Background:To investigate the topographic measurements and densitometry of corneas in Wilson's disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-se...Background:To investigate the topographic measurements and densitometry of corneas in Wilson's disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-sectional study included 20 WD patients without a KF-r(group I),18 WD patients with a KF-r(groupⅠ),and 20 age-matched controls(group Ⅱ).The Pentacam high-resolution imaging system is used to determine corneal topographic measurements and densitometry.Results:Mean age for groups Ⅰ,Ⅱ and Ⅲ was 25.40±6.43 years(14-36 years),25.38±6.96 years(16-39 years),and 23.60±6.56 years(17-35 years),respectively(P=0.623).There was no significant difference between the groups in terms of the anterior corneal densitometry values(P>0.05),while the 6-10 mm and 10-12 mm midstroma and the 2-6 mm,6-10 mm,and 10-12 mm posterior corneal densitometry values in group Ⅱ were significantly higher than those in groups Ⅰ and Ⅱ(for all values,P<0.05).However,the 10-12 mm posterior corneal densitometry values in group Ⅰ were also significantly higher than those in group Ⅲ(P=0.038).The central corneal thickness(CCT),thinnest corneal thickness(tCT),and corneal volume(CV)values in groups Ⅰ and Ⅱ were significantly lower than those in group Ⅱ(for CCT values,P=0.011 and P=0.009;for tCT values,P=0.010 and P=0.005;for CV values,P=0.043 and P=0.029).Conclusion:In WD patients with a KF-r,corneal transparency decreased in the peripheral posterior and midstromal corneal layers;for these patients,corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.展开更多
Background:To investigate the topographic measurements and densitometry of corneas in Wilson’s disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-secti...Background:To investigate the topographic measurements and densitometry of corneas in Wilson’s disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-sectional study included 20 WD patients without a KF-r(group I),18 WD patients with a KF-r(group II),and 20 age-matched controls(group III).The Pentacam high resolution imaging system is used to determine corneal topographic measurements and densitometry.Results:Mean age for groups I,II and III was 25.40±6.43 years(14-36 years),25.38±6.96 years(16-39 years),23.60±6.56 years(17-35 years),respectively(P=0.623).There was no significant difference between the groups in terms of the anterior corneal densitometry values(P>0.05),while the 6-10mm and 10-12 mm mid stroma and the 2-6 mm,6-10 mm,and 10-12 mm posterior corneal densitometry values in group II were significantly higher than those in groups I and III(for all values,P<0.05).However,the 10-12mm posterior corneal densitometry values in group I were also significantly higher than those in group III(P=0.038).The central corneal thickness(CCT),thinnest corneal thickness(tCT),and corneal volume(CV)values in groups I and II were significantly lower than those in group III(for CCT values,P=0.011 and P=0.009;for tCT values,P=0.010 and P=0.005;for CV values,P=0.043 and P=0.029).Conclusion:In WD patients with a KF-r,corneal transparency decreased in the peripheral posterior and mid stromal corneal layers;for these patients,corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.展开更多
BACKGROUND Wilson disease(WD)is the most common genetic metabolic liver disease.Some studies have shown that comorbidities may have important effects on WD.Data on hepatitis B virus(HBV)infection in patients with WD a...BACKGROUND Wilson disease(WD)is the most common genetic metabolic liver disease.Some studies have shown that comorbidities may have important effects on WD.Data on hepatitis B virus(HBV)infection in patients with WD are limited.AIM To investigate the prevalence and clinical impact of HBV infection in patients with WD.METHODS The clinical data of patients with WD were analyzed retrospectively,and the data of patients with concurrent WD and HBV infection were compared with those of patients with isolated WD.RESULTS Among a total of 915 WD patients recruited,the total prevalence of current and previous HBV infection was 2.1%[95%confidence interval(CI):1.2%-3.0%]and 9.2%(95%CI:7.3%-11.1%),respectively.The main finding of this study was the identification of 19 patients with concurrent WD and chronic hepatitis B(CHB)infection.The diagnosis of WD was missed in all but two patients with CHB infection.The mean delay in the diagnosis of WD in patients with concurrent WD and CHB infection was 32.5 mo,which was significantly longer than that in patients with isolated WD(10.5 mo).The rates of severe liver disease and mortality in patients with concurrent WD and CHB infection were significantly higher than those in patients with isolated WD(63.1%vs 19.3%,P=0.000 and 36.8%vs 4.1%,P<0.001,respectively).Binary logistic regression analysis revealed a significantly higher risk of severe liver disease at the diagnosis of WD in patients with current HBV infection[odds ratio(OR)=7.748;95%CI:2.890-20.774;P=0.000)]or previous HBV infection(OR=5.525;95%CI:3.159-8.739;P=0.000)than in patients with isolated WD.CONCLUSION The total prevalence of current HBV infection in patients with WD was 2.1%.The diagnosis of WD in CHB patients is usually missed.HBV infection is an independent risk factor for severe liver disease in WD patients.The diagnosis of WD should be ruled out in some patients with CHB infection.展开更多
文摘Background:To investigate the topographic measurements and densitometry of corneas in Wilson's disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-sectional study included 20 WD patients without a KF-r(group I),18 WD patients with a KF-r(groupⅠ),and 20 age-matched controls(group Ⅱ).The Pentacam high-resolution imaging system is used to determine corneal topographic measurements and densitometry.Results:Mean age for groups Ⅰ,Ⅱ and Ⅲ was 25.40±6.43 years(14-36 years),25.38±6.96 years(16-39 years),and 23.60±6.56 years(17-35 years),respectively(P=0.623).There was no significant difference between the groups in terms of the anterior corneal densitometry values(P>0.05),while the 6-10 mm and 10-12 mm midstroma and the 2-6 mm,6-10 mm,and 10-12 mm posterior corneal densitometry values in group Ⅱ were significantly higher than those in groups Ⅰ and Ⅱ(for all values,P<0.05).However,the 10-12 mm posterior corneal densitometry values in group Ⅰ were also significantly higher than those in group Ⅲ(P=0.038).The central corneal thickness(CCT),thinnest corneal thickness(tCT),and corneal volume(CV)values in groups Ⅰ and Ⅱ were significantly lower than those in group Ⅱ(for CCT values,P=0.011 and P=0.009;for tCT values,P=0.010 and P=0.005;for CV values,P=0.043 and P=0.029).Conclusion:In WD patients with a KF-r,corneal transparency decreased in the peripheral posterior and midstromal corneal layers;for these patients,corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.
文摘Background:To investigate the topographic measurements and densitometry of corneas in Wilson’s disease(WD)patients with or without a Kayser-Fleischer ring(KF-r)compared to healthy individuals.Methods:This cross-sectional study included 20 WD patients without a KF-r(group I),18 WD patients with a KF-r(group II),and 20 age-matched controls(group III).The Pentacam high resolution imaging system is used to determine corneal topographic measurements and densitometry.Results:Mean age for groups I,II and III was 25.40±6.43 years(14-36 years),25.38±6.96 years(16-39 years),23.60±6.56 years(17-35 years),respectively(P=0.623).There was no significant difference between the groups in terms of the anterior corneal densitometry values(P>0.05),while the 6-10mm and 10-12 mm mid stroma and the 2-6 mm,6-10 mm,and 10-12 mm posterior corneal densitometry values in group II were significantly higher than those in groups I and III(for all values,P<0.05).However,the 10-12mm posterior corneal densitometry values in group I were also significantly higher than those in group III(P=0.038).The central corneal thickness(CCT),thinnest corneal thickness(tCT),and corneal volume(CV)values in groups I and II were significantly lower than those in group III(for CCT values,P=0.011 and P=0.009;for tCT values,P=0.010 and P=0.005;for CV values,P=0.043 and P=0.029).Conclusion:In WD patients with a KF-r,corneal transparency decreased in the peripheral posterior and mid stromal corneal layers;for these patients,corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.
文摘BACKGROUND Wilson disease(WD)is the most common genetic metabolic liver disease.Some studies have shown that comorbidities may have important effects on WD.Data on hepatitis B virus(HBV)infection in patients with WD are limited.AIM To investigate the prevalence and clinical impact of HBV infection in patients with WD.METHODS The clinical data of patients with WD were analyzed retrospectively,and the data of patients with concurrent WD and HBV infection were compared with those of patients with isolated WD.RESULTS Among a total of 915 WD patients recruited,the total prevalence of current and previous HBV infection was 2.1%[95%confidence interval(CI):1.2%-3.0%]and 9.2%(95%CI:7.3%-11.1%),respectively.The main finding of this study was the identification of 19 patients with concurrent WD and chronic hepatitis B(CHB)infection.The diagnosis of WD was missed in all but two patients with CHB infection.The mean delay in the diagnosis of WD in patients with concurrent WD and CHB infection was 32.5 mo,which was significantly longer than that in patients with isolated WD(10.5 mo).The rates of severe liver disease and mortality in patients with concurrent WD and CHB infection were significantly higher than those in patients with isolated WD(63.1%vs 19.3%,P=0.000 and 36.8%vs 4.1%,P<0.001,respectively).Binary logistic regression analysis revealed a significantly higher risk of severe liver disease at the diagnosis of WD in patients with current HBV infection[odds ratio(OR)=7.748;95%CI:2.890-20.774;P=0.000)]or previous HBV infection(OR=5.525;95%CI:3.159-8.739;P=0.000)than in patients with isolated WD.CONCLUSION The total prevalence of current HBV infection in patients with WD was 2.1%.The diagnosis of WD in CHB patients is usually missed.HBV infection is an independent risk factor for severe liver disease in WD patients.The diagnosis of WD should be ruled out in some patients with CHB infection.
