Clinical characteristics | Primary hyperoxaluria type 1 (PH1) | ||||
---|---|---|---|---|---|
PH1 without NC or KS (n = 10) | PH1 with NC (n = 6) | PH1 with KS (n = 9) | Total (n = 25) | p value | |
Age (years) | 21 (18, 25) | 16 (16, 17) | 20 (17, 24) | 18 (16, 22) | 0.13 |
Sex | Â | Â | Â | Â | Â |
 Female (%) | 7 (70%) | 3 (50%) | 2 (22%) | 12 (48%) | 0.14 |
 Male (%) | 3 (30%) | 3 (50%) | 7 (78%) | 13 (12%) |  |
Body mass index (kg/m2) | 24 (20, 33) | 24 (23, 27) | 24 (22, 28) | 24 (22, 29) | 0.94 |
Systolic blood pressure (mmHg) | 111 (106, 116) | 112 (100, 120) | 112 (105, 118) | 112 (105, 118) | 0.84 |
Diastolic blood pressure (mmHg) | 73 (67, 74) | 73 (51, 74) | 65 (56, 68) | 68 (58, 74) | 0.26 |
Serum creatinine (mg/dL) | 0.9 (0.9, 1.2) | 1.1 (0.8, 1.3) | 1.3 (1.1, 1.4) | 1.2 (0.9, 1.3) | 0.16 |
eGFR (mL/min/1.73Â m2) | 79 (73, 91) | 66 (55, 94) | 68 (53, 76) | 73 (55, 91) | 0.66 |
Urine biochemistry | Â | Â | Â | Â | Â |
Urine volume (mL/24Â h) | 2944 (2415, 3388) | 3819 (3228, 5766) | 2880 (2423, 3510) | 3037 (2423, 3774) | 0.19 |
pH | 6.2 (6.0, 6.6) | 6.6 (6.4, 6.8) | 6.3 (6.2, 6.7) | 6.4 (6.2, 6.7) | 0.32 |
Osmolality (mOsm/kg) | 610 (441, 632) | 286 (237, 336) | 316 (183, 516) | 355 (248, 610) | 0.06 |
Calcium (mg/24Â h) | 96 (67, 118) | 90 (47, 148) | 113 (59, 126) | 105 (59, 130) | 0.98 |
Citrate (mg/24Â h) | 482 (273, 537) | 416 (386, 533) | 300 (168, 467) | 384 (249, 517) | 0.53 |
Creatinine (mg/mL) | 0.52 (0.32, 0.83) | 0.43 (0.28, 0.44) | 0.58 (0.50, 0.71) | 0.50 (0.31, 0.71) | 0.59 |
Total protein (mg/dL) | 3.5 (0.32, 7.7) | 10.0 (2.5, 17.0) | 1.0 (1.0, 3.0) | 3.0 (1.0, 9.0) | 0.29 |
Urine oxalate (mmol/24Â h/1.73Â m2) | 0.60 (0.35, 1.0) | 1.0 (0.8, 1.7) | 1.0 (0.6, 1.4) | 0.8 (0.6, 1.5) | 0.32 |