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Pediatric Syndromes

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https://www.readbyqxmd.com/read/29040243/toxic-shock-syndrome-still-a-timely-diagnosis
#1
Kira L Gossack-Keenan, April J Kam
BACKGROUND: Toxic shock syndrome (TSS) is an acute, severe, toxin-mediated disease, characterized by fever, hypotension, and multiorgan system involvement. Toxic shock syndrome has made headlines because of its high associated morbidity and mortality rate in previously healthy young females. Incidence peaked in the early 1980s owing to increased usage of ultra-absorbent tampons. After improved patient education and tampon labeling, the incidence of menstrual TSS has declined. CASE: A previously healthy 14-year-old girl presented to an urgent care center with a 2-day history of fever, erythematous maculopapular rash, vomiting, diarrhea, and malaise...
October 16, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29040012/outcome-of-acute-lymphoblastic-leukemia-in-children-with-down-syndrome-polish-pediatric-leukemia-and-lymphoma-study-group-report
#2
Joanna Zawitkowska, Teresa Odój, Katarzyna Drabko, Agnieszka Zaucha-Prażmo, Julia Rudnicka, Michał Romiszewski, Michał Matysiak, Kinga Kwiecińska, Magdalena Ćwiklińska, Walentyna Balwierz, Joanna Owoc-Lempach, Katarzyna Derwich, Jacek Wachowiak, Maciej Niedźwiecki, Elżbieta Adamkiewicz-Drożyńska, Joanna Trelińska, Wojciech Młynarski, Andrzej Kołtan, Mariusz Wysocki, Renata Tomaszewska, Tomasz Szczepański, Marcin Płonowski, Maryna Krawczuk-Rybak, Tomasz Ociepa, Tomasz Urasiński, Agnieszka Mizia-Malarz, Grażyna Sobol-Milejska, Grażyna Karolczyk, Jerzy Kowalczyk
Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group-14 patients, an intermediate-risk group-24, a high-risk group-3...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29034507/screening-for-sturge-weber-syndrome-a-state-of-the-art-review
#3
REVIEW
Michaela Zallmann, Richard J Leventer, Mark T Mackay, Michael Ditchfield, Philip S Bekhor, John C Su
Infants with a high-risk distribution of port-wine stains are commonly screened for Sturge-Weber syndrome using brain magnetic resonance imaging. There is no consensus about which port-wine stain phenotypes to screen, optimal timing, screening sensitivity, or whether presymptomatic diagnosis improves neurodevelopmental outcomes. This state-of-the-art review examines the evidence in favor of screening for Sturge-Weber syndrome, based on its effect on neurodevelopmental outcomes, against the risks and limitations of screening magnetic resonance imaging and electroencephalography...
October 16, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#4
Anne K Dettmar, Thorsten Wiech, Markus J Kemper, Armin Soave, Michael Rink, Jun Oh, Rolf A K Stahl, Elion Hoxha
BACKGROUND: Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but is less frequent in children. Antibodies against four antigens leading to MN have been described in children: phospholipase A2 receptor 1 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), neutral endopeptidase (NEP), and cationic bovine serum albumin (BSA). METHODS: Twelve children with MN were included in this study. Sera of all patients were analyzed for antibodies against PLA2R1, THSD7A, NEP, and BSA...
October 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29034009/a-multicenter-randomized-study-of-decitabine-as-epigenetic-priming-with-induction-chemotherapy-in-children-with-aml
#5
Lia Gore, Timothy J Triche, Jason E Farrar, Daniel Wai, Christophe Legendre, Gerald C Gooden, Winnie S Liang, John Carpten, David Lee, Frank Alvaro, Margaret E Macy, Carola Arndt, Philip Barnette, Todd Cooper, Laura Martin, Aru Narendran, Jessica Pollard, Soheil Meshinchi, Jessica Boklan, Robert J Arceci, Bodour Salhia
BACKGROUND: Decitabine is a deoxycytidine nucleoside derivative inhibitor of DNA-methyltransferases, which has been studied extensively and is approved for myelodysplastic syndrome in adults but with less focus in children. Accordingly, we conducted a phase 1 multicenter, randomized, open-label study to evaluate decitabine pre-treatment before standard induction therapy in children with newly diagnosed AML to assess safety and tolerability and explore a number of biologic endpoints. RESULTS: Twenty-four patients were fully assessable for all study objectives per protocol (10 in Arm A = epigenetic priming induction, 14 in Arm B = standard induction)...
2017: Clinical Epigenetics
https://www.readbyqxmd.com/read/29032616/a-survey-on-hematology-oncology-pediatric-aieop-centres-the-challenge-of-posterior-reversible-encephalopathy-syndrome
#6
Daniele Zama, Pietro Gasperini, Massimo Berger, Mariagrazia Petris, Maria D De Pasquale, Simone Cesaro, Maria E Guerzoni, Elena Mastrodicasa, Francesca Savina, Ottavio Ziino, Valentina Kiren, Paola Muggeo, Rosa M Mura, Fraia Melchionda, Giulio A Zanazzo
Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurological complications in hematology-oncology pediatric patients. Despite an increasingly recognized occurrence, no clear consensus exists regarding how best to manage the syndrome, because most cases of PRES have reported in single case reports or small series. Aim of this paper is to identify incidence, clinical features, management and outcome of PRES in a large series of hematology-oncology pediatric patients METHODS: The cases of PRES occurred in twelve centres of the Italian Association of Pediatric Haematology and Oncology were reported RESULTS: 124 cases of PRES in 112 pediatric patients were recorded with an incidence of 2...
October 15, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29031489/-pediatric%C3%A2-nephrology-what%C3%A2-should-an%C3%A2-adult%C3%A2-nephrologist-know-about%C3%A2-these%C3%A2-diseases
#7
Sandrine Lemoine, Pierre Cochat, Aurélia Bertholet-Thomas, Charlène Levi, Catherine Bonnefoy, Anne-Laure Sellier-Leclerc, Justine Bacchetta
In nephrology, some diseases begin specifically during childhood; however, they must be known by adult nephrologists so that to ensure continuity and homogeneity for their management. The aim of this review is therefore to propose a brief overview of the main pediatric diseases, for which a specific knowledge is warranted, and notably pediatric idiopathic nephrotic syndrome, cystinosis, primary hyperoxaluria and hereditaries tubulopathies.
October 11, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29030698/biliary-atresia-and-liver-transplantation-results-and-thoughts-for-primary-liver-transplantation-in-select-patients
#8
REVIEW
Riccardo Superina
Biliary atresia (BA) is one of the most common indications for liver transplantation in children. Despite advances in biliary atresia surgical techniques, most children will ultimately require liver transplantation. Possible pre-operative predictors of outcome after the Kasai operation are: 1. Age at operation 2. Presence of the biliary atresia splenic malformation syndrome (BASM) 3. Center specific factors 4. Liver histology and 5. Anatomic pattern of bile ducts found at surgery.Age at surgery is considered a strong predictor of success after portoenterostomy...
October 13, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29028962/clinical-features-of-acute-flaccid-myelitis-temporally-associated-with-an-enterovirus-d68-outbreak-results-of-a-nationwide-survey-of-acute-flaccid-paralysis-in-japan-august-december-2015
#9
Pin Fee Chong, Ryutaro Kira, Harushi Mori, Akihisa Okumura, Hiroyuki Torisu, Sawa Yasumoto, Hiroyuki Shimizu, Tsuguto Fujimoto, Nozomu Hanaoka, Susumu Kusunoki, Toshiyuki Takahashi, Kazunori Oishi, Keiko Tanaka-Taya
Backgroud: Acute flaccid myelitis (AFM) is an acute flaccid paralysis (AFP) syndrome with spinal motor neuron involvement of unknown etiology. We investigated the characteristics and prognostic factors of AFM clusters coincident with an enterovirus D68 (EV-D68) outbreak in Japan during autumn 2015. Methods: An AFM case series study was conducted following a nationwide survey from August to December 2015. Radiographic and neurophysiologic data were subjected to centralized review, and virology studies were conducted for available specimens...
October 6, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29028723/does-driving-pressure-matter-in-pediatric-acute-respiratory-distress-syndrome-strain-to-find-the-answer
#10
Yu Inata, Muneyuki Takeuchi
No abstract text is available yet for this article.
November 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29028699/pediatric-acute-respiratory-distress-syndrome-in-asia-none-of-us-is-as-smart-as-all-of-us
#11
H Michael Ushay
No abstract text is available yet for this article.
November 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29027608/ivabradine-in-postural-orthostatic-tachycardia-syndrome-preliminary-experience-in-children
#12
Grazia Delle Donne, Ferran Rosés Noguer, Jan Till, Tushar Salukhe, Sanjay K Prasad, Piers E F Daubeney
OBJECTIVE: Ivabradine is a selective and specific inhibitor of the I(f) current in the sinoatrial and atrioventricular nodes. It decreases heart rate and myocardial oxygen consumption at rest and during exercise. It is used in adults for management of heart failure and angina, but promising results have been obtained in postural orthostatic tachycardia syndrome (POTS). There is little experience of ivabradine in childhood, although it is used on a compassionate basis. Our aim was to review our experience of ivabradine in a retrospective evaluation of pediatric patients with POTS...
October 13, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/29026471/pertussis-and-pertussis-like-illness-pediatric-experience-in-oman
#13
Amal Al Maani, Abdullah Al Qayoudhi, Hanan Fawzi Nazir, Heba Omar, Amina Al Jardani, Zakariya Al Muharrmi, Yasser Wali
OBJECTIVES: A resurgence of pertussis or whooping cough has been observed worldwide despite broad vaccination coverage. Pertussis like illness (PLI) refers to a clinical syndrome compatible with pertussis infection but lacking laboratory confirmation or an epidemiological link to a confirmed case. Our study aimed to estimate the contribution of Bordetella pertussis infection and identifying predictors of its diagnosis in a cohort of children with PLI. METHODS: Demographic and clinical information were retrospectively collected from the medical records of children < 13 years old and hospitalized for PLI in two pediatric units in Oman from 1 January 2012 to 31 December 2013...
September 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/29023277/diagnosis-and-treatment-of-slipping-rib-syndrome
#14
Cassidy M Foley, Dai Sugimoto, David P Mooney, William P Meehan, Andrea Stracciolini
OBJECTIVE: (1) To investigate the clinical presentation, diagnosis, and treatment of slipping rib syndrome in athletes; (2) to investigate the hooking maneuver for diagnosis of slipping rib syndrome. DESIGN: Retrospective chart review of 362 athletes with rib pain. SETTING: Pediatric-based sports medicine clinic between January 1, 1999, and March 1, 2014. PATIENTS: Costochondritis, Tietze, fractures, rib tip syndrome, and unclear diagnoses were excluded...
October 6, 2017: Clinical Journal of Sport Medicine: Official Journal of the Canadian Academy of Sport Medicine
https://www.readbyqxmd.com/read/29023232/early-onset-obesity-and-risk-of-metabolic-syndrome-among-chilean-adolescents
#15
Lorena Sonia Pacheco, Estela Blanco, Raquel Burrows, Marcela Reyes, Betsy Lozoff, Sheila Gahagan
INTRODUCTION: Obesity and metabolic syndrome (MetS) indicators have increased globally among the pediatric population. MetS indicators in the young elevate their risk of cardiovascular disease and metabolic disorders later in life. This study examined early onset obesity as a risk factor for MetS risk in adolescence. METHODS: A cohort of Chilean participants (N = 673) followed from infancy was assessed at age 5 years and in adolescence (mean age, 16.8 y). Adiposity was measured at both time points; blood pressure and fasting blood samples were assessed in adolescence only...
October 12, 2017: Preventing Chronic Disease
https://www.readbyqxmd.com/read/29022557/two-childhood-pheochromocytoma-cases-due-to-von-hippel-lindau-disease-one-associated-with-pancreatic-neuroendocrine-tumor-a-rare-manifestation
#16
Aydilek Dağdeviren Çakır, Hande Turan, Ayça Aykut, Asude Durmaz, Oya Ercan, Olcay Evliyaoğlu
(VHL) disease is an autosomal dominantly inherited disorder characterized by hemangioblastomas of retina and central nervous system (CNS); renal cysts, clear cell carcinoma; PCC; endolymphatic sac tumors; cystadenomas of the epididymis in males, broad ligament of uterus in females; pancreatic cysts, cystadenomas and neuroendocrine tumors. We here report two cases of VHL disease presented with PCC as the first manifestation. Hemangioblastoma of CNS in the first case and PNET in the second case developed during follow- up and led to the diagnosis of VHL disease...
October 12, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29022095/laparoscopic-portoenterostomy-for-biliary-atresia-single-center-experience-and-review-of-literatures
#17
Joel Cazares, Hiroyuki Koga, Hiroshi Murakami, Hiroki Nakamura, Geoffrey Lane, Atsuyuki Yamataka
AIM: The aim of this report was to present the laparoscopic portoenterostomy (LapPE) procedure developed by the Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine (JLapPE). We also attempted to obtain an understanding of the current status of laparoscopic portoenterostomy in the world as reported in the English literature to compare with our experience. METHODS: There were 22 BA patients who had JLapPE between 2009 and 2016...
October 11, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29020116/leptin-adiponectin-imbalance-as-a-marker-of-metabolic-syndrome-among-chinese-children-and-adolescents-the-bcams-study
#18
Ge Li, Linxin Xu, Yanglu Zhao, Lujiao Li, Junling Fu, Qian Zhang, Naishi Li, Xinhua Xiao, Changhong Li, Jie Mi, Shan Gao, Ming Li
PURPOSE: Leptin and adiponectin have opposite effects on subclinical inflammation and insulin resistance, both involved in the development of metabolic syndrome (MS). We aimed to investigate whether leptin/adiponectin ratio (L/A), as a marker of these two adipokines imbalance, may improve diagnosis of MS in children and adolescents, and determined its cut-off value in the diagnosis of MS. METHODS: A total of 3,428 subjects aged 6-18 years were selected from Beijing Child and Adolescent Metabolic Syndrome study...
2017: PloS One
https://www.readbyqxmd.com/read/29019758/sonographic-evaluation-of-pediatric-thyroid-nodules
#19
Alex C Essenmacher, Peter H Joyce, Simon C Kao, Monica Epelman, Liuska M Pesce, Michael P D'Alessandro, Yutaka Sato, Craig M Johnson, Daniel J Podberesky
A thyroid nodule detected clinically or incidentally at medical imaging is a common indication for ultrasonography (US) in the adult population. This scenario is less frequently the case in pediatric patients, and the approach to evaluation of thyroid nodules deserves modification in these patients because of the increased probability of malignancy in children, compared with adults. Evaluating a thyroid nodule with US in a systematic way requires familiarity with a number of features that can be assessed and the terms that the radiologist uses in each category...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29019756/imaging-of-skeletal-disorders-caused-by-fibroblast-growth-factor-receptor-gene-mutations
#20
Kiran M Sargar, Achint K Singh, Simon C Kao
Fibroblast growth factors and fibroblast growth factor receptors (FGFRs) play important roles in human axial and craniofacial skeletal development. FGFR1, FGFR2, and FGFR3 are crucial for both chondrogenesis and osteogenesis. Mutations in the genes encoding FGFRs, types 1-3, are responsible for various skeletal dysplasias and craniosynostosis syndromes. Many of these disorders are relatively common in the pediatric population, and diagnosis is often challenging. These skeletal disorders can be classified based on which FGFR is affected...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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