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Child adversity is often associated with poor quality of life in pediatric gastrointestinal disorders, including non-allergic food reactions (food intolerances), which may be improved using mind-body interventions. We conducted an observational study to (1) describe child adversity (stressors) and resilience factors in children with food intolerances, and (2) explore the association between stressors and self-reported use of integrative modalities. A retrospective chart review of children ≥4-years-old presenting to a pediatric food intolerances clinic from 2017 to 2020 was performed (n = 130). Use of integrative medicine at intake, demographic, illness, and social history data were collected. Qualitative analysis identified exposure to stressors and resilience strategies. Correlation was assessed using a chi-square test. Management of the medical condition was the most common stressor, indicating impact on quality of life. Resilience strategies included themes of self-coping and social support. Individuals with one or more stressors were more likely to be using an integrative modality (most commonly, mind-body interventions) prior to their visit (X2 = 8.1, p = 0.004). Our hypothesis-generating study suggests that screening for child adversity and integrative medicine use may be used to better address quality of life and personalized approaches to treat pediatric food intolerances.

Food is essential for life. Yet, poor food choices may cause poor health. Dietary manipulation is frequently integrated into the management of common chronic pediatric conditions. Parents seek dietary information to have more control over child's condition and to avoid side effects of medicine. This article reviews selected diets for a few common pediatric disorders including eczema, attention deficit hyperactivity disorder, headache and migraine, non-celiac gluten sensitivity, and irritable bowel syndrome.

Objectives To identify predictors of portal hypertension, liver transplantation, and death in North American youth with alpha-1-antitrypsin (AAT) deficiency, and compare with patients with AAT deficiency elsewhere. Study design The Childhood Liver Disease Research Network Longitudinal Observational Study of Genetic Causes of Intrahepatic Cholestasis is a prospective, cohort study of pediatric cholestatic liver diseases, including AAT deficiency, enrolling PIZZ and PISZ subjects 0-25 years of age seen since November 2007 at 17 tertiary care centers in the US and Canada. Data from standard-of-care baseline and annual follow-up visits were recorded from medical records, history, physical examination, and laboratory studies. Participants with portal hypertension were identified based on data collected. Results We enrolled 350 participants (60% male) with a native liver; 278 (79%) entered the cohort without portal hypertension and 18 developed portal hypertension during follow-up. Thirty participants required liver transplantation; 2 patients died during 1077 person-years of follow-up. There was no difference in participants with or without preceding neonatal cholestasis progressing to transplantation or death during the study (12% vs 7%; P = .09), or in experiencing portal hypertension (28% vs 21%; P = .16); the hazard ratio for neonatal cholestasis leading to portal hypertension was P = .04. Development of portal hypertension was associated with a reduced height Z-score. Conclusions Portal hypertension in youth with AAT deficiency impacts growth measures. Progression to liver transplantation is slow and death is rare, but the risk of complications and severe liver disease progression persists throughout childhood. A history of neonatal cholestasis is a weak predictor of severe disease.

Objective Serious pediatric illness places great stress on families. Parents who learn coping skills may better manage these stressors. This study sought to develop and refine a stress coping intervention for parents of hospitalized children, assess the intervention acceptability among these parents, and gather preliminary data on stress, negative and positive affect, anxiety, depression, and self-efficacy. Methods We conducted an observational study in 2 phases, enrolling parents of children who were inpatients with serious illness, 10 in Phase 1 and 40 in Phase 2. All parents completed at baseline measures of stress and psychological well-being and were introduced to the Coping Kit for Parents. Follow-up interviews were conducted at 1 week (all parents) and 1 month (Phase 2 parents only) regarding the acceptability of the intervention. Results At baseline, parents reported that stressful situations were frequent (mean = 30.6, standard deviation [SD] = 6.8) and difficult (mean = 26.2, SD = 7.1) and revealed elevated levels of negative affect (mean = 27.3, SD = 7.7), depression (mean = 8.5, SD = 3.7), and anxiety (mean = 11.3, SD = 3.1) and moderate levels of self-efficacy related to their child's illness (mean = 3.3, SD = 0.5). The majority of parents used the kit regularly and on a scale of 1 to 7 agreed that the kit was helpful (mean = 6.0, SD = 0.9), interesting (mean = 5.7, SD = 1.3), practical (mean = 5.7, SD = 1.4), enjoyable (mean = 6.0, SD = 1.3), and they would recommend it to other parents (mean = 6.4, SD = 0.9). Conclusions The Coping Kit for Parents is an acceptable stress management intervention that could be made available to parents of children with serious illness at pediatric hospitals with minimal staff training or time commitment.

zfhz1b is the causative gene for Mowat-Wilson syndrome, in which patients demonstrate developmental delay and Hirschsprung disease, as well as other anomalies.We identified a patient with Mowat-Wilson syndrome who also developed cholestasis and histopathologic features consistent with biliary atresia, suggesting that mutations involving zfhz1b may lead to biliary developmental anomalies or injury to the biliary tract. We used the zebrafish model system to determine whether zfhx1b has a role in vertebrate biliary development.Using zebrafish we determined that zfhx1b was expressed in the developing liver during biliary growth and remodeling, and that morpholino antisense oligonucleotide-mediated knockdown of zfhx1b led to defects in biliary development. These findings were associated with decreased expression of vhnf1, a transcription factor known to be important in biliary development in zebrafish and in mammals.Our studies underscore the importance of genetic contributions in the etiology of infantile hepatobiliary disorders, including biliary atresia.

Objective: The objectives of the present study were to assess the prevalence, frequency and type of complementary and alternative medicine (CAM) used by children with chronic viral hepatitis infection, and to determine correlates of use and estimates of nondisclosure regarding CAM use. Patients and Methods: In this cross-sectional pilot study, families of 68 children receiving care for chronic viral hepatitis at a tertiary medical center were administered a survey regarding use of CAM. Results: Forty-six percent of these families reported using CAM for their child at least once since diagnosis with chronic viral hepatitis and 31% used CAM monthly or more frequently. Of all of the CAM therapies, biologically based products such as herbals and dietary supplements were used most often. Use of CAM was independently associated with the current or previous use of antiviral medications for viral hepatitis, parent's use of CAM, and child having a nonliver comorbidity. Rates of physician nondisclosure regarding CAM use were >60%. Conclusions: This is the first report of CAM use in children with chronic viral hepatitis. Use of CAM in this population is common, and despite published adult reports, there is infrequent dialogue between patients and pediatric health care providers regarding use of CAM.

We present a case report of a boy with biliary atresia who, after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools. Nuclear medicine imaging study showed no excretion. Broad-spectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.

Objectives To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life. Study design Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 μM) at any time in the first 3 months (TB Results Fifty percent (68/137) of infants had TB P P P P P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P P Conclusions Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB Trial registration ClinicalTrials.gov: NCT00061828 and NCT00294684.

We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.