Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with...Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with medications,autoimmune disorders,cancer,transplantation,and infections,the specific mechanisms of disease are not known.To date,only 4 cases of VBDS have been reported in human immunodeficiency virus(HIV) infected patients.We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases.Presentation includes hyperbilirubinemia,normal liver imaging,and negative viral and autoimmune hepatitis studies.In HIV-infected subjects,VBDS occurred at a range of CD4+ T-cell counts,in some cases following initiation or change in antiretroviral therapy.Lymphoma was associated with two cases;nevirapine,antibiotics,and viral co-infection were suggested as etiologies in the other cases.In HIV-positive patients with progressive cholestasis,early identification of VBDS and referral for transplantation may improve outcomes.展开更多
AIM: To define the magnetic resonance imaging(MRI) parameters differentiating urethral hypermobility(UH) and intrinsic sphincter deficiency(ISD) in women with stress urinary incontinence(SUI).METHODS: The static and d...AIM: To define the magnetic resonance imaging(MRI) parameters differentiating urethral hypermobility(UH) and intrinsic sphincter deficiency(ISD) in women with stress urinary incontinence(SUI).METHODS: The static and dynamic MR images of 21 patients with SUI were correlated to urodynamic(UD) findings and compared to those of 10 continent controls. For the assessment of the urethra and integrity of the urethral support structures, we applied the highresolution endocavitary MRI, such as intraurethral MRI, endovaginal or endorectal MRI. For the functional imaging of the urethral support, we performed dynamic MRI with the pelvic phased array coil. We assessed the following MRI parameters in both the patient and thevolunteer groups:(1) urethral angle;(2) bladder neck descent;(3) status of the periurethral ligaments,(4) vaginal shape;(5) urethral sphincter integrity, length and muscle thickness at mid urethra;(6) bladder neck funneling;(7) status of the puborectalis muscle;(8) pubo-vaginal distance. UDs parameters were assessed in the patient study group as follows:(1) urethral mobility angle on Q-tip test;(2) Valsalva leak point pressure(VLPP) measured at 250 cc bladder volume; and(3) maximum urethral closure pressure(MUCP). The UH type of SUI was defined with the Q-tip test angle over 30 degrees, and VLPP pressure over 60 cm H2 O. The ISD incontinence was defined with MUCP pressure below 20 cm H2 O, and VLPP pressure less or equal to 60 cm H2 O. We considered the associations between the MRI and clinical data and UDs using a variety of statistical tools to include linear regression, multivariate logistic regression and receiver operating characteristic(ROC) analysis. All statistical analyses were performed using STATA version 9.0(Stata Corp LP, College Station, TX).RESULTS: In the incontinent group, 52% have history of vaginal delivery trauma as compared to none in control group(P < 0.001). There was no difference between the continent volunteers and incontinent patients in body habitus as assessed by the body mass index. Pubovaginal distance and periurethral ligament disruption are significantly associated with incontinence; periurethral ligament symmetricity reduces the odds of incontinence by 87%. Bladder neck funneling and length of the suprapubic urethral sphincter are significantly associated with the type of incontinence on UDs; funneling reduced the odds of pure UH by almost 95%; increasing suprapubic urethral sphincter length at rest is highly associated with UH. Both MRI variables result in a predictive model for UDs diagnosis(area under the ROC = 0.944). CONCLUSION: MRI may play an important role in assessing the contribution of hypermobility and sphincteric dysfunction to the SUI in women when considering treatment options.展开更多
Almost all clinically important RBC antigens are defined at the molecular level.The expression of protein-and sugar-based antigens on the RBC surface can be predicted by determining the blood group gene variants (alle...Almost all clinically important RBC antigens are defined at the molecular level.The expression of protein-and sugar-based antigens on the RBC surface can be predicted by determining the blood group gene variants (alleles).Most of the time,a single nucleotide polymorphism (SNP) distinguishes the allele,which determines an antigen and hence allows predicting the antigen.PCR with sequence specific priming (PCR-SSP) followed by gel electrophoresis was the original technique widely applied for blood group genotyping.Real-time PCR obviated the need for gels.The proof of principle for high-throughput platforms was a hybridization based DNA chip,in quick succession followed by Beadchip,SNPstream,SNaPshot and Luminex technology.Each of these platforms differs in technical details,which can put one of them at an advantage over the others depending on the specific tasks and needs that it is expected to cover.展开更多
基金Supported by The Intramural Research Programs of the National Institutes of Health Clinical Center and the National Institute of Allergy and Infectious Diseases
文摘Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with medications,autoimmune disorders,cancer,transplantation,and infections,the specific mechanisms of disease are not known.To date,only 4 cases of VBDS have been reported in human immunodeficiency virus(HIV) infected patients.We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases.Presentation includes hyperbilirubinemia,normal liver imaging,and negative viral and autoimmune hepatitis studies.In HIV-infected subjects,VBDS occurred at a range of CD4+ T-cell counts,in some cases following initiation or change in antiretroviral therapy.Lymphoma was associated with two cases;nevirapine,antibiotics,and viral co-infection were suggested as etiologies in the other cases.In HIV-positive patients with progressive cholestasis,early identification of VBDS and referral for transplantation may improve outcomes.
基金Supported by The Radiological Society of North America and the Society of Computed Body Tomography and Magnetic Resonance
文摘AIM: To define the magnetic resonance imaging(MRI) parameters differentiating urethral hypermobility(UH) and intrinsic sphincter deficiency(ISD) in women with stress urinary incontinence(SUI).METHODS: The static and dynamic MR images of 21 patients with SUI were correlated to urodynamic(UD) findings and compared to those of 10 continent controls. For the assessment of the urethra and integrity of the urethral support structures, we applied the highresolution endocavitary MRI, such as intraurethral MRI, endovaginal or endorectal MRI. For the functional imaging of the urethral support, we performed dynamic MRI with the pelvic phased array coil. We assessed the following MRI parameters in both the patient and thevolunteer groups:(1) urethral angle;(2) bladder neck descent;(3) status of the periurethral ligaments,(4) vaginal shape;(5) urethral sphincter integrity, length and muscle thickness at mid urethra;(6) bladder neck funneling;(7) status of the puborectalis muscle;(8) pubo-vaginal distance. UDs parameters were assessed in the patient study group as follows:(1) urethral mobility angle on Q-tip test;(2) Valsalva leak point pressure(VLPP) measured at 250 cc bladder volume; and(3) maximum urethral closure pressure(MUCP). The UH type of SUI was defined with the Q-tip test angle over 30 degrees, and VLPP pressure over 60 cm H2 O. The ISD incontinence was defined with MUCP pressure below 20 cm H2 O, and VLPP pressure less or equal to 60 cm H2 O. We considered the associations between the MRI and clinical data and UDs using a variety of statistical tools to include linear regression, multivariate logistic regression and receiver operating characteristic(ROC) analysis. All statistical analyses were performed using STATA version 9.0(Stata Corp LP, College Station, TX).RESULTS: In the incontinent group, 52% have history of vaginal delivery trauma as compared to none in control group(P < 0.001). There was no difference between the continent volunteers and incontinent patients in body habitus as assessed by the body mass index. Pubovaginal distance and periurethral ligament disruption are significantly associated with incontinence; periurethral ligament symmetricity reduces the odds of incontinence by 87%. Bladder neck funneling and length of the suprapubic urethral sphincter are significantly associated with the type of incontinence on UDs; funneling reduced the odds of pure UH by almost 95%; increasing suprapubic urethral sphincter length at rest is highly associated with UH. Both MRI variables result in a predictive model for UDs diagnosis(area under the ROC = 0.944). CONCLUSION: MRI may play an important role in assessing the contribution of hypermobility and sphincteric dysfunction to the SUI in women when considering treatment options.
文摘Almost all clinically important RBC antigens are defined at the molecular level.The expression of protein-and sugar-based antigens on the RBC surface can be predicted by determining the blood group gene variants (alleles).Most of the time,a single nucleotide polymorphism (SNP) distinguishes the allele,which determines an antigen and hence allows predicting the antigen.PCR with sequence specific priming (PCR-SSP) followed by gel electrophoresis was the original technique widely applied for blood group genotyping.Real-time PCR obviated the need for gels.The proof of principle for high-throughput platforms was a hybridization based DNA chip,in quick succession followed by Beadchip,SNPstream,SNaPshot and Luminex technology.Each of these platforms differs in technical details,which can put one of them at an advantage over the others depending on the specific tasks and needs that it is expected to cover.
