English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Zendy Open

Presents the pdf analysis as premium feature

PDF Analysis

Insights

Presents the summarisation as premium feature

Summarisation

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the zaia usage as premium feature

ZAIA

Zendy Tools

Zendy Plus

Presents the access of premium content as premium feature

Premium Content

We report on two patients with end-stage renal disease (ESRD) due to primary hyperoxaluria type 1 (PH1) who underwent liver-kidney transplant (LKT), using different approaches and consequently with different outcomes. The first patient is a 32-year-old woman with ESRD on intensive daily hemodialysis (HD) due to PH-1 (homozygous mutation g.12261G>T) with severe systemic oxalosis. Three years after the beginning of dialysis, she underwent a combined simultaneous LKT from a deceased 48-year-old donor. The postoperative period was uneventful and she had immediate diuresis and excellent hepatic function. In an attempt to decrease the serum oxalate pool, continuous venovenous hemodiafiltration was performed for the first 72 h, followed by intensive HD. Her plasma oxalate levels (pOx) progressively decreased while her urinary oxalate levels (uOx) increased at the same rate (Table 1). Three months post-transplant her serum creatinine (sCr) was 1.6 mg/dL pre HD and a renal graft biopsy was performed revealing oxalate deposits on the tubules and intersticium. Intermittent HD was continued for six months and after stopping dialysis she was kept under immunosuppression, bicarbonate therapy and high fluid intake. Although her pOx reached low levels (17 mol/L, normal range = 3–11 mol/dL), one year after LKT, graft dysfunction was present with sCr 4.5 mg/dL. Nevertheless, skin oxalosis, refractory anemia and ventricular dysfunction secondary to oxalate deposits had completely disappeared. The second case is a 26-year-old female with ESRD due to genetic confirmed PH-1 (homozygous mutation p.I244T)

Combined liver and kidney transplantation in two women with primary hyperoxaluria: Different roads led to different outcomes