Table 1.
Patients’ clinical data
| Cystic disease | |||||||
|---|---|---|---|---|---|---|---|
| ADPKD | DNAJB11-PKD | ||||||
| Characteristics | n | Mean ± SD | % | n | Mean ± SD | % | P-value |
| Patients | 42 | 27 | |||||
| Age at last follow-up (years) | 42 | 55.0 ± 16.8 | 27 | 74.8 ± 12.3 | 0.000 | ||
| Males/females | 26/16 | 38.1 | 12/15 | 55.6 | 0.22 | ||
| ESKD (y/n) | 16/26 | 16/26 | 38.1 | 12/15 | 44.4 | 0.62 | |
| Age at ESKD (years) | 15 | 59.9 ± 11.4 | 12 | 71.1 ± 4.5 | 0.006 | ||
| Kidney diameter (cm)a | 33 | 16.4 ± 4.6 | 22 | 10.3 ± 1.6 | <0.001 | ||
| Larger kidney cyst (cm) | 29 | 5.7 ± 2.9 | 22 | 3.0 ± 2.5 | <0.001 | ||
| Pancreatic cysts (y/n) | 2/38 | 5.0 | 1/22 | 4.3 | 1.00 | ||
| Liver cysts (y/n) | 30/10 | 75.0 | 11/12 | 47.8 | 0.06 | ||
| Kidney stones (y/n) | 12/29 | 29.3 | 16/10 | 61.5 | 0.01 | ||
| Hb (g/dL)b | 29 | 12.9 ± 2.3 | 23 | 11.0 ± 2.6 | 0.006 | ||
| PTH (pg/mL)c | 19 | 188.5 ± 196.5 | 14 | 305.8 ± 276.8 | 0.09 | ||
| 24-h proteinuria >00.5 g (y/n) | 6/28 | 17.6 | 12/10 | 54.5 | 0.007 | ||
| ESA use | 5/28 | 15.2 | 9/16 | 36 | 0.12 | ||
| Diabetes type 2 (y/n) | 0/41 | 0.0 | 5/21 | 19.2 | 0.007 | ||
| Cancer (y/n) | 4/37 | 9.8 | 8/16 | 33.3 | 0.02 | ||
| Valvular defectsd (y/n) | 21/20 | 51.2 | 1/22 | 4.3 | <0.001 | ||
| Cerebrovascular disease (y/n) | 0/41 | 0 | 2/21 | 8.7 | 0.12 | ||
| Cardiovascular disease (y/n) | 1/39 | 2.5 | 5/18 | 21.7 | 0.02 | ||
| Hypertension | 25/15 | 62.5 | 16/7 | 69.6 | 0.78 | ||
aKidney size is assessed using pole-to-pole diameter on ultrasound or pole-to-pole diameter on a sagittal scan on CT or MRI.
bFor 10 ADPKD patients, data were collected just before the beginning of dialysis; the other 19 patients had a mean eGFR of 80 mL/min/1.73 m2 using the CKD-EPI equation. Among 5 DNAJB11-PKD patients, data were collected just before dialysis; the other 10 had a mean eGFR of 50 mL/min/1.73 m2.
cSeven ADPKD patients were starting dialysis; among others, the mean eGFR was 71 mL/min/1.73 m2. Five DNAJB11-PKD patients were starting dialysis and the other eight had a mean eGFR of 51 mL/min/1.73 m2.
dThe valvular defects considered were mitral valve insufficiency, mitral valve stenosis, aortic valve insufficiency, aortic valve stenosis and tricuspid valve insufficiency. Each of these defects could be mild, moderate or severe in different patients. Tricuspid valve stenosis and pulmonary valve defects were not reported among patients.
Hb, hemoglobin; PTH, parathormone; ESA, erythropoietin-stimulating agent.
Values in bold are statistically significant.