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  • ISSN: 2373-9312
    Volume 3, Issue 9
    Research Article
    Andreanne Villeneuve, Jean-Luc Bigras, Christian Lachance, Denis Berube, Keith J. Barrington, Anie Lapointe, Ahmed Moussa*
    Abstract
    Objective : To characterize the incidence of pulmonary hypertensionin a cohort of patients with bronchopulmonary dysplasia [BPD] and tocorrelate echocardiographic markers of pulmonary artery pressure [PAP] with prolonged oxygen supplementation, blood oxygen saturation [SpO2], pH and pCO2. Study design: We prospectively studied 29 infants admitted to a level 3 Neonatal Intensive Care unit [NICU] between February 2006 and August 2007. Neonates born at less than 28 weeks of gestation and requiring oxygen supplementation at 34-36 weeks of postmenstrual age were included. Echocardiographic estimation of pulmonary arterial pressure [PAP] was done with interventricle septal motion, tricuspid regurgitation jet velocity, right ventricular pre-ejection period/ejection time ratio [RVPEP/RVET] and right ventricular acceleration time/ejection time ratio [RVAT/RVET]] at weeks 0, 1, 4 and 6 of study. These echocardiographic measurements were assessed for correlation with duration of oxygen therapy, SpO2, pH and pCO2.
    Results: Twenty-nine patients were enrolled at a mean postmenstrual age of 35 weeks and 3 days [±6 days [SD]]. BPD was moderate in 62% and severe in 38%. Twenty-four patients required prolonged oxygen therapy [oxygen needed past 44 weeks postmenstrual age]. RVPEP/RVET ratio was 0.21 in these patients compared to 0.13 [p=0.02] in those that did not require prolonged oxygen therapy. RVPEP/RVET ratio correlated with low pH [p=0.02] and high pCO2 [p=0.04]. It did not correlate with SpO2 levels. Conclusion: In infants with BPD, the RVPEP/RVET ratio at 34-42 weeks of postmenstrual agewas higher in infants requiring prolonged oxygen therapy, and correlated with pH and pCO2. This RVPEP/RVET ratio could help with early identification of patientsthat will require prolonged oxygen therapy.
    Kawsari Abdullah, Gerald Lebovic, Catherine S. Birken, Jonathon L. Maguire, Jennifer Jenkins, Patricia C. Parkin* and on behalf of the TARGet Kids! Collaboration
    Abstract
    Objective: To determine whether there is an association between parent reported child temperament and nutrition risk in preschool aged children.
    Methods: This was a cross-sectional study. Healthy children aged between 3-5 years were recruited from community-based primary care practices. Temperament was assessed using the Children's Behavior Questionnaire Very Short Form (CBQ-VSQ) which identifies three domains: Negative Affectivity, Effortful Control, and Extraversion/Surgency. Nutrition risk was assessed using the Nutrition Screening Tool for Every Pre-schooler Questionnaire (NutriSTEPTM). Adjusted logistic regression was used to measure the association between individual temperament domain and nutrition risk.
    Result: 3468 children were included (mean age 3.75 years, 48 % female) in the study. The mean (SD) of Negative Affectivity, Effortful Control, and Extraversion/Surgency, were 3.79 (0.85), 5.46 (0.72) and 4.56 (0.79) respectively. About 14% (n=472) of the children had high nutrition risk. Children who scored high in Negative Affectivity and high in Effortful Control had an odds ratio of 1.47 (95% CI: 1.30, 1.66, p = <0.001) and 0.73 (95% CI: 0.64, 0.84, p = <0.001) respectively for high nutrition risk.
    Conclusion: This study found children whose temperament is characterized by Negative Affectivity (discomfort, fear, anger/frustration, sadness and poor soothability) are more likely to be associated with high nutrition risk. These concepts can alert physicians to behavior patterns in children that may predispose them to poor nutrition and guide clinical decision making through laboratory testing for nutrition status, counseling and close monitoring.
    Cynthia Lewis*, Anil Kumar Swayampakula, Alexandra Smith, Richard Neugebauer and Ayoade Adeniyi
    Abstract
    Objective: To assess pediatric residents' knowledge of and attitudes about IPV at a community hospital in the South Bronx before and after IPV training.
    Methods: A seven-hour IPV training program on IPV relevant screening, education, policies, and resources was offered in July/August 2010 and again in July/August 2011. Residents completed a pre- and post-training survey consisting of 60 questions divided into 14 distinct themes/competencies areas. Mean scores were calculated for each theme/competency. Paired sample t-tests were used to evaluate participants' pre-test and post-test change in survey responses. After the 2011 training module, a convenience sample of caregivers and adolescents were assessed with a separate health visit exit-survey focusing on IPV and provider interview.
    Results: Thirty-six residents were trained and surveyed in 2010 (PGY-1 and 2), 18 (50%) received a second training module in 2011. The baseline survey showed that 60% of the residents lacked prior IPV-specific training, and 50% were unaware of relevant community resources. Mean values for 12 out of the 14 IPV-relevant themes improved significantly after the initial training module and exhibited further improvement in several areas of theme/competency including self-efficacy, referral, screening, workplace limitations, legal requirements, and victim understanding after the 2nd training module in 2011. Among the teenager sample (n=113) (mean age [SD] of 14.2 [2.2] years), 86% indicated that they would ask their provider for help if they were victims of IPV; while 80% of the caregivers (n=101) said they would discuss healthy relationships with their teenagers.
    Conclusion: This IPV-specific training module for residents improved significantly their IPV-relevant competencies. Our findings demonstrate that IPV-specific training would be a beneficial addition to the Pediatric Residency Training Curriculum.
    Case Report
    Anna CE Hurst*, Crescenda L Williams, Katherine R Nelson, Elliot S Stolerman, Nathaniel H Robin
    Abstract
    Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) is an autosomal dominant condition caused by mutations in the PTCH1 gene. It is characterized by distinct skeletal and craniofacial anomalies with an increased risk for basal cell carcinomas. We present a 5-year-old male with a family history of NBCCS, found to have a previously unreported 2.7kb partial duplication in the PTCH1 gene. The patient has several classical craniofacial findingsof NBCCS (macrocephaly and frontal bossing), but he also displays additional manifestationsof nystagmus and immobile first digit interphalangeal joints due to hypoplastic flexor muscles with underlying normal bony anatomy. Pathogenic mutations are typically protein truncating due to deletions or missense mutations; there have only been three previous reports of duplications in PTCH1. Although nystagmus is frequently listed as an eye anomaly associated with NBCC, it has been reported in just 3 members of the same family (all with rotary nystagmus). Hand abnormalities such as polydactyly, metacarpal shortening, and cutaneous 2,3 syndactyly have been reported, but thumb abnormalities are rare, and there are no reports of immobile thumbs to due hypoplastic flexor muscles. This patient's novel genetic duplication and combination of classical and uncommon features of NCBBS offer the opportunity for both expansion of the NBCCS phenotype and further genotype-phenotype correlations. This case also reiterates the importance of copy number analysis for both duplication and deletion events in the PTCH1 gene.
    Bhaskar Roy and Francis J. Di Mario Jr*
    Abstract
    Refractory epilepsy in childhood is a common presentation of cortical heterotopia, a form of neuronal migration disorders. Subcortical band heterotopia or double cortex syndrome, subependymal heterotopia and subcortical heterotopia are three common types of cortical heterotopia.Here we present a case of 10 year old non dysmorphic boy with static encephalopathy who was evaluated for refractory epilepsy.He had significant cognitive impairment, developmental delay, aggressive behavioral outbursts and self mutilating behavior. He started to have seizures approximately at age six month and over the years seizures evolved to different patterns representing Lennox Gastaut syndrome. His EEG reflected a slow and disorganized pattern with bitemporal slow spikes. His brain MRI revealed band cortical heterotopia without hippocampal pathology or mesial temporal lobe sclerosis. He did not have any genetic abnormality on karyotyping, chromosome micro-array or whole exome sequencing. He was dignosed with double cortex syndrome based on clinioradiological findings without any identifiable genetic abnormality. This case reportalong with review of literature reinforce the different spectrum of clinical manifestations of different types of cortical heterotopia.
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