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HEMOGLOBIN‐OXYGEN AFFINITY IN HYPOPHOSPHATEMIC RICKETS
Author(s) -
MUNK PAUL,
FREEDMAN MELVIN H.,
GREENBERG MARK L.,
LEVISON HENRY
Publication year - 1976
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1976.tb04413.x
Subject(s) - medicine , hypophosphatemic rickets , hypophosphatemia , rickets , percentile , endocrinology , hemoglobin , hypoxia (environmental) , short stature , diphosphoglycerate , oxygen , vitamin d and neurology , chemistry , statistics , mathematics , organic chemistry
. 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 μmol/ml of red blood cells respectively. Moreover, the mean P 50 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.