Cobalamin C disease presenting as hemolytic-uremic syndrome in the neonatal period.

Anew case of cobalamin C disease associated with hemolytic-uremic syndrome (HUS) in the neonatal period is described. A 28-day-old boy presented with failure to thrive, hypotonia, pancytopenia, and features of HUS (microangiopathic hemolytic anemia, thrombocytopenia, and renal failure). The possibility of the diagnosis of an underlying vitamin B12 disorder was prompted by evidence of megaloblastic changes on the peripheral smear and by finding in the literature a suggested association of neonatal HUS with this cobalamin-related metabolic disorder.

Electrogastrographic norms in children: toward the development of standard methods, reproducible results, and reliable normative data.

Background: Surface electrogastrography (EGG) is a noninvasive technique that detects gastric myoelectrical electric activity, principally the underlying pacemaker activity generated by the specialized interstitial cells of Cajal. Interest in the use of this methodology has grown because of its potential applications in describing functional gastrointestinal disorders, particularly as a tool in the evaluation of nausea, anorexia, and other dyspeptic symptoms.

Methods: Fifty-five healthy volunteers (27 female), ranging in age from 6 to 18 years (mean, 11.

Effects of quality of energy intake on growth and metabolic response of enterally fed low-birth-weight infants.

Carbohydrate and fat may vary in their ability to support protein accretion and growth. If so, variations in the source of nonprotein energy might be used to therapeutic advantage in enterally fed low-birth-weight infants. To test the hypothesis that high-carbohydrate diets are more effective than isocaloric high-fat diets in promoting growth and protein accretion, low-birth-weight infants weighing 750-1600 g at birth were randomized in a double blind study to receive one of five formulas differing only in the quantity and quality of nonprotein energy.

Does cisapride influence cardiac rhythm? Results of a United States multicenter, double-blind, placebo-controlled pediatric study.

Background: Major concerns about serious cardiac side effects underlie the recent decision by the FDA and Janssen Pharmaceutica (Titusville, NJ) to make cisapride available only through a limited access program. Concerns have grown despite the fact that most instances of prolonged QTc and other ventricular arrhythmias occurred while the drug was used concomitantly with contraindicated drugs. This study sought to analyze electrocardiograms (ECGs) from a multicenter pediatric study and to identify abnormalities in QTc interval associated with cisapride use.

Novel therapeutics for the treatment of paediatric pulmonary arterial hypertension.

The treatment of paediatric pulmonary arterial hypertension is challenging due to the serious nature of the disease, its rapid progression and the limited treatment options available. However, recent advances in the treatment of pulmonary arterial hypertension may offer significant improvements for patients suffering from this condition. Novel treatment options include prostacyclin analogues and endothelin receptor antagonists.

Bulboventricular foramen resection: hemodynamic and electrophysiologic results.

Background: The two major surgical approaches to the relief of bulboventricular foramen (BVF) obstruction in patients with single left ventricle (LV) are the Damus-Kaye-Stansel (DKS) procedure or direct BVF resection. Theoretical advantages of the DKS include better out-flow gradient relief, lower potential incidences of postoperative heart block and lower incidences of reoperation. Potential disadvantages of this approach include increased semilunar valvar insufficiency, lack of feasibility when attempting septation-type operations for univentricular hearts, and a technically more difficult operation.

Nursing considerations of the neonate with congenital heart disease.

Nursing care of the neonate with CHD incorporates a knowledge base of the anatomy and physiology of congenital heart defects, surgical repair, complications associated with CHD, diagnostic testing, medical therapy, and psychosocial support. The neonatal nurse is a vital member of the cardiac team in providing accurate assessments, implementing medical therapies, and supporting the family.

Prevalence and reversibility of lower airway obstruction in children with sickle cell disease.

Objective: To determine the prevalence and reversibility of lower airway obstruction (LAO) in children and adolescents with hemoglobin SS sickle cell disease (HbSS SCD).

Study Design: Retrospective evaluation of lung function in a cross-section of 35 African American and 28 Hispanic children and adolescents with HbSS SCD. Lung function was evaluated with maximal respiratory flow-volume curves and body plethysmography.

Use of unconventional therapies by children with cancer at an urban medical center.

Purpose: The aim of this study was to determine the prevalence, modalities, and determinants of use of unconventional therapies among children with cancer receiving conventional treatment at an urban academic medical center in the United States.

Patients And Methods: We interviewed the parents of patients and/or patients who were receiving or had received conventional therapy for treatment of childhood cancer. Of 78 patients/ parents asked, 75 consented to the interview, which included demographic factors, life events, and use of unconventional therapies.

Continuous intravenous diltiazem infusion for short-term ventricular rate control in children.

Intravenous diltiazem was administered to 10 pediatric patients with primary atrial tachyarrhythmias with rapid ventricular response. Rapid, consistent, and safe temporary ventricular rate control was obtained in all patients given this medication.

Thalamic and hypothalamic tumors of childhood: endocrine late effects.

Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.

A new technique for endomyocardial biopsy in infants and small children.

Endomyocardial biopsy (EMB) is usually performed from the femoral veins in infants and small children. This requires the use of a long vascular sheath to direct a bioptome to the ventricular septum. We describe a technique of EMB in this population that obviates the need for the use of a long vascular sheath.

Continuous positive airway pressure (CPAP).

Progress in neonatal intensive care is closely linked to improvements in the management of respiratory failure in small infants. This applies to the care of the preterm infants with immature lungs, and also to treatment of the preterm or full term infants with specific diseases that are associated with respiratory failure. Respiratory distress of the newborn continues to account for significant morbidity in the intensive care unit.

Anti-VEGF antibody suppresses primary tumor growth and metastasis in an experimental model of Wilms' tumor.

Background/purpose: Vascular endothelial growth factor (VEGF) has been shown previously to correlate with tumor growth and metastasis in an experimental model of anaplastic Wilms' tumor. The authors hypothesized that treatment with anti-VEGF antibodies would suppress both primary tumor growth and metastasis in this model.

Methods: Tumors were induced in the right kidneys of nude mice by the injection of cultured Wilms' tumor cells.

Randomised controlled trial of thiopental for intubation in neonates.

Aims: To determine the effects of premedication with thiopental on heart rate, blood pressure, and oxygen saturation during semi-elective nasotracheal intubation in neonates.

Methods: A randomised, placebo controlled, non-blinded study design was used to study 30 neonates (mean birthweight 3.27 kg) requiring semi-elective nasotracheal intubation.

Pathological angiogenesis in a murine model of human Wilms' tumor.

Background/purpose: Pathological vascular architecture is a feature of neoangiogenic processes such as diseases of the retina and tumor growth. The authors hypothesized that experimental human Wilms' tumors would display a vascular architecture similar to retinal diseases that are driven by vascular endothelial growth factor (VEGF).

Methods: Human Wilms' tumors were established in the right kidneys of nude mice.

Should people with nocturnal leg cramps drink tonic water and bitter lemon?

A literature search from 1993 to 1997 using MEDLINE and key-words beverages, muscle cramp, and quinine was performed. Three beverages containing quinine were examined in grocery stores. Analysis indicate that leg cramps are a common phenomenon associated with many comorbid disorders especially peripheral vascular and neurologic disorders.

Pervasive developmental delay in children presenting as possible hearing loss.

Objective/hypothesis: Children who fail to develop adequate language skills and/or appropriate social skills by age 2 years often are referred to the department of otolaryngology for otolaryngologic examination and evaluation of possible hearing deficits. Discovering a gross disparity between hearing function and language ability often uncovers an underlying developmental disorder satisfying criteria for diagnosis on the spectrum of autism and pervasive developmental delay (PDD). The otolaryngologist has a unique opportunity to identify these autistic children and initiate their evaluation and management.

Retropharyngeal abscess and Epstein-Barr virus infection in children.

A 6-year retrospective chart review was conducted of all pediatric patients with the diagnosis of retropharyngeal abscess (RPA) at the Columbia-Presbyterian Medical Center. The charts were examined for signs, symptoms, and serologic findings consistent with recent acute Epstein-Barr virus (EBV) infection. Of the 7 patients we studied, 4 had elevated immunoglobulin G antibodies that were consistent with recent acute EBV infection, and 3 patients were not tested.

Ameliorating anthracycline cardiotoxicity in children with cancer: clinical trials with dexrazoxane.

Anthracyclines have major activity against a broad range of childhood cancers. Concern over the risk of long-term cardiotoxicity associated with their use has called into question the role of these agents in the frontline treatment of many patients. Dexrazoxane was developed as a specific cardioprotectant "antidote" which can prevent anthracycline cardiotoxicity without inhibiting its antitumor effect.