Systematic review: MRI enterography for assessment of small bowel involvement in paediatric Crohn's disease.

BACKGROUND: Barium meal enteroclysis (BM) is the recommended imaging technique for small bowel inaccessible by ileo-colonoscopy when diagnosing paediatric-onset inflammatory bowel disease, but it has poor sensitivity and involves ionising radiation. MRI enterography (MRE) is an alternative methodology. AIMS: To critically appraise the published evidence on MRE in the assessment of Paediatric inflammatory bowel disease by systematic review. METHODS: Review of all English language data reporting MRE for the investigation of patients <18 years with known or suspected IBD. Primary searches of Medline (Jan 1950-April 2012), Cinahl (1966-April 2012) and Pubmed (Jan 1950-April 2012) were performed using keyword and MeSH terms; IBD; Magnetic resonance imaging; small bowel imaging; EMBASE was then searched. Two authors independently assessed the quality of studies using the quality assessment of diagnostic accuracy studies tool. RESULTS: Searches yielded 930 035 hits, combination word searches limited to 1983 titles. Fifty-two studies were fully reviewed, 41 were excluded due to lack of paediatric data. Eleven studies of 496 children were included. All studies used endoscopy as the reference test. 10/496 patients required jejunal intubation for bowel preparation. Meta-analysis of six comparable studies gave a pooled sensitivity and specificity for MRE detection of active terminal ileal Crohn's disease of 84% and 97% respectively. Studies displayed heterogeneity in bowel preparation, scanning technique, reporting methodology and timing of ileo-colonoscopy in relation to MRE. In three studies comparing BM, MRE had greater sensitivity and specificity. CONCLUSIONS: MRE is a sensitive and specific tool for diagnosis in paediatric inflammatory bowel disease. Technical considerations require refinement and standardisation; however, MRE has no radiation. Current data suggest that MRE should supersede BM as the SB imaging technique in centres with appropriate expertise.

Clinical progress in the two years following a course of exclusive enteral nutrition in 109 paediatric patients with Crohn's disease.

BACKGROUND: Exclusive enteral nutrition (EEN) is an effective first line treatment for active paediatric Crohn's disease (CD). AIM: To examine the effect of EEN on short- and long-term clinical outcome together with anthropometric measurements. METHODS: Retrospective case-note review in newly diagnosed CD (<16 years) who completed 8 weeks of EEN. Demographics, anthropometry, disease characteristics and inflammatory markers were collected at EEN initiation and at 1, 2, 6, 12 & 24 months post treatment initiation. EEN response was determined by a patient global assessment. RESULTS: One hundred and nine patients were included (Males 68; Median age: 11.2 years). After 8 weeks EEN, 65 were in remission, 32 improved and 12 had no improvement. By 4 weeks, mean weight/BMI z-score (s.d.) increased (P < 0.02) and between 4 and 8 weeks (P < 0.05). Baseline inflammatory markers all improved significantly by week 4 (albumin, CRP and platelets; all P < 0.01) and ESR (P < 0.00001). 63/109(58%) relapsed during follow-up. 44/63(70%) patients completed a second course of EEN with similar response rate, but lower weight gain (3.3 vs. 5.1 kg, P < 0.05). Height z-score did not change significantly over the 24 months. Introduction of azathioprine within 6 months of diagnosis did not improve height outcomes at 24 months. CONCLUSIONS: Weight and BMI z-score improved with EEN and changes are sustained to 2 years, but height z-score did not. Seventy per cent of patients who relapsed during 2-year follow-up managed a 2nd course of EEN. The optimal therapeutic strategies for length of EEN course and to improve linear growth are awaited.

Rationalized assessment of prolonged jaundice is safe and cost-effective.

Prolonged jaundice (PJ) in healthy term neonates is common and frequently benign. It can, however, be the earliest manifestation of underlying liver disease. Its management requires a balanced approach, avoiding over-investigation of well babies while ensuring the early identification of those with pathology. Currently marked heterogeneity exists in the assessment of PJ. Over a two-year period we prospectively audited the management of PJ in two Level 3 neonatal units prior to and after the introduction of a rationalized investigation algorithm in keeping with the recently published British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) guidelines (i.e. clinical examination and stool inspection combined with measurement of split bilirubin). In this study we reviewed initial practice and then evaluated the impact of our change in practice. A total of 197 babies, 1.5% of live births, were referred with PJ. Of these, 105 babies were included in the first part of the study and 92 babies were included in the second part. No pathology relating to PJ, such as infection, hepatitis or liver disease, was identified. Following the introduction of our rationalized algorithm, we demonstrated a statistically significant reduction in the number of return appointments (28 versus 7; P < 0.0009) and repeat investigations (37 versus 7; P < 0.0001). This represented a saving of £1575-2625 per year in laboratory costs alone. Contemporaneously, three infants presented with biliary atresia, none of whom were identified by PJ screening and all of whom were over seven weeks old at diagnosis. A rationalized approach to the assessment of PJ reduces workload and is cost-effective; however, the limitations of selective screening, irrespective of how streamlined it is, remain--if babies are not identified and referred, they cannot be screened. Population-based methodologies offer an alternative approach to the identification of cholestatic liver disease and are worthy of further consideration.

Cycled enteral antibiotics reduce sepsis rates in paediatric patients on long-term parenteral nutrition for intestinal failure.

BACKGROUND: Long-term parenteral nutrition has transformed the prognosis for children suffering from intestinal failure. However, parenteral nutrition itself is associated with considerable morbidity and mortality including that caused by sepsis. AIM: To examine a strategy of cycled enteral antibiotics in reducing the incidence of sepsis in paediatric intestinal failure patients. METHODS: Retrospective analysis of the incidence of sepsis rates of patients on long-term parenteral nutrition, at a tertiary paediatric hospital. Patients were separated into those who received cycled enteral antibiotics and a control group. Sepsis rates before and during cycled enteral antibiotics were compared with comparable timeframes between the cycled enteral antibiotics and control groups. Central venous catheter removal rates were also compared. RESULTS: Fifteen patients (eight cycled enteral antibiotics, & seven controls) received 9512 parenteral nutrition days, with a total of 132 sepsis episodes. All eight patients of the treatment group demonstrated a decrease in the frequency of episodes of sepsis following the introduction of cycled enteral antibiotics. The cycled enteral antibiotics group had a significant reduction in infection rate during the treatment period (from 2.14 to 1.06 per 100 parenteral nutrition days, P = 0.014: median effect size -1.04 CI 95%-1.93, -0.22), whereas the controls had no significant change (1.91 - 2.36 per 100 parenteral nutrition days P = 0.402: median effect size 0.92 CI 95%-1.96, 4.17). The central venous catheter survival rates increased in the cycled enteral antibiotics group from 0.44 central venous catheter removals per 100 parenteral nutrition days to 0.27 central venous catheter removals per 100 parenteral nutrition days, although this was not statistically significant. CONCLUSIONS: Cycled enteral antibiotics significantly reduced the rate of sepsis in a small group of paediatric intestinal failure patients. Larger well-designed prospective studies are warranted to further explore this finding.

Systematic review: medical and nutritional interventions for the management of intestinal failure and its resultant complications in children.

BACKGROUND: Intestinal failure (IF) affects a growing number of children due to increasing numbers of preterm infants surviving intestinal resection for necrotising enterocolitis and improving surgical techniques for congenital gut anomalies. Parenteral nutrition (PN) is the mainstay of therapy; enteral nutrition may have trophic effects on the gut. AIM: To review systematically evidence for the effectiveness of medical and nutritional interventions in the treatment of IF in children. METHODS: Retrieval of data from studies of patients aged <18 years and receiving >28 days of PN. Outcome measures were improvement in intestinal function, intestinal adaptation, growth, prevention and treatment of IF-associated liver disease, and mortality. Cochrane Database (November 2009), MEDLINE (1950-November 2009) and CINAHL (1982-November 2009) electronic database searches were made using keyword and subject headings (MeSH): IF, Short Bowel Syndrome (SBS), PN and Child. The level of the evidence (EL) was assessed using SIGN (Scottish Intercollegiate Guidelines Network) methodology (http://www.sign.ac.uk). RESULTS: From 1 607 620 hits, 720 abstracts were reviewed. Thirty-three original articles were included. No studies were of high methodological quality. CONCLUSIONS: The evidence base for medical and nutritional interventions in paediatric IF is limited and of poor quality. In the absence of randomised-controlled trials, this evidence base can improve through case control and cohort research; and with better multiagency communication, the study of inter-centre differences is possible. Achievable short-term goals would include the study of: optimal ursodeoxycholic usage, novel intralipid formulations, cycled enteral antibiotics, enteral probiotics and new enteral feeding strategies.

Cerebral thromboembolic events in pediatric patients with inflammatory bowel disease.

BACKGROUND: There is a recognized association between pediatric inflammatory bowel disease (IBD) and cerebral thromboembolic events (CTEs). Historical reporting had described the association as strongest between ulcerative colitis (UC), rather than Crohn's disease (CD). We describe the incidence and outcome of CTE in pediatric IBD patients from a single center over 5 years and the relative proportion of stroke reported in the literature in patients with UC and CD before and after January 2000. METHODS: Demographic data were extracted on all newly diagnosed cases of IBD in our center from January 2003 to January 2008 to ascertain patient characteristics, disease type, risk factors for CTE, modality of neuroimaging, and outcome. A literature search was performed to identify all articles describing stroke in pediatric IBD. All identified studies were stratified into those published before and after January 1 2000. RESULTS: In all, 154 new patients diagnosed with IBD (male 56%) (UC 30%, CD 64%, IBD unclassified [IBDU] 6%) were reviewed. Four cases of CTE occurred in our population over 5 years (2.6%). All patients had a risk factor for CTE. Fifteen case series were identified with 32 patients. There was a significant increase in the proportion strokes affecting patients with CD reported after January 2000 (P = 0.02). CONCLUSIONS: CTE affects a proportion of pediatric IBD patients. Although resolution of physical impairment is the norm, significant morbidity exists. Our study suggests a secular trend toward CTE in CD. Primary prevention with the identification and amelioration of identifiable risk factors should be the clinical objective in future studies.

Regionally acquired intestinal failure data suggest an underestimate in national service requirements.

UNLABELLED: OBJECTIVES, SETTING AND PATIENTS: With complete case referral for prolonged parenteral nutrition (PN) beyond term equivalent, serving a stable population of 1.25 million people, we describe the long-term outcome and survival of patients referred to an intestinal failure (IF) nutrition support team over the first 8 years of existence at a regional paediatric centre, and extrapolate to potential numbers of national home parenteral nutrition (HPN) cases and intestinal transplantation data. DESIGN AND OUTCOME MEASURES: Retrospective analysis detailing patient demographics, interventions, use of HPN, occurrence of intestinal failure-associated liver disease (IFALD), and outcomes of enteral adaptation, survival, and referral for and receipt of organ transplantation. RESULTS: 23 patients were referred over 8 years, 20 being PN dependent within the neonatal period. Diagnoses included short bowel syndrome (SBS) (18), neuromuscular abnormalities (4) and congenital enterocyte disorder (1). 12 696 days of PN were delivered with 314 confirmed episodes of sepsis at a median of 12 episodes per patient. 144 central venous catheters (CVCs) were required at a median of four per patient. IFALD occurred in 17 (73%) patients, with 10 (44%) referred for transplant assessment. Thirteen (56%) children received HPN. Overall mortality was 44%. A significant predictor for survival in the SBS group was residual bowel >40 cm (82% vs 28%, p = 0.049). CONCLUSIONS: Survival for IF at 56% was lower than reported from non-UK supra-regional centres, and nationally collected data, possibly reflecting pre-selected referral populations. Data from regional centres with complete ascertainment may be important both when counselling parents and when planning regional and national HPN and IF specialist services.

Successful implementation of a nurse-led teaching programme to independently administer subcutaneous methotrexate in the community setting to children with Crohn's disease.

BACKGROUND: Methotrexate is increasingly used as a third line immunosuppression agent in children with Crohn's disease (CD). Methotrexate is traditionally administered in the hospital setting. AIM: To set up a nurse-led education programme to teach children/their parents to administer subcutaneous methotrexate in the community. METHODS: All patients were given methotrexate over a 16-month period. Patient demographics including previous treatments were collected. A competency based teaching package was implemented by the inflammatory bowel disease nurse. Distances and travel times together with costings were calculated. RESULTS: Thirty two patients (19 male; 13 female) with a median treatment age of 11.96 years (IQR 10.67-13.92) were studied. Thirty of 32 (17 children, 13 parents) were independently administering methotrexate. The median return journey distance to hospital was 23 miles (IQR 14.4-42.4) taking a median time of 52 min (IQR 41.0-73.5) for each injection. The total patient travel saving was £10,537 (average £730 per patient) and nursing time saving was £12,808 with home administration (total saving £23,345). CONCLUSIONS: This paediatric study demonstrates that methotrexate injections can be given successfully in the majority (94%) of patients with CD independently in the community, resulting in significant time and money savings for patients and health professionals alike.

Pulmonary hypertension--a new manifestation of mitochondrial disease.

Mitochondrial respiratory chain (RC) abnormalities in children can present as multiorgan disease, including liver failure, usually within the first year of life. Cardiorespiratory complications have previously been described in association with RC defects; however, to our knowledge no cases of pulmonary hypertension have been described. We discuss two patients with proven mitochondrial RC liver disease who developed severe pulmonary hypertension, one subsequent to cadaveric orthotopic liver transplantation, the second in the neonatal period. It is our contention that pulmonary hypertension should now be included as another potential manifestation of paediatric mitochondrial disease.

Teaching life-long learning skills in a fourth-year medical curriculum.

For more than twenty years, the Ruth Lilly Medical Library has been a traditional part of the Indiana University School of Medicine curriculum. Recently, following changes to the curriculum, the Library's role has evolved to include responsibility for developing and teaching a Medical Informatics rotation as part of the senior year clerkships. Heavy emphasis is placed on acquiring life-long learning skills, especially on locating and critically appraising the best clinical evidence in the medical literature. In its first four months, the rotation has been quite favorably received by both students and faculty, but will continue changing to keep pace with future curriculum alterations and new technology.