Internal Medicine

0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:13

756 Hyperoxaluria

insufficiency, intestinal surgery, alteration in intestinal flora),

increased dietary oxalate (e.g., chocolate, TPN in SGA neonates)

management

What to Do First

■EKG

■assess severity of renal disease

■trial of vitamin B6 (pyridoxine): PHI may respond to pharmacologic

doses of vitamin B6

■consider administration of magnesium and citrate (100 mg/kg/d in

4 divided doses) to prevent further crystal formation

General Measures

■high fluid intake (>2 liters/m2/day)

■decrease dietary oxalate

■monitor cardiac function for heart block

■lithotripsy for urolithiasis

■ophthalmology evaluation

■reduce calcium oxalate crystal formation: sodium or potassium cit-

rate 100–150 mg/kg/d

specific therapy

■vitamin B6 (pyridoxine) in responsive PHI patients (10–40%): start

with 3–5 mg/kg with stepwise increase up to 15 mg/kg

■hemodialysis or peritoneal dialysis in anuric patients (temporary

measure: dialysis unable to keep pace with endogenous oxalate pro-

duction)

■liver transplantation+/−kidney transplantation in patients with

end-stage renal disease

■kidney transplantation: temporary measure (25% graft 3 year sur-

vival)

■liver+kidney transplantation: definitive correction

Side Effects & Contraindications

■operative morbidity and mortality

■immunosuppressive therapy complications

follow-up

During Treatment

■monitor blood/urine oxalate during vitamin B6 administration to

check responsiveness (NB: response may be gradual and take weeks)