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This document is a reply to a letter regarding a review on chronic intestinal pseudo-obstruction (CIPO) in adult patients. The authors express gratitude for the message that more effective treatments require a better understanding of the underlying causes of the disease. They discuss some clinical presentations of mitochondrial disease and the association of CIPO with familial degenerative intestinal neuropathy. However, they disagree with the list of genes and diseases that should be added to the causes of CIPO, stating that further investigation is needed to establish their relevance. They also mention that certain conditions and diseases associated with CIPO require clarification regarding their causal relationship. The authors hope that increased availability of genetic evaluations and histologic examinations will help in selecting more effective treatments and clarifying the role of causal factors in the pathophysiology of CIPO. [Extracted from the article]

Our aim was to analyze the outcomes in children with short‐bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure–associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007–2018. We retrospectively reviewed charts of 135 patients with SBS‐PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2–4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty‐eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%–38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy‐three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of <100,000/μl(median 50,500/μl) and median CB of 21 mg/dl, seven achieved CB normalization and had improved platelet count. Overall survival was 97% (censored 96.3%). We demonstrate high transplant‐free survival and enteral autonomy rates among children with SBS‐IFALD relying on low‐dose soybean lipid emulsion. [ABSTRACT FROM AUTHOR]

The glucagon‐like peptide‐2 (GLP‐2) analogue teduglutide is a medical treatment option for patients with short‐bowel syndrome–associated chronic intestinal failure. Because studies in mice have shown that GLP‐2 analogues may promote the growth of colonic neoplasms, surveillance colonoscopies before and during teduglutide therapy were recommended. The occurrence of small‐intestinal neoplasms has not been reported so far, except for a recent report about de novo development of hamartomatous duodenal polyps. We report a case of de novo development of small‐intestinal premalignant adenomatous polyps in both bulbar duodenum and distal jejunum in a patient treated with teduglutide for 41 months. Therefore, additional endoscopic surveillance of the upper gastrointestinal tract may be advised during teduglutide therapy for early detection and removal of potential small‐bowel adenomas. [ABSTRACT FROM AUTHOR]

Background: Intestinal barrier plays an essential role in maintaining gastrointestinal health. This study aimed to explore the effects of a soluble mediator preparation derived from Lactobacillus rhamnosus Gorbach-Goldin (LGG) on intestinal barrier function in a rat model of short bowel syndrome (SBS).Methods: Six-week-old male Sprague-Dawley rats underwent 80% small-bowel resection (SBR) and then were supplemented with water (SBS), 5 × 108 colony-forming unit viable LGG (SBS+LGG), or the LGG soluble mediators (SBS+LSM) in an equivalent dose to LGG by intragastric gavage daily from day 2 throughout day 14 after operation. Rats that underwent bowel transection and reanastomosis were used as the sham group. Body weight, ileum histology, intestinal permeability and bacterial translocation, inflammatory cytokines, and tight junction protein expressions of ileum were evaluated.Results: Animals undergoing SBR showed higher intestinal permeability and decreased expression of tight junction proteins in the ileum than sham group. Both SBS+LGG and SBS+LSM groups had reduced bacterial translocation and intestinal permeability as compared with the SBS group, with lower levels of serum endotoxin and tumor necrotizing factor alpha in ileum tissues. Moreover, the SBS+LSM group showed better body weight gain, lower endotoxin and FD-40 levels, and higher expressions of claudin-1 and claudin-4 in ileum than the SBS+LGG group.Conclusion: Enteral supplementation of LSMs or viable LGG can ameliorate intestinal barrier disruption in a rat model of SBS. The LSM preparation not only mimicked biological effects of viable LGG but also was revealed to be more effective in reducing inflammation and supporting intestinal barrier function. [ABSTRACT FROM AUTHOR]

Objective: To review all cases of parenteral support (PS)-dependent patients with short bowel syndrome (SBS) treated with teduglutide (Gattex, Shire) and to evaluate its efficacy and adverse effects.Methods: This is a retrospective descriptive cohort of SBS patients treated with teduglutide. Demographics, bowel length, primary diagnosis, PS volume/duration, teduglutide dose, and side effects were collected prospectively.Results: Six SBS patients (4 females, 2 males) received teduglutide. Mean age was 46.3 years (range 26-71). SBS etiology was vascular (n = 3), multiple resections (n = 2), and strangulation (n = 1). Length of residual small bowel was between 30-120 cm. The bowel anatomy was colon present (n = 3) and stoma n = 3 (ileostomy, 2; colostomy, 1). PS duration was 1.5-14 years. Weekly PS volume was mean 7.7 liters/week (1-14). Number of PS days per week ranged 1-7 days. Mean duration of teduglutide therapy was 31 months (24-36). All patients achieved ≥20% reduction in PS weekly volume within 6 months. PS was weaned in all patients. Adverse effects included abdominal bloat/discomfort (n = 3), stoma enlargement (n = 3), bowel obstruction (n = 1), and congestive heart failure (n = 1).Conclusions: All PS-dependent SBS patients treated with teduglutide were weaned off PS. Patients with colon in continuity and lower PS weekly volume requirements were weaned off PS sooner than those with end-stomas and higher PS volume requirements. Teduglutide was well tolerated. Additional clinical studies of teduglutide in SBS patients with marginal PS requirements are needed. [ABSTRACT FROM AUTHOR]

Background: Intestinal failure is a chronic condition related to loss of bowel length and/or function, resulting in dependence on central venous catheters for fluids and nutrition. Catheter use can be associated with significant complications, including catheter-related bloodstream infections (CRBSIs), which can lead to loss of vascular access, advancing intestinal failure associated-liver disease and death. Our objective was to evaluate the effectiveness and safety of ethanol locks as compared with standard heparin locks in pediatric intestinal failure.Methods: Databases, including MEDLINE and EMBASE, were searched until March 2017. Titles and abstracts were reviewed independently and relevant articles reassessed by full-text review. The main outcome was the rate of CRBSIs, while secondary outcomes were catheter replacement and repair.Results: Nine observational studies were included. The mean difference in rate of CRBSIs was 6.27 per 1000 catheter days (95% CI, 4.89-7.66) favoring ethanol locks, with a 63% overall reduction in infection rate. The mean difference in catheter replacement rate (per 1000 catheter days) was 4.56 (95% Cl, 2.68-6.43) favoring ethanol locks. The overall effect on catheter repair rate (per 1000 catheter days) was -1.67 (95% CI, -2.30 to -1.05), indicating lower repair rate with heparin locks.Conclusion: Sufficient evidence was noted showing that ethanol locks reduced CRBSIs and catheter replacements. Our findings raise questions about the effect of the ethanol lock on catheter integrity based on the noted increase in repair rate. This requires further prospective evaluation and may support selective application of ethanol locks to patients with documented CRBSIs. [ABSTRACT FROM AUTHOR]

Background: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB).Design: This study enrolled IFALD children who received 6 months of FO. Blood samples were collected prior to FO, and after 2 weeks and 3 and 6 months of FO. The primary outcome was 3-month vs baseline biomarker concentrations.Results: At study initiation, the median patient age was 3 months (interquartile range, 3-17 months), and mean ± standard deviation DB was 5.6 ± 0.7 mg/dL (n = 14). Cholestasis reversed in 79% of subjects. Eicosapentaenoic and docosahexaenoic acid was greater than baseline (P < .001, all time points). Linoleic and arachidonic acid and sitosterol and stigmasterol were less than baseline (P < .05, all time points). Three- and 6-month interleukin-8 (IL-8) and total and conjugated bile acids were less than baseline (P < .05). Baseline IL-8 was correlated with baseline DB (r = 0.71, P < .01). Early changes in stigmasterol and IL-8 were correlated with later DB changes (r = 0.68 and 0.75, P < .05).Conclusion: Specific fat emulsion components may play a role in IFALD. Stigmasterol and IL-8 may predict FO treatment response. [ABSTRACT FROM AUTHOR]

Background: Minimal enteral nutrition (MEN) may induce a diet-dependent stimulation of gut adaptation following intestinal resection. Bovine colostrum is rich in growth factors, and we hypothesized that MEN with colostrum would stimulate intestinal adaptation, compared with formula, and would be well tolerated in patients with short bowel syndrome.Methods: In experiment 1, 3-day-old piglets with 50% distal small intestinal resection were fed parenteral nutrition (PN, n = 10) or PN plus MEN given as either colostrum (PN-COL, n = 5) or formula (PN-FORM, n = 9) for 7 days. Intestinal nutrient absorption and histomorphometry were performed. In experiment 2, tolerance and feasibility of colostrum supplementation were tested in a pilot study on 5 infants who had undergone intestinal resection, and they were compared with 5 resected infants who served as controls.Results: In experiment 1, relative wet-weight absorption and intestinal villus height were higher in PN-COL vs PN (53% vs 23% and 362 ± 13 vs 329 ± 7 µm, P < .05). Crypt depth and tissue protein synthesis were higher in PN-COL (233 ± 7 µm, 22%/d) and PN-FORM (262 ± 13 µm, 22%/d) vs PN (190 ± 4 µm, 9%/d, both P < .05). In experiment 2, enteral colostrum supplementation was well tolerated, and no infants developed clinical signs of cow's milk allergy.Conclusion: Minimal enteral nutrition feeding with bovine colostrum and formula induced similar intestinal adaptation after resection in piglets. Colostrum was well tolerated by newly resected infants, but the clinical indication for colostrum supplementation to infants subjected to intestinal resection remains to be determined. [ABSTRACT FROM AUTHOR]

Background: Teduglutide is a glucagon-like peptide 2 (GLP-2) analog that has been approved for the treatment of adult short bowel syndrome (SBS)-associated intestinal failure (IF; SBS-IF). Teduglutide increases villus height and crypt depth in the small bowel mucosa, promoting nutrition absorption and enteral independence from parenteral nutrition (PN). We aim to report our single-center experience with teduglutide in adult patients with SBS to provide real-world context to its use.Method: We conducted a retrospective analysis on patients managed within our tertiary-level intestinal rehabilitation program to identify patients with SBS-IF treated with teduglutide from 2009-2015. The current report includes all patients at our center who had any exposure to teduglutide, including those who received commercial drug after approval by the Food and Drug Administration (FDA) and outside the scope of clinical trials.Results: A total of 18 patients were treated with teduglutide. Eleven patients (61%) achieved complete enteral independence from PN and/or intravenous fluids (IV) at a median time of 10 months (range: 3-36 months). PN/IV volume requirement was reduced in all patients except two. Ten of the 11 patients (91%) who achieved enteral autonomy had colon. All patients off PN/IV required additional oral vitamins and electrolyte supplementations.Conclusion: Our preliminary experience is consistent with prior reports of successful partial or complete weaning from PN/IV with teduglutide treatment in adult patients with SBS. The presence of colon appears to be favorable in obtaining enteral independence from PN/IV, regardless of residual small bowel length. Patients on teduglutide may remain at high risk of micronutrient deficiencies. [ABSTRACT FROM AUTHOR]

Background and Aims: Intestinal failure (IF) is a serious and common complication of short bowel syndrome with patients depending on parenteral nutrition (PN) support. Effective nutrition management requires an accurate estimation of the patient's basal metabolic rate (BMR) to avoid underfeeding or overfeeding. However, indirect calorimetry, considered the gold standard for BMR assessment, is a time- and resource-consuming procedure. Consequently, several equations for prediction of BMR have been developed in different settings, but their accuracy in patients with IF are yet to be investigated. We evaluated the accuracy of predicted BMR in clinically stable patients with IF dependent on home parenteral nutrition (HPN).Methods: In total, 103 patients with IF were included. We used indirect calorimetry for assessment of BMR and calculated predicted BMR using different equations based on anthropometric and/or bioelectrical impedance parameters. The accuracy of predicted BMR was evaluated using Bland-Altman analysis with measured BMR as the gold standard.Results: The average measured BMR was 1272 ± 245 kcal/d. The most accurate estimations of BMR were obtained using the Harris-Benedict equation (mean bias, 14 kcal/d [ P = .28]; limits of agreement [LoA], -238 to 266 kcal/d) and the Johnstone equation (mean bias, -16 kcal/d [ P = .24]; LoA, -285 to 253 kcal/d). For both equations, 67% of patients had a predicted BMR from 90%-110% All other equations demonstrated a statistically and clinically significant difference between measured and predicted BMR.Conclusions: The Harris-Benedict and Johnstone equations reliably predict BMR in two-thirds of clinically stable patients with IF on HPN. [ABSTRACT FROM AUTHOR]

Background The aim of this study was to ascertain if prospective determination of specific gut hormones and growth factors could predict bowel adaptation in children with short bowel syndrome ( SBS). Methods We studied independence from parenteral nutrition ( PN) as the short-term result and discontinuation of enteral nutrition ( EN) as the long-term result from a retrospective chart review of seven patients with SBS, who were managed in the absence of growth retardation. The correlation between increased number of enteral feeds or enteral nutrients and fasting serum gastrin, glucagon-like peptide 2 ( GLP-2), citrulline, and D-amino acid oxidase ( DAO) activity was analyzed. Five patients were weaned from PN, and two from EN. Results Fasting serum gastrin was significantly higher and serum GLP-2 lower in the PN-dependent patients than in the patients weaned from EN. The upper limit of fasting serum gastrin for PN independence and for EN independence was 300 and 200 pg/ mL, respectively. The lower limit of fasting serum citrulline for PN independence was 15 μmol/L. The relationship between serum citrulline and DAO and the course of bowel adaptation, however, was poor. Conclusions Serum citrulline is a predictor of PN independence in children with SBS. Fasting serum gastrin and GLP-2 are indicators for adaptation of the residual intestine, but this was a small study and further larger prospective trials are required to confirm these results. [ABSTRACT FROM AUTHOR]

Background: In phase III clinical studies, treatment with teduglutide was associated with clinically meaningful reductions (≥20% from baseline) in parenteral support (PS; parenteral nutrition and/or intravenous fluids) requirements in adult patients with intestinal failure associated with short bowel syndrome (SBS-IF). This analysis reports clinical characteristics of patients who achieved complete independence from PS during teduglutide treatment.Materials and Methods: Post hoc analysis of adult patients who achieved complete PS independence during treatment with teduglutide 0.05 mg/kg/d. Data were pooled from 5 teduglutide clinical trials (2 phase III placebo-controlled trials [NCT00081458 and NCT00798967] and their respective extension studies [NCT00172185, NCT00930644, NCT01560403]). Descriptive statistics were used; no between-group comparisons were performed because of the small sample size and lack of comparator.Results: Of 134 patients, 16 gained oral or enteral autonomy after a median of 5 years of PS dependence and 89 weeks of teduglutide treatment. Demographic and baseline disease characteristics varied among patients (median age, 55 years; 50% men; median baseline PS volume, 5.1 L/wk; median residual small intestine length, 52.5 cm). Most patients who achieved PS independence had colon-in-continuity; however, there was no significant difference in the frequency of PS independence among patients who maintained colon-in-continuity vs those who did not.Conclusion: Findings from this post hoc analysis suggest that oral or enteral autonomy is possible for some patients with SBS-IF who are treated with teduglutide, regardless of baseline characteristics and despite long-term PS dependence. [ABSTRACT FROM AUTHOR]

Background and Aims: A glucagon-like peptide 2 (GLP-2) analogue is approved for adults with intestinal failure, but no studies of GLP-2 have included children. This study examined the pharmacokinetics, safety, and nutritional effects of GLP-2 in children with intestinal failure.Methods: Native human GLP-2(1-33) was synthesized following good manufacturing practices. In an open-label trial, with parental consent, 7 parenteral nutrition-dependent pediatric patients were treated with subcutaneous GLP-2 (20 µg/kg/d) for 3 days (phase 1) and, if tolerated, continued for 42 days (phase 2). Nutritional treatment was directed by the primary caregivers. Patients were followed to 1 year.Results: Seven patients were enrolled (age: 4.0 ± 0.8 years; bowel length, mean ± SEM: 24% ± 4% of predicted). All were parenteral nutrition dependent since birth, receiving 44% ± 5% of calories by parenteral nutrition. GLP-2 treatment had no effect on vital signs (blood pressure, heart rate, and temperature) and caused no significant adverse events. Peak GLP-2 levels were 380 pM (day 3) and 295 pM (day 42), with no change in half-life or endogenous GLP-2 levels. Nutritional indices showed a numeric improvement in z scores and citrulline levels; the z score was maintained while citrulline levels returned to baseline once GLP-2 was discontinued.Conclusions: GLP-2 was well tolerated in children, with a pharmacokinetic profile similar to that of adults. There were no changes in endogenous GLP-2 release or metabolism. These results suggest that GLP-2 ligands may be safely used in pediatric patients; larger trials are suggested to investigate nutritional effects. [ABSTRACT FROM AUTHOR]

Background: There is a lack of large database research relating to the epidemiology and health resource utilization associated with short bowel syndrome (SBS) in the United States.Methods: We analyzed the Kids' Inpatient Database for the year 2012 and utilized International Classification of Diseases, Ninth Revision, and Clinical Modification ( ICD-9-CM) diagnosis codes to identify patients 0-3 years of age with SBS, who were matched by age and sex to children without SBS. The study variables included patient and hospital demographics, All Patient Refined Diagnosis Related Groups, in-hospital mortality, hospital length of stay, and hospitalization costs. We also determined the most frequent ICD-9-CM diagnostic and procedural codes associated with SBS.Results: Children with SBS demonstrated a higher rate of mortality than that of children without SBS (1.6% vs 0.7%; P < .001). Children with SBS also experienced a longer length of stay (median days [interquartile range]: 8 [15] vs 2 [3]; P < .001) and higher hospital costs ($17,000 [$34,000] vs $3000 [$5000]; P < .001). The most frequent medical diagnoses associated with SBS were infection (62%), anemia (29%), and liver disease (17%). Children with SBS also demonstrated more severe illness as assessed by an All Patient Refined Diagnosis Related Group score of 3 or 4 (94.30% vs 16.20%; P < .001).Conclusions: Children hospitalized with SBS have a high severity of illness and experience complicated inpatient courses related to their disease. Our study represents the first use of national U.S. data to study the epidemiology and health resource utilization associated with SBS. [ABSTRACT FROM AUTHOR]

Background: Since 2005, the Canadian home parenteral nutrition (HPN) registry has collected data on patients' demography, outcomes, and HPN clinical practice. At annual meetings, Canadian HPN programs review and discuss results.Aim: To evaluate changes over time in patient demography, outcomes, and HPN clinical practice using the registry data.Methods: This retrospective study evaluated 369 patients who were prospectively entered in the registry. Two periods were compared for the first data entry: 2005-2008 (n = 182) and 2011-2014 (n = 187). Patient demography, indications for HPN, HPN regimen, nutrition assessment, vascular access, and number of line sepsis per 1000 catheter days were evaluated.Results: For 2011-2014 compared with 2005-2008, indications for HPN changed significantly, with an increased proportion of patients with cancer (37.9% vs 16.7%) and with fewer cases of short bowel syndrome (32% vs 65.5%); line sepsis rate decreased from 1.58 to 0.97 per 1000 catheter days; and the use of tunneled catheters decreased from 64.3% to 38.0% and was no longer the most frequently chosen vascular access method. In contrast, the proportion of peripherally inserted central catheters increased from 21.6% to 52.9%. In addition, there was a reduction in number and days of hospitalizations related to HPN, and favorable changes were noted in the prescription of energy, proteins, and trace elements.Conclusion: The Canadian HPN registry is useful in tracking trends in demography, outcomes, and clinical practice. Results suggest a shift in patient demography and line access with improvement in line sepsis, hospitalizations, and HPN prescriptions. [ABSTRACT FROM AUTHOR]

Background: Teduglutide, a glucagon-like peptide-2 (GLP-2) analogue, is available for long-term use by parenteral nutrition (PN)-dependent adults to promote intestinal adaptation but is not approved for use in pediatric patients. The objective of this study was to assess teduglutide-stimulated induced intestinal adaptation, potential synergies with partial enteral nutrition (PEN), and distinct temporal markers of adaptation in a neonatal piglet model of short bowel syndrome (SBS).Materials and Methods: Neonatal piglets (48 hours old; n = 72) underwent an 80% jejunoileal resection and were randomized to 1 of 4 treatment groups, in a 2 × 2 factorial design, with PN or PEN (80% standard PN/20% standard enteral nutrition) and teduglutide (0.1 mg/kg/d) or control. Piglets received nutrient infusions for 4 hours, 48 hours, or 7 days.Results: Teduglutide improved ( P < .05) mucosal surface area (villus height: duodenum, jejunum, ileum; crypt depth: ileum, colon; proliferation: duodenum, jejunum, ileum; colon; apoptosis: jejunum, ileum, colon) and acute nutrient processing capacity (glucose: duodenum, jejunum, ileum; glutamine: duodenum, jejunum). These effects were complemented and synergistically enhanced by PEN in both site and timing of action. Structural adaptations preceded functional adaptations, but crypt depth remained a strong indicator of adaptation, regardless of time.Conclusions: The combination of teduglutide and PEN enhances intestinal adaptation beyond that of either therapy alone. [ABSTRACT FROM AUTHOR]

Background/aims: The objective of this study was to describe a clinically well-defined, single-center, intestinal failure (IF) cohort based on a template of definitions and classifications endorsed by the European Society for Clinical Nutrition and Metabolism (ESPEN).Methods: A cross-sectional, retrospective, adult IF cohort, receiving parenteral support (PS), was extracted from the Copenhagen IF database at the tertiary IF center, Copenhagen University Hospital, Rigshospitalet, Denmark.Results: Rigshospitalet provided PS to 188 adult patients with IF on December 31, 2011. Six patients received only fluids and electrolytes, while 97% required parenteral energy (17 ± 12 kcal/kg/d). Although 92% of the cohort had undergone intestinal resection, only 53% were classified as patients with short bowel syndrome (SBS) according to the pathophysiological classification. In the remaining cohort, patients were distributed as 5% with intestinal fistula, 12% with intestinal dysmotility, 5% with mechanical obstruction, and 14% with mucosal diseases. Twelve percent had a combination of pathophysiological causes. The patients with SBS (n = 100) were subdivided according to bowel anatomy into group 1 (jejuno/ileostomy, n = 82), group 2 (jejuno-colonic-anastomosis, n = 16), and group 3 (jejuno-ileo-colonic-anastomosis, n = 2). When evaluating the cohort requirements for PS using the ESPEN chronic IF classification based on the need for fluid volume and energy, 53% of the patients with IF were distributed in the maximum categories.Conclusion: The orphan condition of IF with its large patient heterogeneity mandates establishment of uniform definitions and a harmonization of classifications. As illustrated, the ESPEN-endorsed definitions and classifications are well designed and may serve as a common uniform template to facilitate both intra- and intercenter comparisons between reference centers and thus outcome results. [ABSTRACT FROM AUTHOR]

Aim: We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowel in extensive necrotizing enterocolitis (NEC). Methods: Extensive NECs managed surgically in a tertiary centre in 2006-2009 were retrospectively studied to include patients planned for an SLL. End points were bowel salvage rate and survival outcomes. Results were median (ranges), and statistical significance was P < 0.05. Main results: In 4 years, 34 NECs required a laparotomy, and 9 extensive NECs who required an SLL were included. The gestation at birth was 27 (24-38) weeks, birth weight was 1120 (580-2835) g, and first laparotomy performed on day 34 (2-77) of life, with SLL performed 2 (1-3) days after initial laparotomy. Commonest indications for SLL were doubtful bowel viability and physiological instability. 3 died before SLL. Patients who survived to have an SLL ( n = 6) had remaining small bowel length of 41 (25-70) cm, overall small bowel salvage rate 51 % (0-100 %), and 30-day survival 5/6 (83 %). Four patients survived for 1 year, their length of NICU stay was 114 (76-120) postoperative days, time on PN was 84 postoperative days (71 days-17 months), including one patient with short bowel syndrome who achieved enteral autonomy at 17 months; one late mortality had short bowel syndrome after further bowel resection for bowel obstruction, developed intestinal failure associated liver disease, and died before 1 year of life following liver transplant. Conclusion: SLL is a viable approach for extensive NEC. It offered bowel salvage rate of 51 % and long-term PN-free survival of 44 %, in the patient group who would have had significant risk of mortality and major morbidity. [ABSTRACT FROM AUTHOR]

The aim of this study was to assess the effect of ursodeoxycholic acid (UDCA) on the morphological and functional adaptive response of the jejunal remnant after massive intestinal resection in a cat model of short bowel syndrome (SBS). UDCA was administered to animals at a daily oral dose of 15 mg/kg for 6 weeks following a 85% jejunoileal resection. Resection alone caused extensive hyperplasia of jejunal mucosa, as evidenced by a significant increase in the weight of jejunal mucosa per unit length as well as by significant increases in DNA and protein concentration but no change in the protein/DNA ratio. Morphometric analysis using microscopy revealed no changes in jejunal mucosa thickness, jejunal crypt depth, villus height and villus surface area, although villus thickness was increased. The specific activities of jejunal sucrase and alkaline phosphatase were unaffected. UDCA treatment of resected animals, using doses that caused no toxicity, as evidenced by the absence of serum biochemistry abnormalities and histopathology, did not induce, compared to resection alone, any changes in mucosal cellularity and did not affect villus morphometry. On the other hand, UDCA administration increased crypt depth and, also, induced a profound increase in the specific activity of sucrase. UDCA improved diarrhoea, a core SBS symptom, reflected in a considerably reduced frequency of defaecation and improved form and texture of faeces. It is concluded that UDCA administration may enhance the natural adaptive response of the intestinal remnant following massive jejunoileal resection and may, thus, be beneficial in SBS treatment. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 21-year-old primiparous woman who had been treated for short bowel syndrome was referred to the present center for perinatal management at 33 weeks' gestation. Topics include the Vitamin K deficiency bleeding (VKDB) is a devastating morbidity in newborns, the universal vitamin K (VK) prophylaxis is recommended to prevent VKDB, and the mechanism of VK transportation from mother to fetus is unclear, but fat-soluble vitamins.

No abstract is available for this article. [ABSTRACT FROM AUTHOR]