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https://www.readbyqxmd.com/read/29166293/applicability-of-the-calcaneal-apophysis-ossification-staging-system-to-the-modern-pediatric-population
#1
Samuel Q Li, Allen D Nicholson, Daniel R Cooperman, Raymond W Liu
BACKGROUND: The calcaneal apophysis ossification staging system is a novel method for assessing skeletal maturity. However, it was created using the same historic patient population that was used to create the Greulich and Pyle atlas of the hand and wrist, predominantly white children. It is unclear if the calcaneal apophysis ossification staging system is still applicable to the modern pediatric population and to children of other races. METHODS: We retrospectively studied 1327 benign lateral foot x-rays from modern white and black children...
November 21, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29164473/clinicopathological-and-immunohistochemical-study-of-head-and-neck-langerhans-cell-histiocytosis-from-latin-america
#2
Natália Rocha Bedran, Román Carlos, Bruno Augusto Benevenuto de Andrade, Ana Paula Silva Bueno, Mário José Romañach, Cristiane Bedran Milito
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplastic proliferation with variable clinical behavior caused by the accumulation of CD1a(+)/CD207(+) histiocytes, associated with a variable number of eosinophils, lymphocytes, plasma cells and multinucleated giant cells, most commonly observed in male children. LCH is uncommon in the head and neck region, occurring as ulcerated and reddened plaques or nodules that cause destruction of adjacent soft tissues and bone. The exact etiology of LCH is still unknown and controversial, with possible etiologic role of viruses, including Epstein-Barr virus (EBV)...
November 21, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/29164082/acute-septic-arthritis-of-the-knee-caused-by-kingella-kingae-in-a-5-year-old-cameroonian-boy
#3
Nawal El Houmami, Dimitri Ceroni, Karine Codjo Seignon, Jean-Christophe Pons, Cédric Lambert, Guillaume André Durand, Philippe Minodier, Léopold Lamah, Philippe Bidet, Jacques Schrenzel, Didier Raoult, Pierre-Edouard Fournier
Kingella kingae is an important cause of invasive infections in young children from Western countries. Although increasing reports indicate that this organism is the leading agent of bone and joint infections in early childhood, data on K. kingae infections from resource-limited settings are scarce, and none has yet been reported in Africa. We herein report the diagnostic and epidemiological investigations of the first case of K. kingae arthritis identified in a child from sub-Saharan Africa. A 5-year-old Cameroonian boy presented with a sudden painful limp which appeared in the course of a mild rhinopharyngitis...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29163809/distinct-signaling-events-promote-resistance-to-mitoxantrone-and-etoposide-in-pediatric-aml-a-children-s-oncology-group-report
#4
Xin Long, Robert B Gerbing, Todd A Alonzo, Michele S Redell
Despite aggressive chemotherapy including mitoxantrone and etoposide, relapse occurs for almost half of children with acute myeloid leukemia (AML). Since both drugs inhibit topoisomerase II and cause DNA double strand breaks, resistance could be achieved by enhanced DNA damage repair (DDR), via homologous recombination (HR) and/or non-homologous end joining (NHEJ). An important source of extrinsic chemoresistance is the bone marrow stroma. We aimed to reveal intrinsic and stroma-induced signaling pathways that contribute to chemoresistance...
October 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/29163677/trim37-promotes-tumor-cell-proliferation-and-drug-resistance-in-pediatric-osteosarcoma
#5
Yanling Tao, Meiyun Xin, Huanchen Cheng, Zongxuan Huang, Tiantian Hu, Teng Zhang, Jianlong Wang
Osteosarcoma (OS) is among the most frequently occurring bone tumors, particularly in children. Clinical treatment of OS is limited due to several factors including resistance to chemotherapy drugs and metastasis, and the underlying molecular mechanisms remain unclear. In the present study, tripartite motif containing 37 (TRIM37) expression levels were upregulated in tumor samples and associated with the development of drug resistance in OS. Furthermore, chemotherapy drug treatment (doxorubicin, cisplatin and methotrexate) induced TRIM37 expression in OS cells in vitro...
December 2017: Oncology Letters
https://www.readbyqxmd.com/read/29159075/enzyme-replacement-therapy-in-perinatal-hypophosphatasia-case-report-of-a-negative-outcome-and-lessons-for-clinical-practice
#6
Gregory Costain, Aideen M Moore, Lauren Munroe, Alison Williams, Randi Zlotnik Shaul, Cheryl Rockman-Greenberg, Martin Offringa, Peter Kannu
Enzyme replacement therapy (ERT) is a newly approved disease-modifying treatment for hypophosphatasia (HPP), a rare metabolic bone disorder. With an orphan drug and ultra-rare disease, sharing information about responders and non-responders is particularly important, as any one centre's familiarity with its use will be limited. Nearly all published data in infants and very young children with life-threatening HPP are from three small clinical trials that have reported generally positive outcomes. We describe in detail a patient with perinatal HPP for whom treatment with ERT was not successful...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29157625/pediatric-type-1-cartilage-tympanoplasty-outcomes-a-comparison-of-short-and-long-term-hearing-results
#7
Isa Kaya, Murat Benzer, Sercan Gode, Furkan Sahin, Cem Bilgen, Tayfun Kirazli
OBJECTIVE: Tympanoplasty is a commonly used procedure in children as in adults. The purposes of this study were to evaluate and report the long term results of type 1 cartilage tympanoplasty in pediatric population. Short term and long term hearing outcomes were compared according to age and perforation location. METHODS: We retrospectively evaluated a total of 76 of 93 patients who had regularly come to visits (38 male and 38 female) with chronic otitis media (COM) and who were younger than 16 years (range, 9-16 years) and underwent a primary type 1 tympanoplasty in tertiary medical center...
November 17, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/29156943/assessment-of-biochemical-bone-turnover-markers-and-bone-mineral-density-in-thin-and-normal-weight-children
#8
Jadwiga Ambroszkiewicz, Joanna Gajewska, Grazyna Rowicka, Witold Klemarczyk, Magdalena Chelchowska
Objective There is scant research examining the prevalence of thinness in early childhood, despite its potential negative consequences for health and development across the life course. The objective of this study was to assess bone status through measurement of bone mineral density and biochemical bone turnover markers, with special attention paid to carboxylated (c-OC) as well as undercarboxylated (uc-OC) forms of osteocalcin, in the groups of thin and normal-weight children. Design The study included 80 healthy prepubertal children (median age 7...
January 1, 2017: Cartilage
https://www.readbyqxmd.com/read/29156780/baicalein-inhibits-progression-of-osteosarcoma-cells-through-inactivation-of-the-wnt-%C3%AE-catenin-signaling-pathway
#9
Guo Dai, Di Zheng, Qianliang Wang, Jian Yang, Gaiwei Liu, Qi Song, Xiangran Sun, Chunjie Tao, Qingzhu Hu, Tian Gao, Ling Yu, Weichun Guo
Osteosarcoma is a very common type of malignant bone tumor in children and young adults and aberrant activation of Wnt/β-catenin signaling pathway has been discovered in osteosarcoma. The traditional Chinese medicine baicalein was proved to have anti-proliferative and anti-metastatic properties in osteosarcoma, but the mechanism remained poorly understood. In the present study, we assessed the effects of baicalein on osteosarcoma and detected the potential molecular mechanism. We found that baicalein significantly suppressed the proliferation of osteosarcoma cells in a concentration- and time-dependent manner...
October 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29156746/tssc3-represses-self-renewal-of-osteosarcoma-stem-cells-and-nanog-expression-by-inhibiting-the-src-akt-pathway
#10
Guang-Ning Yan, Xue-Feng Tang, Xian-Chao Zhang, Ting He, Yu-Sheng Huang, Xi Zhang, Gang Meng, De-Yu Guo, Yang-Fan Lv, Qiao-Nan Guo
Osteosarcoma is the most common type of bone cancer, and the second leading cause of cancer-related death in children and young adults. Osteosarcoma stem cells are essential for osteosarcoma initiation, metastasis, chemoresistance and recurrence. In the present study, we report that: 1) higher TSSC3 expression indicates a better prognosis for osteosarcoma patients, and; 2) overexpression of TSSC3 significantly decreases sphere-forming capacity, tumor initiation, stemness-related surface markers and Nanog expression in osteosarcoma cells...
October 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29155317/treosulfan-fludarabine-conditioning-for-hsct-in-children-with-primary-immunodeficiency-uk-experience
#11
Mary A Slatter, Kanchan Rao, Intan Juliana Abd Hamid, Zohreh Nademi, Robert Chiesa, Reem Elfeky, Mark S Pearce, Persis Amrolia, Austen Worth, Terence Flood, Mario Abinun, Sophie Hambleton, Waseem Qasim, Hubert B Gaspar, Andrew J Cant, Andrew R Gennery, Paul Veys
We previously published results of 70 children who received treosulfan with cyclophosphamide (30) or fludarabine (40) before haematopoietic stem cell transplantation (HSCT) for Primary Immunodeficiency (PID). Toxicity was lower and T cell chimerism better in those receiving fludarabine, but numbers were relatively small and follow-up short. We now report outcome of 160 children who received homogeneous conditioning with treosulfan, fludarabine mostly with alemtuzumab (n=124). Median age at transplant was 1...
November 16, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29155316/influence-of-age-on-acute-and-chronic-gvhd-in-children-receiving-hla-identical-sibling-bmt-for-acute-leukemia-implications-for-prophylaxis
#12
Muna Qayed, Tao Wang, Michael T Hemmer, Stephen Spellman, Mukta Arora, Daniel Couriel, Amin Alousi, Joseph Pidala, Hisham Abdel-Azim, Mahmoud Aljuf, Mouhab Ayas, Menachem Bitan, Mitchell Cairo, Sung Won Choi, Christopher Dandoy, David Delgado, Robert Peter Gale, Gregory Hale, Haydar Frangoul, Rammurti T Kamble, Mohamed Kharfan-Dabaja, Leslie Lehman, John Levine, Margaret MacMillan, David I Marks, Taiga Nishihori, Richard F Olsson, Peiman Hematti, Olov Ringden, Ayman Saad, Prakash Satwani, Bipin N Savani, Kirk R Schultz, Sachiko Seo, Shalini Shenoy, Edmund K Waller, Lolie Yu, Mary M Horowitz, John Horan
Relapse remains the major cause of mortality post hematopoietic cell transplantation (HCT) for pediatric acute leukemia. Previous research suggests that reducing the intensity of calcineurin inhibitor based graft versus host disease (GVHD) prophylaxis may be an effective strategy in abrogating the risk of relapse in pediatric patients undergoing matched sibling donor (MSD) HCT. We reasoned that benefits of this strategy could be maximized by selectively applying it to those patients least likely to develop GVHD...
November 16, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29154388/systematic-review-of-the-effect-of-denosumab-on-children-with-osteogenesis-imperfect-showed-inconsistent-findings
#13
Guowei Li, Yanling Jin, Mitchell A H Levine, Heike Hoyer-Kuhn, Leanne Ward, Jonathan D Adachi
Osteogenesis imperfecta (OI) is a rare, inherited disorder characterised by increased bone fragility, reduced bone mass and often short stature. Most cases are due to mutations in one of the two genes encoding collagen type-1 - COL1A1 and COL1A2 (1) - and the signs and symptoms vary substantially with the disease severity. The risk of fractures is 100 times higher than the general population, even in patients with mild OI. Bisphosphonates are the current standard pharmacotherapy for managing and treating patients with severe OI, but concerns about their long-term safety have been raised (1)...
November 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29154266/rehabilitation-of-neglected-monteggia-fracture-dislocations-in-children
#14
Azad Yıldırım, Kemal Nas
PURPOSE: There are limited studies related to the rehabilitation of neglected Monteggia fracture-dislocations. This study reports the results of the rehabilitation of neglected Monteggia fractures and dislocations and the best treatment options available. MATERIAL AND METHODS: Thirteen children were rehabilitated between 2009 and 2012. A retrospective chart review was conducted to record the following: age, gender, anatomic region of fractures, time delay from symptom onset to fracture, Bado classification, Mayo Elbow Performance Index (MEPI) which includes pain, range of motion and daily life comfort, surgeries, length of hospitalization, location and pattern of fracture, length of follow-up and complications...
November 6, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/29154020/skeletal-surveys-in-young-injured-children-a-systematic-review
#15
REVIEW
Christine W Paine, Joanne N Wood
Skeletal surveys (SSs) have been identified as a key component of the evaluation for suspected abuse in young children, but variability in SS utilization has been reported. Thus, we aimed to describe the utilization patterns, yield, and risks of obtaining SS in young children through a systematic literature review. We searched PubMed/MEDLINE and CINAHL databases for articles published between 1990 and 2016 on SS. We calculated study-specific percentages of SS utilization and detection of occult fractures and examined the likelihoods that patient characteristics predict SS utilization and detection of occult fractures...
November 15, 2017: Child Abuse & Neglect
https://www.readbyqxmd.com/read/29153191/atresiaplasty-in-congenital-aural-atresia-what-the-facial-plastic-surgeon-needs-to-know
#16
REVIEW
Douglas S Ruhl, Bradley W Kesser
Patients with microtia and congenital aural atresia should have a comprehensive hearing assessment early in life. Options for hearing habilitation should be presented, and children with bilateral aural atresia should be fitted with a bone conducting hearing device to support normal speech and language development. If atresia surgery is pursued, the microtia surgeon must be aware of certain principles. This article presents recommendations on options for potentially improving hearing in children with congenital aural atresia: assessing surgical candidacy; chronology and timing of surgeries; functional importance of certain ear structures; and understanding the possible locations of an aberrant facial nerve to avoid injury in these patients...
February 2018: Facial Plastic Surgery Clinics of North America
https://www.readbyqxmd.com/read/29152441/trabecular-variant-juvenile-ossifying-fibroma-of-the-maxilla
#17
Ashwan Paranthaman, Vandana Shenoy, Senthil Kumar, Laavanya Marimuthu, Sakthivel Velusubbiah, Shonali Vijayaraj
Juvenile ossifying fibroma (JOF) is a benign, bone-forming neoplasm occurring primarily in children and adolescents. JOF is an aggressive variant of ossifying fibroma of the jaw with a variable clinical behavior and a high tendency for recurrence. Early detection and prompt treatment are required to treat JOF successfully. This case report describes JOF in a 13-year-old girl presenting with a year-long, gradually progressive swelling on the right side of her face with typical clinical, radiological, and histopathological features...
September 14, 2017: Curēus
https://www.readbyqxmd.com/read/29152060/inhibition-of-chk1-sensitizes-ewing-sarcoma-cells-to-the-ribonucleotide-reductase-inhibitor-gemcitabine
#18
Kelli L Goss, Stacia L Koppenhafer, Kathryn M Harmoney, William W Terry, David J Gordon
Ewing sarcoma is a bone and soft tissue sarcoma that occurs in children and young adults. The EWS-FLI1 gene fusion is the driver mutation in most Ewing sarcoma tumors and functions, in part, as an aberrant transcription factor. We recently identified that Ewing sarcoma cells are sensitive to inhibition of ribonucleotide reductase (RNR), which catalyzes the formation of deoxyribonucleotides from ribonucleotides. In this report, we show that Ewing sarcoma cells are sensitive to treatment with clofarabine, which is a nucleoside analogue and allosteric inhibitor of RNR...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29150647/impact-of-multidecadal-climate-variability-on-united-kingdom-rickets-rates
#19
Haris Majeed, G W K Moore
Children who receive inadequate exposure to sunlight have reduced levels of vitamin D, resulting in rickets, a disease that is characterized by bone deformity, stunted growth, and long term pronounced disability. The United Kingdom rickets incidence rates declined from the early 1960's to mid-1990's, after which there was a dramatic increase. The reason for this change is not well understood. Here we show that an important low frequency mode of climate variability, the Atlantic Multidecadal Oscillation (AMO), with a period of ~60-80 years, has an impact on rickets incidence rates in the United Kingdom through changes in sea level pressure, cloud cover and sunshine duration...
November 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29150356/cryptococcosis-manifesting-as-isolated-biliary-infection-case-report-and-brief-review-of-literature
#20
Hua-Ying Zhou, Xiao-Bo Zeng, Sheng-Long Shi, Min Wang, Da-Min Li
BACKGROUND: Biliary cryptococci infection is rare, which is frequently diagnosed by exploratory laparotomy, preoperative diagnosis is difficult. CASE PRESENTATION: A 14-year-old girl presented with intermittent jaundice for 6 years. She had no pruritus, anorexia, nausea or vomiting, fever, abdominal pain, or clay stools. Laboratory tests showed obstructive jaundice, eosinophilia, and increased IgE levels. The patient was ultimately diagnosed as Cryptococcal infection by bone marrow culture...
November 14, 2017: Clinics and Research in Hepatology and Gastroenterology
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