title: Sickle cell disease-related organ damage occurs irrespective of pain rate: implications for clinical practice.
abstract: In daily clinical practice, the frequency of painful crises (pain rate) is an important parameter of sickle cell disease severity. We assessed the prevalence of sickle cell disease-related organ damage and complications and their relation to pain rate. Organ damage and history of vaso-occlusive complications were obtained via systematic screening of consecutive patients and by chart review. In 104 adult sickle cell patients pain rate was related to a history of acute chest syndromes, avascular osteonecrosis, iron overload, priapism and cholelithiasis. However, major disease-related complications, such as microalbuminuria and pulmonary hypertension, were detected in 23% and 24% respectively of patients without painful crises in the study period underlining the importance of systematic screening for developing organ damage in sickle cell patients irrespective of pain rate.
authors: Department of Haematology, F4-224, Academic Medical Center, PO box 22660, 1100 DD Amsterdam, The Netherlands. b.j.biemond@amc.uva.nl
authors: Eduard J
authors: EJ
authors: van Beers
authors: Charlotte F J
authors: CF
authors: van Tuijn
authors: Melvin R
authors: MR
authors: Mac Gillavry
authors: Anna
authors: A
authors: van der Giessen
authors: John-John B
authors: JJ
authors: Schnog
authors: Bart J
authors: BJ
authors: Biemond
mesh_terms: D000328:Adult; D000418:Albumins / Q000378:metabolism; D000755:Anemia, Sickle Cell / Q000150:complications / Q000473:pathology* / Q000628:therapy; D015897:Comorbidity; D003404:Creatinine / Q000652:urine; D005260:Female; D005838:Genotype; D006801:Humans; D006976:Hypertension, Pulmonary; D019190:Iron Overload; D008297:Male; D010020:Osteonecrosis / Q000473:pathology; D010146:Pain; D010147:Pain Measurement; D013789:Thalassemia / Q000150:complications / Q000473:pathology* / Q000628:therapy
chemical_list: D000418:Albumins; D003404:Creatinine