✦ LIBER ✦

The lung in sickle cell disease

✍ Scribed by Jennifer Knight; Thomas M. Murphy; Iley Browning

Publisher: John Wiley and Sons
Year: 1999
Tongue: English
Weight: 241 KB
Volume: 28
Category: Article
ISSN: 8755-6863
DOI: 10.1002/(sici)1099-0496(199909)28:3<205::aid-ppul8>3.0.co;2-z

No coin nor oath required. For personal study only.

✦ Synopsis

Sickle cell disease is the most common inherited disorder in African-Americans. Although the primary defect is hematological, the changes in the erythrocytes lead to a vasculopathy with multiorgan injury. The pulmonary complications, i.e., acute chest syndrome and chronic sickle cell lung disease, are significant causes of morbidity and mortality. The pulmonary manifestations result from a unique constellation of factors which come into play in sickle cell disease. Based on the growing understanding of the molecular and cellular biology of sickle cell disease, new therapies are being developed that are likely to ameliorate the natural history of this disease and its complications.

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