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]

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]

• Hemophilia A

  In "Acute Chest Syndrome in Children with Sickle Cell Disease," coauthors Shilpa Jain, Women and Children's Hospital of Buffalo and Hemophilia Center of Western New York, Buffalo, and Nitya Bakshi and Lakshmanan Krishnamurti, Children's Healthcare of [medicalxpress.com]

  Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia [medscape.com]

  In " Acute Chest Syndrome in Children with Sickle Cell Disease ," coauthors Shilpa Jain, Women and Children's Hospital of Buffalo and Hemophilia Center of Western New York, Buffalo, and Nitya Bakshi and Lakshmanan Krishnamurti, Children's Healthcare of [eurekalert.org]

• Pain

  Painful episodes which require hospitalization are most often treated with opioids, which may then influence the development of ACS. Nalbuphine is a parenteral opioid which effectively treats pain and may cause less ACS. [ncbi.nlm.nih.gov]

  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]

  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]

  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]

• Fever

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

  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]

• Hypoxemia

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

  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]

  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]

  Society. 76, 96 NSAIDs solely can be used to treat mild–moderate pain with rapid addition of opioids if there is progression to severe pain. [ncbi.nlm.nih.gov]

• Falling

  In acute chest syndrome, arterial blood oxygen saturation commonly falls to a greater degree than that seen in simple pneumonia of the same magnitude. [medscape.com]

  This results in the fall in average temperatures to as low as 13°C with many daily temperature values reaching as low as 3°C especially in the evenings and night times. [42] Treatment of acute chest syndrome In most cases, treatment for ACS is largely [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]

  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]

  They may also have a fever, cough or trouble breathing. ACS can be triggered by a lung infection like pneumonia.It may also happen before, during or after a pain episode. [nationwidechildrens.org]

• Pneumonia

  Explore these free sample topics: -- The first section of this topic is shown below -- PATHOGENS The most common etiologies of acute chest syndrome are Unknown (46%) Infarction (16%) Fat Embolism (9%) Chlamydia pneumoniae (7%) Mycoplasma pneumonia (7% [hopkinsguides.com]

  H. influenzae, Mycoplasma pneumoniae and Chlamydia pneumoniae. [cancertherapyadvisor.com]

  S pneumoniae –preventable by pneumococcal vaccine, Mycoplasma pneumoniae, and others. [medical-dictionary.thefreedictionary.com]

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

  Diagnosis [ edit ] The diagnosis of acute chest syndrome is made difficult by its similarity in presentation with pneumonia. Both may present with a new opacification of the lung on chest x-ray. [en.wikipedia.org]

• Dyspnea

  A 41-year-old African male with previously unknown hemoglobinopathy developed an acute episode of fever, chest pain and dyspnea right after surgery for retinal detachment. The patient was suspected of having pulmonary thromboembolism. [ncbi.nlm.nih.gov]

  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]

  Dyspnea and chest pain are found to be significant predictors of acute chest syndrome, P < 0.05. In addition to asthma, history of splenectomy and surgery (P= 0.001, and < 0.05 respectively). [pjms.com.pk]

  This prompted us to treat him with nebulized albuterol, which improved his dyspnea and aeration. [jamanetwork.com]

  […] 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, or dyspnea [icd9data.com]

• 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]

  […] 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]

  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]

  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]

  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]

  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]

• Chest Pain

  Acute chest syndrome, characterized by fever, chest pain and pulmonary infiltrates, is a known complication of hemoglobin SC disease. [ncbi.nlm.nih.gov]

  Acute chest syndrome can occur in any haemoglobinopathy—e.g., Hb SS, Hb SC, Hb S ß+-thalassemia, Hb S ß0-thalassemia, etc. Clinical findings In children: fever, tachycardia, chest pain, leukocytosis, and pulmonary infiltrates. [medical-dictionary.thefreedictionary.com]

  Acute chest syndrome (ACS) is a pulmonary illness with fever, chest pain, leukocytosis and new pulmonary opacity in a patient with sickle cell disease. [link.springer.com]

  Pain treatment with heavy doses of opioid medicine. Treatment of acute chest syndrome includes: Encouraging deep breathing. This is especially important when a person has chest pain or drowsiness and shallow breathing from opioid medicine. [healthlinkbc.ca]

• 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]

• Seizure

  His course was complicated by a seizure associated with MRI abnormalities, although the outcome has been excellent. [ncbi.nlm.nih.gov]

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

As part of the initial workup, a child with SCD presenting with fever should have a CBC, reticulocyte count, blood culture, basic biochemistry, type and crossmatch, and a chest X-ray, irrespective of respiratory symptoms. [ncbi.nlm.nih.gov]

• 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]

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

• Hypercapnia

  However, both hypoxemia and hypercapnia were observed in our patients. Increased dead space ventilation due to vasooclusion in pulmoner microcirculation and respiratory muscular fatigue because of hyper ventilation may cause hypercapnia. [ispub.com]

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

treatment;Further objectives are to determine whether there are important variations in efficacy and safety:3. for different treatment regimens,4. by participant age, or geographical location of the clinical trials. [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]

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]

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]

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