Impact of exclusive enteral nutrition on the gut microbiome of children with medical complexity.

BACKGROUND: Children with medical complexity (CMC) often require enteral tube feedings to meet their nutrition needs. Many, however, experience symptoms of feeding intolerance, such as vomiting and pain. The goal of this analysis was to examine the relationship between diet and the gut microbiome, controlling for medications, among CMC receiving enteral tube feedings, CMC consuming oral nutrition, and healthy controls. Given the variety of available commercial formula preparations, we were also interested in examining the impact of different formula types on the CMC microbiome. METHODS: Fecal samples from 91 children (57 CMC and 34 healthy controls) were collected and analyzed. Parents completed clinical and dietary questionnaires. 16S ribosomal RNA amplicon sequencing was completed using the QIIME2 pipeline. RESULTS: A significant decrease in alpha diversity among CMC receiving exclusive enteral nutrition (CMC EEN) compared with healthy controls (Shannon P = 0.006 and Faith's phylogenetic distance P = 0.006) was found that was not observed between CMC receiving oral nutrition and healthy controls. Significant differences in beta diversity were also observed between CMC EEN and healthy controls, with CMC EEN having a greater relative abundance of Enterobacteriaceae and obligate anaerobes. Differences were also noted between CMC EEN and CMC receiving oral nutrition (Aitchison distance P = 0.001); however, no differences were observed between CMC receiving oral nutrition and healthy controls. CONCLUSION: Despite similarities in medication profiles, CMC EEN have decreased alpha diversity and differences in beta diversity compared with healthy controls not observed in CMC receiving oral nutrition, highlighting the impact of diet over medications.

Empowering Pediatric Palliative Homecare Patients and Caregivers with Symptom Management Plans.

Pediatric palliative home-based care has been shown to improve symptoms, quality of life, and coordination of care. Despite these successes, hospital utilization in our own palliative home-based care population remained high as some caregivers lacked confidence to manage symptoms at home and had difficulty in recalling or accessing "sick care plans." Our team developed the Symptom Management Plan (SMP), a multi-system "sick care plan," as a quality improvement project with the aim of improving caregiver confidence to manage symptoms at home. An Electronic Health Record-based SMP template was created for common symptoms: respiratory distress, seizures, feeding intolerance, and constipation with core subspecialists' input. Individualized SMPs were created and reviewed with caregivers at every subsequent palliative home nursing visit. Caregivers were surveyed on their confidence 3 and 6-months post-implementation. Resource utilization was analyzed throughout implementation. At 6 months, 73% of caregivers reported "better" or "much better" confidence in managing their child's symptoms after using the SMP, and 76% of caregivers perceived the SMP prevented urgent care or emergency department (ED) visits. After the SMP was launched, the rate of ED visits decreased from 0.86 to 0.47 per 100 patient-days, and admissions decreased from 0.56 to 0.39 per 100 patient-days. These rates further decreased to 0.31 ED visits and 0.19 admissions per 100 patient-days within 4 and 6 months. Introducing the SMP for our home-based palliative care patients was associated with improved caregiver confidence in managing acute symptoms at home and a reduction in hospital utilization.

A unique pancreatic phenotype in a child with a WDR19-related ciliopathy: A case report and literature review of pancreatic involvement in ciliopathies.

Ciliopathies are a group of genetic disorders caused by ciliary dysfunction. Thirty-five distinct multi-organ phenotypes have been recognized, with 187 genes associated. We performed a literature review of pancreatic involvement in ciliopathies and found that pancreatic disease is an uncommon phenotype described in only a handful of these genetic disorders. We present a case report of a pediatric patient with WDR19-related ciliopathy whose degree of pancreatic disease exceeds what has previously been reported in the literature for WDR19-related ciliopathies. WDR19 is one member of the nephronophthisis (NPHP)-related ciliopathy gene family and encodes an intra-flagellar transport protein (IFT144). Our patient presented with restrictive and obstructive lung disease, short rib thoracic dysplasia, end-stage renal disease (ESRD), developmental delay, hepatic fibrosis, and severe recurrent pancreatitis. Whole-exome sequencing (GeneDx) showed two likely pathogenic WDR19 variants in trans (maternally inherited: c.742G > A, p.G248S; paternally inherited: c.617 T > C, p.L206P). Among WDR19-related ciliopathies, pancreatic involvement is rarely reported and there have been no cases of severe, recurrent pancreatitis. Through this case report and literature review we hope to emphasize that pancreatic involvement is a rare yet important clinical phenotype to recognize in ciliopathies, especially in WDR19-related ciliopathies.

Impact of Home-Based Pediatric Palliative Care on Hospital and Emergency Department Utilization at a Single Institution.

Background: There is limited data on home-based pediatric palliative care (PPC) demographics and utilization outcomes. Objective: Describe who receives home-based PPC and compare emergency department visits, hospital admissions, and hospital days admitted in the one year before and after initiation of home-based PPC. Design: Exploratory retrospective medical chart review. Settings/Subjects: Patients, from birth to their 21st birthday, who received home-based PPC during January 1, 2015 to July 31, 2016 at a single site. Measurements: Demographics and hospital utilization were extracted from the medical chart. Results:N = 154. Comparing one year before and after initiation of home-based PPC, the median number of hospitalizations decreased from 2 to 1 (p < 0.001), and the median total number of hospital days admitted decreased from 16 to 4 days (p < 0.001). Conclusions: Children enrolled in a home-based PPC program experienced a significant decrease in the number of hospital admissions and hospital days admitted.

Detection of Neisseria gonorrhoeae from Joint Aspirate by Metagenomic Sequencing in Disseminated Gonococcal Infection.

Disseminated gonococcal infection (DGI) often manifests as gonococcal arthritis and may carry significant morbidity. However, diagnosis remains elusive due to limited sensitivity of available diagnostic tests. We used metagenomic next-generation sequencing to detect Neisseria gonorrhoeae from culture-negative joint aspirates of 2 patients with clinically diagnosed DGI.