Martínez Mejía S, Alonso Melgar A, Melgosa Hijosa M, Fernandez Camblor C, Peña Carrión A, García Meseguer C, Espinosa Román L
Nefrología Infantil La Paz, Madrid, Spain.
Nefrología Infantil La Paz, Madrid, Spain.
Transplant Proc. 2015 Jan-Feb;47(1):38-41. doi: 10.1016/j.transproceed.2014.11.022.
The aim of this work was to analyze the evolution of the 1st renal transplantation in children with nephrotic syndrome in the 1st year of life (NSFL).
In this retrospective study of 15 patients (8 women and 7 men) with NSFL receiving transplants from 1989 to 2013, 9 had NS of Finnish type, 4 diffuse mesangial sclerosis, 1 minimal changes, and 1 collapsing glomerulopathy. We analyzed the clinical and analytic situation at 4 time points: before dialysis, before transplantation, 3 months after transplantation, and long-term evolution.
Mean follow-up was 72.8 months (range, 1 month to 16.9 years); mean age at diagnosis was 2.21 months (range, 0-8.2 months); mean age at onset of replacement therapy was 22.9 ± 16.4 months (range, 3.8-55.4 months); and mean time on dialysis was 14.9 months (range, 2-44 months). Age at transplantation was 3.1 years (range, 1.8 to 7.7 years), with 6 living-donor transplantations (LDTs) and 9 cadaveric (CDTs). Ten patients required nephrectomy before transplantation (9 bilateral) to control proteinuria after 3.1 ± 3.8 months on dialysis, 1 during transplantation, and 3 after transplantation (2 persistent proteinuria, 1 hypertension). Mean time on dialysis for LDTs was 5.4 ± 2.7 months versus 13.2 ± 6.9 months for CDTs (P < .005). Mean age of cadaveric donors was 6.2 ± 2.4 years and that of living donors 35.5 ± 7.9 years. As complications, there was 1 bleeding from venous anastomosis and 1 urinary leakage after surgery. After 6 ± 5.2 years of evolution, actuarial survival at both 1 and 7 years was 92.9%. One graft was lost owing to acute rejection 1 month after transplantation and 2 others owing to chronic rejection >9 years after transplantation. None had disease recurrence.
Short-term complications did not differ from the rest of population if transplantation occurred with standard albumin levels, for which most required pre-transplantation nephrectomy because dialysis failed to reduce proteinuria.
本研究旨在分析1岁以内肾病综合征患儿（NSFL）首次肾移植术后第一年的病情演变情况。
本回顾性研究纳入了1989年至2013年间接受肾移植的15例NSFL患者（8例女性，7例男性），其中9例为芬兰型肾病，4例为弥漫性系膜硬化，1例为微小病变，1例为塌陷性肾小球病。我们分析了4个时间点的临床和分析情况：透析前、移植前、移植后3个月以及长期演变情况。
平均随访时间为72.8个月（范围为1个月至16.9年）；诊断时的平均年龄为2.21个月（范围为0至8.2个月）；替代治疗开始时的平均年龄为22.9±16.4个月（范围为3.8至55.4个月）；透析的平均时间为14.9个月（范围为2至44个月）。移植时的年龄为3.1岁（范围为1.8至7.7岁），其中6例为活体供肾移植（LDT），9例为尸体供肾移植（CDT）。10例患者在透析3.1±3.8个月后、1例在移植期间、3例在移植后（2例持续性蛋白尿，1例高血压）需要在移植前进行肾切除术以控制蛋白尿。LDT的平均透析时间为5.4±2.7个月，而CDT为13.2±6.9个月（P <.005）。尸体供者的平均年龄为6.2±2.4岁，活体供者为35.5±7.9岁。作为并发症，术后有1例静脉吻合口出血和1例尿漏。经过6±5.2年的演变，1年和7年的精算生存率均为92.9%。1例移植肾在移植后1个月因急性排斥反应丢失，另外2例在移植后9年以上因慢性排斥反应丢失。无一例疾病复发。
如果在标准白蛋白水平下进行移植，短期并发症与其他人群无差异，其中大多数患者因透析未能降低蛋白尿而需要在移植前进行肾切除术。
