Acute chest syndrome

Potentially lethal blockage of lung vasculature in sickle cell anaemia

Acute chest syndrome
[[File:|250px|alt=|]]
Synonyms
Pronounce
Field
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths

The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell anemia. This condition commonly manifests with a new opacification of the lung(s) on a chest x-ray.<ref name="Pace2007">{{{last}}},

 Betty Pace, 
  
 Renaissance of Sickle Cell Disease Research in the Genome Era. online version, 
  
 Imperial College Press, 
 2007, 
  
  
 ISBN 978-1-86094-645-5, 
  
  
  
 Pages: 81–,</ref>

Signs and symptoms

The crisis is a common complication in sickle-cell patients and can be associated with one or more symptoms including fever, cough, excruciating pain, sputum production, shortness of breath, or low oxygen levels.<ref> Johnson, CS. Sickle-Cell Disease: The Acute Chest Syndrome(link). {{{website}}}.

</ref>

Cause

Acute chest syndrome is often precipitated by a lung infection, and the resulting inflammation and loss of oxygen saturation leads to further sickling of red cells, thus exacerbating pulmonary and systemic hypoxemia, sickling, and vaso-occlusion. ^{(July 2020)}

Diagnosis

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. The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome. 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. ^{(July 2020)}

Prevention

Hydroxyurea is a medication that can help to prevent acute chest syndrome. It may cause a low white blood cell count, which can predispose the person to some types of infection.<ref>Sickle cell disease (SCD). Centers for Disease Control and Prevention. Retrieved January 7, 2015.</ref>

Treatment

Broad spectrum antibiotics to cover common infections such as Streptococcus pneumoniae and mycoplasma, pain control, and blood transfusion. Acute chest syndrome is an indication for exchange transfusion.

Bronchodilators may be useful but have not been well studied.<ref>,

 Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease., 
 The Cochrane Database of Systematic Reviews, 
 
 Vol. 8(Issue: 8),
 pp. CD003733,
 DOI: 10.1002/14651858.CD003733.pub3,
 PMID: 25086371,</ref>

Prognosis

It may result in death,<ref>"acute chest syndrome" at Dorland's Medical Dictionary </ref> and it is one of the most common causes of death for people with sickle cell anemia.<ref>Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631</ref>

External links

   This article is a medical stub. You can help WikiMD by expanding it!