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https://www.readbyqxmd.com/read/28938474/response-to-letter-the-effect-of-whole-body-vibration-training-on-bone-and-muscle-function-in-children-with-osteogenesis-imperfecta
#1
Wolfgang Högler, Nick Bishop, Peter Nightingale, Nick Shaw, Raja Padidela, Nicola Crabtree
No abstract text is available yet for this article.
August 29, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28938436/comment-on-the-effect-of-whole-body-vibration-training-on-bone-and-muscle-function-in-children-with-osteogenesis-imperfecta
#2
Heike Hoyer-Kuhn, Eckhard Schoenau, Oliver Semler
No abstract text is available yet for this article.
August 30, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28938420/bone-structural-characteristics-and-response-to-bisphosphonate-treatment-in-children-with-hajdu-cheney-syndrome
#3
Sophia Sakka, Rachel I Gafni, Justin H Davies, Bart Clarke, Peter Tebben, Mark Samuels, Vrinda Saraff, Klaus Klaushofer, Nadja Fratzl-Zelman, Paul Roschger, Frank Rauch, Wolfgang Högler
Context: Hajdu-Cheney syndrome (HJCYS) is a rare, multisystem, bone disease caused by heterozygous mutations in the NOTCH2 gene. Histomorphometric and bone ultrastructural analyses in children have not been reported and sparse evidence exists on response to bisphosphonate (BP) therapy. Objective: To investigate clinical and bone histomorphometric characteristics, bone matrix mineralization and the response of bone geometry and density to BP therapy. Patients: Five children with HJCYS (3 males) aged between 6...
September 8, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28938290/body-composition-changes-in-severely-burned-children-during-icu-hospitalization
#4
Janos Cambiaso-Daniel, Ioannis Malagaris, Eric Rivas, Gabriel Hundeshagen, Charles D Voigt, Elizabeth Blears, Ron P Mlcak, David N Herndon, Celeste C Finnerty, Oscar E Suman
OBJECTIVES: Prolonged hospitalization due to burn injury results in physical inactivity and muscle weakness. However, how these changes are distributed among body parts is unknown. The aim of this study was to evaluate the degree of body composition changes in different anatomical regions during ICU hospitalization. DESIGN: Retrospective chart review. SETTING: Children's burn hospital. PATIENTS: Twenty-four severely burned children admitted to our institution between 2000 and 2015...
September 21, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28938257/childhood-visceral-leishmaniasis-distinctive-features-and-diagnosis-of-a-re-emerging-disease-an-11-year-experience-from-a-tertiary-referral-center-in-athens-greece
#5
Panagiotis Krepis, Adamantia Krepi, Ioanna Argyri, Anastasios Aggelis, Alexandra Soldatou, Maria Tsolia
BACKGROUND: Visceral leishmaniasis (VL) remains a public health issue in Greece. The aim of this study was to describe the clinical and epidemiological characteristics of pediatric VL in our region as well as to evaluate the laboratory findings and the diagnostic techniques that are applied. METHODS: We retrospectively reviewed the medical records of all children diagnosed with VL in an 11-year period at a tertiary public hospital in the region of Athens. Demographic features, clinical information and laboratory findings were accessed...
September 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28938256/chronic-q-fever-infections-in-israeli-children-a-25-year-nationwide-study
#6
Nimrod Sachs, Yafit Atiya-Nasagi, Adi Beth-Din, Itzhak Levy, Shalom Ben-Shimol, Diana Tasher, Galia Grisaru-Soen, Haleema Dabaja, Imad Kassis, Shiri Spilman, Efraim Bilavsky
BACKGROUND: Q fever is a zoonosis caused by the bacterium Coxiella burnetii (C. burnetii) with a worldwide distribution. Our aim was to assess the epidemiology, clinical manifestations and treatment regimens of chronic Q fever infections in Israeli children during the past 25 years. METHODS: Cases were collected from the national Q fever reference laboratory database. Demographic, epidemiologic, and clinical data were reviewed using a structured questionnaire sent to the referring physician...
September 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28937024/celiac-disease-related-osteopathy-among-saudi-celiac-patients-are-we-adherent-to-recommendations
#7
Mona A Fouda
BACKGROUND/AIMS: There are no reports from Saudi Arabia documenting the picture of osteopathy in celiac disease (CD) and the adherence of physicians to the guidelines and recommendations to screen for bone disease. We conducted this study to document the prevalence of CD-related osteopathy and the Saudi physicians' adherence to the screening recommendations. PATIENTS AND METHODS: We identified the biopsy proven CD cases diagnosed between 2003 and 2012. In addition to demographic data, we collected laboratory (serum calcium, phosphate, alkaline phosphatase, 25-dihydroxy vitamin D, and parathyroid hormone levels) and imaging [Dual-energy X-ray absorptiometry (DEXA)] data...
September 2017: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
https://www.readbyqxmd.com/read/28936809/association-between-vitamin-d-and-carboxy-terminal-cross-linked-telopeptide-of-type-i-collagen-in-children-during-growth-hormone-replacement-therapy
#8
Ewelina Witkowska-Sędek, Anna Stelmaszczyk-Emmel, Anna Kucharska, Urszula Demkow, Beata Pyrżak
Growth hormone and insulin-like growth factor-1 (IGF-1) play a crucial role in the regulation of bone turnover. Adequate vitamin D status supports proper bone remodeling, leading to normal longitudinal bone growth and normal peak bone mass. The aim of this study was to evaluate the association between serum 25-hydroxyvitamin D [25(OH)D] and carboxy-terminal cross-linked telopeptide of type I collagen (ICTP) in children and adolescents with growth hormone deficiency at baseline and during recombinant human growth hormone (rhGH) replacement therapy...
September 22, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28934267/patient-derived-osteosarcoma-cells-are-resistant-to-methotrexate
#9
Amanda Dos Santos Cavalcanti, Walter Meohas, Gabriele de Oliveira Ribeiro, Ana Cristina de Sá Lopes, Sharareh Gholamin, Mostafa Razavi, Taís Hanae Kasai Brunswick, Amir Avan, João Antonio Matheus Guimarães, Maria Eugenia Leite Duarte, Suzana Assad Kahn
Osteosarcoma is the most common primary bone tumor in children and young adults. The median survival of osteosarcoma patients has not significantly improved since 1990, despite administration of different classes of chemotherapy agents, such as methotrexate, cisplatin and doxorubicin. Cancer stem cells (CSCs) are responsible for the resistance of osteosarcoma to chemotherapy and OCT4, SOX2 and SSEA4 have been used to identify CSCs in osteosarcoma. Here, we used low-passage patient-derived osteosarcoma cells and osteosarcoma cells directly isolated from patients before and after chemotherapy treatments to evaluate the effects of chemotherapy on stem cell markers expression...
2017: PloS One
https://www.readbyqxmd.com/read/28933808/pediatric-orthopedic-injuries-evidence-based-management-in-the-emergency-department-digest
#10
Jamie Lien, Kathryn H Pade
Upper and lower extremity injuries are common in children, with an overall risk of fracture estimated at just under 1 in 5 children. Pediatric bone anatomy and physiology produce age specific injury patterns and conditions that are unique to children, which can make accurate diagnosis difficult for emergency clinicians. This issue reviews the etiology and pathophysiology of child-specific fractures, as well as common injuries of the upper and lower extremities. Evidence-based recommendations for management of pediatric fractures, including appropriate diagnostic studies and treatment, are also discussed...
September 22, 2017: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/28933358/substrate-deprivation-therapy-to-reduce-glycosaminoglycan-synthesis-improves-aspects-of-neurological-and-skeletal-pathology-in-mps-i-mice
#11
Ainslie L K Derrick-Roberts, Matilda R Jackson, Carmen E Pyragius, Sharon Byers
Mucopolysaccharidosis type I (MPS I) is the most common form of the MPS group of genetic diseases. MPS I results from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to accumulation of undegraded heparan and dermatan sulphate glycosaminoglycan (GAG) chains in patient cells. MPS children suffer from multiple organ failure and die in their teens to early twenties. In particular, MPS I children also suffer from profound mental retardation and skeletal disease that restricts growth and movement. Neither brain nor skeletal disease is adequately treated by current therapy approaches...
February 23, 2017: Diseases (Basel)
https://www.readbyqxmd.com/read/28932646/reconstitution-of-multifunctional-cd56-low-cd16-low-natural-killer-cell-subset-in-children-with-acute-leukemia-given-%C3%AE-%C3%AE-t-cell-depleted-hla-haploidentical-haematopoietic-stem-cell-transplantation
#12
Stabile Helena, Nisti Paolo, Peruzzi Giovanna, Fionda Cinzia, Pagliara Daria, Pomonia Brescia Letizia, Merli Pietro, Locatelli Franco, Angela Santoni, Angela Gismondi
We recently described the CD56(low)CD16(low) subset of Natural Killer (NK) cells that both mediate cytotoxic activity and produce IFNγ, being more abundant in bone marrow (BM) than in peripheral blood (PB) of pediatric normal subjects. Given the multifunctional properties of this subset, we examined its development and functional recovery in a cohort of children undergoing α/β T-cell depleted HLA-haploidentical haematopoietic stem cell transplantation (HSCT). The results obtained indicate that CD56(low)CD16(low) NK cells are present in both PB and BM already at one month post-HSCT, with an increased frequency in BM of graft recipients as compared with normal subjects...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28932332/juvenile-psammomatoid-ossifying-fibroma-jpof-of-proximal-radius-a-rare-entity
#13
Jagadish Prabhu, Veena Nagaraj, Iftikhar Ahmed Mukhtar
BACKGROUND: Juvenile psammomatoid ossifying fibroma (JPOF) is a rare fibro-osseous lesion that usually occurs in the facial bones. The pathognomonic histopathologic feature is the presence of spherical ossicles, which are similar to psammoma bodies. It is considered to be a unique lesion because of its reported tendency to occur in children and adolescents and its tendency for locally aggressive growth. Because this lesion is aggressive in nature with high recurrence rate, early detection and complete surgical excision are essential...
2017: Open Orthopaedics Journal
https://www.readbyqxmd.com/read/28932154/malpositioned-canine-treatment-with-autotransplantation-and-laser
#14
Serap Keskin Tunc, Mehmet Savas Kayasan, Esma Ozeroglu, Cennet Neslihan Eroglu
Children and young adults often have tooth loss due to congenital tooth deficiency, trauma, or caries. Autotransplantation has many benefits. The transplanted tooth can be moved orthodontically, maintains alveolar bone growth potential during eruption, and functional periodontal ligament function also permits tooth eruption, allows the defected areas to be filled with the bones; gingival contour is much more successful than the one obtained with prosthesis. In this paper, treatment steps and follow-up results of autotransplantation case supported with biostimulation are mentioned...
July 2017: European Journal of Dentistry
https://www.readbyqxmd.com/read/28929265/-fractures-of-the-lower-extremities-in-childhood-part-1-fractures-of-the-thigh-and-in-the-proximity-of-the-knee
#15
L Kremer, M Voth, I Marzi
Fractures in children are a major challenge in everyday clinical practice. The decision about the correct treatment of fractures follows the consideration of individual factors. The age of the child and the resulting residual growth play an essential role. This article aims to facilitate a structured approach with respect to examination, diagnostics and treatment in clinical practice. This first part of this series deals with fractures of the thigh and in the proximity of the knee joint in children. The peculiarity of pediatric bone lies in its growth, which results not only in a much faster healing tendency than in adults but also possesses a greater correction potential...
September 19, 2017: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/28929078/primary-ewing-s-sarcoma-of-the-temporal-bone-a-rare-case-report-and-literature-review
#16
Divya Gupta, Achal Gulati, Purnima
Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy...
September 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28927975/pediatric-arterial-catheters-complications-and-associated-risk-factors
#17
Ferdynand Hebal, Hayley T Sparks, Karen L Rychlik, Meredith Bone, Sifrance Tran, Katherine A Barsness
BACKGROUND/PURPOSE: Arterial catheter complications are a common problem in a pediatric critical care setting, but reported complication rates and risk factors associated with peripheral arterial catheter complications vary. We conducted a retrospective cohort study to identify risk factors in a pediatric patient population. METHODS: We performed a detailed abstraction of provider notes in the electronic medical records of inpatients ≤18years of age who underwent arterial line placement between January 1, 2008 and January 1, 2013 at a university-affiliated standalone pediatric hospital...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28926829/continuous-subcutaneous-recombinant-parathyroid-hormone-1-34-infusion-in-the-management-of-childhood-hypoparathyroidism-associated-with-malabsorption
#18
Vrinda Saraff, Anya Rothenbuhler, Wolfgang Högler, Agnès Linglart
BACKGROUND/AIMS: Hypoparathyroidism associated with malabsorption can be particularly challenging to manage due to limited and erratic intestinal absorption of calcium and vitamin D analogues, resulting in episodes of hypo- or hypercalcaemia. We evaluated the role of continuous subcutaneous recombinant parathyroid hormone (rhPTH 1-34) infusion (CSPI) in children with hypoparathyroidism associated with intestinal malabsorption resistant to conventional therapy. METHOD: Four patients (8-13 years of age), with symptomatic hypocalcaemia resistant to conventional therapy, were started on CSPI (follow-up 3-8 years) in two paediatric endocrinology units in Europe...
September 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28924803/patient-derived-xenografts-as-preclinical-neuroblastoma-models
#19
REVIEW
Noémie Braekeveldt, Daniel Bexell
The prognosis for children with high-risk neuroblastoma is often poor and survivors can suffer from severe side effects. Predictive preclinical models and novel therapeutic strategies for high-risk disease are therefore a clinical imperative. However, conventional cancer cell line-derived xenografts can deviate substantially from patient tumors in terms of their molecular and phenotypic features. Patient-derived xenografts (PDXs) recapitulate many biologically and clinically relevant features of human cancers...
September 19, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28921128/precision-of-bone-density-and-micro-architectural-properties-at-the-distal-radius-and-tibia-in-children-an-hr-pqct-study
#20
C E Kawalilak, A T Bunyamin, K M Björkman, J D Johnston, S A Kontulainen
Precision errors need to be known when monitoring bone micro-architecture in children with HR-pQCT. Precision errors for trabecular bone micro-architecture ranged from 1 to 8% when using the standard evaluation at the radius and tibia. Precision errors for cortical bone micro-architecture ranged from 1 to 11% when using the advanced cortical evaluation. INTRODUCTION: Our objective was to define HR-pQCT precision errors (CV%RMS) and least significant changes (LSCs) at the distal radius and tibia in children using the standard evaluation and the advanced cortical evaluation...
September 18, 2017: Osteoporosis International
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