Niemczyk Mariusz, Niemczyk Stanisław, Bujko Maciej, Pączek Leszek
Department of Immunology, Transplant Medicine, and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.
Department of Internal Diseases, Nephrology and Dialysis, Military Institute of Medicine, Warsaw, Poland.
Neurol Neurochir Pol. 2015;49(2):126-8. doi: 10.1016/j.pjnns.2015.02.002. Epub 2015 Feb 27.
Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder that affects 1 in 1.000 people in the general population, and is connected to increased risk for intracranial aneurysms (ICANs). In this work, we made an attempt to identify risk factors for subarachnoid hemorrhage (SAH) due to a rupture of an ICAN.
Retrospective analysis of series of cases including 8 ADPKD patients who passed a nonfatal stroke due to the rupture of an ICAN.
The mean age at SAH was 40.13 years. In 88% of patients the renal function at SAH was normal. At least 63% of patients were hypertensive. The family history for ICAN and/or SAH was positive in 38% of patients, and in 63% of patients there were neurological symptoms preceding SAH, with headache as the most common one.
Similarly to ICAN and/or SAH in the family history, headache and/or other neurological symptoms should be indication for screening for ICANs in all ADPKD patients irrespectively of their age. We propose a set of criteria, which may enable detection of most cases of ICANs in ADPKD patients.
常染色体显性多囊肾病（ADPKD）是一种遗传性疾病，在普通人群中发病率为千分之一，且与颅内动脉瘤（ICANs）风险增加有关。在本研究中，我们试图确定因ICAN破裂导致蛛网膜下腔出血（SAH）的危险因素。
对8例因ICAN破裂发生非致命性卒中的ADPKD患者进行病例系列回顾性分析。
SAH发生时的平均年龄为40.13岁。88%的患者SAH发生时肾功能正常。至少63%的患者患有高血压。38%的患者ICAN和/或SAH家族史呈阳性，63%的患者SAH前有神经症状，其中头痛最为常见。
与家族史中的ICAN和/或SAH类似，无论年龄大小，头痛和/或其他神经症状都应作为所有ADPKD患者筛查ICAN的指征。我们提出了一套标准，可用于检测大多数ADPKD患者的ICAN病例。
