Table 1 Patient characteristics.
From: Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD
Total (n = 456) | |
|---|---|
Clinical information | |
Sex, n (%) | |
Male | 230/456 (50%) |
Female | 226/456 (50%) |
Last available CKD stage, n (%) | |
G1 | 102/446 (23%) |
G2 | 95/446 (21%) |
G3 | 92/446 (21%) |
G4 | 37/446 (8%) |
G5 without KRT | 19/446 (4%) |
G5 with KRT | 101/446 (23%) |
Age at first visit (yrs), n | 456 |
Median (IQR) | 1.7 (0.2–7.4) |
Age at last visit (yrs), n | 456 |
Median (IQR) | 8.2 (3.6–14.6) |
Number of visits, n | 456 |
Median (IQR) | 3.0 (2.0–7.0) |
Follow-up time, n | 456 |
Median (IQR) | 2.3 (0.4–7.3) |
Age at initial diagnosis (yrs), n | 418 |
Median (IQR) | 0.3 (0.1–1.6) |
Gestational age at birth (weeks), n | 341 |
Median (IQR) | 38.0 (36.0–39.0) |
Perinatal assisted breathing, n (%) | 94/402 (23%) |
Patients with documented nephrectomies, n (%) | 45/456 (10%) |
Age at first or bilateral nephrectomy (yrs), n | 45 |
Median (IQR) | 1.1 (0.1–5.6) |
Genetic information | |
Genetic confirmation, n (%) | |
No PKHD1 testing, no PKHD1 variant detected | 260/456 (57%) |
ARPKD genetically confirmed | 116/456 (25%) |
ARPKD genetically probable | 35/456 (8%) |
ARPKD genetically unknown | 45/456 (10%) |
Functional PKHD1 variant classifications, n (%) | |
Null/null | 11/196 (6%) |
Null/missense | 50/196 (26%) |
Missense/missense | 85/196 (43%) |
Others | 24/196 (12%) |
Single variant | 26/196 (13%) |
