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Pediatric Syndromes

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https://www.readbyqxmd.com/read/28821624/a-sweet-case-of-mycoplasma
#1
Jackie Hsieh, Ali Yalcindag, Daniel T Coghlin
Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory disorder marked by fever and swelling of the skin that can be very painful. It is especially rare in the pediatric population. Infection is a well-known trigger for Sweet syndrome, but this entity has, to our knowledge, never been described after Mycoplasma infection. Herein, we describe the first pediatric case of febrile neutrophilic dermatosis associated with Mycoplasma infection.
August 18, 2017: Pediatrics
https://www.readbyqxmd.com/read/28820871/genetic-predisposition-to-fetal-alcohol-syndrome-association-with-congenital-disorders-of-n-glycosylation
#2
María E de la Morena-Barrio, María J Ballesta-Martínez, Raquel López-Gálvez, Ana I Antón, Vanessa López-González, Laia Martínez-Ribot, José Padilla, Antonia Miñano, Oscar García-Algar, Miguel Del Campo, Javier Corral, Encarna Guillén-Navarro, Vicente Vicente
BACKGROUND: Fetal alcohol syndrome (FAS) is caused by maternal alcohol consumption during pregnancy, although additional factors must be involved, as development and severity are not directly related to alcohol intake. The abnormal glycosylation caused by alcohol might play a role in FAS according to the clinical similarities shared with congenital disorders of glycosylation (CDG). Thus, mutations underlying CDG, affecting genes involved in glycosylation, could also be involved in FAS...
August 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28820041/a-report-of-7-year-experience-on-pediatric-continuous-renal-replacement-therapy
#3
Ayse Filiz Yetimakman, Selman Kesici, Murat Tanyildiz, Umut Selda Bayrakci, Benan Bayrakci
BACKGROUND: Continuous renal replacement therapies (CRRTs) either as continuous venovenous hemofiltration (CVVH) or hemodiafiltration (CVVHD) are used frequently in critically ill children. Many clinical variables and technical issues are known to affect the result. The factors that could be modified to increase the survival of renal replacement are sought. As a contribution, we present the data on 104 patients who underwent CRRT within a 7-year period. MATERIALS AND METHOD: A total of 104 patients admitted between 2009 and 2016 were included in the study...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28819780/preliminary-research-on-syndrome-types-of-chinese-medicine-in-children-with-primary-nephrotic-syndrome
#4
Wen Sun, Jian Yu, Gu-Lan Zeng, Bing-Feng Zhang
OBJECTIVE: To provide an objective reference for the syndrome types of Chinese medicine (CM) associated with pediatric primary nephrotic syndrome (PNS). METHODS: A cross-sectional study was performed. Data on clinical symptoms, CM syndrome types, biochemical indices, and medications used were collected from 98 children with PNS. Then, the correlation between CM syndromes and biochemical indices, as well as medications used, was analyzed. RESULTS: The four most common symptoms in children with PNS were brown urine, red tongue, excessive sweating, and swelling of the face and limbs...
August 17, 2017: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/28818172/clinical-profile-and-short-term-outcome-of-pediatric-hyperleukocytic-acute-leukemia-from-a-developing-country
#5
Syed Ali Shazif Baqari, Anwarul Haque, Muhammad Shamvil Ashraf, Muhammad Matloob Alam, Zehra Fadoo
This study was conducted to determine the frequency, clinical profile, and short-term outcome of children with hyperleukocytosis at two pediatric oncology centers in Karachi. Of a total 1,045 patients, 13.97% (n=146) patients had hyperleukocytosis. Majority (61.7%, n=90) were under 10 years of age and 76% (n=146) were male. The symptom duration before diagnosis was more than 30 days in 49.3% (n=72). The median WBC count was 181 x109/L(IQR=130.45298.3) and extreme hyperleukocytosis (>200 x109/L) was observed in 44...
July 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28817819/progressive-kyphosis-associated-with-tethered-cord-syndrome-treated-by-posterior-vertebral-column-resection-in-a-pediatric-patient
#6
Kadir Oktay, Kerem Mazhar Ozsoy, Yurdal Gezercan, Nuri Eralp Cetinalp, Tahsin Erman
Posterior vertebral column resection is a novel surgical approach for the treatment of progressive kyphosis associated with tethered cord syndrome that was first treated with untethering surgery. A patient with tethered cord syndrome associated with kyphosis first underwent untethering surgery, resulting in progressive kyphosis. Posterior vertebral column resection was performed to correct the kyphosis while shortening the spinal column to prevent the spinal cord from stretch injury. Good correction of kyphosis and reduction of tension on the neural elements were achieved without any neurological deficits...
August 18, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28817534/radius-regeneration-after-open-fracture-and-extrusion-a-case-report
#7
Kristopher B McCall, David Y Chong
A pediatric patient presenting with an open forearm fracture with segmental defect is rare, and the pediatric orthopedic literature to guide treatment is sparse. There are several described methods to treat the defect, including bone grafting and bone transport techniques. In addition, there are reports of fibular regeneration after resection if the periosteum is left intact. We present a case report of complete regeneration of over half of a pediatric radial shaft with an intact periosteum, after traumatic extrusion from an open fracture...
August 16, 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/28816920/similar-metabolic-innate-immunity-and-adipokine-profiles-in-adult-and-pediatric-sepsis-versus-systemic-inflammatory-response-syndrome-a-pilot-study
#8
Theonymfi Tavladaki, Anna Maria Spanaki, Helen Dimitriou, Efmorfia Kondili, Christianna Choulaki, Dimitris Georgopoulos, George Briassoulis
OBJECTIVES: To examine whether the septic profiles of heat shock protein 72, heat shock protein 90α, resistin, adiponectin, oxygen consumption, CO2 production, energy expenditure, and metabolic pattern, along with illness severity, nutritional, and inflammatory indices, differ between adult and pediatric patients compared with systemic inflammatory response syndrome and healthy controls. To evaluate whether these biomolecules may discriminate sepsis from systemic inflammatory response syndrome in adult and pediatric patients...
August 12, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28816870/spinal-cord-injury-what-are-the-controversies
#9
Christopher S Ahuja, Gregory D Schroeder, Alexander R Vaccaro, Michael G Fehlings
Traumatic spinal cord injuries have a tremendous impact on individuals, families, and society as a whole. Substantial heterogeneity in the patient population, their presentation and underlying pathophysiology has sparked debates along the care spectrum from initial assessment to definitive treatment. This article reviews spinal cord injury (SCI) management followed by a discussion of the salient controversies in the field. Current care practices modeled on the American Association of Neurological Surgeons/Congress of Neurological Surgeons joint section guidelines are highlighted including key recommendations regarding immobilization, avoidance of hypotension, early International Standards for Neurological Classification of SCI examination and intensive care unit treatment...
September 2017: Journal of Orthopaedic Trauma
https://www.readbyqxmd.com/read/28815624/morbidity-and-mortality-in-children-undergoing-bronchoscopy-for-foreign-body-removal
#10
Christopher A Roberts, Michele M Carr
OBJECTIVES/HYPOTHESIS: Analyze morbidity and mortality among children undergoing bronchoscopy for foreign body removal. STUDY DESIGN: Multicenter retrospective review using the American College of Surgeons Pediatric National Surgical Quality Improvement Program from 2014 and 2015. METHODS: Patients were identified using Current Procedural Terminology code 31635. Demographics, time to surgery, operative times, hospitalization time, and complications were collected...
August 17, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28814627/prematurity-and-sudden-unexpected-infant-deaths-in-the-united-states
#11
Barbara M Ostfeld, Ofira Schwartz-Soicher, Nancy E Reichman, Julien O Teitler, Thomas Hegyi
BACKGROUND AND OBJECTIVES: Prematurity, a strong risk factor for sudden unexpected infant death (SUID), was addressed in recommendations by the American Academy of Pediatrics in 2011 for safe sleep education in NICUs. We documented associations between gestational age (GA) and SUID subsequent to these guidelines. METHODS: Using the 2012-2013 US linked infant birth and death certificate period files, we documented rates per live births of sudden infant death syndrome, ill-defined and unspecified causes, accidental suffocation and strangulation in bed, and overall SUID by GA in postneonatal, out-of-hospital, and autopsied cases; compared survivors and cases; and estimated logistic regression models of associations between GA and SUID...
June 5, 2017: Pediatrics
https://www.readbyqxmd.com/read/28814538/implications-of-the-fmr1-premutation-for-children-adolescents-adults-and-their-families
#12
Anne Wheeler, Melissa Raspa, Randi Hagerman, Marsha Mailick, Catharine Riley
BACKGROUND AND OBJECTIVES: Given the nature of FMR1 gene expansions, most biological mothers, and often multiple other family members of children with fragile X syndrome (FXS), will have a premutation, which may increase individual and family vulnerabilities. This article summarizes important gaps in knowledge and notes potential implications for pediatric providers with regard to developmental and medical risks for children and adolescents with an FMR1 premutation, including possible implications into adulthood...
June 2017: Pediatrics
https://www.readbyqxmd.com/read/28811575/biological-and-functional-characterization-of-bone-marrow-derived-mesenchymal-stromal-cells-from-patients-affected-by-primary-immunodeficiency
#13
Nadia Starc, Daniela Ingo, Antonella Conforti, Valeria Rossella, Luigi Tomao, Angela Pitisci, Fabiola De Mattia, Immacolata Brigida, Mattia Algeri, Mauro Montanari, Giuseppe Palumbo, Pietro Merli, Paolo Rossi, Alessandro Aiuti, Franco Locatelli, Maria Ester Bernardo
Mesenchymal stromal cells (MSCs) represent a key component of bone marrow (BM) microenvironment and display immune-regulatory properties. We performed a detailed analysis of biological/functional properties of BM-MSCs derived from 33 pediatric patients affected by primary immune-deficiencies (PID-MSCs): 7 Chronic Granulomatous Disease (CGD), 15 Wiskott-Aldrich Syndrome (WAS), 11 Severe Combined Immunodeficiency (SCID). Results were compared with MSCs from 15 age-matched pediatric healthy-donors (HD-MSCs). Clonogenic and proliferative capacity, differentiation ability, immunophenotype, immunomodulatory properties were analyzed...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811041/post-thrombotic-syndrome-in-children
#14
Marisol Betensky, Neil A Goldenberg
The post-thrombotic syndrome (PTS) is the most common long-term complication of pediatric deep venous thrombosis (DVT). It is a burdensome condition that can lead to severe disability and poor quality-of-life of affected children. Although its pathophysiology remains poorly understood, it is thought to be the result of chronic venous hypertension. Recent studies have shown that the inflammatory response associated with an acute DVT likely plays a key role in the development of PTS. The Manco-Johnson Instrument and the modified Villalta Scale are the most widely used instruments for the diagnosis of pediatric PTS...
July 24, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28808789/palliative-care-for-children-with-a-yet-undiagnosed-syndrome
#15
Jessica I Hoell, Jens Warfsmann, Gabriele Gagnon, Laura Trocan, Stefan Balzer, Prasad T Oommen, Arndt Borkhardt, Gisela Janßen, Michaela Kuhlen
The number of children without a diagnosis in pediatric palliative home care and the process of decision-making in these children are widely unknown. The study was conducted as single-center retrospective cohort study. Between January 2013 and September 2016, 198 children and young adults were cared for; 27 (13.6%) of these were without a clear diagnosis at the start of pediatric palliative home care. A definite diagnosis was ultimately achieved in three children. Median age was 7 years (0-25), duration of care 569 days (2-2638), and number of home visits 7...
August 14, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28808787/malignant-transformation-of-a-conservatively-managed-incidental-childhood-cerebral-mass-lesion-controversy-regarding-management-paradigm
#16
Jehuda Soleman, Jonathan Roth, Zvi Ram, Michal Yalon, Shlomi Constantini
BACKGROUND: Incidental findings on neuroimaging in the pediatric population are an emerging treatment challenge. Treatment options for these incidental childhood brain mass lesions, which radiologically may be assumed to be low-grade gliomas (LGG), vary, ranging from careful conservative "wait and scan" treatment to surgical biopsy, gross total resection, and upfront radiation and/or chemotherapy. As malignant transformation of LGG in children is extremely rare, some series advocate careful conservative management of these lesions; however, universal treatment protocols are not totally agreed upon...
August 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28807357/development-and-initial-validation-of-the-macrophage-activation-syndrome-primary-hemophagocytic-lymphohistiocytosis-score-a-diagnostic-tool-that-differentiates-primary-hemophagocytic-lymphohistiocytosis-from-macrophage-activation-syndrome
#17
Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli, AnnaCarin Horne
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample...
August 11, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28806468/an-overview-of-hemophagocytic-lymphohistiocytosis
#18
Ysabella M Esteban, Jill L O de Jong, Melissa S Tesher
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases...
August 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28806467/the-history-of-home-cardiorespiratory-monitoring
#19
Gary E Freed, Francis Martinez
Home cardiorespiratory monitoring has changed significantly since it was first introduced in the 1970s. It has improved from a simple alarm system to a sophisticated piece of equipment capable of monitoring the patient's electrocardiogram, respiratory effort, and oxygen saturations. In addition, the indications for using a monitor have also changed. The home monitor was initially used to reduce the incidence of sudden infant death syndrome (SIDS). Although there were several studies demonstrating the reduction of SIDS rates in communities where apnea programs existed, none was a prospective, double-blinded study or had adequate numbers to be clinically significant...
August 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28806464/beyond-back-to-sleep-ways-to-further-reduce-the-risk-of-sudden-infant-death-syndrome
#20
Fern R Hauck, Kawai O Tanabe
Sudden infant death syndrome (SIDS) remains the leading cause of postneonatal mortality in the United States, despite reduction in rates of more than 50% since the initiation of the "Back to Sleep" (now called "Safe to Sleep") campaign in 1994. In recent years, the rate of decline in SIDS deaths has plateaued, even with the ongoing educational efforts that promote safe sleep and other risk reduction measures. The 2016 American Academy of Pediatrics guidelines for reducing the risk of SIDS focus heavily on sleep practices, bedding, and location, but also include factors that often receive less attention (ie, prenatal care, maternal smoking, alcohol and drug use, and childhood vaccinations)...
August 1, 2017: Pediatric Annals
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