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常染色体显性遗传多囊肾病早、晚期的肾功能储备能力。

Kidney Function Reserve Capacity in Early and Later Stage Autosomal Dominant Polycystic Kidney Disease.

机构信息

Departments of Nephrology and.

Urology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; and.

出版信息

Clin J Am Soc Nephrol. 2018 Nov 7;13(11):1680-1692. doi: 10.2215/CJN.03650318. Epub 2018 Sep 25.

Abstract

BACKGROUND AND OBJECTIVES

It is assumed that in autosomal dominant polycystic kidney disease (ADPKD), kidney function remains in the normal range for several decades because of hyperfiltration of remnant nephrons. In this study, we investigate the extent to which patients with ADPKD hyperfilter.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this cross-sectional study, we measured GFR as urinary clearance using continuous infusion of I-iothalamate. Kidney function reserve capacity was determined as increase in measured GFR after adding a dopamine infusion of 4.4-6 mg/h. Potential kidney donors were used as healthy controls and matched by age and sex to patients with ADPKD for comparisons across age groups and CKD stages. Hyperfiltration was defined by a loss of kidney function reserve capacity compared with healthy controls.

RESULTS

A total of 300 participants were studied. In the youngest age group (18-29 years), measured GFR was not different between patients with ADPKD and healthy controls (103±21 versus 111±9 ml/min per 1.73 m; =0.14). In this age group kidney function reserve capacity was higher compared with healthy controls (11.1%±8.3% versus 5.3%±6.5%; =0.04). Moreover, kidney function reserve capacity was similar to healthy controls in patients with ADPKD with early-stage disease (eGFR≥60 ml/min per 1.73 m), either overall or when divided into fast or slow progressors according to their Mayo height-adjusted total kidney volume class. However, in patients with ADPKD, lower measured GFR was associated with lower kidney function reserve capacity (=1.0 [95% confidence interval, 0.5 to 1.5] % per 10 ml/min per 1.73 m; <0.001). Kidney function reserve capacity was therefore lower compared with healthy controls at older age and later CKD stages.

CONCLUSIONS

Patients with early-stage ADPKD, either classified as having rapidly or slowly progressive disease, are able to increase their GFR in response to dopamine. Hyperfiltration, defined by a loss of kidney function reserve capacity, may therefore not be an early phenomenon in ADPKD.

摘要

背景与目的

据推测,在常染色体显性多囊肾病(ADPKD)中,由于残余肾单位的超滤,肾功能在几十年内仍保持在正常范围内。在这项研究中,我们调查了 ADPKD 患者超滤的程度。

设计、地点、参与者和测量:在这项横断面研究中,我们使用连续输注碘海醇测量 GFR。通过添加 4.4-6mg/h 的多巴胺输注来确定肾功能储备能力的增加,作为测量 GFR 的增加。潜在的肾脏供体被用作健康对照组,并按年龄和性别与 ADPKD 患者相匹配,以便在不同年龄组和 CKD 阶段进行比较。与健康对照组相比,肾功能储备能力的丧失被定义为超滤。

结果

共研究了 300 名参与者。在最年轻的年龄组(18-29 岁),ADPKD 患者和健康对照组之间的测量 GFR 没有差异(103±21 与 111±9 ml/min/1.73 m;=0.14)。在这个年龄组,肾功能储备能力高于健康对照组(11.1%±8.3%与 5.3%±6.5%;=0.04)。此外,在疾病早期(eGFR≥60ml/min/1.73 m)的 ADPKD 患者中,无论是整体还是根据 Mayo 身高调整的总肾脏体积分类将其分为快速或缓慢进展者,肾功能储备能力与健康对照组相似。然而,在 ADPKD 患者中,较低的测量 GFR 与较低的肾功能储备能力相关(=1.0[95%置信区间,0.5 至 1.5]%/每 10ml/min/1.73 m;<0.001)。因此,与健康对照组相比,肾功能储备能力在年龄较大和较晚的 CKD 阶段较低。

结论

无论是被归类为快速还是缓慢进展性疾病的早期 ADPKD 患者,都能够增加他们的 GFR 以响应多巴胺。因此,超滤(定义为肾功能储备能力的丧失)可能不是 ADPKD 的早期现象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3bc3/6237049/0ec376646617/CJN.03650318absf1.jpg

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