Table 4.
Prognosis of mitochondrial nephropathy with m.3243 A>G depending on the time of onset of renal manifestations
| Outcomes | All (N = 63) | Onset timing of renal manifestationsa |
||
|---|---|---|---|---|
| Childhood (0–19 yr) (n = 28) |
Adulthood (≥20 yr) (n = 35) |
P | ||
| Observation time for initiation of RRT, median (IQR) (yr)b | 11.0 (5.0–17.0) | 13.0 (7.0–18.5) | 9.0 (4.0–15.0) | 0.12 |
| Initiation of RRT, n (%) | 32 (50.8) | 16 (57.1) | 16 (45.7) | 0.37 |
| Observation time for all-cause mortality, median (IQR) (yr)b | 12.0 (7.0–23.0) | 14.5 (8.0–25.5) | 12.0 (6.0–21.0) | 0.22 |
| All-cause mortality, n (%) | 16 (25.4) | 7 (25.0) | 9 (25.7) | 0.95 |
| eGFR declining slope (ml/min per 1.73 m2/yr), median (IQR) | 5.4 (2.9–9.2) | 7.4 (4.0–10.6) | 4.7 (1.7–8.2) | 0.16 |
Cre, creatinine; eGFR, estimated glomerular filtration rate; IQR, interquartile range; RRT, renal replacement therapy.
a
Time at the first detection of at least one of the following 3 renal manifestations was defined as the onset timing of renal manifestations: proteinuria (≥0.15 g/g Cre), reduced eGFR (<60 ml/min per 1.73 m2), or Fanconi syndrome.
b
Time from onset of renal manifestations.