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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28737601/new-ways-to-detect-pediatric-sickle-cell-retinopathy-a-comprehensive-review
#1
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/28730452/transcranial-color-doppler-in-stroke-free-adult-patients-with-sickle-cell-disease
#2
G Graziadei, F M Casoni, F Annoni, I Cortinovis, P Ridolfi, I Gandolfi, A Marcon, E Di Pierro, M D Cappellini
The threshold velocity ≥200 cm/s at transcranial Doppler (TCD) evaluation is a useful cut-off for preventing the stroke (STOP trial) in pediatric patients with sickle cell disease (SCD), term including different types of sickle genotypes. Scanty data are available for adult SCD patients. We compared intracranial blood flow velocities between adult SCD patients and controls using transcranial color Doppler (TCCD), measuring the peak of systolic velocity (PSV) with the insonation angle correction and the pulsatility index (PI), an indicator of endothelial elasticity...
July 20, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28698782/prevalence-and-etiology-of-bacteremia-in-febrile-children-with-sickle-cell-disease-at-a-nigeria-tertiary-hospital
#3
Biobele Brown, Hannah Dada-Adegbola, Catherine Trippe, Olufunmilayo Olopade
BACKGROUND & OBJECTIVES: As a result of immune defects in Sickle cell disease (SCD), affected individuals are prone to infection from encapsulated bacterial pathogens like Streptococcus Pneumoniae. Studies on the etiological agents of bacteremia in children with SCD in Nigeria are few and have revealed a spectrum of organisms that is different from those recorded in other parts of the world. AIM AND OBJECTIVES: The objectives of this study were to determine the prevalence of bacteremia, etiological agents and antibiotic susceptibility pattern in febrile children with SCD attending the University College Hospital (UCH), Ibadan, Nigeria...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28698351/subdissociative-intranasal-ketamine-plus-standard-pain-therapy-versus-standard-pain-therapy-in-the-treatment-of-paediatric-sickle-cell-disease-vaso-occlusive-crises-in-resource-limited-settings-study-protocol-for-a-randomised-controlled-trial
#4
James R Young, Hendry Robert Sawe, Juma A Mfinanga, Ernest Nshom, Ethan Helm, Charity G Moore, Michael S Runyon, Stacy L Reynolds
INTRODUCTION: Pediatric sickle cell disease, highly prevalent in sub-Saharan Africa, carries great morbidity and mortality risk. Limited resources and monitoring make management of acute vaso-occlusive crises challenging. This study aims to evaluate the efficacy and safety of subdissociative intranasal ketamine as a cheap, readily available and easily administered adjunct to standard pain therapy. We hypothesise that subdissociative, intranasal ketamine may significantly augment current approaches to pain management in resource-limited settings in a safe and cost-effective manner...
July 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28697028/sciatic-popliteal-fossa-catheter-for-pediatric-pain-management-of-sickle-cell-crisis-a-case-report
#5
Garret Weber, Sherry Liao, Micah Alexander Burns
Sickle cell crisis, or vaso-occlusive crisis (VOC), is a major cause of hospitalizations for adults and children with sickle cell disease, and is associated with increased morbidity and mortality. Despite prompt pharmacological treatment and multimodal pain management, acute pain during a VOC is often not adequately controlled in the pediatric population. We placed a continuous popliteal sciatic nerve block under ultrasound guidance in a pediatric patient for localized refractory pain during a VOC, resulting in improved pain control with preserved sensorimotor function...
July 10, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28689245/imaging-of-renal-medullary-carcinoma-in-children-and-young-adults-a-report-from-the-children-s-oncology-group
#6
Jesse K Sandberg, Elizabeth A Mullen, Mariana M Cajaiba, Ethan A Smith, Sabah Servaes, Elizabeth J Perlman, James I Geller, Peter F Ehrlich, Nicholas G Cost, Jeffrey S Dome, Conrad V Fernandez, Geetika Khanna
BACKGROUND: Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children. OBJECTIVE: To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation. MATERIALS AND METHODS: We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children's Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016...
July 8, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28686127/effectiveness-of-surgical-revascularization-for-stroke-prevention-in-pediatric-patients-with-sickle-cell-disease-and-moyamoya-syndrome
#7
Wuyang Yang, Risheng Xu, Jose L Porras, Clifford M Takemoto, Syed Khalid, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander L Coon, Rafael J Tamargo, Judy Huang, Edward S Ahn
OBJECTIVE Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors' aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy. METHODS The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013...
July 7, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28677113/plasma-chitotriosidase-and-carotid-intima-media-thickness-in-children-with-sickle-cell-disease
#8
Normeen A Kaddah, Dalia A Saied, Hanan A Alwakeel, Rania H Hashem, Sara M Rowizak, Mohamed A Elmonem
The relationship between chronic hemolysis with subsequent iron overload, inflammation, and premature atherosclerosis has been documented in hemolytic anemias, particularly β-thalassemia. However, no such relationship has been established in sickle cell disease (SCD). We sought to evaluate SCD as a risk factor for early vascular insult by measuring carotid intima-media thickness (CIMT) and plasma chitotriosidase and to assess the role of the latter as a potential quantitative indicator of vascular inflammation and atherogenesis...
July 4, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28670947/genetic-background-of-the-sickle-cell-disease-pediatric-population-of-dakar-senegal-and-characterization-of-a-novel-frameshift-%C3%AE-thalassemia-mutation-hbb-c-265_266del-p-leu89glufs-2
#9
Fatou Gueye Tall, Cyril Martin, El Hadji Malick Ndour, Indou Déme Ly, Céline Renoux, Louis Chillotti, Nicolas Veyrenche, Philippe Connes, Papa Madieye Gueye, Rokhaya Ndiaye Diallo, Philippe Lacan, Ibrahima Diagne, Pape Amadou Diop, Aynina Cissé, Philomène Lopez Sall, Philippe Joly
Sickle cell disease is a genetic disorder with a large variability in the pattern and severity of clinical manifestations. Different genetic modulators have been identified but very few epidemiologic data are available on these modifier genes in Senegal. This study aimed to determine their prevalence in a Senegalese sickle cell disease pediatric population. The following genetic parameters were genotyped in 295 sickle cell disease children of the Dakar pediatric hospital: sickle cell disease genotype [β(S)/β(S) (HBB: c...
March 2017: Hemoglobin
https://www.readbyqxmd.com/read/28667380/variants-in-the-non-coding-region-of-the-tlr2-gene-associated-with-infectious-subphenotypes-in-pediatric-sickle-cell-anemia
#10
Susana David, Pedro Aguiar, Liliana Antunes, Alexandra Dias, Anabela Morais, Anavaj Sakuntabhai, João Lavinha
Sickle cell anemia (SCA) is characterized by chronic hemolysis, severe vasoocclusive crises (VOCs), and recurrent often severe infections. A cohort of 95 SCA pediatric patients was the background for genotype-to-phenotype association of the patient's infectious disease phenotype and three non-coding polymorphic regions of the TLR2 gene, the -196 to -174 indel, SNP rs4696480, and a (GT)n short tandem repeat. The infectious subphenotypes included (A) recurrent respiratory infections and (B) severe bacterial infection at least once during the patient's follow-up...
June 30, 2017: Immunogenetics
https://www.readbyqxmd.com/read/28662607/integrating-fat-embolism-syndrome-scoring-indices-in-sickle-cell-disease-a-practice-management-review
#11
Keneisha Bailey, Jagila Wesley, Adebayo Adeyinka, Louisdon Pierre
Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28637291/pediatric-to-adult-care-transition-perspectives-of-young-adults-with-sickle-cell-disease
#12
Jerlym S Porter, Kimberly M Wesley, Mimi S Zhao, Rebecca J Rupff, Jane S Hankins
The aim of this study was to explore perspectives of transition and transition readiness of young adult patients (YAs) with sickle cell disease (SCD) who have transitioned to adult health care. In all, 19 YAs with SCD (ages 18-30 years) participated in one of three focus groups and completed a brief questionnaire about transition topics. Transcripts were coded and emergent themes were examined using the social-ecological model of adolescent and young adult readiness for transition (SMART). Themes were consistent with most SMART components...
June 15, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28626540/acute-crises-and-complications-of-sickle-cell-anemia-among-patients-attending-a-pediatric-tertiary-unit-in-kinshasa-democratic-republic-of-congo
#13
Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28606098/determining-the-longitudinal-validity-and-meaningful-differences-in-hrql-of-the-pedsql%C3%A2-sickle-cell-disease-module
#14
Julie A Panepinto, J Paul Scott, Oluwakemi Badaki-Makun, Deepika S Darbari, Corrie E Chumpitazi, Gladstone E Airewele, Angela M Ellison, Kim Smith-Whitley, Prashant Mahajan, Sharada A Sarnaik, T Charles Casper, Larry J Cook, Julie Leonard, Monica L Hulbert, Elizabeth C Powell, Robert I Liem, Robert Hickey, Lakshmanan Krishnamurti, Cheryl A Hillery, David C Brousseau
BACKGROUND: Detecting change in health status over time and ascertaining meaningful changes are critical elements when using health-related quality of life (HRQL) instruments to measure patient-centered outcomes. The PedsQL™ Sickle Cell Disease module, a disease specific HRQL instrument, has previously been shown to be valid and reliable. Our objectives were to determine the longitudinal validity of the PedsQL™ Sickle Cell Disease module and the change in HRQL that is meaningful to patients...
June 12, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28602692/psychological-characteristics-and-pain-frequency-are-associated-with-experimental-pain-sensitivity-in-pediatric-patients-with-sickle-cell-disease
#15
Nitya Bakshi, Ines Lukombo, Helen Shnol, Inna Belfer, Lakshmanan Krishnamurti
Sickle Cell Disease (SCD) is associated with episodes of severe vasocclusive pain beginning in infancy with a subset of patients with SCD transitioning to chronic pain. Response to experimental pain using Quantitative Sensory Testing (QST) in these patients suggest altered pain processing. The objectives of this study were to characterize sensitivity to multiple modalities of experimental pain stimuli and to interrogate the relationship of psychological covariates, clinical pain burden and pain-related outcomes to experimental pain sensitivity in children with SCD as compared to healthy individuals of similar age and sex...
June 5, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28599856/-bilateral-pulmonary-embolism-mimicking-acute-chest-syndrome-in-an-adolescent-with-sickle-cell-disease
#16
P Mornand, F Chalard, A-S Romain, M Rohr, C Paluel-Marmont, A Niakaté, B Quinet, E Grimprel, M-H Odièvre-Montanié
Pulmonary embolism is a life-threatening and potentially lethal disease. Its incidence in children with sickle cell disease is probably underestimated and pediatric case reports in the literature are rare. Moreover, symptoms can mimic an acute chest syndrome. We report on the case of a 17-year-old boy with SS sickle cell disease, admitted for chest pain with dyspnea and tachycardia. Pulmonary angiography revealed a partial bilateral obstructive pulmonary embolism. We did not find any deep venous thrombosis or thrombophilia...
June 6, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28578536/population-pharmacokinetic-and-exposure-response-analyses-of-prasugrel-in-pediatric-patients-with-sickle-cell-anemia
#17
Brian A Moser, Elizabeth S LaBell, Emmanuel Chigutsa, Joseph A Jakubowski, David S Small
BACKGROUND AND OBJECTIVE: Prasugrel, a P2Y12 adenosine diphosphate (ADP) receptor antagonist, inhibits ADP-mediated platelet activation and aggregation in patients with sickle cell anemia (SCA). We developed a population pharmacokinetic (popPK) model in pediatric patients from 2 to <18 years of age with SCA, and performed exposure-response evaluations to characterize the effects of prasugrel in a subset of these patients who weighed 19 kg or more and experienced at least two episodes of vaso-occlusive crises (VOC) in the past year...
June 3, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28578441/prevalence-of-obstructive-sleep-apnea-in-children-with-sickle-cell-disease-at-a-tertiary-hospital-in-saudi-arabia
#18
Tahani Al-Otaibi, Mansour Al-Qwaiee, Huda Faraidi, Fatima Batniji, Faisal Al-Otaibi, Adel Al-Harbi
To assess the prevalence of obstructive sleep apnea in Saudi children with sickle cell disease at a tertiary hospital in Kingdom of Saudi Arabia (KSA) using nocturnal polysomnography. Methods: A prospective cross-section study was conducted between 2012 and 2016 in 65 children aged between 2-14 years at Prince Sultan Military Medical City, Riyadh, KSA with sickle cell disease. Patients answered a pediatric sleep questionnaire with the help of an accompanying caregiver and underwent polysomnography in the same night...
June 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28576670/validation-of-the-youth-acute-pain-functional-ability-questionnaire-yapfaq-in-children-and-adolescents-undergoing-inpatient-surgery
#19
Jennifer A Rabbitts, Rachel V Aaron, William T Zempsky, Tonya M Palermo
Half of children admitted after surgery experience intense pain in hospital, and many experience continued pain and delayed functional recovery at home. However, there is a gap in tools available to measure acute functional ability in pediatric postsurgical settings. We aimed to validate the Youth Acute Pain Functional Ability Questionnaire (YAPFAQ) in a large inpatient pediatric surgical population, evaluate its responsiveness to expected functional recovery, and develop a short form for broad clinical implementation...
May 30, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28555355/laparoscopic-cholecystectomy-for-cholelithiasis-in-children-with-sickle-cell-disease
#20
REVIEW
Yousef Al Talhi, Bader Hamza Shirah, Muteb Altowairqi, Yasmin Yousef
Patients with sickle cell disease (SCD) suffer from an increased incidence of gallstone formation due to hemolysis of sickled red blood cells; this leads to an increased level of bilirubin in secreted bile that becomes a nidus for pigment stone formation. Laparoscopic cholecystectomy (LC) is considered a standard operative procedure for gallstone disease mainly due to lower postoperative wound complaints, faster recovery, better postoperative cosmetic results, shorter hospital stay, and earlier return to work...
August 2017: Clinical Journal of Gastroenterology
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