Bacchetta Justine, Basmaison Odile, Leclerc Anne-Laure, Bertholet-Thomas Aurélia, Cochat Pierre, Ranchin Bruno
Centre de Référence des Maladies Rénales Rares, Service de Néphrologie et Rhumatologie Pédiatriques, Hôpital Femme Mère Enfant, 59 Bd Pinel, 69677, Bron Cedex, France,
Pediatr Nephrol. 2014 Oct;29(10):2061-4. doi: 10.1007/s00467-014-2842-3. Epub 2014 Jun 9.
The management of tubulopathies after renal transplantation (RTx) may require high doses of sodium and bicarbonate, reducing the quality of life and therapeutic compliance of the patient. Some studies on adult patients have highlighted the benefits of fludrocortisone (fludro) in the treatment of severe tubulopathies.
This study was a retrospective review of the medical charts of 15 children, aged 12.4 (range 3.6-17.4) years who received fludro after RTx.
With the administration of fludro, both sodium bicarbonate and chloride supplementation decreased, from 10 (range 0-14) to 0 (0-5) g/day, and from 9 (0-20) to 0 (0-3) g/day, respectively (both p < 0.001). Serum potassium also significantly decreased (4.6 ± 0.4 vs. 3.3 ± 0.6 mmol/L; p < 0.001), but there was no significant effect on renal function. Both systolic and diastolic blood pressure increased significantly. Fludro therapy was stopped in six patients due to side-effects (arterial hypertension, hypokalemia during acute diarrhea, gastric pain, n = 3), parental decision (n = 1), inefficacy and/or non-compliance (n = 1) and scheduled withdrawal (n = 1). Four of these patient had subsequent increasing requirements for bicarbonate and/or sodium supplementation, which ultimately required the re-introduction of fludro in two of these patients.
Based on our findings, fludro would appear to be an effective therapy in most cases of severe tubulopathy after RTx. Further prospective studies are required to validate this indication and to determine the optimal dose and timing of treatment to avoid side-effects as well as the clinical and biological follow-up.
肾移植（RTx）后肾小管疾病的管理可能需要高剂量的钠和碳酸氢盐，这会降低患者的生活质量和治疗依从性。一些针对成年患者的研究强调了氟氢可的松（fludro）在治疗严重肾小管疾病方面的益处。
本研究是一项回顾性研究，对15名RTx后接受fludro治疗的儿童（年龄12.4岁，范围3.6 - 17.4岁）的病历进行了回顾。
随着fludro的使用，碳酸氢钠和氯化物的补充量均减少，分别从10（范围0 - 14）克/天降至0（0 - 5）克/天，以及从9（0 - 20）克/天降至0（0 - 3）克/天（均p < 0.001）。血清钾也显著降低（4.6 ± 0.4对3.3 ± 0.6毫摩尔/升；p < 0.001），但对肾功能无显著影响。收缩压和舒张压均显著升高。6名患者因副作用（动脉高血压、急性腹泻时低钾血症、胃痛，n = 3）、家长决定（n = 1）、无效和/或不依从（n = 1）以及计划停药（n = 1）而停止fludro治疗。其中4名患者随后对碳酸氢盐和/或钠补充剂的需求增加，最终其中2名患者需要重新使用fludro。
基于我们的研究结果，fludro在大多数RTx后严重肾小管疾病病例中似乎是一种有效的治疗方法。需要进一步的前瞻性研究来验证这一适应症，并确定最佳治疗剂量和时间，以避免副作用以及临床和生物学随访。
