Rigi10™ Malleable Penile Prosthesis
Also Known As
Oxaluria, Hyperoxalosis (when referring to systemic oxalate deposition), Primary hyperoxaluria (PH1, PH2, PH3 for genetic forms), Enteric hyperoxaluria, Dietary hyperoxaluria, Idiopathic hyperoxaluria
Definition
Hyperoxaluria is a metabolic disorder characterized by excessive oxalate excretion in the urine, typically defined as urinary oxalate excretion exceeding 40 mg (440 μmol) per 24 hours.1 Oxalate is a natural chemical produced by the body and found in certain foods that, when present in high concentrations, forms crystals with calcium in the kidneys.2 These calcium oxalate crystals can lead to kidney stone formation, nephrocalcinosis (calcium deposits in kidney tissue), and in severe cases, kidney damage and failure.3 Hyperoxaluria can be classified into primary forms (inherited genetic disorders of glyoxylate metabolism) and secondary forms (resulting from increased dietary intake or intestinal absorption of oxalate).4
Clinical Context
Hyperoxaluria is a significant contributor to nephrolithiasis (kidney stones), with urinary oxalate being the strongest chemical promoter of kidney stone formation.1 The clinical presentation varies based on the type and severity of hyperoxaluria, but common manifestations include recurrent kidney stones, hematuria (blood in urine), urinary tract infections, and flank pain.2
Primary hyperoxaluria (PH) is a rare inherited disorder with three main types (PH1, PH2, PH3), each caused by different genetic mutations affecting liver enzymes involved in glyoxylate metabolism.3 PH1, the most severe form, is caused by mutations in the AGXT gene, resulting in deficient alanine-glyoxylate aminotransferase activity.4 Without treatment, PH1 often progresses to end-stage renal disease (ESRD) in childhood or early adulthood.5
Secondary hyperoxaluria includes enteric hyperoxaluria (resulting from intestinal diseases like Crohn’s disease or after bariatric surgery), dietary hyperoxaluria (from excessive consumption of high-oxalate foods), and idiopathic hyperoxaluria.6
Management strategies depend on the underlying cause and severity but generally include increased fluid intake (targeting >3L of urine output daily), dietary oxalate restriction, calcium supplementation with meals, urinary alkalinization with potassium citrate, and in severe cases, specialized treatments like pyridoxine (vitamin B6) for responsive PH1 patients.7 For primary hyperoxaluria, newer RNA interference therapies like lumasiran have shown promise in reducing oxalate production.8 In advanced cases with kidney failure, intensive dialysis and combined liver-kidney transplantation may be necessary, particularly for PH1.9