Primary Hyperoxaluria (PH) | Diagnosis & Treatments
How is primary hyperoxaluria diagnosed?
Children with PH are often diagnosed because they develop multiple kidney stones and have loss of kidney function. Sometimes evaluations for frequent urinary tract infections (UTI), blood in the urine, or flank (side) or back pain will lead to a diagnosis. PH might be diagnosed if your child has:
- Kidney stones forming early in childhood
- Recurrent kidney stones
- Nephrocalcinosis (calcium deposits in the kidney tissue)
- Reduced kidney function or kidney failure with unclear cause
- A family history of kidney problems or kidney stones
- A family history of PH
However, it is important to note that PH1 is the most common form of PH. To prevent kidney stones and the risk of kidney damage to your child, it’s important to diagnose PH as soon as possible so that effective therapies can be started.
How is primary hyperoxaluria in children treated?
At Boston Children’s Hospital, we treat PH with a multidisciplinary approach. Your child’s care team may include:
- Nephrologists, who manage the medical therapies for PH
- Urologists, who help manage the surgical treatments
- Dietitians, who help with any dietary changes that your child’s nephrologist may recommend
Initial PH treatment includes very high daily fluid intake and medications, like potassium citrate, that can help calcium oxalate crystals dissolve in the body. Treatment with pyridoxine (vitamin B6) or RNA interference drugs may lower urine oxalate and blood oxalate levels in PH1. Children with PH who develop kidney failure may require dialysis or transplantation.
Research can help us better understand how to treat all forms of PH. Our team frequently participates in clinical trials to help further our knowledge of PH and its treatment.
A primary hyperoxaluria care Center of Excellence
Boston Children’s is the first pediatric center designated as a Center of Excellence through the Oxalosis and Hyperoxaluria Foundation’s (OHF) Care Center Network.
As a PH Center of Excellence, we are constantly working to broaden awareness of PH, to advance research into new PH therapies, and to improve the outcomes of children with PH under our care.