A case series of successfully managing exomphalos major with awake graduated compression dressing and early enteral feeding.

Introduction: Exomphalos anomaly is defined as the herniation of abdominal viscera into the base of the umbilical cord, with only a membranous sac covering these contents. It has an incidence of approximately 1 in 4000-6000 births. Management of exomphalos major (EM) remains controversial and limited, with very few studies to guide decision-making.

The experiences of adolescents living with a central venous access device: A qualitative analysis.

Background: Central venous access devices are used in paediatric populations for specific chronic conditions requiring long-term treatment. Very little isknown about how young people experience living with such devices.

Aim: To gain a deep understanding of adolescents' (aged 12-17 years) experiences of living with a central venous access device from the perspective of the adolescents themselves, and of one of their parents.

Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature.

Introduction: Treatment of patients with Haemophilia A has improved significantly in recent years since the advent of novel therapeutic agents such as emicizumab. The low annualised bleeding rates associated with emicizumab have liberated many patients from the need for central venous access devices (CVAD). Optimal peri-operative management of CVAD removal is not currently known and there are no specific formal recommendations available.

Multi-Organ Dysfunction in Cerebral Palsy.

Cerebral Palsy (CP) describes a heterogenous group of non-progressive disorders of posture or movement, causing activity limitation, due to a lesion in the developing brain. CP is an umbrella term for a heterogenous condition and is, therefore, descriptive rather than a diagnosis. Each case requires detailed consideration of etiology.

Nucleic acid cytokine responses in obese children and infants of obese mothers.

Almost a third of Irish children are now overweight and the country ranks 58th out of 200 countries for its proportion of overweight youths. With the rising obesity epidemic, and the impaired immune responses of this population, it is vital to understand the effects that obesity has on the immune system and to design future therapeutics, adjuvants and vaccines with overweight and obese populations in mind. Many current vaccines use adjuvants that have been found to be less effective at stimulating the immune response in children compared with adults and there is now substantial effort to design paediatric-focused adjuvants.

Type 1 IFN Induction by Cytosolic Nucleic Acid Is Intact in Neonatal Mononuclear Cells, Contrasting Starkly with Neonatal Hyporesponsiveness to TLR Ligation Due to Independence from Endosome-Mediated IRF3 Activation.

Two million infants die each year from infectious diseases before they reach 12 mo; many of these diseases are vaccine preventable in older populations. Pattern recognition receptors represent the critical front-line defense against pathogens. Evidence suggests that the innate immune system does not fully develop until puberty, contributing to impaired response to infection and impaired vaccine responses in neonates, infants, and children.

Neonatal scrotal wall necrotizing fasciitis (Fournier gangrene): a case report.

Introduction: Necrotizing fasciitis in neonates is rare and is associated with almost 50% mortality. Although more than 80 cases of neonates (under one month of age) with necrotizing fasciitis have been reported in the literature, only six of them are identified as originating in the scrotum.

Case Presentation: We report the case of a four-week-old, full-term, otherwise-healthy Caucasian baby boy who presented with an ulcerating lesion of his scrotal wall.

Age-related changes in the myenteric plexus of the porcine bowel.

Introduction: Myenteric plexus (MP) is well recognized as an important regulator of peristaltic activity. Knowledge regarding prenatal and postnatal normal morphological changes is important when interpreting histopathologic findings in motility disorders of childhood. The aim of this study was to determine the neuronal density and morphology of the myenteric plexus (MP) of the porcine bowel from fetal life to adulthood.

New insights into the neuromuscular anatomy of the ileocecal valve.

The neuroanatomy of the ileocecal valve (ICV) is poorly understood. A better understanding of this important functional component of the gastrointestinal tract would enable surgeons to reconstruct an effective valve following surgical resection of the ICV. ICVs were examined in young pigs (N = 5) using frontal and transverse paraffin embedded and frozen sections.

Postnatal changes in enteric plexus axonal thickness.

Purpose: Chronological age has not always been an accurate predictor of gut motility in newborns. We hypothesized that the enteric plexus is immature at birth. We studied whole-mount preparations of the myenteric plexus in pigs from mid-point of gestation to adulthood.

Correlation of enteric NADPH-d positive cell counts with the duration of incubation period in NADPH-d histochemistry.

Nicotinamide adenine dinucleotide phosphate-diaphorase (NADPH-d) staining can be used in the enteric nervous system to determine nitrergic neuronal counts, critical in motility disorders such as intestinal neuronal dysplasia and hypoganglionosis. The reported incubation periods of specimens with NADPH-d staining solution has varied from 2 to 24 h. The aim of this study is to investigate the impact of the incubation period on the overall NADPH-d positive cell counts in porcine rectal submucosal plexus.

New hypothesis on the pathogenesis of ileocecal intussusception.

Purpose: Ileocecal intussusception is a relatively common surgical emergency in infants and young children. The etiology of intussusception is not clearly understood. Nitric oxide (NO) is a major inhibitory neurotransmitter in the enteric nervous system, which causes relaxation of the smooth muscles.

Long-term results of delayed primary anastomosis for pure oesophageal atresia: a 27-year follow up.

The management of a newborn with pure oesophageal atresia continues to be challenging. We started treating babies with pure oesophageal atresia by delayed primary anastomosis in 1977. The purpose of this study was to review the long-term outcome in infants with pure oesophageal atresia (EA) treated by delayed primary anastomosis with special emphasis on gastroesophageal reflux (GOR) related morbidity.

Developmental changes of the adrenergic network in the myenteric plexus of the porcine small bowel.

Knowledge about the foetal development of the normal enteric nervous system (ENS) is crucial for the understanding of congenital and acquired functional abnormalities of the gut. The ENS is the largest and most complex division of the peripheral nervous system and consists of intrinsic and extrinsic components. Although previous studies have described sympathetic innervation of the myenteric plexus, little is known regarding its age-related changes.

The effect of age on colocalization of acetylcholinesterase and nicotinamide adenine dinucleotide phosphate diaphorase staining in enteric neurons in an experimental model.

Purpose: Cholinergic and nitrergic neurons form 2 main subpopulations of the myenteric neurons, and they have been the targets of detailed morphological investigations in bowel motility disorders. However, little is known regarding the colocalization of neurotransmitters within the same enteric neurons. The aim of this study was to determine the histochemical colocalization of cholinergic and nitrergic neurons in the porcine distal large bowel myenteric plexus from fetal to adulthood.

Developmental changes in submucosal nitrergic neurons in the porcine distal colon.

Background/purpose: As our understanding of the enteric nervous system improves, it becomes clear that it is no longer sufficient to simply determine whether enteric ganglion cells are present but also to determine whether correct number and types of ganglion cells are present. Nitric oxide is recognized as a potent mediator of inhibitory nerves responsible for the relaxation of the smooth muscle of the gastrointestinal tract. The aim of this study was to determine the normal nitrergic neuronal density and morphology in the submucosal plexus of the porcine distal bowel from fetal life to adulthood.

Mucinous cystadenoma of the ovary in perimenarchal girls.

Ovarian masses in children are an uncommon occurrence. They represent less than 2% of all tumours in girls less than 16 years of age. Mucinous tumours of the ovary occur principally in middle adult life and are extremely rare prior to menarche.

New technique for fixation of Broviac catheters.

Broviac catheters are in common use for administration of parenteral nutrition, cancer chemotherapy, and antibiotic therapy within the paediatric population. Inadvertent dislodgement of these catheters is common during the initial weeks before the Dacron cuff is anchored by the ingrowth of fibrous tissue. The authors describe a technique in which an internal fixation suture is placed to prevent dislodgement or migration.