埃及患有类固醇抵抗性肾病综合征的儿科患者中的线粒体复合物I缺乏症
Mitochondrial Complex I Deficiency among Egyptian Pediatric Patients with Steroid-Resistant Nephrotic Syndrome.
作者信息
Abdou Doaa M, Mohamed AbdelAal, Abdulhay Mohamed, El Khateeb Sara
机构信息
Clinical and Chemical Pathology, Faculty of Medicine, Cairo University, Cairo 11562, Egypt.
Internal Medicine Department, Faculty of Medicine, Cairo University, Cairo 11562, Egypt.
出版信息
Int J Nephrol. 2021 May 18;2021:6645373. doi: 10.1155/2021/6645373. eCollection 2021.
RESULTS
Positive consanguinity was a remarkable finding in 44 patients among the SRNS group (73%), compared with 33 patients among the SSNS group (55%). Complex I activity was significantly lower in the SRNS group (0.2657 ± 0.1831 nmol/ml/min), than in the SSNS group (0.4773 ± 0.1290 nmol/ml/min) ( < 0.001). There was a significant positive correlation between complex I activity and the heaviness of proteinuria among the SRNS group ( 0.344, < 0.001). There were statistically significant differences in serum C3 and C4 levels between both groups ( < 0.001, 0.053, respectively).
CONCLUSION
Mitochondrial complex I deficiency in patients who have a nephrotic syndrome complaint may play a role in their responsiveness to steroid therapy and the development of SRNS and even the prognosis of their illness.
结果
在类固醇抵抗型肾病综合征（SRNS）组的44例患者中（73%），阳性血缘关系是一个显著发现，而在类固醇敏感型肾病综合征（SSNS）组中为33例患者（55%）。SRNS组的复合体I活性（0.2657±0.1831纳摩尔/毫升/分钟）显著低于SSNS组（0.4773±0.1290纳摩尔/毫升/分钟）（<0.001）。在SRNS组中，复合体I活性与蛋白尿严重程度之间存在显著正相关（r = 0.344，<0.001）。两组之间血清C3和C4水平存在统计学显著差异（分别为<0.001，0.053）。
结论
患有肾病综合征的患者线粒体复合体I缺乏可能在其对类固醇治疗的反应性、SRNS的发展乃至疾病预后中起作用。
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