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https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#1
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29045353/impaired-il-10-receptor-mediated-suppression-in-monocyte-from-patients-with-crohn-s-disease
#2
Moran Y Nunberg, Lael Werner, Uri Kopylov, Yael Haberman, Avishay Lahad, Batia Weiss, Dror S Shouval
OBJECTIVES: Interleukin-10 (IL-10) is an immunoregulatory cytokine that has a central role in suppressing pro-inflammatory responses. Patients with deleterious mutations in IL-10 or IL-10 receptor (IL-10R) genes develop severe colitis and perianal disease in the first months of life. Whether IL-10R expression and signaling in pediatric- or adult-onset Crohn's disease (CD) are altered is unknown. The objective of this study was to characterize IL-10R expression and IL-10R-mediated suppression in patients with CD...
October 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29045351/awareness-and-implementation-of-the-2014%C3%A2-espghan-naspghan-guideline-for-childhood-functional-constipation
#3
Ilan J N Koppen, M H Vriesman, M M Tabbers, C Di Lorenzo, M A Benninga
OBJECTIVE: To assess if physicians approach children with functional constipation according to the 2014 ESPGHAN/NASPGHAN guideline. METHODS: We invited pediatricians and pediatric gastroenterologists in the Netherlands and the U.S. to participate in this anonymous survey using a self-developed questionnaire containing 19 multiple-choice questions concerning evaluation and treatment of children with constipation. RESULTS: We included 328 physicians (67% from the U...
October 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29045276/impact-of-required-versus-self-directed-use-of-virtual-patient-cases-on-clerkship-performance-a-mixed-methods-study
#4
Sarang Kim, Laura R Willett, Wilbur J Pan, Joyce Afran, John A Walker, Judy A Shea
PURPOSE: To explore how students use and benefit from virtual patient cases (VPCs). METHOD: In academic years 2013-2014 and 2014-2015, cohorts of students in pediatrics (Peds), family medicine (FM), and internal medicine (IM) clerkships were allocated to either core required use (CRU) or self-directed use (SU) of MedU VPCs. Outcomes included number and time of case review, student perception of learning from VPCs, National Board of Medical Examiners (NBME) subject examination scores, and summative clinical ratings for medical knowledge and differential diagnoses/problem solving...
October 17, 2017: Academic Medicine: Journal of the Association of American Medical Colleges
https://www.readbyqxmd.com/read/29045268/early-nutrition-intervention-attenuates-weight-gain-for-pediatric-acute-lymphoblastic-leukemia-patients-in-maintenance-therapy
#5
Rachel Hill, Tyler Hamby, Lisa Bashore, Stefanie Rapisand, Kari Galipp, Kenneth Heym, W Paul Bowman
Obesity following treatment of pediatric acute lymphoblastic leukemia (ALL) has become a significant long-term concern. Excessive weight gain often occurs during treatment, particularly during induction and the first 6 months of maintenance therapy, and it may be potentially modifiable. This retrospective study aimed to evaluate the impact of an early, 3-visit nutrition intervention on weight gain during maintenance therapy in ALL patients. Medical records of the intervention group were compared with historical controls who were treated on the same ALL treatment protocols during an earlier time period...
October 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29045180/quantitative-liver-mri-biopsy-correlation-in-pediatric-and-young-adult-patients-with-nonalcoholic-fatty-liver-disease-can-one-be-used-to-predict-the-other
#6
Jonathan R Dillman, Andrew T Trout, Emma N Costello, Suraj D Serai, Kristin S Bramlage, Rohit Kohli, Stavra A Xanthakos
OBJECTIVE: The purpose of this study is to determine the relationships between quantitative liver MRI measurements and liver biopsy findings in pediatric and young adult patients with nonalcoholic fatty liver disease (NAFLD). MATERIALS AND METHODS: Data were obtained from a registry that prospectively enrolls pediatric and young adult patients with biopsy-confirmed NAFLD at our tertiary medical center with parent or guardian and subject informed consent, as appropriate...
October 18, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/29044597/short-and-long-term-complications-of-in-utero-exposure-to-lamotrigine
#7
Merav Cohen-Israel, Itai Berger, Einat Y Martonovich, Gil Klinger, Bracha Stahl, Nehama Linder
AIM: The present study evaluates the effect of antenatal lamotrigine exposure, on short and long term pediatric outcome. METHODS: The study included the children of 83 epileptic women treated with lamotrigine during pregnancy, at a tertiary medical center between 2004-2014. All newborns were monitored for vital signs, congenital malformations and Finnegan score. In addition, the parents completed a questionnaire regarding child's development and health up to the age of 12 years...
October 18, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29044088/management-of-idiopathic-intracranial-hypertension-in-an-infant-with-bilateral-congenital-cataract-and-associated-comitant-sensory-esotropia
#8
Jyoti Himanshu Matalia, Sheetal Shirke, Hemant Anaspure, Pooja Ghalla, Minal Kekatpure
In this report, we describe the management of a child with bilateral cataract, nystagmus, and comitant sensory esotropia. Routine ultrasonography done before cataract surgery revealed bilateral disc edema confirmed as idiopathic intracranial hypertension by a pediatric neurologist. The primary intervention for cataract surgery was followed by nonresolving papilledema, despite maximum medical therapy. To salvage the optic nerve function in a nonverbal child, bilateral optic nerve sheath decompression was planned with simultaneous medial rectus recessions for the persistent esotropia with the satisfactory postoperative outcome...
October 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29043693/-post-appendectomy-intra-abdominal-abscesses-six-years-experience-in-a-pediatric-surgery-department
#9
A Coelho, C Sousa, A S Marinho, J Barbosa-Sequeira, M Recaman, F Carvalho
OBJECTIVES: Post-appendectomy intra-abdominal abscesses are estimated to complicate up to 4.2% of acute non-perforated appendicitis and between 6.7% and 28% acute perforated appendicitis. This study was performed with the aim to review and characterize post-appendectomy intra-abdominal abscesses in our Pediatric Surgery department; to calculate their incidence and to evaluate the efficacy of the treatment modality. MATERIAL AND METHODS: A retrospective analysis of all patients, under 18 years and with the diagnosis of intra-abdominal abscess post-appendectomy, between January 2010 and December 2015 was performed, taking in account initial surgical approach, type of acute appendicitis, length of hospital stay and the treatment applied...
July 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/29043231/voriconazole-plus-caspofungin-for-treatment-of-invasive-fungal-infection-in-children-with-acute-leukemia
#10
Kyu Ho Lee, Young Tae Lim, Jeong Ok Hah, Yu Kyung Kim, Chae Hoon Lee, Jae Min Lee
BACKGROUND: Invasive fungal infections (IFIs) are a life-threatening problem in immunocompromised patients. Despite timely diagnosis and appropriate antifungal therapy, clinical outcomes of IFIs remain unsatisfactory, necessitating treatment with a combination of antifungal agents. Therefore, childhood leukemic patients treated with voriconazole plus caspofungin were evaluated for the safety and efficacy of the combination antifungal therapy to treat IFIs. METHODS: In this retrospective study, medical records were retrieved for patients admitted to the Pediatric Department of Yeungnam University Hospital, Daegu, South Korea, between April 2009 and May 2013...
September 2017: Blood Research
https://www.readbyqxmd.com/read/29042840/warfarin-rifampin-drug-interaction-in-a-pediatric-patient
#11
Michael Poon, Brady S Moffett, Donald L Yee
Rifampin has been documented to significantly attenuate the effect of warfarin in adult patients. No data have been presented on the use of rifampin and warfarin in a pediatric patient. We report an extreme case of increased warfarin metabolism in a pediatric patient who was concomitantly receiving rifampin, despite receiving other medications that significantly decrease warfarin metabolism. The inhibitory effect of rifampin on warfarin therapy may be amplified in pediatric patients.
September 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29042838/dose-banding-of-intravenous-piperacillin-tazobactam-in-pediatric-surgical-inpatients
#12
Indrajit S Karande, Zoy Goff, Jacqueline Kewley, Shailender Mehta, Thomas Snelling
BACKGROUND: Antimicrobial doses in children are often prescribed by using an individually calculated dose per weight (e.g., mg/kg) or based on body surface area. Dosing errors are the most commonly reported medication errors in children. A "dose-banding" strategy is frequently used for some over-the-counter drugs to prevent dosing errors. It could also lead to efficiencies by enabling batch preparation of intravenous (IV) medications in hospitals. OBJECTIVES: To evaluate whether use of dose-banding for IV piperacillin-tazobactam results in acceptable dose variation from standard practice of individualized prescription of 100 mg/kg in children...
September 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29042835/double-blind-randomized-placebo-controlled-trial-of-single-dose-intravenous-acetaminophen-for-pain-associated-with-adenotonsillectomy-in-pediatric-patients-with-sleep-disordered-breathing
#13
Arlyne K Thung, Charles A Elmaraghy, N'Diris Barry, Dmitry Tumin, Kris R Jatana, Julie Rice, Vidya Raman, Tarun Bhalla, David P Martin, Marco Corridore, Joseph D Tobias
OBJECTIVES: Adequate pain control is an important component in the postoperative outcome for pediatric adenotonsillectomy patients with sleep-disordered breathing (SDB). Intravenous acetaminophen appears to be a favorable analgesic adjunct owing to its predictable pharmacokinetics and opioid-sparing effects; however, its role in pediatric adenotonsillectomy pain management remains unclear. METHODS: In this prospective, randomized, double-blinded, controlled study, subjects with the diagnosis of SDB, aged 2 to 8 years, who required extended postoperative admission, received intravenous acetaminophen (15 mg/kg) or saline placebo intraoperatively in addition to morphine (0...
September 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29042833/impact-of-a-pharmacist-led-chemotherapy-education-program-on-the-knowledge-of-pediatric-hematology-oncology-nurses
#14
Christine M Fisher, Abby J Kim, Joshua J Elder
OBJECTIVES: Medication errors involving chemotherapy are a serious source of avoidable medical harm that can result in chemotherapy-related adverse drug events. Efforts to reduce errors in the past decade have largely focused on chemotherapy errors at the prescriber level, using computerized or automated technology, but little has been done to ensure chemotherapy is administered accurately at the nursing level. The current study implemented a pharmacist-led, supplemental, institution-specific training program to nursing staff regarding the use of chemotherapy and to address knowledge deficits in newly hired nursing personnel...
September 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29042752/spotlight-on-perampanel-in-the-management-of-seizures-design-development-and-an-update-on-place-in-therapy
#15
REVIEW
Michele A Faulkner
PURPOSE: Perampanel is a first-in-class antiepileptic medication approved for the treatment of partial (focal) seizures, and as adjunctive treatment for primarily generalized tonic-clonic seizures. The pharmacology, efficacy data, adverse-effect profile, pharmacokinetics and place in therapy are reviewed. SUMMARY: Perampanel is indicated for use in patients with epilepsy who are 12 years of age or older. It is the first medication designed specifically to be a non-competitive antagonist at post-synaptic α-amino-3-hydroxyl-5-methyl-4-isoxazole-propionate receptors...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/29042521/-transition-of-children-with-epilepsy-to-adult-care-current-status-and-challenge
#16
Hidemoto Kubota
Thirty percent of the patients consulting a pediatric neurologist have already reached adulthood as patients and their families do not like transfer to the adult clinics; moreover, there are no suitable clinics. Pediatric neurologists find it difficult to examine adult patients with childhood-onset epilepsy as they are unfamiliar with the psychiatric and psychological symptoms manifested in adulthood, and the common diseases of adulthood. On the other hand, adult neurologists have difficulty obtaining a complete picture of the patient's clinical history since childhood, and are unfamiliar with childhood-specific epilepsy syndrome...
October 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29042343/systems-based-training-in-graduate-medical-education-for-service-learning-in-the-state-legislature-in-the-united-states-pilot-study
#17
Shikhar H Shah, Maureen D Clark, Kimberly Hu, Jalene A Shoener, Joshua Fogel, William C Kling, James Ronayne
BACKGROUND: There is a dearth of advocacy training in graduate medical education in the United States. To address this void, the Legislative Education and Advocacy Development (LEAD) course was developed as an interprofessional experience, partnering a cohort of pediatrics residents, fourth-year medical students, and public health students to be trained in evidence-informed health policy making. OBJECTIVE: The objective of our study was to evaluate the usefulness and acceptability of a service-based legislative advocacy course...
October 17, 2017: JMIR Medical Education
https://www.readbyqxmd.com/read/29041934/h-syndrome-5-new-cases-from-the-united-states-with-novel-features-and-responses-to-therapy
#18
Jessica L Bloom, Clara Lin, Lisa Imundo, Stephen Guthery, Shelly Stepenaskie, Csaba Galambos, Amy Lowichik, John F Bohnsack
BACKGROUND: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations in SLC29A3, a gene located on chromosome 10q23, which encodes the human equilibrative transporter 3 (hENT3). Less than 100 patients with H syndrome have been described in the literature, with the majority being of Arab descent, and only a few from North America. CASE PRESENTATION: Here we report five pediatric patients from three medical centers in the United States who were identified to have H syndrome by whole exome sequencing...
October 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29040512/fertility-related-knowledge-and-reproductive-goals-in-childhood-cancer-survivors-short-communication
#19
V Lehmann, M C Keim, L Nahata, E L Shultz, J L Klosky, M A Tuinman, C A Gerhardt
STUDY QUESTION: Do young adult survivors of childhood cancer know their fertility status, in the context of their parenthood goals and screening for gonadal functioning? SUMMARY ANSWER: While 80% of survivors (who were without children) wanted children in the future, most did not know their fertility status, and screening for gonadal functioning was underutilized. WHAT IS KNOWN ALREADY: Survivors of childhood cancer are at risk for infertility, but fertility counseling and assessment are underutilized...
September 29, 2017: Human Reproduction
https://www.readbyqxmd.com/read/29040242/epidemiology-and-predictors-of-orbital-fractures-in-children
#20
Andrew F Miller, Daniel M Elman, Paul L Aronson, Amir A Kimia, Mark I Neuman
OBJECTIVES: Data regarding clinical predictors of orbital fractures in children are limited. We sought to describe the epidemiology of pediatric orbital fractures and identify the signs and symptoms of orbital fractures in children. METHODS: We performed a retrospective cohort study of children younger than 22 years who underwent a facial or orbital computed tomography (CT) scan to evaluate for orbital fracture. We included patients presenting to an emergency department of a tertiary care children's hospital between January 2009 and May 2013...
October 16, 2017: Pediatric Emergency Care
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