Peak ground acceleration(PGA) estimation is an important task in earthquake engineering practice.One of the most well-known models is the Boore-Joyner-Fumal formula,which estimates the PGA using the moment magnitude,t...Peak ground acceleration(PGA) estimation is an important task in earthquake engineering practice.One of the most well-known models is the Boore-Joyner-Fumal formula,which estimates the PGA using the moment magnitude,the site-to-fault distance and the site foundation properties.In the present study,the complexity for this formula and the homogeneity assumption for the prediction-error variance are investigated and an effi ciency-robustness balanced formula is proposed.For this purpose,a reduced-order Monte Carlo simulation algorithm for Bayesian model class selection is presented to obtain the most suitable predictive formula and prediction-error model for the seismic attenuation relationship.In this approach,each model class(a predictive formula with a prediction-error model) is evaluated according to its plausibility given the data.The one with the highest plausibility is robust since it possesses the optimal balance between the data fi tting capability and the sensitivity to noise.A database of strong ground motion records in the Tangshan region of China is obtained from the China Earthquake Data Center for the analysis.The optimal predictive formula is proposed based on this database.It is shown that the proposed formula with heterogeneous prediction-error variance is much simpler than the attenuation model suggested by Boore,Joyner and Fumal(1993).展开更多
PKHD1 mutations are generally considered to cause autosomal recessive polycystic kidney disease(ARPKD).ARPKD is a rare disorder and one o f the most severe conditions leading to end-stage renal disease in childhood.Wi...PKHD1 mutations are generally considered to cause autosomal recessive polycystic kidney disease(ARPKD).ARPKD is a rare disorder and one o f the most severe conditions leading to end-stage renal disease in childhood.With the biallelic deletion mutation,patients have difficulty in surviving the perinatal period,resulting in perinatal or neonatal death.This study retrospectively analyzed patient characteristics,imaging characteristics,laboratory examinations and family surveys from 7 Chinese children with different PKHD1 gene mutations diagnosed by high-throughput sequencing from January 2014 to February 2018.O f the 7 children,there were 3 males and 4 females.Eight missense mutations,two frameshift mutations,two deletion mutations,and two intronic slicing mutations were identified.Six of the mutations have not previously been identified.In the literature search,we identified a total of 29 Chinese children with PKHD1 mutations.The missense mutation c.2507T>C in exon 24 was found in one patient in our study,and five patients with liver fibrosis but normal renal function were reported in the literature.The missense mutation c.5935G>A in exon 37 was found in two patients in our study and three cases in the literature.Four patients had renal failure at an age as young as 1 year of those five patients with the missense mutation c.5935G>A in exon 37.It was concluded that:(1)Kidney length more than 2-3 SDs above the mean and early-onset hypertension might be associated with PKHDI-associated ARPICD;(2)The more enlarged the kidney size is,the lower the renal function is likely to be;(3)c.5935G>A may be a hot spot that leads to early renal failure in Chinese children with PKHD1 mutations;(4)c.2507T>C may be a hot-spot mutation associated with hepatic lesions in Chinese children with PKHD1.展开更多
基金Research Committee of University of Macao under Research Grant No.MYRG081(Y1-L2)-FST13-YKVthe Science and Technology Development Fund of the Macao SAR government under Grant No.012/2013/A1
文摘Peak ground acceleration(PGA) estimation is an important task in earthquake engineering practice.One of the most well-known models is the Boore-Joyner-Fumal formula,which estimates the PGA using the moment magnitude,the site-to-fault distance and the site foundation properties.In the present study,the complexity for this formula and the homogeneity assumption for the prediction-error variance are investigated and an effi ciency-robustness balanced formula is proposed.For this purpose,a reduced-order Monte Carlo simulation algorithm for Bayesian model class selection is presented to obtain the most suitable predictive formula and prediction-error model for the seismic attenuation relationship.In this approach,each model class(a predictive formula with a prediction-error model) is evaluated according to its plausibility given the data.The one with the highest plausibility is robust since it possesses the optimal balance between the data fi tting capability and the sensitivity to noise.A database of strong ground motion records in the Tangshan region of China is obtained from the China Earthquake Data Center for the analysis.The optimal predictive formula is proposed based on this database.It is shown that the proposed formula with heterogeneous prediction-error variance is much simpler than the attenuation model suggested by Boore,Joyner and Fumal(1993).
基金This project was supported by the National Natural Science Foundation of China(No.81873596).
文摘PKHD1 mutations are generally considered to cause autosomal recessive polycystic kidney disease(ARPKD).ARPKD is a rare disorder and one o f the most severe conditions leading to end-stage renal disease in childhood.With the biallelic deletion mutation,patients have difficulty in surviving the perinatal period,resulting in perinatal or neonatal death.This study retrospectively analyzed patient characteristics,imaging characteristics,laboratory examinations and family surveys from 7 Chinese children with different PKHD1 gene mutations diagnosed by high-throughput sequencing from January 2014 to February 2018.O f the 7 children,there were 3 males and 4 females.Eight missense mutations,two frameshift mutations,two deletion mutations,and two intronic slicing mutations were identified.Six of the mutations have not previously been identified.In the literature search,we identified a total of 29 Chinese children with PKHD1 mutations.The missense mutation c.2507T>C in exon 24 was found in one patient in our study,and five patients with liver fibrosis but normal renal function were reported in the literature.The missense mutation c.5935G>A in exon 37 was found in two patients in our study and three cases in the literature.Four patients had renal failure at an age as young as 1 year of those five patients with the missense mutation c.5935G>A in exon 37.It was concluded that:(1)Kidney length more than 2-3 SDs above the mean and early-onset hypertension might be associated with PKHDI-associated ARPICD;(2)The more enlarged the kidney size is,the lower the renal function is likely to be;(3)c.5935G>A may be a hot spot that leads to early renal failure in Chinese children with PKHD1 mutations;(4)c.2507T>C may be a hot-spot mutation associated with hepatic lesions in Chinese children with PKHD1.
