Unidad de Gestión Clínica de Nefrología, Hospital Universitario de Jaén, Jaén, Spain.
Unidad de Gestión Clínica de Nefrología, Hospital Regional Universitario "Reina Sofía" de Córdoba, Córdoba, Spain.
J Am Soc Nephrol. 2023 Jun 1;34(6):944-950. doi: 10.1681/ASN.0000000000000130. Epub 2023 Mar 30.
Autosomal dominant polycystic kidney disease (ADPKD) is a disease characterized by a progressive kidney growth due to the development of cysts that lead to gradual destruction of the surrounding parenchyma. In the first stage, the estimated GFR will remain stable despite the reduction of the renal parenchyma because of an increase in glomerular hyperfiltration. The total kidney volume (TKV) measured with computed tomography or magnetic resonance imaging is related to the future GFR decline. Thus, TKV has become an early marker to be analyzed in all patients with ADPKD. In addition, in recent years, it has been pointed out that kidney growth rate estimated with a single TKV measurement can be a clear prognostic marker for future glomerular filtration decline. However, there is no consensus on how to measure kidney volume growth in ADPKD, so each author has used different models that, not having the same meaning, have been handled as if they produced similar values. This may lead to erroneous estimates of kidney growth rate with the consequent prognostic error. The Mayo Clinic classification is now the most widely accepted prognostic model in clinical practice to predict patients who will deteriorate faster and to decide what patients should be treated with tolvaptan. However, some aspects of this model have not been discussed in depth. Our aim in this review was to present the models that can be used to estimate kidney volume growth rate in ADPKD, to facilitate their applicability in daily clinical practice.
常染色体显性多囊肾病（ADPKD）是一种以囊肿发展导致周围实质逐渐破坏为特征的进行性肾脏增大的疾病。在第一阶段，尽管由于肾小球高滤过导致肾实质减少，但估计的肾小球滤过率（eGFR）仍保持稳定。通过计算机断层扫描或磁共振成像测量的总肾体积（TKV）与未来的 eGFR 下降有关。因此，TKV 已成为所有 ADPKD 患者分析的早期标志物。此外，近年来，有人指出，单次 TKV 测量估计的肾脏生长速度可以作为未来肾小球滤过下降的明确预后标志物。然而，目前尚无关于如何在 ADPKD 中测量肾脏体积增长的共识，因此每位作者都使用了不同的模型，这些模型没有相同的含义，但被视为产生相似值的模型。这可能导致对肾脏生长速度的错误估计，从而导致预后错误。目前，Mayo 临床分类是临床实践中最广泛接受的预测模型，用于预测病情恶化较快的患者，并决定哪些患者应接受托伐普坦治疗。然而，该模型的某些方面尚未深入讨论。我们在本综述中的目的是介绍可用于估计 ADPKD 中肾脏体积增长率的模型，以促进其在日常临床实践中的适用性。
