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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/29350437/diffusion-tensor-imaging-mri-of-sickle-cell-kidney-disease-initial-results-and-comparison-with-iron-deposition
#1
Shannon B Donnola, Connie M Piccone, Lan Lu, Joshua Batesole, Jane Little, Katherine M Dell, Chris A Flask
Chronic kidney disease (CKD) occurs in over one-third of patients with sickle cell disease (SCD) and can progress to end-stage renal disease. Unfortunately, current clinical assessments of kidney function are insensitive to early-stage CKD. Previous studies have shown that diffusion magnetic resonance imaging (MRI) can sensitively detect regional renal microstructural changes associated with early-stage CKD. However, previous MRI studies in patients with SCD have been largely limited to the detection of renal iron deposition assessed by T2 * relaxometry...
January 19, 2018: NMR in Biomedicine
https://www.readbyqxmd.com/read/29331393/oral-health-considerations-for-pediatric-patients-with-sickle-cell-disease
#2
REVIEW
Sahar Alrayyes, Anne-Ashley Compton, Nadia Kawar
No abstract text is available yet for this article.
January 10, 2018: Disease-a-month: DM
https://www.readbyqxmd.com/read/29324574/sleep-problem-risk-for-adolescents-with-sickle-cell-disease-sociodemographic-physical-and-disease-related-correlates
#3
Cecelia R Valrie, Krystal L Trout, Kayzandra E Bond, Rebecca J Ladd, Nichelle L Huber, Kristen J Alston, Alicia M Sufrinko, Erik Everhart, Beng R Fuh
The aims of the current study were to investigate whether SCD incurs an additional risk for poor sleep over and above the influence of sociodemographic factors (ie, race and sex) during adolescence, and to explore the relationships between sociodemographic, physical (ie, age and pubertal status), and disease-related factors (ie, SCD genotype and hydroxyurea use) on sleep problem risk during adolescence. Black adolescents (age, 12 to 17 y) with SCD (n=53) were recruited from regional pediatric SCD clinics in the southeast and a sample of healthy black adolescents (n=160) were recruited from middle and high schools...
January 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29323695/lead-toxicity-in-the-pediatric-patient-with-sickle-cell-disease-unique-risks-and-management
#4
Josephine Misun Jung, Radhika Peddinti
Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described...
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29323687/pediatric-endocrinology-lead-toxicity-in-sickle-cell-disease-and-hiv-pre-exposure-prophylaxis
#5
EDITORIAL
Joseph R Hageman
No abstract text is available yet for this article.
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#6
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29282441/platelet-transfusion-thresholds-among-children-admitted-to-a-pediatric-intensive-care-unit
#7
Batool Alsheikh, Madhuradhar Chegondi, Balagangadhar Totapally
Objective To evaluate the threshold for platelet transfusion in children admitted to a pediatric intensive care unit (PICU). This is a retrospective chart review study, conducted at our tertiary level PICU and is related to critically ill pediatric patients who required platelet transfusion. Methods We retrieved the target patient population data from our blood bank database. The patients were subdivided into four subgroups: hematologic (hematologic malignancies, bone marrow suppression, hemolytic anemia, and sickle cell disease), surgical (post-surgical, trauma and acute bleeding), the unstable fraction of inspired oxygen (FiO2 > 0...
October 24, 2017: Curēus
https://www.readbyqxmd.com/read/29279789/asthma-screening-in-pediatric-sickle-cell-disease-a-clinic-based-program-using-questionnaires-and-spirometry
#8
Sara C Sadreameli, Rachel O Alade, Peter J Mogayzel, Sharon McGrath-Morrow, John J Strouse
A clinician diagnosis of asthma is associated with increased morbidity and mortality in people with sickle cell disease (SCD). We hypothesized that a screening program would help identify children with asthma needing referral to pulmonary clinic. We conducted a single-center project to screen patients with SCD for asthma using a previously validated questionnaire (Breathmobile) and for pulmonary function abnormalities with portable spirometry. Participants with a positive questionnaire and/or abnormal spirometry were referred to pediatric pulmonary clinic...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#9
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
https://www.readbyqxmd.com/read/29222303/responsiveness-of-patient-reported-outcome-measurement-information-system-promis-pain-domains-and-disease-specific-patient-reported-outcome-measures-in-children-and-adults-with-sickle-cell-disease
#10
REVIEW
Susanna Curtis, Amanda M Brandow
Case 1: A 33-year-old man with hemoglobin SS (homozygous hemoglobin S) disease presents for his regular clinic visit. He had 6 hospital admissions for pain over the past year. He also has avascular necrosis of the right hip. He takes daily hydroxyurea with hematologic changes indicative of compliance. He also takes morphine sustained release twice daily and morphine immediate release every 6 hours as needed for pain. He feels that more optimal pain control at home would help him reduce his number of hospital admissions in the upcoming year and improve his daily functioning at home...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29210071/whole-exome-sequencing-of-sickle-cell-disease-patients-with-hyperhemolysis-syndrome-suggests-a-role-for-rare-variation-in-disease-predisposition
#11
Savannah Mwesigwa, Joann M Moulds, Alice Chen, Jonathan Flanagan, Vivien A Sheehan, Alex George, Neil A Hanchard
BACKGROUND: Hyperhemolysis syndrome (HHS) is an uncommon, but life-threatening, transfusion-related complication of red blood cell transfusion. HHS has predominantly been described in patients with sickle cell disease (SCD) and is difficult to diagnose and treat. The pathogenesis of HHS, including its occurrence in only a subset of apparently susceptible individuals, is poorly understood. We undertook whole-exome sequencing (WES) of 12 SCD-HHS patients to identify shared genetic variants that might be relevant to the development of HHS...
December 6, 2017: Transfusion
https://www.readbyqxmd.com/read/29201539/assessment-of-cerebral-blood-flow-with-magnetic-resonance-imaging-in-children-with-sickle-cell-disease-a-quantitative-comparison-with-transcranial-doppler-ultrasonography
#12
Paula L Croal, Jackie Leung, Przemyslaw Kosinski, Manohar Shroff, Isaac Odame, Andrea Kassner
Introduction: Transcranial Doppler ultrasonography (TCD) is a clinical tool for stratifying ischemic stroke risk by identifying abnormal elevations in blood flow velocity (BFV) in the middle cerebral artery (MCA). However, TCD is not effective at screening for subtle neurologic injury such as silent cerebral infarcts. To better understand this disparity, we compared TCD measures of BFV with tissue-level cerebral blood flow (CBF) using arterial spin-labeling MRI in children with and without sickle cell disease, and correlated these measurements against clinical hematologic measures of disease severity...
November 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29200151/recurrent-acute-chest-syndrome-in-pediatric-sickle-cell-disease-clinical-features-and-risk-factors
#13
Gaylen D Patterson, Hafsat Mashegu, Jordan Rutherford, Samantha Seals, David Josey, Cynthia Karlson, Melissa McNaull, Warren May, Clinton Carroll, Frederick E Barr, Suvankar Majumdar
Acute chest syndrome (ACS) is a common and serious lung complication in sickle cell disease. A retrospective medical chart review was performed over a 6-year period in all pediatric ACS patients to investigate whether factors during the initial hospitalization were associated with recurrent ACS episodes. There were 386 episodes of ACS: 149 had only 1 episode of ACS, and 76 had >1 episode of ACS; 172 (76.4%) had hemoglobin SS, and 39 (17.3%) had hemoglobin SC. The most common presenting features were fever (83%), pain (70%), and cough (61%), which changed with the number of ACS episodes...
January 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29185057/implications-of-a-paediatrician-psychologist-tandem-for-sickle-cell-disease-care-and-impact-on-cognitive-functioning
#14
Adrienne Lerner, Hervé Picard, Adrien May, Vincent Gajdos, Louise Malou-Dhaussy, Flaviana Maroja-Cox, Laurence Salomon, Marie-Hélène Odièvre
Sickle cell disease (SCD), a life-threatening chronic disease, necessitates a paediatric treatment plan that considers the influence of psychological, family and intercultural factors. At the Louis-Mourier Hospital (APHP) in Colombes, France, we introduced an original paediatric-psychological partnership where a clinical psychologist accompanies the paediatrician at programmed consultations. We evaluated children and their parents treated in Colombes and in two other paediatric units using standardized culture-free tools and clinical interviews to evaluate the psychological repercussions of SCD...
November 29, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29183008/correlates-of-pulmonary-function-in-children-with-sickle-cell-disease-and-elevated-fetal-hemoglobin
#15
Adekunle D Adekile, Asmaa Azab, Abdullah Owayed, Mousa Khadadah
OBJECTIVE: The current study was carried out to compare pulmonary function tests (PFT) in pediatric Kuwaiti sickle cell disease (SCD) patients to age-matched normal controls and to investigate the association of PFTS to selected clinical and laboratory parameters. SUBJECTS AND METHODS: There were 38 patients with SCD and 36 controls in the study. The patients were recruited from the Pediatric Hematology Clinics of Mubarak Al-Kabeer and Al-Amiri Hospitals, Kuwait and were studied in steady state...
November 28, 2017: Medical Principles and Practice: International Journal of the Kuwait University, Health Science Centre
https://www.readbyqxmd.com/read/29176462/feasibility-and-acceptability-of-internet-delivered-cognitive-behavioral-therapy-for-chronic-pain-in-adolescents-with-sickle-cell-disease-and-their-parents
#16
Tonya M Palermo, Joanne Dudeney, James P Santanelli, Alexie Carletti, William T Zempsky
Pain is a clinical hallmark of sickle cell disease (SCD), and is rarely optimally managed. Cognitive-behavioral therapy (CBT) for pain has been effectively delivered through the Internet in other pediatric populations. We tested feasibility and acceptability of an Internet-delivered CBT intervention in 25 adolescents with SCD (64% female, mean age=14.8 y) and their parents randomized to Internet CBT (n=15) or Internet Pain Education (n=10). Participants completed pretreatment/posttreatment measures. Eight dyads completed semistructured interviews to evaluate treatment acceptability...
November 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29134776/acute-chest-syndrome-among-children-hospitalized-with-vaso-occlusive-crisis-a-nationwide-study-in-the-united-states
#17
Takuto Takahashi, Yusuke Okubo, Atsuhiko Handa
PURPOSE: Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication. METHODS: Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database...
November 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29117957/nocturnal-oxyhemoglobin-desaturation-and-arteriopathy-in-a-pediatric-sickle-cell-disease-cohort
#18
Nomazulu Dlamini, Dawn E Saunders, Michael Bynevelt, Sara Trompeter, Timothy C Cox, Romola S Bucks, Fenella J Kirkham
OBJECTIVE: The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO2) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia. METHODS: Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9...
November 8, 2017: Neurology
https://www.readbyqxmd.com/read/29115052/dose-selection-based-on-modeling-and-simulation-for-rivipansel-in-pediatric-patients-aged-6-to-11-years-with-sickle-cell-disease
#19
Brinda K Tammara, Lutz O Harnisch
This modeling and simulation exercise aimed to provide dosing recommendations for rivipansel phase 3 studies in children aged 6 to 11 years with sickle cell disease (SCD). Pharmacokinetic data from 109 patients aged 12 to 51 years who received rivipansel (2-40 mg/kg) in previous studies (3 phase 1, one phase 2) were integrated to build a 3-compartmental simulation model. Renal clearance simulations across the age range accounted for renal function development and postulated hyperfiltration in SCD. Simulated demographic distributions for the pediatric SCD population were used to predict concentration-time profiles from 3 dosing regimens, which were then compared against efficacious average steady-state concentrations observed in phase 2...
November 8, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#20
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
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