Quantitative trait mapping reveals a regulatory axis involving peroxisome proliferator-activated receptors, PRDM16, transforming growth factor-β2 and FLT3 in hematopoiesis.

Hematopoiesis is the process whereby BM HSCs renew to maintain their number or to differentiate into committed progenitors to generate all blood cells. One approach to gain mechanistic insight into this complex process is the investigation of quantitative genetic variation in hematopoietic function among inbred mouse strains. We previously showed that TGF-β2 is a genetically determined positive regulator of hematopoiesis.

Transient enhancement of pericardium, peritoneum, soft tissues and perhaps lymphatics after large doses of contrast administration: an overlooked phenomenon?

An infant with complex cyanotic congenital heart disease was recently encountered whose radiographs seemed to show enhancement of pericardium, peritoneal mesothelium and body wall fascial planes without enhancement of the liver or spleen after very large doses of intravenous contrast. Although patterns of postcontrast enhancement have been described previously, this pattern seems to be unique. We report the unusual postcontrast opacification pattern and speculate about its underlying mechanism.

Preventive strategies for central line-associated bloodstream infections in pediatric hematopoietic stem cell transplant recipients.

Background: Few studies have described preventive strategies for central line-associated bloodstream infections (CLABSIs) in pediatric hematopoietic stem cell transplantation (HSCT) recipients.

Methods: We performed a pilot intervention study in our pediatric HSCT population in 2006-2008 and compared CLABSI rates before and after implementation of preventive strategies (ie, training staff and caregivers in procedures for dressing changes and drawing blood) in the inpatient, outpatient, and non-health care (ie, home) settings. We also studied the pathogens associated with hospital-onset versus community-onset CLABSIs.

Survival and long-term neurodevelopmental outcome of the extremely preterm infant. A systematic review.

Survival rates among live births in North American tertiary perinatal centers since 1990 were 28% at 23 weeks, 52% at 24, 70% at 25 and 83% at 26 weeks. However, there is wide variation among centers. Survival rates in 2010 among tertiary centers in the United States participating in the Vermont-Oxford Network were 34% at 23 weeks, 61% at 24, 79% at 25, and 87% at 26.

Simplifying the diagnosis of 4 common voiding conditions using uroflow/electromyography, electromyography lag time and voiding history.

Purpose: Noninvasive uroflowmetry with simultaneous electromyography is useful to triage cases of lower urinary tract symptoms into 4 urodynamically defined conditions, especially when incorporating short and long electromyography lag times in the analysis. We determined the prevalence of these 4 conditions at a single referral institution and the usefulness of uroflowmetry with simultaneous electromyography and electromyography lag time to confirm the diagnosis, guide treatment and monitor response.

Materials And Methods: We retrospectively reviewed the records of 100 consecutive normal children who presented with persistent lower urinary tract symptoms, underwent uroflowmetry with electromyography as part of the initial evaluation and were diagnosed with 1 of 4 conditions based on certain uroflowmetry/electromyography features.

Testicular symmetry and adolescent varicocele--does it need followup?

Purpose: Appropriate management for adolescent varicocele with testicular symmetry is rarely discussed. We examined the natural history of varicocele in patients presenting with testicular symmetry to achieve better understanding of the clinical course.

Materials And Methods: Our varicocele registry was queried for adolescent boys who presented with varicocele in association with less than 15% testicular asymmetry and who underwent at least 1 testicular asymmetry assessment 12 or more months later.

Prenatal lung-head ratio: threshold to predict outcome for congenital diaphragmatic hernia.

Objective: The literature suggests that lung-head ratio (LHR) and liver position may inconsistently predict outcome for congenital diaphragmatic hernia (CDH). We reviewed our inborn neonates with isolated left-sided CDH to determine whether these variables predicted survival and to estimate the optimal LHR threshold.

Methods: Prenatal LHR and liver position were obtained from 2002 to 2009.

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study.

Introduction: Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates.

Primary squamous cell carcinoma arising at a gastrostomy tube site.

Gastrostomy tubes are commonly used in children with feeding impairment. Postoperative complications such as the formation of granulation tissue are common and represent a type of chronic nonhealing wound. We present a case of a 24-year-old man with no history of malignancy who developed a primary cutaneous squamous cell carcinoma at a long-term gastrostomy tube site.

Congenital adrenal hyperplasia: an update in children.

Purpose Of Review: Congenital adrenal hyperplasia (CAH) in children, the majority of which is due to 21-hydroxylase deficiency, represents a group of disorders in which there is impaired cortisol synthesis and abnormalities in adrenal hormonal profiles. There continues to be debate regarding the optimal management of and treatment for these children. This review will highlight the most recent advances in neonatal screening for CAH, as well as the timeliest recommendations for the treatment and management of 21-hydroxylase deficiency, both the classic and nonclassic forms of the disorder.

Impact of fetal cardiac intervention on congenital heart surgery.

Fetal cardiac interventions performed by interventional cardiologists are currently in a clinical experimental phase. The 3 most frequent interventions are: 1) aortic balloon valvuloplasty for critical aortic stenosis with a small left ventricle or with a normal size left ventricle but poor function; 2) atrial septostomy for highly restrictive or intact atrial septum in hypoplastic left heart syndrome; and 3) pulmonary valvuloplasty for pulmonary atresia and hypoplastic right ventricle. Current impact on everyday congenital heart surgery practice is small, but may increase in the future.

Current and novel therapeutics in the treatment of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with significant clinical heterogeneity. Recent advances in our understanding of the genetic, molecular, and cellular bases of autoimmune diseases and especially SLE have led to the application of novel and targeted treatments. Although many treatment modalities are effective in lupus-prone mice, the situation is more complex in human subjects.

Ablation of supraventricular tachycardia allows more liberal therapy in some children with attention-deficit-hyperactivity disorder.

Background: First-line therapy for children with attention-deficit-hyperactivity disorder (ADHD) is stimulant medication, which may have potential cardiovascular side-effects. In patients with supraventricular tachycardia or Wolf-Parkinson-White syndrome (WPW), therapy for ADHD could become challenging. The purpose of the present study was to review the authors' experience of performing electrophysiologic study (EPS) with or without ablation to determine how it affected ADHD therapy.

Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease.

Purpose: Thoracoscopic lobectomy for congenital cystic lung lesions is an accepted technique in pediatric surgery. Since an increasing number of these lesions are detected prenatally, the safety and efficacy of infant resections have been questioned. We reviewed our experience over a 10-year period to evaluate early resection of these lesions.

The role of posterior spinal osteotomies in pediatric spinal deformity surgery: indications and operative technique.

The primary goals of spinal deformity surgery are to prevent the progression of further deformity and to improve sagittal and coronal balance. Although increasingly powerful instrumentation has greatly facilitated these treatment goals, osteotomy of the spine is sometimes necessary to address more significant deformity. This review provides a detailed overview of posterior spinal osteotomies, which have gained increased attention as an adjunct to the treatment of complex pediatric spine deformity.

Clinical and radiological distinction between spondylothoracic dysostosis (Lavy-Moseley syndrome) and spondylocostal dysostosis (Jarcho-Levin syndrome).

In 1938, Saul Jarcho and Paul Levin from Johns Hopkins Hospital reported cases of thoracic insufficiency due to vertebral and rib anomalies. Nearly 30 years later, in 1966, Norman Lavy and associates from Indiana University reported a similar syndrome in a family from Puerto Rico. Lavy's description was followed by a report by John E.

Clinical research careers: reports from a NHLBI pediatric heart network clinical research skills development conference.

Background: Wyman W. Lai, MD, MPH, and Victoria L. Vetter, MD, MPH.

Role of immunosuppression regimen in post-transplant lymphoproliferative disorder in pediatric heart transplant patients.

Background: Post-transplant lymphoproliferative disorder (PTLD) contributes to morbidity and mortality after transplantation. We examined the effect of immunosuppressive regimen on the risk of developing PTLD after pediatric heart transplantation.

Methods: The 324 pediatric heart transplant patients at 2 children's hospitals were retrospectively reviewed for the primary outcome of PTLD development.

Arrhythmogenic causes of chest pain in children.

Although cardiac causes of chest pain in children are infrequent, arrhythmias are implicated in most cardiac related cases. The most common arrhythmias associated with chest pain are supraventricular tachycardias, but more ominous rhythms, such as ventricular tachycardia or bradycardias, can manifest as chest pain. Investigation of all children with chest pain suspected of arrhythmia should include detailed history and physical examination and a 12- or 15-lead electrocardiogram.

Long-duration (>4 weeks) continuous renal replacement therapy in critical illness.

Introduction: Decreased pediatric survival has been reported with long-duration (>4 weeks) continuous renal replacement therapy (CRRT), though the practice has not been well-described.

Methods: Retrospective chart review in a children's hospital of all patients treated with CRRT over 2 years (2003-4), including those who underwent long (group 1) and shorter duration (group 2) therapy.

Results: We identified 39 patients: median age was 6 years (range: 0.

Limb ischemia after common femoral artery cannulation for venoarterial extracorporeal membrane oxygenation: an unresolved problem.

Purpose: Extracorporeal Life Support Organization Registry data confirm that the number of pediatric patients being supported by extracorporeal membrane oxygenation (ECMO) is increasing. To minimize the potential neurologic effects of carotid artery ligation, the common femoral artery (CFA) is frequently being used for arterial cannulation. The cannula has the potential for obstructing flow to the lower limb, thus increasing ischemia and possible limb loss.

Update on the management of adolescent varicocele.

The management of adolescent varicocele has evolved over the last 30 years. This article briefly reviews some of the current issues regarding the adolescent varicocele, including (a) the progressive nature of the adolescent varicocele, (b) the degree of asymmetry that necessitates surgery, (c) the wide variability in percentage of repairs performed bilaterally in published adolescent series, and (d) the choice of surgical procedure to be performed.