Diseases Commons^™

Evaluation Of Kawasaki Disease Risk-Scoring Systems For Intravenous Immunoglobulin Resistance., Lynn A. Sleeper, L Luann Minich, Brian M. Mccrindle, Jennifer S. Li, Wilbert Mason, Steven D. Colan, Andrew M. Atz, Beth F. Printz, Annette Baker, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.

STUDY DESIGN: We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant.

RESULTS: For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower …

Noncoronary Cardiac Abnormalities Are Associated With Coronary Artery Dilation And With Laboratory Inflammatory Markers In Acute Kawasaki Disease., Beth F. Printz, Lynn A. Sleeper, Jane W. Newburger, L Luann Minich, Timothy Bradley, Meryl S. Cohen, Deborah Frank, Jennifer S. Li, Renee Margossian, Girish S. Shirali, Masato Takahashi, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis.

BACKGROUND: Left ventricular (LV) dysfunction, mitral regurgitation (MR), and aortic root dilation occur early after diagnosis; their associations with coronary artery dilation and inflammatory markers have not been well-described.

METHODS: Centrally interpreted echocardiograms were obtained at KD diagnosis and 1 and 5 weeks after diagnosis on 198 subjects in the National Institutes of Health-sponsored Pediatric Heart Network KD pulsed steroid trial. Regression models were constructed to investigate the relationships among early LV dysfunction, MR, and aortic …

Efficacy And Safety Of Once-Daily Aclidinium In Chronic Obstructive Pulmonary Disease, Paul W. Jones, Stephen I. Rennard, Alvar Agusti, Pascal Chanez, Helgo Magnussen, Leonardo Fabbri, James F. Donohue, Eric D. Bateman, Nicholas J. Gross, Rosa Lamarca, Cynthia Caracta, Esther Garcia Gil

Journal Articles: Pulmonary & Critical Care Med

BACKGROUND: The long-term efficacy and safety of aclidinium bromide, a novel, long-acting muscarinic antagonist, were investigated in patients with moderate to severe chronic obstructive pulmonary disease (COPD).

METHODS: In two double-blind, 52-week studies, ACCLAIM/COPD I (n=843) and II (n=804), patients were randomised to inhaled aclidinium 200 μg or placebo once-daily. Patients were required to have a post-bronchodilator forced expiratory volume in 1 second (FEV1)/forced vital capacity ratio of ≤70% and FEV1< 80% of the predicted value. The primary endpoint was trough FEV1 at 12 and 28 weeks. Secondary endpoints were health status measured by St George's Respiratory Questionnaire (SGRQ) and time to first moderate or severe COPD exacerbation.

RESULTS: At 12 and 28 weeks, aclidinium improved trough FEV1 versus placebo in ACCLAIM/COPD I (by 61 and 67 mL; both p< 0.001) and ACCLAIM/COPD II (by 63 and 59 mL; both p< 0.001). More patients had a SGRQ improvement≥4 units at 52 weeks with aclidinium versus placebo in ACCLAIM/COPD I (48.1% versus 39.5%; p=0.025) and ACCLAIM/COPD II (39.0% versus 32.8%; p=0.074). The time to first exacerbation was significantly delayed by aclidinium in ACCLAIM/COPD II (hazard ratio [HR] 0.7; 95% confidence interval [CI] 0.55 to 0.92; p=0.01), but not ACCLAIM/COPD I (HR 1.0; 95% CI 0.72 to 1.33; p=0.9). Adverse events were minor in both studies.

CONCLUSION: Aclidinium is effective and well tolerated …

Reduction Of Exacerbations By The Pde4 Inhibitor Roflumilast--The Importance Of Defining Different Subsets Of Patients With Copd, Stephen I. Rennard, Peter M.A. Calverley, Udo M. Goehring, Dirk Bredenbröker, Fernando J. Martinez

Journal Articles: Pulmonary & Critical Care Med

BACKGROUND: As chronic obstructive pulmonary disease (COPD) is a heterogeneous disease it is unlikely that all patients will benefit equally from a given therapy. Roflumilast, an oral, once-daily phosphodiesterase 4 inhibitor, has been shown to improve lung function in moderate and severe COPD but its effect on exacerbations in unselected populations was inconclusive. This led to the question of whether a responsive subset existed that could be investigated further.

METHODS: The datasets of two previous replicate, randomized, double-blind, placebo-controlled, parallel-group studies (oral roflumilast 500 μg or placebo once daily for 52 weeks) that were inconclusive regarding exacerbations were combined in …

Genetics Of Sputum Gene Expression In Chronic Obstructive Pulmonary Disease, Weiliang Qiu, Michael H. Cho, John H. Riley, Wayne H. Anderson, Dave Singh, Per Bakke, Amund Gulsvik, Augusto A. Litonjua, David A. Lomas, James D. Crapo, Terri H. Beaty, Bartolome R. Celli, Stephen I. Rennard, Ruth Tal-Singer, Steven M. Fox, Edwin K. Silverman, Craig P. Hersh, Eclipse Investigators

Journal Articles: Pulmonary & Critical Care Med

Previous expression quantitative trait loci (eQTL) studies have performed genetic association studies for gene expression, but most of these studies examined lymphoblastoid cell lines from non-diseased individuals. We examined the genetics of gene expression in a relevant disease tissue from chronic obstructive pulmonary disease (COPD) patients to identify functional effects of known susceptibility genes and to find novel disease genes. By combining gene expression profiling on induced sputum samples from 131 COPD cases from the ECLIPSE Study with genomewide single nucleotide polymorphism (SNP) data, we found 4315 significant cis-eQTL SNP-probe set associations (3309 unique SNPs). The 3309 SNPs were tested …

The Cardiac Atlas Project--An Imaging Database For Computational Modeling And Statistical Atlases Of The Heart, Carissa G. Fonseca, Michael Backhaus, David A. Bluemke, Randall D. Britten, Jae Do Chung, Brett R. Cowan, Alan H. Kadish

Office of the President Publications and Research

MOTIVATION: Integrative mathematical and statistical models of cardiac anatomy and physiology can play a vital role in understanding cardiac disease phenotype and planning therapeutic strategies. However, the accuracy and predictive power of such models is dependent upon the breadth and depth of noninvasive imaging datasets. The Cardiac Atlas Project (CAP) has established a large-scale database of cardiac imaging examinations and associated clinical data in order to develop a shareable, web-accessible, structural and functional atlas of the normal and pathological heart for clinical, research and educational purposes. A goal of CAP is to facilitate collaborative statistical analysis of regional heart shape …

Usefulness Of The Echocardiographic Multi-Parameter Score (Emps) In Evaluating Left Ventricular Global Heart Function, Bingyuan Zhou, Junhua Yang, Xiangjun Yang, Yongming He, Caiming Zhao, Sudan Xu, Huifeng Wang

The Texas Heart Institute Journal

In this study, we established a new index by combining several echocardiographic parameters to quantify heart failure. We selected 233 consecutive patients who underwent both echocardiographic and plasma B-type natriuretic peptide (BNP) tests within 24 hours after referral for suspected heart failure. The echocardiographic parameters included systolic function, diastolic function, left ventricular chamber remodeling, valvular lesions, systolic pulmonary arterial pressure, and regional wall-motion abnormality. Each factor was scored from 1 to 3 points according to its severity. The total point from these 6 factors is the echocardiographic multi-parameter score (EMPS).

The EMPS for 37, 51, 77, and 38 patients from …

Left Atrial Ablation For Atrial Fibrillation: Creating The “Box Lesion” With A Bipolar Radiofrequency Device, Leonid Sternik, Hartzel V Schaff, David Luria, Michael Glikson, Alexander Kogan, Ateret Malachy, Maya First, Ehud Raanani

The Texas Heart Institute Journal

The maze procedure is the gold standard for the ablation of atrial fibrillation, and the “box lesion” around the pulmonary veins is the most important part of this procedure. We have created this lesion with a bipolar radiofrequency ablator, abandoning the usual use of this device (to achieve bilateral epicardial isolation of the pulmonary veins).

From March 2004 through the end of May 2010, we performed surgical ablation of atrial fibrillation in 240 patients. Of this number, 205 underwent operation by a hybrid maze technique and the remaining 35 (our study cohort) underwent the creation of a box lesion around …

Internal Medicine Problems As They Present To The Cardiologist, Ira Martin Grais

The Texas Heart Institute Journal

No abstract provided.

Rosuvastatin Therapy Does Not Affect Serum Mmp-13 Or Timp-1 Levels In Hypercholesterolemic Patients, Cihan Cevik, Kenneth Nugent, Gary Meyerrose, Mohammad Otahbachi, Cemil Izgi, Mark Lyte, R David Fish

The Texas Heart Institute Journal

Matrix metalloproteinases degrade the collagen content of atherosclerotic plaque and reduce plaque stability. In tissue sections of atherosclerotic plaque, the expression of matrix metalloproteinases is increased. 3-Hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) decrease the tissue expression of matrix metalloproteinases-1, -2, -3, and -9 in atheromatous plaque by attenuating the inflammatory process that leads to increased expression. However, it is not known whether statins decrease levels of matrix metalloproteinase-13—an enzyme crucial to the initiation of collagen degradation—as part of their plaque-stabilizing effect.

We prospectively examined the effect of statin therapy on serum levels of matrix metalloproteinase-13, tissue inhibitor of metalloproteinase-1, and …

Prehypertension: A Meta-Analysis Of The Epidemiology, Risk Factors, And Predictors Of Progression, Xiaofan Guo, Liling Zou, Xingang Zhang, Jue Li, Liqiang Zheng, Zhaoqing Sun, Jian Hu, Nathan D Wong, Yingxian Sun

The Texas Heart Institute Journal

We investigated the prevalence and risk factors of prehypertension, as well as the predictors of progression from prehypertension to hypertension. To do this, we performed a systematic review and meta-analysis of cross-sectional and longitudinal studies, after unrestricted searches of PubMed and The Cochrane Library through September 2010. In addition, we reviewed references, major textbooks, and review articles. Pooled prevalence, standardized mean differences, and odds ratios were estimated by using a random-effects model.

Twenty-six articles met our inclusion criteria; these included 20 cross-sectional and 6 longitudinal studies, with a total sample of 250,741 individuals. The overall pooled prevalence of prehypertension was …

Interposition Grafting Of Large Extracranial Carotid Aneurysm, Marlous Huyzer, Michel M P J Reijnen, Johannes E M Sybrandy, Jacob Buth, Clark J Zeebregts

The Texas Heart Institute Journal

Extracranial carotid aneurysms are a rare entity and carry an inherent risk of thromboembolic complications. Treatment options consist of endovascular and conventional surgical techniques. We describe the cases of 3 patients who were treated with an interposition graft for a large extracranial carotid aneurysm.

The patients had presented with an extracranial carotid aneurysm with a diameter of 30 to 43 mm. In all cases, the aneurysm was excluded by means of an interposition graft, without major perioperative complications. There was 1 case of temporary paresis of the facial nerve and another of temporary paresis of the vocal cord. After a …

Closure Of A Para-Valvular Aortic Leak With The Use Of 2 Amplatzer Devices And Real-Time 2− And 3-Dimensional Transesophageal Echocardiography, Kurt S Hoffmayer, Christian Zellner, Damon M Kwan, Suma Konety, Elyse Foster, Phillip Moore, Yerem Yeghiazarians

The Texas Heart Institute Journal

Paravalvular leaks are well-recognized sequelae of mechanical aortic valve implantation. Clinical manifestations include hemolysis, arrhythmias, and congestive heart failure. Frequently, patients who receive mechanical aortic valves are poor candidates for repeat valve surgery, and the reoperative mortality rate is high. Percutaneous intervention has recently become an alternative to surgery in selected patients. Herein, we describe the percutaneous closure of an aortic paravalvular leak in a 45-year-old man who had undergone 2 aortic valve replacements with mechanical valves. The patient, who was at high surgical risk due to comorbidities, underwent the implantation of 2 AMPLATZER devices with the use of real-time …

Takayasu Arteritis With Coronary Aneurysms Causing Acute Myocardial Infarction In A Young Man, Sana Ouali, Slim Kacem, Fatma Ben Fradj, Rim Gribaa, Elyes Naffeti, Fahmi Remedi, Chedia Laaoueni, Essia Boughzela

The Texas Heart Institute Journal

Takayasu arteritis is an inflammatory condition that involves the large cardiac vessels, predominantly the aorta and its main branches. It typically affects young women (age, ≤40 yr), most often Asians and Latin Americans. Herein, we describe a rare manifestation of Takayasu arteritis in a 19-year-old black Tunisian man who presented with acute inferior myocardial infarction and complete atrioventricular block after occlusion from a giant aneurysm in the right coronary artery. The coronary artery disease was associated with aneurysmal dilations in the carotid, vertebral, and right renal arteries. Medical therapy improved Thrombolysis in Myocardial Infarction flow in the area of the …

Surgical Management Of Double-Chambered Right Ventricle In Adults, Ahmad K Darwazah, Mohammed Eida, Vivian Bader, Mohammed Khalil

The Texas Heart Institute Journal

Double-chambered right ventricle is a congenital anomaly in which the right ventricle is divided into 2 portions by anomalous muscle bundles. These cases often present in children, but rarely in adults.

We discuss 2 cases of double-chambered right ventricle, in patients aged 42 and 35 years. When cases remain asymptomatic until adulthood, they can present with unusual symptoms that lead to incorrect diagnosis. Our cases represent 2 very different manifestations of double-chambered right ventricle, which differ in presentation, in the site of abnormal obstructive muscle bundles, and in the presence of associated lesions. Both of our patients underwent successful surgical …

A Dosing Study Of Bone Marrow Mononuclear Cells For Transendocardial Injection In A Pig Model Of Chronic Ischemic Heart Disease, Guilherme V Silva, Marlos R Fernandes, Cristiano O Cardoso, Ricardo R Sanz, Edie M Oliveira, Pilar Jimenez-Quevedo, Javier Lopez, Franca S Angeli, Yi Zheng, James T Willerson, Emerson C Perin

The Texas Heart Institute Journal

We studied the effect of the dose of bone marrow mononuclear cells, delivered via transendocardial injection, upon capillary density and fibrosis in pigs with chronic ischemic heart disease.

Pigs (n = 16) that had undergone ameroid constrictor placement (left circumflex coronary artery) to induce chronic ischemia were divided equally into 4 groups on the basis of bone marrow mononuclear cell dose: control (saline injection) and 50, 100, or 200 × 106 bone marrow mononuclear cells. Thirty days after ameroid placement, each pig received 13 transendocardial NOGA-guided injections. An implantable loop recorder monitored possible arrhythmias caused by cell transplantation. Thirty days …

High-Risk Diabetic Patients With Unprotected Left Main Coronary Artery Disease: Characteristics And Medium-Term Outcomes Of Percutaneous Revascularization With Drug-Eluting Stents, Jose F Díaz Fernández, Carlos Sánchez González, Manuel Jiménez Navarro, Juan C Fernández Guerrero, Antonio Gómez Menchero, Juan H Alonso Briales, Rosa María Cardenal Piris, Juan Herrador, Manuel Guzmán Herrera, Jose M Hernández García

The Texas Heart Institute Journal

Percutaneous coronary intervention with drug-eluting stents is an alternative for patients with high-risk unprotected left main coronary artery disease; those with diabetes mellitus are at even higher risk. Recent advances in percutaneous coronary intervention could lead to better results. The aim of this study was to evaluate medium-term results in a real-world sample of high-risk diabetic patients undergoing percutaneous coronary intervention with drug-eluting stents for unprotected left main coronary artery disease.

From 3 tertiary hospitals, we retrospectively identified 334 high-risk patients, of whom 141 (42%) were diabetic and 193 (58%) were nondiabetic. The diabetes mellitus group showed a higher prevalence …

Micro-Ultrasonographic Imaging Of Atherosclerotic Progression And Correlation With Inflammatory Markers In Apolipoprotein-E Knockout Mice, Rong-Juan Li, Ya Yang, Yan-Hong Wang, Jin-Jie Xie, Li Song, Zheng Wang, Yao-Zhong Zhang, Yan-Wen Qin, Zhi-An Li, Xiao-Shan Zhang

The Texas Heart Institute Journal

We studied prospectively whether atherosclerotic progression in apolipoprotein-E knockout mice could be noninvasively and accurately measured by use of high-resolution ultrasonographic biomicroscopy. We examined the correlation between the ultrasonographic characterization of ascending aortic atherosclerotic plaque and plasma C-reactive protein, interleukin-1, and interleukin-6 levels in these mice.

In 4 age groups (8, 16, 24, and 32 wk) of 8 male knockout mice each (atherosclerotic groups) and age-matched male C57BL/6 mice (control groups), we used ultrasonographic biomicroscopy to measure maximal plaque thickness or intima-media thickness in the ascending aorta. We compared the findings with corresponding histologic measurements, and we measured plasma C-reactive …

Sirolimus-Eluting Stents Versus Bare-Metal Stents In Routine Clinical Use, Muhammad Munir, Jonathan Aliota, Amany Ahmed, Anwarullah Mohammed, Vei Vei Lee, Macarthur A Elayda, James M Wilson

The Texas Heart Institute Journal

Conflicting patient outcomes have been reported after the use of sirolimus-eluting stents or bare-metal stents. In this nonrandomized study, we examine the outcomes after placement of sirolimus-eluting versus bare-metal stents in an unselected population of patients who underwent percutaneous coronary revascularization.

We used THIRD-base, a longitudinal data registry of patients who underwent revascularization at our institution, to compare demographics and outcomes in patients treated with a sirolimus-eluting or bare-metal stent from January 2001 through June 2006. Outcome measures included major acute coronary and cerebral events at 30 days, target-vessel failure at 9 months and at 3 years, and stent thrombosis. …

Endovascular Repair Of Aortoesophageal And Aortobronchial Fistulae, Roberto Chiesa, Andrea Kahlberg, Yamume Tshomba, Enrico M Marone, Massimiliano M Marrocco-Trischitta, Germano Melissano

The Texas Heart Institute Journal

Aortoesophageal fistulae (AEF) and aortobronchial fistulae (ABF) are uncommon, but are in general fatal if left untreated. Conventional treatment requires surgical replacement of the thoracic aorta, along with several adjunctive procedures to repair the esophageal or tracheobronchial lesion.The mortality rates associated with open surgery reach 61% in cases of primary fistula and 78% in secondary fistula.

Thoracic endovascular aortic repair (TEVAR) has been proposed as an alternative strategy to surgical management.Although less invasive, this technique has important limitations in treating AEF and ABF, the chief of which is the high risk of graft contamination. Moreover, we still lack sufficient data …

Predictors Of Coronary Artery Visualization In Kawasaki Disease., Renee Margossian, Minmin Lu, L Luann Minich, Timothy J. Bradley, Meryl S. Cohen, Jennifer S. Li, Beth F. Printz, Girish S. Shirali, Lynn A. Sleeper, Jane W. Newburger, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Background: Echocardiography is the imaging modality of choice for the evaluation of coronary artery (CA) abnormalities in Kawasaki disease. Small series have established high specificity and sensitivity for detecting abnormalities, yet visualization rates of individual CA segments and factors associated with success are unknown.

Methods: In the Pediatric Heart Network's randomized trial of primary steroid treatment for Kawasaki disease, echocardiograms were interpreted locally and by a core laboratory. Univariate and multivariate predictors of CA visualization by the local lab as determined by the core lab were explored, and agreement of CA size measured locally and by the core lab was …

Relation Of Size Of Secondary Ventricles To Exercise Performance In Children After Fontan Operation., Ashwin Prakash, Thomas G. Travison, Mark A. Fogel, Lynne M. Hurwitz, Andrew J. Powell, Beth F. Printz, Michael D. Puchalski, Girish S. Shirali, Shi-Joon Yoo, Tal Geva, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

The effects of the nondominant or secondary ventricle on the Fontan circulation are not known. The present study used cardiac magnetic resonance imaging to investigate the relations between secondary ventricular size and global cardiac performance. The Fontan cross-sectional study collected data from 7 centers participating in the Pediatric Heart Network. Subjects with complete cardiac magnetic resonance imaging data and an identifiable secondary ventricle were included in the analysis. Relationships between body surface area-adjusted parameters of the secondary ventricle (mass, end-diastolic volume, mass/volume ratio, and stroke volume) and the following measures were assessed. These measures included the percentage of predicted peak …

Prenatal Diagnosis And Risk Factors For Preoperative Death In Neonates With Single Right Ventricle And Systemic Outflow Obstruction: Screening Data From The Pediatric Heart Network Single Ventricle Reconstruction Trial(∗)., Andrew M. Atz, Thomas G. Travison, Ismee A. Williams, Gail D. Pearson, Peter C. Laussen, William T. Mahle, Amanda L. Cook, Joel A. Kirsh, Mark Sklansky, Svetlana Khaikin, Caren Goldberg, Michele Frommelt, Catherine Krawczeski, Michael D. Puchalski, Jeffrey P. Jacobs, Jeanne M. Baffa, Jack Rychik, Richard G. Ohye, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.

METHODS: Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.

RESULTS: …

Should We Be Concerned About Jejunoileal Atresia During Repair Of Duodenal Atresia?, Shawn D. St Peter, Danny C. Little, Katherine A. Barsness, Daniel R. Copeland, Casey M. Calkins, Suzanne Yoder, Steve S. Rothenberg, Saleem Islam, Kuojen Tsao, Daniel J. Ostlie

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: During repair for duodenal atresia, it has been emphasized that inspection of the small bowel to identify a second atresia is required. The laparoscopic approach for repair of duodenal atresia has been criticized for its limitation to perform this step. Given that duodenal atresia and jejunoileal atresias do not share common embryologic origins, we question the validity of this concern. Therefore, we conducted a multicenter retrospective review of duodenal atresia patients to quantify the incidence of jejunoileal atresia in this population.

METHODS: After institutional review board approval (IRB #07-12-187X), a retrospective review was conducted on all patients who have …

Cardiovascular Risk Associated With Interactions Among Polymorphisms In Genes From The Renin-Angiotensin, Bradykinin, And Fibrinolytic Systems, John P. Bentley, Folkert W. Asselbergs, Christopher S. Coffey, Patricia R. Hebert, Jason H. Moore, Hans L. Hillege, Wiek H. Van Gilst

Dartmouth Scholarship

Background: Vascular fibrinolytic balance is maintained primarily by interplay of tissue plasminogen activator (t-PA) and plasminogen activator inhibitor type 1 (PAI-1). Previous research has shown that polymorphisms in genes from the renin- angiotensin (RA), bradykinin, and fibrinolytic systems affect plasma concentrations of both t-PA and PAI-1 through a set of gene-gene interactions. In the present study, we extend this finding by exploring the effects of polymorphisms in genes from these systems on incident cardiovascular disease, explicitly examining two-way interactions in a large population- based study.

Methodology/Principal Findings: Data from the population-based PREVEND study in Groningen, The Netherlands (n = 8,138) …

Partial And Transitional Atrioventricular Septal Defect Outcomes., L Luann Minich, Andrew M. Atz, Steven D. Colan, Lynn A. Sleeper, Seema Mital, James Jaggers, Renee Margossian, Ashwin Prakash, Jennifer S. Li, Meryl S. Cohen, Ronald V. Lacro, Gloria L. Klein, John A. Hawkins, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.

METHODS: We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.

RESULTS: Median age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive …

Sputum Neutrophils As A Biomarker In Copd: Findings From The Eclipse Study, Dave Singh, Lisa Edwards, Ruth Tal-Singer, Stephen I. Rennard

Journal Articles: Pulmonary & Critical Care Med

INTRODUCTION: The percentage of neutrophils in sputum are increased in COPD patients, and may therefore be a biomarker of airway inflammation. We studied the relationships between sputum neutrophils and FEV1, health status, exacerbation rates, systemic inflammation and emphysema, and long term variability at 1 year.

METHODS: Sputum samples were obtained from 488 COPD patients within the ECLIPSE cohort. 359 samples were obtained at baseline, and 297 after 1 year. 168 subjects provided samples at both visits. Serum interleukin-6 (IL-6), IL-8, surfactant protein D and C-reactive protein levels were measured by immunoassays. Low-dose CT scans evaluated emphysema.

RESULTS: Sputum neutrophil % …

Characterisation Of Copd Heterogeneity In The Eclipse Cohort, Alvar Agusti, Peter M.A. Calverley, Bartolome Celli, Harvey O. Coxson, Lisa D. Edwards, David A. Lomas, William Macnee, Bruce E. Miller, Steve I. Rennard, Edwin K. Silverman, Ruth Tal-Singer, Emiel Wouters, Julie C. Yates, Jørgen Vestbo, The Evaluation Of Copd Longitudinally To Identify Predictive Surrogate Endpoints (Eclipse) Investigators

Journal Articles: Pulmonary & Critical Care Med

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a complex condition with pulmonary and extra-pulmonary manifestations. This study describes the heterogeneity of COPD in a large and well characterised and controlled COPD cohort (ECLIPSE).

METHODS: We studied 2164 clinically stable COPD patients, 337 smokers with normal lung function and 245 never smokers. In these individuals, we measured clinical parameters, nutritional status, spirometry, exercise tolerance, and amount of emphysema by computed tomography.

RESULTS: COPD patients were slightly older than controls and had more pack years of smoking than smokers with normal lung function. Co-morbidities were more prevalent in COPD patients than in …

Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets With Alopecia Resulting From A Novel Missense Mutation In The Dna-Binding Domain Of The Vitamin D Receptor., Peter J. Malloy, Jining Wang, Tarak Srivastava, David Feldman

Manuscripts, Articles, Book Chapters and Other Papers

The rare genetic recessive disease, hereditary vitamin D resistant rickets (HVDRR), is caused by mutations in the vitamin D receptor (VDR) that result in resistance to the active hormone 1,25-dihydroxyvitamin D(3) (1,25(OH)(2)D(3) or calcitriol). In this study, we examined the VDR from a young boy with clinical features of HVDRR including severe rickets, hypocalcemia, hypophosphatemia and partial alopecia. The pattern of alopecia was very unusual with areas of total baldness, adjacent to normal hair and regions of scant hair. The child failed to improve on oral calcium and vitamin D therapy but his abnormal chemistries and his bone X-rays normalized …

Parent- Versus Child-Reported Functional Health Status After The Fontan Procedure., Linda M. Lambert, L Luann Minich, Jane W. Newburger, Minmin Lu, Victoria L. Pemberton, Ellen A. Mcgrath, Andrew M. Atz, Mingfen Xu, Elizabeth Radojewski, Darlene Servedio, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: We sought to compare perceptions of functional health status between children who had undergone a Fontan procedure and their parents.

METHODS: Fontan procedure survivors 10 to 18 years of age were included in the study if the child completed the Child Health Questionnaire (CHQ) and the parent completed the parent form to assess the child's functional health status. Comparisons were made between raw domain scores for the parent- and child-completed CHQs.

RESULTS: Between March 2003 and April 2004, 1078 Fontan survivors were screened. Of the 546 eligible and consented patients, 354 were 10-18 years of age and 328 parent/child …