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https://www.readbyqxmd.com/read/28728258/-follow-up-and-genetic-study-of-43-chinese-children-with-type-%C3%A2-alexander-disease
#1
T T Ban, Y Wu, Z B Zhang, L L Zang, J M Wang, Y W Jiang
Objective: To identify the clinical and genetic characteristics in 43 Chinese children diagnosed with type Ⅰ Alexander disease (AxD). Method: Forty-three type Ⅰ AxD cases identified by glial fibrillary acidic protein (GFAP) gene mutations in Peking University First Hospital from 2005 to 2016 were followed up. The data of medical history, physical examination and magnetic resonance imaging (MRI) were collected. All these patients were followed up in December 2010, Febury 2012, June 2014 and January 2016, respectively...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28721492/peristaltic-like-motion-of-the-human-fetal-right-ventricle-and-its-effects-on-fluid-dynamics-and-energy-dynamics
#2
Hadi Wiputra, Guat Ling Lim, Khong Chun Chua, R Nivetha, Sanah Merchant Soomar, Arijit Biwas, Citra Nurfarah Zaini Mattar, Hwa Liang Leo, Choon Hwai Yap
In both adult human and canine, the cardiac right ventricle (RV) is known to exhibit a peristaltic-like motion, where RV sinus (inflow region) contracts first and the infundibulum (outflow region) later, in a wave-like contraction motion. The delay in contraction between the sinus and infundibulum averaged at 15% of the cardiac cycle and was estimated to produce an intra-ventricular pressure difference of 15 mmHg. However, whether such a contractile motion occurs in human fetuses as well, its effects on hemodynamics remains unknown, and are the subject of the current study...
July 18, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/28717864/diagnosing-developmental-dysplasia-of-the-hip-using-the-graf-ultrasound-method-risk-and-protective-factor-analysis-in-11-820-universally-screened-newborns
#3
Mohammad Schams, Rob Labruyère, Anne Zuse, Mikolaj Walensi
The essential role of ultrasound examinations in diagnosis and treatment of developmental dysplasia of the hip (DDH) is widely accepted while the weighting and correlation of protective factors and perinatal risk for DDH still give rise to debate. Our aim was to investigate the impact of single and twofold combined risk and protective factors on the newborns' hip maturity, assessed with the Graf ultrasound method. Therefore, data sets of 11,820 universally screened newborns were analyzed. Univariate and logistic regression analyses were performed to correlate risk and protective factors with mature or immature but appropriate for age and pathologic hip types...
July 17, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28716012/case-reports-of-juvenile-gm1-gangliosidosisis-type-ii-caused-by-mutation-in-glb1-gene
#4
Parvaneh Karimzadeh, Samaneh Naderi, Farzaneh Modarresi, Hassan Dastsooz, Hamid Nemati, Tayebeh Farokhashtiani, Bibi Shahin Shamsian, Soroor Inaloo, Mohammad Ali Faghihi
BACKGROUND: Type II or juvenile GM1-gangliosidosis is an autosomal recessive lysosomal storage disorder, which is clinically distinct from infantile form of the disease by the lack of characteristic cherry-red spot and hepatosplenomegaly. The disease is characterized by slowly progressive neurodegeneration and mild skeletal changes. Due to the later age of onset and uncharacteristic presentation, diagnosis is frequently puzzled with other ataxic and purely neurological disorders. Up to now, 3-4 types of GM1-gangliosidosis have been reported and among them type I is the most common phenotype with the age of onset around 6 months...
July 17, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28715158/loss-to-follow-up-among-children-and-adolescents-growing-up-with-hiv-infection-age-really-matters
#5
Katharina Kranzer, John Bradley, Joseph Musaazi, Mary Nyathi, Hilary Gunguwo, Wedu Ndebele, Mark Dixon, Mbongeni Ndhlovu, Andrea Rehman, Palwasha Khan, Florian Vogt, Tsitsi Apollo, Rashida Abbas Ferrand
INTRODUCTION: Globally, increasing numbers of HIV-infected children are reaching adolescence due to antiretroviral therapy (ART). We investigated rates of loss-to-follow-up (LTFU) from HIV care services among children as they transition from childhood through adolescence. METHODS: Individuals aged 5-19 years initiated on ART in a public-sector HIV clinic in Bulawayo, Zimbabwe, between 2005 and 2009 were included in a retrospective cohort study. Participants were categorized into narrow age-bands namely: 5-9 (children), 10-14 (young adolescents) and 15-19 (older adolescents)...
July 17, 2017: Journal of the International AIDS Society
https://www.readbyqxmd.com/read/28707233/inflammatory-follicular-cysts-associated-to-necrotic-primary-teeth
#6
N P Marques, N C T Marques, V T Sakai, J A C Hanemann
BACKGROUND: Cysts are pathological cavities lined by epithelium and supported by connective tissue, containing fluid or semi-fluid substances and presenting developmental or inflammatory origins. CASE REPORT: This study aimed to evaluate a case series of inflammatory follicular cysts in children to clarify their nomenclature, diagnosis, clinical implications and treatment outcomes. Prevalence, aetio-pathogenesis, clinical, radiographic and microscopic findings, treatments and sequelae of this condition were assessed by using the records of patients treated between 2000 and 2015...
July 13, 2017: European Archives of Paediatric Dentistry: Official Journal of the European Academy of Paediatric Dentistry
https://www.readbyqxmd.com/read/28705692/how-early-do-social-determinants-of-health-begin-to-operate-results-from-the-fragile-families-and-child-wellbeing-study
#7
Anao Zhang, Yolanda C Padilla, Yeonwoo Kim
From a life course perspective, important insights about how social determinants of health operate can be gained by analyzing the various forms that social climate can take in different life periods. For children, a critical aspect of social climate is exposure to bullying. Bullying can serve as a proxy for power imbalance and social exclusion analogous to adult social climate of discrimination and racism. We used the Year 9 follow-up data of the Fragile Families and Child Wellbeing Study (N=3301) that, for the first time included interviews with the children...
July 10, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28701062/prediction-of-the-requirement-of-open-reduction-for-developmental-dysplasia-of-the-hip
#8
Nabil Alassaf
Objective Closed reduction (CR) is a noninvasive treatment for developmental dysplasia of the hip (DDH), and this treatment is confirmed intraoperatively. This study aimed to develop a preoperative estimation model of the probability of requiring open reduction (OR) for DDH. Methods The study design was cross-sectional by screening all patients younger than 2 years who had attempted CR between October 2012 and July 2016 by a single surgeon. Potential diagnostic determinants were sex, age, side, bilaterality, International Hip Dysplasia Institute (IHDI) grade, and acetabular index (AI)...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28698723/socioeconomic-status-index-to-interpret-inequalities-in-child-development
#9
Mahbobeh Ahmadi Doulabi, Firoozeh Sajedi, Roshanak Vameghi, Mohammad Ali Mazaheri, Alireza Akbarzadeh Baghban
OBJECTIVE: There have been contradictory findings on the relationship between Socioeconomic Status (SES) and child development although SES is associated with child development outcomes. The present study intended to define the relationship between SES and child development in Tehran kindergartens, Iran. MATERIALS & METHODS: This cross-sectional survey studied 1036 children aged 36-60 month, in different kindergartens in Tehran City, Iran, in 2014-2015. The principal factor analysis (PFA) model was employed to construct SES indices...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28694615/prevalence-of-autism-spectrum-disorder-among-rural-urban-and-tribal-children-1-10-years-of-age
#10
Sunil Kumar Raina, Vishav Chander, Ashok K Bhardwaj, Dinesh Kumar, Seema Sharma, Vipasha Kashyap, Mitasha Singh, Amit Bhardwaj
INTRODUCTION: Studies on autism spectrum disorders (ASDs) have largely focused on children in specific settings. The current scenario of research in ASDs is limited largely to clinic-based case reports, case series, and retrospective chart reviews. The present study is the first population-based prevalence study conducted across rural, urban, and tribal populations in India. MATERIALS AND METHODS: A cross-sectional two-phase study was conducted covering children in the age group of 1-10 years of age across geographical regions representing rural, urban, and tribal populations...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28693965/maternal-pre-gravid-cardiometabolic-health-and-infant-birthweight-a-prospective-pre-conception-cohort-study
#11
R Retnakaran, S W Wen, H Tan, S Zhou, C Ye, M Shen, G N Smith, M C Walker
BACKGROUND AND AIMS: Both low birthweight and high birthweight have been associated with the development of cardiometabolic disease in adulthood, possibly reflecting the effect of intrauterine fetal programming. As developmental programming can begin before conception, pre-gravid factors that predict birthweight may be relevant in this context. However, little is known about such factors. Thus, we established a pre-conception cohort to identify maternal pre-gravid cardiometabolic determinants of infant birthweight...
May 23, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/28692578/turning-1-year-of-age-in-a-low-socioeconomic-environment-a-portrait-of-disadvantage
#12
Hallam Hurt, Laura M Betancourt
In the United States today, 16 million children are growing up poor. Few studies report multiple environmental factors associated with poverty during the first year of life and effects on infant development. OBJECTIVES: To evaluate maternal, home, and neighborhood environment of low and higher socioeconomic status (SES) infants from birth to 1 year and to evaluate the impact of SES and environment on infant developmental outcome at 1 year. METHODS: Low (n = 30) and higher SES (n = 30) African-American mothers and their healthy term gestation female infants were prospectively compared for environmental characteristics and infant developmental outcome...
June 29, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28691180/examining-the-association-between-language-expository-discourse-and-offending-behaviour-an-investigation-of-direction-strength-and-independence
#13
Thomas Hopkins, Judy Clegg, Joy Stackhouse
BACKGROUND: A high prevalence of Developmental Language Disorder (DLD) is reported in the population of Young Offenders (YO). However, little is known about the extent of the association between language and offending behaviour relative to social disadvantage, education attendance and non-verbal intelligence (IQ), and neither has this association been investigated with particular reference to the expository discourse abilities of YOs on community orders in the UK. AIMS: This study aimed to examine the direction and strength of the association between language and offending behaviour by comparing the receptive and expressive language and expository discourse abilities of male and female YOs and non-offenders in the UK, relative to the confounds of social disadvantage, years of education attended and non-verbal IQ...
July 10, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/28688968/are-surgical-milestone-assessments-predictive-of-in-training-examination-scores
#14
Mary K Kimbrough, Carol R Thrush, Emily Barrett, Frederick R Bentley, Kevin W Sexton
OBJECTIVES: With the recent utilization of Accreditation Council for Graduate Medical Education developmental milestones as part of resident evaluation, we sought to explore whether milestone-based ratings were associated with American Board of Surgery In-Training Examination (ABSITE) scores. METHODS: Mid-year milestone ratings were obtained from the Accreditation Council for Graduate Medical Education Accreditation Database System for years 2014, 2015, and 2016 for all postgraduate years 1-5 general surgery residents in our program and paired with ABSITE scores (n = 69) from January of the following year...
July 5, 2017: Journal of Surgical Education
https://www.readbyqxmd.com/read/28688787/retrospective-analysis-of-predictors-of-proficient-power-mobility-in-young-children-with-severe-motor-impairments
#15
Shelley R H Mockler, Irene R McEwen, Maria A Jones
OBJECTIVE: The primary objective was to determine if (a) child characteristics, (b) maternal education, (c) intervention parameters, (d) type of wheelchair control mechanism, or a combination of these variables, were associated with proficient power mobility skills in children with severe motor impairments aged 14 to 30 months. The secondary purpose was to determine if performance on the Wheelchair Skills Checklist (WSC) was associated with performance on the Powered Mobility Program (PMP)...
July 5, 2017: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/28683424/prenatal-phenolic-compounds-exposure-and-neurobehavioral-development-at-2-and-7years-of-age
#16
Ching-Chun Lin, Chun-Ju Chien, Meng-Shan Tsai, Chia-Jung Hsieh, Wu-Shiun Hsieh, Pau-Chung Chen
BACKGROUND: Phenolic compounds such as bisphenol A (BPA), nonylphenol (NP), and octylphenol (OP) are known as endocrine-disrupting compounds and are commonly used. Their impacts on the neurodevelopment of children are inconclusive. The current study aims to investigate the association between umbilical cord blood levels of BPA, NP, OP and neurodevelopmental outcomes at 2 and 7years of age. METHODS: The study was based on the Taiwan Birth Panel Study, a prospective birth cohort...
July 3, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28672093/does-age-matter-comparing-post-treatment-psychosocial-outcomes-in-young-adult-and-older-adult-cancer-survivors-with-their-cancer-free-peers
#17
Michael Lang, Janine Giese-Davis, Scott Patten, David J T Campbell
OBJECTIVE: Adolescents and Young Adult cancer survivors (AYA) are a unique sub-population with high levels of distress and unmet need. To date studies have not disentangled distress due to developmental life stage from distress due to cancer survivorship. This population-based study allowed a direct comparison between AYA cancer survivors, Older Adult (OA) cancer survivors and their cancer-free peers. METHODS: We combined four annual cycles of the Canadian Community Health Survey (CCHS, 2007-2010) to obtain a final sample of 239,316 respondents...
July 3, 2017: Psycho-oncology
https://www.readbyqxmd.com/read/28671981/parents-with-mental-health-problems-and-their-children-in-a-german-population-based-sample-results-of-the-bella-study
#18
Angela Plass-Christl, Anne-Catherine Haller, Christiane Otto, Claus Barkmann, Silke Wiegand-Grefe, Heike Hölling, Michael Schulte-Markwort, Ulrike Ravens-Sieberer, Fionna Klasen
BACKGROUND: Mental health problems (MHP) of parents are associated with an increased risk of psychological and developmental difficulties in their children. This study aims at analyzing population-based data of parents with MHP and their children and the effects of associated risk factors in order to further targeted preventive and therapeutic interventions. METHODS: The BELLA study is the mental health module of the German National Health Interview and Examination Survey among Children and Adolescents...
2017: PloS One
https://www.readbyqxmd.com/read/28671587/inborn-errors-of-metabolism-and-epilepsy-current-understanding-diagnosis-and-treatment-approaches
#19
REVIEW
Suvasini Sharma, Asuri N Prasad
Inborn errors of metabolism (IEM) are a rare cause of epilepsy, but seizures and epilepsy are frequently encountered in patients with IEM. Since these disorders are related to inherited enzyme deficiencies with resulting effects on metabolic/biochemical pathways, the term "metabolic epilepsy" can be used to include these conditions. These epilepsies can present across the life span, and share features of refractoriness to anti-epileptic drugs, and are often associated with co-morbid developmental delay/regression, intellectual, and behavioral impairments...
July 2, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28668758/gender-differences-and-stage-specific-influence-of-parent-adolescent-conflicts-on-adolescent-suicidal-ideation
#20
Yu-Ching Chiu, Chin-Yuan Tseng, Fu-Gong Lin
This study examined familial and peer related factors as predictors of suicidal ideation in school students. Total 2896 participants were included from Taiwan Youth Project released data, a longitudinal survey of adolescent suicidal ideation at ages 15, 18, and 20. Logistic regression analysis risk factors associated with adolescent suicidal ideation reveled differences during the developmental stages. After adjusted for psychological symptoms, effect of quarrels with parents on suicidal ideation lasts in early and middle stages; in the late adolescent stage, only cigarette or alcohol use remained significant...
June 27, 2017: Psychiatry Research
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