Pediatrics Commons^™

Does Initial Shunt Type For The Norwood Procedure Affect Echocardiographic Measures Of Cardiac Size And Function During Infancy?: The Single Vventricle Reconstruction Trial., Peter C. Frommelt, Lin T. Guey, L Luann Minich, Majeed Bhat, Tim J. Bradley, Steve D. Colan, Greg Ensing, Jessica Gorentz, Haleh Heydarian, J Blaine John, Wyman W. Lai, Jami C. Levine, William T. Mahle, Stephen G. Miller, Richard G. Ohye, Gail D. Pearson, Girish S. Shirali, Pierre C. Wong, Meryl S. Cohen, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The Pediatric Heart Network trial comparing outcomes in 549 infants with single right ventricle undergoing a Norwood procedure randomized to modified Blalock-Taussig shunt or right ventricle-pulmonary artery shunt (RVPAS) found better 1-year transplant-free survival in those who received RVPAS. We sought to compare the impact of shunt type on echocardiographic indices of cardiac size and function up to 14 months of age.

METHODS AND RESULTS: A core laboratory measured indices of cardiac size and function from protocol exams: early after Norwood procedure (age 22.5 ± 13.4 days), before stage II procedure (age 4.8 ± 1.8 months), and at 14 …

Process Evaluation Of A Positive Youth Development Program In Hong Kong Based On Different Cohorts, Ben M. F. Law, Daniel T. L. Shek

Pediatrics Faculty Publications

There are only a few process evaluation studies on positive youth development programs, particularly in the Chinese context. This study aims to examine the quality of implementation of a positive youth development program (the Project P.A.T.H.S.: Positive Adolescent Training through Holistic Social Programmes) and investigate the relationships among program adherence, process factors, implementation quality, and perceived program success. Process evaluation of 97 classroom-based teaching units was conducted in 62 schools from 2005 to 2009. Findings based on different cohorts generally showed that there were high overall program adherence and implementation quality. Program adherence and implementation process were highly correlated with …

Secondary Data Analyses Of Subjective Outcome Evaluation Data Based On Nine Databases, Daniel T. L. Shek

Pediatrics Faculty Publications

The purpose of this study was to evaluate the effectiveness of the Tier 1 Program of the Project P.A.T.H.S. (Positive Adolescent Training through Holistic Social Programmes) in Hong Kong by analyzing 1,327 school-based program reports submitted by program implementers. In each report, program implementers were invited to write down five conclusions based on an integration of the subjective outcome evaluation data collected from the program participants and program implementers. Secondary data analyses were carried out by aggregating nine databases, with 14,390 meaningful units extracted from 6,618 conclusions. Results showed that most of the conclusions were positive in nature. The findings …

Interim Evaluation Of The Project P.A.T.H.S.: Findings Based On Different Datasets, Daniel T. L. Shek, Lu Yu

Pediatrics Faculty Publications

Interim evaluation studies were carried out in order to examine the implementation details of the Tier 1 Program of the Project P.A.T.H.S. (Positive Adolescent Training through Holistic Social Programmes) in Hong Kong. Quantitative results of the interim evaluation findings based on eight datasets collected from 2006 to 2009 are reported in this paper. Three hundred and seventy-eight schools were randomly selected to provide information on the implementation details of the program via face-to-face interviews, telephone interviews, and self-completed questionnaires. Results showed that a majority of the workers perceived that the students had positive responses to the program and the program …

The Systemic Inflammatory Response After Spinal Cord Injury In The Rat Is Decreased By Α4Β1 Integrin Blockade, Feng Bao, Vanessa Omana, Arthur Brown, Lynne C. Weaver

Paediatrics Publications

The systemic inflammatory response syndrome (SIRS) follows spinal cord injury (SCI) and causes damage to the lungs, kidney, and liver due to an influx of inflammatory cells from the circulation. After SCI in rats, the SIRS develops within 12 h and is sustained for at least 3 days. We have previously shown that blockade of CD11d/CD18 integrin reduces inflammation-driven secondary damage to the spinal cord. This treatment reduces the SIRS after SCI. In another study we found that blockade of α4β1 integrin limited secondary cord damage more effectively than blockade of CD11d/CD18. Therefore we considered it important to assess the …

Qualitative Evaluation Of Project P.A.T.H.S.: An Integration Of Findings Based On Program Participants, Daniel T. L. Shek, Rachel C. F. Sun

Pediatrics Faculty Publications

An integration of the qualitative evaluation findings collected in different cohorts of students who participated in Project P.A.T.H.S. (Positive Adolescent Training through Holistic Social Programmes) (n = 252 students in 29 focus groups) was carried out. With specific focus on how the informants described the program, results showed that the descriptions were mainly positive in nature, suggesting that the program was well received by the program participants. When the informants were invited to name three metaphors that could stand for the program, positive metaphors were commonly used. Beneficial effects of the program in different psychosocial domains were also voiced by …

Factor Structure Of A Multidimensional Gender Identity Scale In A Sample Of Chinese Elementary School Children, Lu Yu, Dong Xie, Daniel T. L. Shek

Pediatrics Faculty Publications

This study examined the factor structure of a scale based on the four-dimensional gender identity model (Egan and Perry, 2001) in 726 Chinese elementary school students. Exploratory factor analyses suggested a three-factor model, two of which corresponded to "Felt Pressure" and "Intergroup Bias" in the original model. The third factor "Gender Compatibility" appeared to be a combination of "Gender Typicality" and "Gender Contentment" in the original model. Follow-up confirmatory factor analysis (CFA) indicated that, relative to the initial four-factor structure, the three-factor model fits the current Chinese sample better. These results are discussed in light of cross-cultural similarities and differences …

Spinal Muscular Atrophy:Clinical Spectrum And Genetic Mutations In Pakistani Children, S. H. Ibrahim, Tariq Moatter, Ali Faisal Saleem

Department of Paediatrics and Child Health

Background: In Pakistan the rate of consanguineous marriages is high, thus, the chance of incidence of autosomal recessive disorders is likely to be high. The aim of this study is to investigate the clinical characteristics and genetics of spinal muscular atrophy (SMA) in children who presented to Aga Khan University, Karachi.
Materials and Methods: This study was a retrospective review of the medical charts of children (neonate: 15 years) with discharge diagnosis of SMA during last 10 years. Demographic features, consanguinity, and diagnostic analysis (including genetic analysis) were noted.
Results: During the study period 67 children had a discharge diagnosis …

Case Report: Rapid Spontaneous Recovery From Severe Hypothyroidism In 2 Teenage Girls., Paul B. Kaplowitz

Pediatrics Faculty Publications

Background

While it is recognized that patients sometimes recover from autoimmune hypothyroidism, little is known about how rapidly this may occur.

Case reports

Two 13 year old girls had severe primary hypothyroidism (total T4 14.2 nmol/L with TSH 468 miU/L and total T4 7.7 nmol/L with TSH 183 miU/L) accompanied by goiter and positive thyroid peroxidase antibodies. There were delays in starting thyroid hormone replacement, and complete reversal of hypothyroidism was documented within 2 months in both cases. One of the girls had recurrence of severe hypothyroidism after being euthyroid for 18 months.

Review of literature

There are few published …

Time-Series Model To Predict Impact Of H1n1 Influenza On A Children's Hospital., Michael C. Spaeder, Jonathan R. Stroud, Xiaoyan Song

Pediatrics Faculty Publications

The spring of 2009 witnessed the emergence of a novel influenza A(H1N1) virus resulting in the first influenza pandemic since 1968. In autumn of 2010, the 2009 novel H1N1 influenza strain re-emerged. We performed a retrospective time-series analysis of all patients with laboratory-confirmed H1N1 influenza who presented to our institution during 2009. Cases of influenza were assembled into 3-day aggregates and forecasting models of H1N1 influenza incidence were created. Forecasting estimates of H1N1 incidence for the 2010–2011 season were compared to actual values for our institution to assess model performance. Ninety-five percent confidence intervals calculated around our model's forecasts were …

Association Of Cartilage-Specific Deletion Of Peroxisome Proliferator-Activated Receptor Γ With Abnormal Endochondral Ossification And Impaired Cartilage Growth And Development In A Murine Model, Roxana Monemdjou, Faezeh Vasheghani, Hassan Fahmi, Gemma Perez, Meryem Blati, Noboru Taniguchi, Martin Lotz, René St-Arnaud, Jean Pierre Pelletier, Johanne Martel-Pelletier, Frank Beier, Mohit Kapoor

Paediatrics Publications

Objective Long bones develop through the strictly regulated process of endochondral ossification within the growth plate, resulting in the replacement of cartilage by bone. Defects in this process can result in skeletal abnormalities and a predisposition to degenerative joint diseases such as osteoarthritis (OA). Studies suggest that activation of the transcription factor peroxisome proliferator-activated receptor γ (PPARγ) is an important therapeutic target in OA. To devise PPARγ-related therapies in OA, it is critical to identify the role of this transcription factor in cartilage biology. Therefore, this study sought to determine the in vivo role of PPARγ in endochondral ossification and …

The Dark Side Of Neuroplasticity, Arthur Brown, Lynne C. Weaver

Paediatrics Publications

Whether dramatic or modest, recovery of neurological function after spinal cord injury (SCI) is greatly due to neuroplasticity - the process by which the nervous system responds to injury by establishing new synaptic connections or by altering the strength of existing synapses. However, the same neuroplasticity that allows locomotor function to recover also produces negative consequences such as pain and dysfunction of organs controlled by the autonomic nervous system. In this review we focus specifically on structural neuroplasticity (the growth of new synaptic connections) after SCI and on the consequent development of pain and autonomic dysreflexia, a condition of episodic …

Paediatricians, Social Media And Blogs: Ethical Considerations, Thérèse St-Laurent-Gagnon, Kevin W. Coughlin

Paediatrics Publications

The use of blogs, Facebook and similar social networking sites is rapidly expanding and, when compared with e-mail, may be having a significantly different impact on the traditional doctor-patient relationship. Characteristics specific to these online platforms have major implications for professional relationships, including the ‘Facebook effect’ (the relative permanence of postings) and the ‘online disinhibition effect’. The present practice point illustrates relevant ethical considerations and provides guidance to paediatricians and others concerning the prudent professional and personal use of social networking media.

L’utilisation des blogues, de Facebook et de sites de réseautage social similaires prend une expansion rapide. Par rapport …

Program Implementers' Evaluation Of The Project P.A.T.H.S.: Findings Based On Different Datasets Over Time, Daniel T. L. Shek, Cecilia M. S. Ma

Pediatrics Faculty Publications

This paper integrates the evaluation findings based on program implementers in nine datasets collected from 2005 to 2009 (244 schools and 7,926 implementers). Using consolidated data with schools as the unit of analysis, results showed that program implementers generally had positive perceptions of the program, themselves, and benefits of the program, with more than four-fifths of the implementers regarding the program as beneficial to the program participants. The subjective outcome evaluation instrument was found to be internally consistent. Multiple regression analyses revealed that perceived qualities of the program and program implementers predicted perceived effectiveness of the program. In conjunction with …

Recognition For Positive Behavior As A Critical Youth Development Construct: Conceptual Bases And Implications On Youth Service Development, Ben M. F. Law, Andrew M. H. Siu, Daniel T. L. Shek

Pediatrics Faculty Publications

Recognition for positive behavior is an appropriate response of the social environment to elicit desirable external behavior among the youth. Such positive responses, rendered from various social systems, include tangible and intangible reinforcements. The following theories are used to explain the importance of recognizing positive behavior: operational conditioning, observational learning, self-determination, and humanistic perspective. In the current work, culturally and socially desirable behaviors are discussed in detail with reference to Chinese adolescents. Positive behavior recognition is especially important to adolescent development because it promotes identity formation as well as cultivates moral reasoning and social perspective thinking from various social systems. …

Novel Napi-Iic Mutations Causing Hhrh And Idiopathic Hypercalciuria In Several Unrelated Families: Long-Term Follow-Up In One Kindred., Y Yu, S R. Sanderson, M Reyes, A Sharma, N Dunbar, Tarak Srivastava, H Jüppner, C Bergwitz

Manuscripts, Articles, Book Chapters and Other Papers

Homozygous and compound heterozygous mutations in SLC34A3, the gene encoding the sodium-dependent co-transporter NaPi-IIc, cause hereditary hypophosphatemic rickets with hypercalciuria (HHRH), a disorder characterized by renal phosphate-wasting resulting in hypophosphatemia, elevated 1,25(OH)(2) vitamin D levels, hypercalciuria, rickets/osteomalacia, and frequently kidney stones or nephrocalcinosis. Similar albeit less severe biochemical changes are also observed in heterozygous carriers, which are furthermore indistinguishable from those encountered in idiopathic hypercalciuria (IH). We now searched for SLC34A3 mutations (exons and introns) in two previously not reported HHRH kindreds, which resulted in the identification of three novel mutations. The affected members of kindred A were compound heterozygous …

Early Developmental Outcome In Children With Hypoplastic Left Heart Syndrome And Related Anomalies: The Single Ventricle Reconstruction Trial., Jane W. Newburger, Lynn A. Sleeper, David C. Bellinger, Caren S. Goldberg, Sarah Tabbutt, Minmin Lu, Kathleen A. Mussatto, Ismee A. Williams, Kathryn E. Gustafson, Seema Mital, Nancy Pike, Erica Sood, William T. Mahle, David S. Cooper, Carolyn Dunbar-Masterson, Catherine Dent Krawczeski, Alan Lewis, Shaji C. Menon, Victoria L. Pemberton, Chitra Ravishankar, Teresa W. Atz, Richard G. Ohye, J William Gaynor, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass.

METHODS AND RESULTS: In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean …

Significant Differences In Markers Of Oxidant Injury Between Idiopathic And Bronchopulmonary-Dysplasia-Associated Pulmonary Hypertension In Children, Kimberly B. Vera, Donald Moore, English Flack, Michael Liske, Marshall Summar

Pediatrics Faculty Publications

While oxidant stress is elevated in adult forms of pulmonary hypertension (PH), levels of oxidant stress in pediatric PH are unknown. The objective of this study is to measure F₂-isoprostanes, a marker of oxidant stress, in children with idiopathic pulmonary hypertension (IPH) and PH due to bronchopulmonary dysplasia (BPD). We hypothesized that F₂-isoprostanes in pediatric IPH and PH associated with BPD will be higher than in controls. Plasma F₂-isoprostanes were measured in pediatric PH patients during clinically indicated cardiac catheterization and compared with controls. F₂-Isoprostane levels were compared between IPH, PH due …

School Age Children: The Effects Of Hunger, Dacia Bessler

Honors Capstones

Capstone submitted as a graduation requirement for the BSU Honors Program.

Spirituality As A Positive Youth Development Construct: A Conceptual Review, Daniel T. L. Shek

Pediatrics Faculty Publications

The concept of spirituality as a positive youth development construct is reviewed in this paper. Both broad and narrow definitions of spirituality are examined and a working definition of spirituality is proposed. Regarding theories of spirituality, different models pertinent to spiritual development and the relationship between spirituality and positive youth development are highlighted. Different ecological factors, particularly family and peer influences, were found to influence spirituality. Research on the influence of spirituality on adolescent developmental outcomes is examined. Finally, ways to promote adolescent spirituality are discussed.