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Childrens Bones

Ellen G H M van den Heuvel, Jan M J M Steijns
The relevance of dairy produce for the diminishment of osteoporotic risk is still a matter of scientific debate due to the outcome of a few single observational studies. This review will address the most robust point estimate on the role of dairy products, as reported in systematic reviews and meta-analyses on randomised controlled trials in the case of bone mineralisation or prospective studies in the case of fracture risk. Plain dairy products or those fortified with Ca and/or vitamin D improve total body bone mineral content (BMC) by 45-50 g over 1 year when the daily baseline Ca intake is lower than 750 mg in Caucasians and Chinese girls...
March 21, 2018: Nutrition Research Reviews
Maria L Stunkel, Scott E Brodie, Artur V Cideciyan, Wanda L Pfeifer, Elizabeth L Kennedy, Edwin M Stone, Samuel G Jacobson, Arlene V Drack
PURPOSE: GUCY2D has been associated with autosomal recessive Leber Congenital Amaurosis and autosomal dominant cone-rod dystrophy. This report expands the phenotype of autosomal recessive mutations to congenital night blindness which may slowly progress to retinitis pigmentosa. DESIGN: Retrospective case series. METHODS: Multicenter study of five patients (3 male, 2 female). RESULTS: All presented with night blindness since childhood...
March 17, 2018: American Journal of Ophthalmology
Alexia J Murphy-Alford, Melinda White, Liane Lockwood, Andrew Hallahan, Peter S W Davies
AIM: To describe the body composition, dietary intake and physical activity and of paediatric, adolescent and young adult childhood cancer survivors (CCS) and examine the factors that impact body composition after treatment. METHODS: This prospective cross-sectional study involved 74 subjects who were at least three years post treatment. Measurements included anthropometry, whole body potassium counting, air displacement plethysmography, and three day physical activity and diet diaries...
March 7, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Alma V Huidobro-Chávez, Gianmarco D Vigo Pareja, Carlos Pachas-Peña, Karina Patiño-Calla
The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. We present an 18-month-old infant who consulted due to periorbital edema and proptosis of the right eye, with two months of evolution. The computed tomography and the magnetic resonance imaging showed a maxillary sinus tumor mass of expansive growth and erosion of the roof of the orbit...
April 1, 2018: Archivos Argentinos de Pediatría
Despina Piatopoulou, Margaritis Avgeris, Ioanna Drakaki, Antonios Marmarinos, Marieta Xagorari, Margarita Baka, Apostolos Pourtsidis, Lydia Kossiva, Dimitrios Gourgiotis, Andreas Scorilas
Although childhood acute lymphoblastic leukemia (ALL) is characterized by high remission rates, there are still patients who experience poor response to therapy or toxic effects due to intensive treatment. In the present study, we examined the expression profile of miR-143 and miR-182 in childhood ALL and evaluated their clinical significance for patients receiving Berlin-Frankfurt-Münster (BFM) protocol. Bone marrow specimens from 125 childhood ALL patients upon diagnosis and the end-of-induction (EoI; day 33), as well as from 64 healthy control children undergone RNA extraction, polyadenylation, and reverse transcription...
March 20, 2018: Annals of Hematology
Judith Sebestyen VanSickle, Tarak Srivastava, Uri S Alon
The calcium-sensing receptor (CaSR) plays an important role in the homeostasis of serum ionized calcium by regulating parathyroid hormone (PTH) secretion and tubular calcium handling. Calcimimetics, which act by allosteric modulation of the CaSR, mimic hypercalcemia resulting in suppression of PTH release and increase in calciuria. Mostly used in children to treat secondary hyperparathyroidism associated with advanced renal failure, we have shown that calcimimetics can also be successfully used in children with bone and mineral disorders in which elevated PTH plays a detrimental role in skeletal pathophysiology in the face of normal kidney function...
March 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Nashwa M Samra, Shaimaa Emad El Abrak, Hanaa H El Dash, Mona El Said El Raziky, Manal A El Sheikh
BACKGROUND: Hepatic osteodystrophy caused by vitamin D and calcium malabsorption is thought to develop in children with cholestatic liver disease leading to secondary hyperparathyroidism and rickets or osteomalacia. The aim of this study was to evaluate the dental and bone mineral densities and the serum level of vitamin D in cholestatic infants and children and to correlate this process with clinical and laboratory parameters. METHODS: This is a cross-sectional study that include 50 patients presenting with cholestasis...
March 15, 2018: Clinics and Research in Hepatology and Gastroenterology
Rachael F Grace, Paola Bianchi, Eduard J van Beers, Stefan W Eber, Bertil Glader, Hassan M Yaish, Jenny M Despotovic, Jennifer A Rothman, Mukta Sharma, Melissa M McNaull, Elisa Fermo, Kimberly Lezon-Geyda, D Holmes Morton, Ellis J Neufeld, Satheesh Chonat, Nina Kollmar, Christine M Knoll, Kevin Kuo, Janet L Kwiatkowski, Dagmar Pospíŝilová, Yves D Pastore, Alexis A Thompson, Peter E Newburger, Yaddanapudi Ravindranath, Winfred C Wang, Marcin W Wlodarski, Heng Wang, Susanne Holzhauer, Vicky R Breakey, Joachim Kunz, Sujit Sheth, Melissa J Rose, Heather A Bradeen, Nolan Neu, Dongjing Guo, Hasan Al-Sayegh, Wendy B London, Patrick G Gallagher, Alberto Zanella, Wilma Barcellini
An international, multicenter registry was established to collect retrospective and prospective clinical data on patients with pyruvate kinase (PK) deficiency, the most common glycolytic defect causing congenital non-spherocytic hemolytic anemia. Medical history and laboratory and radiologic data were retrospectively collected at enrollment in 254 patients with molecularly confirmed PK deficiency. Perinatal complications were common, including anemia requiring transfusions, hyperbilirubinemia, hydrops, and prematurity...
March 16, 2018: Blood
Joseph P Herbert, Sidish S Venkataraman, Ali H Turkmani, Liang Zhu, Marcia L Kerr, Rajan P Patel, Irma T Ugalde, Stephen A Fletcher, David I Sandberg, Charles S Cox, Ryan S Kitagawa, Arthur L Day, Manish N Shah
OBJECTIVE The objective of this study was to assess the incidence, diagnosis, and treatment of pediatric blunt cerebrovascular injury (BCVI) at a busy Level 1 trauma center and to develop a tool for accurately predicting pediatric BCVI and the need for diagnostic testing. METHODS This is a retrospective cohort study of a prospectively collected database of pediatric patients who had sustained blunt trauma (patient age range 0-15 years) and were treated at a Level 1 trauma center between 2005 and 2015. Digital subtraction angiography, MR angiography, or CT angiography was used to confirm BCVI...
March 16, 2018: Journal of Neurosurgery. Pediatrics
Jun Bian, Dan Cao, Jie Shen, Bo Jiang, Dan Chen, Lanzheng Bian
N-methyl pyrrolidone (NMP), a small bioactive molecule, has the potential to stimulate bone formation and inhibit osteoclast differentiation. The aim of the present study was to investigate the effect of NMP on the inflammatory response and underlying molecular mechanisms in MG-63 cells. The mRNA and protein expression of cytokines from peripheral blood in children with or without ankle fracture were determined by reverse transcription-quantitative polymerase chain reaction (RT-qPCR) and ELISA, respectively...
April 2018: Experimental and Therapeutic Medicine
Adam Gassas, Ponni Sivaprakasam, Michelle Cummins, Patricia Breslin, Katharine Patrick, Mary Slatter, Roderick Skinner, Geof Shenton, Brenda Gibson, Sarah Lawson, Toni Petterson, Michael Potter, Beki James, Rachael Hough, Prashant Hiwarkar, Ajay Vora, Paul Veys, Josu De La Fuente, Robert Wynn, Persis Amrolia
Paediatric therapy-related acute myeloid leukaemia (t-AML) is rare and the outcome is poor. While allogeneic haematopoietic stem cell transplantation (HSCT) is generally the accepted modality of treatment, data regarding salvage chemotherapy, remission induction, conditioning regimens, transplant-related mortality and outcome is scarce. Between 2000 and2016, 36 children with t-AML were treated in seven UK paediatric HSCT centres. The most common salvage protocol for remission induction was FLAG with or without idarubicin and 28 patients were in complete morphological remission prior to BMT...
March 15, 2018: Bone Marrow Transplantation
Qingkai Dai, Xiaojuan Liu, Hui Yang, Siqi Guo, Yuefang Wang, Luyun Peng, Lei Ye, Lan Chen, Chunqi Lai, Qi Chen, Ge Zhang, Yongmei Jiang
Detection of aberrant antigen expression in acute lymphoblastic leukemia (ALL) by flow cytometric is proposed for the quantification of minimal residual disease (MRD). There are few studies that investigate the stability of the antigen expression in children with B lineage ALL at the end of remission induction therapy and determine its prognostic impact. Between 2010 and 2015, 691 bone marrow specimens of childhood ALL were sent at diagnosis for immunophenotypic characterization, and follow-up samples for MRD were analyzed on day 33...
March 2, 2018: Leukemia Research
Diana Olvera, Rachel Stolzenfeld, Joan C Marini, Michelle S Caird, Kenneth M Kozloff
Osteogenesis imperfecta (OI) is a genetic disorder characterized by altered bone quality and imbalanced bone remodeling, leading to skeletal fractures which are most prominent during childhood. Treatments for OI have focused on restoring pediatric bone density and architecture to recover functional strength and consequently reduce fragility. Though antiresorptive agents like bisphosphonates (BP) are currently the most common intervention for the treatment of OI, a number of studies have shown efficacy of sclerostin antibody (SclAb) in inducing gains in bone mass and reducing fragility in OI mouse models...
March 15, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
A A Yarovoy, O V Golubeva, S S Kleyankina, T V Yanchenko
Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. JX can develop in any part of the eye and its appendages. Taking into consideration how rare this pathology is, its diagnosis and treatment are complicated...
2018: Vestnik Oftalmologii
Nataliya Zelinska, Iryna Shevchenko, Evgenia Globa
BACKGROUND: We investigated the prevalence of Turner syndrome (TS) in Ukrainian population, the frequency of karyotype variants, the age of children at diagnosis, the degree of short stature and phenotypic features in TS girls. MATERIAL AND METHODS: A retrospective analyses was made in 538 TS girls aged 0.11-18.2 years old within the period of 2005-2015 with detailed examination of 150 patients. RESULTS AND DISCUSSION: The prevalence of TS in Ukraine is 77...
February 28, 2018: Journal of Clinical Research in Pediatric Endocrinology
Elvira Larqué, Eva Morales, Rosaura Leis, José E Blanco-Carnero
BACKGROUND: To what extent does the circulating 25-hydroxyvitamin D (25[OH]D) concentration help to meet the physiological needs of humans is an ongoing subject of debate. Remaining unexposed to the sun to reduce melanoma cancer risk, current lifestyle with less out door activities, and increasing obesity rates, which in turn increases the storage of vitamin D in the adipose tissue, are presumably factors that contribute to the substantial upsurge in the prevalence of vitamin D deficiency in humans...
March 13, 2018: Annals of Nutrition & Metabolism
Désirée van der Heijde, David I Daikh, Neil Betteridge, Gerd R Burmester, Afton L Hassett, Eric L Matteson, Ronald van Vollenhoven, Sharad Lakhanpal
A European League Against Rheumatism-American College of Rheumatology working group consisting of practicing and academic rheumatologists, a rheumatology researcher, and a patient representative created a succinct general statement describing rheumatic and musculoskeletal diseases (RMDs) in adults and children in language that can be used in conversations with the lay public, media, healthcare providers, and other stakeholders. Based on the literature review, several elements were deemed important for inclusion in the description of RMDs...
March 13, 2018: Arthritis & Rheumatology
Luis A Aponte-Tinao, Jose I Albergo, Miguel A Ayerza, D Luis Muscolo, Federico Milano Ing, German L Farfalli
BACKGROUND: Preservation of limb function after resection of malignant bone tumors in skeletally immature children is challenging. Resection of bone sarcomas and reconstruction with an allograft in patients younger than 10 years old is one reconstructive alternative. However, long-term studies analyzing late complications and limb length discrepancy at skeletal maturity are scarce; this information would be important, because growth potential is altered in these patients owing to the loss of one physis during tumor resection...
March 2018: Clinical Orthopaedics and related Research
Beata Znorko, Dariusz Pawlak, Ewa Oksztulska-Kolanek, Tomasz Domaniewski, Anna Pryczynicz, Alicja Roszczenko, Joanna Rogalska, Paweł Lipowicz, Michał Doroszko, Malgorzata Michalina Brzoska, Krystyna Pawlak
Osteoprotegerin (OPG), receptor activator of NF-κB ligand (RANKL), and parathyroid hormone (PTH) play a central role in the regulation of bone turnover in chronic kidney disease (CKD), but their influence on bone mineral density (BMD) and strength remains unclear, particularly in children. We studied the clinical significance of OPG and RANKL in relation to PTH, femur weight, BMD, and bone biomechanical properties in growing rats after one month (CKD-1) and three months (CKD-3) of surgically-induced mild CKD...
March 9, 2018: Cytokine
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