A new extended distribution called the Odd Exponential Generalized Exponential-Exponential distribution(EOEGE-E)is proposed based on generalization of the odd generalized exponential family(OEGE-E).The statistical pro...A new extended distribution called the Odd Exponential Generalized Exponential-Exponential distribution(EOEGE-E)is proposed based on generalization of the odd generalized exponential family(OEGE-E).The statistical properties of the proposed distribution are derived.The study evaluates the accuracy of six estimation methods under complete samples.Estimation techniques include maximumlikelihood,ordinary least squares,weighted least squares,maximumproduct of spacing,Cramer vonMises,and Anderson-Darling methods.Twomethods of estimation for the involved parameters are considered based on progressively type Ⅱ censored data(PTⅡC).These methods are maximum likelihood and maximum product of spacing.The proposed distribution’s effectiveness was evaluated using different data sets from various fields.The proposed distribution provides a better fit for these datasets than existing probability distributions.展开更多
BACKGROUND Progressive familial intrahepatic cholestasis type 1(PFIC-1)is a genetic cholestatic disease causing end-stage liver disease,which needs liver transplantation(LT).Simultaneous biliary diversion(BD)was recom...BACKGROUND Progressive familial intrahepatic cholestasis type 1(PFIC-1)is a genetic cholestatic disease causing end-stage liver disease,which needs liver transplantation(LT).Simultaneous biliary diversion(BD)was recommended to prevent allograft steatosis after transplantation,while increasing the risk of infection.Here,an attempt was made to perform BD using appendix to prevent bacterial translocation after LT.CASE SUMMARY An 11-month-old boy diagnosed with PFIC-1 received ABO compatible living donor LT due to refractory jaundice and pruritus.His mother donated her left lateral segment with a graft-to-recipient weight ratio of 2.9%.Internal BD was constructed during LT using the appendix by connecting its proximal end with the intrahepatic biliary duct and the distal end with colon.Biliary leakage was suspected on the 5th day after transplantation and exploratory laparotomy indicated biliary leakage at the cutting surface of liver.The liver function returned to normal on the 9th day post-operation and maintained normal during the 15-month follow-up.Cholangiography at 10 months after transplantation confirmed the direct secretion of bile into colon.Computerized tomography scan(4 months and 10 months)and liver biopsy(10 months)indicated no steatosis in the allograft.No complaint of recurrent diarrhea,infection or growth retardation was reported during follow-up.CONCLUSION Internal BD using appendix during LT is effective in preventing allograft steatosis and post-transplant infection in PFIC-1 recipients.展开更多
BACKGROUND Mutations that occur in the ABCB4 gene,which encodes multidrug-resistant protein 3,underlie the occurrence of progressive familial intrahepatic cholestasis type 3(PFIC3).Clinical signs of intrahepatic chole...BACKGROUND Mutations that occur in the ABCB4 gene,which encodes multidrug-resistant protein 3,underlie the occurrence of progressive familial intrahepatic cholestasis type 3(PFIC3).Clinical signs of intrahepatic cholestasis due to gene mutations typically first appear during infancy or childhood.Reports of PFIC3 occurring in adults are rare.CASE SUMMARY This is a case study of a 32-year-old infertile female Chinese patient with a 15-year history of recurrent abnormal liver function.Her primary clinical signs were elevated levels of alkaline phosphatase andγ-glutamyl transpeptidase.Other possible reasons for liver dysfunction were eliminated in this patient,resulting in a diagnosis of PFIC3.The diagnosis was confirmed using gene detection and histological analyses.Assessments using genetic sequencing analysis indicated the presence of two novel heterozygous mutations in the ABCB4 gene,namely,a 2950C>T;p.A984V mutation(exon 24)and a 667A>G;p.I223V mutation(exon 7).After receiving ursodeoxycholic acid(UDCA)treatment,the patient's liver function indices improved,and she successfully became pregnant by in vitro fertilization.However,the patient developed intrahepatic cholestasis of pregnancy in the first trimester.Fortunately,treatment with UDCA was safe and effective.CONCLUSION These novel ABCB4 heterozygous mutations have a variety of clinical phenotypes.Continued follow-up is essential for a comprehensive understanding of PFIC3.展开更多
The present investigation is concerned with the reaction of barium and iron nitrates mixtures using three different molar ratios, 1:1 (Ⅰ), 1:2 (Ⅱ) and 2:1 (Ⅲ) at different temperatures as pointed out from the DTA d...The present investigation is concerned with the reaction of barium and iron nitrates mixtures using three different molar ratios, 1:1 (Ⅰ), 1:2 (Ⅱ) and 2:1 (Ⅲ) at different temperatures as pointed out from the DTA data. The reaction products exhibit 12 compounds namely, Ba(NO3)2, αFe2O3, Fe3O4, BaFeO3, BaFeO2.9, hexagonal BaFeO3-x, tetragonal BaFeO3-x, BaFe2O4, αBaFe2O4, Ba2Fe6O11, Ba5Fe14O26 and BaFe12O19. The formation of these products depend on the molar ratio between the reactants and the reaction temperature. The reaction products were studied by DTA and TG techniques and characterized by X-ray diffraction patterns, magnetic susceptibility data and scanning electron microscopy, SEM.展开更多
Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by low serum γ-glutamyltransferase levels due to mutation inATP8B1.We present a 23-year-old male who experienced persistent...Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by low serum γ-glutamyltransferase levels due to mutation inATP8B1.We present a 23-year-old male who experienced persistent marked pruritus for eighteen years and recurrent jaundice for thirteen years,in addition to cholestasis that eventually became fatal.Genetic sequencing studies of the entire coding(exon) sequences of ATP8B1 and ABCB11 uncovered a novel heterozygous missense 3035G>T mutation(S1012I) and a synonymous 696T>C mutation in ATP8B1.The patient's progression was associated with not only impaired familial intrahepatic cholestasis 1(FIC1) function but also impaired bile salt export pump expression due to the impaired FIC1 function.Our findings show that patients with intermittent cholestasis can develop progressive liver disease even after several decades and require regular follow up.展开更多
In this article,we consider the statistical inferences of the unknown parameters of a generalized inverted exponential distribution based on the Type II progressively hybrid censored sample.By applying the expectation...In this article,we consider the statistical inferences of the unknown parameters of a generalized inverted exponential distribution based on the Type II progressively hybrid censored sample.By applying the expectation–maximization(EM)algorithm,the maximum likelihood estimators are developed for estimating the unknown parameters.The observed Fisher information matrix is obtained using the missing information principle,and it can be used for constructing asymptotic con-fidence intervals.By applying the bootstrapping technique,the confidence intervals for the parameters are also derived.Bayesian estimates of the unknown parameters are obtained using the Lindley’s approximation.Monte Carlo simulations are imple-mented and observations are given.Finally,a real data set representing the spread factor of micro-drops is analyzed to illustrative purposes.展开更多
Introduction Progressive familial intrahepatic cholestasis Type 3(PFIC3)is a rare,autosomal recessive,and hepatocellular-originating cholestatic liver disease caused by mutations in the ABCB4 gene,which encodes the mu...Introduction Progressive familial intrahepatic cholestasis Type 3(PFIC3)is a rare,autosomal recessive,and hepatocellular-originating cholestatic liver disease caused by mutations in the ABCB4 gene,which encodes the multidrug resistance protein 3(MDR3)[1].The function of the MDR3 protein is to translocate phosphatidylcholine from the inner lipid layer to the outer lipid layer of the bile canaliculus[1].Phosphatidylcholine combines with bile salts to form mixed micelles,which protect the biliary epithelium from the detergent effects of bile acids.In the absence of MDR3 protein,this translocation is impaired,leaving the biliary epithelium exposed to the detergent effects of bile acids[2].This disruption leads to cholestasis and progressive liver damage[2].展开更多
Progressive familial intrahepatic cholestasis type 2(PFIC2),also known as bile salt export pump(BSEP)deficiency disease,is a rare autosomal recessive inherited liver disease caused by mutations in the ABCB11 gene(loca...Progressive familial intrahepatic cholestasis type 2(PFIC2),also known as bile salt export pump(BSEP)deficiency disease,is a rare autosomal recessive inherited liver disease caused by mutations in the ABCB11 gene(located on chromosome 2q24-31),leading to impaired bile secretion.1 Over 200 distinct mutations in the ABCB11 gene have been identified,including missense,nonsense,insertion,deletion,and splice site mutations.1 Compared with other types of PFIC,patients with BSEP deficiency are at a higher risk of progressing to cirrhosis and liver failure.Current treatment options for PFIC2 remain limited and frustrating.Liver transplantation,the only effective intervention,remains the sole definitive treatment for PFIC2.Nevertheless,its application is severely limited by the prohibitive cost and the scarcity of suitable liver donors.Addressing the pathogenesis of PFIC2 poses a significant clinical challenge.However,a recent study has shown that sirolimus may partially restore the bile excretion ability of ABCB11 mutants in abcb11 knockout Zebrafish models.2 This case report describes a patient with BSEP deficiency disease who responded favorably to sirolimus treatment.展开更多
Lifetime performance index is a powerful and effective way to analyze whether a product achieves the specified standards.In this paper,we investigate the lifetime performance index for the inverted exponential Rayleig...Lifetime performance index is a powerful and effective way to analyze whether a product achieves the specified standards.In this paper,we investigate the lifetime performance index for the inverted exponential Rayleigh distribution using progressive type II censored sample data.The censored sample is able to greatly save the cost of the experiment and speed up the experiment.We derive the estimation value of lifetime performance index using the maximum likelihood method,and conduct the hypothesis test.Based on extensive numerical simulation,the power function is utilized to assess effectiveness of hypothesis testing.The simulation results show that lifetime performance index is good for determining whether the lifetime of the product reaches the criterion.Finally,a practical dataset is provided to give a demonstration for the procedures of lifetime performance evaluation.展开更多
文摘A new extended distribution called the Odd Exponential Generalized Exponential-Exponential distribution(EOEGE-E)is proposed based on generalization of the odd generalized exponential family(OEGE-E).The statistical properties of the proposed distribution are derived.The study evaluates the accuracy of six estimation methods under complete samples.Estimation techniques include maximumlikelihood,ordinary least squares,weighted least squares,maximumproduct of spacing,Cramer vonMises,and Anderson-Darling methods.Twomethods of estimation for the involved parameters are considered based on progressively type Ⅱ censored data(PTⅡC).These methods are maximum likelihood and maximum product of spacing.The proposed distribution’s effectiveness was evaluated using different data sets from various fields.The proposed distribution provides a better fit for these datasets than existing probability distributions.
基金Supported by the National Natural Science Foundation of China,No.82471804.
文摘BACKGROUND Progressive familial intrahepatic cholestasis type 1(PFIC-1)is a genetic cholestatic disease causing end-stage liver disease,which needs liver transplantation(LT).Simultaneous biliary diversion(BD)was recommended to prevent allograft steatosis after transplantation,while increasing the risk of infection.Here,an attempt was made to perform BD using appendix to prevent bacterial translocation after LT.CASE SUMMARY An 11-month-old boy diagnosed with PFIC-1 received ABO compatible living donor LT due to refractory jaundice and pruritus.His mother donated her left lateral segment with a graft-to-recipient weight ratio of 2.9%.Internal BD was constructed during LT using the appendix by connecting its proximal end with the intrahepatic biliary duct and the distal end with colon.Biliary leakage was suspected on the 5th day after transplantation and exploratory laparotomy indicated biliary leakage at the cutting surface of liver.The liver function returned to normal on the 9th day post-operation and maintained normal during the 15-month follow-up.Cholangiography at 10 months after transplantation confirmed the direct secretion of bile into colon.Computerized tomography scan(4 months and 10 months)and liver biopsy(10 months)indicated no steatosis in the allograft.No complaint of recurrent diarrhea,infection or growth retardation was reported during follow-up.CONCLUSION Internal BD using appendix during LT is effective in preventing allograft steatosis and post-transplant infection in PFIC-1 recipients.
基金Supported by Natural Science Foundation of Gansu Province,No.21JR7RA410.
文摘BACKGROUND Mutations that occur in the ABCB4 gene,which encodes multidrug-resistant protein 3,underlie the occurrence of progressive familial intrahepatic cholestasis type 3(PFIC3).Clinical signs of intrahepatic cholestasis due to gene mutations typically first appear during infancy or childhood.Reports of PFIC3 occurring in adults are rare.CASE SUMMARY This is a case study of a 32-year-old infertile female Chinese patient with a 15-year history of recurrent abnormal liver function.Her primary clinical signs were elevated levels of alkaline phosphatase andγ-glutamyl transpeptidase.Other possible reasons for liver dysfunction were eliminated in this patient,resulting in a diagnosis of PFIC3.The diagnosis was confirmed using gene detection and histological analyses.Assessments using genetic sequencing analysis indicated the presence of two novel heterozygous mutations in the ABCB4 gene,namely,a 2950C>T;p.A984V mutation(exon 24)and a 667A>G;p.I223V mutation(exon 7).After receiving ursodeoxycholic acid(UDCA)treatment,the patient's liver function indices improved,and she successfully became pregnant by in vitro fertilization.However,the patient developed intrahepatic cholestasis of pregnancy in the first trimester.Fortunately,treatment with UDCA was safe and effective.CONCLUSION These novel ABCB4 heterozygous mutations have a variety of clinical phenotypes.Continued follow-up is essential for a comprehensive understanding of PFIC3.
文摘The present investigation is concerned with the reaction of barium and iron nitrates mixtures using three different molar ratios, 1:1 (Ⅰ), 1:2 (Ⅱ) and 2:1 (Ⅲ) at different temperatures as pointed out from the DTA data. The reaction products exhibit 12 compounds namely, Ba(NO3)2, αFe2O3, Fe3O4, BaFeO3, BaFeO2.9, hexagonal BaFeO3-x, tetragonal BaFeO3-x, BaFe2O4, αBaFe2O4, Ba2Fe6O11, Ba5Fe14O26 and BaFe12O19. The formation of these products depend on the molar ratio between the reactants and the reaction temperature. The reaction products were studied by DTA and TG techniques and characterized by X-ray diffraction patterns, magnetic susceptibility data and scanning electron microscopy, SEM.
文摘Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by low serum γ-glutamyltransferase levels due to mutation inATP8B1.We present a 23-year-old male who experienced persistent marked pruritus for eighteen years and recurrent jaundice for thirteen years,in addition to cholestasis that eventually became fatal.Genetic sequencing studies of the entire coding(exon) sequences of ATP8B1 and ABCB11 uncovered a novel heterozygous missense 3035G>T mutation(S1012I) and a synonymous 696T>C mutation in ATP8B1.The patient's progression was associated with not only impaired familial intrahepatic cholestasis 1(FIC1) function but also impaired bile salt export pump expression due to the impaired FIC1 function.Our findings show that patients with intermittent cholestasis can develop progressive liver disease even after several decades and require regular follow up.
文摘In this article,we consider the statistical inferences of the unknown parameters of a generalized inverted exponential distribution based on the Type II progressively hybrid censored sample.By applying the expectation–maximization(EM)algorithm,the maximum likelihood estimators are developed for estimating the unknown parameters.The observed Fisher information matrix is obtained using the missing information principle,and it can be used for constructing asymptotic con-fidence intervals.By applying the bootstrapping technique,the confidence intervals for the parameters are also derived.Bayesian estimates of the unknown parameters are obtained using the Lindley’s approximation.Monte Carlo simulations are imple-mented and observations are given.Finally,a real data set representing the spread factor of micro-drops is analyzed to illustrative purposes.
文摘Introduction Progressive familial intrahepatic cholestasis Type 3(PFIC3)is a rare,autosomal recessive,and hepatocellular-originating cholestatic liver disease caused by mutations in the ABCB4 gene,which encodes the multidrug resistance protein 3(MDR3)[1].The function of the MDR3 protein is to translocate phosphatidylcholine from the inner lipid layer to the outer lipid layer of the bile canaliculus[1].Phosphatidylcholine combines with bile salts to form mixed micelles,which protect the biliary epithelium from the detergent effects of bile acids.In the absence of MDR3 protein,this translocation is impaired,leaving the biliary epithelium exposed to the detergent effects of bile acids[2].This disruption leads to cholestasis and progressive liver damage[2].
基金supported by the 5010 Cultivation Program of Clinical Research of Sun Yat-Sen University(No.2018024)Guangzhou Science and Technology Plan Project(2023A03J0807).
文摘Progressive familial intrahepatic cholestasis type 2(PFIC2),also known as bile salt export pump(BSEP)deficiency disease,is a rare autosomal recessive inherited liver disease caused by mutations in the ABCB11 gene(located on chromosome 2q24-31),leading to impaired bile secretion.1 Over 200 distinct mutations in the ABCB11 gene have been identified,including missense,nonsense,insertion,deletion,and splice site mutations.1 Compared with other types of PFIC,patients with BSEP deficiency are at a higher risk of progressing to cirrhosis and liver failure.Current treatment options for PFIC2 remain limited and frustrating.Liver transplantation,the only effective intervention,remains the sole definitive treatment for PFIC2.Nevertheless,its application is severely limited by the prohibitive cost and the scarcity of suitable liver donors.Addressing the pathogenesis of PFIC2 poses a significant clinical challenge.However,a recent study has shown that sirolimus may partially restore the bile excretion ability of ABCB11 mutants in abcb11 knockout Zebrafish models.2 This case report describes a patient with BSEP deficiency disease who responded favorably to sirolimus treatment.
文摘Lifetime performance index is a powerful and effective way to analyze whether a product achieves the specified standards.In this paper,we investigate the lifetime performance index for the inverted exponential Rayleigh distribution using progressive type II censored sample data.The censored sample is able to greatly save the cost of the experiment and speed up the experiment.We derive the estimation value of lifetime performance index using the maximum likelihood method,and conduct the hypothesis test.Based on extensive numerical simulation,the power function is utilized to assess effectiveness of hypothesis testing.The simulation results show that lifetime performance index is good for determining whether the lifetime of the product reaches the criterion.Finally,a practical dataset is provided to give a demonstration for the procedures of lifetime performance evaluation.
