Endothelial nitric oxide synthase polymorphisms associated with abnormal nitric oxide production are not over-represented in children with Down syndrome.

Objective: Down syndrome patients are at increased risk for developing pulmonary hypertension (PHTN). Nitric oxide (NO) is an important factor for pulmonary vasoreactivity. Various endothelial nitric oxide synthase (eNOS) polymorphisms have been shown to affect NO.

Palliation via hybrid procedure of a 1.4-kg patient with a hypoplastic left heart.

Despite improvements in survival of patients with hypoplastic left heart syndrome (HLHS) with various palliative procedures, certain risk factors, such as weight less than 2.5 kg, continue to predict increased mortality. We report the palliation of a patient with HLHS weighing 1.

Treatment of Susac's Syndrome.

Susac's syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. It usually affects women aged 20 to 40, but men are also affected, and the age range extends from 9 to 72 years. It tends to be unrecognized, even in major academic centers.

Adequacy of two ambulatory care surveillance systems for tracking childhood obesity practice patterns.

Background: The National Ambulatory Medical Care Surveys (NAMCS) and National Hospital Ambulatory Medical Care Surveys (NHAMCS) are surveillance systems in the USA that track provider practice patterns at ambulatory care visits. This study investigated the adequacy of the NAMCS/NHAMCS for surveillance of childhood obesity practice patterns.

Methods: The frequency of obesity visits in the 1997-2000 NAMCS/NHAMCS (outpatient component) was compared with obesity prevalence among children who reported a physician visit in the preceding 12 months in the National Health and Nutrition Examination Survey (NHANES) 1999-2000.

In vitro drug adsorption and plasma free hemoglobin levels associated with hollow fiber oxygenators in the extracorporeal life support (ECLS) circuit.

The purpose of this study was to identify the percentage of fentanyl or morphine sulfate lost from adhesion to either the polyvinylchloride (PVC) tubing or the surface of two different hollow fiber oxygenators used in current extracorporeal life support circuits and to identify any difference in the plasma free hemoglobin (PFH) levels generated when using these oxygenator and/or drug combinations. For each drug examined, six simple circuits were assembled; for each drug, two circuits contained tubing without an oxygenator (control), two circuits contained the Jostra Quadrox D (Maquet Cardiopulmonary, AG Hirrlingen, Germany), and two circuits contained the Terumo Baby Rx (Terumo Cardiovascular Systems Corp., Ann Arbor, MI).

Stenting complex aortic arch obstructions.

Objective: To present our institutional experience of endovascular stent therapy in patients with complex aortic arch lesions.

Background: The management of aortic arch obstructions is complex and many physicians are either reluctant to treat mild-moderate aortic arch lesions associated with systemic hypertension or instead refer to high-risk surgical alternatives. Reported data on transcatheter stent therapy of complex aortic arch lesions are scarce.

A review of the management of two common clinical problems found in patients with cystic fibrosis: cystic fibrosis-related diabetes and poor growth.

Background: Cystic fibrosis-related diabetes (CFRD) and glucose intolerance often occur in teens and adults with CF. The initial deficiency is an impaired first-phase insulin response; as patients age, peak insulin response is delayed and less robust than normal. Decreased insulin sensitivity (insulin resistance) is also present in patients with CF.

Triple diabetes: coexistence of type 1 diabetes mellitus and a novel mutation in the gene responsible for MODY3 in an overweight adolescent.

We report an interesting and unique case of an overweight adolescent with a novel mutation of the maturity-onset diabetes of the young (MODY)3 gene [hepatocyte nuclear factor-1 alpha (HNF-1alpha)] and positive islet cell autoantibodies. The patient is a 17-yr-old Caucasian female, who was diagnosed with type 2 diabetes mellitus, treated with metformin and glipizide, with poor control for 18 months prior to being referred to the Endocrinology clinic. Family history was strongly positive for type 2 diabetes (father, paternal aunts, uncles, and grandmother).

Serum markers of systemic right ventricular function and exercise performance.

The right ventricle, which faces systemic pressure due to congenital heart disease, often develops premature systolic dysfunction. Cardiac hormones might be useful to identify patients with such systolic dysfunction. This prospective study investigated the relationship of atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) levels with right ventricular ejection fraction (RVEF) and exercise performance in patients with a systemic right ventricle.

Frena tears and abusive head injury: a cautionary tale.

Tears of the lingual and labial frena have been associated with accidental and nonaccidental injury. Three cases of infants are presented who were evaluated in the hospital with frena tears which were not recognized as manifestations of abuse, discharged home, and subsequently returned with manifestations of severe abusive head injury.

Requirements for accurately diagnosing chronic partial upper urinary tract obstruction in children with hydronephrosis.

Successful management of hydronephrosis in the newborn requires early accurate diagnosis to identify or exclude ureteropelvic junction obstruction. However, the presence of hydronephrosis does not define obstruction and displays unique behavior in the newborn. The hydronephrotic kidney usually has nearly normal differential renal function at birth, has not been subjected to progressive dilation and except for pelvocaliectasis does not often show signs of high-grade obstruction.

Isolated fallopian tube torsion: sonographic and CT features.

Background: Isolated fallopian tube torsion (IFTT) rarely occurs in pediatric patients and is difficult to diagnose preoperatively.

Objective: To determine the common sonographic and CT findings in girls with IFTT.

Materials And Methods: We retrospectively reviewed the hospital charts and imaging studies of the eight girls diagnosed with and treated for IFTT at Columbus Children's Hospital between January 1995 and June 2006 whose final diagnosis was IFTT.

Pharyngeal swallowing: defining pharyngeal and upper esophageal sphincter relationships in human neonates.

Objective: To test the hypothesis that the sensorimotor characteristics of the reflexes evoked on stimulation with air and water infusions differ by studying the effect of pharyngeal stimulation on pharyngeal-upper esophageal sphincter (UES) interactions in healthy neonates.

Study Design: Pharyngo-UES-esophageal manometry was recorded in 10 neonates at 39 +/- 4 weeks postmenstrual age. Pharyngeal infusions (n = 155) of air (0.

Current therapies in childhood and adolescent migraine.

This review summarizes recent guidelines for diagnosis of migraine in children and adolescents and inherent issues regarding their headaches that influence evaluation of therapies. With the shorter duration of pediatric migraine attacks and the prominent placebo responder rate, design of randomized clinical trials becomes more problematic than in the adult population. Regarding abortive therapies, several agents have appreciable efficacy in randomized controlled trials.

Effects of ischemic preconditioning and adenosine pretreatment on myocardial function and energetics in a clinically relevant model.

Preconditioning (PC) is a potential approach to myocardial protection. We hypothesize that brief ischemia or adenosine given prior to an extended period of warm ischemia may prevent myocardial stunning by altering myocardial metabolism. Using a global ischemia model, 19 dogs were subjected to no PC(control), two episodes of ischemia (2 min of global ischemia followed by 3 min of reperfusion) (IPC), or 30 min of pulmonary artery adenosine infusion (AP), to a maximum of 350 microg/kg/min, followed by 20 min of global warm ischemia on cardiopulmonary bypass.

Biomarker discovery in human SLE nephritis.

The treatment of systemic lupus erythematosus (SLE) nephritis, while effective, is associated with significant morbidity and mortality. These side effects could be mitigated if the onset, severity, and response of renal flare could be predicted, and therapy modified accordingly. In this review, an approach to derive prediction equations of SLE nephritis flare is discussed.

Use of wound V.A.C. therapy in pediatric patients with infected spinal wounds: a retrospective review.

Background: Deep wound infection in patients that have had a posterior spinal fusion for scoliosis is a major complication in pediatric spine surgery.

Purpose: To explore characteristics of pediatric patients who had a posterior spinal fusion with segmental spinal instrumentation and bone graft and subsequently developed deep spinal wound infections that were treated with wound vacuum-assisted closure (V.A.

Ventriculosubgaleal shunts at Columbus Children's Hospital: Neurosurgical implant placement in the neonatal intensive care unit.

Object: The authors review all cases in which ventriculosubgaleal (VSG) shunts were placed at Columbus Children's Hospital for the treatment of posthemorrhagic hydrocephalus in order to assess the surgical procedure, effectiveness of surgery, and complications of cerebrospinal fluid diversion to the subgaleal space. The purpose of the review is to make a comparison between cases in which shunts were placed in the operating room (OR) and those in which they were placed in the neonatal intensive care unit (NICU). Considerations and complications specific to patient transport to the OR or surgical implantation in the NICU are discussed.

Bench-to-bedside review: Developmental influences on the mechanisms, treatment and outcomes of cardiovascular dysfunction in neonatal versus adult sepsis.

Sepsis is a significant cause of morbidity and mortality in neonates and adults, and the mortality rate doubles in patients who develop cardiovascular dysfunction and septic shock. Sepsis is especially devastating in the neonatal population, as it is one of the leading causes of death for hospitalized infants. In the neonate, there are multiple developmental alterations in both the response to pathogens and the response to treatment that distinguish this age group from adults.

Improving processes of care in patient-controlled analgesia: the impact of computerized order sets and acute pain service patient management.

Background: In an effort to combat opioid induced side effects within the first 24 h of patient-controlled analgesia (PCA) induction, it has been recommended that care be provided by an Acute Pain Treatment Service (APS) and that computerized PCA order sets with patient monitoring requirements be implemented. To date, there are few published studies on the role of computerized order sets or APS in improving the quality and safety of pediatric PCA use. This retrospective analysis sought to determine if the implementation of computerized order sets would increase appropriate monitoring and problem recognition.

A translational approach for limb vascular delivery of the micro-dystrophin gene without high volume or high pressure for treatment of Duchenne muscular dystrophy.

Background: Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder with monogenic mutations setting the stage for successful gene therapy treatment. We have completed a study that directly deals with the following key issues that can be directly adapted to a gene therapy clinical trial using rAAV considering the following criteria: 1) A regional vascular delivery approach that will protect the patient from widespread dissemination of virus; 2) an approach to potentially facilitate safe passage of the virus for efficient skeletal muscle transduction; 3) the use of viral doses to accommodate current limitations imposed by vector production methods; 4) and at the same time, achieve a clinically meaningful outcome by transducing multiple muscles in the lower limb to prolong ambulation.

Methods: The capacity of AAV1, AAV6 or AAV8 to cross the vascular endothelial barrier carrying a micro-dystrophin cDNA was compared under identical conditions with delivery through a catheter placed in the femoral artery of the mdx mouse.

Acceptance of the use of diazepam rectal gel in school and day care settings.

This study was conducted to identify how often parents of children with epilepsy encounter barriers to the use of diazepam rectal gel in day care or school settings and how these barriers affect the child and family. Sixty-four parents completed an 18-item questionnaire documenting their experience with asking their child's school to administer diazepam rectal gel. No data regarding its actual use in the school was obtained.

A survey of parent satisfaction with pediatric neuropsychological evaluations.

Satisfaction with pediatric neuropsychological evaluations was surveyed by asking parents or guardians of children who completed pediatric neuropsychological evaluations at a large children's hospital over a 2-year period to complete a 30-item rating scale. The scale included items drawn from published measures of consumer satisfaction, and incorporated a well-validated measure of general satisfaction. A total of 338 surveys were distributed, with 117 completed, for a return rate of 35%.