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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.

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.

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.

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., (Copyright © 2013 Mosby, Inc. All rights reserved.)

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., (© 2013 John Wiley & Sons A/S.)

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.

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.

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.

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.

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.

Objective: To compare the efficacy of two commonly used solutions in the rehydration of infants with mild to moderate dehydration caused by acute diarrhea in the United States., Design and Setting: Double-blind, parallel-group, randomized study performed at Children's Hospital Medical Center., Patients: Sixty infant boys (< or = 2 years old), with mild (< or = 5%) or moderate (6 to 9%) dehydration caused by acute diarrhea of less than 1 week's duration were included in the study., Interventions: Infants were randomly assigned to receive treatment with either a glucose-based oral rehydration solution (ORS) (Pedialyte, Ross Laboratories, Columbus, OH) or a rice syrup solids-based ORS (Infalyte, Mead Johnson Nutritional Group, Evansville, IN). After rehydration was achieved, patients entered a maintenance phase during which, in addition to a maintenance ORS, breast milk or a soy-based formula was offered; infants older than 1 year were also given a lactose-free diet., Outcome Measures: Rehydration was judged clinically. Infants remained on a metabolic bed during the study in to separate and quantitate urine and stool output. Therefore, in addition to clinical outcome, we compared intake, output and apparent absorption and retention of fluid, sodium, and potassium between groups., Results: All patients were successfully rehydrated using an ORS without the use of intravenous fluids. No differences were detected between treatment groups in time to rehydration, percentage of weight gain after rehydration, consumption of ORS to achieve rehydration, or stool output. However, the apparent sodium absorption (net intake less fecal output) was greater in the Infalyte group than the Pedialyte group during the first 24 hours., Conclusion: The two maintenance oral electrolyte solutions (Pedialyte and Infalyte) most commonly used in the United States are effective as rehydration solutions for infants with mild to moderate dehydration. We speculate that a strategy for oral rehydration therapy in the United States, based on the use of a single solution during the rehydration and maintenance phase, might gain additional acceptance by practicing pediatricians and family physicians.

To determine the contribution of the colon in Escherichia coli heat-stable enterotoxin-mediated diarrheal disease, toxin binding, guanyl cyclase activation, and toxin-induced water flux in the rat colon and ileum were compared. Scatchard analysis suggested a single class of heat-stable enterotoxin receptors with an affinity constant of binding of 10(9) L/mol in both colonocytes and ileocytes; however, the number of toxin receptors per cell was 3.5-fold greater in coloncytes than ileocytes (8.32 +/- 1.33 x 10(5) vs. 2.33 +/- 0.28 x 10(5) receptors per cell; P = 0.02). Heat-stable enterotoxin stimulated guanyl cyclase activation in an identical dose-dependent manner in proximal colonic and ileal membranes, with similar sensitivity and maximum response. Heat-stable enterotoxin also inhibited net water flux to a similar degree in both colon and ileum (-47.8 vs. -48.4 microL.cm-1.h-1, respectively) at a dose of 8 nmol/L. At this dose in the colon, because of a higher baseline of absorption, absorption continued, but at a diminished level. At this dose in the ileum, heat-stable enterotoxin induced net secretion. These data are consistent with the concept that heat-stable enterotoxin-induced diarrheal disease results from a decreased absorptive capacity in the colon in the face of increased small intestinal fluid secretion.

Hypomagnesemia has been associated with multiple neurologic abnormalities, including irritability, seizures, paralysis, and choreiform movements. A patient with short gut syndrome who developed severe hypomagnesemia during an intercurrent diarrheal illness is reported. This patient had a transient neurologic syndrome consisting of hemiparesis, mental status changes, and aphasia which resolved as the magnesium levels returned to normal. Neurologic abnormalities of this child were largely secondary to severe hypomagnesemia and should be included in the list of neurologic abnormalities that can result from this metabolic derangement.

Pigs demonstrate an increased sensitivity and susceptibility to Escherichia coli heat-stable enterotoxin (STa) in the 1st wk of life and immediately after weaning. To determine the possible mechanisms for this increased susceptibility, we compared STa binding, guanylate cyclase activation, and photoaffinity cross-linking to porcine jejunal brush border membranes prepared from immature (less than or equal to 1 wk of age) versus adult pigs as well as 3-wk-old weaned versus unweaned pigs. The STa binding capacity of immature pigs was nearly twice that of adult pigs (11.73 +/- 1.52 versus 6.00 +/- 0.96 x 10(-11) mol/L, p less than 0.001), and the STa binding capacity of weaned pigs was nearly three times greater than that of unweaned pigs (17.48 +/- 2.10 versus 4.86 +/- 1.02 x 10(-11) mol/L, p less than 0.001). Scatchard analysis suggested a single class of STa receptor, with an association of binding constant of approximately 10(9) L/mol at all ages. Maximum guanylate cyclase response (expressed as pmol cyclic GMP generated/mg brush border membrane protein/min) was greater in immature versus adult pigs (1312 +/- 831 versus 320 +/- 92, p less than 0.02). Weaned pigs had a greater maximum guanylate cyclase activation than unweaned pigs (1126 +/- 692 versus 624 +/- 298); however, this difference was not statistically significant. Autoradiograms demonstrated specific cross-linking of 125I-STa to a number of distinct radiolabeled bands (62, 66, 84, 92, 160, and 165 kD). There was a difference in the size and trypsin sensitivity of these radiolabeled bands as a function of age and weaning. Treatment with trypsin decreased the intensity of the 160 to 165-kD bands while increasing the intensity of the 62- to 66- and 84- to 92-kD bands. These differences in STa binding, guanylate cyclase activation, and STa receptor size may increase the susceptibility of pigs during the 1st wk of life and at weaning to STa-mediated diarrheal disease.

We studied the incidence of hypophosphatemia in patients during the nutritional recovery syndrome. The charts of 150 patients receiving a complete nutritional assessment for 18 months were reviewed; 45 met established nutritional risk criteria. Only 9 of these 45 had serial phosphorus values measured during nutritional repletion, and 5 of these 9 patients had hypophosphatemia (phosphorus levels less than 0.97 mmol/L). Anthropometric measurements of arm circumference and arm muscle circumference were less than the fifth percentile in all patients developing hypophosphatemia. We concluded that hypophosphatemia is an underrecognized complication of nutritional repletion and that anthropometric measurements may be predictive of patients at risk. All patients with significant malnutrition should be evaluated for this complication of refeeding.