Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review

Bailey, Keneisha; Wesley, Jagila; Adeyinka, Adebayo; Pierre, Louisdon

doi:10.1177/0885066617712676

Cited by 9 publications

(5 citation statements)

References 71 publications

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“…Three major causes of ACS have been proposed: pulmonary infection, embolization of bone marrow fat, and intravascular pulmonary sequestration of sickled erythrocytes resulting in lung injury and infarction [ 4 ]. Pulmonary fat embolism (PFE), the blockage of pulmonary microvasculature by bone marrow fat, causes a more severe and distinct form of ACS via vascular obstruction and bone marrow necrosis [ 10 - 12 ]. The bone marrow undergoes necrosis and its contents including fat, are released into the bloodstream and travel to the lung where they cause severe lung inflammation and hypoxemia [ 13 - 15 ].…”

Section: Introductionmentioning

confidence: 99%

Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease

Wamkpah¹,

Shrestha²,

Salzman³

et al. 2022

Cureus

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Rationale Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Current treatment is supportive-supplemental oxygen, transfusions, and antibiotics. Prevention of ACS may reduce morbidity and mortality in patients with SCD. Acute chest syndrome appears similar to pulmonary fat embolism (PFE), a complication of severe skeletal trauma or orthopedic procedures from pulmonary micro-vessel blockage by bone marrow fat. Vascular obstruction and bone marrow necrosis occur in PFE and ACS. Pulmonary fat embolism rat models have shown that angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II receptor blockers (ARB) mitigate damage in PFE. These medications could work similarly in ACS. We hypothesize that time to readmission after one hospitalization for ACS will be reduced in patients taking ACEI or ARB compared to patients who are not. Methods This is a retrospective cohort study. Inclusion criteria are adults (18 to 100 years) with sickle cell anaemia (HbSS), hemoglobin SC (HbSC) disease, sickle cell thalassemia (HbSβThal), hospitalized with ACS over 16 years (January 1, 2000, to March 31, 2016); patients who take and don’t take ACEI or ARB. Children (<18 years old), elderly adults (>100 years old), pregnant patients, and patients with sickle cell trait were excluded. Data was collected from the Health Facts database, which contains de-identified information from the electronic medical records of hospitals in which Cerner© has a data use agreement. Kaplan-Meier estimates explored a time-to-event model of ACS readmission. Multivariable analysis (age, gender, smoking history) was conducted using Cox proportional hazards regression. Results were reported around a 95% confidence interval. Results There were 6972 patients in total. Of which, 9.6% (n = 667) reported taking ACEI or ARB. Results for the covariates were: average age of 38 years old; 63% female (n = 4366/6969); 16% smokers (n = 1132). Readmission rates were higher for patients not taking ACEI/ARB than those who did: 0.44 (95% CI 0.43, 0.46) versus 0.28 (95% CI 0.24, 0.31) at one year, and 0.56 (95% CI 0.55, 0.58) versus 0.33 (95% CI 0.29, 0.37) at two years. Age had the strongest effect on readmission rates for patients taking ACEI/ARB (adjusted hazards ratio 0.78 [95% CI 0.68, 0.91]). Conclusion Patients with SCD who reported taking ACEI or ARB had lower readmission rates for ACS; age was the strongest covariate. Our results may have a significant impact on the prevention of ACS. Prospective studies comparing ACEI or ARB therapy versus placebo are needed to confirm this preventative effect.

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Section: Introductionmentioning

confidence: 99%

Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease

Wamkpah¹,

Shrestha²,

Salzman³

et al. 2022

Cureus

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“…While the diagnosis of FES is clinically based on the presence of the triad of pulmonary symptoms such as hypoxemia, CNS symptoms such as altered mental status, and petechiae, it is important to note that all findings may not be present at the outset and that the diagnosis is usually reached with a high degree of suspicion. A study conducted by Bailey et al on FES, in general, has concluded that it is useful to use either the Gurd and Wilson or the Schonfeld fat embolism criteria as diagnostic tools when there is a high index of suspicion for FES [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

Fat Embolization Syndrome Secondary to Steroid Treatment in a Case of Sickle Cell Vaso-Occlusive Crisis

Thirugnanasambandam,

Maraikayar,

Liu

et al. 2023

Case Reports in Hematology

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Fat embolization syndrome (FES) is often seen as a complication of fractures and has been known to cause respiratory failure, rashes of the skin, thrombocytopenia, and neurological damage. Nontraumatic FES is uncommon and occurs due to bone marrow necrosis. Vaso-occlusive crisis in sickle cell patients secondary to steroid therapy is a rare entity and not widely acknowledged. We report a case of FES secondary to steroid therapy administered for a patient with intractable migraine. FES is an uncommon yet serious complication that occurs due to bone marrow necrosis and is usually associated with increased mortality or damaging neurologic sequelae for the surviving patient. Our patient was initially admitted for intractable migraine and worked up to rule out any acute emergency conditions. She was then given steroids for her migraine which did not subside with the initial treatment. Her condition worsened, and she developed respiratory failure along with altered mental status requiring care in the intensive care unit (ICU). Imaging studies showed microhemorrhages throughout the cerebral hemispheres, brainstem, and cerebellum. The imaging of her lungs confirmed severe acute chest syndrome. The patient also had hepatocellular and renal injuries indicative of multiorgan failure. The patient was treated with a red cell exchange transfusion (RBCx) leading to an almost complete recovery in a few days. The patient, however, had residual neurological sequelae with the presence of numb chin syndrome (NCS). This report thus highlights the need to recognize potential multiorgan failure secondary to steroid treatment and the importance of initiating treatment with red cell exchange transfusions to decrease the risk of such complications secondary to steroids.

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“…Patients with SCD are at an increased risk of developing thromboembolic diseases [5]. Fat embolism syndrome marked with abnormal coagulation profiles used in fat embolism scoring indices is an early warning sign of fulminant deterioration [9]. The pathophysiology of sickle cell-associated thromboembolic events is unclear but it is speculated that sickled RBCs increase the production of thrombin and its complexes, thereby increasing the hypercoagulability [10].…”

Section: Discussionmentioning

confidence: 99%

COVID-19 Infection and Acute Pulmonary Embolism in an Adolescent Female With Sickle Cell Disease

Kasinathan¹,

Ashraf²,

Minkowitz³

et al. 2020

Cureus

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Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review

Cited by 9 publications

References 71 publications

Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease

Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease

Fat Embolization Syndrome Secondary to Steroid Treatment in a Case of Sickle Cell Vaso-Occlusive Crisis

COVID-19 Infection and Acute Pulmonary Embolism in an Adolescent Female With Sickle Cell Disease

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