Under the heading of bleeding disorders aregrouped a number of diseases which have hemorrhagicproblems. Bleeding disorders are very common, involv-ing about one third of patients in the hematological con-sultation. Th...Under the heading of bleeding disorders aregrouped a number of diseases which have hemorrhagicproblems. Bleeding disorders are very common, involv-ing about one third of patients in the hematological con-sultation. The hemorrhagic mechanism are complex andmay be somewhat simplistically divided into three parts:platelet deficiencies, deficiencies in the plasma coagula-展开更多
HIV-associated neurocognitive disorders (HAND) are chronic complications of HIV infection in the central nervous system. Clinical presentations include asymptomatic neurocognitive impairment (ANI), mild neurocognitive...HIV-associated neurocognitive disorders (HAND) are chronic complications of HIV infection in the central nervous system. Clinical presentations include asymptomatic neurocognitive impairment (ANI), mild neurocognitive impairment (MND), and HIV-associated dementia (HAD). In the era of combination antiretroviral therapy (cART), the prevalence of HAD has significantly decreased, but the rates of ANI and MND have increased, impairing patients’ daily functioning, medical adherence, employment, driving abilities, risk of HIV transmission, overall quality of life, and posing challenges to society, economy, families, and public health. This article reviews the latest research findings regarding the pathogenesis, clinical diagnosis and treatment, neuroimaging, and neuropsychological assessment of HAND, aiming to provide insights into the prevention and management of HAND.展开更多
AIM To investigate factors predicting treatment completion and treatment outcome of the Reasoning and Rehabilitation Mental Health Programme(R&R2MHP) cognitive skills programme for mentally disordered offenders. M...AIM To investigate factors predicting treatment completion and treatment outcome of the Reasoning and Rehabilitation Mental Health Programme(R&R2MHP) cognitive skills programme for mentally disordered offenders. METHODS Secondary analysis of data previously obtained from 97 male patients who were sectioned and detained under the United Kingdom Mental Health Act in low, medium and high security hospitals and who had completed R&R2MHP. Predictors of treatment completion included background variables and five outcome measures: Four self-reported measures of violent attitudes, social problem-solving skills, reactive anger and locus of control and an objective measure of behaviour on theward that was completed by staff. Completion of the 16 session programme, which was delivered on a weekly basis, was classified as ≥ 12 sessions.RESULTS It was found that the R&R2MHP is appropriate for delivery to participants of different ages, ethnic background, and at different levels of security without the completion rate or treatment effectiveness being compromised. Participants taking oral typical psychotropic medication were over seven times more likely to complete the programme than other participants. Behavioural disturbance on the ward prior to commencing the programme predicted non-completion(medium effect size). As far as treatment completion was concerned, none of the background factors predicted treatment effectiveness(age, ethnic background, level of security, number of previous convictions and number of previous hospital admissions). The best predictor of treatment effectiveness was attitude towards violence suggesting that this should be the primary outcome measure in future research evaluating outcomes of the R&R2MHP cognitive skills program. CONCLUSION The findings suggest that a stable mental state is a key factor that predicts treatment completion.展开更多
Fabry Disease (FD) is a rare lysosomal storage disorder characterized by α-galactosidase A (α-Gal A) enzyme deficiency, resulting in glycosphingolipid accumulation. Its clinical spectrum ranges from severe classical...Fabry Disease (FD) is a rare lysosomal storage disorder characterized by α-galactosidase A (α-Gal A) enzyme deficiency, resulting in glycosphingolipid accumulation. Its clinical spectrum ranges from severe classical to milder nonclassical or late-onset phenotypes. Renal involvement, termed Fabry Nephropathy (FN), can vary from mild proteinuria to kidney failure. FN diagnosis, especially in nonclassical cases with a genetic Variant of Unknown Significance (VUS) in the GLA gene, poses challenges. Measurement of plasma lyso-Gb3 levels is gaining importance in FN diagnosis, while renal biopsy with electron microscopy remains the gold standard in equivocal cases. Treatment options include Enzyme Replacement Therapy (ERT) and chaperone therapy, demanding careful candidate selection due to high treatment costs. Research has predominantly focused on classical FD, revealing modest treatment benefits. However, evidence for treating patients, especially females, with milder nonclassical or late-onset phenotypes is scarce, emphasizing the necessity for placebo-controlled clinical trials in these subgroups. Meanwhile, participation in global FD registries can improve our understanding of disease management. Case Presentation: A woman in her late sixties presented with moderate chronic kidney disease, mild proteinuria, and microscopic hematuria. Her family history included a prevalence of renal, cardiac and cerebrovascular diseases. Kidney biopsy revealed characteristic myelin figures and zebra bodies in podocytes, strongly suggestive of FN. Genetic analysis identified a VUS in the GLA gene (c.655A > C, p.Ile219Leu), introducing diagnostic uncertainty. Further investigations revealed severe cardiac involvement. Considering the recurring difficulty presented by the finding of a VUS in the GLA gene during FN assessments, along with the uncertainty regarding the need for treatment in nonclassical or late-onset FD phenotypes, especially in women, this case becomes a central focus for a thorough review of the literature. This review aims to propose a practical algorithm that integrates clinical, biochemical, and genetic markers for FN screening and diagnosis. Additionally, it explores treatment benefits in nonclassical or late-onset FD phenotypes, with a focus on female patients.展开更多
Objective:According to the World Health Organization,the number of infected people with human immunodeficiency virus(HIV)in 2019 was about 38 million.Using combinational antiretroviral therapy in recent years has incr...Objective:According to the World Health Organization,the number of infected people with human immunodeficiency virus(HIV)in 2019 was about 38 million.Using combinational antiretroviral therapy in recent years has increased life expectancy in these people and HIV has been changed from a deadly disease to a chronic one.HIV-associated neurological disorders(HAND)include asymptomatic neurological disorders,Motor neurological disorders,and HIV-associated dementia.Methods:We searched 3 databases(PubMed,Science Direct and Scopus databases)from January 2015 to March 2021 for the keywords;HIV associated with a neurocognitive disorder,HAND and combinational antiretroviral therapy to provide careful consideration of various aspects of these disorders,including;risk factors,their diagnostic methods,their epidemiology,and finally the treatment of HAND.Results:In the initial search,we came across 1351 articles.Three authors did the title-abstract screening,and 270 articles were entered into full-text screening from the previous step.Four authors did the full-text screening,and the screening outcome was 21 articles.We categorized the results of our study into sub-categories,including:risk factors,pathogenicity,clinical manifestations,diagnosis,and epidemiology of HAND.Conclusions:Although the HAND subheading has made great strides,many questions about the disease remain unanswered,and many of the diagnosing established methods are not 100%accurate and as a result of that,this epidemy can’t be fully endured yet.We hope to find and apply further advances and more definitive solutions in the coming decades.展开更多
Obsessive-compulsive disorder is a disorder which is resistant to the existing treatments and to compensate this defect, it is necessary to design and search for the new treatments. This study aims to develop and eval...Obsessive-compulsive disorder is a disorder which is resistant to the existing treatments and to compensate this defect, it is necessary to design and search for the new treatments. This study aims to develop and evaluate the effectiveness of the behavioral-cognitive-metacognitive intervention on the symptoms of obsessive-compulsive disorder. In order to achieve objectives of the study, after developing the behavioral-cognitive-metacognitive intervention, it was evaluated in a single-subject procedure. OCI-R scores at baseline were obtained by following the treatment on 3 individuals. The study was a single-subject research and the data were analyzed by drawing the graph. Graph drawing results showed that the behavioral-cognitive-metacognitive intervention reduced the symptoms of obsessive-compulsive disorder (OCD), and reduction of OCD symptoms was also clinically significant. As the intervention of the present study is multi-dimensional and takes into account all the three behavioral, cognitive and metacognitive aspects and considers them interdependent and not apart or contradictory, it is considered as a new intervention.展开更多
The functional gastrointestinal disorders (FGIDs) are a group of diseases mainly manifested as gastrointestinal functional disorders,including 45 kinds of different diseases,such as functional dyspepsia(FD), irritable...The functional gastrointestinal disorders (FGIDs) are a group of diseases mainly manifested as gastrointestinal functional disorders,including 45 kinds of different diseases,such as functional dyspepsia(FD), irritable bowel syndrome (IBS),functional constipation展开更多
文摘Under the heading of bleeding disorders aregrouped a number of diseases which have hemorrhagicproblems. Bleeding disorders are very common, involv-ing about one third of patients in the hematological con-sultation. The hemorrhagic mechanism are complex andmay be somewhat simplistically divided into three parts:platelet deficiencies, deficiencies in the plasma coagula-
文摘HIV-associated neurocognitive disorders (HAND) are chronic complications of HIV infection in the central nervous system. Clinical presentations include asymptomatic neurocognitive impairment (ANI), mild neurocognitive impairment (MND), and HIV-associated dementia (HAD). In the era of combination antiretroviral therapy (cART), the prevalence of HAD has significantly decreased, but the rates of ANI and MND have increased, impairing patients’ daily functioning, medical adherence, employment, driving abilities, risk of HIV transmission, overall quality of life, and posing challenges to society, economy, families, and public health. This article reviews the latest research findings regarding the pathogenesis, clinical diagnosis and treatment, neuroimaging, and neuropsychological assessment of HAND, aiming to provide insights into the prevention and management of HAND.
基金supported by the National Institute for Health Research (NIHR) Imperial Biomedical Research Centre
文摘AIM To investigate factors predicting treatment completion and treatment outcome of the Reasoning and Rehabilitation Mental Health Programme(R&R2MHP) cognitive skills programme for mentally disordered offenders. METHODS Secondary analysis of data previously obtained from 97 male patients who were sectioned and detained under the United Kingdom Mental Health Act in low, medium and high security hospitals and who had completed R&R2MHP. Predictors of treatment completion included background variables and five outcome measures: Four self-reported measures of violent attitudes, social problem-solving skills, reactive anger and locus of control and an objective measure of behaviour on theward that was completed by staff. Completion of the 16 session programme, which was delivered on a weekly basis, was classified as ≥ 12 sessions.RESULTS It was found that the R&R2MHP is appropriate for delivery to participants of different ages, ethnic background, and at different levels of security without the completion rate or treatment effectiveness being compromised. Participants taking oral typical psychotropic medication were over seven times more likely to complete the programme than other participants. Behavioural disturbance on the ward prior to commencing the programme predicted non-completion(medium effect size). As far as treatment completion was concerned, none of the background factors predicted treatment effectiveness(age, ethnic background, level of security, number of previous convictions and number of previous hospital admissions). The best predictor of treatment effectiveness was attitude towards violence suggesting that this should be the primary outcome measure in future research evaluating outcomes of the R&R2MHP cognitive skills program. CONCLUSION The findings suggest that a stable mental state is a key factor that predicts treatment completion.
文摘Fabry Disease (FD) is a rare lysosomal storage disorder characterized by α-galactosidase A (α-Gal A) enzyme deficiency, resulting in glycosphingolipid accumulation. Its clinical spectrum ranges from severe classical to milder nonclassical or late-onset phenotypes. Renal involvement, termed Fabry Nephropathy (FN), can vary from mild proteinuria to kidney failure. FN diagnosis, especially in nonclassical cases with a genetic Variant of Unknown Significance (VUS) in the GLA gene, poses challenges. Measurement of plasma lyso-Gb3 levels is gaining importance in FN diagnosis, while renal biopsy with electron microscopy remains the gold standard in equivocal cases. Treatment options include Enzyme Replacement Therapy (ERT) and chaperone therapy, demanding careful candidate selection due to high treatment costs. Research has predominantly focused on classical FD, revealing modest treatment benefits. However, evidence for treating patients, especially females, with milder nonclassical or late-onset phenotypes is scarce, emphasizing the necessity for placebo-controlled clinical trials in these subgroups. Meanwhile, participation in global FD registries can improve our understanding of disease management. Case Presentation: A woman in her late sixties presented with moderate chronic kidney disease, mild proteinuria, and microscopic hematuria. Her family history included a prevalence of renal, cardiac and cerebrovascular diseases. Kidney biopsy revealed characteristic myelin figures and zebra bodies in podocytes, strongly suggestive of FN. Genetic analysis identified a VUS in the GLA gene (c.655A > C, p.Ile219Leu), introducing diagnostic uncertainty. Further investigations revealed severe cardiac involvement. Considering the recurring difficulty presented by the finding of a VUS in the GLA gene during FN assessments, along with the uncertainty regarding the need for treatment in nonclassical or late-onset FD phenotypes, especially in women, this case becomes a central focus for a thorough review of the literature. This review aims to propose a practical algorithm that integrates clinical, biochemical, and genetic markers for FN screening and diagnosis. Additionally, it explores treatment benefits in nonclassical or late-onset FD phenotypes, with a focus on female patients.
文摘Objective:According to the World Health Organization,the number of infected people with human immunodeficiency virus(HIV)in 2019 was about 38 million.Using combinational antiretroviral therapy in recent years has increased life expectancy in these people and HIV has been changed from a deadly disease to a chronic one.HIV-associated neurological disorders(HAND)include asymptomatic neurological disorders,Motor neurological disorders,and HIV-associated dementia.Methods:We searched 3 databases(PubMed,Science Direct and Scopus databases)from January 2015 to March 2021 for the keywords;HIV associated with a neurocognitive disorder,HAND and combinational antiretroviral therapy to provide careful consideration of various aspects of these disorders,including;risk factors,their diagnostic methods,their epidemiology,and finally the treatment of HAND.Results:In the initial search,we came across 1351 articles.Three authors did the title-abstract screening,and 270 articles were entered into full-text screening from the previous step.Four authors did the full-text screening,and the screening outcome was 21 articles.We categorized the results of our study into sub-categories,including:risk factors,pathogenicity,clinical manifestations,diagnosis,and epidemiology of HAND.Conclusions:Although the HAND subheading has made great strides,many questions about the disease remain unanswered,and many of the diagnosing established methods are not 100%accurate and as a result of that,this epidemy can’t be fully endured yet.We hope to find and apply further advances and more definitive solutions in the coming decades.
文摘Obsessive-compulsive disorder is a disorder which is resistant to the existing treatments and to compensate this defect, it is necessary to design and search for the new treatments. This study aims to develop and evaluate the effectiveness of the behavioral-cognitive-metacognitive intervention on the symptoms of obsessive-compulsive disorder. In order to achieve objectives of the study, after developing the behavioral-cognitive-metacognitive intervention, it was evaluated in a single-subject procedure. OCI-R scores at baseline were obtained by following the treatment on 3 individuals. The study was a single-subject research and the data were analyzed by drawing the graph. Graph drawing results showed that the behavioral-cognitive-metacognitive intervention reduced the symptoms of obsessive-compulsive disorder (OCD), and reduction of OCD symptoms was also clinically significant. As the intervention of the present study is multi-dimensional and takes into account all the three behavioral, cognitive and metacognitive aspects and considers them interdependent and not apart or contradictory, it is considered as a new intervention.
基金Supported by the Key Research ltem of Beijing Science and Technology Committee(No.Z0005190043711)the CapitalDevelopment Fund(No.SF-2005-9)
文摘The functional gastrointestinal disorders (FGIDs) are a group of diseases mainly manifested as gastrointestinal functional disorders,including 45 kinds of different diseases,such as functional dyspepsia(FD), irritable bowel syndrome (IBS),functional constipation
