Laboratory characteristics of patient groups
| Variable . | CSSCD, n = 3380 . | BMC, n = 140 . | NIH-PHT, n = 210 . | |||
|---|---|---|---|---|---|---|
| Mean . | SD . | Mean . | SD . | Mean . | SD . | |
| Age, y | 20.00 | 14.04 | 21.40 | 14.91 | 35.99* | 11.45 |
| ALT level, (Sgpt), U/L | 35.03 | 52.53 | 25.72* | 24.37 | 27.09* | 14.49 |
| AST level, (Sgot), U/L | 44.51 | 23.74 | 45.14 | 30.88 | 41.71 | 21.74 |
| Bilirubin level, mg/dL | 2.71 | 1.89 | 3.10 | 3.19 | 2.75 | 1.74 |
| BUN level, mg/dL | 9.36 | 5.36 | 9.68 | 4.73 | 10.76 | 10.62 |
| Creatinine level, mg/dL* | 0.70 | 0.64 | 0.48* | 0.35 | 0.93† | 1.30 |
| Hb level, g/dL | 9.13 | 1.81 | 11.71 | 24.37 | 9.44 | 1.88 |
| % HbF | 6.12 | 5.78 | 6.15 | 7.57 | 7.41 | 6.96 |
| LDH level, U/L† | 459.51 | 206.34 | 406.7† | 289.89 | 361.9* | 164.04 |
| MCV, fL | 87.42 | 8.75 | 88.03 | 11.36 | 91.94* | 11.30 |
| Platelet count, 109/L | 399.30 | 131.17 | 372.70 | 156.30 | 365.30 | 140.13 |
| Reticulocyte count, % RRC | 9.56 | 5.85 | 9.05 | 9.11 | 250.1† | 130.13 |
| Sys BP, mmHg* | 105.70 | 12.35 | 115.9* | 14.91 | 120.5* | 17.82 |
| WBC count, 109/L | 11.17 | 3.31 | 10.94 | 4.73 | 10.39† | 3.62 |
| Variable . | CSSCD, n = 3380 . | BMC, n = 140 . | NIH-PHT, n = 210 . | |||
|---|---|---|---|---|---|---|
| Mean . | SD . | Mean . | SD . | Mean . | SD . | |
| Age, y | 20.00 | 14.04 | 21.40 | 14.91 | 35.99* | 11.45 |
| ALT level, (Sgpt), U/L | 35.03 | 52.53 | 25.72* | 24.37 | 27.09* | 14.49 |
| AST level, (Sgot), U/L | 44.51 | 23.74 | 45.14 | 30.88 | 41.71 | 21.74 |
| Bilirubin level, mg/dL | 2.71 | 1.89 | 3.10 | 3.19 | 2.75 | 1.74 |
| BUN level, mg/dL | 9.36 | 5.36 | 9.68 | 4.73 | 10.76 | 10.62 |
| Creatinine level, mg/dL* | 0.70 | 0.64 | 0.48* | 0.35 | 0.93† | 1.30 |
| Hb level, g/dL | 9.13 | 1.81 | 11.71 | 24.37 | 9.44 | 1.88 |
| % HbF | 6.12 | 5.78 | 6.15 | 7.57 | 7.41 | 6.96 |
| LDH level, U/L† | 459.51 | 206.34 | 406.7† | 289.89 | 361.9* | 164.04 |
| MCV, fL | 87.42 | 8.75 | 88.03 | 11.36 | 91.94* | 11.30 |
| Platelet count, 109/L | 399.30 | 131.17 | 372.70 | 156.30 | 365.30 | 140.13 |
| Reticulocyte count, % RRC | 9.56 | 5.85 | 9.05 | 9.11 | 250.1† | 130.13 |
| Sys BP, mmHg* | 105.70 | 12.35 | 115.9* | 14.91 | 120.5* | 17.82 |
| WBC count, 109/L | 11.17 | 3.31 | 10.94 | 4.73 | 10.39† | 3.62 |
Patients enrolled in the CSSCD included children aged younger than 1 year and adults with sickle cell anemia with or without coincident α thalassemia (deletion of 1 or 2 α-globin genes [HBA2, HBA1]) and also patients with HbSC disease. Two additional independent, longitudinally followed contemporaneous patient groups were examined. One-hundred forty patients were from BMC and 210 patients participated in the NIH-PHT. These patients also had either sickle cell anemia or HbSC disease. The NIH-PHT patients had echocardiographic assessment of pulmonary hypertension. Reticulocyte counts in the NIH-PHT patients are presented as absolute numbers. This data was not available in the CSSCD. To convert to SI units: bilirubin, ×17.1 (μmol/L); BUN, ×0.357 (mmol/L); creatinine, ×88.4 (μmol/L); hemoglobin, ×10 (g/L). The reticulocytes are expressed as a percent of all red blood cells.
CSSCD indicates Cooperative Study of Sickle Cell Disease; BMC, Boston Medical Center; NIH-PHT, NIH-Pulmonary Hypertension Screening Study; ALT, alanine aminotransferase; Sgpt, serum glutamic pyruvic transaminase; AST, aspartate aminotransferase; Sgop, serum glutamic oxoloacetic transaminase; BUN, blood urea nitrogen; Hb, hemoglobin; and Sys BP, systolic blood pressure.
P < .001 relative to the CSSCD data.
01 < P < .05 relative to the CSSCD data.