肝肾联合移植治疗儿童原发性高草酸尿症1型1例并文献复习
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南京医科大学附属儿童医院

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Combined liver and kidney transplantation for primary hyperoxaluria type 1 in children: a case report and literature review
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    摘要:

    目的:探讨肝肾联合移植治疗儿童原发性高草酸尿症1型的疗效。方法:回顾性分析南京医科大学附属儿童医院肾脏科1例行肝肾联合移植术的原发性高草酸尿症1型患儿的术前及术后临床资料,并复习相关文献。结果:患儿,男,16岁。1岁时发现双肾多发结石,曾在外院行碎石、取石等治疗,效果不佳。10岁时外院基因检测示AGXT基因复合杂合突变,此时已出现肾功能不全,并于半年内快速进展至终末期肾病。在我院规律行高通量血液透析2年后,于外院行肝肾联合移植,术后一周出现少尿及尿中间断排石,复查血肌酐200μmol/L,口服氢氯噻嗪、枸橼酸钾等治疗,血肌酐维持在140~150μmol/L。肝肾联合移植术后1.5年行移植肾肾活检,病理提示移植肾临界改变。肝肾联合移植术后3.5年,血肌酐稳定在140μmol/L左右。检索到6篇文献共10例原发性高草酸尿症1型患者,接受肝肾联合移植术同时切除双侧自体肾,随访1~9年,移植肾肾功能均正常。结论:肝肾联合移植术是治疗儿童原发性高草酸尿症1型终末期肾病的有效手段,但自体肾作为全身草酸盐主要储库,术后草酸盐可能被动员到血浆和尿液中,导致移植肾肾功能不全。接受肝肾联合移植术同时切除双侧自体肾,可能更有效保护移植肾肾功能。

    Abstract:

    Objective: To investigate the effect of combined liver and kidney transplantation on primary hyperoxaluria type 1 (PH1) in children. Methods: The preoperative and postoperative clinical data of a patient with PH1 who underwent combined liver and kidney transplantation in the Kidney Department of the Children""s Hospital of Nanjing Medical University were analyzed retrospectively, and the related literature was reviewed. Results: The patient was a 16 years old boy. At the age of 1, he found multiple stones in both kidneys. He had been treated with lithotripsy and stone removal in other hospitals, and the effect was poor. At the age of 10, the gene detection showed that the AGXT gene had a complex heterozygous mutation. At this time, renal insufficiency had occurred, and it rapidly progressed to end-stage renal disease within half a year. Two years after regular high flux hemodialysis in our hospital, he performed combined liver and kidney transplantation in another hospital.One week after the operation, oliguria and urinary incontinence occurred, and the serum creatinine was rechecked at 200μmol/L. After oral hydrochlorothiazide, potassium citrate and other treatments, serum creatinine maintained at 140~150μmol/L. One and a half years after combined liver and kidney transplantation, renal biopsy of the transplanted kidney was performed. Pathology showed that the transplanted kidney had critical changes. 3.5 years after combined liver and kidney transplantation, serum creatinine was stable at 140μmol/L. A total of 10 patients with PH1 received combined liver and kidney transplantation and bilateral autologous kidneys were reviewed. The patients were followed up for 1-9 years, and the renal function of the transplanted kidneys was normal. Conclusion: Combined liver and kidney transplantation is an effective method for the treatment of end-stage renal disease caused by PH1 in children. However, since the autologous kidney is the main repository of oxalate in the whole body, oxalate may be mobilized into the plasma and urine after surgery, resulting in renal dysfunction of the transplanted kidney. It may be more effective to protect the renal function of the transplanted kidney by simultaneous resection of bilateral autologous kidneys after combined liver and kidney transplantation.

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  • 收稿日期:2023-02-07
  • 最后修改日期:2023-04-11
  • 录用日期:2023-08-29
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