Indices of inorganic phosphate, parathyreoid hormone of blood and renal tubular reabsorption of phosphate in children with hereditary hypophosphatemic rickets
Abstract
THE AIM OF STUDY: To evaluate the growth, indices of inorganic phosphate, parathyreoid hormone, 25(OH)2D3 in blood, renal tubular reabsorption, glomerular filtration rate (GFR) in children suffering from hereditary hypophosphatemic rickets (HR). PATIENTS AND METHODS: Examined are 80 patients from 79 families with hereditary HR. Detected are 38 Х-linked and AD HR cases, of which 4 cases having hypophosphatemic bone disease, 42 AR HR cases, of which 4 cases having HR with hypercalciuria. Used are the formulas for calculation of indices of FEP, TRP, TmP, U P/Cr, U Ca/Cr. RESULTS: Of the 80 patients with HR, the centile values of body length (cm) in 33 boys and in 47 girls aged 1 to 17 years were in the “low” level - zone No. 2 (10% of the centile). A considerable growth retardation is detected, more pronounced in boys, with AR HR. The inorganic phosphate of blood in the 80 patients with HR (1.00±0.09 mmol/l) is reliably less than normal, high activity of alkaline phosphotase 1149.63±76.07 U/l, phosphaturia 28.91±2.65 mg/kg/day. The indices of tubular reabsorption of phosphate (TRP) (67.46±3.40 %), maximum tubular reabsorption of phosphate (TmP) (0.69±0.09 mmol/l) are less than normal. The fractional phosphate excretion (FPE) is higher than normal (31.54±5.64 %), a correlation dependence between the level of phosphate of blood and the FEP is discovered. The indices of U P/Cr in children with HR were 1.38 ± 0.28 (n 0.76 ± 0.31). The index of U Ca/Cr is less than normal in 3 groups, higher than normal in the group of children with AR with hypercalciuria. Of the 80 patients with HR, the level of PTH and 25(OH)2D in blood was no different from the normal indices in 76 (49.7±4.4 pg/ml, 30.3±8.3 and 44.5 pg/ml), р>0.05, in those with HR with hypercalciuria is less than normal (28.5±0.8 pg/ml and 44.5 pg/ml). CONCLUSION: The evaluation of indices of FEP, TRP, TmP, U P/Cr using the calculation formulas is very informative of defect of transport of phosphates in proximal renal tubules, as it takes into account their concentration in plasma, and the level of creatinine in blood and in urine.
About the Authors
Zh. G. Leviashvili
Санкт-Петербургский государственный университет
Russian Federation
Russian Federation
N. D. Savenkova
Санкт-Петербургский государственный университет
Russian Federation
Russian Federation
A. V. Musayeva
Санкт-Петербургский государственный университет
Russian Federation
Russian Federation
D. Yu. Belov
Санкт-Петербургский государственный университет
Russian Federation
Russian Federation
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Review
For citations:
Leviashvili Zh.G., Savenkova N.D., Musayeva A.V., Belov D.Yu. Indices of inorganic phosphate, parathyreoid hormone of blood and renal tubular reabsorption of phosphate in children with hereditary hypophosphatemic rickets. Nephrology (Saint-Petersburg). 2014;18(3):45-56. (In Russ.)
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