Table 1.

Hematologic and iron parameters in patients with GSD1a and anemia

SubjectSexAge, yHgb, g/dLHct, %MCV, fLRDW, %Fe, μg/dLTIBC, μg/dLSaturation, %Ft, ng/mL
Female 19 9.8 29.4 80.2 15.3 20 301 6.6 67.2 
Male 22 8.5 26.6 64.0 N/A 20 330 6.1 N/A 
Male 27 4.6 16.6 50.4 20.7 349 1.7 23.7 
Male 13 8.4 26.7 61.8 16.4 13 411 3.2 47.9 
Male 24 7.7 24.4 70.8 18.3 N/A N/A N/A 15.3 
SubjectSexAge, yHgb, g/dLHct, %MCV, fLRDW, %Fe, μg/dLTIBC, μg/dLSaturation, %Ft, ng/mL
Female 19 9.8 29.4 80.2 15.3 20 301 6.6 67.2 
Male 22 8.5 26.6 64.0 N/A 20 330 6.1 N/A 
Male 27 4.6 16.6 50.4 20.7 349 1.7 23.7 
Male 13 8.4 26.7 61.8 16.4 13 411 3.2 47.9 
Male 24 7.7 24.4 70.8 18.3 N/A N/A N/A 15.3 

We obtained hematologic data from 5 patients with GSD1a on oral iron therapy prior to intravenous iron dextran infusions. All patients had large adenomas at the time of these studies. One representative set of data is shown for each patient. Subjects 1 and 2 are patients A and B, respectively. Data were not available for the sixth patient because he was treated at another institution and was not covered by our human studies protocol. Hgb indicates hemoglobin (normal value ranges for adult females, 12-16 g/dL; adult males, 13-18 g/dL; 13-year-old males, 13-16 g/dL); Hct, hematocrit (normal value ranges for adult females, 37%-48%; adult males, 42%-52%; 13-year-old males, 37%-49%); MCV, mean corpuscular volume (normal value range for adults, 90 ± 10 fL; 13-year-old males, 88 ± 10 fL); RDW, red blood cell distribution width (normal, 13%-15%); Fe, serum iron concentration (normal, 50-150 μg/dL); TIBC, total iron-binding capacity (normal, 250-370 μg/dL); saturation, the ratio of iron to total iron-binding capacity (normal, 20%-45%); Ft, serum ferritin (normal values for 19-year-old females, 6-40 ng/mL; 13-year-old males, 23-70 ng/mL; adult males, 20-300 ng/mL, increasing with age); N/A, not available.

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