Table.
Case Reports of MYH9-related Nephropathy Treated with RAS Blockade Therapy.
| Case | Age*/Sex | Genotype | Domain | U-P* | U-B* | S-Cre* (mg/dL) | Treatment (mg/day) | Follow-up period | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Present case 2019 | 19/M | E1841K | coiled-coil | 0.78 g/gCr | (-) | 0.84 | enalapril 5 | 29 months | U-P: decreased to 0.25 g/gCr S-Cre: stable |
| [12], 2018 | 13/M | R702C | motor | <1 g/gCr | NA | normal | telmisartan 40 | ESRD at the age of 19 years | |
| 11/M | R702C | motor | 1-2 g/gCr | NA | normal | lisinopril 10 valsartan 160 | ESRD at the age of 15 years | ||
| 11/M | R702C | motor | 1 g/gCr | NA | normal | lisinopril 10 valsartan 160 | ESRD at the age of 17 years | ||
| [13], 2015 | 36/F | E1841K | coiled-coil | 0.926 g/day | NA | 0.7 | ARB | 1 year | U-P: decreased to 0.224 g/gCr S-Cre: stable |
| [14], 2013 | 22/M | Q1068_ L1074del | coiled-coil (deletion) | 2.18 g/day | (-) | 1.55 | candesartan 6 | ESRD at the age of 27 years | |
| [15], 2011 | 17/M | S96L | motor | (+) | (+) | 3.0 | ACEI | ESRD at the age of 23 years | |
| 12/F | S96L | motor | (+) | (+) | NA | ACEI | CKD4 at the age of 19 years | ||
| 1/M | R718W | motor | (+) | (-) | 0.3 | ACEI+ARB | ESRD at the age of 7 years | ||
| [6], 2010 | 3/F | R702C | motor | 0.5-0.7 g/gCr | (-) | CKD1 | valsartan 20 | 10 months | U-P: decreased to 0.1 g/gCr |
| NA/M | R702C | motor | NA | NA | NA | ARB and/or ACEI | The effect was transient. ESRD at the age of 20 years | ||
| NA/M | R702C | motor | NA | NA | NA | ARB and/or ACEI | The effect was not conclusive. | ||
| [16], 2008 | 39/M | D1424H | coiled-coil | 1.231 g/day | NA | 1.8 | ramipril 10 telmisartan 80 | 68 months | U-P: decreased to 0.09 g/gCr S-Cr: stable |
| 42/M | D1424H | coiled-coil | 1.570 g/day | NA | 1.2 | ramipril 10 telmisartan 80 | 16 months | U-P: decreased to 0.137 g/gCr S-Cr: stable | |
| 18/M | D1424H | coiled-coil | 0.768 g/day | NA | 0.9 | ramipril 10 losartan 50 | 11 months | U-P: decreased to 0.143 g/gCr S-Cr: stable | |
| 38/F | N93K | motor | 1.280 g/day | NA | 0.9 | ramipril 5 | 40 months | U-P: decreased to 0.39 g/gCr S-Cr: stable | |
| [17], 2004 | 27/F | K910Q + D1424H | coiled-coil (double mutations) | non-nephrotic | (+) | 3.3 | ACEI | ESRD |
RAS: renin-angiotensin system, U-P: urinary protein, U-B: urinary occult blood, S-Cre: serum creatinine, ESRD: end-stage renal disease, ARB: angiotensin II receptor blocker, ACEI: angiotensin-converting enzyme inhibitor, CKD: chronic kidney disease, NA: not available
* At the time of starting RAS blockade therapy