Achieving Enteral Autonomy in Children with Intestinal Failure Following Inpatient Admission: A Case Series.

We describe cases of intestinal failure wherein inpatient admission was critical toward enteral autonomy. We performed a retrospective chart review of 6 children with long-term parenteral nutrition dependence who were weaned from parenteral nutrition after admission. Admissions included feeding and medication titration, interdisciplinary care, and a home parenteral nutrition team consultation.

Interprofessional educators' competencies, assessment, and training - IPEcat: protocol of a global consensus study.

Contemporary practice in interprofessional education (IPE) has evolved predominantly focusing on the competencies for interprofessional collaboration (IPC) that learners must acquire. Competencies that educators need to successfully deliver IPC have been overlooked. This lack of attention is further confounded by a field replete with inconsistent terminology and standards and no global consensus on the core competencies needed for IPE facilitation.

Generic and Disease-specific Health-related Quality of Life in Pediatric Intestinal Failure.

Objective: The aim of the study was to assess overall and disease-specific health-related quality of life (hrQOL) in patients with pediatric intestinal failure (PIF) and caregivers and elucidate differences from healthy and chronic gastrointestinal (GI) illness cohorts.

Methods: Cross-sectional study of patients with PIF and their caregivers managed at a multidisciplinary intestinal rehabilitation program using the PedsQL Generic Core and the Gastrointestinal Symptoms Module to assess generic and disease-specific hrQOL, respectively. These data were compared to established healthy and chronic GI disease controls.

CXCR-4 expression by circulating endothelial progenitor cells and SDF-1 serum levels are elevated in septic patients.

Background: Endothelial progenitor cell (EPC) numbers are increased in septic patients and correlate with survival. In this study, we investigated, whether surface expression of chemokine receptors and other receptors important for EPC homing is upregulated by EPC from septic patients and if this is associated with clinical outcome.

Methods: Peripheral blood mononuclear cells from septic patients ( = 30), ICU control patients ( = 11) and healthy volunteers ( = 15) were isolated by Ficoll density gradient centrifugation.

Is Serum Methylmalonic Acid a Reliable Biomarker of Vitamin B12 Status in Children with Short Bowel Syndrome: A Case Series.

We describe 3 patients with short bowel syndrome who had persistently elevated serum methylmalonic acid (MMA) levels while being treated for vitamin B12 deficiency. Following treatment for presumed small bowel bacterial overgrowth, MMA levels normalized. Among patients with short bowel syndrome, MMA levels may have limited specificity for vitamin B12 deficiency.

High Prevalence of Eosinophilic Gastrointestinal Disease in Children With Intestinal Failure.

Objectives: The aim of the present study was to describe the prevalence and clinical features of gastrointestinal (GI) eosinophilic inflammation among pediatric patients with intestinal failure (IF).

Methods: Medical records of all patients studied in our institution's IF program who underwent GI endoscopy over a 15-year period were reviewed, and clinical, pathologic, nutrition, and laboratory data collected.

Results: One hundred five patients underwent 208 GI endoscopic procedures with biopsy.

Ibuprofen intake increases exercise time to exhaustion: A possible role for preventing exercise-induced fatigue.

Although the intake of nonsteroidal anti-inflammatory drugs (NSAIDs) intake by athletes prevents soreness, little is known concerning their role in exercise performance. This study assessed the effects of ibuprofen intake on an exhaustive protocol test after 6 weeks of swimming training in rats. Animals were divided into sedentary and training groups.

[Severe local anesthetic intoxication in an infant undergoing circumcision].

Local anesthetic intoxication is an uncommon complication of regional anesthesia. We report the case of a 4-month-old infant who presented with generalized tonic-clonic seizure complicated by cardiac arrest secondary to a severe intoxication to local anesthesia. These complications were observed after a bilateral dorsal penile nerve block with lidocaine for circumcision in a non-hospital setting.

Gastrointestinal bleeding as a complication of serial transverse enteroplasty.

Purpose: Serial transverse enteroplasty (STEP) lengthens and tapers bowel in patients with intestinal failure. Evaluation and treatment of serious late gastrointestinal bleeding (GIB) in three STEP patients are described.

Methods: Patients participating in an interdisciplinary intestinal rehabilitation program were reviewed to identify those who underwent STEP and had GIB requiring transfusion.

Stac3 is a component of the excitation-contraction coupling machinery and mutated in Native American myopathy.

Excitation-contraction coupling, the process that regulates contractions by skeletal muscles, transduces changes in membrane voltage by activating release of Ca(2+) from internal stores to initiate muscle contraction. Defects in excitation-contraction coupling are associated with muscle diseases. Here we identify Stac3 as a novel component of the excitation-contraction coupling machinery.

Swimming training induces liver mitochondrial adaptations to oxidative stress in rats submitted to repeated exhaustive swimming bouts.

Background And Aims: Although acute exhaustive exercise is known to increase liver reactive oxygen species (ROS) production and aerobic training has shown to improve the antioxidant status in the liver, little is known about mitochondria adaptations to aerobic training. The main objective of this study was to investigate the effects of the aerobic training on oxidative stress markers and antioxidant defense in liver mitochondria both after training and in response to three repeated exhaustive swimming bouts.

Methods: Wistar rats were divided into training (n = 14) and control (n = 14) groups.

Acute creatine administration improves mitochondrial membrane potential and protects against pentylenetetrazol-induced seizures.

A growing body of evidence indicates that creatine (Cr) exerts beneficial effects on a variety of pathologies where energy metabolism and oxidative stress play an etiological role. However, the benefits of Cr treatment for epileptics are still shrouded in controversy. In the present study, we found that acute Cr treatment (300 mg/kg, p.

Creatine increases hippocampal Na(+),K(+)-ATPase activity via NMDA-calcineurin pathway.

Achievements made over the past few years have demonstrated the important role of the creatine and phosphocreatine system in the buffering and transport of high-energy phosphates into the brain; however, the non-energetic processes elicited by this guanidine compound in the hippocampus are still poorly understood. In the present study we disclosed that the incubation of rat hippocampal slices with creatine (10mM) for 30 min increased Na(+),K(+)-ATPase activity. In addition, intrahippocampal injection of creatine (5 nmol/site) also increased the above-mentioned activity.

Healthcare-associated infection prevention in pediatric intensive care units: a review.

The objective of this review was to summarize the current knowledge base on the prevention of nosocomial infections in pediatric intensive care units (PICUs). Healthcare-associated infections (HAIs) are a crucial problem in PICUs because of their impact on patient outcome, length of hospital stay, and costs. Studies published between 1998 and 2011 were identified using the MEDLINE and Cochrane databases.

[Kidney transplantation and infection in childhood].

Infectious risk is more important in the transplanted child than in adult because children are less often immunised against pathogens ant more exposed than adults to numerous infectious agents (virus but also bacteria including pneumococcus). The application of the standard immunisation schedule must be a permanent concern of transplantation (Tx) teams. Some vaccines that are not planned in the standard immunization schedule are particularly advised for the child and his family circle, as well as for caregivers.

Prompt and successful toxin-targeting treatment of three patients with necrotizing pneumonia due to Staphylococcus aureus strains carrying the Panton-Valentine leukocidin genes.

Three patients with extensive necrotizing pneumonia due to Panton-Valentine leukocidin-positive Staphylococcus aureus strains and with aggravating factors (leukopenia count of less than 3x10(9)/liter in all three cases and hemoptysis in two cases) were successfully treated with toxin-suppressing agents introduced rapidly after hospital admission.

Novel congenital myopathy locus identified in Native American Indians at 12q13.13-14.1.

Background: Native American myopathy (NAM) is an autosomal recessive congenital myopathy first reported in the Lumbee Indian people. Features of NAM include congenital weakness, cleft palate, ptosis, short stature, and susceptibility to malignant hyperthermia provoked by anesthesia.

Method: We identified five individuals with NAM from the Lumbee population, and hypothesized that these affected individuals have disease alleles shared identical-by-descent inherited from common ancestry.

Native American myopathy: congenital myopathy with cleft palate, skeletal anomalies, and susceptibility to malignant hyperthermia.

Native American myopathy (NAM) [OMIM 255995], a putative autosomal recessive disorder, was first reported in the Lumbee Indians of North Carolina. NAM features include congenital weakness and arthrogryposis, cleft palate, ptosis, short stature, kyphoscoliosis, talipes deformities, and susceptibility to malignant hyperthermia (MH) provoked by anesthesia. This report documents the phenotypic complexity and natural history of this rare congenital disorder in fourteen individuals with NAM.

Non-invasive ventilation as primary ventilatory support for infants with severe bronchiolitis.

Objective: To report our experience of non-invasive ventilation (NIV) as primary ventilatory support strategy in infants admitted for severe bronchiolitis.

Design And Setting: Retrospective study in a paediatric intensive care unit of an university hospital.

Patients: Infants aged less than 12 months, admitted for bronchiolitis during 2003-2004 and 2004-2005 winter epidemics.

Refinement of 2q and 7p loci in a large multiplex NTD family.

Background: NTDs are considered complex disorders that arise from an interaction between genetic and environmental factors. NTD family 8776 is a large multigenerational Caucasian family that provides a unique resource for the genetic analysis of NTDs. Previous linkage analysis using a genome-wide SNP screen in family 8776 with multipoint nonparametric mapping methods identified maximum LOD* scores of approximately 3.

High-density single nucleotide polymorphism screen in a large multiplex neural tube defect family refines linkage to loci at 7p21.1-pter and 2q33.1-q35.

Background: Neural tube defects (NTDs) are considered complex, with both genetic and environmental factors implicated. To date, no major causative genes have been identified in humans despite several investigations. The first genomewide screen in NTDs demonstrated evidence of linkage to chromosomes 7 and 10.

Age and diet act through distinct isoforms of the class II transactivator gene in mouse intestinal epithelium.

Background & Aims: Normal weaning induces class II major histocompatibility complex (Ia) and invariant chain (Ii) expression in the mouse intestinal epithelium. Because the class II transactivator protein (CIITA) induces Ia and Ii in most cell types, we hypothesized that diet-induced expression of these genes was through CIITA.

Methods: Mouse litters were split and weaned onto an elemental diet or a normal (complex) chow diet.