Original Research: Acute chest syndrome in sickle cell disease: Effect of genotype and asthma

Pahl, Kristy S.; Mullen, Craig A.

doi:10.1177/1535370216636720

Cited by 9 publications

(9 citation statements)

References 18 publications

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“…2, 3 This results in significant RBC membrane damage and deformation; and may result in RBC lysis. 4, 5 The complexity of SCD can result in a number of clinical manifestations such as stroke 6, 7 , pulmonary hypertension 8 , cognitive deficiency 9 , and acute chest syndrome 10 which further contributes to increase morbidity and mortality in these patients. Healthy human red blood cells (RBCs) have the ability to change their morphology and structural rigidity in a reversible manner during oxygen transport.…”

Section: Introductionmentioning

confidence: 99%

Shear dependent red blood cell adhesion in microscale flow

2018

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Non-adherence and deformability are the key intrinsic biomechanical features of the red blood cell (RBC), which allow it to tightly squeeze and pass through even the narrowest of microcirculatory networks. Blockage of microcirculatory flow, also known as vaso-occlusion, is a consequence of abnormal cellular adhesion to the vascular endothelium. In sickle cell disease (SCD), an inherited anaemia, even though RBCs have been shown to be heterogeneous in adhesiveness and deformability, this has not been studied in the context of physiologically relevant dynamic shear gradients at the microscale. We developed a microfluidic system that simulates physiologically relevant shear gradients of microcirculatory blood flow at a constant single volumetric flow rate. Using this system, shear dependent adhesion of RBCs from 28 subjects with SCD and from 11 healthy subjects was investigated using vascular endothelial protein functionalized microchannels. We defined a new term, RBC Shear Gradient Microfluidic Adhesion (SiGMA) index to assess shear dependent RBC adhesion in a subject-specific manner. We have shown for the first time that shear dependent adhesion of RBCs is heterogeneous in a microfluidic flow model, which correlates clinically with inflammatory markers and iron overload in subjects with SCD. This study reveals the complex dynamic interactions between RBC-mediated microcirculatory occlusion and clinical outcomes in SCD. These interactions may also be relevant to other microcirculatory disorders and microvascular diseases.

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Section: Introductionmentioning

confidence: 99%

Shear dependent red blood cell adhesion in microscale flow

2018

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“…The majority of patients in the SCD-A-P group had the homozygous genotype of SCD type SS, and this group had higher rates of hospital visits for asthma and ACS compared to group SCD-A. Patients with this genotype are known to have more severe disease than patients with the heterozygous genotype (SCD type SC) 1,15 , and they are at higher risk for ACS compared to other SCD genotypes 27 . Thus, patients with SCD type SS should perhaps be referred to the pulmonologist more readily compared to patients with other genotypes.…”

Section: Discussionmentioning

confidence: 94%

Benefit of Pulmonary Subspecialty Care for Children with Sickle Cell Disease and Asthma

Saxena

Afolabi‐Brown

Ballester

et al. 2021

Preprint

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Objective: Asthma is a recognized comorbidity in children with sickle cell disease (SCD). It increases risk of acute chest syndrome (ACS), vaso-occlusive episodes, and early mortality. We aim to determine whether evaluation and management of children with sickle cell disease (SCD) and asthma by a pulmonologist reduces 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 01/01/2015 and 12/31/2018 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 before/after initial pulmonology visit. Results: SCD-A-P (n=70) had lower baseline pulmonary function, hemoglobin, and hematocrit compared to SCD-A (n=122). SCD-A-P had a higher average rate of hospital visits for asthma exacerbation and ACS per year compared to SCD-A: 0.84 [0.56-1.12] versus 0.31 [0.18-0.43], (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] before initial pulmonary visit to 0.85 [0.48-1.23] after the visit (p=0.014). Conclusion: SCD-A-P had more severe baseline disease and higher rates of ED visits and hospitalizations 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. Further studies are needed to help establish evidence-based asthma guidelines for children with SCD.

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“…In our study, the most significant genotypes were HbSS (57%) and Hb SB (43%). Pahl et al [22] evaluated 109 patients and observed that 50% of HbSS and HbSB genotype patients were diagnosed with acute chest syndrome (ACS) at least once during hospitalization due to painful crisis. In our patients, 50.6% were diagnosed with ACS during painful crisis.…”

Section: Discussionmentioning

confidence: 99%

Patient-controlled analgesia and morphine consumption in sickle cell anemia painful crises: A new protocol

Bakır

Atıcı

Yıldırım

et al. 2020

Agri

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The sudden and rapidly increasing severity of pain in sickle cell anemia painful crises frequently requires the use of strong opioids. Patients require continuous administrations of various doses (increased/decreased) within the following hours. This study aims to retrospectively evaluate the effects of a structured protocol based on standardized Visual Analogue Scale (VAS) and Patient-controlled analgesia (PCA) patient demand count on morphine consumption in painful crises. Methods: A total of 177 painful crises of 93 patients who were administered morphine using the PCA method according to appropriate analgesia protocol between 2004-2018 were evaluated in this study. The demographic data, hemoglobin chromatography and genotypes, painful episode follow-up time, VAS scores before and after treatment, and daily morphine consumption of the patients were recorded. Morphine consumption during the crisis according to age groups and sex were compared. Results: Of the patients, 57% were homozygous hemoglobin type SS (HbSS). Mean morphine consumption with PCA method was 56.9±35.4 mg (min-max: 10-232 mg) and mean follow-up time was 3.4±2.1 days (min.-max.: 1-11). VAS scores were significantly lower after treatment (6.8±2.3 pre-treatment; 0.8±0.6 post-treatment) (p<0.05). Conclusion: To our knowledge, our study is the first structured protocol based on VAS and PCA demand data. We believe lower morphine dosage using PCA protocol according to the rapidly changing pain levels of the patients will provide effective analgesia. Prospective studies with fewer limitations will more effectively demonstrate the effectiveness of this protocol.

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Original Research: Acute chest syndrome in sickle cell disease: Effect of genotype and asthma

Cited by 9 publications

References 18 publications

Shear dependent red blood cell adhesion in microscale flow

Shear dependent red blood cell adhesion in microscale flow

Benefit of Pulmonary Subspecialty Care for Children with Sickle Cell Disease and Asthma

Patient-controlled analgesia and morphine consumption in sickle cell anemia painful crises: A new protocol

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