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https://www.readbyqxmd.com/read/28738049/atypical-hemolytic-uremic-syndrome-achieving-positive-patient-outcomes-with-early-diagnosis-and-appropriate-management%C3%A2
#1
Dmitriy Sverdlin, Brenda Peters-Watral
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS), a condition found in adult and pediatric populations, can be idiopathic or acquired as a result of major systemic changes. aHUS presents with a wide array of symptoms that can be attributed to other less dangerous conditions. Because of its complex nature and rare occurrence, it is typically diagnosed in later stages and with multiple organ involvement.
. OBJECTIVES: This article provides an overview of aHUS and available interventions...
August 1, 2017: Clinical Journal of Oncology Nursing
https://www.readbyqxmd.com/read/28738026/effect-of-red-blood-cell-storage-time-on-markers-of-hemolysis-and-inflammation-in-transfused-very-low-birth-weight-infants
#2
Tamara G Kalhan, David A Bateman, Rakhee M Bowker, Eldad A Hod, Sudha Kashyap
BACKGROUND: Prolonged storage of transfused red blood cells (RBCs) is associated with hemolysis in healthy adults and inflammation in animal models. We aimed to determine whether storage duration affects markers of hemolysis (e.g., serum bilirubin, iron, and non-transferrin-bound iron (NTBI)) and inflammation (e.g., interleukin (IL)-8 and monocyte chemoattractant protein (MCP)-1) in transfused very low birth weight (VLBW) infants. METHODS: Blood samples from 23 independent transfusion events were collected by heel stick before and 2-6 h after transfusion...
July 24, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28737867/pediatric-dermatopathology-an-overview
#3
Fatma S Afsar, Gulden Diniz, Safiye Aktas
Dermatopathology is a subspecialty of pathology and dermatology involving correlation of clinical information with microscopic observations of skin biopsies to provide diagnostic information. Pediatric dermatology is a subspecialty of dermatology for which specific points need to be known for evaluating and managing skin disorders in children. The histopathological approach and other important factors for definitive diagnoses in pediatric dermatopathology are reviewed. Skin diseases in children are not necessarily smaller versions of those that develop in adults and some diaseases may be confined to pediatric age group...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28737601/new-ways-to-detect-pediatric-sickle-cell-retinopathy-a-comprehensive-review
#4
Daniel A Pahl, Nancy S Green, Monica Bhatia, Royce W S Chen
Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography...
July 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28737193/-vertebral-fractures-in-children-with-type-i-osteogenesis-imperfecta
#5
Andrea M Sepúlveda, Claudia V Terrazas, Josefina Sáez, María L Reyes
Osteogenesis imperfecta (OI) is an hereditary disease affecting conective tissue, mainly associated to growth retardation and pathological fractures. OI type I (OI type I), is the mildest, most often, and homogeneous in its fenotype. Vertebral fractures are the most significant complications, associated to skeletical and cardiopulmonary morbidity. OBJECTIVES: To characterize clinically a cohort of children with OI type I. PATIENTS AND METHODS: A cohort of OI type I children younger than 20 year old was evaluated...
June 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28733870/rethinking-childhood-ependymoma-a-retrospective-multi-center-analysis-reveals-poor-long-term-overall-survival
#6
Amanda E Marinoff, Clement Ma, Dongjing Guo, Matija Snuderl, Karen D Wright, Peter E Manley, Hasan Al-Sayegh, Claire E Sinai, Nicole J Ullrich, Karen Marcus, Daphne Haas-Kogan, Liliana Goumnerova, Wendy B London, Mark W Kieran, Susan N Chi, Jason Fangusaro, Pratiti Bandopadhayay
Ependymoma is the third most common brain tumor in children, but there is a paucity of large studies with more than 10 years of follow-up examining the long-term survival and recurrence patterns of this disease. We conducted a retrospective chart review of 103 pediatric patients with WHO Grades II/III intracranial ependymoma, who were treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center and Chicago's Ann & Robert H. Lurie Children's Hospital between 1985 and 2008, and an additional 360 ependymoma patients identified from the Surveillance Epidemiology and End Results (SEER) database...
July 21, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28728892/-pediatric-preparations-of-blood-products
#7
C Boulat
The transfusion in pediatrics may require specific preparations to adapt to the various characteristics from preterm neonates to big child: weight, immaturity, immune system, blood volume, long and short-term prognosis, and to meet the needs of pathologies related to the perinatal period. The knowledge of the process for preparation, conservation and administration of these particular products shall permit a transfusion in safe conditions by supporting the prescribers in a personalized blood management.
July 17, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28727973/pediatric-xanthogranulomatous-cystitis-the-first-described-case-in-a-pre-pubertal-male-and-review-of-the-literature
#8
Janae Preece, Vinay Prasad, Daryl McLeod
Xanthogranulomatous cystitis (XC) is a rare condition affecting the bladder thought to be caused by chronic inflammation. Fewer than 30 cases have been reported in the literature since being first described in 1932. We present the first reported case of XC in a pre-pubertal male and review the literature. A 10-year-old boy presenting with dysuria subsequently developed frequency. After failure of conservative management, an ultrasound of the bladder revealed a mass. The patient underwent partial cystectomy...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28727248/vascular-anomaly-cases-for-the-pediatric-hematologist-oncologists-an-interdisciplinary-review
#9
REVIEW
Denise M Adams, Leonardo R Brandão, Caitlin M Peterman, Anita Gupta, Manish Patel, Steven Fishman, Cameron C Trenor
Vascular anomalies (VAs) are classified as tumors or malformations depending on their clinical characteristics, pathological diagnosis, and genomic information. Diagnosis can be challenging because of the heterogeneity of clinical presentation; thus, the best diagnosis and care are provided by an interdisciplinary team of specialists. Over the past 10 years, an increasing number of pediatric hematologist/oncologists are caring for patients with VAs secondary to new medical therapy options and clinical trials...
July 20, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28726587/repair-of-tympanic-membrane-perforations-with-customized-bioprinted-ear-grafts-using-chinchilla-models
#10
Che-Ying Kuo, Emmanuel Wilson, Andrew Fuson, Nidhi Gandhi, Reza Monfaredi, Audrey Jenkins, Maria Romero, Marco Santoro, John P Fisher, Kevin Cleary, Brian Reilly
The goal of this work is to develop an innovative method that combines bioprinting and endoscopic imaging to repair tympanic membrane perforations (TMPs). TMPs are a serious health issue because they can lead to both conductive hearing loss and repeated otitis media. TMPs occur in 3 to 5% of cases after ear tube placement as well as in cases of acute otitis media (the second most common infection in pediatrics), chronic otitis media with or without cholesteatoma, or as a result of barotrauma to the ear. About 55,000 tympanoplasties, the surgery performed to reconstruct TMPs, are performed every year and the commonly used cartilage grafting technique has a success rate between 43% to 100%...
July 20, 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/28726262/double-aortic-arch-anomalies-in-children-a-systematic-20-year-single-center-study
#11
M Kaldararova, I Simkova, I Varga, P Tittel, M Kardos, M Ondriska, V Vrsanska, J Masura
INTRODUCTION: Aortic arch anomalies underlie numerous congenital disorders. Effectively diagnosing and treating them requires close understanding of cardiovascular embryology. As our Center serves the entire pediatric population of our country, we performed a comprehensive retrospective analysis of all aortic arch anomalies diagnosed at our Center over the past 20 years. MATERIALS AND METHODS: We analyzed 40 children with aortic arch anomalies, distinguishing two defect types: Group 1 displayed ring-forming anomalies, and Group 2 other types of aortic arch anomalies that did not form a vascular ring...
July 20, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/28724792/pediatric-and-adult-dilated-cardiomyopathy-represent-distinct-pathological-entities
#12
Meghna D Patel, Jayaram Mohan, Caralin Schneider, Geetika Bajpai, Enkhsaikhan Purevjav, Charles E Canter, Jeffrey Towbin, Andrea Bredemeyer, Kory J Lavine
Pediatric dilated cardiomyopathy (DCM) is the most common indication for heart transplantation in children. Despite similar genetic etiologies, medications routinely used in adult heart failure patients do not improve outcomes in the pediatric population. The mechanistic basis for these observations is unknown. We hypothesized that pediatric and adult DCM comprise distinct pathological entities, in that children do not undergo adverse remodeling, the target of adult heart failure therapies. To test this hypothesis, we examined LV specimens obtained from pediatric and adult donor controls and DCM patients...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28721598/a-retrospective-review-of-34-cases-of-pediatric-pituitary-adenoma
#13
Nannan Zhang, Peizhi Zhou, Yu Meng, Feng Ye, Shu Jiang
PURPOSE: The purpose of this paper is to study invasiveness, tumor features and clinical symptoms of pediatric pituitary adenoma, and to discuss some inconclusive results in prior studies. METHODS: We retrospectively reviewed 34 cases of children (<20 year-old) who were pathologically diagnosed with pituitary adenoma and surgically treated from 2010 to 2017. Data of general information, clinical symptoms, invasive behaviors, surgery approaches, and tumor features were collected and analyzed...
July 18, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28719732/genome-wide-association-meta-analysis-reveals-novel-juvenile-idiopathic-arthritis-susceptibility-loci
#14
Laura A McIntosh, Miranda C Marion, Marc Sudman, Mary E Comeau, Mara L Becker, John F Bohnsack, Tasha E Fingerlin, Thomas A Griffin, J Peter Haas, Daniel J Lovell, Lisa A Maier, Peter A Nigrovic, Sampath Prahalad, Marilynn Punaro, Carlos D Rosé, Carol A Wallace, Carol A Wise, Halima Moncrieffe, Timothy D Howard, Carl D Langefeld, Susan D Thompson
OBJECTIVE: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and has a strong genomic component. To date, JIA genetic association studies have had limited sample sizes, used heterogeneous patient populations, or included only candidate regions. This study focuses on identifying new associations among oligoarticular and polyarticular rheumatoid factor negative (RF(-) ) JIA, which are clinically similar and the most prevalent JIA disease subtypes. METHODS: Three cohorts totaling 2,751 oligoarticular and polyarticular RF(-) JIA cases were genotyped on the Affymetrix Genome-Wide SNP Array 6...
July 18, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28717867/totally-laparoscopic-living-donor-left-hepatectomy-for-liver-transplantation-in-a-child
#15
Helayel Almodhaiberi, Seok-Hwan Kim, Ki-Hun Kim
BACKGROUND: Minimally invasive surgery has been validated to be a new standard in living donor hepatectomy for adult-to-pediatric transplantation with less morbidity [1]. Laparoscopic donor hepatectomy can reduce the major concerns about pain and morbidity associated with open surgery and a slow return to daily activities of donors [2]. Herein, we present one case of totally laparoscopic living donor left hepatectomy including the middle hepatic vein (MHV). DONOR AND METHOD: A 37-year-old mother volunteered to donate to her 3-year-old son with biliary atresia (PELD score 7)...
July 17, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28717752/challenges-of-treating-childhood-medulloblastoma-in-a-country-with-limited-resources-20-years-of-experience-at-a-single-tertiary-center-in-malaysia
#16
Revathi Rajagopal, Sayyidatul Abd-Ghafar, Dharmendra Ganesan, Anita Zarina Bustam Mainudin, Kum Thong Wong, Norlisah Ramli, Vida Jawin, Su Han Lum, Tsiao Yi Yap, Eric Bouffet, Ibrahim Qaddoumi, Shekhar Krishnan, Hany Ariffin, Wan Ariffin Abdullah
PURPOSE: Pediatric medulloblastoma (MB) treatment has evolved over the past few decades; however, treating children in countries with limited resources remains challenging. Until now, the literature regarding childhood MB in Malaysia has been nonexistent. Our objectives were to review the demographics and outcome of pediatric MB treated at the University Malaya Medical Center between January 1994 and December 2013 and describe the challenges encountered. METHODS: Fifty-one patients with childhood MB were seen at University Malaya Medical Center...
April 2017: Journal of Global Oncology
https://www.readbyqxmd.com/read/28717738/collaborative-pediatric-bone-tumor-program-to-improve-access-to-specialized-care-an-initiative-by-the-lebanese-children-s-oncology-group
#17
Raya Saab, Zeina Merabi, Miguel R Abboud, Samar Muwakkit, Peter Noun, Gladys Gemayel, Elie Bechara, Hassan Khalifeh, Roula Farah, Nabil Kabbara, Tarek El-Khoury, Rasha Al-Yousef, Rachid Haidar, Said Saghieh, Toufic Eid, Samir Akel, Nabil Khoury, Layal Bayram, Matthew J Krasin, Sima Jeha, Hassan El-Solh
BACKGROUND: Children with malignant bone tumors have average 5-year survival rates of 60% to 70% with current multimodality therapy. Local control modalities aimed at preserving function greatly influence the quality of life of long-term survivors. In developing countries, the limited availability of multidisciplinary care and limited expertise in specialized surgery and pediatric radiation therapy, as well as financial cost, all form barriers to achieving optimal outcomes in this population...
February 2017: Journal of Global Oncology
https://www.readbyqxmd.com/read/28715257/association-between-testicular-microlithiasis-and-testicular-neoplasia-large-multicenter-study-in-a-pediatric-population
#18
Andrew T Trout, Jeanne Chow, Erin R McNamara, Kassa Darge, Raul Ramirez Grueso, Marthe Munden, Sarah M Rothan, Oscar M Navarro, Marta Tijerín Bueno, Kevin E Bove, Kudakwashe R Chikwava, Amer Heider, M John Hicks, Gino R Somers, Bin Zhang, Jonathan R Dillman
Purpose To retrospectively define the strength of association between testicular microlithiasis and testicular neoplasia in a large geographically diverse pediatric population. Materials and Methods Retrospective review of scrotal ultrasonographic (US) examination reports and pathology specimens obtained between January 2000 and May 2014 at six academic pediatric hospitals in North America was performed. Reported cases were reviewed to confirm microlithiasis. Radiology and pathology data bases were searched for pathology-proven testicular tumors (benign or malignant germ cell or stromal tumors)...
July 17, 2017: Radiology
https://www.readbyqxmd.com/read/28714114/split-liver-transplantation-for-retroperitoneal-immature-teratoma-masquerading-as-hepatoblastoma
#19
Dongkyu Oh, Nam-Joon Yi, Sanghee Song, Ok Kyung Kim, Suk Kyun Hong, Kyung Chul Yoon, Sung Woo Ahn, Hyo-Sin Kim, Hyeyoung Kim, Hyun-Young Kim, Hyoung Jin Kang, Myungsu Lee, Kyoung Bun Lee, Kwang-Woong Lee, Kyung Suk Suh
Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features...
July 16, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28711168/surgical-treatment-of-intestinal-complications-of-graft-versus-host-disease-in-the-pediatric-population-case-series-and-review-of-literature
#20
REVIEW
Camille A Gutierrez, Mehul V Raval, Hannah R Vester, Sonali Chaudhury, Daniel von Allmen, David H Rothstein
BACKGROUND/PURPOSE: Intestinal complications of acute graft-versus-host disease (aGVHD) include hemorrhage and perforation in the short-term, and stricture with bowel obstruction in the long-term. As medical management of severe aGVHD has improved, more patients are surviving even advanced stages of intestinal aGVHD. This review summarizes the available pediatric literature on surgical treatment of complications of intestinal GVHD. METHODS: A systematic review was performed using PubMed, Cochrane, Embase, and Scopus databases...
June 28, 2017: Journal of Pediatric Surgery
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