Risk Categorization Predicts Disability in Pain-associated Functional Gastrointestinal Disorders After 6 Months.

INTRODUCTION: For a large portion of youth, pain-associated functional gastrointestinal disorders (FGIDs) are associated with significant impairment over time. Clinically feasible methods to categorize youth with FGIDs at greatest risk for persistent pain-related impairment have not yet been identified. METHODS: Measures of functional disability, pain intensity, and anxiety were collected on 99 patients with FGIDs (ages 8-18) during a visit to a pediatric gastroenterology office to assess for the presence of risk. Follow-up data were obtained on a subset of this sample (n = 64) after 6 months, either in person or via mail. The present study examined whether a greater number of risk factors at baseline predicted greater pain-related disability at follow-up. RESULTS: Patients were divided into 4 groups based on number of risk factors present at the initial assessment: 0 (18.2%), 1 (24.2%), 2 (26.3%), and 3 (31.3%). The presence of 2 or 3 risk factors significantly predicted greater disability at follow-up compared to those with 0 risk factors (R = 0.311) and those with just 1 risk factor (Cohen's d values of -1.07 and -1.44, respectively). DISCUSSION: A simple approach to risk categorization can identify youth with FGIDs who are most likely to report increased levels of pain-related impairment over time. These findings have important clinical implications that support the utility of a brief screening process during medical care to inform referral for targeted treatment approaches to FGIDs.

Concordant parent-child reports of anxiety predict impairment in youth with functional abdominal pain.

BACKGROUND: Functional abdominal pain (FAP) is associated with significant anxiety and impairment. Prior investigations of child anxiety in youth with FAP are generally limited by small sample sizes, based on child report, and use lengthy diagnostic tools. It is unknown whether a brief anxiety-screening tool is feasible, whether parent and child reports of anxiety are congruent, and whether parent and child agreement of child anxiety corresponds to increased impairment. The purpose of this investigation was to examine anxiety characteristics in youth with FAP using parent and child reports. Parent-child agreement of child anxiety symptoms was examined in relation to pain and disability. METHODS: One hundred patients with FAP (8-18 years of age) recruited from pediatric gastroenterology clinics completed measures of pain intensity (Numeric Rating Scale) and disability (Functional Disability Inventory). Patients and caregivers both completed a measure of child anxiety characteristics (Screen for Child Anxiety and Related Disorders). RESULTS: Clinically significant anxiety symptoms were more commonly reported by youth (54%) than their parents (30%). Panic/somatic symptoms, generalized anxiety, and separation anxiety were most commonly endorsed by patients, whereas generalized anxiety, separation anxiety, and school avoidance were most commonly reported by parents. The majority (65%) of parents and children agreed on the presence (26%) or absence (39%) of clinically significant anxiety. Parent-child agreement of clinically significant anxiety was related to increased impairment. CONCLUSIONS: A brief screening instrument of parent and child reports of anxiety can provide clinically relevant information for comprehensive treatment planning in children with FAP.

Multiple micronutrient deficiencies among patients with intestinal failure during and after transition to enteral nutrition.

OBJECTIVES: To determine the prevalence of deficiencies of specific micronutrients (iron, zinc, magnesium, phosphorus, selenium, copper, folate, and vitamins A, D, E, and B12) in children with intestinal failure (IF), and to identify risk factors associated with developing these deficiencies. STUDY DESIGN: This study was a retrospective review of prospectively collected data from 178 children with IF managed by the Intestinal Care Center of Cincinnati Children's Hospital Medical Center between August 1, 2007, and July 31, 2012. Transition to full enteral nutrition (FEN) was defined as the period during which the patient received between 20% and 100% of estimated required nutrition enterally. FEN was defined as the patient's ability to tolerate 100% estimated required nutrition enterally for >2 weeks. RESULTS: Necrotizing enterocolitis was the most common cause of IF (27.5%). Iron was the most common micronutrient deficiency identified both during (83.9%) and after (61%) successful transition to FEN, with a significant reduction in the percentage of patients with iron deficiency between these 2 periods (P = .003). Predictors of micronutrient deficiency after successful transition to FEN included birth weight (P = .03), weight percentile (P = .02), height percentile (P = .04), and duration of parenteral nutrition (PN) (P = .013). After multivariate adjustments, only duration of PN remained statistically significant (P = .03). CONCLUSION: Micronutrient deficiencies persist in patients with IF during and after transition to FEN. These data support the need for routine monitoring and supplementation of these patients, especially those on prolonged PN.

Importance of addressing anxiety in youth with functional abdominal pain: suggested guidelines for physicians.

Functional abdominal pain (FAP) is a common pediatric disorder associated with impairment in functioning that may persist for the long term. Anxiety is common in youth with FAP, and may be an important factor in predicting youth who are at greatest risk for increased impairment because of pain symptoms. In this article, we examine the relation between anxiety and impairment in youth with FAP. Furthermore, we explore various biopsychosocial factors (eg, neurobiological substrates, coping strategies, social factors) that may be implicated in the relation among FAP, anxiety, and increased impairment. Finally, we propose physician guidelines for screening and treatment of youth with FAP and co-occurring anxiety. Youth with FAP and co-occurring anxiety may benefit from cognitive-behavioral therapy in the context of multidisciplinary care.

Vitamin D deficiency and low bone mineral density in pediatric and young adult intestinal failure.

OBJECTIVES: The aim of the present study was to determine the prevalence and predisposing factors for vitamin D deficiency and low bone mineral density (BMD) in patients with intestinal failure (IF). METHODS: A retrospective review of patients with IF managed at the Cincinnati Children's Hospital Medical Center. IF was defined as history of parenteral nutrition (PN) >30 days. Vitamin D deficiency was defined as serum 25-hydroxyvitamin D (25 (OH) D) <20 ng/dL. Reduced BMD was defined using dual x-ray absorptiometry z score ≤-2. A binary logistic regression model was used to test for association of significant risk factors and the outcome variables after univariate analyses. RESULTS: One hundred and twenty-three patients with median age of 4 years (range 3-22 years) were evaluated. Forty-nine (39.8%) patients had at least a documented serum 25 (OH) D deficiency during the study interval, whereas 10 of 80 patients (12.5%) with dual x-ray absorptiometry scans completed had a low BMD z score. Age at study entry was associated with both 25 (OH) D deficiency (P = 0.01) and low BMD z score (P = 0.03). Exclusive PN at study entry was associated with reduced bone mass (P = 0.03). There was no significant association between vitamin D deficiency and low BMD z score (P = 0.31). CONCLUSIONS: The risk of 25 (OH) D deficiency and low BMD z score increases with age among patients with IF. Strategies for monitoring and preventing abnormal bone health in older children receiving exclusive PN need to be developed and evaluated.

Micronutrient deficiencies in pediatric and young adult intestinal transplant patients.

Intestinal transplant recipients are at risk of micronutrient deficiency due to the slow process of post-transplant adaptation. Another contributing factor is calcineurin inhibitor-induced renal tubular dysfunction. Patients are typically supplemented with micronutrients during PN; however, the risk of deficiency may persist even after a successful transition to FEN. The goal was to determine the prevalence of, and associated risk factors for, iron, zinc, magnesium, phosphorus, selenium, copper, folate, and vitamins A, D, E, and B12 deficiency in pediatric intestinal transplant recipients after successful transition to FEN. A retrospective review of prospectively collected data from children who underwent intestinal transplantation at Cincinnati Children's Hospital Medical Center was done. Deficiencies of various micronutrients were defined using the hospital reference values. Twenty-one intestinal transplant recipients, aged one to 23 yr, who were successfully transitioned to FEN were included in the study. The prevalence of micronutrient deficiency was 95.2%. The common deficient micronutrients were iron (94.7%) and magnesium (90.5%). Age ≤ 10 yr (p = 0.002) and tube feeding (p = 0.02) were significant risk factors for micronutrient deficiencies. Pediatric intestinal transplant recipients have a high risk of micronutrient and mineral deficiencies. These deficiencies were more common among younger patients and those who received jejunal feeding.

Medical update and potential advances in the treatment of pediatric intestinal failure.

Short bowel syndrome (SBS) and intestinal failure are chronic malabsorption disorders with considerable nutritional and growth consequences in children. Intestinal failure occurs when the functional gastrointestinal mass is reduced even if there is normal anatomical gastrointestinal length. A number of management strategies are often utilized to achieve successful intestinal rehabilitation and maintain adequate nutrition to avoid intestinal transplant. These strategies include minimizing the effect of parenteral associated liver disease, limiting catheter complications, and treating bacterial overgrowth in the remaining small intestine. In addition, there continues to be significant research interest in enhancing intestinal adaptation with targeted therapies. The purpose of this review is to discuss current perspectives and to highlight recent medical advances in novel investigational therapies.

Crohn's disease limited to the appendix: a case report in a pediatric patient.

In the original description of Crohn's disease, the appendix was not believed to be involved in the inflammatory process. Later on, case reports started to appear in publications demonstrating that the appendix could be involved in the inflammatory changes of Crohn's disease, and it could also be the primary or the sole manifestation of the disease. Being that appendectomies are one of the most common procedures performed by pediatric surgeons, the knowledge about this diagnosis, all be it rare, is important. Our aim was to report a case and discuss the results of our literature review in order to elucidate the probability of a pediatric patient subsequently developing full Crohn's disease and the follow up that is indicated in such patients. A 12-year-old male patient presented with a history of chronic abdominal pain (3-4 times per week) for 1 year, crampy in nature, localized in the left lower quadrant, and associated with diarrhea (2 episodes per day). There were no extraintestinal manifestations of Crohn's, such as arthralgia or uveitis. Important family history included two paternal uncles with ulcerative colitis both of whom currently have stomas. The only abnormal laboratory value in our patient was an elevated fecal calprotectin level. An esophagogastroduodenoscopy and colonoscopy were performed and found to be unremarkable except for the cecum where it appeared that an exudate was emanating from the appendiceal orifice. A magnetic resonance enterography was ordered and showed an enlarged enhancing appendix. An exploratory laparoscopy identified an appendix with macroscopic cobblestone or lymphoid reaction that histologically was consistent with Crohn's disease. It appears that the Crohn's appendix is more indolent than Crohn's disease of the ileum or colon, with a recurrence rate in the largest series of 8%. The interval time from diagnosis to recurrence varied from 1 to 48 months with an average of 19 months. Some authors debate the need of follow up at all in those patients, believing that the appendectomy alone is curative in the majority of patients. Others recommend follow up for up to 5 years.

Decreasing Door-to-Door Times for Infliximab Infusions in a Children's Hospital Observation Unit.

INTRODUCTION: Children with inflammatory bowel disease (IBD) often require infliximab infusions to manage their disease. Infusions administered in the hospital setting require the patient and their families to devote many hours away from home. Changing to a rapid infusion protocol has been shown in the literature to be safe and has the potential to decrease time spent in the hospital receiving infusions. METHODS: We describe stepwise changes made over a 4-month period to improve infliximab infusion efficiency and lessen the time spent in the hospital by IBD patients and their families. These changes included the implementation of a standardized order set, defaulting to rapid infusions for eligible patients, eliminating the post-infusion observation window, and improving the pharmacy's efficiency in preparing infusion medications. We utilized several established quality improvement tools, including a smart aim, key driver diagram, plan-do-study-act cycles, and statistical process control charts to measure these interventions. RESULTS: Within three months of starting, the average door-to-door time patients spent in the hospital decreased by 128 minutes (2 hours 8 minutes). This improvement amounts to 768 minutes (12 hours 48 minutes) per year of time returned for normal childhood activities outside of the hospital. There were no infusion reactions during the period monitored. CONCLUSIONS: Implementation of a rapid infliximab infusion protocol made an impressive impact on freed family time without sacrificing patient safety. The changes we implemented could be helpful to other centers interested in decreasing in-hospital time for patients with IBD and their families.

Beriberi disease in an 11-year-old girl with total colectomy.

Beriberi or vitamin B1 deficiency is a rare disease in the developed world and more common in developing countries due to poverty and malnutrition. It usually presents with neurological manifestations (dry beriberi) or cardiovascular signs (wet beriberi). We report a case of dry beriberi in an 11-year-old girl with total colectomy who presented to our hospital with ataxic gait and muscle weakness. Her symptoms started 1 week after she underwent cholecystectomy for her chronic abdominal pain. Nerve conduction study showed sensory demyelinating neuropathy and she had low levels of serum vitamin B1. Her condition improved significantly after taking vitamin B1 supplementation. To the best of our knowledge, this is the first case report of thiamine deficiency in a child with a total colectomy.

A Quality Improvement Initiative to Decrease Time to Antibiotics for Children with Intestinal Failure, Fever, and a Central Line.

INTRODUCTION: Pediatric intestinal failure (IF) patients experience significant morbidity, including sepsis related to central line-associated bloodstream infections. Adult studies of sepsis demonstrate an association between time to antibiotic administration (TTA) and mortality. To overcome challenges in treating pediatric IF patients in an emergency department (ED), we appropriated an existing, reliable system for febrile immunocompromised oncology/bone marrow transplant children. We describe the translation of this process to febrile IF patients in the ED and steps toward sustained improvement. METHODS: We formed a multidisciplinary team and used the Model for Improvement to define aims and identify key drivers. The goal was to use an existing improvement process to increase the percentage of patients with IF who receive antibiotics within 60 minutes of arrival to the ED from 46% to 90%. Key drivers included pre- and postarrival processes, staff and family engagement, and a preoccupation with failure. We performed Plan-Do-Study-Act cycles targeting family engagement, prearrival efficiency, and postarrival consistency. RESULTS: Two hundred seventy-six encounters involving febrile IF patients between November 2012 and March 2017 were evaluated. There was a sustained reduction in the median time from arrival to antibiotic administration (71-45 minutes). We decreased TTA to less than 60 minutes for 77% of febrile IF patients. CONCLUSIONS: The basic tenets of process improvement for 1 high-risk population can be translated to another high-risk population but must be adjusted for variability in characteristics.

Body mass index in children with newly diagnosed inflammatory bowel disease: observations from two multicenter North American inception cohorts.

OBJECTIVE: To conduct a systematic review of children with newly diagnosed inflammatory bowel disease (IBD) from 2 prospective inception cohorts to examine body mass index (BMI) status at presentation. STUDY DESIGN: Clinical, demographic, and BMI data were obtained from 783 patients with newly diagnosed IBD. National Health and Nutrition Examination Survey data for 2748 healthy children were used as a control. RESULTS: Most children with Crohn's disease and ulcerative colitis had a BMI in the normative range (5%-84%). Low BMI (<5%) was seen in 22% to 24% of children with Crohn's disease and 7% to 9% of children with ulcerative colitis. Ten percent of children with Crohn's disease and 20% to 30% of children with ulcerative colitis had a BMI at diagnosis consistent with overweight or risk for overweight. CONCLUSION: Children with IBD are affected by current population trends toward overweight. A significant subgroup of children with newly diagnosed IBD has a BMI categorized as overweight or at risk for overweight. Clinicians should be aware of possible IBD diagnosis in the presence increased BMI.

Health-related quality of life in the first year after a diagnosis of pediatric inflammatory bowel disease.

BACKGROUND AND AIMS: Assessment of health-related quality of life (HRQOL) is of increasing importance in the evaluation of new therapies for inflammatory bowel disease (IBD). Available data concerning HRQOL in pediatric patients are sparse and uniformly cross-sectional. The aim of this study was to describe HRQOL and influential factors in newly diagnosed pediatric patients with Crohn's disease and ulcerative colitis during the first 12 months after diagnosis. MATERIALS AND METHODS: Participants were drawn from a large, prospectively derived observational IBD registry of pediatric patients studied through 18 U.S. and Canadian centers. Patients who had completed a baseline IMPACT questionnaire and for whom there were 12 months of follow-up data available were included. In addition to description of cohort, factors that were believed to influence HLQOL were assessed during the course of the year from diagnosis. RESULTS: Two hundred eighteen children met inclusion criteria (77% Crohn's disease, 23 % ulcerative colitis, mean age 12.7 +/- 1.9 years). Mean total IMPACT score at baseline was 154, 181 at 6 months, and 191 at 1 year (possible range 0-238, with increasing scores representing better quality of life). Repeated measures analysis showed that age and disease severity significantly negatively affected the IMPACT scores during the course of the year. CONCLUSIONS: In this large prospective pediatric IBD cohort, significant improvement in HRQOL is noted during the year from diagnosis. Mean IMPACT scores varied significantly depending on the disease severity and also decreased with increasing age.

An unusual cause of postfundoplication vomiting.

This article demonstrates a previously unreported late occurring complication of fundoplication that leads to vomiting. A patient with cerebral palsy had the placement of a transesophageal suture, which, after ingestion of his family dogs' hair over time, created a significant-sized esophageal trichbezoar formed and caught around the suture. Upon removal of the bezoar via upper gastrointestinal endoscopy, the patient experienced immediate relief of his symptoms. This case should be considered in the appropriate clinical setting where the patient has demonstrated pica. An evaluation with contrast upper gastrointestinal studies proved to be critical in this case of postfundoplication vomiting.

Hepatitis B virus screening for internationally adopted children.

OBJECTIVES: The objectives of this study were to estimate the prevalence of hepatitis B virus protection, infection, and recovery among internationally adopted children and to examine the need for repeat testing 6 months after arrival in the United States. METHODS: From November 1999 through October 2006, 1282 international adoptees were screened for hepatitis B virus, and results were examined with regard to age, gender, and birth country. The prevalence of hepatitis B virus protection, infection, and recovery was determined. RESULTS: The prevalence of hepatitis B virus in internationally adopted children at our large international adoption center was 4%, including 1.1% with acute or chronic infection and 2.9% with resolved infection. Overall, 64% of internationally adopted children had evidence of hepatitis B virus immunization, with protective antibodies. We also report a case that highlights the need for repeat serological testing to detect hepatitis B virus infection or immunization in internationally adopted children who might have been infected or vaccinated just before adoption and thus not have serological evidence in initial testing. CONCLUSIONS: These data reinforce the American Academy of Pediatrics recommendations regarding hepatitis B virus screening and infection control measures for international adoptees.

Effect of early immunomodulator use in moderate to severe pediatric Crohn disease.

BACKGROUND: The immunomodulators (IMs) 6-mercaptopurine and azathioprine decrease corticosteroid dependence and maintain remission in Crohn's disease (CD). We describe IM use in newly diagnosed pediatric CD, comparing outcomes of "early" versus "late" initiation of therapy. METHODS: Data were obtained from pediatric CD patients enrolled in a prospective, multicenter observational study. Moderate/severe disease patients treated with IM were compared for outcomes of remission, corticosteroid use, infliximab therapy, hospitalizations, and CD-related surgery based on timing of initiation of IM therapy. RESULTS: In all, 247 children met the criteria (60% male, mean age 11.9 years); 199 were treated with IM within 1 year of diagnosis; 150 between 0-3 months (early), 49 between 3-12 months (late). Both groups showed a decrease in corticosteroid use by 12 months, at which time proportionately fewer early group patients had received corticosteroids in the preceding quarter (22%) than late groups patients (41%)(P = 0.013). The number of hospitalizations per patient was also noted to be significantly lower in the early group over the 2-year follow-up (P = 0.03). No difference was noted in the rates of remission, infliximab use over time, or surgery. CONCLUSIONS: 80% of children with newly diagnosed moderate to severe CD are treated with IM within 1 year. Early IM use is associated with reduced corticosteroid exposure and possibly fewer hospitalizations per patient.

Laboratory values for children with newly diagnosed inflammatory bowel disease.

OBJECTIVE: The goal was to determine how often common laboratory tests yield normal results at the time of diagnosis for children with inflammatory bowel disease. METHODS: Data were obtained from a registry of children with newly diagnosed inflammatory bowel disease who were enrolled prospectively in 18 US/Canadian centers. Laboratory values investigated included hemoglobin level, platelet count, albumin level, and erythrocyte sedimentation rate. Disease severity was categorized by physician global assessment. RESULTS: A total of 526 children (mean age: 11.6 years; 58% male; 392 with Crohn disease and 134 with ulcerative colitis) were studied. All 4 values were normal for 21% of patients with mild Crohn disease and 54% with mild ulcerative colitis. In contrast, only 3.8% of children with moderate/severe Crohn disease and 4.3% with moderate/severe ulcerative colitis had normal results for all 4 tests. The erythrocyte sedimentation rate was least likely to be normal; overall, 26% of patients with inflammatory bowel disease had a normal erythrocyte sedimentation rate, including 18% with moderate/severe disease. Hemoglobin levels were normal for 32%, platelet counts for 50%, and albumin levels for 60%. There was no clear association between Crohn disease location and either severity or number of normal laboratory values. In contrast, there were direct correlations between ulcerative colitis disease severity and both the extent of bowel inflammation and the number of abnormal laboratory tests. CONCLUSION: The presence of normal screening laboratory studies should not dissuade clinicians from considering a diagnosis of inflammatory bowel disease.

Use of wireless capsule endoscopy in the management of severe Henoch-Schonlein purpura.

Henoch-Schonlein purpura is a multisystem vasculitis that primarily affects children. Characteristic symptoms include purpura of the lower extremities and buttocks, abdominal pain, arthralgias, and hematuria. Gastrointestinal bleeding occurs in approximately 50% of children and, although often self-limiting, can be significant. Wireless capsule endoscopy has been found to be safe and effective for children over 10 years of age. It is now the preferred imaging modality for evaluating gastrointestinal bleeding from the small intestine. Here we report an unusual case of chronic Henoch-Schonlein purpura vasculitis, primarily affecting the gastrointestinal tract, in which wireless capsule endoscopy was helpful in evaluating and directing treatment options.