1.Diagnoses and treatments of pediatric intracranial aneurysms:a clinical analysis of 16 cases
Jie ZHOU ; Changren HUANG ; Luotong LIU ; Jinghu DONG ; Jian ZHOU ; Chengyuan DONG ; Yong JIANG ; Yang MING ; Ligang CHEN
Chinese Journal of Neuromedicine 2016;15(7):713-717
Objective To investigate the clinical features, treatments and prognoses of pediatric intracranial aneurysms. Methods The clinical and follow-up data of 16 consecutive patients with pediatric intracranial aneurysms (≤16 years), admitted to our hospital from January 2003 to December 2014, were analyzed retrospectively. Results Pediatric intracranial aneurysms in this study accounted for 0.78%of all intracranial aneurysms. Of the 16 children, 14 were male, 2 were female. There were 12 anterior circulation aneurysms and 5 posterior circulation aneurysms; there were 4 large aneurysms (diameter 11-25 mm) and 2 giant aneurysms (diameter≥25 mm);there were 14 complex aneurysms. At a mean follow-up duration of 16.8 months, no death was noted. Of the 12 patients received microsurgical therapy, 10 patients had favorable outcomes (modified Rankin scale [mRS] 0-1) and 2 patients had some sequelae: different degrees of disability (mRS 2-4). Of the 4 patients received endovascular therapy, 3 patients had favorable outcomes (mRS 0-1) and one patient had hemiparesis (mRS 2). Conclusions Pediatric intracranial aneurysms are different from adult intracranial aneurysms. The treatment effects and prognosis are relatively well when we select individualized treatment mode according to the clinical features of pediatric intracranial aneurysms.
2.Preliminary mechanism study on the X-linked parkinsonism caused by RAB39B gene mutation c.536dupA
Changhe SHI ; Mengmeng SHI ; Yu FAN ; Zhihua YANG ; Yali DONG ; Chengyuan MAO ; Jing YANG ; Yuming XU
Chinese Journal of Neurology 2020;53(6):416-422
Objective:To detect the expression level of RAB39B gene and the effect of RAB39B on autophagy and α-synuclein, and then investigate the role of RAB39B gene mutation c.536dupA in the pathogenesis of Parkinson′s disease.Methods:Based on the novel RAB39B gene c.536 dupamutation identified in the previous work, the recombinant expression plasmid (pcDNA3.1-HA-RAB39B-536) of RAB39B gene with this mutation and wild-type recombinant expression plasmid (pcDNA3.1-HA-RAB39B) of RAB39B gene were constructed, and the recombinant expression plasmid was transfected into N2a cells with liposome as experimental group. The control group was made up with N2a cells transfected with plasmid pcDNA3.1-HA-RAB39B. Real-time polymerase chain reaction, Western blotting, immunofluorescence and immunoprecipitation techniques were used to detect the expression level of mutant RAB39B gene and the effects of RAB39B on autophagy and α-synuclein.Results:In the N2a cell model, the transcription level of mutant RAB39B was about twice that of wild type RAB39B, while the protein level of mutant RAB39B (0.30±0.00) was significantly lower than that of wild type (1.50±0.25, t=8.313, P<0.05). After adding proteasome inhibitor MG132, the protein level of mutant RAB39B increased (0.70±0.10, t=6.925, P<0.05); the level of microtubule-associated protein 1 light chain 3 BⅡ/Ⅰ of mutant RAB39B (3.11±0.30) was significantly lower than that of wild type (7.03±0.20, t=18.831, P<0.05); overexpression of wild type and mutant RAB39B did not affect the level of endogenous α-synuclein; overexpression of wild-type RAB39B resulted in elevated level of exogenous wild-type (p.A53T; from 0.60±0.11 to 1.25±0.08, t=8.254, P<0.05) and mutant (from 0.55±0.08 to 1.15±0.08, t=9.293, P<0.05) α-synuclein. Conclusions:The stability of the RAB39B protein decreased with the appearance of c.536 dupA mutation, the mutant protein may be degraded through the ubiquitin-proteasome pathway, and this mutation may affect the autophagy level of cells. RAB39B protein may interact with α-synuclein in vivo and may be involved in the maintenance of the stable level of α-synuclein.
3.Two decomposition algorithms of dual-energy cone beam CT and their dependence on the phantom sizes
Chenguang LI ; Tianye NIU ; Li ZHOU ; Jun DENG ; Chengyuan ZOU ; Sha LI ; Hongjia LIU ; Zhengkun DONG ; Ling HUA ; Yichen PU ; Liangzi QU ; Qiao LI ; Yibao ZHANG
Chinese Journal of Radiological Medicine and Protection 2022;42(4):269-276
Objective:To analyze the effects of two decomposition algorithms of dual-energy cone beam CT (DECBCT) (direct decomposition and iterative decomposition) on the image quality and material decomposition accuracy of different sizes of phantoms.Methods:Different sizes of imaging parts of patients were simulated using the combination of CatPhan604 phantoms and customized annuluses. CBCT with high energy of 140 kVp and low energy of 100 kVp were acquired using the Varian Edge CBCT system. Then the material decomposition of DECBCT images was performed using the two algorithms. The electron density (ED) and contrast-to-noise ratio (CNR) of each material in the CTP682 module were calculated. They were used to assess the decomposition accuracy and image quality of the two algorithms.Results:Based on the values in the Catphan604 manual, both algorithms have high ED accuracy. Only the ED accuracy of four materials of the smallest sized phantom showed statistical difference ( z = -4.21, 4.30, 2.87, 5.45, P < 0.05), but the average relative error was less than 1%. The CNR of the iterative decomposition algorithm was significantly higher than that of the direct decomposition, increasing by 51.8%-703.47%. The increase in the phantom size significantly reduced the accuracy of ED, and the increased amplitude of the relative error was up to a maximum of 2.52%. The large phantom size also reduced the image quality of iterative decomposition, and the decreased amplitude of CNR was up to a maximum of 39.71. Conclusions:Compared with the direct decomposition, the iterative decomposition algorithm can significantly reduce the image noise and improve the contrast without losing the accuracy of electron density in the DECBCT construction of different sizes of phantoms.