在患有多囊性病的儿童中,脏测量和淋巴膜过率的演变
Ramona Stroescu1,2, Mihai Gafencu2,3, Ruxandra Maria Steflea2,3
1Department XI of Pediatrics-1st Pediatric Discipline, Center for Research on Growth and Developmental Disorders in Children, "Victor Babes" University of Medicine and Pharmacy Timisoara, Eftimie Murgu Sq. No. 2, 300041 Timisoara, Romania.
Children (Basel, Switzerland)
|May 25, 2024
概括
儿童自身主导性多囊性病 (ADPKD) 经常表现为正常的功能,尽管脏膨胀. 膜过率 (qGFR) 的二次估计比施瓦茨公式提供了更准确的评估,避免了虚假的过分类.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 儿科脏病学 儿科脏病学
- 医学遗传学 医学遗传学
背景情况:
- 自体主导多囊性病 (ADPKD) 是一种遗传性疾病,导致管囊扩张和扩大.
- 在ADPKD中,囊性扩张减少了功能性脏,导致功能逐渐下降,并导致诸如膜过,白色素尿,蛋白尿和血液尿等并发症.
- 儿童的ADPKD呈现有很大差异,从无症状病例到快速进展,早期发病 (<2年) 显示功能下降的速度更快.
研究的目的:
- 在患有ADPKD的儿童中,探索膜过率 (GFR) 动态和脏超声波调整的百分位.
- 为了比较修改的施瓦茨公式的准确性与二次方程来估计儿科ADPKD患者的GFR.
- 用不同的GFR估计方法评估ADPKD儿童球过的患病率.
主要方法:
- 对16名患有ADPKD的儿科患者进行了回顾性分析.
- 使用修改的施瓦茨公式和二次方程 (qGFR) 调整血清肌素后,测量球过率 (GFR).
- 评估脏大小使用脏超声波调整百分位数和与GFR动态的相关性在1年的随访.
主要成果:
- 在16名患者中,有14名患者的百分比超过90%.
- 在二次方程 (qGFR) 估计较低的GFR值 (111.95±12.43毫升/分钟/1.73米2),与施瓦茨eGFR (126.28±33.07毫升/分钟/1.73米) 相比,p = 0.14.
- 使用qGFR,没有患者达到淋巴细胞过的值,GFR呈现线性或略有下降的趋势 (ΔeGFR = -0.32 ± 5.78 mL/min/1.73 m2),与施瓦茨公式不同,它可能错误地表明过 (ΔeGFR = 7.51 ± 19.46 mL/min/1.73 m2),p = 0.019.
结论:
- 患有ADPKD的儿童经常有缩的脏,但可能保持正常或接近正常的功能.
- 二次方程为儿科ADPKD的GFR提供了更可靠的估计,准确地反映了功能动态,避免了过的错误分类.
- 准确的GFR评估对于管理儿科ADPKD,指导治疗策略和预测疾病进展至关重要.
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