Longitudinal Analysis of Patient Specific Predictors for Mortality in Sickle Cell Disease

Curtis, Susanna A; Danda, Neeraja; Etzion, Zipora; Cohen, Hillel W.; Billett, Henny H.

doi:10.1371/journal.pone.0164743

Cited by 9 publications

(9 citation statements)

References 30 publications

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“…However, 6MWT better mimics the ADLs because it is a submaximal test that is easy to perform and has no risk for VOC in SCA patients ( 4 , 5 ). Because there has been a significant improvement in the survival of patients with SCA over recent decades ( 40 ), we believe that the N 2 SBW test may be a complementary tool to traditional PFTs, especially for the early diagnosis of pulmonary involvement, which tends to become more frequent with aging in this patient population.…”

Section: Discussionmentioning

confidence: 99%

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Lopes

Marinho

Alves

et al. 2017

Braz J Med Biol Res

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Sickle cell anemia (SCA) causes dysfunction of multiple organs, with pulmonary involvement as a major cause of mortality. Recently, there has been growing interest in the nitrogen single-breath washout (N2SBW) test, which is able to detect ventilation heterogeneity and small airway disease when the results of other pulmonary function tests (PFTs) are still normal. Thus, the objectives of the present study were to assess the heterogeneity in the ventilation distribution in adults with SCA and to determine the association between the ventilation distribution and the clinical, cardiovascular, and radiological findings. This cross-sectional study included 38 adults with SCA who underwent PFTs, echocardiography, computed tomography (CT), and 6-min walk test. To evaluate the ventilation heterogeneity, the patients were categorized according to the phase III slope of the N2SBW (SIIIN2). Compared with adults with lower SIIIN2 values, adults with higher SIIIN2 values showed lower hemoglobin levels (P=0.048), a history of acute chest syndrome (P=0.001), an elevated tricuspid regurgitation velocity (P=0.039), predominance of a reticular pattern in the CT (P=0.002), a shorter 6-min walking distance (6MWD) (P=0.002), and lower peripheral oxygen saturation (SpO2) after exercise (P=0.03). SIIIN2 values correlated significantly with hemoglobin (rs=-0.344; P=0.034), forced vital capacity (rs=-0.671; P<0.0001), diffusing capacity for carbon monoxide (rs=-0.376; P=0.019), 6MWD (rs=-0.554; P=0.0003), and SpO2 after exercise (P=0.040). Heterogeneity in the ventilation distribution is one of the most common pulmonary dysfunctions in adults with SCA. Moreover, relationships exist between ventilation heterogeneity, worsening of pulmonary structural damage, and reduced tolerance for exercise.

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Section: Discussionmentioning

confidence: 99%

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Lopes

Marinho

Alves

et al. 2017

Braz J Med Biol Res

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“…However, with this strategy, changes over time in the laboratory parameter are not taken into account. For example, in the study conducted by Curtis et al [17], which enrolled 359 patients with sickle cell anaemia, lower baseline levels of fetal hemoglobin were associated with increased mortality, however a signi cant risk of increased mortality, based on longitudinal changes in percentage of fetal hemoglobin, was not detected.…”

Section: Discussionmentioning

confidence: 99%

Laboratory parameters in prognostic sickle cell disease cohort studies in children: a systematic review and lessons for an appropriate strategy for analysis

Sommet

Roux

Koehl

et al. 2019

Preprint

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BACKGROUND: There is a variability of the results in the published prognostic sickle cell disease (SCD) studies. The aim of this article is to assess and demonstrate how the methods used for analysis of laboratory parameters impact the prognostic study results in children with sickle cell disease. METHODS: Firstly, a systematic review of published studies was conducted to identify the methods used in statistical analyses on laboratory parameters. Secondly, four different identified analysis were successively applied on laboratory data collected prospectively in our cohort of newborns with SCD in order to evaluate the association between laboratory parameters and cerebral vasculopathy, depending on the analysis strategy. RESULTS: Thirty-one studies were included in the systematic review in which hemoglobin and reticulocyte counts were the most frequently collected parameters. Through this review, we noted four different statistical strategies. In almost one half of the cohort studies, laboratory parameter was collected only one time per patient. When several values were available, only one statistical analysis considered the time variation. When we applied these four different statistical strategies to our cohort of SCD children, we highlighted that the conclusion on prognostic value of parameters (either as protective or as risk factor) depends on the analysis strategies applied. CONCLUSION: The laboratory parameter prognostic value depends on the analysis strategy chosen for its analysis. It raises fundamental questions about methodological approaches that concern clinician researchers. Identifying prognostic factors is currently essential for better understanding of SCD. Taking into account longitudinal data and standardization of analyses in prognostic cohort studies should allow drawing valuable and reproducible conclusions.

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“…The decreased red blood cell count, evidenced by low hematocrit percentage, besides the low Hb level and reticulocytosis 34,44,45 present themselves as risk factors for death in several researches, as well as a higher average corpuscular volume. 46 The increasing white cell numbers 44,45,47,48 have also been associated with a higher risk of death.…”

Section: Discussionmentioning

confidence: 99%

Fatores de risco para mortalidade em pacientes com doença falciforme: uma revisão integrativa

et al. 2020

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RESUMO Objetivo Sumarizar fatores de risco e indicadores de mortalidade em pacientes com doença falciforme. Método Revisão integrativa em periódicos indexados nas bases de dados CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO e Web of Science. A questão norteadora foi elaborada por meio da estratégia Population, variable, outcome (PVO). A busca ocorreu no portal de periódicos da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior entre outubro e novembro de 2018. Resultados Dos 19 artigos, 18 eram coorte e um ensaio clínico randomizado. A amostra foi constituída, em sua maioria, pelo sexo feminino e genótipo HbSS. Se repetiram mais a taxa de mortalidade cumulativa e a curva de mortalidade global. Sete estudos identificaram fatores de risco com associação estatisticamente significativa para morte. Os mais frequentes foram o baixo nível de hemoglobina, variáveis hepáticas (enzimas fosfatase alcalina e transaminase glutâmico oxalacética) e cardiovasculares (velocidade de regurgitação da válvula tricúspide ≥ 2,5m/s). Conclusão e implicações para a prática Indicadores de mortalidade constituem ferramentas de manejo de pacientes com doença falciforme e prevenção de riscos e complicações. Há necessidade de estudos sobre os fatores relacionados à mortalidade desses pacientes. A prevenção do óbito, certamente, promoverá uma melhoria na qualidade de vida e na sobrevida dessa população.

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Longitudinal Analysis of Patient Specific Predictors for Mortality in Sickle Cell Disease

Cited by 9 publications

References 30 publications

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Laboratory parameters in prognostic sickle cell disease cohort studies in children: a systematic review and lessons for an appropriate strategy for analysis

Fatores de risco para mortalidade em pacientes com doença falciforme: uma revisão integrativa

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