J Pediatr Gastroenterol Nutr
September 2024
Background: Infants with small bowel stomas (SBstoma) frequently struggle with absorption and rely on parenteral nutrition (PN). Intestinal absorption is difficult to predict based solely on intestinal anatomy. The purpose of this study was to characterize the microbiota and metabolic by-products within stoma effluent and correlate with clinical features and intestinal absorption.
View Article and Find Full Text PDFPurpose: Neonates with intestinal failure (IF) are at risk for infection due to central venous access, and intestinal surgery. Infection can cause systemic inflammation and sepsis, potentially affecting growth. The purpose of this study was to identify risk factors for, and the potential impact of infection to help with preventative strategies.
View Article and Find Full Text PDFBackground: Nutrition plays a vital role in shaping the intestinal microbiome. However, many hospitalized children undergo periods of fasting during medical treatment. Changes to the small intestinal microbiota in early life in the setting of enteral deprivation have not been well described.
View Article and Find Full Text PDFBackground: Vitamin D deficiency is present in 40%-70% of children with intestinal failure (IF), yet there are no published guidelines for repleting and maintaining vitamin D levels in this population. The purpose of this study is to evaluate the efficacy of a standardized vitamin D algorithm in reducing the incidence of deficiency.
Methods: A retrospective chart review was performed in children with IF who had at least one serum vitamin D (25-hydroxyvitamin D ) measurement.
Background/purpose: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.
View Article and Find Full Text PDFNuclear factor one X (NFIX) is a transcription factor required for normal ependymal development. Constitutive loss of in mice () is associated with hydrocephalus and sloughing of the dorsal ependyma within the lateral ventricles. Previous studies have implicated NFIX in the transcriptional regulation of genes encoding for factors essential to ependymal development.
View Article and Find Full Text PDFEur J Cell Biol
August 2022
Proteins of the Wiskott-Aldrich syndrome protein (WASP) family play a central role in regulating actin cytoskeletal dynamics in a wide range of cellular processes. Genetic mutations or misregulation of these proteins are tightly associated with many diseases. The WASP-family proteins act by transmitting various upstream signals to their conserved WH2-Central-Acidic (WCA) peptide sequence at the C-terminus, which in turn binds to the Arp2/3 complex to stimulate the formation of branched actin networks at membranes.
View Article and Find Full Text PDFChildren who require surgical interventions are subject to physiologic stress, necessitating a period of healing when nutrition needs may temporarily change. Providing appropriate nutrition to children before and after surgery is an important part of minimizing surgical morbidity. There is a clear link between poor nutrition and surgical outcomes, therefore providing good reason for ensuring an appropriate nutrition plan is in place for children requiring surgery.
View Article and Find Full Text PDFObjective: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts.
Summary Background Data: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known.
Purpose: The origin of congenital abdominal cysts in the female fetus often dictates management. While most arise from the ovary and are often managed non-operatively, some are non-ovarian and are frequently removed. We analyzed a national sample of female infants with congenital abdominal cysts to elucidate prenatal and postnatal factors associated with the diagnosis of a non-ovarian cyst.
View Article and Find Full Text PDFPurpose: Urine sodium (UNa) is a measure of total body sodium in infants with intestinal failure (IF) but can be misleading as it does not reflect volume status. Urine sodium to urine creatinine ratio (UNa:UCr) may offer a more accurate measure, but is not routinely used. This study compares UNa:UCr to UNa as a maker of sodium status in infants with IF.
View Article and Find Full Text PDFBackground: Necrotizing enterocolitis remains a leading cause of morbidity and mortality in premature infants. The role of prophylactic probiotics in its prevention is unclear. This study evaluates the effect of routine probiotics on the incidence and severity of necrotizing enterocolitis in infants with very low birth weight in the neonatal intensive care unit.
View Article and Find Full Text PDFPurpose: Infants with short bowel syndrome (SBS) wean from parenteral nutrition (PN) support at variable rates. Small bowel length is a predictor, but the importance of the ileocecal valve (ICV) and colon are unclear. We aim to determine if the ICV and/or colon predict enteral autonomy.
View Article and Find Full Text PDFMultidisciplinary intestinal rehabilitation (IR) teams have transformed care in pediatric intestinal failure (IF). Although most children with IF are identified in the neonatal intensive care unit (NICU), IR teams may not be involved at this stage. We describe our collaborative model, blending NICU and IR expertise to optimize care.
View Article and Find Full Text PDFBackground: Children with intestinal failure (IF) require parenteral nutrition (PN) at home, delivered through a central venous catheter (CVC) to support growth. CVC-related complications including infection, breakage, and blockage are the most common cause of readmission to the hospital. The objective of this study was to evaluate the use of instructional videos as part of the caregiver home PN-teaching program to reduce CVC-related complications.
View Article and Find Full Text PDFPediatric intestinal failure (IF) is the critical reduction of intestinal mass or function below the amount necessary for normal growth in children. Short-bowel syndrome (SBS) is the most common cause of IF in infants and children and is caused by intestinal resection. Enteral autonomy and freedom from parenteral nutrition is the mainstay of nutrition management in SBS.
View Article and Find Full Text PDFBackground: Children with short bowel syndrome (SBS) frequently struggle with malabsorption and poor growth. The intestinal microbiota plays an important role in gut function, and children with SBS have known deficiencies in some commensal gut microbes. One strategy to enhance the gut microbiota is by taking probiotics.
View Article and Find Full Text PDFPurpose: Cholestasis is problematic for infants with intestinal failure (IF). The soy-based lipid Intralipid® (IL) has been implicated. An alternative, Smoflipid® (SMOF), is increasingly used.
View Article and Find Full Text PDFA jaw lesion reported in mink exposed to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) and TCDD-like chemicals is considered a potential indicator of exposure to these chemicals. Many of the effects of TCDD-like chemicals are induced through interaction with the aryl hydrocarbon receptor. The present study indicates that mink dosed with β-naphthoflavone, which is an aryl hydrocarbon receptor ligand but not a TCDD-like chemical, also develop the lesion.
View Article and Find Full Text PDFChildren with short bowel syndrome have significant changes to their intestinal microbiota after intestinal loss. The purpose of this article is to understand the potential implications of these changes on gut function, hepatic cholestasis and overall nutrition. Possible therapies to restore the commensal bacterial community in these patients will also be reviewed.
View Article and Find Full Text PDFBackground: Pediatric breast masses can be a diagnostic challenge. Nearly all are benign, but there is no consensus on which should be removed. We hypothesized that children with asymptomatic breast lesions can be safely managed nonoperatively.
View Article and Find Full Text PDFPurpose: The purpose of this study was to investigate whether anticoagulation (AC) results in thrombus resolution and increased line longevity in children with intestinal failure (IF) and catheter-associated central venous thrombosis (CVT).
Methods: A retrospective, single institution review was performed of children with IF who were dependent on parenteral nutrition with known CVT between 2006 and 2017. Frequency of catheter-related complications including infection, occlusion, and breakage were compared 18months prior to and after starting AC.
Background: Parental leave is linked to health benefits for both child and parent. It is unclear whether surgeons at academic centers have access to paid parental leave. The aim of this study was to determine parental leave policies at the top academic medical centers in the United States to identify trends among institutions.
View Article and Find Full Text PDFPediatric patients with intestinal failure often require central venous catheters for extended periods of time for parenteral nutrition, blood sampling, and medication administration, increasing morbidity, mortality, and costs. In 2007, we reported a central line-associated bloodstream infection rate of 7.0 per 1,000 catheter line-days in our pediatric patients with intestinal failure.
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