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Pediatric pathology

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https://www.readbyqxmd.com/read/27926496/the-therapeutic-potential-of-targeting-the-pi3k-pathway-in-pediatric-brain-tumors
#1
REVIEW
Hazel A Rogers, Jasper Estranero, Keshni Gudka, Richard G Grundy
Central nervous system tumors are the most common cancer type in children and the leading cause of cancer related deaths. There is therefore a need to develop novel treatments. Large scale profiling studies have begun to identify alterations that could be targeted therapeutically, including the phosphoinositide 3-kinase (PI3K) signaling pathway, which is one of the most commonly activated pathways in cancer with many inhibitors under clinical development. PI3K signaling has been shown to be aberrantly activated in many pediatric CNS neoplasms...
December 2, 2016: Oncotarget
https://www.readbyqxmd.com/read/27924361/prevalence-of-non-cardiovascular-findings-on-ct-angiography-in-children-with-congenital-heart-disease
#2
Archana Malik, Jeffrey C Hellinger, Sabah Servaes, Mathew C Schwartz, Marc S Keller, Monica Epelman
BACKGROUND: CT angiography is gaining broader acceptance in the evaluation of children with known or suspected congenital heart disease. These studies include non-cardiovascular structures such as the mediastinum, lung parenchyma and upper abdominal organs. It is important to inspect all these structures for potential abnormalities that might be clinically important and, in some cases, may impact care plans. OBJECTIVE: To determine the prevalence of non-cardiovascular findings in CT angiography of children with congenital heart disease...
December 6, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27911891/severe-carbamazepine-intoxication-in-children-analysis-of-a-40-case-series
#3
Mehmet Acikgoz, M Sukru Paksu, Ahmet Guzel, Abdurrahman Alacam, Fatma Alacam
BACKGROUND We compared the factors that might impact the severity and the prognosis of carbamazepine (CBZ) intoxication in children, as well as the efficacy levels of the treatment options. MATERIAL AND METHODS Demographic information and clinical and laboratory findings for 40 patients were evaluated retrospectively. Predictive parameters for the development of serious complications were studied. RESULTS Median age of patients was 14 years; 65% of the patients were female. The most common pathological clinical finding and laboratory abnormality were inability to awaken the patient and hyperglycemia (45% and 60%, respectively)...
December 2, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/27911767/phosphatidylinositol-3-kinase-inhibition-restores-ca2-release-defects-and-prolongs-survival-in-myotubularin-deficient-mice
#4
Candice Kutchukian, Mirella Lo Scrudato, Yves Tourneur, Karine Poulard, Alban Vignaud, Christine Berthier, Bruno Allard, Michael W Lawlor, Ana Buj-Bello, Vincent Jacquemond
Mutations in the gene encoding the phosphoinositide 3-phosphatase myotubularin (MTM1) are responsible for a pediatric disease of skeletal muscle named myotubular myopathy (XLMTM). Muscle fibers from MTM1-deficient mice present defects in excitation-contraction (EC) coupling likely responsible for the disease-associated fatal muscle weakness. However, the mechanism leading to EC coupling failure remains unclear. During normal skeletal muscle EC coupling, transverse (t) tubule depolarization triggers sarcoplasmic reticulum (SR) Ca(2+) release through ryanodine receptor channels gated by conformational coupling with the t-tubule voltage-sensing dihydropyridine receptors...
November 28, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27911069/-urological-pathology-of-lymphatic-origin
#5
A Domènech, A Serrano, I Forner-Cordero, F Gómez, D Maldonado, C Domínguez
OBJECTIVES: Lymphatic disease is a rarely cause of some very unspecific genitourinary manifestations, assuming a diagnostic challenge in most cases. The aim of this paper is to warn about the possible etiology of these urological lymphatic presentations and discuss its management. METHODS: Retrospective review of clinical data in pediatric patients with urological pathology of lymphatic origin between 2008-2014. Three patients, two boys and a girl, were included...
January 25, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27904844/cardiothoracic-surgical-experience-in-ghana
#6
REVIEW
Mark Tettey, Martin Tamatey, Frank Edwin
Ghana is one of the few low-to-middle-income countries in sub-Saharan Africa able to consistently sustain a cardiothoracic program with locally trained staff for more than two decades. Cardiothoracic surgery practice in Ghana started in 1964 but faltered from a combination of political and the economic problems. In 1989, Dr. Kwabena Frimpong-Boateng, a Ghanaian cardiothoracic surgeon trained in Hannover, rekindled interest in cardiothoracic surgery and in establishing a National Cardiothoracic Centre. His vision and leadership has brought cardiothoracic surgery practice in Ghana to its current high level...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27903347/-clinical-genetic-and-pathological-features-of-neuronal-ceroid-lipofuscinosis-in-5-chinese-patients
#7
S C Ren, B Q Gao, Y J Wang, X J Wu, Z X Tian, Y L Sun
Objective: To report the clinical, genetic and ultrastructural pathological features of neuronal ceroid lipofuscinosis (NCLs) in 5 Chinese patients. Methods: A total of 5 patients with NCLs were collected from 2013 to 2015 diagnosed by the department of pediatrics of Beijing Tian Tan Hospital. Their clinical, electrophysiological and neuroimaging data of the patients were reviewed.A total of 9 underlying genes of NCLs were tested in 4 cases and their parents.Ultrastructural pathology by skin biopsy was performed in 3 cases respectively...
November 22, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27900491/treatable-renal-disease-in-children-with-silent-lupus-nephritis-detected-by-baseline-biopsy-association-with-serum-c3-levels
#8
Hiroyuki Wakiguchi, Syuji Takei, Tomohiro Kubota, Akinori Miyazono, Yoshifumi Kawano
Lupus nephritis is identified in up to 75% of patients with juvenile systemic lupus erythematosus and may present with abnormal urinary findings (overt lupus nephritis) or be apparent only upon renal biopsy (silent lupus nephritis). We investigated whether serum complement levels correlate with renal pathology in pediatric patients with silent lupus nephritis. We performed baseline renal biopsy in 45 children diagnosed with juvenile systemic lupus erythematosus who were admitted to Kagoshima University Hospital between January 2000 and June 2015...
November 30, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27897381/pathologic-fracture-in-childhood-and-adolescent-osteosarcoma-a-single-institution-experience
#9
Lindsay Haynes, Sue C Kaste, Kirsten K Ness, Jianrong Wu, Lucia Ortega-Laureano, Michael Bishop, Michael Neel, Bhaskar Rao, Israel Fernandez-Pineda
PURPOSE: Pathologic fractures occur in 5-10% of pediatric osteosarcoma (OS) cases and have historically been considered a contraindication to limb salvage. Our purpose was to describe the radiographic features of pathologic fracture and examine its impact on local recurrence rates, functional outcomes, and overall survival. METHODS: We retrospectively analyzed patients at our institution from 1990 to 2015 with pathologic fracture at diagnosis or during neoadjuvant chemotherapy...
November 5, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#10
Yi Zhang, Weiling Zhang, Suoqin Tang, Liping Chen, You Yi, Pinwei Zhang, Aiping Liu, Tian Zhi, Dongsheng Huang
Hepatoblastoma is a malignant liver tumor generally diagnosed in infants and children <3 years old. The current retrospective study aimed to investigate the associations of tumor stage, pathological type, metastasis and chemotherapy with clinical outcomes. In the current study, a total of 102 patients with hepatoblastoma were enrolled between September 2006 and June 2014. Clinical records and follow-up information for each of patient were obtained to conduct a Kaplan-Meier survival analysis and log-rank test...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27894766/risk-and-relevance-of-open-lung-biopsy-in-pediatric-ecmo-patients-the-dutch-experience
#11
Robert Jan Houmes, Chantal A Ten Kate, Enno D Wildschut, Rob M Verdijk, René M H Wijnen, Ivo de Blaauw, Dick Tibboel, Arno F van Heijst
BACKGROUND: Open lung biopsy can help differentiate between reversible and irreversible lung disease and may guide therapy. To assess the risk-benefit ratio of this procedure in pediatric extracorporeal membrane oxygenation (ECMO) patients, we reviewed data of all patients who underwent an open lung biopsy during ECMO in one of the two pediatric ECMO centers in a nationwide study in the Netherlands. RESULTS: In nineteen neonatal and six pediatric patients (0-15...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27894445/usual-and-unusual-manifestations-of-systemic-and-central-nervous-system-vasculitis
#12
REVIEW
James J Nocton
The idiopathic vasculitides are a group of inflammatory and immune-mediated conditions associated with inflammation of blood vessels. They affect multiple organ and body systems, and vary in their clinical manifestations, severity, prognosis, and pathology. They frequently present a diagnostic challenge for clinicians because of their complexity, overlapping features, and similar findings to other noninflammatory, genetic, or infectious conditions. This article summarizes some of the common pediatric vasculitides, emphasizing both the characteristic and unusual clinical manifestations of these diseases...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27894435/pediatric-appendicitis
#13
REVIEW
Rebecca M Rentea, Shawn D St Peter
Appendicitis is one of the most common surgical pathologies in children. It can present with right lower quadrant pain. Scoring systems in combination with selective imaging and surgical examination will diagnose most children with appendicitis. Clinical pathways should be used. Most surgical interventions for appendicitis are now almost exclusively laparoscopic, with trials demonstrating better outcomes for children who undergo index hospitalization appendectomies when perforated. Nonoperative management has a role in the treatment of both uncomplicated and complicated appendicitis...
February 2017: Surgical Clinics of North America
https://www.readbyqxmd.com/read/27893197/importance-to-question-sinonasal-symptoms-and-to-perform-rhinoscopy-and-rhinomanometry-in-cystic-fibrosis-patients
#14
J M Bock, M Schien, C Fischer, L Naehrlich, M Kaeding, O Guntinas-Lichius, A Gerber, C Arnold, J G Mainz
OBJECTIVES: Cystic fibrosis (CF) patients almost regularly reveal sinonasal pathology. The purpose of this study was to assess association between objective and subjective measurements of sinonasal involvement comparing nasal airflow obtained by active anterior rhinomanometry (AAR), nasal endoscopic findings, and symptoms assessed with the Sino-Nasal Outcome Test-20 (SNOT-20). METHODS: Nasal cavities were explored by anterior rigid rhinoscopy and findings were compared to inspiratory nasal airflow measured by AAR to quantify nasal patency and subjective health-related quality of life in sinonasal disease obtained with the SNOT-20 questionnaire...
November 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27891787/evaluation-of-the-western-australian-register-of-developmental-anomalies-thirty-five-years-of-surveillance
#15
Wendy N Nembhard, Carol Bower
BACKGROUND: The birth defects component of the Western Australian Register for Developmental Anomalies (WARDA-BD) was evaluated to assess its efficiency, effectiveness, and data quality. METHODS: WARDA-BD was evaluated using the Centers for Disease Control and Prevention Guidelines for Evaluating Public Health Surveillance Systems and Data Quality Standards from the National Birth Defects Prevention Network. The evaluation included interviews with Register staff, local community organizations, parents, clinicians, and researchers; process observation; and secondary data analyses...
November 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
https://www.readbyqxmd.com/read/27891487/a-rare-case-of-pyogenic-granuloma-with-a-natal-tooth
#16
Nidhi Agarwal, Dipanshu Kumar, Alaka Vaish, Ashish Anand
Natal teeth are teeth present in the oral cavity at the time of birth. It is extremely rare to find natal teeth in association with pathological conditions of the oral cavity. Pyogenic granuloma is a type of inflammatory hyperplasia that appears as an over exuberant connective tissue response to a stimulus or injury, in the present case the injurious agent is the natal tooth. The parents of the eight day old male infant brought the child with a natal tooth associated with a soft tissue lesion growing from the gum pad...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27891279/multiple-gastric-erosion-early-after-a-3%C3%A2-v-lithium-battery-cr2025-ingestion-in-an-18-month-old-male-patient-consideration-about-the-proper-time-of-intervention
#17
Ioannis Patoulias, Christos Kaselas, Dimitrios Patoulias, Konstantinos Farmakis, Eleni Papacrivou, Maria Kalogirou, Thomas Feidantsis
Introduction. Button battery ingestion is considered an emergency situation in pediatric patients that needs to be managed promptly; otherwise, it may lead to serious and potentially lethal complications, especially when it is impacted in the esophagus. Less attention has been given in cases where the battery passes into the stomach, with guidelines for emergency intervention being based on the presence of symptoms. Case Report. We present a case of an 18-month-old male patient who presented to our emergency department after button battery ingestion...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27890780/a-clinical-score-to-predict-appendicitis-in-older-male-children
#18
Anupam B Kharbanda, Michael C Monuteaux, Richard G Bachur, Nanette C Dudley, Lalit Bajaj, Michelle D Stevenson, Charles G Macias, Manoj K Mittal, Jonathan E Bennett, Kelly Sinclair, Peter S Dayan
OBJECTIVE: To develop a clinical score to predict appendicitis among older, male children who present to the emergency department (ED) with suspected appendicitis. METHODS: Patients with suspected appendicitis were prospectively recruited at 9 pediatric EDs. A total 2,625 patients enrolled; a subset of 961 males, age 8-18 were analyzed in this secondary analysis. Outcomes determined by pathology, operative reports and follow-up calls. Clinical and laboratory predictors with < 10% missing data and Kappa > 0...
November 24, 2016: Academic Pediatrics
https://www.readbyqxmd.com/read/27890307/-french-society-of-pediatrics-forensic-pathology-speciality-s-union-to-protect-minors
#19
M Balençon, C Garnier-Jardin, M Lemesle, A P Michard Lenoir, F Paysant, C Rambaud, C Rey-Salmon, V Scolan, B Tisseron, N Vabres
No abstract text is available yet for this article.
December 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27889838/changes-of-brain-metabolite-concentrations-during-maturation-in-different-brain-regions-measured-by-chemical-shift-imaging
#20
Eva Bültmann, Thomas Nägele, Heinrich Lanfermann, Uwe Klose
INTRODUCTION: We examined the effect of maturation on the regional distribution of brain metabolite concentrations using multivoxel chemical shift imaging. METHODS: From our pool of pediatric MRI examinations, we retrospectively selected patients showing a normal cerebral MRI scan or no pathologic signal abnormalities at the level of the two-dimensional 1H MRS-CSI sequence and an age-appropriate global neurological development, except for focal neurological deficits...
November 26, 2016: Neuroradiology
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