Pathologic findings in control and SS mice
. | Genotype . | . | . | |
|---|---|---|---|---|
. | Control; n = 16 . | Sickle; n = 15 . | P . | |
| Spleen | ||||
| Gross infarcts, % positive | 0 | 27 | .04 | |
| Micros infarct, % positive | 0 | 40 | .007 | |
| Diameter, mm | 2.2 | 5.7 | < .001 | |
| Iron, all ages | 1.9 | 4.4 | < .001 | |
| 2 y or younger | 0 | 2.7 | NA | |
| Older than 2 y | 2.6 | 5.9 | NA | |
| Hematopoiesis | 0.3; n = 6 | 5.9 | < .001 | |
| Vascular ectasia, all ages | 0.8 | 4.4 | < .001 | |
| 2 y or younger | 0.5 | 3.7 | NA | |
| Older than 2 y | 0.9 | 5.0 | NA | |
| Liver | ||||
| Mononuclear, % positive | 6 | 67 | < .001 | |
| Gross infarcts, % positive | 0 | 27 | .04 | |
| Micros infarcts, % positive | 0; n = 6 | 100 | < .001 | |
| Iron, % positive | 0 | 100 | < .001 | |
| Iron, mean of positive scores | NA | 4.1 | NA | |
| Calcification, % positive | 0 | 60 | < .001 | |
| Perivenous pallor, % positive | 6 | 20 | NS | |
| Kidney | ||||
| Cortical width, μm | 25.8 | 42.1 | < .001 | |
| Gross infarcts, % positive | 0 | 20 | .10 | |
| Micros infarcts, % positive | 0 | 67 | < .001 | |
| Iron, % positive* | 6 | 93 | < .001 | |
| Iron, mean of positive scores | 1.0 | 4.1 | NA | |
| Glomerular size, U | 2.7 | 4.5 | < .001 | |
| Arterial wall, μm | 1.7 | 2.1 | NS | |
| Mononuclear infiltrates, % positive | 0 | 40 | .007 | |
| Lungs | ||||
| Lymphs, % positive | 12.5 | 20 | NS | |
| Iron, all ages, % positive | 0 | 80 | < .001 | |
| 2 y or younger | 0 | 57 | NA | |
| Older than 2 y | 0 | 100 | NA | |
| Iron, all ages, mean of positive scores | NA | 1.75 | NA | |
| Capillary congestion, % positive | 0; n = 6 | 93 | .001 | |
| Pulmonary artery wall, μm | 1.8 | 2.7 | .002 | |
| Hemorrhage, % positive | 0; n = 6 | 60 | .02 | |
| Heart | ||||
| Micros infarcts, % positive | 0 | 33 | .02 | |
| Iron, % positive | 6 | 33 | .08 | |
| Iron, mean of positive scores | 1.0 | 1.2 | NA | |
| Blood vessel diameter, μm† | 3.7; n = 15 | 4.9 | .02 | |
| Blood vessel wall thickness, % greater than 1 μm | 12.5 | 80 | < .001 | |
| Brain and eye | ||||
| Pyknotic Purkinje cells, % | 0 | 93 | < .001 | |
| Retinal degeneration, % | 0 | 100 | < .001 | |
| Bone marrow | ||||
| Iron, % | 17 | 67 | .02 | |
| Iron, mean of positive scores | 1.5 | 1.3 | NA | |
| Cellularity score | 5.1 | 4.2 | < .001 | |
| Erythroid hyperplasia score | 0.88 | 4.8 | < .001 | |
. | Genotype . | . | . | |
|---|---|---|---|---|
. | Control; n = 16 . | Sickle; n = 15 . | P . | |
| Spleen | ||||
| Gross infarcts, % positive | 0 | 27 | .04 | |
| Micros infarct, % positive | 0 | 40 | .007 | |
| Diameter, mm | 2.2 | 5.7 | < .001 | |
| Iron, all ages | 1.9 | 4.4 | < .001 | |
| 2 y or younger | 0 | 2.7 | NA | |
| Older than 2 y | 2.6 | 5.9 | NA | |
| Hematopoiesis | 0.3; n = 6 | 5.9 | < .001 | |
| Vascular ectasia, all ages | 0.8 | 4.4 | < .001 | |
| 2 y or younger | 0.5 | 3.7 | NA | |
| Older than 2 y | 0.9 | 5.0 | NA | |
| Liver | ||||
| Mononuclear, % positive | 6 | 67 | < .001 | |
| Gross infarcts, % positive | 0 | 27 | .04 | |
| Micros infarcts, % positive | 0; n = 6 | 100 | < .001 | |
| Iron, % positive | 0 | 100 | < .001 | |
| Iron, mean of positive scores | NA | 4.1 | NA | |
| Calcification, % positive | 0 | 60 | < .001 | |
| Perivenous pallor, % positive | 6 | 20 | NS | |
| Kidney | ||||
| Cortical width, μm | 25.8 | 42.1 | < .001 | |
| Gross infarcts, % positive | 0 | 20 | .10 | |
| Micros infarcts, % positive | 0 | 67 | < .001 | |
| Iron, % positive* | 6 | 93 | < .001 | |
| Iron, mean of positive scores | 1.0 | 4.1 | NA | |
| Glomerular size, U | 2.7 | 4.5 | < .001 | |
| Arterial wall, μm | 1.7 | 2.1 | NS | |
| Mononuclear infiltrates, % positive | 0 | 40 | .007 | |
| Lungs | ||||
| Lymphs, % positive | 12.5 | 20 | NS | |
| Iron, all ages, % positive | 0 | 80 | < .001 | |
| 2 y or younger | 0 | 57 | NA | |
| Older than 2 y | 0 | 100 | NA | |
| Iron, all ages, mean of positive scores | NA | 1.75 | NA | |
| Capillary congestion, % positive | 0; n = 6 | 93 | .001 | |
| Pulmonary artery wall, μm | 1.8 | 2.7 | .002 | |
| Hemorrhage, % positive | 0; n = 6 | 60 | .02 | |
| Heart | ||||
| Micros infarcts, % positive | 0 | 33 | .02 | |
| Iron, % positive | 6 | 33 | .08 | |
| Iron, mean of positive scores | 1.0 | 1.2 | NA | |
| Blood vessel diameter, μm† | 3.7; n = 15 | 4.9 | .02 | |
| Blood vessel wall thickness, % greater than 1 μm | 12.5 | 80 | < .001 | |
| Brain and eye | ||||
| Pyknotic Purkinje cells, % | 0 | 93 | < .001 | |
| Retinal degeneration, % | 0 | 100 | < .001 | |
| Bone marrow | ||||
| Iron, % | 17 | 67 | .02 | |
| Iron, mean of positive scores | 1.5 | 1.3 | NA | |
| Cellularity score | 5.1 | 4.2 | < .001 | |
| Erythroid hyperplasia score | 0.88 | 4.8 | < .001 | |
Measurements are mean values of all grading scores unless otherwise designated as percentage of animals with positive scores and corresponding mean of the positive scores.
NA indicates not applicable; NS, not significant.
Among SS animals, iron scores in the kidney increased by .85 for each month of age (P < .001)
Among SS animals, sex and age were independent factors associated with cardiac vascular ectasia (coefficient = 1.5 for male sex [P = .03] and .44 for each month of age [P = .03])