletions and/or depletion of mitochondrial DNA (13 patients). All the tested patients presenting with a complete MNGIE had increased thymidine levels in plasma and urine, and no TP activity. The group with pseudo-obstruction syndrome had normal or partial reduction of TP activity. We found pathogenic mutations on TP gene only in the MNGIE syndrome group: all the MNGIE patients were compound heterozygous or homozygous for mutations in the TP gene. Eight of these mutations are yet unreported, confirming the lack of genotype/phenotype correlation in this syndrome. Enzymatic activity and thymidine level are thus rapid diagnosis tests to detect MNGIE affected patients prior to genetic testing for patients with gastrointestinal symptoms.