The Role of Liver Biopsy in Investigation of Cholestatic Liver Disease in Infancy.

OBJECTIVES: The objective of this study was to assess the diagnostic yield and impact on management of liver biopsy in infants with cholestatic jaundice. METHODS: A retrospective cohort study of infants with cholestasis who underwent liver biopsy before one year of age between December 2002 and December 2013 at the Montreal Children's Hospital was conducted. Biopsies were reviewed by a single pathologist. The diagnostic yield of the biopsy was assessed in terms of its role in establishing a diagnosis, excluding an important diagnosis or changing management. RESULTS: Seventy-nine biopsies were performed within the time frame outlined, with 58 fulfilling inclusion criteria. Liver biopsies were found to add novel information in 21 cases (36.2%). The diagnostic yield of the biopsy was unrelated to the severity of direct hyperbilirubinemia, age at biopsy, age at admission, co-morbidities, stool color at presentation and TPN exposure. Among infants under 90 days of age, 21 also underwent cholangiography, the results of which were consistent with biopsy findings. There were four (6.9%) documented complications from biopsies, including bleeding and accumulation of free fluid in the peri-hepatic area. CONCLUSIONS: Liver biopsy is an invasive test used with other clinical modalities to determine the etiology of neonatal cholestasis. These results suggest that biopsy added novel information to the diagnostic workup in 36.2% of cases with a complication rate of 6.9%. Consequently, the role and timing of liver biopsy need to be reassessed to determine which patients would most benefit from this procedure.

Outcomes of Patients with Intestinal Failure after the Development and Implementation of a Multidisciplinary Team.

Aim. A multidisciplinary team was created in our institution to manage patients with intestinal failure (INFANT: INtestinal Failure Advanced Nutrition Team). We aimed to evaluate the impact of the implementation of the team on the outcomes of this patient population. Methods. Retrospective chart review of patients with intestinal failure over a 6-year period was performed. Outcomes of patients followed up by INFANT (2010-2012) were compared to a historical cohort (2007-2009). Results. Twenty-eight patients with intestinal failure were followed up by INFANT while the historical cohort was formed by 27 patients. There was no difference between the groups regarding remaining length of small and large bowel, presence of ICV, or number of infants who reached full enteral feeds. Patients followed up by INFANT took longer to attain full enteral feeds and had longer duration of PN, probably reflecting more complex cases. Overall mortality (14.8%/7.1%) was lower than other centers, probably illustrating our population of "early" intestinal failure patients. Conclusions. Our data demonstrates that the creation and implementation of a multidisciplinary program in a tertiary center without an intestinal and liver transplant program can lead to improvement in many aspects of their care.

Canadian Association of Gastroenterology consensus guidelines on safety and quality indicators in endoscopy.

BACKGROUND: Increasing use of gastrointestinal endoscopy, particularly for colorectal cancer screening, and increasing emphasis on health care quality, highlight the need for clearly defined, evidence-based processes to support quality improvement in endoscopy. OBJECTIVE: To identify processes and indicators of quality and safety relevant to high-quality endoscopy service delivery. METHODS: A multidisciplinary group of 35 voting participants developed recommendation statements and performance indicators. Systematic literature searches generated 50 initial statements that were revised iteratively following a modified Delphi approach using a web-based evaluation and voting tool. Statement development and evidence evaluation followed the AGREE (Appraisal of Guidelines, REsearch and Evaluation) and GRADE (Grading of Recommendations, Assessment, Development and Evaluation) guidelines. At the consensus conference, participants voted anonymously on all statements using a 6-point scale. Subsequent web-based voting evaluated recommendations for specific, individual quality indicators, safety indicators and mandatory endoscopy reporting fields. Consensus was defined a priori as agreement by 80% of participants. RESULTS: Consensus was reached on 23 recommendation statements addressing the following: ethics (statement 1: agreement 100%), facility standards and policies (statements 2 to 9: 90% to 100%), quality assurance (statements 10 to 13: 94% to 100%), training, education, competency and privileges (statements 14 to 19: 97% to 100%), endoscopy reporting standards (statements 20 and 21: 97% to 100%) and patient perceptions (statements 22 and 23: 100%). Additionally, 18 quality indicators (agreement 83% to 100%), 20 safety indicators (agreement 77% to 100%) and 23 recommended endoscopy-reporting elements (agreement 91% to 100%) were identified. DISCUSSION: The consensus process identified a clear need for high-quality clinical and outcomes research to support quality improvement in the delivery of endoscopy services. CONCLUSIONS: The guidelines support quality improvement in endoscopy by providing explicit recommendations on systematic monitoring, assessment and modification of endoscopy service delivery to yield benefits for all patients affected by the practice of gastrointestinal endoscopy.

Indicators of safety compromise in gastrointestinal endoscopy.

INTRODUCTION: The importance of quality indicators has become increasingly recognized in gastrointestinal endoscopy. Patient safety requires the identification and monitoring of occurrences associated with harm or the potential for harm. The identification of relevant indicators of safety compromise is, therefore, a critical element that is key to the effective implementation of endoscopy quality improvement programs. OBJECTIVE: To identify key indicators of safety compromise in gastrointestinal endoscopy. METHODS: The Canadian Association of Gastroenterology Safety and Quality Indicators in Endoscopy Consensus Group was formed to address issues of quality in endoscopy. A subcommittee was formed to identify key safety indicators. A systematic literature review was undertaken, and articles pertinent to safety in endoscopy were identified and reviewed. All complications and measures used to document safety were recorded. From this, a preliminary list of 16 indicators was compiled and presented to the 35-person consensus group during a three-day meeting. A revised list of 20 items was subsequently put to the consensus group for vote for inclusion on the final list of safety indicators. Items were retained only if the consensus group highly agreed on their importance. RESULTS: A total of 19 indicators of safety compromise were retained and grouped into the three following categories: medication-related - the need for CPR, use of reversal agents, hypoxia, hypotension, hypertension, sedation doses in patients older than 70 years of age, allergic reactions and laryngospasm/bronchospasm; procedure-related early - perforation, immediate postpolypectomy bleeding, need for hospital admission or transfer to emergency department from the gastroenterology unit, instrument impaction, severe persistent abdominal pain requiring evaluation proven to not be perforation; and procedure-related delayed - death within 30 days of procedure, 14-day unplanned hospitalization, 14-day unplanned contact with a health provider, gastrointestinal bleeding within 14 days of procedure, infection or symptomatic metabolic complications. CONCLUSIONS: The 19 indicators of safety compromise in endoscopy, identified by a rigorous, evidence-based consensus process, provide clear outcomes to be recorded by all facilities as part of their continuing quality improvement programs.

Zoledronic acid for the treatment of osteopenia in pediatric Crohn's disease.

BACKGROUND: Pediatric patients with Crohn's disease often have low bone mass (osteopenia) for age. No randomized, placebo-controlled trials using zoledronic acid have ever been performed in this population. The objective of this study was to assess the efficacy of zoledronic acid in children with Crohn's disease and osteopenia. METHODS: A double-blind, randomized, placebo-controlled design was used. Thirteen adolescents received either a single intravenous dose of zoledronic acid (0.066 mg/kg, max 4 mg, n= 7) or saline placebo (n= 6). The primary outcome was change in lumbar spine bone mineral density (LSBMD) z-score at 6 months. Secondary outcomes included bone markers and adverse events. RESULTS: At 6 months, the change in LSBMD z-score was significantly higher in the zoledronic acid group compared to placebo (0.7 vs 0.1, P < 0.001). Volumetrically adjusted LSBMD z-score also significantly increased in the treated group. This significant difference persisted until 12 months. With zoledronic acid, urinary C-telopeptide excretion decreased by 50% at 6 months and remained suppressed at 12 months (P= 0.02), but no changes were observed with placebo. Both groups had similar adverse events which included transient fever, arthralgias, and nausea (3/7 treated, 2/6 placebo, P= NS). CONCLUSIONS: In this study, zoledronic acid demonstrated a significant increase in LSBMD at 6 and 12 months following a well-tolerated infusion.

Foveolar hyperplasia secondary to cow's milk protein hypersensitivity presenting with clinical features of pyloric stenosis.

Pyloric stenosis is one of several causes of infantile nonbilious vomiting. A 7-week-old boy presenting with symptoms suggestive of this was found to have a prepyloric lobular mass causing near-complete gastric outlet obstruction. The histology showed localized hypertrophic gastropathy with hyperplasia and tortuosity of foveolar cells reminiscent of Menetrier changes. Postsurgical excision, the child continued vomiting until removal of bovine and soy proteins from his diet. The clinical presentation, pathology, and response to restrictive diet suggested cow's milk protein allergy as key. The literature describing infantile focal foveolar hyperplasia and Menetrier disease is reviewed, stressing the rarity of allergy as cause.

Pregnancy outcome of women who failed appointments at a teratogen information service clinic.

The effect of failed appointments at a teratogen information service (TIS) clinic on pregnancy outcome is not known. The objectives of our study were 1) to identify predictors of failed appointments and 2) to evaluate if failed appointments were associated with a higher rate of adverse pregnancy outcome among women booked for counseling at a TIS clinic over 5 consecutive months. Of 242 patients scheduled to come to clinic, 154 were followed-up at 20 weeks gestational age. Women who were pregnant at 20 weeks were more likely to have attended the clinic (81% attendance rate), than women who had a spontaneous abortion, a therapeutic abortion, or an ectopic pregnancy (54% attendance rate) (P = 0.003). Independent predictors of failed appointments included exposure to illicit drugs, history of previous spontaneous abortion, and a main concern that was a nonmedication exposure. In conclusion, pregnant women who failed appointments at a TIS clinic are at greater risk for not being pregnant at 20 weeks gestational age than those who attended the clinic. Telephone counseling should be considered in selected cases to ensure that all women get relevant information at the time of appointment booking.