No presenting complaint or physical finding predicted which patients would succumb, however the lowest presenting hemoglobins (mean, 4.9 g/dL) were seen in the patients who died within 24 hours of presentation. [bloodjournal.org]

Areas covered: This review evaluates the epidemiology, clinical and laboratory presentation, etiology and pathogenesis of ACS. It also reviews the standard treatments as well as experimental treatments in ACS. [uncch.pure.elsevier.com]

Poster presented at: 2015 American Society of Hematology 57th Annual Meeting & Exposition. December 5, 2015; Orange County, CA. Shah VP, Tunik MG, Tsung JW. [emdocs.net]

Greater recognition of this syndrome in the growing number of adult sickle cell patients presenting for cardiac surgery may help improve their outcome. [ncbi.nlm.nih.gov]

The most common presenting symptoms of ACS are fever, non-productive cough, tachypnea, chest pain and shortness of breath. Arm and leg pain, abdominal pain and rib or sternal pain may also be present. [cancertherapyadvisor.com]

• Pain

  In patients admitted with a painful crisis, there is need for a high index of suspicion, as pain episodes may be a prodrome for the development of ACS. [uncch.pure.elsevier.com]

  Chest Pain 2. Fever > 38.5 3. Tachypnea, wheezing, cough, or increased work of breathing 4. Hypoxemia Management of Acute Chest Syndrome: 1. [errolozdalga.com]

  Pain Control Pain leads to hypoventilation that exacerbates ACS severity. To counteract pain, opioid analgesics are often used. 20 If opioids are to be used, attention should be given to proper dosing to prevent over-sedation and hypoventilation. [healio.com]

  No relationship was identified between allele lengths and pain rate. We provide evidence that genetic variation in HMOX1 is associated with decreased rates of hospitalization for ACS, but not pain. [ncbi.nlm.nih.gov]

• Fever

  He is tachycardic to 115 bpm and demonstrates fever to 38.3° C. His exam is otherwise unremarkable. Empiric antibiotics are administered and the patient is admitted for pain management and sepsis. [emdocs.net]

  Introduction: Acute chest syndrome (ACS), a leading cause of morbidity and mortality in sickle cell disease (SCD), is an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on a chest X-ray. [uncch.pure.elsevier.com]

  Acute chest syndrome (ACS) is defined as fever, respiratory symptoms and a new pulmonary infiltrate in an individual with sickle cell disease (SCD). [ncbi.nlm.nih.gov]

  Clinical findings In children: fever, tachycardia, chest pain, leukocytosis, and pulmonary infiltrates. [medical-dictionary.thefreedictionary.com]

• Hypoxemia

  Hypoxemia Management of Acute Chest Syndrome: 1. IVFs – similar to routine pain crises, goal is to achieve euvolemia first with isotonic fluids, then maintenance fluids. Avoid overhydration which can lead to pulmonary edema. 2. [errolozdalga.com]

  Severe acute chest syndrome afflicts patients with sickle cell disease and can cause hypoxemia refractory to conventional treatments. [ncbi.nlm.nih.gov]

  Correct hypoxemia with supplemental oxygen: goal PaO2 > 70 mmHg. [cancertherapyadvisor.com]

• Severe Pain

  General indications for exchange transfusion: Acute chest syndrome with severe pain refractory to simple transfusion, severe hypoxemia, multi-lobar disease, previous history of severe acute chest syndrome, or severe cardiopulmonary disease. [errolozdalga.com]

  Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. [bloodjournal.org]

  Pain Control Pain leads to hypoventilation that exacerbates ACS severity. To counteract pain, opioid analgesics are often used. 20 If opioids are to be used, attention should be given to proper dosing to prevent over-sedation and hypoventilation. [healio.com]

  […] episodes, treatment with the low molecular weight heparin, Tinzaparin, resulted in a significant reduction in the overall duration of painful crisis, number of days with the most severe pain scores, and duration of hospitalization compared with placebo [ssajm.org]

• Cough

  The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome. [en.wikipedia.org]

  The most common presenting symptoms were fever, cough, and chest pain (Table 1 ). While less common, many patients experienced shortness of breath, wheezing, hemoptysis, chills, and productive cough. [bloodjournal.org]

  Definition (MSH) Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. [fpnotebook.com]

  It is clinically defined as any new infiltrate in an SCA patient, associated with one or more new symptoms—e.g., fever, cough, sputum production, dyspnoea, or hypoxia. [medical-dictionary.thefreedictionary.com]

  The most common presenting features were fever (83%), pain (70%), and cough (61%), which changed with the number of ACS episodes. Children [ncbi.nlm.nih.gov]

• Tachypnea

  Definition (MSH) Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. [fpnotebook.com]

  Tachypnea, wheezing, cough, or increased work of breathing 4. Hypoxemia Management of Acute Chest Syndrome: 1. IVFs – similar to routine pain crises, goal is to achieve euvolemia first with isotonic fluids, then maintenance fluids. [errolozdalga.com]

  […] acute chest syndrome Sickle cell thalassemia disease with acute chest syndrome Clinical Information Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea [icd9data.com]

  See Sickle cell anemia. acute chest syndrome A complication of sickle cell disease resulting from vascular occlusion or infection in the lungs and marked by chest pain, tachypnea, fever, rales and rhonchi, leukocytosis, and lobar consolidation. [medical-dictionary.thefreedictionary.com]

  ACS is defined by the appearance of a new pulmonary infiltrate on CXR, associated with fever and respiratory symptoms such as tachypnea, wheezing, cough and pleuritic chest pain. [cancertherapyadvisor.com]

• Rales

  See Sickle cell anemia. acute chest syndrome A complication of sickle cell disease resulting from vascular occlusion or infection in the lungs and marked by chest pain, tachypnea, fever, rales and rhonchi, leukocytosis, and lobar consolidation. [medical-dictionary.thefreedictionary.com]

  Physical examination may reveal tachypnea, dyspnea, hypoxia, decreased air entry, wheezes, and rales. However, it is common not to find any of these physical signs in some young patients, particularly at ACS onset. [healio.com]

  66 42.9 Tachypnea 106 68.8 Productive cough 50 32.5 Retractions 18 11.7 Shortness of breath 38 24.7 Tachycardia 90 58.4 Whitish sputum 35 22.7 Dullness to percussion 11 7.1 Yellowish sputum 13 8.4 Decreased breath sounds 52 33.8 Greenish sputum 2 1.3 Rales [annsaudimed.net]

  The most frequent physical exam findings were rales and dullness to percussion. Notably, the second most common auscultory finding was a normal lung exam, which occurred in 35% of patients. [bloodjournal.org]

• Tachycardia

  Clinical findings In children: fever, tachycardia, chest pain, leukocytosis, and pulmonary infiltrates. [medical-dictionary.thefreedictionary.com]

  Raised temperature, tachypnea and tachycardia were the most common findings. ACS was associated with painful crisis (46.8%) and infections (13%). It was mild in 31.2%, moderate in 57.1% and severe in 11.7% of admissions. [annsaudimed.net]

  Also, commonly used bronchodilators can have side effects such as tachycardia, nervousness and hyperactivity and, in one reported case, myocardial infarction ( Corso 2005 ). [doi.org]

• Back Pain

  The EBG year reported higher use of hydroxyurea (P CONCLUSION: Mandatory IS for sickle cell disease patients admitted without respiratory complaints reduces transfusions and ACS, particularly for those presenting with back pain. [ncbi.nlm.nih.gov]

  The 2003 BCSH guidelines strongly advocate the use of incentive spirometry for patients with chest or back pain. [38] Did this answer your question? Yes No Additional feedback? (Optional) Thank you for your feedback! [medscape.com]

  When one administers opioids, it is tricky in patients with chest and back pain to achieve analgesia adequate to reduce respiratory splinting 53 while avoiding respiratory depression. [bloodjournal.org]

  During nitric oxide therapy, nitrogen dioxide was Case 2 A 9-yr-old girl with sickle cell disease presented with a 3-day history of fever, cough, and back pain. She developed right lower and middle lobe infiltrates and right pleural effusion. [anesthesiology.pubs.asahq.org]

• Leg Pain

  The most common presenting symptoms of ACS are fever, non-productive cough, tachypnea, chest pain and shortness of breath. Arm and leg pain, abdominal pain and rib or sternal pain may also be present. [cancertherapyadvisor.com]

Diagnostic workup includes chest x-ray, complete cell count, reticulocyte count, ECG, and blood and sputum cultures. Patients may also require additional blood tests or imaging (e.g. a CT scan) to exclude a heart attack or other pulmonary pathology. [en.wikipedia.org]

[…] deoxygenation of HbS, leading to its polymerization and to red blood cell sickling with subsequent vaso-occlusion, ischemia, and endothelial dysfunction. 10 A specific cause for ACS, however, is identified in less than half of the cases despite an extensive workup [healio.com]

• Pulmonary Infiltrate

  Clinicians should consider acute chest syndrome and sickle cell trait in the differential diagnosis of patients with unexplained respiratory failure and pulmonary infiltrates. [ncbi.nlm.nih.gov]

  infiltrate on a chest X-ray. [uncch.pure.elsevier.com]

  Definition (MSH) Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. [fpnotebook.com]

  Clinical findings In children: fever, tachycardia, chest pain, leukocytosis, and pulmonary infiltrates. [medical-dictionary.thefreedictionary.com]

• Air Bronchogram

  Consolidation with air bronchograms and a disrupted pleural line in longitudinal plane (C) and transverse plane (D). (Daswani et al. 2016). [emdocs.net]

• Staphylococcus Aureus

  Possible bacterial pathogens were identified in sputum cultures from less than half of the episodes; no pneumococci were found, and Staphylococcus aureus was the only bacterium associated with a longer illness than that seen when only normal flora were [jamanetwork.com]

  aureus Streptococcus pneumoniae Mycoplasma hominis Haemophilus influenza Legionella pneumophilia E. [hopkinsguides.com]

  Other organisms isolated were Hemophilus influenzae (1 of 11), Staphylococcus aureus (1 of 11), Staphylococcus epidermidis (1 of 11), Bacillus species (1 of 11) and both Enterobacter agglomerans and Acenetobacter (1 of 11). [annsaudimed.net]

  In this group, other causes of bacteremia included Staphylococcus aureus, Salmonella, Enterobacter, Hemophilus influenza, and Clostridia. [bloodjournal.org]

  In another ACS study, a single infectious agent was identified in 30% of patients. [14] In these patients, the most common cause of infection was Chlamydia (30%) followed by Mycoplasma (21%), respiratory syncytial virus (10%), Staphylococcus aureus (4% [ssajm.org]

• Blood Culture Positive

  Second, associated bacterial sepsis was common with nine patients having blood cultures positive for pneumococcus, three with gram negative rods, and one with group B Streptococcal sepsis. [bloodjournal.org]

Clinical trials using novel drugs for the treatment of ACS are greatly warranted. [uncch.pure.elsevier.com]

“Current approaches to antibiotic treatment in children with ACS (acute chest syndrome) vary widely, so more robust dissemination and implementation of existing treatment guidelines may reduce readmissions in this high-risk population,” the researchers [acepnow.com]

Standard treatment may include intravenous hydration, oxygen as treatment for hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given. [ncbi.nlm.nih.gov]

Bronchodilators may be useful but have not been well studied. [4] Prognosis [ edit ] It may result in death, [5] and it is one of the most common causes of death for people with sickle cell anemia. [6] References [ edit ] External links [ edit ] [en.wikipedia.org]

The overall prognosis for patients with sickle hemoglobinopathies has continued to improve. However, a large proportion of deaths occur during an episode of ACS. [ 1 ] The specific inciting etiology of ACS is difficult to discern. [anesthesiology.pubs.asahq.org]

The presence of multilobular, or bilateral, infiltrates is associated with a poorer prognosis 1. Patients with ACS generally characterized with severe hypoxemia. However, both hypoxemia and hypercapnia were observed in our patients. [ispub.com]

Prognosis The National Acute Chest Syndrome Study Group identified 538 patients with 671 episodes of ACS. The following outcomes were observed: Nearly half of the patients were initially admitted for another reason, usually pain crisis. [cancertherapyadvisor.com]

There is increasing knowledge regarding the etiology and pathogenesis of ACS in SCD. A high index of suspicion is required for the diagnosis of ACS. [uncch.pure.elsevier.com]

In the remainder of episodes (13 %), an identifiable etiology was found, usually bacterial pneumonia. [link.springer.com]

Etiologies of Acute Chest Syndrome: Majority are idiopathic. But, of those with identified cause: 1. Infection – both bacterial (including atypicals) and viral etiologies 2. Pulmonary Infarction 3. [errolozdalga.com]

Areas covered: This review evaluates the epidemiology, clinical and laboratory presentation, etiology and pathogenesis of ACS. It also reviews the standard treatments as well as experimental treatments in ACS. [uncch.pure.elsevier.com]

AREAS COVERED: This review evaluates the epidemiology, clinical and laboratory presentation, etiology and pathogenesis of ACS. It also reviews the standard treatments as well as experimental treatments in ACS. [ncbi.nlm.nih.gov]

It includes original translational, clinical, and epidemiologic research; public health, quality improvement, and case control studies; patient education research; and the latest research and standards of care for functional and genetic immune deficiencies [eurekalert.org]

Epidemiology One in every 650 African Americans is born with SCD and approximately 8% are heterozygous for the sickle cell gene. [cancertherapyadvisor.com]

This review comprehensively covers the epidemiology, clinical presentation, pathophysiology, risk factors, diagnosis, management, prevention, and outcome of ACS. [healio.com]

Disruption of microvascular integrity is critical to the pathophysiology of ACS, but the factors governing its phenotypic variability are incompletely understood. [ncbi.nlm.nih.gov]

The pathophysiology and management of SCA, vaso-occlusive crisis, and pulmonary and neurologic complications of the disease are discussed. [nursingcenter.com]

Patients with a diagnosis of ACS should be aggressively managed to prevent clinical deterioration. [uncch.pure.elsevier.com]

Risk factors of ACS and effective preventive and therapeutic strategies to prevent recurrent episodes in children with sickle cell disease are presented in a new article published in Pediatric Allergy, Immunology, and Pulmonology. [medicalxpress.com]

Help prevent ACS: Take all medicines as directed. You may need to take medicine to help your body make red blood cells that are less likely to sickle. This may help prevent ACS. [drugs.com]

She’ll give you regular blood tests, and she may need to adjust your dosage to prevent heart problems, infections, and seizures. [webmd.com]