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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28637291/pediatric-to-adult-care-transition-perspectives-of-young-adults-with-sickle-cell-disease
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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...
May 29, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/28548215/protein-z-and-endothelin-1-genetic-polymorphisms-in-pediatric-egyptian-sickle-cell-disease-patients
#10
Mervat M Khorshied, Nohair S Mohamed, Rania S Hamza, Rasha M Ali, Mona K El-Ghamrawy
BACKGROUND: Sickle cell disease (SCD) is a monogenic disease associated with multisystem morbidity. Vasculopathy caused by delicate imbalance between coagulation and endothelial systems plays a pivotal role in disease course. As Protein Z and Endothelin-1 genetic polymorphisms may increase the thrombotic risk, the aim of the current work was to verify the possible impact of Protein Z (PROZ G79A) and Endothelin-1 (EDN1 G5665T) polymorphisms on the clinic-laboratory features of the SCD in a cohort of Egyptian pediatric patients...
May 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28538501/invasive-pneumococcal-disease-in-patients-with-sickle-cell-disease
#11
Pournima Navalkele, Bülent Özgönenel, Eric McGrath, Paul Lephart, Sharada Sarnaik
BACKGROUND: Patients with sickle cell disease (SCD) are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD. This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. pneumoniae alongside routine childhood immunization. But compared with the general African American pediatric population, the rate of invasive pneumococcal disease (IPD) in patients with SCD still remains high, resulting in hospitalization and fatality...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28500682/red-blood-cell-transfusions-during-sickle-cell-anemia-vaso-occlusive-crises-a-report-from-the-magnesium-in-crisis-magic-study
#12
Monica L Hulbert, Julie A Panepinto, J Paul Scott, Robert I Liem, Lawrence J Cook, Timothy Simmons, David C Brousseau
BACKGROUND: Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy. STUDY DESIGN AND METHODS: This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β(0) thalassemia...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28490230/acceptability-of-in-utero-hematopoietic-cell-transplantation-for-sickle-cell-disease
#13
Ryan M Antiel, Scott D Halpern, Evelyn M Stevens, Jesse D Vrecenak, Chavis A Patterson, Trudy Tchume-Johnson, Kim Smith-Whitley, William H Peranteau, Alan W Flake, Lamia P Barakat
BACKGROUND: In utero hematopoietic cell transplantation (IUHCT) has curative potential for sickle cell disease (SCD) but carries a risk of fetal demise. METHODS: We assessed the conditions under which parents of children with SCD and young adults with SCD would consider IUHCT in a future pregnancy, given a 5% fixed risk of fetal demise. Participants were randomized to consider a hypothetical cure rate (20%, 40%, or 70%). Subsequently, cure rate was either increased or decreased depending on the previous answer to reveal the lowest acceptable rate...
May 1, 2017: Medical Decision Making: An International Journal of the Society for Medical Decision Making
https://www.readbyqxmd.com/read/28488469/extramedullary-hematopoiesis-presenting-as-bilateral-glomus-tympanicum-first-case-report-in-a-pediatric-patient-and-with-bilateral-presentation
#14
Leonard N Chen, Alexandra Espinel, Brian Reilly, Dragos C Luca
To the best of our knowledge, this is the first case report of middle ear extramedullary hematopoiesis (EMH) in a pediatric patient as well as the first bilateral presentation reported in both children and adults. We report a 13-year-old African-American female with sickle cell disease who developed bilateral hearing loss, with magnetic resonance imaging findings consistent with bilateral glomus tympanicum (GT). Upon excisional biopsy, however, EMH was diagnosed histologically. Besides its novelty, this case highlights the importance of considering EMH in the differential diagnosis of GT including cases with bilateral presentation that may be otherwise highly suggestive of the familial form of GT...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28481580/neurocognitive-deficits-in-children-with-chronic-health-conditions
#15
Bruce E Compas, Sarah S Jaser, Kristen Reeslund, Niral Patel, Janet Yarboi
Over 4 million children in the United States suffer from chronic health conditions, including cancer, sickle cell disease, and diabetes. Because of major advances in the early identification and treatment of these conditions, survival rates for these children continue to rise, and the majority now lives into adulthood. However, increases in survival have come with costs related to long-term effects of disease processes and treatments. Foremost among these consequences is impairment in brain development and neurocognitive function that may affect a substantial portion of children with chronic health conditions and follow many into adulthood...
May 2017: American Psychologist
https://www.readbyqxmd.com/read/28475298/a-modified-noninvasive-screening-protocol-for-pulmonary-hypertension-in-children-with-sickle-cell-disease-who-should-be-sent-for-invasive-evaluation
#16
Christian Lilje, Jordan Harry, Kelly K Gajewski, Renee V Gardner
BACKGROUND: Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. OBJECTIVES: We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies...
May 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28450986/-major-sickle-cell-syndromes-and-infections-associated-with-this-condition-in-children-in-burkina-faso
#17
Sonia Douamba, Kisito Nagalo, Laure Tamini, Ismaël Traoré, Madibèlè Kam, Fla Kouéta, Diarra Yé
INTRODUCTION: This study aims to investigate infections in children with major sickle cell syndrome. METHODS: We conducted a monocentric descriptive retrospective hospital study in Ouagadougou, Burkina Faso, over a ten-year period. All children with major sickle cell syndrome (homozygous SS and double heterozygous SC, SDPunjab, Sβ thalassemic, SOArab and SE) hospitalized for microbiologically confirmed infections were enrolled in the study. RESULTS: One hundred and thirty-three patients met our inclusion criteria...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28444215/behavioral-and-pharmacological-adherence-in-pediatric-sickle-cell-disease-parent-child-agreement-and-family-factors-associated-with-adherence
#18
Page H Klitzman, Julia K Carmody, Mary H Belkin, David M Janicke
This study aimed to evaluate agreement between children and parents on a measure of behavioral and pharmacological adherence in children with sickle cell disease (SCD), and the associations among family factors (i.e., problem-solving skills, routines, communication) and adherence behaviors. In all, 85 children (aged 8-18 years) with SCD and their parents completed questionnaires assessing individual and family factors. Overall parent-child agreement on an adherence measure was poor, particularly for boys and older children...
April 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#19
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28417347/-serological-markers-of-viral-hepatitis-b-and-c-in-children-with-sickle-cell-disease-monitored-in-the-pediatrics-department-at-the-university-hospital-of-bobo-dioulasso-burkina-faso
#20
S A Kissou, M Koura, A Sawadogo, A-S Ouédraogo, H Traoré, E Kamboulé, W W F Zogona, B Nacro
Viral hepatitis B and C are universal public health problems. Burkina Faso is a high endemic area for hepatitis B. Patients with sickle cell disease are at risk. The aim of this study was to investigate and quantify the serological markers of viral hepatitis B and C among pediatric patients with sickle cell disease. This was a descriptive cross-sectional study, which took place from July to November 2014 at the Department of Pediatrics, University Hospital of Bobo- Dioulasso. The study included 44 SS and 26 SC sickle cell patients...
April 17, 2017: Bulletin de la Société de Pathologie Exotique
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