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https://www.readbyqxmd.com/read/29920145/a-descriptive-study-of-decision-making-conversations-during-pediatric-intensive-care-unit-family-conferences
#1
Michael A Smith, Marla L Clayman, Joel Frader, Melanie Arenson, Natalie Haber-Barker, Claire Ryan, Linda Emanuel, Kelly Michelson
BACKGROUND: Little is known about how decision-making conversations occur during pediatric intensive care unit (PICU) family conferences (FCs). OBJECTIVE: Describe the decision-making process and implementation of shared decision making (SDM) during PICU FCs. DESIGN: Observational study. SETTING/SUBJECTS: University-based tertiary care PICU, including 31 parents and 94 PICU healthcare professionals involved in FCs. MEASUREMENTS: We recorded, transcribed, and analyzed 14 PICU FCs involving decision-making discussions...
June 19, 2018: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/29916983/mandibular-reconstruction-for-pediatric-patients
#2
Loreto Castellon, Daniel Jerez, Jaime Mayorga, Alejandra Gallego, Carlos Fuenzalida, German Laissle
: Mandibular reconstruction in pediatric patients is controversial. The scant pediatric experience and the infrequent occurrence of this type of pathology in children complicate therapeutic decisions. The literature contains different treatment protocols and describes the bone graft alternatives to be used. MATERIALS AND METHOD: This is a retrospective review of patients under the age of 15 who were subjected to resection and reconstruction. RESULTS: A total of 18 patients were included in the study: 8 women and 10 men...
June 18, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29916974/evaluation-of-fronto-orbital-advancement-using-titanium-based-internal-fixation-for-corrective-pediatric-craniofacial-surgery
#3
Matthias Kreppel, Martin Kauke, Andrea Grandoch, Ali-Farid Safi, Hans-Joachim Nickenig, Joachim Zöller
BACKGROUND: For successful reshaping of the cranial vault in terms of corrective fronto-orbital advancement (FOA) efficient and reliable internal fixation systems for stabilization of bone fragments are indispensable. The rate of complications and feasibility of corrective FOA with the usage of titanium-based rigid fixation systems is rarely analyzed and discussed. METHODS: The authors retrospectively reviewed the medical records of 42 patients who received corrective FOA with the implementation of titanium internal rigid fixation and consecutive secondary surgery for removal...
June 18, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29914177/effect-of-nicotine-on-cyp2b1-expression-in-a-glioma-animal-model-and-analysis-of-cyp2b6-expression-in-pediatric-gliomas
#4
Sonia Nava-Salazar, Saúl Gómez-Manzo, Jaime Marcial-Quino, Alfonso Marhx-Bracho, Bryan V Phillips-Farfán, Carlos Diaz-Avalos, America Vanoye-Carlo
Cyclophosphamide (CPA) is a pro-drug commonly used in the chemotherapeutic schemes for glioma treatment but has high toxicity and the side effects include brain damage and even death. Since CPA is activated mainly by CY2B6, over-expression of the enzyme in the tumor cells has been proposed to enhance CPA activation. In this study, we explored the induction of the Cyp2b1 (homologous to CYP2B6 ) by nicotine in an animal rat model with glioma. Gene expression and protein levels were analyzed by RT-PCR and Western blot...
June 16, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29913001/efficacy-of-rituximab-vs-tacrolimus-in-pediatric-corticosteroid-dependent-nephrotic-syndrome-a-randomized-clinical-trial
#5
Biswanath Basu, Anja Sander, Birendranath Roy, Stella Preussler, Shilpita Barua, T K S Mahapatra, Franz Schaefer
Importance: Calcineurin inhibitors are an established first-line corticosteroid-sparing therapy for patients with corticosteroid-dependent nephrotic syndrome (CDNS), whereas B-lymphocyte-depleting therapy is mostly used as a rescue for calcineurin inhibitor-resistant cases. The positive efficacy and safety profile of rituximab raises the question of whether it could be used as a first-line alternative to calcineurin inhibitor therapy. Objective: To compare the efficacy of rituximab and tacrolimus in maintaining relapse-free survival among children with CDNS...
June 18, 2018: JAMA Pediatrics
https://www.readbyqxmd.com/read/29909939/renal-artery-embolization-in-a-child-with-delayed-hemodynamic-instability-from-penetrating-knife-wound
#6
M Barras, E Pearson, I Cousin, C Le Rouzic, M Thepaut, J-C Gentric, J-M Roue, S Yevich, P de Vries
Penetrating laceration injury in the pediatric population may present as an acute or delayed life-threatening injury. Although emergent intra-arterial embolization is commonly utilized in adults, few cases have been reported for children. Surgical treatment for severe renal laceration injuries may require complete nephrectomy; an unfortunate outcome for a pediatric patient if a renal-preserving alternative is feasible. We present a case of penetrating renal laceration in a 10-year-old boy treated with intra-arterial embolization of the lacerated dominant renal artery and subsequent renal perfusion by an uninjured accessory renal artery allowing for renal preservation...
June 14, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29904533/using-the-tools-of-informal-science-education-to-connect-science-and-the-public
#7
April Killikelly
Traditional modes of communication within the scientific community, including presentation of data at conferences and in peer-reviewed publications, use technical jargon that limits public engagement. While altering word choice is an important method for increasing public engagement, the data itself may not be enough. For example, communicating the lack of evidence that vaccines cause autism did not convince many reluctant parents to vaccinate their kids (Nyhan, Reifler, Richey, Freed, Pediatrics 133:e835-e842, 2014)...
2018: Journal of Microbiology & Biology Education: JMBE
https://www.readbyqxmd.com/read/29901540/a-computed-tomography-study-of-the-c7-vertebra-screws-fixation-in-children
#8
Junhao Liu, Wei Ji, Zucheng Huang, Ganggang Kong, Qi Liu, Zhiping Huang, Xiuhua Wu, Qingan Zhu
STUDY DESIGN: Retrospective analysis of computed tomographic images of 92 normal pediatric cervical vertebrae. OBJECTIVE: To ascertain the feasibility of the pedicle, intralaminar, and lateral mass screws fixation in children. SUMMARY OF BACKGROUND DATA: Fixation techniques of C7 vertebrae is technically challenging in the pediatric population. Although the techniques offer alternatives to screws placement at C7 spine in adult, there is no consensus to apply these screws in children...
June 12, 2018: Spine
https://www.readbyqxmd.com/read/29895895/missense-variants-in-atp1a3-and-fxyd-gene-family-are-associated-with-childhood-onset-schizophrenia
#9
Boris Chaumette, Vladimir Ferrafiat, Amirthagowri Ambalavanan, Alice Goldenberg, Alexandre Dionne-Laporte, Dan Spiegelman, Patrick A Dion, Priscille Gerardin, Claudine Laurent, David Cohen, Judith Rapoport, Guy A Rouleau
Childhood-onset schizophrenia (COS) is a rare and severe form of schizophrenia defined as onset before age of 13. Here we report on two unrelated cases diagnosed with both COS and alternating hemiplegia of childhood (AHC), and for whom two distinct pathogenic de novo variants were identified in the ATP1A3 gene. ATP1A3 encodes the α-subunit of a neuron-specific ATP-dependent transmembrane sodium-potassium pump. Using whole exome sequencing data derived from a cohort of 17 unrelated COS cases, we also examined ATP1A3 and all of its interactors known to be expressed in the brain to establish if variants could be identified...
June 12, 2018: Molecular Psychiatry
https://www.readbyqxmd.com/read/29895509/usefulness-of-chromogenic-assays-for-potency-assignment-and-recovery-of-plasma-derived-fviii-and-fix-concentrates-or-their-recombinant-long-acting-therapeutic-equivalents-with-potential-application-in-treated-pediatric-hemophiliac-patients
#10
REVIEW
Jean Amiral, Jerard Seghatchian
On demand and prophylaxis usage of FVIII/ FIX concentrates for the therapeutic management of hemophilia has greatly changed quality of life, and healthy life span of affected patients. Availability of recombinant therapeutic FVIII and FIX products, and of their long-acting variants, further improves the treatment constraints, and progressively permits to hemophiliacs to have an almost normal way of life. Unlimited amounts of recombinant or engineered substitutive products become available, and open new avenues for extending the benefits of prophylaxis to all hemophiliac patients, not only in economically advanced territories, but also in emerging and developing countries, worldwide...
May 16, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29895017/transition-in-pediatric-and-adolescent-hypogonadal-girls-gynecological-aspects-estrogen-replacement-therapy-and-contraception
#11
Anette Tønnes Pedersen, Line Cleemann, Katharina M Main, Anders Juul
Hypogonadism may be suspected if puberty is delayed. Pubertal delay may be caused by a normal physiological variant, by primary ovarian insufficiency (Turner syndrome), or reflect congenital hypogonadotropic hypogonadism (HH; genetic) or acquired HH (brain lesions). Any underlying chronic disease like inflammatory bowel disease, celiac disease, malnutrition (anorexia or orthorexia), or excessive physical activity may also result in functional HH. Thus, girls with delayed puberty should be evaluated for an underlying pathology before any treatment, including oral contraception, is initiated...
2018: Endocrine Development
https://www.readbyqxmd.com/read/29892808/use-of-endoscopic-balloon-dilation-for-benign-esophageal-stenosis-in-children-our-11-year-experience
#12
Tatsuya Osuga, Yoshihiro Ikura, Kazunori Hasegawa, Seiichi Hirano
Endoscopic balloon dilation (EBD) is an established therapy for esophageal stenosis. To assess its usefulness in children, we reviewed 11 years of pediatric cases of EBD in our hospital. Over the last 11 years, EBD was performed on 14 pediatric patients (10 boys and four girls; 7 months-11 years) at our hospital. All EBD sessions were performed under general anesthesia and fluoroscopic monitoring. The sessions were repeated every week or alternate weeks until resolution of dysphagia or development of smooth endoscope passage...
January 2018: Esophagus: Official Journal of the Japan Esophageal Society
https://www.readbyqxmd.com/read/29890870/economic-burden-work-and-school-productivity-in-individuals-with-tuberous-sclerosis-and-their-families
#13
Anne M Skalicky, Anne M Rentz, Zhimei Liu, Qayyim Said, Jo Anne Nakagawa, Michael D Frost, James W Wheless, David W Dunn
AIMS: Tuberous sclerosis complex (TSC) is a multi-organ autosomal-dominant, genetic disorder with incomplete penetrance. The multiple manifestations of TSC and impacts to numerous organ systems represent significant disease, health care and treatment burden. The economic and employment burden of the disease on individuals and their families is poorly understood. This study assessed the cost of illness and work and school productivity burden associated with TSC in a cross-sectional web-survey sample...
June 11, 2018: Journal of Medical Economics
https://www.readbyqxmd.com/read/29889582/mutant-atrx-uncovering-a-new-therapeutic-target-for-glioma
#14
Santiago Haase, María Belén Garcia-Fabiani, Stephen Carney, David Altshuler, Felipe J Núñez, Flor M Méndez, Fernando Núñez, Pedro R Lowenstein, Maria G Castro
ATRX is a chromatin remodeling protein whose main function is the deposition of the histone variant H3.3. ATRX mutations are widely distributed in glioma, and correlate with alternative lengthening of telomeres (ALT) development, but they also affect other cellular functions related to epigenetic regulation. Areas covered: We discuss the main molecular characteristics of ATRX, from its various functions in normal development to the effects of its loss in ATRX syndrome patients and animal models. We focus on the salient consequences of ATRX mutations in cancer, from a clinical to a molecular point of view, focusing on both adult and pediatric glioma...
June 11, 2018: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/29888625/electron-microscopy-can-still-have-a-role-in-the-diagnosis-of-selected-inborn-errors-of-metabolism
#15
Mana Taweevisit, Paul S Thorner
Many anatomic pathology laboratories no longer have electron microscopy facilities. A retrospective review of autopsies was performed to identify cases of inborn errors of metabolism (IEM) and determine the contribution of electron microscopy in making the diagnosis in those cases. Over a period of 17 years, there were 900 perinatal and pediatric autopsies. There were 7 cases (1%) of IEM, including 4 cases of Pompe disease, 1 case of I-cell disease, 1 case of bile acid synthesis defect, and 1 case of mitochondrial disease (Leigh syndrome)...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29886909/two-for-one-a-change-in-hand-positioning-during-low-dose-spinal-stereoradiography-allows-for-concurrent-reliable-sanders-skeletal-maturity-staging
#16
Taylor J Jackson, Daniel Miller, Susan Nelson, Patrick J Cahill, John M Flynn
STUDY DESIGN: Prospective survey. OBJECTIVES: To evaluate the reliability of low-dose stereoradiography compared to standard hand bone age films for assessing Sanders skeletal maturity stage in patients with idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: The Sanders skeletal maturity staging system is a valuable tool in the care of juvenile and adolescent spine scoliosis, but obtaining dedicated hand films adds additional time, radiation, and expense to the clinic visit...
July 2018: Spine Deformity
https://www.readbyqxmd.com/read/29886008/medial-patellofemoral-ligament-repair-restores-stability-in-pediatric-patients-when-compared-to-reconstruction
#17
Jessica Bryant, Nirav Pandya
BACKGROUND: Pediatric patellar instability has a high recurrence rate with non-operative care, and medial patellofemoral ligament (MPFL) reconstruction has known complications. MPFL repair offers an alternative method to restore patellar stability. This study's purpose was to assess the outcomes of MPFL repair in a pediatric cohort, and to compare these outcomes to a cohort of pediatric patients who underwent MPFL reconstruction. METHODS: One surgeon performed 16 MPFL repairs on pediatric patients for traumatic patellar instability, with an average follow-up of 1...
June 6, 2018: Knee
https://www.readbyqxmd.com/read/29884845/williams-syndrome-neuroanatomical-score-associates-with-gtf2ird1-in-large-scale-magnetic-resonance-imaging-cohorts-a-proof-of-concept-for-multivariate-endophenotypes
#18
Chun Chieh Fan, Andrew J Schork, Timothy T Brown, Barbara E Spencer, Natacha Akshoomoff, Chi-Hua Chen, Joshua M Kuperman, Donald J Hagler, Vidar M Steen, Stephanie Le Hellard, Asta Kristine Håberg, Thomas Espeseth, Ole A Andreassen, Anders M Dale, Terry L Jernigan, Eric Halgren
Despite great interest in using magnetic resonance imaging (MRI) for studying the effects of genes on brain structure in humans, current approaches have focused almost entirely on predefined regions of interest and had limited success. Here, we used multivariate methods to define a single neuroanatomical score of how William's Syndrome (WS) brains deviate structurally from controls. The score is trained and validated on measures of T1 structural brain imaging in two WS cohorts (training, n = 38; validating, n = 60)...
June 8, 2018: Translational Psychiatry
https://www.readbyqxmd.com/read/29883628/duplex-real-time-polymerase-chain-reaction-assay-for-the-detection-of-human-kipyv-and-wupyv-in-nasopharyngeal-aspirate-pediatric-samples
#19
Marco Ligozzi, Liliana Galia, Maria Carelli, Pier Paolo Piccaluga, Erica Diani, Davide Gibellini
In this study, we describe a duplex real-time PCR assay for the simultaneous detection of KIPyV and WUPyV polyomaviruses based on TaqMan probes. This assay detected 500 copies/mL both for KIPyV and WUPyV in 100% of tested positive samples. We assessed this technique on 482 nasopharyngeal aspirate specimens from hospitalized pediatric patients with respiratory symptoms, previously analyzed with commercial multiplex assay for 16 major respiratory viruses. Our assay detected KIPyV genome in 15 out of 482 samples (3...
June 5, 2018: Molecular and Cellular Probes
https://www.readbyqxmd.com/read/29881194/evaluation-of-angle-trunk-rotation-measurements-to-improve-quality-and-safety-in-the-management-of-adolescent-idiopathic-scoliosis
#20
Jill E Larson, Maximilian A Meyer, Barrett Boody, John F Sarwark
Purpose: The evaluation, management and follow-up of adolescent idiopathic scoliosis (AIS) occur frequently within a pediatric orthopedic surgery practice. Curve status can be assessed with Scoliometer measurements of angle trunk rotation (ATR), which are reliable and reproducible to within 3°. This study assessed the longitudinal efficacy, safety and cost savings of integrating ATR measurements to monitor curve status and progression in AIS, and suggests a quality-based management strategy...
June 2018: Journal of Orthopaedics
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