Response to rhPTH1 34 20 μg Daily for 28 Days in Case 1 with X Linked Hypophosphatemia with Hypoparathyroidism
| Variables | Pre day 1 | Post day 1 | Pre day 28 | Post day 28 | Reference intervals |
| TmP/GFR, mmol/L | 1.14 | 1.20 | 0.66 | 0.48 | 0.81, 1.35 |
| NcAMP, nmol/L | 5.7 | 85.1 | 24.5 | 140.7 | 22.2, 30.4 |
| cFGF23, RU/L | 6700 | 7300 | 6750 | 7050 | <100 |
| iFGF23, ng/L | 11,900 | 12,200 | 10,400 | 9650 | 10, 50 |
| α Klotho, ng/L | 346.4 | 408.7 | 407.7 | 408.3 | 505, 1175 |
| Ionized calcium, mmol/L | 1.08 | 1.07 | 1.07 | 1.16 | 1.19, 1.35 |
| PTH, pmol/L | <0.6 | <0.6 | <0.6 | <0.6 | 1.6, 6.9 |
| PINP, μg/L | 41.5 | 41.4 | 89.8 | 89.2 | 22, 96 |
| OC(1–43), μg/L | 15.2 | 14.3 | 32.7 | 33.9 | 11, 43 |
| Bone ALP, μg/L | 5.5 | 8.8 | 16 | 15.1 | 2.9, 20.9 |
| CTX, μg/L | 0.370 | 0.437 | 0.641 | 1.060 | 0.016, 0.584 |
| uNTX/Cr, nmol BCE/mmol Cr | 9.9 | 3.6 | 38.2 | 38.3 | 14, 74 |
| uCa/Cr mmol/mmol | 0.41 | 0.47 | 0.65 | 0.33 | 0.07, 0.41 |
| 25OHD, nmol/L | 98 | 113 | 84 | 90 | 30, 125 |
| 24,25(OH)2D, nmol/L | 3.5 | 3.6 | 2.8 | 3.5 | 1.0, 13.0 |
| 1,25(OH)2D, pmol/L | 57 | 63 | 53 | 51 | 55, 139 |
| 25OHD:24,25(OH)2D | 28 | 31 | 30 | 26 | 7, 25 |
| 1,25(OH)2D:24,25(OH)2D | 16 | 18 | 19 | 15 | 9, 132 |
| Variables | Pre day 1 | Post day 1 | Pre day 28 | Post day 28 | Reference intervals |
| TmP/GFR, mmol/L | 1.14 | 1.20 | 0.66 | 0.48 | 0.81, 1.35 |
| NcAMP, nmol/L | 5.7 | 85.1 | 24.5 | 140.7 | 22.2, 30.4 |
| cFGF23, RU/L | 6700 | 7300 | 6750 | 7050 | <100 |
| iFGF23, ng/L | 11,900 | 12,200 | 10,400 | 9650 | 10, 50 |
| α Klotho, ng/L | 346.4 | 408.7 | 407.7 | 408.3 | 505, 1175 |
| Ionized calcium, mmol/L | 1.08 | 1.07 | 1.07 | 1.16 | 1.19, 1.35 |
| PTH, pmol/L | <0.6 | <0.6 | <0.6 | <0.6 | 1.6, 6.9 |
| PINP, μg/L | 41.5 | 41.4 | 89.8 | 89.2 | 22, 96 |
| OC(1–43), μg/L | 15.2 | 14.3 | 32.7 | 33.9 | 11, 43 |
| Bone ALP, μg/L | 5.5 | 8.8 | 16 | 15.1 | 2.9, 20.9 |
| CTX, μg/L | 0.370 | 0.437 | 0.641 | 1.060 | 0.016, 0.584 |
| uNTX/Cr, nmol BCE/mmol Cr | 9.9 | 3.6 | 38.2 | 38.3 | 14, 74 |
| uCa/Cr mmol/mmol | 0.41 | 0.47 | 0.65 | 0.33 | 0.07, 0.41 |
| 25OHD, nmol/L | 98 | 113 | 84 | 90 | 30, 125 |
| 24,25(OH)2D, nmol/L | 3.5 | 3.6 | 2.8 | 3.5 | 1.0, 13.0 |
| 1,25(OH)2D, pmol/L | 57 | 63 | 53 | 51 | 55, 139 |
| 25OHD:24,25(OH)2D | 28 | 31 | 30 | 26 | 7, 25 |
| 1,25(OH)2D:24,25(OH)2D | 16 | 18 | 19 | 15 | 9, 132 |
1,25(OH)2D = 1,25 hydroxyvitamin D; 25OHD = 25 hydroxyvitamin D; ALP = alkaline phosphatase; BAP = bone specific alkaline phosphatase; BCE = bone collagen equivalent; cFGF23 = C terminal FGF23; Ca = calcium; Cr = creatinine; CTX = C terminal telopeptide of type I collagen; iFGF23 = intact FGF23; OC(1–43) = N mid fragment osteocalcin; PINP = procollagen type I N propeptide; NcAMP = nephrogenous cyclic adenosine monophosphate; NTX = N terminal telopeptides of type I collagen; RU = relative unit; TmP/GFR = maximum renal tubular phosphate reabsorption rate per volume of glomerular filtrate; uNTX = urine N terminal telopeptides of type I collagen.
Response to rhPTH1 34 20 μg Daily for 28 Days in Case 1 with X Linked Hypophosphatemia with Hypoparathyroidism
| Variables | Pre day 1 | Post day 1 | Pre day 28 | Post day 28 | Reference intervals |
| TmP/GFR, mmol/L | 1.14 | 1.20 | 0.66 | 0.48 | 0.81, 1.35 |
| NcAMP, nmol/L | 5.7 | 85.1 | 24.5 | 140.7 | 22.2, 30.4 |
| cFGF23, RU/L | 6700 | 7300 | 6750 | 7050 | <100 |
| iFGF23, ng/L | 11,900 | 12,200 | 10,400 | 9650 | 10, 50 |
| α Klotho, ng/L | 346.4 | 408.7 | 407.7 | 408.3 | 505, 1175 |
| Ionized calcium, mmol/L | 1.08 | 1.07 | 1.07 | 1.16 | 1.19, 1.35 |
| PTH, pmol/L | <0.6 | <0.6 | <0.6 | <0.6 | 1.6, 6.9 |
| PINP, μg/L | 41.5 | 41.4 | 89.8 | 89.2 | 22, 96 |
| OC(1–43), μg/L | 15.2 | 14.3 | 32.7 | 33.9 | 11, 43 |
| Bone ALP, μg/L | 5.5 | 8.8 | 16 | 15.1 | 2.9, 20.9 |
| CTX, μg/L | 0.370 | 0.437 | 0.641 | 1.060 | 0.016, 0.584 |
| uNTX/Cr, nmol BCE/mmol Cr | 9.9 | 3.6 | 38.2 | 38.3 | 14, 74 |
| uCa/Cr mmol/mmol | 0.41 | 0.47 | 0.65 | 0.33 | 0.07, 0.41 |
| 25OHD, nmol/L | 98 | 113 | 84 | 90 | 30, 125 |
| 24,25(OH)2D, nmol/L | 3.5 | 3.6 | 2.8 | 3.5 | 1.0, 13.0 |
| 1,25(OH)2D, pmol/L | 57 | 63 | 53 | 51 | 55, 139 |
| 25OHD:24,25(OH)2D | 28 | 31 | 30 | 26 | 7, 25 |
| 1,25(OH)2D:24,25(OH)2D | 16 | 18 | 19 | 15 | 9, 132 |
| Variables | Pre day 1 | Post day 1 | Pre day 28 | Post day 28 | Reference intervals |
| TmP/GFR, mmol/L | 1.14 | 1.20 | 0.66 | 0.48 | 0.81, 1.35 |
| NcAMP, nmol/L | 5.7 | 85.1 | 24.5 | 140.7 | 22.2, 30.4 |
| cFGF23, RU/L | 6700 | 7300 | 6750 | 7050 | <100 |
| iFGF23, ng/L | 11,900 | 12,200 | 10,400 | 9650 | 10, 50 |
| α Klotho, ng/L | 346.4 | 408.7 | 407.7 | 408.3 | 505, 1175 |
| Ionized calcium, mmol/L | 1.08 | 1.07 | 1.07 | 1.16 | 1.19, 1.35 |
| PTH, pmol/L | <0.6 | <0.6 | <0.6 | <0.6 | 1.6, 6.9 |
| PINP, μg/L | 41.5 | 41.4 | 89.8 | 89.2 | 22, 96 |
| OC(1–43), μg/L | 15.2 | 14.3 | 32.7 | 33.9 | 11, 43 |
| Bone ALP, μg/L | 5.5 | 8.8 | 16 | 15.1 | 2.9, 20.9 |
| CTX, μg/L | 0.370 | 0.437 | 0.641 | 1.060 | 0.016, 0.584 |
| uNTX/Cr, nmol BCE/mmol Cr | 9.9 | 3.6 | 38.2 | 38.3 | 14, 74 |
| uCa/Cr mmol/mmol | 0.41 | 0.47 | 0.65 | 0.33 | 0.07, 0.41 |
| 25OHD, nmol/L | 98 | 113 | 84 | 90 | 30, 125 |
| 24,25(OH)2D, nmol/L | 3.5 | 3.6 | 2.8 | 3.5 | 1.0, 13.0 |
| 1,25(OH)2D, pmol/L | 57 | 63 | 53 | 51 | 55, 139 |
| 25OHD:24,25(OH)2D | 28 | 31 | 30 | 26 | 7, 25 |
| 1,25(OH)2D:24,25(OH)2D | 16 | 18 | 19 | 15 | 9, 132 |
1,25(OH)2D = 1,25 hydroxyvitamin D; 25OHD = 25 hydroxyvitamin D; ALP = alkaline phosphatase; BAP = bone specific alkaline phosphatase; BCE = bone collagen equivalent; cFGF23 = C terminal FGF23; Ca = calcium; Cr = creatinine; CTX = C terminal telopeptide of type I collagen; iFGF23 = intact FGF23; OC(1–43) = N mid fragment osteocalcin; PINP = procollagen type I N propeptide; NcAMP = nephrogenous cyclic adenosine monophosphate; NTX = N terminal telopeptides of type I collagen; RU = relative unit; TmP/GFR = maximum renal tubular phosphate reabsorption rate per volume of glomerular filtrate; uNTX = urine N terminal telopeptides of type I collagen.
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