Chest
Clinical InvestigationsDIFFUSE LUNG DISEASEAcute Chest Syndrome in Adults With Sickle Cell Disease
Section snippets
Patients
All patients were recruited from the Sickle Cell Disease Center at the Henri Mondor University Hospital, Créteil, France, which follows a cohort of 800 adult patients. All consecutive ACS episodes in this cohort were recorded from 1991 to 1997. Hemoglobin (Hb) phenotype was determined by standard procedure to be homozygous Hb SS, Hb SC, or Hb Sβ-thalassemia. β-globin cluster haplotypes were determined as previously described.20 The diagnosis of ACS was based on the presence of fever or chest
Results
One hundred seven consecutive episodes of ACS were considered in 77 patients. Of these patients, 52 had one ACS episode, 17 had two episodes, and 7 had three episodes. For 14 patients (20%), ACS was the first severe acute event related to SCD. Table 1 shows the baseline characteristics of the 77 patients before the first episode. The haplotype distribution of patients presenting ACS was similar to that of the total patient population followed at our center. Only three patients received chronic
Discussion
The aim of this study was to determine the characteristics and outcome of ACS in adult patients. Recent studies, particularly the report of the Cooperative Study of Sickle Cell Disease (CSSCD), have described ACS clinical features in large numbers of patients.5, 1415, 1617, 1819 However, most of these studies have focused on patients < 16 years old. In contrast, our monocentric study included only adult patients treated according to an uniform protocol in which transfusion was used only for the
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Cited by (97)
Acute chest syndrome in adult sickle cell patients
2022, Revue de Medecine InterneHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease
2022, American Journal of Emergency MedicineCitation Excerpt :Common findings on chest radiograph include lobar, segmental, or multilobar consolidations in the lower lobes, as well as atelectasis with pleural effusion (Fig. 1) [30,76-78]. Pediatric patients more commonly demonstrate upper or middle lobe disease but are less likely to have pleural effusions [3,12,79]. Chest radiograph has a sensitivity over 85% and specificity <60% for finding a radiodensity [80], and radiographic signs may be absent early in the course of the disease and underestimates the degree of hypoxemia [6,81,82].
Molecular testing for respiratory pathogens in sickle cell disease adult patients presenting with febrile acute chest syndrome
2020, Medecine et Maladies InfectieusesCitation Excerpt :An empirical antibiotic course was introduced in 52 patients (85.2%) and was later adapted according to the bacterial detection results in nine patients. The mean time from initial empirical therapy to adapted specific therapy was 3.1 [2–7] days. Overall, prescribed antibiotics were amoxicillin (n = 30), amoxicillin-clavulanic acid (n = 9), cefotaxime (n = 18), spiramycin (n = 22), telithromycin (n = 8), levofloxacin (n = 1), piperacillin-tazobactam (n = 1), oxacillin (n = 1), and linezolid (n = 1).
Recommendations for the use of red blood cell exchange in sickle cell disease
2019, Transfusion and Apheresis ScienceAcute Hypoxemia and Coma in a Patient With Hemoglobin SC Disease
2019, ChestCitation Excerpt :Since its features are nonspecific, other causes of hypoxemia such as pneumonia, aspiration, pulmonary edema, and thromboembolism must be excluded. More specific evidence of FES may be provided by the presence of abundant fat in macrophages obtained by bronchoalveolar lavage,3 brain MRI that shows a typical “starfield” pattern of diffuse, punctate, hyperintense lesions on diffusion weighted imaging,4 or retinal lesions on dilated eye examination. In the present case, the finding of acute cor pulmonale on bedside cardiac ultrasound with a negative CT pulmonary angiogram provided compelling evidence for FES and led to prompt institution of exchange transfusion, a potentially life-saving intervention in patients with catastrophic FES.1,5
Manifestations of sickle cell disease on thoracic imaging
2018, Clinical Imaging