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  • Title: Hemoglobin-oxygen affinity in hypophosphatemic rickets.
    Author: Munk P, Freedman MH, Greenberg ML, Levison H.
    Journal: Acta Paediatr Scand; 1976 Jan; 65(1):97-9. PubMed ID: 1251728.
    Abstract:
    In 14 patients with simple X-linked hypophosphatemic rickets, 5 were below the third percentile in height and 9 were between the third and twenty-fifth percentile. Although the mean serum inorganic phosphorus level was only 2.01 +/- 0.65 (normal range for all age groups is 3.8 to 6.0 mg/100 ml), both the mean values for red cell 2,3-diphosphoglycerate (2,3-DPG) and adenosine triphosphate (ATP) were normal at 4.78 +/- 1.23 and 1.02 +/- 0.17 mumol/ml of red blood cells respectively. Moreover, the mean P50 value was normal at 26.4 +/- 0.9 mmHg. These normal oxygen transport data make unlikely any proposal that short stature seen in these patients is secondary to chronic tissue hypoxia. They also indicate that the intra-erythrocytic organic phosphate levels are maintained at normal levels despite profound chronic hypophosphatemia.
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