August 2026 Research Update

These studies add a clearer picture of why transplant planning matters in primary hyperoxaluria type 1 (PH1): when the liver keeps making oxalate, oxalate can keep building up even after a kidney transplant. In a small pediatric series, combined liver-kidney transplant had early complications but all children were alive with working grafts at follow-up. In contrast, one adult case diagnosed after a kidney-only transplant showed oxalate crystals in the transplanted kidney and progressed to irreversible graft failure. [1] [2]

In the pediatric series of four children receiving combined liver-kidney transplantation (including PH1 among the indications), all had functioning liver and kidney grafts at last follow-up, despite infections, bleeding, and episodes of rejection that were treated successfully. This helps show that a combined transplant approach can be feasible in real-world care, while still involving meaningful short-term risks that required active management. [1]

The adult case highlights how PH1 may be missed even when urine oxalate tests look normal, and how that can affect a transplanted kidney. After living-donor kidney transplantation, biopsy showed widespread calcium oxalate crystal deposits in the graft, and genetic testing later identified an AGXT mutation consistent with PH1; the patient later died in the setting of severe catheter-related infection after graft failure. This single report cannot predict what will happen to others, but it illustrates the possible consequences of identifying PH1 only after a kidney transplant has already occurred. [2]

For patients and caregivers, this month mainly adds context about transplant outcomes and the importance of recognizing PH1 as the cause of kidney failure when possible. The combined liver-kidney transplant series suggests that, in a small group, long-term graft function and survival were achievable even though early complications were common and needed treatment. The kidney-only transplant case shows that ongoing oxalate production can damage a transplanted kidney if PH1 is not recognized, and it also shows that normal urine oxalate did not rule out PH1 in that person; how often this happens remains uncertain from a single case. [1] [2]

This month, case reports reinforced that PH1 can become a whole-body condition when oxalate builds up, not just a kidney stone disease. Reports described oxalate deposits affecting soft tissues, bones, joints, eyes, and other areas, especially after kidney function worsens. One report also introduced an imaging approach that lit up many bones and joints, interpreted as the body actively trying to repair oxalate-related damage. [3] [4]

In a report of two brothers with PH1 from a rare AGXT gene change, both developed end-stage kidney disease in young adulthood and showed extensive extra-kidney deposits on imaging along with problems such as vision loss, tooth loss, and mobility issues. The authors also noted that the brother receiving more intensive blood-cleaning approaches appeared to have lower measured plasma oxalate than the brother on standard hemodialysis, though this was an observation within one family rather than a controlled comparison. [3]

A separate single-patient report used a 68Ga-FAPI-04 PET/CT scan and found strong signal in multiple bones and joints. The authors interpreted this pattern as an active fibroblast-driven repair response triggered by oxalate crystal deposits, suggesting the scan may visualize tissue responses to oxalate in places that can be hard to assess. It is not yet clear how this imaging would compare with existing tests or how often this pattern occurs in PH1. [4]

For patients, these reports mainly help explain why symptoms in PH1 can extend beyond the kidneys, especially as oxalate accumulates in the body over time. The family report shows the range of possible daily-life impacts once systemic deposits occur, while also highlighting that oxalate burden can differ even among relatives with the same condition. The new imaging report is early research in a single person, so it does not change what is routinely done now, but it points to a possible future tool for seeing where oxalate-related tissue injury and repair may be happening. [3] [4]