ENDOGLIN is dispensable for vasculogenesis, but required for vascular endothelial growth factor-induced angiogenesis.

ENDOGLIN (ENG) is a co-receptor for transforming growth factor-β (TGF-β) family members that is highly expressed in endothelial cells and has a critical function in the development of the vascular system. Mutations in Eng are associated with the vascular disease known as hereditary hemorrhagic telangiectasia type l. Using mouse embryonic stem cells we observed that angiogenic factors, including vascular endothelial growth factor (VEGF), induce vasculogenesis in embryoid bodies even when Eng deficient cells or cells depleted of Eng using shRNA are used.

Pharmacologic therapy of heart failure in children: part of a special series on paediatric pharmacology, guest edited by Gianvincenzo Zuccotti, Emilio Clementi, and Massimo Molteni.

Paediatric cardiomyopathy and heart failure are distinct but frequently associated conditions, which have a high mortality. Traditional medical therapy has evolved to incorporate newer classes of heart failure drugs, although the evidence to support efficacy in children is limited. This perspective article discusses the rationale, benefits and limitations of the various classes of drug therapy used in paediatric heart failure due to cardiomyopathy or congenital heart disease.

Toxic megacolon in children with inflammatory bowel disease: clinical and radiographic characteristics.

Background: Toxic megacolon (TMC) denotes a rare clinical syndrome accompanied by colonic dilatation, and is a serious complication of inflammatory bowel disease (IBD). This study assessed the clinical and radiologic characteristics of TMC in children with IBD.

Methods: A systematic search identified patients with IBD-associated TMC and matched them by age to controls with ulcerative colitis without evidence of TMC.

Outcomes and associated risk factors for aortic valve replacement in 160 children: a competing-risks analysis.

Background: We sought to define patient characteristics, outcomes, and associated risk factors after aortic valve replacement (AVR) in children.

Methods And Results: Clinical records from children undergoing AVR from 1974 to 2004 at our institution were reviewed. Competing-risks methodology determined the time-related prevalence of 3 mutually exclusive end states: death, repeated replacement, and survival without subsequent AVR and their associated risk factors.

Can Canada sustain paediatric phase I trials? A national survey of cancer relapse in children.

Background: Paediatric phase I trials are critical to the evaluation of new agents using standardized methodology. However a large proportion of paediatric patients in Canada do not have access to phase I therapy.

Objectives: A National Paediatric Cancer Relapse Survey was conducted to collect preliminary data to evaluate the feasibility of multi-centre paediatric phase I trials within Canada.

Three-dimensional echocardiography improves the understanding of left atrioventricular valve morphology and function in atrioventricular septal defects undergoing patch augmentation.

Objectives: We sought to address the role of 3-dimensional echocardiography in the evaluation of the left atrioventricular valve in children with an atrioventricular septal defect who underwent patch augmentation of their valve for either regurgitation or left ventricular outflow tract obstruction.

Methods: Five children whose ages ranged between 4.5 and 9.

Management of a patient with an accessory maxilla and congenital facial fistula.

Although accessory jaws are a rare occurrence, the presence of such accessory tissue may cause some bothersome symptoms. This case report helps identify these unusual developmental lesions so that dentists can refer such patients for definitive care and management.

Glycine conjugation of para-aminobenzoic acid (PABA): a pilot study of a novel prognostic test in acute liver failure in children.

Background: Fulminant hepatic failure (FHF) is associated with high mortality; few patients survive without liver transplantation. It is important to have a sensitive, specific early predictor of outcome to distinguish potential survivors (S) from nonsurvivors (NS).

Objective: Because we had previously shown that glycine conjugation of para-aminobenzoic acid (PABA) quantitatively reflects liver function in children with chronic liver disease, in this pilot study we wanted to determine whether the measurement of the glycine conjugates of PABA could distinguish S from NS in FHF in comparison with standard prognostic indices.

Music skills and the expressive interpretation of music in children with Williams-Beuren syndrome: pitch, rhythm, melodic imagery, phrasing, and musical affect.

This paper studied music in 14 children and adolescents with Williams-Beuren syndrome (WBS), a multi-system neurodevelopmental disorder, and 14 age-matched controls. Five aspects of music were tested. There were two tests of core music domains, pitch discrimination and rhythm discrimination.

Dose-response relationship and infusion requirement of cisatracurium besylate in infants and children during nitrous oxide-narcotic anesthesia.

Background: To determine the effect of age on the dose-response relation and infusion requirement of cisatracurium besylate in pediatric patients, 32 infants (mean age, 0.7 yr; range, 0.3-1.

Snow-related recreational injuries in children: assessment of morbidity and management strategies.

Purpose: The aim of this study was to investigate the causes, clinical course, and financial impact of snow-related sport injuries in children.

Methods: Reports of snow-related injuries (skiing, toboganning, snowboarding) occurring in 147 consecutive children (< or =16 years of age) admitted from 1991 through 1997 were collected prospectively and assessed retrospectively. During the last year of the study, outpatients treated and released from the emergency department (1996 through 1997) were examined in parallel (n = 101).

Primum atrial septal defect in children: early results, risk factors, and freedom from reoperation.

Background: Repair of primum atrial septal defect in children usually is associated with a low operative mortality, except for a subgroup of children with congestive heart failure. To determine the early mortality and incidence of reoperation in children with primum atrial septal defect, we analyzed retrospectively the results of patients who underwent repair of this defect.

Methods: Between July 1982 and December 1996, 180 children underwent repair of primum atrial septal defect.

Echocardiographic clues and accuracy in the diagnosis of scimitar syndrome.

Scimitar syndrome was diagnosed in 27 patients seen between July 1974 and May 1993. All available echocardiograms taken before surgery or death were reviewed. Age at presentation ranged from 1 day to 14 years.

Pyrogen - prostaglandin coupling in the pathogenesis of fever: evidence against a role for nitric oxide.

There is much debate on the mechanism by which blood-borne pyrogens trigger prostaglandin E2 (PGE2) synthesis in brain and fever. This investigation was undertaken to determine whether nitric oxide qualifies as a signal transducer for pyrogens at the interface between blood and brain. Experiments were carried out in vitro and in vivo using, respectively, preparations of cerebral tissue and microvessels from the rat, and the conscious, chronically instrumented cat.