scholarly journals Pulmonary Complications and Lung Function Abnormalities in Children with Sickle Cell Disease

Author(s):  
Anne Greenough
2019 ◽  
Vol 54 (5) ◽  
pp. 610-619 ◽  
Author(s):  
Azza A. Tantawy ◽  
Amira A. Adly ◽  
Fatma S. E. Ebeid ◽  
Eman A. Ismail ◽  
Mahitab M. Hussein ◽  
...  

2007 ◽  
Vol 42 (6) ◽  
pp. 483-488 ◽  
Author(s):  
Anastassios C. Koumbourlis ◽  
Donna J. Lee ◽  
Ada Lee

Hematology ◽  
2002 ◽  
Vol 2002 (1) ◽  
pp. 10-34 ◽  
Author(s):  
Mark C. Walters ◽  
Arthur W. Nienhuis ◽  
Elliott Vichinsky

Abstract In this update, selected clinical features of sickle cell disease and their management are reviewed. In addition, the current status of interventions that have curative potential for sickle cell disease is discussed, with particular attention focused on indications, methodology, recent results, and challenges to wider clinical application. In Section I, Dr. Nienhuis describes recent improvements in vector technology, safety, and replacement gene expression that are creating the potential for clinical application of this technology. In Section II, Dr. Vichinsky reviews our current understanding of the pathophysiology and treatment of pulmonary injury in sickle cell disease. The acute and chronic pulmonary complications of sickle cell disease, modulators and predictors of severity, and conventional and novel treatment of these complications are discussed. In Section III, Dr. Walters reviews the current status of hematopoietic cell transplantation for sickle cell disease. Newer efforts to expand its availability by identifying alternate sources of stem cells and by reducing the toxicity of transplantation are discussed.


2014 ◽  
Vol 15 (1) ◽  
pp. 13-16 ◽  
Author(s):  
Jennifer Knight-Madden ◽  
Anne Greenough

2020 ◽  
Vol 48 (5) ◽  
pp. 450-457
Author(s):  
Andrea Angel ◽  
Gustavo Falbo Wandalsen ◽  
Dirceu Solé ◽  
Fernanda C. Lanza ◽  
Carolina L.N. Cobra ◽  
...  

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3759-3759
Author(s):  
Onyinye C. Onyekwere ◽  
Andrew Campbell ◽  
James Williams ◽  
Peter Gaskin ◽  
Sohail Rana ◽  
...  

Abstract Despite the high prevalence of PHTN in adults with SCD, the prevalence in the pediatric population with SCD is not known. We hypothesized that elevated pulmonary artery pressures may be found in SCD adolescents with history of pulmonary complications, such as acute chest syndrome (ACS), obstructive sleep apnea (OSA), asthma, and reactive airway disease. Thirty such sickle cell disease adolescents were screened at Howard University or University of Michigan for PHTN with Doppler echocardiography. We defined PHTN as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/sec (corresponding to a pulmonary artery systolic pressure greater than 35 mm Hg). PHTN was found in 16 SCD patients (53.3%) and 5 (16.7%) had TRV > 3.0 m/sec. Clinical findings according to the presences or absence of PHTN are shown in the table. Potential factors contributing to PHTN in patients with SCD include chronic hemolysis and chronic hypoxia. Our results suggest that PHTN is common among SCD adolescents with a history of pulmonary complications. Consideration should be given to screening such patients for PHTN and exploring treatment options. Further studies are urgently needed to clarify the prevalence and mechanisms of PHTN in adolescents with SCD. Clinical and demographic data of 30 SCD adolescents with pulmonary findings who underwent echocardiography at Howard University Hospital or University of Michigan PHTN (N = 16) No PHTN (N = 14) P Age in years (mean +/− SD) 15.9 +/− 3.2 17.4 +/− 2.3 0.17 Females (no. and %) 5 (31.3) 7 (50) 0.5 Hemoglobin SS Phenotype (no and %) 14 (87.5 11 (78.6) 0.5 Hemoglobin concentration (mean +/− SD) 8.0 +/− 2.1 9.3 +/−1.9 0.11 White blood cells (mean +/− SD) 10.9 +/− 2.9 9.7 +/− 3.7 0.4 Platelet (mean +/− SD) 475 +/− 172 364 +/− 240 0.17 Hemoglobin F percent (mean +/− SD) 5.1 +/− 3.5 6.4 +/− 5.5 0.6 Lactate dehydrogenase (mean +/− SD) 505 +/− 162 264 +/− 50 0.002 Total bilirubin (mean +/− SD) 4.1 +/− 2.6 3.4 +/− 2.6 0.5 Creatinine concentration (mean +/− SD) 0.6 +/− 0.2 0.7 +/− 0.2 0.18 Aspartate transaminase (mean +/− SD) 48 +/− 27 36 +/− 16 0.18 Alanine transaminase (mean +/− SD) 51 +/− 37 39 +/− 20 0.3


Lung ◽  
2010 ◽  
Vol 188 (6) ◽  
pp. 499-504 ◽  
Author(s):  
Jennifer M. Knight-Madden ◽  
Terrence S. Forrester ◽  
Norma A. Lewis ◽  
Anne Greenough

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