Catheter salvage from central line-related bloodstream infections in pediatric intestinal failure.

Objectives: Patients with intestinal failure require central venous access which puts them at risk for central line-associated bloodstream infections (CLABSI). Maintaining vascular patency is critical for this population to receive nutrition support. When CLABSIs occur line salvage can help maintain vascular access.

Impact of genetic and non-genetic factors on phenotypic diversity in NBAS-associated disease.

Biallelic pathogenic variants in neuroblastoma-amplified sequence (NBAS) cause a pleiotropic multisystem disorder. Three clinical subgroups have been defined correlating with the localisation of pathogenic variants in the NBAS gene: variants affecting the C-terminal region of NBAS result in SOPH syndrome (short stature, optic atrophy, Pelger-Huët anomaly), variants affecting the Sec 39 domain are associated with infantile liver failure syndrome type 2 (ILFS2) and variants affecting the ß-propeller domain give rise to a combined phenotype. However, there is still unexplained phenotypic diversity across the three subgroups, challenging the current concept of genotype-phenotype correlations in NBAS-associated disease.

Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA.

Background And Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study.

Use of Cidofovir for Safe Transplantation in a Toddler with Acute Liver Failure and Adenovirus Viremia.

Background: Since October 2021, there have been more than 500 cases of severe hepatitis of unknown origin in children reported worldwide, including 180 cases in the U.S. The most frequently detected potential pathogen to date has been adenovirus, typically serotype 41.

Pediatric Acute Liver Failure.

Pediatric acute liver failure is a rare process that results from many different diseases including toxin ingestion and drug overdose, infections, metabolic and genetic disorders, immune-mediated diseases, and ischemia. Up to 50% of children with acute liver failure will never have an underlying cause found. Early identification, supportive care, and disease-directed therapy are critical.

Improving the rate of anthropometric measurements in the pediatric intensive care unit.

Background: Malnutrition occurs in approximately 25% of pediatric intensive care patients and correlates with increased length of stay, prolonged ventilation, and mortality. Anthropometric measurements should be obtained at admission and throughout hospitalization to evaluate nutrition status. We aimed to increase documentation, reporting, and discussion of anthropometric measurements, including height/length, weight, and occipital frontal circumference (OFC) within 24 hours of admission and weekly.

Transient hyperphosphatasemia following pediatric liver transplantation in a patient with hepatic and skeletal abnormalities.

Background: We report a unique case of transient hyperphosphatasemia in a pediatric patient with a history of hepatic and skeletal abnormalities.

Patient And Methods: A 2-month old male was diagnosed with progressive familial intrahepatic cholestasis type-2 and osteoporosis after marked increases in liver function tests were noted at 1 month of age. He underwent a second liver transplantation at 1 y.

Body composition assessment after pediatric liver transplant.

Background: Pediatric liver transplantation generally restores metabolic function; yet after transplantation, some children remain malnourished, have increased adiposity, and develop obesity. Measurement of body composition in the assessment of nutrition status could reduce adverse consequences in children.

Methods: Anthropometric measurements, multiple-frequency bioelectrical impedance analysis, air displacement plethysmography, and ultrasound measurements were conducted on children recruited from the liver transplant program at the University of Minnesota Masonic Children's Hospital.

Body Composition Post Pediatric Liver Transplant: Implications and Assessment.

Liver disease has a negative influence on growth and development of children. Measurement of body composition as a component of nutrition status assessment in children before and after transplant would facilitate tailoring of nutrition therapy. A comprehensive literature search on pediatric liver transplant and body composition assessment was performed using a modified systematic approach.

Rapid Infliximab Infusion in the Pediatric Population.

Objectives: To compare infusion reaction rates between rapid infliximab (REMICADE, Janssen Biotech Inc) infusions and previous standard 2- to 3-hour infusions; additionally, to assess patient satisfaction and reduction in chair time associated with rapid infliximab infusions.

Methods: Pediatric rheumatology and gastroenterology patients receiving maintenance infliximab therapy using a standard 2- to 3-hour titrated infusion had the opportunity to enroll in the non-titrated rapid 1-hour infusion protocol following tolerance of induction dosing at 0, 2, and 6 weeks. Patients were included from December 1, 2017, to March 31, 2018, via retrospective chart review and patient satisfaction surveys.

Pediatric retransplantation of the liver: A prognostic scoring tool.

Few prognostic models have been created in children that receive liver retransplantation (rLT). We examined the SRTR database of 731 children that underwent second liver transplant between 2002 and 2018. Proportional hazards models using backward variable selection were used to identify recipient, donor, and surgical characteristics associated with survival.

NASPGHAN Nutrition University (N2U): A Survey of its Efficacy as Continuing Nutrition Education for North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition.

The North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) developed NASPGHAN Nutrition University (N2U) in 2012 to improve nutrition education for pediatric gastroenterology providers. A total of 543 providers (physicians, registered dietitians, and advanced practice nurses) have applied to N2U and 285 have attended this 2-day course. We used survey methodology to compare attendees to applicants who did not attend.

Neonatal Acute Liver Failure.

Neonatal acute liver failure (NALF) is a rare disease with a few known primary causes: gestational alloimmune liver disease (GALD), viral infections, metabolic diseases, and ischemic injury. Many cases still do not have a known cause. Laboratory evaluation may suggest a diagnosis.

Defining clinical subgroups and genotype-phenotype correlations in NBAS-associated disease across 110 patients.

Purpose: Pathogenic variants in neuroblastoma-amplified sequence (NBAS) cause an autosomal recessive disorder with a wide range of symptoms affecting liver, skeletal system, and brain, among others. There is a continuously growing number of patients but a lack of systematic and quantitative analysis.

Methods: Individuals with biallelic variants in NBAS were recruited within an international, multicenter study, including novel and previously published patients.

Malnutrition in Children With Chronic Disease.

Malnutrition occurs when nutrient intake does not meet the needs for normal body functions and as a consequence leads to alterations of growth and development in children. Chronic illness puts children at risk for developing malnutrition. Because of children's rapid periods of growth and development, early diagnosis, prevention, and management of malnutrition are paramount.

Defining Pediatric Failure to Thrive in the Developed World: Validation of a Semi-Objective Diagnosis Tool.

There is no concordance between current diagnostic criteria for failure to thrive (FTT). We analyzed validity of the Semi-Objective Failure to Thrive (SOFTT) diagnosis tool, which uses a combination of subjective and objective components to make the diagnosis of FTT. The tool was used to diagnose FTT in 94 patients who met 1 of 7 accepted criteria for FTT.

Body composition and cognition in preschool-age children with congenital gastrointestinal anomalies.

Background: Children with congenital gastrointestinal anomalies (CGIAs) experience multiple stressors while hospitalized in neonatal intensive care units during an essential time of growth and development. Early stress and inadequate nutrition are linked to altered growth patterns and later neurodevelopmental delays. In other at-risk populations, improved fat-free mass (FFM) accretion is associated with improved cognitive outcomes.

Laboratory Monitoring of Children on Home Parenteral Nutrition: A Prospective Study.

Background: The primary hypothesis of this article is that a team approach in creating a protocolized laboratory monitoring schedule for home parenteral nutrition (PN) patients improves patient safety by decreasing the occurrence of nutrition deficiencies and is cost-effective.

Methods: In this prospective cohort study of home PN patients, each patient followed an established protocol of laboratory monitoring and weekly review by an interdisciplinary team of dietitians, nurses, and physicians. Data collected included anthropometric measurements, laboratory results, deviations from laboratory protocols, laboratory charges, PN shortage information, and means of ameliorating such shortages.

Hospitalization for Failure to Thrive: A Prospective Descriptive Report.

Failure to thrive (FTT) is a common symptom leading to hospitalization of children. Most literature describing this population is from 30 years ago. Since that time the hospitalized population has become more medically complex.

Langerhans cell histiocytosis of the digestive tract identified on an upper gastrointestinal examination.

Langerhans cell histiocytosis (LCH) with involvement of the gastrointestinal tract is rare and typically identified in patients with systemic disease. We describe a 16-month-old girl who initially presented with bilious vomiting, failure to thrive and a rash. An upper gastrointestinal (GI) examination revealed loss of normal mucosal fold pattern and luminal narrowing within the duodenum, prompting endoscopic biopsy.

Clinical Review of Failure to Thrive in Pediatric Patients.

Failure to thrive (FTT) is a common problem that occurs when caloric intake is insufficient to maintain growth. For the majority of children it can be reversed with behavioral modifications and increased caloric provisions. In a minority of cases, FTT is the symptom of underlying organic disease.

Failure to Thrive: A Prospective Study in a Pediatric Gastroenterology Clinic.

Objectives: We aimed to describe the clinical characteristics, diagnostic work-up, interventions, and outcomes of children referred to a pediatric gastroenterology clinic with the diagnosis of failure to thrive (FTT).

Methods: We prospectively enrolled 110 children seen for the first time in our pediatric gastroenterology clinic for FTT. Standard demographic information, history, and anthropometric data were collected at initial and follow-up visits.