344.2.3.2 Primary Hyperoxaluria
- AGXT, GRHPR, HOGA1 mutations
- Severe; ESKD
- Lumasiran (Oxlumo, FDA 2020) â siRNA for AGXT
- Nedosiran â RGRPR siRNA emerging
- Liver-kidney transplant
Calcium Oxalate: - Hypercalciuria: thiazide - Hyperoxaluria: calcium with meals - Hypocitraturia: potassium citrate
Uric Acid: - Alkalinize urine (potassium citrate to pH 6.5-7.0) - Allopurinol
Struvite: - Antibiotics (urease bacteria â Proteus, Klebsiella, Pseudomonas, S. aureus) - Surgical removal (PCNL)
Cystine: - Hydration (> 4 L/d) - Alkalinize (pH > 7.5) - Tiopronin, penicillamine
| Stone Type | % | pH | Imaging | Bacteria | Prevention |
|---|---|---|---|---|---|
| Ca oxalate | 70-80 | Variable | CT | None | Thiazide, K citrate, â fluid |
| Ca phosphate | 5-10 | Alkaline | CT | None | K citrate (cautious) |
| Uric acid | 5-10 | < 5.5 | CT (radiolucent on KUB) | None | Alkalinize, allopurinol |
| Struvite | 5-10 | > 7.5 | CT | Urease bacteria | Antibiotics + surgery |
| Cystine | 1 | Variable | CT (radiopaque, dense) | None | Hydration + alkalinize + tiopronin |
| Drug | Variable | Variable | CT | None | Stop drug |