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https://www.readbyqxmd.com/read/29775451/-recurrent-urolithiasis-as-a-symptom-of-primary-hyperparathyroidism-in-a-16-year-old-boy
#1
Monika Wojciechowska, Beata Bieniaś, Aleksandra Sobieszczańska-Droździel, Anna Wieczorkiewicz-Płaza, Iwona Beń-Skowronek, Przemysław Sikora
Primary hyperparathyroidism is one of the most common endocrine diseases, however, it is rare in children. In most cases, it is caused by adenoma of these organs. Its most common complications include urolithiasis, nephrocalcinosis and osteoporosis. CASE REPORT: A 16-year-old patient was admitted to our Clinic because of his first-ever renal colic. The ultrasound examination revealed rightsided hydronephrosis caused by the presence of 9 mm stone in the upper part of the right ureter. In addition, the presence of 8 mm stone in the middle calyx of the left kidney was found...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29772353/cardiac-profile-of-chimeric-antigen-receptor-car-t-cell-therapy-in-children-a-single-institution-experience
#2
Danielle Burstein, Shannon Maude, Stephen Grupp, Heather Griffis, Joseph Rossano, Kimberly Lin
BACKGROUND: Immunotherapy with chimeric antigen receptor (CAR)-modified T-cells targeting CD19 for pediatric acute lymphoblastic leukemia (ALL) has demonstrated significant efficacy. The principle toxicity is cytokine release syndrome with resultant hypotension. However, the spectrum of cardiovascular effects associated with CAR T-cell therapy has not been systematically evaluated. METHODS: We reviewed all patients who received CD19-directed CAR T-cells at the Children's Hospital of Philadelphia between April 2012 and September 2016...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29769040/novel-aggrecan-variant-p-gln2364pro-causes-severe-familial-nonsyndromic-adult-short-stature-and-poor-growth-hormone-response-in-chinese-children
#3
Dandan Xu, Chengjun Sun, Zeyi Zhou, Bingbing Wu, Lin Yang, Zhuo Chang, Miaoying Zhang, Li Xi, Ruoqian Cheng, Jinwen Ni, Feihong Luo
BACKGROUND: Mutations in the aggrecan (ACAN) gene can cause short stature (with heterogeneous clinical phenotypes), impaired bone maturation, and large variations in response to growth hormone (GH) treatment. For such cases, long-term longitudinal therapy data from China are still scarce. We report that a previously unknown ACAN gene variant reduces adult height and we analyze the GH response in children from an affected large Chinese family. METHODS: Two children initially diagnosed with idiopathic short stature (ISS) and a third mildly short child from a large Chinese family presented with poor GH response...
May 16, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29768519/influence-of-growth-hormone-replacement-on-neurological-and-psychomotor-development-case-report
#4
Felipe Motta, Adriana Pasmanik Eisencraft, Lindiane Gomes Crisostomo
The height response to the use of growth hormone in short height cases has already been confirmed in the literature. The influence of the insulin-like growth factor 1 (GH-IGF1) axis components on development, function, regeneration, neuroprotection, cognition, and motor functions has been evaluated in experimental studies and in adults with central nervous system lesions. However, there is still little research on the clinical impact of hormone replacement on neurological and psychomotor development. This report presents the case of a patient with excellent weight-height recovery and, even more surprisingly, neurological and psychomotor development in response to use of growth hormone...
May 14, 2018: Einstein
https://www.readbyqxmd.com/read/29766835/are-consumption-of-dairy-products-and-physical-activity-independently-related-to-bone-mineral-density-of-6-year-old-children-longitudinal-and-cross-sectional-analyses-in-a-birth-cohort-from-brazil
#5
Renata M Bielemann, Juliana Dos S Vaz, Marlos R Domingues, Alicia Matijasevich, Iná S Santos, Ulf Ekelund, Bernardo L Horta
OBJECTIVE: To evaluate cross-sectional and longitudinal associations of consumption of dairy products and physical activity (PA) with bone mineral density (BMD). DESIGN: Cohort study with children from the 2004 Pelotas (Brazil) Birth Cohort. SETTING: Pelotas, a medium-sized Brazilian city. SUBJECTS: The study started in 2004 and mothers/children were interviewed/measured periodically from birth to age 6 years. PA was measured by maternal proxy at 4 and 6 years and by accelerometry at 6 years...
May 16, 2018: Public Health Nutrition
https://www.readbyqxmd.com/read/29766672/ribcage-deformity-and-the-altered-breathing-pattern-in-children-with-osteogenesis-imperfecta
#6
Antonella LoMauro, Paolo Fraschini, Simona Pochintesta, Marianna Romei, Maria G D'Angelo, Andrea Aliverti
AIM: Osteogenesis Imperfecta (OI) is a genetic disease characterized by bones fragility and progressive deformity. Life expectancy is reduced in the non-lethal most severe type III form before the age of 10 years. The main cause of death in OI is respiratory insufficiency resulting from impaired thoracic function worsened by ribcage deformity and scoliosis. METHODS: We used opto-electronic plethysmography to study chest geometry, the ventilatory, and the thoraco-abdominal pattern at rest in supine position in children younger than 10 years...
May 15, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29766567/melatonin-attenuates-osteosarcoma-cell-invasion-by-suppression-of-c-c-motif-chemokine-ligand-24-through-inhibition-of-the-c-jun-n-terminal-kinase-pathway
#7
Ko-Hsiu Lu, Shih-Chi Su, Chiao-Wen Lin, Yi-Hsien Hsieh, Ya-Chiu Lin, Ming-Hsien Chien, Russel J Reiter, Shun-Fa Yang
Osteosarcoma, with its high metastatic potential, is the most prevalent malignant bone tumor in children and adolescents. Melatonin possesses multiple tumor-suppressing properties for a myriad of tumors, but little is known about the effects of melatonin on osteosarcoma metastasis. In this study, we demonstrated that melatonin elicited very low cytotoxicity and significantly inhibited cellular motility, migration, and invasion in human osteosarcoma U2OS and HOS cells. Moreover, using RNA sequencing technology, we revealed that melatonin repressed C-C motif chemokine ligand 24 (CCL24) gene expression in U2OS cells...
May 16, 2018: Journal of Pineal Research
https://www.readbyqxmd.com/read/29764966/risk-factors-for-intraarticular-heterotopic-bone-formation-in-the-temporomandibular-joint-in-juvenile-idiopathic-arthritis
#8
Matthew L Stoll, Dina Amin, Kathlyn K Powell, Catherine H Poholek, Rachel H Strait, Inmaculada Aban, Timothy Beukelman, Daniel W Young, Randy Q Cron, Peter D Waite
OBJECTIVE: Intraarticular corticosteroid (IAC) injections are often used to treat temporomandibular joint (TMJ) arthritis associated with juvenile idiopathic arthritis (JIA). One potential complication of IA therapy is heterotopic bone formation (HBF). The purpose of our study was to evaluate risk factors for HBF development in children with JIA who received IA therapy for TMJ arthritis. METHODS: This was a retrospective study of children with JIA who had received ≥ 1 IAC injection into the TMJ...
May 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29764626/25-hydroxvitamin-d-concentrations-are-not-lower-in-children-with-bronchial-asthma-atopic-dermatitis-obesity-or-attention-deficient-hyperactivity-disorder-than-in-healthy-children
#9
Thomas Reinehr, Christian Langrock, Eckard Hamelmann, Thomas Lücke, Cordula Koerner-Rettberg, Martin Holtmann, Tanja Legenbauer, Stephanie Gest, Mirjam Frank, Börge Schmidt, Katharina Radkowski, Karl-Heinz Jöckel
Vitamin D (vitD) is involved in immune regulation, and its receptor has been identified in several tissues including lung, adipose tissue, brain, and skin. Based on these observations, it has been suggested that vitD has an essential role not only in bone metabolism but also in other diseases such as atopic dermatitis (AD), bronchial asthma (BA), attention-deficit/hyperactivity disorder (ADHD), and obesity because the affected tissues express vitD receptors. Furthermore, obesity, AD, and BA are regarded as inflammatory diseases...
April 2018: Nutrition Research
https://www.readbyqxmd.com/read/29764021/-misdiagnosic-analysis-and-treatment-of-pyriform-sinus-fistula-in-children
#10
J Ma, C Ming, F Lou, M L Wang, K Lin, W J Zeng, Z C Li, X F Liu, T S Zhang
Objective: To discuss the misdiagnosis of pyriform sinus fistula and to better understand this kind of illness. Methods: The analysis was based on twenty-eight patients with congenital pyriform sinus fistula aged from 11 months to 14 years, with the median age of 5 years, and who were surgically treated from January 2013 to January 2017 in Kunming Children's Hospital.Twenty patients were misdiagnosed in other hospital.After the routine examination of neck ultrasound and enhanced CT, internal fistula was found by self-retaining laryngoscope, traced by methylene blue, and excised by high ligation...
May 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29761075/transforming-growth-factor-%C3%AE-signaling-plays-a-pivotal-role-in-the-interplay-between-osteosarcoma-cells-and-their-microenvironment
#11
REVIEW
Franck Verrecchia, Françoise Rédini
Osteosarcomas are the most frequent form of primary bone tumors and mainly affect children, adolescents, and young adults. Despite encouraging progress in therapeutic management, including the advent of multidrug chemotherapy, the survival rates have remained unchanged for more than four decades: 75% at 5 years for localized disease, but two groups of patients are still at high risk: metastatic at diagnosis (overall survival around 40% at 5 years) and/or poor responders to chemotherapy (20% at 5 years)...
2018: Frontiers in Oncology
https://www.readbyqxmd.com/read/29755469/urinary-peptides-as-a-novel-source-of-t-cell-allergen-epitopes
#12
Ricardo da Silva Antunes, John Pham, Curtis McMurtrey, William H Hildebrand, Elizabeth Phillips, Simon Mallal, John Sidney, Paula Busse, Bjoern Peters, Véronique Schulten, Alessandro Sette
Mouse allergy in both laboratory workers and in inner-city children is associated with allergic rhinitis and asthma, posing a serious public health concern. Urine is a major source of mouse allergens, as mice spray urine onto their surroundings, where the proteins dry up and become airborne on dust particles. Here, we tested whether oligopeptides that are abundant in mouse urine may contribute to mouse allergic T cell response. Over 1,300 distinct oligopeptides were detected by mass spectrometry analysis of the low molecular weight filtrate fraction of mouse urine (LoMo)...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29755234/the-substantiation-of-the-elastic-viscoplastic-model-of-the-human-spine-for-modeling-the-correction-process-of-kyphoscoliotic-deformation
#13
Konstantin S Sergeev, Valery V Piven
Purpose: The relevance of the problem is caused by an increase in the number of spine-related diseases among children, including scoliosis. Currently, there are no methodologies for the treatment of scoliosis, which ensure an unambiguous positive result. The purpose of the article is to justify the spinal model as an elastic viscoplastic body for further mathematical modeling of the process of spine correction and search for its optimal conditions. Methodology: The leading approach to the study of this problem is the development of techniques for the surgical treatment of deformities of the vertebral column with the aid of an external fixation device for the spine, providing for a rigid connection of the elements of the apparatus with each other and with the spine...
January 2018: Journal of Craniovertebral Junction and Spine
https://www.readbyqxmd.com/read/29754886/langerhans-cell-histiocytosis-in-children-diagnosis-differential-diagnosis-treatment-sequelae-and-standardized-follow-up
#14
REVIEW
Jolie Krooks, Milen Minkov, Angela G Weatherall
A definitive diagnosis of Langerhans cell histiocytosis (LCH) requires a combination of clinical presentation, histology, and immunohistochemistry. The inflammatory infiltrate contains various proportions of LCH cells, the disease hallmark, which are round and have characteristic "coffee-bean" cleaved nuclei and eosinophilic cytoplasm. Positive immunohistochemistry staining for CD1a and CD207 (langerin) are required for a definitive diagnosis. Isolated cutaneous disease should only be treated when symptomatic, because spontaneous resolution is common...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29754885/langerhans-cell-histiocytosis-in-children-history-classification-pathobiology-clinical-manifestations-and-prognosis
#15
REVIEW
Jolie Krooks, Milen Minkov, Angela G Weatherall
Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Clinical manifestations range from isolated disease with spontaneous resolution to life-threatening multisystem disease...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29753895/infected-intradural-dermoid-cyst-with-complete-dermal-sinus-of-posterior-fossa
#16
Félix K K Ségbédji, A J Tokpo, A A Nubukpo-Guménu, N K Alaoui, L M A Quenum, K Chakour, M F Chaoui, M Benzagmout
BACKGROUND: Intracranial dermoid cysts represent benign embryologic tumors. Children and young adults are most affected. Infected intradural dermoid cyst with complete dermal sinus of posterior fossa is exceptional. The main feature of this dermal sinus leads from the midline intradural cyst through an opening in the occipital bone to be attached to the skin. METHOD: We report two cases of infected intradural dermoid cyst with complete dermal sinus of posterior fossa in children, with special attention to the clinical, anatomical features of this rare combination...
May 10, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29753543/increased-fracture-risk-with-furosemide-use-in-children-with-congenital-heart-disease
#17
Ji Haeng Heo, Karen L Rascati, Keila N Lopez, Brady S Moffett
OBJECTIVES: To determine the association of furosemide therapy with the incidence of bone fractures in children with congenital heart disease. STUDY DESIGN: We conducted a retrospective cohort study with data extracted from the 2008-2014 Texas Medicaid databases. Pediatric patients aged <12 years diagnosed with congenital heart disease, cardiomyopathy, or heart failure were included. Patients taking furosemide were categorized into a furosemide-adherent group (medication possession ratio of ≥70%), and a furosemide-nonadherent group (medication possession ratio of <70%)...
May 9, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29753157/treosulfan-fludarabine-and-low-dose-total-body-irradiation-for-children-and-young-adults-with-acute-myeloid-leukemia-or-myelodysplastic-syndrome-undergoing-allogeneic-hematopoietic-cell-transplantation-a-prospective-phase-ii-trial-of-the-pediatric-blood-and
#18
Eneida R Nemecek, Ralf A Hilger, Alexia Adams, Bronwen E Shaw, Deidre Kiefer, Jennifer Le-Rademacher, John E Levine, Gregory Yanik, Wing Leung, Julie-An Talano, Paul Haut, David Delgado, Neena Kapoor, Aleksandra Petrovic, Roberta Adams, Rabi Hanna, Hemalatha Rangarajan, Jignesh Dalal, Joseph Chewning, Michael R Verneris, Stacy Epstein, Lauri Burroughs, Evelio D Perez-Albuerne, Michael A Pulsipher, Colleen Delaney
This multicenter study evaluated a treosulfan-based regimen in children and young adults with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS) undergoing allogeneic hematopoietic cell transplant (HCT). Forty patients with median age 11 years (1-19) underwent allogeneic HCT for AML in first (n=18), second (n=11), third or greater remission (n=3); or MDS (n=8) using bone marrow (n=25), peripheral blood stem cells (n=5) or cord blood (n=9). The regimen consisted of body surface area (BSA)-based treosulfan 10 g/m2 /day (BSA ≤ 0...
May 9, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29751634/rho-a-regulates-epidermal-growth-factor-induced-human-osteosarcoma-mg63-cell-migration
#19
Jinyang Wang, Lei Zhang, Rongmei Qu, Lin Zhang, Wenhua Huang
Osteosarcoma, the most common primary bone tumor, occurs most frequently in children and adolescents and has a 5-year survival rate, which is unsatisfactory. As epidermal growth factor receptor (EGFR) positively correlates with TNM (tumor-node-metastasis) stage in osteosarcoma, EGFR may play an important role in its progression. The purpose of this study was to explore potential mechanisms underlying this correlation. We found that EGF promotes MG63 cell migration and invasion as well as stress fiber formation via Rho A activation and that these effects can be reversed by inhibiting Rho A expression...
May 11, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29750745/successful-treatment-of-osteosarcoma-without-methotrexate-in-a-13-year-old-boy-with-down-syndrome
#20
Emma Sims, Audrey Nath, Aaron Sugalski
Osteosarcoma is the most common primary bone tumor in children, and only 1 article in the literature describes a case of osteosarcoma in a patient with Down syndrome. Although osteosarcoma is generally treated with chemotherapy regimens that include high-dose methotrexate, patients with Down syndrome have heightened sensitivity to the toxicities of methotrexate. The patient from the aforementioned case study died from sepsis soon after treatment with high-dose methotrexate. This case report describes the successful treatment of osteosarcoma in a pediatric patient with Down syndrome without methotrexate...
May 10, 2018: Journal of Pediatric Hematology/oncology
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