Determining the longitudinal validity and meaningful differences in HRQL of the PedsQL™ Sickle Cell Disease Module.

Background: Detecting change in health status over time and ascertaining meaningful changes are critical elements when using health-related quality of life (HRQL) instruments to measure patient-centered outcomes. The PedsQL™ Sickle Cell Disease module, a disease specific HRQL instrument, has previously been shown to be valid and reliable. Our objectives were to determine the longitudinal validity of the PedsQL™ Sickle Cell Disease module and the change in HRQL that is meaningful to patients.

Inter-center variation in death or tracheostomy placement in infants with severe bronchopulmonary dysplasia.

Objective: To estimate the presence and sources of inter-center variation (ICV) in the risk of death or tracheostomy placement (D/T) among infants with severe bronchopulmonary dysplasia (sBPD)Study design:We analyzed the Children's Hospitals Neonatal Database between 2010 and 2013 to identify referred infants born <32 weeks' gestation with sBPD. The association between center and the primary outcome of D/T was analyzed by multivariable modeling. Hypothesized diagnoses/practices were included to determine if these explained any observed ICV in D/T.

Predicting death or tracheostomy placement in infants with severe bronchopulmonary dysplasia.

Objective: To estimate the risk of death or tracheostomy placement (D/T) in infants with severe bronchopulmonary dysplasia (sBPD) born < 32 weeks' gestation referred to regional neonatal intensive care units.

Study Design: We conducted a retrospective cohort study in infants born < 32 weeks' gestation with sBPD in 2010-2011, using the Children's Hospital Neonatal Database. sBPD was defined as the need for FiO2 ⩾ 0.

The effects of early parenteral amino acids on sick premature infants.

Objectives: To study the effects of early parenteral amino acid administration on body weight, fluid compartments and metabolic parameters during the first week of life in sick premature infants.

Methods: Appropriate for gestational age, sick premature infants were randomized into two groups. Group A infants (n=8, birth weight 1258±339 g) were supplemented with amino acids starting within 24 h of birth and advanced to 2.

Colonic volvulus in children with intestinal motility disorders.

Background: Chronic intestinal pseudo-obstruction (CIP) is a condition characterized by symptoms of bowel obstruction in the absence of an anatomical cause. Patients with CIP and chronic intractable constipation (CIC) can also develop anatomical obstruction, and the presenting symptoms mimic those of underlying pseudo-obstruction.

Objectives: Our objectives were to evaluate the incidence, clinical presentation, and diagnostic investigations of colonic volvulus in children with intestinal motility disorders and to differentiate these episodes of colonic volvulus from the underlying motility disorder based on clinical presentation and imaging techniques.

Superior mesenteric artery syndrome in children: a 20-year experience.

Objectives: Superior mesenteric artery syndrome (SMAS), Wilkie syndrome or cast syndrome is a rare condition that usually presents with symptoms of mid to upper gastrointestinal obstruction due to the compression of the duodenum between the abdominal aorta, posteriorly, and the superior mesenteric artery, anteriorly. The aim of this study was to analyze the clinical characteristics, means of diagnosis and management of SMAS in a pediatric population.

Methods: Retrospective chart review of all patients at the Children's Hospital of Wisconsin with SMAS from 1985 to 2005.

Safety of aprotinin use and re-use in pediatric cardiothoracic surgery.

Background: Hypersensitivity reactions to aprotinin have been reported in adult cardiac surgical patients undergoing initial and re-exposure to the medication. This study describes the incidence and impact of aprotinin hypersensitivity reactions in children undergoing cardiothoracic surgery.

Methods And Results: In this retrospective review of our entire experience with aprotinin (n=865), 681 first exposures, 150 second exposures, and 34 third or higher exposures were examined.

Esophageal biopsy does not predict clinical outcome after percutaneous endoscopic gastrostomy in children.

Clinically symptomatic gastroesophageal reflux may occur after percutaneous endoscopic gastrostomy (PEG). Preoperative evaluation for gastroesophageal reflux does not reliably predict those individuals who will develop reflux unresponsive to medical management after PEG. Esophageal histology at the time of PEG might be used to identify patients at risk for developing intractable gastroesophageal reflux.

What is the definition of cure for aplastic anemia?

Treatment with immune suppression and bone marrow transplantation has improved the response rates and survival of patients with aplastic anemia. Measurement of response requires that common endpoints be recorded at specific times. There has been no agreement on such parameters for patients with aplastic anemia.

Neuronal nitric oxide synthase mediates halothane-induced cerebral microvascular dilation.

Background: The causes of volatile anesthetic-induced cerebral vasodilation include direct effects on smooth muscle and indirect effects via changes in metabolic rate and release of mediators from vascular endothelium and brain parenchyma. The role of nitric oxide and the relative importance of neuronal and endothelial nitric oxide synthase (nNOS and eNOS, respectively) are unclear.

Methods: Rat brain slices were superfused with oxygenated artificial cerebrospinal fluid.