Pediatric Hematology Unit, Emek Medical Center, 21 Yitzhak Rabin St, Afula, Israel.
The Ruth and Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel.
BMC Nephrol. 2021 Dec 20;22(1):418. doi: 10.1186/s12882-021-02630-5.
Renal injury in transfusion dependent β thalassemia patients (TDT) has been attributed to iron overload, chronic anemia and iron-chelation therapy (ICT) toxicity. We studied renal function in TDT patients treated with two different ICT regimes.
We studied 36 TDT patients: 26 received deferasirox (DFX) and 10 were treated with deferoxamine (DFO) +/- deferiprone (DFP).
Increased uNAG was found in 30% of the DFX group vs. 10% of the DFO+/-DFP group, the mean uNAG level in the DFX group was significantly higher than in the DFO+/-DFP group, (P < 0.05). A moderate negative correlation was found between uNAG levels and mean serum ferritin for the prior 10 years (P = 0.03), more pronounced for the DFO+/-DFP group. Twenty nine patients had had their renal function evaluated 10 years earlier; eGFR significantly declined in patients switched to DFX (P = 0.0093) but not in patients who continued DFO+/-DFP.
A high prevalence of renal tubular damage was observed in our TDT patients, particularly those treated with DFX; uNAG was negatively associated with mean 10-year serum ferritin, suggesting ICT's involvement in tubular injury. A significant decline in eGFR compared to a decade earlier was observed only in patients currently treated with DFX. Strict follow-up of renal function in TDT patients is warranted.
依赖输血的β地中海贫血患者(TDT)的肾损伤归因于铁过载、慢性贫血和铁螯合疗法(ICT)毒性。我们研究了接受两种不同 ICT 方案治疗的 TDT 患者的肾功能。
我们研究了 36 名 TDT 患者:26 名接受地拉罗司(DFX)治疗,10 名接受去铁胺(DFO)+/-地拉罗司(DFP)治疗。
DFX 组中有 30%的患者出现 uNAG 升高,而 DFO+/-DFP 组中只有 10%的患者出现 uNAG 升高,DFX 组的 uNAG 水平明显高于 DFO+/-DFP 组(P<0.05)。DFX 组 uNAG 水平与过去 10 年平均血清铁蛋白呈中度负相关(P=0.03),DFO+/-DFP 组更为明显。29 名患者的肾功能在 10 年前进行了评估;与继续 DFO+/-DFP 治疗的患者相比,转换为 DFX 的患者的 eGFR 显著下降(P=0.0093)。
我们的 TDT 患者观察到肾小管损伤的高患病率,特别是那些接受 DFX 治疗的患者;uNAG 与平均 10 年血清铁蛋白呈负相关,表明 ICT 参与了肾小管损伤。与 10 年前相比,只有目前接受 DFX 治疗的患者 eGFR 显著下降。需要对 TDT 患者的肾功能进行严格随访。