MAPK phosphatases--regulating the immune response.

Mitogen-activated protein kinase (MAPK) phosphatases (MKPs) are protein phosphatases that dephosphorylate both the phosphothreonine and phosphotyrosine residues on activated MAPKs. Removal of the phosphates renders MAPKs inactive, effectively halting their cellular function. In recent years, evidence has emerged that, similar to MAPKs, MKPs are pivotal in the regulation of immune responses.

Impairment of reaction time among children awakened during stage 4 sleep.

Study Objective: Sleep inertia refers to impairments in cognitive/motor performance and the tendency to return to sleep after awakening. As part of a project studying the effect of different types of 100 decibel (dB) sounds on awakening children during stage 4 sleep (S4S), we hypothesized that children with the greatest impairment in auditory arousal during S4S, manifested by the inability to either awaken to 100 dB sounds or successfully perform a self-rescue sequence (SRS), would have greater impairments in reaction time (RT) upon awakening than children who awoke and successfully accomplish a SRS.

Design: Observational study.

Physical and emotional health of mothers of youth with functional abdominal pain.

Objective: To determine if mothers of youth with functional abdominal pain (FAP) experience more anxiety, depressive, and somatic symptoms and disorders than mothers of unaffected children.

Design: Case-control study.

Setting: Four primary care pediatric practices in western Pennsylvania.

Assessing heparin dosing in neonates on venoarterial extracorporeal membrane oxygenation.

We studied 12 consecutive neonates placed on venoarterial extracorporeal membrane oxygenation (ECMO) in 2004-2005. Activated clotting times (ACT) and anti-factor Xa levels were measured, and the corresponding heparin drip rate was noted. The mean heparin drip rate was 42.

Efficacy and safety of extended release metoprolol succinate in hypertensive children 6 to 16 years of age: a clinical trial experience.

Objective: To evaluate the efficacy, tolerability, and blood pressure (BP) lowering effect of extended release metoprolol succinate (ER metoprolol) in children 6 to 16 years of age with established hypertension.

Study Design: Patients were randomized to one of four treatment arms: placebo or ER metoprolol (0.2 mg/kg, 1.

Diurnal variation in the chemical clearance of acid gastroesophageal reflux in infants.

Background & Aims: Clearance of acid gastroesophageal reflux is biphasic. During volume clearance, refluxed material is cleared from the esophagus by peristalsis, and during chemical clearance, acidified esophageal mucosa is neutralized by saliva and possibly secretions from the esophageal lumen. In this study, we examined the effects of feeding on the durations of volume clearance and chemical clearance.

Combined clotting factor deficiencies: experience at a single hemophilia treatment center.

We describe a series of patients with combined factor deficiencies and von Willebrand's disease (VWD) at one haemophilia treatment centre. Although the incidence of VWD is at least 1% in the general population, combined coagulation defects have been infrequently described in the medical literature and are likely under diagnosed. This entity should be considered in patients with a known factor deficiency and either an unexpectedly severe bleeding phenotype, or bleeding that is unresponsive to factor replacement.

Anxiety and pain symptoms in children with inflammatory bowel disease and functional gastrointestinal disorders undergoing colonoscopy.

Objective: To compare pain and anxiety scores between children with inflammatory bowel disease (IBD) and those with functional gastrointestinal disorders (FGID) undergoing colonoscopy and to examine the role of anxiety and procedure length in predicting reports of pain.

Materials And Methods: Children ages 10 to 18 years undergoing colonoscopy (general anesthesia) for the first time completed pain and anxiety questionnaires immediately before the procedure and a pain questionnaire 48 hours after colonoscopy. The diagnosis was determined by chart review and physician interview.

A history of robots: from science fiction to surgical robotics.

Surgical robotics is an evolving field with great advances having been made over the last decade. The origin of robotics was in the science-fiction literature and from there industrial applications, and more recently commercially available, surgical robotic devices have been realized. In this review, we examine the field of robotics from its roots in literature to its development for clinical surgical use.

The congenital muscular dystrophies: recent advances and molecular insights.

Over the past decade, molecular understanding of the congenital muscular dystrophies (CMDs) has greatly expanded. The diseases can be classified into 3 major groups based on the affected genes and the location of their expressed protein: abnormalities of extracellular matrix proteins (LAMA2, COL6A1, COL6A2, COL6A3), abnormalities of membrane receptors for the extracellular matrix (fukutin, POMGnT1, POMT1, POMT2, FKRP, LARGE, and ITGA7), and abnormal endoplasmic reticulum protein (SEPN1). The diseases begin in the perinatal period or shortly thereafter.

Limited value of plain radiographs in infant torticollis.

Objective: The purpose of this work was to assess the frequency of clinically relevant findings from plain films of infants evaluated for torticollis.

Patients And Methods: After institutional review board approval, radiology records were searched for infants 0 to 12 months of age who underwent plain film study for torticollis or "head tilt." Infants evaluated for trauma or Down syndrome were excluded.

History and development of trauma registry: lessons from developed to developing countries.

Background: A trauma registry is an integral component of modern comprehensive trauma care systems. Trauma registries have not been established in most developing countries, and where they exist are often rudimentary and incomplete. This review describes the role of trauma registries in the care of the injured, and discusses how lessons from developed countries can be applied toward their design and implementation in developing countries.

Transcatheter devices used in the management of patients with congenital heart disease.

The availability of transcatheter devices has revolutionized the management of patients with congenital heart and vascular disease. Many patients that were in the past exclusively treated through open heart surgical procedures can now be managed using a percutaneous approach. This article describes those devices that are, at present, most frequently used in the USA for transcatheter therapy of congenital heart disease, which have, or are about to receive, US premarket approval.

Pulmonary hyalinizing granuloma and retroperitoneal fibrosis in an adolescent.

We describe a 15-year-old boy who developed pulmonary hyalinizing granuloma (PHG) and retroperitoneal fibrosis (RPF). His PHG and RPF were not associated with histoplasmosis or tuberculosis and appeared to represent idiopathic autoimmune phenomena. This is the first reported case of PHG in a pediatric patient and the fourth reported co-occurrence of PHG and RPF.

Unique application of multislice computed tomography in adults with congenital heart disease.

Purpose: Adults with congenital heart disease (CHD) form a growing population of patients requiring coronary artery assessment. In light of their increased incidence of coronary anomalies and need for reoperations, it is important to define their coronary anatomy. The purpose of this study was to define the spectrum of coronary artery anatomies and feasibility of coronary artery delineation in adults with CHD using 16- and 64-slice computed tomography angiography.

Acid gastroesophageal reflux reports in infants: a comparison of esophageal pH monitoring and multichannel intraluminal impedance measurements.

Twenty-four-hour tracings generated from combined esophageal pH and multichannel intraluminal impedance measurements of 14 infants (nine males, median age 3.5 months) were examined retrospectively. For each tracing, two acid reflux assessment reports were generated using either pH monitoring alone or pH monitoring combined with impedance.

Radiofrequency ablation of a cervical osteoid osteoma: a trans-thyroid approach.

We present a 16-year-old girl with an osteoid osteoma in the vertebral body of C5. Given the difficulty of surgically treating the lesion, radiofrequency ablation was used. Percutaneous needle access to this lesion in the anterior aspect of the cervical vertebral body was limited by the many critical adjacent structures.

Reconstructive surgery of the upper urinary tract.

This paper reviews the major publications over the past year regarding upper urinary tract reconstruction. Controversies in the diagnosis of ureteropelvic junction obstruction and in the surgical repair of the obstructed upper urinary tract are discussed. Special emphasis is placed on issues surrounding minimally invasive techniques.

Pediatric precursors of adult cardiovascular disease: noninvasive assessment of early vascular changes in children and adolescents.

Background: Until recently, our understanding of the childhood antecedents of adult cardiovascular disease was limited mainly to autopsy studies and pathologic findings in teens and young adults who died from accidental causes. Recent advances in the understanding of atherosclerosis and new technologies allowing detection of early events have made it possible to observe anatomic and physiologic evidence of cardiovascular disease in young adults and children.

Objectives: The goal of this article was to introduce pediatricians to new methods for noninvasive measurement of cardiovascular disease and its precursors, to describe the potential application of these techniques in detecting childhood precursors of adult cardiovascular disease, and to summarize knowledge gained from this approach.

Renal anomalies in family members of infants with bilateral renal agenesis/adysplasia.

Renal agenesis/adysplasia is the leading etiology of end stage renal disease in children. The etiology for renal agenesis/adysplasia has not been identified. The purpose of the present study was to determine if renal agenesis/adysplasia occur in a familial pattern.

Injury outcomes in children following automobile, motorcycle, and all-terrain vehicle accidents: an institutional review.

Object: The authors performed an analysis of retrospectively obtained data to compare the outcomes of pediatric patients admitted to their institution for traumatic injuries resulting from car, motorcycle, and all-terrain vehicle (ATV) accidents.

Methods: The authors conducted a retrospective review of Columbus Children's Hospital's Trauma Registry data collected between January 1993 and December 2003. Data obtained in patients admitted with motor vehicle-related injuries were compiled for a total of 1608 patient records.

Image-guided interventions in neonates.

Minimally invasive interventional radiological procedures can be invaluable in the care of neonates and infants. These procedures have proven to be useful in a wide variety of clinical situations, improving patient care, comfort and safety. Most techniques in adult interventional radiology have been adapted for use in pediatric patients, covering the spectrum of diagnostic and therapeutic intervention.

Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.

A 4-year-old girl with pyruvate dehydrogenase deficiency, static encephalopathy, and seizure disorder treated with the ketogenic diet presented in severe diabetic ketoacidosis. Pyruvate dehydrogenase deficiency is a rare genetic defect of mitochondrial energy metabolism that leads to inefficient glucose use and lactic acidosis. The ketogenic diet provides the brain with an alternate fuel source, but its implementation opposes traditional diabetes management.

Using HFMEA to assess potential for patient harm from tubing misconnections.

Background: Reported cases of tubing misconnections and other tubing errors prompted Columbus Children's Hospital to study their potential for harm in its patient population. A Health Failure Mode and Effects Analysis (HFMEA) was conducted in October 2004 to determine the risks inherent in the use and labeling of various enteral, parenteral, and other tubing types in patient care and the potential for patient harm.

Methods: An assessment of the practice culture revealed considerable variability among nurses and respiratory therapists within and between units.