Woo Hyun Ah, Lee Hyeonju, Choi Young Hun, Min Jeesu, Kang Hee Gyung, Ahn Yo Han, Lee Hyun Kyung
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea.
Front Pediatr. 2023 Oct 9;11:1214704. doi: 10.3389/fped.2023.1214704. eCollection 2023.
We evaluated the long-term clinical outcomes of nephrocalcinosis (NC) according to etiology and grade in preschool-age children with NC.
We retrospectively analyzed the clinical outcomes and disease grade of children with NC classified into three groups according to etiology: prematurity, tubular disorders, and others.
Overall, 67 children were diagnosed with NC [median age, 0.76 years; interquartile range (IQR) 0.46-2.14 years]. The etiologies of NC included prematurity (28.4%), tubular disorders (25.4%), and others (46.3%). Moreover, 56 (83.6%) children were asymptomatic and diagnosed accidentally through kidney ultrasonography. Newly diagnosed underlying diseases were greater in the tubular disorders group than in the other two groups ( = 0.001). Significantly more newly diagnosed NCs were grade 3 than grade 1 ( = 0.003). The median estimated glomerular filtration rate (eGFR) changed from 96.1 (IQR 68.8-119.2) ml/min/1.72 m at diagnosis to 90.9 (IQR 76.4-106.4) ml/min/1.72 m at the last follow-up, without a significant difference ( = 0.096). Changes in the kidney function did not differ according to etiology. However, patients without improvement in NC grade showed a decrease in eGFR from 98.1 (IQR 71.1-132.9) to 87.4 (IQR 74.0-104.1) ml/min/1.73 m (= 0.023), while patients with improved NC grade did not show any change in the kidney function.
Early recognition, especially in NC grade 3, can help uncover further diagnoses, such as tubular disorders. Long-term kidney function depends on whether the NC grade improves.
我们根据病因和分级评估了学龄前肾钙质沉着症（NC）患儿的长期临床结局。
我们回顾性分析了根据病因分为三组的NC患儿的临床结局和疾病分级：早产、肾小管疾病和其他病因。
总体而言，67名儿童被诊断为NC[中位年龄，0.76岁；四分位间距（IQR）0.46 - 2.14岁]。NC的病因包括早产（28.4%）、肾小管疾病（25.4%）和其他病因（46.3%）。此外，56名（83.6%）儿童无症状，通过肾脏超声意外诊断。肾小管疾病组新诊断的潜在疾病比其他两组更多（P = 0.001）。新诊断的NC中3级明显多于1级（P = 0.003）。估计肾小球滤过率（eGFR）中位数从诊断时的96.1（IQR 68.8 - 119.2）ml/min/1.72 m²降至末次随访时的90.9（IQR 76.4 - 106.4）ml/min/1.72 m²，无显著差异（P = 0.096）。肾功能变化根据病因无差异。然而，NC分级无改善的患者eGFR从98.1（IQR 71.1 - 132.9）降至87.4（IQR 74.0 - 104.1）ml/min/1.73 m²（P = 0.023），而NC分级改善的患者肾功能无变化。
早期识别，尤其是3级NC，有助于发现进一步的诊断，如肾小管疾病。长期肾功能取决于NC分级是否改善。
