Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Outcomes. The Experience of a Single Thalassemia and Sickle Cell Unit in a University Hospital.

Acute chest syndrome (ACS) is a common cause of death for sickle cell disease patients. This syndrome is defined as: respiratory symptoms, new X-ray findings developed and/or fever; ACS requires prompt treatment to avoid clinical deterioration and death in adults with sickle cell disease. Sixteen episodes of acute chest syndrome were studied in 16 adults with sickle cell disease. The clinical and radiological findings, treatment, response and outcome of the episode were evaluated respectively. The patient's past history and comorbidities were taken into account in the outcome and days of hospitalization. Fourteen patients recovered with no sequelae; one patient who required mechanical ventilation also recovered; one patient died due to pulmonary emboli. The mean hospitalization days were 7.43.

Veno-Venous Extracorporeal Membrane Oxygenation in Adult Patients with Sickle Cell Disease and Acute Chest Syndrome: a Single-Center Experience.

Acute chest syndrome (ACS) in adult patients with sickle cell disease represents a leading cause of death. It is characterized by a new density on chest X-ray accompanied by fever and/or respiratory symptoms. Currently, 49 adult patients with sickle cell disease are registered at our department. By now, 12 patients (24.5%) suffered from ACS and two patients showed multiple/recurrent (>2) episodes. Death in one patient was related to acute respiratory failure secondary to ACS. In three patients with ACS, invasive mechanical ventilation and subsequent veno-venous extracorporeal membrane oxygenation (VV-ECMO) was mandatory. Veno-venous ECMO was applied within 24 hours upon arrival to the intensive care unit (ICU). All patients were treated aggressively for ACS including exchange transfusions [packed red blood cell (pRBC) units 5-16] maintaining a Hb S threshold of <30.0% in addition to broad-spectrum antibiotics, resulting in a successful outcome following decannulation from VV-ECMO (49 hours, 251 hours, 30 min., and 98 hours, respectively). Limited information is presently available on the use of VV-ECMO in adult patients with sickle cell disease in the context of acute respiratory failure secondary to ACS. The adequate timing of the decision to place ECMO in critically ill adults with sickle cell disease, incapable of being treated by conventional mechanical ventilation secondary to very severe vaso-occlusive crisis (VOC), might further reduce mortality rates while treating the underlying condition.

How I Treat Acute Chest Syndrome in Asthmatic Children with Sickle Cell Disease. A Practical Review.

Asthma is associated with increased rate of acute chest syndrome (ACS), pain episodes and premature death. Differentiating between an acute asthma exacerbation and ACS is a challenge clinically as they can present with similar symptoms. Clinicians should be aware of symptoms of asthma or broncho spasm in any children with sickle cell disease, as adequate treatments are required. In this mini-review, we selected 16 clinical studies, published in English between 2004 and 2016, and reviewed all of the abstracts and references of the selected articles. We subsequently selected articles that were focused on asthma in children with sickle cell disease. Given the pathophysiological mechanisms of ACS and the association between asthma and sickle cell disease, the management approach of asthmatic children should be clarified. Bronchodilators should be used if there are clinical features suggestive of a history of asthma or evidence of acute broncho spasm. The indication for cortisone should be reassessed. This literature review failed to conclude on therapeutic modalities of ACS in asthmatic children with sickle cell disease. Only a well designed, multicenter adequately-powered randomized controlled study of each of them will allow assessing their real benefits and risks.

Successful Use of Veno-Venous Extracorporeal Membrane Oxygenation in an Adult Patient with Sickle Cell Anemia and Severe Acute Chest Syndrome.

A 25-year-old female with sickle cell anemia presented with respiratory failure due to acute chest syndrome (ACS). Given her severely compromised cardiopulmonary status, she was started on veno-venous extracorporeal membrane oxygenation (VV-ECMO). After 20 days, the patient's respiratory status improved and she was successfully decannulated. Veno-venous extracorporeal membrane oxygenation can be utilized for severe ACS in adult patients with sickle cell disease. Prompt initiation of this modality may improve outcomes in adult patients with sickle cell disease complicated by life threatening ACS.

Crisis in the Chest: Acute Chest Syndrome as a Sequela of Tricuspid Valve Endocarditis.

The management of acute chest syndrome (ACS) in sickle cell disease occurring concurrently with pulmonary embolism resulting from tricuspid valve endocarditis poses an atypical challenge. We present a case in which this complex interaction occurs and the prompt interventions that were utilized to give the best possible outcome.

Cocaine-Induced Isolated Bradycardic Atrial Flutter: A Rare Presentation in a Patient With a History of Acute Coronary Syndrome.

Six months ago, a middle-aged African American male visited the cardiology clinic for a follow-up on acute coronary syndrome along with atrial fibrillation. The patient was initially diagnosed with unstable angina with palpitation and underwent cardiac catheterization. During the visit, the patient complained of unspecific chest discomfort, palpitation, and reduced exercise tolerance after the use of cocaine for several months. ECG showed the absence of atrial fibrillation but instead showed atrial flutter with bradycardia. Cocaine-induced atrial flutter was suspected. The patient was educated about the imperative need to discontinue cocaine use immediately. Additionally, appropriate measures for rate control and anticoagulation were initiated.

Analysis of Sensitivity, Specificity, and Predictive Values of High-Sensitivity Troponin T in a Secondary Care Setting: A Retrospective Cohort Study.

Background High-sensitivity cardiac troponin (hs-cTn) assays have significantly improved the early detection of myocardial injury and the diagnosis of acute coronary syndrome (ACS). Different diagnostic algorithms exist for the interpretation of hs-cTn in the management of patients with suspected ACS. This study analysed the diagnostic efficacy of hs-cTn using serial and single measurements while also shedding light on the challenges associated with the use of this assay. Methods  We reviewed 189 results belonging to 120 unique patient episodes and records for troponin tests performed in a two-week period obtained from the West Cumberland Hospital, North Cumbria Integrated Care (NCIC), Whitehaven, England. These troponin tests were carried out based on the NCIC trust guidelines for the use of troponin assays in the management of acute coronary syndrome (ACS). A positive troponin test is defined using the NCIC trust guidelines and the National Academy of Clinical Biochemistry (NACB) guidelines. The case notes of the unique patients were reviewed to determine the outcome, which is defined as the clinical diagnosis on discharge of the patient following a cardiologist review. These outcomes were then used to calculate the sensitivity, specificity, and predictive values. We also determined the alternate diagnosis for false-positive tests. Results Using both guidelines to assess the clinical effectiveness of the troponin assay yields slightly varying results, with the single positive test of NACB demonstrating a higher sensitivity of 92.8% (>71.4%) and a slightly better negative predictive value of 97.8% (>96%). However, using the serial troponin measurements as per the NCIC trust guideline demonstrates a better specificity of 95.2% (>42.4%) and a positive predictive value of 66% (>17.5%). False positive results are identified, which are due to alternate diagnoses such as stable angina, myocarditis, heart failure, sepsis, and malignancy. Conclusion  High-sensitivity troponin (hs-cTn) assays play a crucial role in the early detection and management of patients with suspected ACS. This study supports evidence that serial troponin measurements are more diagnostically accurate than single troponin measurements. Although hs-cTn assays offer significant advantages, there remain challenges and limitations that require careful interpretation and clinical correlation.

Unraveling the Link Between Fibromuscular Dysplasia and Acute Coronary Syndrome: A Comprehensive Case Report and Analysis.

We present a rare case of fibromuscular dysplasia (FMD) manifesting in the mid to distal segment of the left anterior descending (LAD) artery, which led to the development of acute coronary syndrome (ACS) in our patient, highlighting the severe consequences of this vascular disorder. During the investigation of the patient's clinical symptoms, an unexpected incidental finding emerged, indicating bilateral FMD involvement of the renal arteries. This serendipitous discovery underscores the importance of comprehensive evaluation and thorough exploration when managing patients with FMD. We aim to shed light on the intriguing nature of FMD and emphasize the need for vigilant assessment to identify potential multi-vessel abnormalities, even beyond the primary affected site. We also aim to highlight the coronary artery manifestation of FMD as ACS and discuss its medical management.

Adverse Drug Effect Profiles of Gp2b/3a Inhibitors: A Comparative Review of the Last Two Decades.

ST-Elevation Myocardial Infarction and non-ST Elevation Myocardial Infarction belong to the acute coronary syndrome group of diseases. These conditions are characterized by the complete or partial blockage of one or several coronary arteries, resulting in myocardial injury or necrosis. Various medications are used in their treatment, with the most recent addition being Glycoprotein IIb/IIIa inhibitors. They work by hindering the activity of glycoprotein IIb/IIIa receptors, which, in turn, prevents the clumping of platelets. Some of the GpIIb/IIIa inhibitors available in this category include abciximab, tirofiban, eptifibatide, roxifiban, and orbofiban. With this comprehensive literature review, we aimed to explore the potential adverse effects of these medications and compare the three in terms of their side effects profile. We searched through PubMed and Google Scholar and pinpointed 13 articles aligned with our inclusion criteria: six articles utilized eptifibatide, four were related to abciximab, and three used tirofiban. In 85% of the cases, a severe drop in platelet count, reaching as low as 1000/µL, was reported. Additionally, several other side effects were noted: one case documented multiple bruising spots appearing around the patient's body, two cases reported diffuse alveolar hemorrhage, and one case described a cardiac tamponade resulting from hemorrhagic pericarditis. Our study highlights the crucial significance of keeping a watchful eye on and comprehending the potential drawbacks linked to these medications in cardiovascular treatment. The necessity of researching these medications and their side effects is also evident, as this will significantly enhance the quality of treatment provided.

Management of acute chest syndrome in patients with sickle cell disease: a systematic review of randomized clinical trials.

INTRODUCTION: Acute chest syndrome (ACS) accounts for the highest mortality in Sickle cell disease patients. Early diagnosis and timely management of ACS results in better outcomes. However, the effectiveness of most treatment modalities for ACS management has not been established. AREAS COVERED: To review the treatment modalities management protocols and highlight the effectiveness of each option a literature search was done. Randomized controlled trials that assessed the efficacy of different treatment modalities in ACS management in SCD patients were chosen and reviewed. EXPERT OPINION: 11 randomized controlled trials were found that evaluated the efficacy of incentive spirometry, positive expiratory pressure device, intravenous dexamethasone, oral vs. intravenous morphine, inhaled nitric oxide, unfractionated heparin, and blood transfusion in the prevention or treatment of ACS. Although there are guidelines for ACS treatment, the available evidence is very limited to delineating the effectiveness of various interventions in ACS management. More high-quality studies and trials with a larger patient population can benefit this area to support the recommendations with stronger evidence.

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

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 (HbSßThal), 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.

Sickle Cell Disease and Its Respiratory Complications.

Sickle cell disease (SCD) is a hematological disorder that is inherited in an autosomal recessive (AR) fashion. It is caused by mutations in the genes encoding for the globin apoprotein of hemoglobin (Hb), leading to diminished oxygen-carrying ability. Its pathophysiologic mechanism affects multiple organ systems, making it crucial to understand the complications of SCD and find the best ways to prevent and treat them. Some important ways that SCD manifests in the respiratory system are acute chest syndrome (ACS), pulmonary hypertension (PH), asthma, and venous thromboembolism (VTE). This article summarizes their salient features, including pathogenesis related to the adverse outcomes, screening practices, and management guidelines, with the intent to provide greater insight into forming better practices that increase the quality of life in SCD patients.

Convergence of Inflammatory Pathways in Allergic Asthma and Sickle Cell Disease.

The underlying pathologies of sickle cell disease and asthma share many characteristics in terms of respiratory inflammation. The principal mechanisms of pulmonary inflammation are largely distinct, but activation of common pathways downstream of the initial inflammatory triggers may lead to exacerbation of both disease states. The altered inflammatory landscape of these respiratory pathologies can differentially impact respiratory pathogen susceptibility in patients with sickle cell disease and asthma. How these two distinct diseases behave in a comorbid setting can further exacerbate pulmonary complications associated with both disease states and impact susceptibility to respiratory infection. This review will provide a concise overview of how asthma distinctly affects individuals with sickle cell disease and how pulmonary physiology and inflammation are impacted during comorbidity.