Sickle Cell Disease and Acute Chest Syndrome: Mechanisms and Pathogenenesis

Adisa, Olufolake; Owusu-Ansah, Amma; Abrahams, Afua O.D.; Ghosh, Samit; Ofori-Acquah, Solomon Fiifi

doi:10.1007/978-3-319-41912-1_3

Cited by 1 publication

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“…Lung consolidations in ACS are due to the presence of fluids, proteinaceous exudates, or other products of disease that replace alveolar airspace; these cause the lung to become solid. [1920]…”

Section: Discussionmentioning

confidence: 99%

Radiological patterns in sickle cell disease patients with acute chest syndrome: Are there age-related differences?

Al-Sharydah

Alshahrani

Al-Dhaferi

et al. 2019

Saudi J Med Med Sci

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Background: Acute chest syndrome is a major cause of pulmonary disease and mortality in sickle cell disease patients. Its diagnosis can be delayed due to differing imaging patterns between children and adults. Objective: The purpose of this study was to describe the pulmonary and extrapulmonary imaging findings in sickle cell disease patients with acute chest syndrome and determine differences in findings between adult and pediatric patients. Patients and Methods: This retrospective study analyzed the data of all sickle cell disease patients who were admitted with a diagnosis of acute chest syndrome to King Fahd Hospital of the University, Al Khobar, Saudi Arabia, between January and June 2015 ( n = 150). After grouping the patients into adults and pediatrics, the pulmonary and extrapulmonary characteristics were identified and the digital radiography, computed tomography and laboratory findings were compared. Results: A total of 116 patients with 163 acute chest syndrome episodes met the inclusion criteria, of which 69 (60%) were adults. In both adult and pediatric patients, the most frequent pulmonary finding was consolidation of the lung parenchyma. The right lung was most frequently involved: the lower lobe in adult patients and the middle lobe in pediatric patients. In addition, pleural effusion was observed in both age groups. Extrapulmonary radiological findings, such as avascular necrosis and cardiomegaly, were significantly more common in adult patients than in pediatric patients ( P < 0.05). Compared with adults, pediatric patients had significantly lower hemoglobin levels ( P = 0.001) and oxygen tension fraction in arterial blood ( P = 0.007). Conclusions: Pediatric and adult sickle cell disease patients with acute chest syndrome typically exhibited similar pulmonary characteristics, whereas extrapulmonary findings were more prominent in adult patients. Furthermore, low levels of hemoglobin and oxygen tension fraction were dependent predictors of acute chest syndrome.

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Section: Discussionmentioning

confidence: 99%