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https://www.readbyqxmd.com/read/28339008/detection-of-lower-levels-of-snap25-using-multiple-microarray-systems-and-its-functional-significance-in-medulloblastoma
#1
Chi-Jung Huang, Chia-Long Lee, Chih-Yi Liu, Shih-Hung Huang, Jia-Woei Hou, Yi-Hou Chen, Chih-Cheng Chien, Chih-Ming Ho, Wen-Cheng Lo, Kun-Long Hung
Medulloblastoma (MB) is the most common pediatric malignant brain tumor and patients with high-risk or recurrent MB respond poorly to current therapies, and have a higher related mortality. For this reason, potential molecules related to MB need be identified in order to develop targets for the development of novel therapeutics. In the present study, we compared MB microarray data obtained using different microarray systems and significant targets were selected by gene annotation and enrichment analysis. Genes for soluble N-ethylmaleimide-sensitive factor attachment protein receptors (SNAREs) annotated with the function 'vesicle' were identified and one of these proteins, synaptosomal-associated protein 25 (SNAP25), was found to have significantly lower expression levels in MB...
March 20, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28337084/therapeutic-drug-monitoring-of-continuous-infusion-doripenem-in-a-pediatric-patient-on-continuous-renal-replacement-therapy
#2
Jeffrey J Cies, Wayne S Moore, Susan B Conley, Paul Shea, Adela Enache, Arun Chopra
An 11-year-old African American male with severe combined immunodeficiency variant, non-cystic fibrosis bronchiectasis, pancreatic insufficiency, chronic mycobacterium avium-intracellulare infection, chronic sinusitis, and malnutrition presented with a 1-week history of fevers. He subsequently developed respiratory decompensation and cefepime was discontinued and doripenem was initiated. Doripenem was the carbapenem used due to a national shortage of meropenem. By day 7 the patient (24.7 kg) had a positive fluid balance of 6925 mL (28% FO), and on days 7 into 8 developed acute kidney injury evidenced by an elevated serum creatinine of 0...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28337077/antithrombin-iii-doses-rounded-to-available-vial-sizes-in-critically-ill-pediatric-patients
#3
Winifred M Stockton, Eimeira Padilla-Tolentino, Carolyn E Ragsdale
OBJECTIVES: Children have decreased levels of antithrombin III (AT III) compared to adults. These levels may be further decreased during acute illness. Administration of exogenous AT III can increase anticoagulant efficacy. The objective of this study was to evaluate AT III doses rounded to available vial sizes compared to partial vial doses in critically ill pediatric patients, including patients receiving extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT)...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28333115/modeling-and-targeting-myc-genes-in-childhood-brain-tumors
#4
REVIEW
Sonja Hutter, Sara Bolin, Holger Weishaupt, Fredrik J Swartling
Brain tumors are the second most common group of childhood cancers, accounting for about 20%-25% of all pediatric tumors. Deregulated expression of the MYC family of transcription factors, particularly c-MYC and MYCN genes, has been found in many of these neoplasms, and their expression levels are often correlated with poor prognosis. Elevated c-MYC/MYCN initiates and drives tumorigenesis in many in vivo model systems of pediatric brain tumors. Therefore, inhibition of their oncogenic function is an attractive therapeutic target...
March 23, 2017: Genes
https://www.readbyqxmd.com/read/28331084/structural-insights-into-emerging-pediatric-pathogens-human-bocaparvoviruses
#5
Mario Mietzsch, Shweta Kailasan, Jamie Garrison, Maria Ilyas, Paul Chipman, Kalle Kantola, Mandy E Janssen, John Spear, Duncan Sousa, Robert McKenna, Kevin Brown, Maria Söderlund-Venermo, Timothy Baker, Mavis Agbandje-McKenna
Bocaparvoviruses are emerging pathogens of the Parvoviridae family. Human bocaviruses 1 (HBoV1) causes severe respiratory infections and HBoV2-4 gastrointestinal infections in young children. Recent reports of life threatening cases, lack of direct treatment or vaccination, and a limited understanding of their disease mechanisms demand the need to study these pathogens on a molecular and structural level for the development of therapeutics. Towards this end, the capsid structures of HBoV1, HBoV3, and HBoV4 were determined to 2...
March 22, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28326084/hiv-infection-and-compromised-mucosal-immunity-oral-manifestations-and-systemic-inflammation
#6
REVIEW
Samantha E Heron, Shokrollah Elahi
Mucosal surfaces account for the vast majority of HIV transmission. In adults, HIV transmission occurs mainly by vaginal and rectal routes but rarely via oral route. By contrast, pediatric HIV infections could be as the result of oral route by breastfeeding. As such mucosal surfaces play a crucial role in HIV acquisition, and spread of the virus depends on its ability to cross a mucosal barrier. HIV selectively infects, depletes, and/or dysregulates multiple arms of the human immune system particularly at the mucosal sites and causes substantial irreversible damage to the mucosal barriers...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28325298/relevance-of-fusion-genes-in-pediatric-cancers-toward-precision-medicine
#7
REVIEW
Célia Dupain, Anne Catherine Harttrampf, Giorgia Urbinati, Birgit Geoerger, Liliane Massaad-Massade
Pediatric cancers differ from adult tumors, especially by their very low mutational rate. Therefore, their etiology could be explained in part by other oncogenic mechanisms such as chromosomal rearrangements, supporting the possible implication of fusion genes in the development of pediatric cancers. Fusion genes result from chromosomal rearrangements leading to the juxtaposition of two genes. Consequently, an abnormal activation of one or both genes is observed. The detection of fusion genes has generated great interest in basic cancer research and in the clinical setting, since these genes can lead to better comprehension of the biological mechanisms of tumorigenesis and they can also be used as therapeutic targets and diagnostic or prognostic biomarkers...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28323979/thyroid-cancer-following-childhood-low-dose-radiation-exposure-a-pooled-analysis-of-nine-cohorts
#8
Jay H Lubin, M Jacob Adams, Roy Shore, Erik Holmberg, Arthur B Schneider, Michael M Hawkins, Leslie L Robison, Peter D Inskip, Marie Lundell, Robert Johansson, Ruth A Kleinerman, Florent de Vathaire, Lena Damber, Siegal Sadetzki, Margaret Tucker, Ritsu Sakata, Lene H S Veiga
Context: The increased use of diagnostic and therapeutic procedures that involve radiation raises concerns about radiation effects, particularly in children and to the radio-sensitive thyroid gland. Objectives: Evaluation of relative risk (RR) trends for thyroid radiation doses <0.2 gray; evidence of a threshold dose; and possible modifiers of the dose-response, e.g., sex, age at exposure, time since exposure. Design and setting: Pooled data from nine cohort studies of childhood external radiation exposure and thyroid cancer with individualized dose estimates, ≥1,000 irradiated subjects or ≥10 thyroid cancer cases, with data limited to individuals receiving doses <0...
March 8, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28323911/monogenic-diabetes-accounts-for-6-3-of-cases-referred-to-15-italian-pediatric-diabetes-centers-during-2007-2012
#9
Maurizio Delvecchio, Enza Mozzillo, Giuseppina Salzano, Dario Iafusco, Giulio Frontino, Patrizia I Patera, Ivana Rabbone, Valentino Cherubini, Valeria Grasso, Nadia Tinto, Sabrina Giglio, Giovanna Contreas, Rosa Di Paola, Alessandro Salina, Vittoria Cauvin, Stefano Tumini, Giuseppe d'Annunzio, Lorenzo Iughetti, Vilma Mantovani, Giulio Maltoni, Sonia Toni, Marco Marigliano, Fabrizio Barbetti
Context: Etiologic diagnosis of diabetes may impact on therapeutic strategy, and prognosis of chronic complications. Objective: The aim of the study was to establish the relative percentage of different diabetes subtypes in patients attending Italian pediatric diabetes centers, and the influence of etiologic diagnosis on therapy. Design, Setting and Patients: This was a retrospective study. Clinical records of 3781 consecutive patients (age: 0-18 years) referred to fifteen pediatric diabetes clinics and diagnosed with diabetes or IFG between Jan/1/2007 and Dec/31/2012 were examined...
February 16, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321350/shigellosis-caused-by-ctx-m-type-esbl-producing-shigella-flexneri-in-two-siblings-of-rural-nepal-first-case-report-from-the-country
#10
Narayan Prasad Parajuli, Govardhan Joshi, Bashu Dev Pardhe, Jyotsna Shakya, Anjeela Bhetwal, Shreena Shakya, Roshan Pandit, Sumesh Shreekhanda Shrestha, Puspa Raj Khanal
Shigellosis is an acute infectious disease characterized as severe bloody diarrhea (dysentery) and is accountable for a significant burden of morbidity and mortality especially in children under the age of 5 years. Antimicrobial therapy is required in the cases of severe dysentery associated with Shigella. However, emergence of multidrug resistant (MDR) strains of Shigella spp. over the last two decades has restricted the use of common therapeutic antimicrobials. In MDR strains, the third-generation cephalosporins have been used for the treatment, but, unfortunately, emerging reports of enzyme mediated β-lactam resistance among Shigella isolates from various parts of the world have greatly compromised the therapy of pediatric dysentery...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/28320870/clinical-considerations-related-to-the-behavioral-manifestations-of-child-maltreatment
#11
Robert D Sege, Lisa Amaya-Jackson
Children who have suffered early abuse or neglect may later present with significant health and behavior problems that may persist long after the abusive or neglectful environment has been remediated. Neurobiological research suggests that early maltreatment may result in an altered psychological and physiologic response to stressful stimuli, a response that deleteriously affects the child's subsequent development. Pediatricians can assist caregivers by helping them recognize the abused or neglected child's emotional and behavioral responses associated with child maltreatment and guide them in the use of positive parenting strategies, referring the children and families to evidence-based therapeutic treatment and mobilizing available community resources...
March 20, 2017: Pediatrics
https://www.readbyqxmd.com/read/28320517/influence-of-age-and-co-medication-on-the-steady-state-pharmacokinetics-of-valproic-acid-in-tunisian-patients-with-epilepsy
#12
L Ben Mahmoud, A Hakim, H Ghozzi, R Atheymen, Z Sahnoun, K Zeghal
AIM: Valproic acid (VPA) is a widely prescribed broad-spectrum antiepileptic drug. However, the use of VPA is complicated in clinical practice by its remarkably wide variability of pharmacokinetics. The objective of this study was to investigate the effects of demographic factors and associated therapies on steady-state plasma VPA concentrations in patients with epilepsy. METHODS: This retrospective cohort study was carried out using the routine therapeutic drug monitoring (TDM) database...
March 17, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28319601/effectiveness-of-double-balloon-enteroscopy-facilitated-polypectomy-in-paediatric-patients-with-peutz-jeghers-syndrome
#13
Dalia Belsha, Arun Urs, Thomas Attard, Mike Thomson
BACKGROUND: Sizable small-bowel (SB) polyps in Peutz-Jeghers syndrome (PJS) pose a high risk for intussusception, often necessitating laparotomy and intraoperative-enteroscopy (IOE). This series examines the effectiveness of double-balloon enteroscopy (DBE) facilitated polypectomy for pediatric patients with PJS. METHODS: Prospective analysis of collected data (6 years) on all patients with PJS referred for DBE-facilitated SB polypectomy at a pediatric tertiary-referral center...
March 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28319075/autologous-hematopoietic-stem-cell-transplantation-for-pediatric-multiple-sclerosis-a-registry-based-study-of-the-autoimmune-diseases-working-party-adwp-and-pediatric-diseases-working-party-pdwp-of-the-european-society-for-blood-and-marrow-transplantation-ebmt
#14
J Burman, K Kirgizov, K Carlson, M Badoglio, G L Mancardi, G De Luca, B Casanova, J Ouyang, R Bembeeva, J Haas, P Bader, J Snowden, D Farge
Autologous hematopoietic stem cell transplantation (aHSCT) is a promising therapy for multiple sclerosis (MS), which has mainly been used in adults. The purpose of this study was to investigate efficacy and adverse events of aHSCT in the treatment of children with MS using data from the European Society for Blood and Marrow Transplantation registry. Twenty-one patients with a median follow-up time of 2.8 years could be identified. PFS at 3 years was 100%, 16 patients improved in expanded disability status scale score and only 2 patients experienced a clinical relapse...
March 20, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28319067/the-histone-demethylase-kdm3a-and-its-downstream-target-mcam-promote-ewing-sarcoma-cell-migration-and-metastasis
#15
M Sechler, J K Parrish, D K Birks, P Jedlicka
Ewing Sarcoma is the second most common solid pediatric malignant neoplasm of bone and soft tissue. Driven by EWS/Ets, or rarely variant, oncogenic fusions, Ewing Sarcoma is a biologically and clinically aggressive disease with a high propensity for metastasis. However, the mechanisms underpinning Ewing Sarcoma metastasis are currently not well understood. In the present study, we identify and characterize a novel metastasis-promotional pathway in Ewing Sarcoma, involving the histone demethylase KDM3A, previously identified by our laboratory as a new cancer-promoting gene in this disease...
March 20, 2017: Oncogene
https://www.readbyqxmd.com/read/28318188/-zosteriform-lichen-aureus-pediatric-clinical-case
#16
Álvaro Rivera-Rodríguez, Sergio Hernández Ostiz, Ana L Morales-Moya, Lucía Prieto-Torres, Marcial Álvarez-Salafranca, Mariano Ara Martín
Lichen aureus is a rare pigmented purpuric dermatosis. We present an unusual case because of the pediatric age and the great number of lesions with zosteriform distribution. He is a 10-yearold boy, with a brownish, smaller than 1 cm, sharp edges, lichenified surface, asymptomatic macule, over the inner aspect of the left leg with a zosteriform distribution. The histology showed a band-like inflammatory infiltrate in the superficial dermis, composed of lymphocytes, histiocytes, erythrocytes and haemosiderin...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28318185/neonatal-acute-liver-failure-a-diagnosis-challenge
#17
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28318054/efficacy-and-safety-of-targeted-high-intensity-medium-band-304-312-nm-ultraviolet-b-light-in-pediatric-vitiligo
#18
Da-Ke Dong, Zhan-Yan Pan, Juan Zhang, Xue-Fen Lu, Cheng Jin, Shi-Qin Tao, Li-Jia Yang
BACKGROUND/OBJECTIVES: Phototherapy is a commonly used treatment for vitiligo that has demonstrated safety and efficacy. High-intensity targeted ultraviolet B (UVB) light (304-312 nm) delivered using a phototherapy device is a useful therapeutic option because it can induce repigmentation in a short time without global exposure to radiation, but information regarding this device in children is limited. METHODS: We performed a retrospective analysis of 95 patches of vitiligo in 27 children treated using a targeted phototherapy device...
March 20, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28317178/successful-third-renal-transplantation-in-a-child-with-an-occluded-inferior-vena-cava-a-novel-technique-to-use-the-venous-interposition-between-the-transplant-renal-vein-and-the-infrahepatic-inferior-vena-cava
#19
Masaki Muramatsu, Seiichiro Shishido, Yusuke Takahashi, Yuko Hamasaki, Hiroshi Yoshimura, Hiroshi Nihei, Yoshihiro Itabashi, Takeshi Kawamura, Atsushi Aikawa
A girl aged 11 years and 3 months with occlusion of the inferior vena cava had experienced two renal transplant graft failures since birth. The third renal transplant from a live donor was carried out. Preoperative evaluation showed that the arteries from the right common to the right external iliac artery were absent, and the ilio-caval vein was occluded below the level of the renal vein. The donor's renal artery was anastomosed to the aorta. The donor's ovarian and large saphenous veins were used to extend the transplant renal vein to the recipient's patent inferior vena cava...
March 19, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#20
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
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