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Abstract: TH-PO862

Frequency of Polycystic Kidney Disease in Patients with Intracranial Aneurysm

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Iwatani, Hirotsugu, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Koizumi, Shintaro, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Yajima, Ayako, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Shigeki, Takatomo, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Kimura, Yoshiki, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Fujinaka, Toshiyuki, National Hospital Organization Osaka National Hospital, Osaka, Japan
  • Yamato, Masafumi, National Hospital Organization Osaka National Hospital, Osaka, Japan
Background

Polycystic kidney disease (PKD) is characterized by the multiple cystic formation in the bilateral kidneys. It is also famous for the highly accompanying extra-renal manifestations such as intracranial aneurysm, valvular heart disease, liver cysts, pancreatic cysts, or intestinal diverticulum. The underlying and unifying mechanism of PKD is the abnormality of cilia. The frequency of intracranial aneurysm in patients with PKD is reported to be approximately 4 to 12%. However, the rate of PKD in patients with intracranial aneurysm is not known. Here, we aimed to investigate the frequency of PKD in patients with intracranial aneurysm.

Methods

Seventy-two patients with intracranial aneurysm who visited department of neurosurgery in our hospital, also visited department of nephrology between Nov 2017 and May 2019 for PKD screening. The screening modality was basically kidney ultrasound. If abdominal CT or MRI was already performed, we utilized them to diagnose whether or not the patients have PKD. In cases where PKD is highly suspected by questionnaire to the patients, we performed abdominal MRI or CT. We also investigated the family history of kidney disease, dialysis, intracranial hemorrhage, intracranial aneurysm, sudden death, or liver cysts. Thus we retrospectively investigated the PKD rate in cases with intracranial aneurysm. The ethics committee in our hospital approved this retrospective study.

Results

The patients’ characteristics were as follows; age median 69 IQR (53-76) y.o., sex F/M 60/12, eGFR 69 (61-81) mL/min/1.73m2. Out of the 72 cases, 43 patients had single intracranial aneurysm and 29 patients had plural intracranial aneurysm. Out of 72 patients, typical PKD was detected in one patient, and atypical PKD in another patient. Family history taking showed that 9 patients had relatives with kidney diseases, 4 had those with dialysis, 7 had those with intracranial aneurysm, 14 had those with intracranial hemorrhage, 14 had those with sudden deaths, and 2 had those with liver cysts.

Conclusion

PKD screening indicated that approximately 1-3% of patents with intracranial aneurysm had PKD.