Pediatric combined living donor liver and kidney transplantation for primary hyperoxaluria type 2

Ramakrishna, Somashekara Hosaagrahara; Hassan, Amel Ahmed; Kasala, Mohan Babu; Karnan, Perumal; Venkategowda, Chaitra; Malleeswaran, Selvakumar; Periasamy, Muthukumar; Muruganandham, K; Patcha, Rajanikanth; Varghese, Joy; Reddy, Mettu Srinivas

doi:10.1016/j.ajt.2023.05.006

Cited by 3 publications

(1 citation statement)

References 13 publications

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“…Arnaud et al reported an illustrative case: a 41-year-old male PH2 patient experienced the rapid and severe recurrence of oxalate nephropathy after isolated KT, leading to early graft loss, yet he was subsequently re-treated with CLKT without relapse and with successful resolution [112]. In the past few years, two successful CLKT treatments in PH2 patients have been reported: a 44-year-old man with frequent stone events and ESRD, and a 12-year-old boy with systemic oxalosis and ESRD [113,114]. The deficient enzyme in PH2 lacks liver specificity, historically suggesting isolated KT for such patients [9].…”

Section: Surgical Interventionsmentioning

confidence: 99%

Navigating the Evolving Landscape of Primary Hyperoxaluria: Traditional Management Defied by the Rise of Novel Molecular Drugs

Huang,

Zhu,

Zhou

et al. 2024

Biomolecules

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Primary hyperoxalurias (PHs) are inherited metabolic disorders marked by enzymatic cascade disruption, leading to excessive oxalate production that is subsequently excreted in the urine. Calcium oxalate deposition in the renal tubules and interstitium triggers renal injury, precipitating systemic oxalate build-up and subsequent secondary organ impairment. Recent explorations of novel therapeutic strategies have challenged and necessitated the reassessment of established management frameworks. The execution of diverse clinical trials across various medication classes has provided new insights and knowledge. With the evolution of PH treatments reaching a new milestone, prompt and accurate diagnosis is increasingly critical. Developing early, effective management and treatment plans is essential to improve the long-term quality of life for PH patients.

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Section: Surgical Interventionsmentioning

confidence: 99%

Navigating the Evolving Landscape of Primary Hyperoxaluria: Traditional Management Defied by the Rise of Novel Molecular Drugs

Huang,

Zhu,

Zhou

et al. 2024

Biomolecules

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Primary hyperoxaluria: Long‐term outcomes of isolated kidney versus simultaneous liver/kidney transplant

Habash,

Jaoudeh,

Hentz

et al. 2024

J. pediatr. gastroenterol. nutr.

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ObjectivesTo compare long‐term transplant outcomes (organ rejection and retransplant) of simultaneous liver/kidney transplant (SLK) versus isolated kidney transplant (IK) for patients with primary hyperoxaluria (PH).MethodsThe Rare Kidney Stone Consortium PH registry was queried to identify patients with PH who underwent SLK or IK from 1999 to 2021. Patient characteristics and long‐term transplant outcomes were abstracted and analyzed. Statistical comparisons were performed with Kaplan–Meier plots and Cox proportional hazards models.ResultsWe identified 250 patients with PH, of whom 35 received care at Mayo Clinic and underwent SLK or IK. Patients who underwent SLK as their index transplant had lower odds of kidney rejection than did those who underwent IK (hazard ratio [HR], 0.29; 95% confidence interval [CI], 0.08–0.99; p = .048). The immunoprotective effect of concomitant liver and kidney transplant appeared to enhance outcomes for patients with PH. Additionally, the odds of retransplant were significantly lower for patients who underwent SLK as their index transplant than for those who underwent IK (HR, 0.08; 95% CI, 0.02–0.42; p = .003). Of five patients who underwent IK and had maintained graft function for at least 5 years after transplant, three (60%) had documented vitamin B6 responsiveness.ConclusionsPatients with PH who underwent SLK had a lower risk of kidney rejection and retransplant than those who underwent IK. Accurate genetic assessment for vitamin B6 responsiveness may optimize IK allocation. Novel therapeutics, such as lumasiran, have been introduced as promising agents for the management of PH.

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Clinical features and mutational spectrum of Chinese patients with primary hyperoxaluria type 2

Liu,

Zhao,

et al. 2024

Urolithiasis

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Pediatric combined living donor liver and kidney transplantation for primary hyperoxaluria type 2

Cited by 3 publications

References 13 publications

Navigating the Evolving Landscape of Primary Hyperoxaluria: Traditional Management Defied by the Rise of Novel Molecular Drugs

Navigating the Evolving Landscape of Primary Hyperoxaluria: Traditional Management Defied by the Rise of Novel Molecular Drugs

Primary hyperoxaluria: Long‐term outcomes of isolated kidney versus simultaneous liver/kidney transplant

Clinical features and mutational spectrum of Chinese patients with primary hyperoxaluria type 2

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