Managing PH1

medical management options

PH1 medical management is based on the provider’s assessment of each patient.

Current management approaches to PH1 aim at enhancing clearance of oxalate, inhibiting oxalate crystallization, or lowering oxalate production by the liver.^1-3

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• Hyperhydration²
  • Recommend daily fluid intake of ≥2-3L/m² of body surface area aims to reduce urinary calcium oxalate crystallization²
• Alkali citrate^2,3
  • Inhibits calcium oxalate crystallization^2,3
• High-Dose Pyridoxine (Vitamin B6) Therapy^1,3,4
  • Can act as an AGT co-factor, which may increase residual AGT enzyme activity and reduce oxalate production by the liver in some patients^1,3
  • A small subset of patients with PH1 may respond to vitamin B6^1,3,4
• RNA interference (RNAi) Therapeutics^5-7
  • Binds to messenger RNA of enzymes involved in oxalate production, preventing their expression via the RNAi pathway^5-7

• DIALYSIS STRATEGIES IN PH1 MANAGEMENT^1,3,8,9
  • To reduce plasma oxalate levels, PH1 patients with compromised kidney function may require up to 6 hemodialysis sessions per week, which may need to be combined with continuous peritoneal dialysis^1,3,8,9
  • Dialysis may either serve as a bridge to transplantation or as an adjunct therapy after liver-kidney transplantation¹

• LIVER-KIDNEY TRANSPLANTATION
  • Liver transplant addresses the defect that causes glyoxylate—an organic compound generated by the liver enzyme GO—to convert into oxalate^2,3
  • As kidney function declines, a kidney transplant may be required along with a liver transplant.^1,3