A physicians survey assessing management of pulmonary airway involvement in sickle cell disease

Agrawal, Sabhyata; Burton, William B.; Manwani, Deepa; Rastogi, Deepa; De, Aliva

doi:10.1002/ppul.24289

Cited by 5 publications

(3 citation statements)

References 38 publications

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“…32 Given the available literature, it is not surprising that a survey conducted among pediatric pulmonologists revealed a lack of consensus on diagnosis and treatment of SCD and airway inflammation. 33 Prospective clinical trials are needed to establish evidence-based guidelines on the use of asthma medications in patients with SCD.…”

Section: Discussionmentioning

confidence: 99%

“…A randomized controlled trial in nonasthmatic adults with SCD showed that inhaled corticosteroids were associated with decreased daily pain, lower biomarker levels for vascular injury, and similar ACT scores compared to placebo 32 . Given the available literature, it is not surprising that a survey conducted among pediatric pulmonologists revealed a lack of consensus on diagnosis and treatment of SCD and airway inflammation 33 . Prospective clinical trials are needed to establish evidence‐based guidelines on the use of asthma medications in patients with SCD.…”

Section: Discussionmentioning

confidence: 99%

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Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma

Saxena

Afolabi‐Brown

Ballester

et al. 2022

Pediatric Pulmonology

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Objective: Asthma is a recognized comorbidity in children with sickle cell disease (SCD). It increases the risk of acute chest syndrome (ACS), vaso-occlusive episodes, and early mortality. We aim to determine whether evaluation and management of children with SCD and asthma by a pulmonologist reduce rate of asthma exacerbation and ACS. Methods:The study included 192 patients with SCD (0-21 years) followed at Children's Hospital of Philadelphia Hematology between January 1, 2015, and December 31, 2018, with a diagnosis of asthma, wheeze, or cough. Patients were placed in two groups: those evaluated by a pulmonologist (SCD-A-P) and those not (SCD-A). Rates of emergency department (ED) visits and hospitalizations for asthma exacerbation and ACS were compared between groups and over time.Results: SCD-A-P patients (n = 70) were predominantly SCD type SS with lower hemoglobin and hematocrit compared to SCD-A patients (n = 122). SCD-A-P started with a higher average rate of hospital visits for asthma exacerbation and ACS per year (2.69 [1.02-4.37]) compared to SCD-A (0.43 [0.24-0.63]), (p < 0.001). For SCD-A-P patients with at least one hospital visit (n = 48), the average rate decreased from 3.93 (1.57-6.29) to 0.85 (0.48-1.23) following pulmonary consultation (p = 0.014) and was comparable to the SCD-A rate by study end.Conclusion: SCD-A-P was mainly SCD type SS and had higher ED/hospitalization rates for asthma exacerbation and ACS compared to SCD-A, but the rates significantly decreased following pulmonology consultation. These findings support the pulmonologist's role in the multidisciplinary care of SCD patients and highlight the need for evidence-based asthma guidelines for children with SCD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma

Saxena

Afolabi‐Brown

Ballester

et al. 2022

Pediatric Pulmonology

View full text Add to dashboard Cite

show abstract

“…Blood culture or other sepsis work-up tests might be obtained depending upon the suspicions of an ongoing infectious process. Determining blood type and crossmatch might be warranted in case of a possible blood transfusion [35][36][37][38]. Besides laboratory tests, radiological imaging might be indicated in certain cases.…”

Section: Diagnostic Evaluationmentioning

confidence: 99%

Correlation of Transfusion Dependence and Its Associated Sequelae to Hematological and Biochemical Parameters in Patients With Sickle Cell Disease and Beta Thalassemia Major in Khobar: A Retrospective Study

Albahout,

Yunus,

Mohammad

et al. 2023

Cureus

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Sickle cell disease (SCD) and beta thalassemia major (βTM) are multisystemic, genetically inherited diseases. They are caused by mutations of hemoglobin, which ultimately cause abnormal functioning of the red blood cells. The morbidity and mortality rates of these diseases are significant, as they may result in severe complications, some of which are quite fatal; hence, early diagnosis and treatment are crucial. The purpose of this study is to collect patients' data in terms of their manifestations and overall clinical picture and correlate them to the laboratory parameters with emphasis on their transfusion dependence and its sequelae in King Fahd Hospital of the University (KFHU), Al-Khobar, Saudi Arabia.After obtaining ethical approval from the institutional review board and in collaboration with the blood bank, patients' data were retrospectively collected from the hospital's database and categorized into two disease groups. Accordingly, data related to the biological and demographic information, clinical picture pattern, laboratory investigations, and therapeutic measures, with emphasis on blood transfusion as a treatment option, were gathered and analyzed. Eventually, the aforementioned data aspects were assessed for the probability of correlations, which were proven to be present to some level as an answer to our cohort study's question. Such findings, which will be depicted later in this study, might represent a ground for having a more comprehensive and extensive approach in terms of the general evaluation of patients with SCD and βTM based on the established level of correlation.During the course of conducting our research, we encountered some limitations, including the sample size and scarce data available during the process of data collection.

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Pulmonary disease burden in Hispanic and non‐Hispanic children with sickle cell disease

Chen

Gong

Matta

et al. 2020

Pediatric Pulmonology

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RationalePulmonary complications are the leading cause of morbidity and mortality in sickle cell disease (SCD) patients. Research in SCD has predominantly been conducted on African‐Americans, and the disease burden of SCD in other races and ethnicities, including Hispanic patients, is not well characterized.ObjectiveTo compare pulmonary disease burden between Hispanic and non‐Hispanic ethnic groups among children with SCD.MethodsIn a retrospective chart review on 566 SCD patients followed at the Children's Hospital at Montefiore, NY, we compared the pulmonary disease burden and disease management in Hispanic patients to their non‐Hispanic counterparts. We also compared the contribution of demographic and clinical variables to acute chest syndrome (ACS), vaso‐occlusive crisis (VOC), and hospitalizations for SCD related complications between the two ethnic groups.ResultsHispanic patients had a greater proportion of ACS, and had lower forced expiratory volume (FEV1), forced vital capacity, and vital capacity, compared to non‐Hispanics. Hispanic patients were more likely to be evaluated in pulmonary clinic and to be on inhaled corticosteroids, short‐acting β agonizts, and leukotriene receptor antagonists. In addition, Hispanic children were more likely to be on hydroxyurea, and receive exchange transfusions. However, the association of asthma with the proportion of ACS did not differ between Hispanics and non‐Hispanics.ConclusionHispanic children with SCD had differences in their pulmonary function profile and received more pulmonary evaluations than non‐Hispanic children.

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A physicians survey assessing management of pulmonary airway involvement in sickle cell disease

Cited by 5 publications

References 38 publications

Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma

Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma

Correlation of Transfusion Dependence and Its Associated Sequelae to Hematological and Biochemical Parameters in Patients With Sickle Cell Disease and Beta Thalassemia Major in Khobar: A Retrospective Study

Pulmonary disease burden in Hispanic and non‐Hispanic children with sickle cell disease

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