Finally, a consensus statement on sickle cell disease manifestations: a critical step in improving the medical care and research agenda for individuals with sickle cell disease.

摘要

The combined effort of the investigators from the Com- i prehensive Sickle Cell Disease Centers should be , applauded for their mission to establish standard definitions of medical complications associated with sickle cell dis- i ease. Their accomplishment, published in this issue of , American Journal of Hematology, stands as a landmark for both clinical and translational research in sickle cell disease [1]. Far too often, families, healthcare providers, and clinicians are compelled to compare the results from one study i to another without knowing whether the primary endpoints are the same. Even worse, the same presentation of a sickle cell complication is often clinician dependent. For example, a child who presents with a new radiodensity on radiograph, fever, and wheezing may be discharged 2 days later with a diagnosis of bronchiolitis by one physician, yet the same presentation may be called acute chest syndrome by another. Patients and their families are only confused when these inconsistencies persist, resulting in miscommu-nication and distrust. A list of the various definitions of acute chest syndrome used by the six most commonly cited manuscripts is described in Table I. Until the article published by Ballas et al., none of the previous definitions addressed the severity of the episode or whether the pul-monic process was unrelated to sickle cell disease.