Caregiver perspectives on the everyday medical and social needs of long-term pediatric liver transplant patients.

Using in-depth interviews, we sought to characterize the everyday medical and social needs of pediatric liver transplant caregivers to inform the future design of solutions to improve care processes. Participants (parents/caregivers of pediatric liver transplant recipients) completed a survey (assessing socioeconomic status, economic hardship, health literacy, and social isolation). We then asked participants to undergo a 60-min virtual, semistructured qualitative interview to understand the everyday medical and social needs of the caregiver and their household. We intentionally oversampled caregivers who reported a social or economic hardship on the survey. Transcripts were analyzed using thematic analysis and organized around the Capability, Opportunity, Motivation-Behavior model. A total of 18 caregivers participated. Of the participants, 50% reported some form of financial strain, and about half had less than 4 years of college education. Caregivers had high motivation and capability in executing transplant-related tasks but identified several opportunities for improving care. Caregivers perceived the health system to lack capability in identifying and intervening on specific family social needs. Caregiver interviews revealed multiple areas in which family supports could be strengthened, including (1) managing indirect costs of prolonged hospitalizations (e.g., food, parking), (2) communicating with employers to support families' needs, (3) coordinating care across hospital departments, and (4) clarifying care team roles in helping families reduce both medical and social barriers. This study highlights the caregiver perspective on barriers and facilitators to posttransplant care. Future work should identify whether these themes are present across transplant centers. Caregiver perspectives should help inform future interventions aimed at improving long-term outcomes for children after liver transplantation.

INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE Cohort Study: Design and Rationale for INSPPIRE 2 From the Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer.

We created the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE (INSPPIRE 2) cohort to study the risk factors, natural history, and outcomes of pediatric acute recurrent pancreatitis and chronic pancreatitis (CP). Patient and physician questionnaires collect information on demographics, clinical history, family and social history, and disease outcomes. Health-related quality of life, depression, and anxiety are measured using validated questionnaires. Information entered on paper questionnaires is transferred into a database managed by Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer's Coordinating and Data Management Center. Biosamples are collected for DNA isolation and analysis of most common pancreatitis-associated genes.Twenty-two sites (18 in the United States, 2 in Canada, and 1 each in Israel and Australia) are participating in the INSPPIRE 2 study. These sites have enrolled 211 subjects into the INSPPIRE 2 database toward our goal to recruit more than 800 patients in 2 years. The INSPPIRE 2 cohort study is an extension of the INSPPIRE cohort study with a larger and more diverse patient population. Our goals have expanded to include evaluating risk factors for CP, its sequelae, and psychosocial factors associated with pediatric acute recurrent pancreatitis and CP.

Detection of bowel inflammation with fused DWI/T2 images versus contrast-enhanced images in pediatric MR enterography with histopathologic correlation.

PURPOSE: To evaluate the fused, colorized diffusion weighted imaging (DWI) and anatomic T2 images compared to routine contrast-enhanced T1 images at pediatric magnetic resonance enterography (MRE). METHODS: Fused, colorized DWI/T2 images were created from patients with magnetic resonance enterography (MRE) and colonoscopy/biopsy. Radiologists noted inflammation in five bowel segments (terminal ileum-rectosigmoid colon) on postcontrast images and DWI/T2 images. Test characteristics and agreement were calculated. RESULTS: For 20 patients, sensitivity/specificity/positive predictive value (PPV)/negative predictive value (NPV) were 0.53/0.90/0.77/0.76 for DWI/T2 and 0.45/0.90/0.72/0.73 for postcontrast images. Intraobserver agreement was Ò¡=0.45-0.73. Interobserver agreement was Ò¡=0.53 for DWI/T2 and Ò¡=0.63 for postcontrast images. CONCLUSION: DWI/T2 images are similar in sensitivity/specificity to contrast-enhanced images and with moderate intra/interobserver reliability.

Nonanesthesia magnetic resonance enterography in young children: feasibility, technique, and performance.

OBJECTIVES: The aim of the present study was to demonstrate the effectiveness and cost savings of a nonanesthesia approach to magnetic resonance enterography (MRE) in 14 young pediatric patients (age 4-7 years) with clinically suspected early-onset inflammatory bowel disease using an MRE protocol. METHODS: MRE was performed using a combination of an abbreviated imaging protocol, magnetic resonance imaging video goggles, and Child Life Services support. MRE results were correlated with both colonoscopy and pathology results using Pearson correlation coefficient. Sensitivity, specificity, and positive and negative predictive values were calculated. RESULTS: MRE was performed successfully in 13 of 14 patients (age range 4 years 0 months to 7 years 6 months). MRE findings matched with results in 12 of 13 patients in whom colonoscopy was successfully performed. Both MRE and colonoscopy demonstrated a high specificity (100%) and a positive predictive value (100%), but a low sensitivity (43%) and a negative predictive value (50%). CONCLUSIONS: MRE can be successfully performed in children ages 4 to 7 years using this approach. In addition to decreased risks to the child, the lack of anesthesia also offers a potential overall cost reduction.

Shooting a moving target: use of real-time cine magnetic resonance imaging in assessment of the small bowel.

The chronic nature of inflammatory bowel disease (IBD) creates a lifelong effect on the morbidity of children affected by the disease. The ability to confidently identify and characterize complications resulting from IBD in the pediatric patient is of critical importance. Magnetic resonance enterography (MRE) is especially valuable in the diagnostic assessment of IBD; however, precise elucidation of complications including strictures can be difficult with standard MRE sequences. The recent development of faster MRI pulse sequences provides rapid, real-time imaging of the intestinal tract. In this review, we describe how the addition of cine MRE confidently pinpoints areas of stricture, aids in lesion detection and diagnosis, and provides valuable information on intestinal motility.

Deficiency of indoleamine 2,3-dioxygenase enhances commensal-induced antibody responses and protects against Citrobacter rodentium-induced colitis.

Indoleamine 2,3-dioxygenase (IDO) is a negative regulator of lymphocyte responses that is expressed predominantly in macrophages and dendritic cells. We detected it at high levels in the small intestine and mesenteric lymph node of young adult mice, suggesting a role in intestinal immunity. Consistent with this idea, we found that IDO-deficient mice had elevated baseline levels of immunoglobulin A (IgA) and IgG in the serum and increased IgA in intestinal secretions. These abnormalities were corrected by a course of broad-spectrum oral antibiotics started at weaning, indicating that they were dependent on the intestinal microbiota. Kynurenine and picolinic acid, two IDO-generated metabolites of tryptophan, were able to inhibit lipopolysaccharide-induced antibody production by splenocytes in vitro, and kynurenine also induced B-cell apoptosis, findings that provide an explanation for the elevated Ig levels in animals lacking IDO. The intestinal secretions of IDO-deficient mice had elevated levels of IgA antibodies that cross-reacted with the gram-negative enteric bacterial pathogen Citrobacter rodentium. In keeping with the functional importance of this natural secretory IgA, the mutant animals were more resistant to intestinal colonization by Citrobacter, developed lower levels of serum Citrobacter-specific IgM and IgG antibodies following oral infection, and had significantly attenuated Citrobacter-induced colitis. Our observations point to an important role for IDO in the regulation of immunity to the gut commensal microbiota that has a significant impact on the response to intestinal pathogens.

Developmentally regulated intestinal expression of IFN-gamma and its target genes and the age-specific response to enteric Salmonella infection.

Young infants are highly susceptible to systemic dissemination of enteric pathogens such as Salmonella typhimurium when compared with older individuals. The mechanisms underlying this differential susceptibility have not been defined clearly. To better understand this phenomenon, we examined the responses of adult mice and preweaned pups to oral infection by S. typhimurium. We found clear age-specific differences, namely, an attenuated intestinal inflammatory response and a higher systemic bacterial burden in the pups compared with the adults. To elucidate the molecular basis for these differences, we obtained a microarray-based profile of gene expression in the small intestines of uninfected adult and preweaned animals. The results indicated a striking age-dependent increase in the intestinal expression of a number of IFN-gamma-regulated genes involved in antimicrobial defense. This finding was confirmed by real-time quantitative PCR, which also demonstrated an age-dependent increase in intestinal expression of IFN-gamma. The developmental up-regulation of the IFN-gamma-regulated genes was dependent on both IFN-gamma and a normal commensal microflora, as indicated by experiments in IFN-gamma-knockout mice and germfree mice, respectively. However, the increase in expression of IFN-gamma itself was independent of the commensal flora. The functional importance of IFN-gamma in the immunological maturation of the intestine was confirmed by the observation that the response of adult IFN-gamma-knockout animals to S. typhimurium infection resembled that of the wild-type pups. Our findings thus reveal a novel role for IFN-gamma in the developmental regulation of antimicrobial responses in the intestine.