Relationship between intracranial aneurysms and the severity of autosomal dominant polycystic kidney disease

Relationship between intracranial aneurysms and the severity of autosomal dominant polycystic kidney disease
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DOI:
10.1007/s00701-017-3316-8
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发表时间:
2017-09
影响因子:
2.4
通讯作者:
H. Yoshida;E. Higashihara;K. Maruyama;K. Nutahara;T. Nitatori;Isao Miyazaki;Y. Shiokawa
H. Yoshida;E. Higashihara;K. Maruyama;K. Nutahara;T. Nitatori;Isao Miyazaki;Y. Shiokawa
中科院分区:
医学3区
文献类型:
--
作者:
H. Yoshida;E. Higashihara;K. Maruyama;K. Nutahara;T. Nitatori;Isao Miyazaki;Y. Shiokawa

文献摘要

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常染色体显性多囊肾病(ADPKD)是一种遗传性肾脏疾病,其特征是无数肾囊肿进行性扩大。尽管颅内动脉瘤(ICANs)与ADPKD的关联是众所周知的,但ICANs与疾病严重程度(包括总肾体积(TKV)和估计肾小球滤过率(eGFR))之间的关系尚不清楚。方法对265例ADPKD患者(平均48.8岁,范围14.9 ~ 88.3岁)进行MR血管造影筛查。既往有ICANs病史的患者被排除在研究之外。评价ADPKD患者ICAN的发生率及特点。通过磁共振成像的体积分析来测量TKV。结果49例患者中检测到65例ICANs,其中女性37例,男性12例,平均年龄52.7岁,范围20.4-86岁。ICANs的发生率为18.5%,女性患者的发生率(23.1%)高于男性患者(11.4%)(p= 0.02)。ICANs患者的年龄明显高于无ICANs患者(p= 0.006),且ICANs的累积诊断风险随年龄增加而增加。ICANs组TKV明显高于无ICANs组(p= 0.001),但两组间eGFR无差异(p= 0.07)。通过多变量分析,只有TKV与ICANs的发生有显著相关性(p= 0.02)。结论ICANs的发生率随着年龄的增长而增加,女性发生率较高,且与ADPKD患者肾脏增大有关。筛查ICANs的必要性在肾大的老年妇女中尤其高。
BackgroundAutosomal dominant polycystic kidney disease (ADPKD) is a hereditary kidney disease characterized by the progressive enlargement of innumerable renal cysts. Although the association of intracranial aneurysms (ICANs) with ADPKD is well known, the relationship between the ICAN and the disease severity including total kidney volume (TKV) and estimated glomerular filtration rate (eGFR) is poorly understood.MethodsWe screened 265 patients with ADPKD (mean age, 48.8 years; range, 14.9–88.3 years) with MR angiography. The patients with a past history related to ICANs were excluded from the study. The incidence and characteristics of ICAN in patients with ADPKD were evaluated. TKV was measured by volumetric analyses of MR imaging.ResultsWe detected 65 ICANs in 49 patients (37 women and 12 men, mean age, 52.7 years; range, 20.4–86 years). The incidence of ICANs was 18.5% and female patients had was higher incidence (23.1%) than male patients (11.4%) (p= 0.02). An age of those with ICANs was significantly higher than those without (p= 0.006), and the cumulative risk of diagnosis of ICANs increased with age. TKV was significantly larger in those with ICANs than those without (p= 0.001), but eGFR was not different between two groups (p= 0.07). By multivariate analyses, only TKV was significantly related to the development of ICANs (p= 0.02).ConclusionsThe incidence of ICANs increased with age, was higher in females, and correlated with kidney enlargement in patients with ADPKD. Necessity of screening ICANs would be particularly high in elderly women with large kidneys.