A Case Report of Takayasu's Arteritis and Ulcerative Colitis in a Pediatric Patient with Chronic Recurrent Multifocal Osteomyelitis Successfully Treated with Infliximab: Diagnostic Clues in Disease Associations and Immune Dysregulation.

BACKGROUND: Takayasu's arteritis with comorbid chronic recurrent multifocal osteomyelitis and ulcerative colitis is rare in the pediatric population. Treatment with anti-TNF alpha agents such as infliximab has been a successful treatment strategy in adults and can be used effectively in the pediatric population. CASE PRESENTATION: We present the case of a 15-year-old Caucasian girl with a history of chronic recurrent multifocal osteomyelitis and ulcerative colitis presenting with hypertensive emergency secondary to Takayasu's arteritis with middle aortic syndrome. She was treated with corticosteroids and methotrexate and ultimately required infliximab infusions of 15 mg/kg every 4 weeks to successfully control her symptoms and normalize her inflammatory markers. CONCLUSIONS: In this case, we discuss the use of infliximab in an adolescent patient with chronic recurrent multifocal osteomyelitis, ulcerative colitis, and Takayasu's arteritis. The significance of this case is determined by the unique occurrence of all three conditions in a pediatric patient, the important consideration of vasculitis in the differential of a pediatric patient presenting with hypertensive emergency, the need for vigilance for detecting diagnostic clues, signs, and symptoms, knowledge of disease associations when evaluating a patient with a predisposition for autoinflammatory conditions, and the use of increasing doses of infliximab to control symptoms.

Poor oral intake in a late preterm twin - usual symptom with an unusual diagnosis.

BACKGROUND: At three weeks of age, a previous 34 weeks' gestation male infant (twin A) was transferred to our regional perinatal center (RPC) with complaints of poor oral feeding and intermittent tachypnea. Twin B was discharged at 37 weeks with an uneventful course. CASE: Twin A briefly required respiratory support but continued to have difficulty transitioning from gavage to oral feeding. Initially, his inability to feed orally was thought to be secondary to nasal congestion and prematurity, but with worsening respiratory distress he was transferred for further evaluation and management. DIAGNOSIS & CONCLUSION: On admission to RPC, the examination prompted a cardiac assessment which revealed a large aortic-pulmonary window type II. After surgery, the infant quickly improved and went home on-demand oral feeds. Cardiac lesions are more common in monochorionic twins but should be suspected in dichorionic twins especially if one twin has a normal course.

Left ventricular three-dimensional global systolic strain by real-time three-dimensional speckle-tracking in children: feasibility, reproducibility, maturational changes, and normal ranges.

BACKGROUND: Three-dimensional (3D) strain analysis may help overcome the limitations of Doppler and two-dimensional strain analysis for the left ventricle and become the method of choice for left ventricular (LV) systolic function. The aims of this study were to evaluate the feasibility and reproducibility of LV global 3D systolic strain by real-time 3D speckle-tracking echocardiography (STE) in children and to establish their maturational growth patterns and normal values. METHODS: A prospective study was conducted in 256 consecutive healthy subjects using real-time 3D echocardiography. Full-volume 3D data were acquired using a 3D matrix-array transducer. Three-dimensional LV peak systolic global strain (GS), global longitudinal strain (GLS), global radial strain (GRS), and global circumferential strain (GCS) values were determined using real-time 3D STE. RESULTS: A total of 228 patients (89%) met the criteria for analysis; 28 (11%) were excluded. The correlations between age and strain variables by real-time 3D STE were poor (R(2) = 0.01-0.05, P < .05). The differences in GLS and GCS among the five age groups were statistically significant but clinically irrelevant. There were no statistical differences in GRS and GS values among the age groups, nor were there statistical differences between the genders for all 3D strain parameters. Intraobserver and interobserver variability ranged from 5.0 ± 4.3% to 10.1 ± 8.5% versus 6.9 ± 6.1% to 17.0 ± 16.2% for coefficients of variation, respectively. Interclass correlation coefficients ranged from 0.78 to 0.87 and from 0.75 to 0.79 for intraobserver and interobserver measurements for GS, GLS, GCS, and GRS, respectively. CONCLUSIONS: LV global 3D systolic strain analysis using the new 3D STE is feasible and reproducible in the pediatric population. There are small maturational changes in GLS and GCS, but not in GRS and GS, that are statistically significant but probably clinically irrelevant. Further investigation is warranted for potential clinical application of this new technology in a pediatric population.

Advances in fetal echocardiography: early imaging, three/four dimensional imaging, and role of fetal echocardiography in guiding early postnatal management of congenital heart disease.

In this article, we review a number of topics that we believe reflect new and exciting aspects of fetal echocardiography. These new advances include early fetal cardiovascular imaging around 14 weeks, the utility of three/four dimensional imaging technology for the fetus, and finally the utility of fetal echocardiography for antenatal and perinatal care of congenital heart diseases to improve and optimize outcome. Finally, we briefly discussed future directions in fetal cardiac intervention.

Real time three-dimensional echocardiography for quantification of ventricular volumes, mass, and function in children with congenital and acquired heart diseases.

Quantitative measurement of left ventricular (LV) volumes, mass, and function is one of the most common and important indications for echocardiography. These measurements are among the most powerful tools for diagnosis and prognosis of congenital and acquired heart diseases and for assessment of medical, percutaneous, and surgical interventions. Awareness is also growing of the importance of right ventricular (RV) volume, mass, and function in many cardiopulmonary diseases. Furthermore, there are challenges and opportunities to measure the volume, mass, and function of complex chambers such as the left atrium, right atrium, and the univentricular heart. As echocardiography continues to be the imaging modality of choice for these measurements, the strengths and limitations of M-mode, two-dimensional (2D), and recently three-dimensional (3D) echocardiographic (3DE) methodologies for accurate and reproducible measurement of these indices have been extensively investigated for congenital and acquired heart diseases. Evidence suggests that 3DE provides improved accuracy and reproducibility over 2D methods for measurement of LV volume and function calculation in adults and in children. Data have accumulated on the utility of 3DE for measuring chamber volumes and function for the RV and for the single ventricle, which may become more widely used in clinical and research arenas in the future. Finally, new advanced modes of analysis such as 3D strain and synchrony analysis by 3DE are promising methodologies that warrant further investigation.

Assessment of biomarkers of cardiovascular risk among HIV type 1-infected adolescents: role of soluble vascular cell adhesion molecule as an early indicator of endothelial inflammation.

Cardiovascular disease (CVD) biomarkers were examined in a cohort of HIV-infected and HIV-uninfected adolescents who participated in Adolescent Trials Network study 083 utilizing samples from the Reaching for Excellence in Adolescent Care cohort, a longitudinal study of youth infected through adult risk behavior. Nonfasting blood samples from 97 HIV-infected and 81 HIV-uninfected adolescents infected by adult risk behaviors were analyzed for total cholesterol, low-density lipoprotein (LDL), high-density lipoprotein (HDL), very low-density lipoprotein (VLDL), triglycerides, apolipoprotein A-I, high-sensitivity C-reactive protein (hsCRP), soluble vascular adhesion molecule-1 (sVCAM-1), myeloperoxidase, and neopterin at baseline and 18 months later. Results were analyzed using ANOVA, Wilcoxon signed-rank, and paired t tests. Among infected subjects 67 received antiretroviral therapy and 30 were treatment naive. The HIV-infected and HIV-uninfected subjects were similar in gender, ethnicity, and cardiovascular risk factors such as smoking and obesity. In all groups lipid parameters were within accepted guidelines for cardiovascular risk. Among HIV-infected youth on antiretroviral therapy (ART), HDL and apoprotein A-I were significantly lower when compared to uninfected youth. hsCRP was not elevated and thus not predictive for risk in any group. sVCAM-1 levels were significantly elevated in both HIV-infected groups: 1,435 ng/ml and 1,492 ng/ml in untreated and treated subjects, respectively, and 1,064 ng/ml in the uninfected group (p<0.0001). Across all groups neopterin correlated with sVCAM at 18 months (Spearman correlation coefficient 0.58, p<0.0001). Only 9% of ART-treated subjects fully suppressed virus. Lipid profiles and hsCRP, traditional markers of cardiovascular disease, are not abnormal among HIV-infected youth but elevated sVCAM may be an early marker of atherosclerosis.

Real time three-dimensional echocardiography for evaluation of congenital heart defects: state of the art.

Real time three-dimensional echocardiography (RT3DE) has been increasingly used in the diagnosis and assessment of congenital heart disease. A growing body of literature suggests that this new technology can be used as an integrated approach to assess the morphology of simple and complex congenital heart defects, flow abnormality, and left, right, and single ventricular function both qualitatively and quantitatively. This review summarizes the available evidence for the use of RT3DE in each of these areas. Future technology refinement in RT3DE and development of practice guidelines will increase the utilization of this new technology as a valuable tool to compliment 2D echocardiography/Doppler in clinical care and research to improve the care and outcome of congenital heart disease.