Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

“Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” is the 3rd edition of clinical practice guideline (CPG) for stroke rehabilitation in Korea, which updates the 2nd edition published in 2014. Forty-two specialists in stroke rehabilitation from 21 universities and 4 rehabilitation hospitals and 4 consultants participated in this update. The purpose of this CPG is to provide optimum practical guidelines for stroke rehabilitation teams to make a decision when they manage stroke patients and ultimately, to help stroke patients obtain maximal functional recovery and return to the society. The recent two CPGs from Canada (2015) and USA (2016) and articles that were published following the 2nd edition were used to develop this 3rd edition of CPG for stroke rehabilitation in Korea. The chosen articles' level of evidence and grade of recommendation were decided by the criteria of Scotland (2010) and the formal consensus was derived by the nominal group technique. The levels of evidence range from 1++ to 4 and the grades of recommendation range from A to D. Good Practice Point was recommended as best practice based on the clinical experience of the guideline developmental group. The draft of the developed CPG was reviewed by the experts group in the public hearings and then revised. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” consists of ‘Chapter 1; Introduction of Stroke Rehabilitation’, ‘Chapter 2; Rehabilitation for Stroke Syndrome, ‘Chapter 3; Rehabilitation for Returning to the Society’, and ‘Chapter 4; Advanced Technique for Stroke Rehabilitation’. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” will provide direction and standardization for acute, subacute and chronic stroke rehabilitation in Korea.
Canada ; Consensus ; Consultants ; Humans ; Korea* ; Practice Guidelines as Topic ; Rehabilitation* ; Scotland ; Specialization ; Stroke*

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.
Adult* ; Biopsy ; Cough ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Diabetes Mellitus* ; Diabetes Mellitus, Type 2 ; Dyspnea ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell* ; Humans ; Lung ; Lung Diseases ; Magnetic Resonance Imaging ; Male* ; Polydipsia ; Smoke ; Smoking ; Smoking Cessation ; Thoracic Surgery, Video-Assisted ; Water Deprivation

"Clinical Practice Guideline for Stroke Rehabilitation in Korea 2012" is a 2nd edition of clinical practice guideline (CPG) for stroke rehabilitation in Korea, which updates the 1st edition published in 2009. After 1st stroke rehabilitation CPG, many studies concerning stroke rehabilitation have been published and the necessity for update has been raised. The Korea Centers for Disease Control and Prevention supported the project "Development of Clinical Practice Guideline for Stroke Rehabilitation" in 2012. Thirty-two specialists in stroke rehabilitation from 18 universities and 3 rehabilitation hospitals and 10 consultants participated in this project. The scope of this CPG included both ischemic and hemorrhagic stroke from the acute to chronic stages. The purpose of this CPG is to provide guidelines for doctors and therapists to make a decision when they manage stroke patients and ultimately, to help stroke patients obtain maximal functional recovery and return to the society. "Clinical Practice Guideline for Stroke Rehabilitation in Korea 2012" consists of 'Chapter 1; Introduction of Stroke Rehabilitation', 'Chapter 2; Rehabilitation for Stroke Syndrome, 'Chapter 3; Rehabilitation for Return to the Society', and 'Chapter 4; Advanced Technique for Stroke Rehabilitation'. Both the adaptation and de novo development methods were used to develop this 2nd edition of CPG. The appraisal of foreign CPGs was performed using 'Korean appraisal of guidelines for research and evaluation II' (K-AGREE II); moreover, four CPGs from Scotland (2010), Austrailia (2010), USA (2010), Canada (2010) were chosen for adaptation. For de novo development, articles that were published following the latest foreign CPGs were searched from the database system, PubMed, Embase, and Cochrane library. Literatures were assessed in the aspect of subjects, study design, study results' consistency, language and application possibility in the Korean society. The chosen articles' level of evidence and grade of recommendation were decided by the criteria of Scotland (2010) and the formal consensus was derived by the nominal group technique. The levels of evidence range from 1++ to 4 and the grades of recommendation range from A to D. GPP (Good Practice Point) was recommended as best practice based on the clinical experience of the guideline developmental group. The draft of the developed CPG was reviewed by the experts group in the public hearings and then revised.
Canada ; Centers for Disease Control and Prevention (U.S.) ; Consensus ; Consultants ; Humans ; Korea* ; Practice Guidelines as Topic ; Rehabilitation* ; Scotland ; Specialization ; Stroke*

Several studies have suggested that a positive lymphocyte cross-matching (XM) is associated with low graft survival rates and a high prevalence of acute rejection after adult living donor liver transplantations (ALDLTs) using a small-for-size graft. However, there is still no consensus on preoperative desensitization. We adopted the desensitization protocol from ABO-incompatible LDLT. We performed desensitization for the selected patients according to the degree of T lymphocyte cross-match titer, model for end-stage liver disease (MELD) score, and graft liver volume. We retrospectively evaluated 230 consecutive ALDLT recipients for 5 yr. Eleven recipients (4.8%) showed a positive XM. Among them, five patients with the high titer (> 1:16) by antihuman globulin-augmented method (T-AHG) and one with a low titer but a high MELD score of 36 were selected for desensitization: rituximab injection and plasmapheresis before the transplantation. There were no major side effects of desensitization. Four of the patients showed successful depletion of the T-AHG titer. There was no mortality and hyperacute rejection in lymphocyte XM-positive patients, showing no significant difference in survival outcome between two groups (P=1.000). In conclusion, this desensitization protocol for the selected recipients considering the degree of T lymphocyte cross-match titer, MELD score, and graft liver volume is feasible and safe.
ABO Blood-Group System/immunology ; Adult ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Desensitization, Immunologic/*methods ; End Stage Liver Disease/surgery ; Female ; Graft Rejection/immunology ; Graft Survival/*immunology ; Histocompatibility Testing ; Humans ; Liver/surgery ; *Liver Transplantation ; Living Donors ; Male ; Middle Aged ; Plasmapheresis ; Preoperative Care ; Retrospective Studies ; Severity of Illness Index ; Survival Rate ; T-Lymphocytes/*immunology ; *Transplant Recipients

BACKGROUND: Simian virus 40 (SV40), a polyomavirus, was discovered as a contaminant of a human polio vaccine in the 1960s. It is known that malignant mesothelioma (MM) is associated with SV40, and that the virus works as a cofactor to the carcinogenetic effects of asbestos. However, the reports about the correlation between SV40 and MM have not been consistent. The purpose of this study is to identify SV40 in MM tissue in Korea through detection of SV40 protein and DNA. METHODS: We analyzed 62 cases of available paraffin-blocks enrolled through the Korean Malignant Mesothelioma Surveillance System and performed immunohistochemistry for SV40 protein and real-time polymerase chain reaction (PCR) for SV40 DNA. RESULTS: Of 62 total cases, 40 had disease involving the pleura (64.5%), and 29 (46.8%) were found to be of the epithelioid subtype. Immunostaining demonstrated that all examined tissues were negative for SV40 protein. Sufficient DNA was extracted for real-time PCR analysis from 36 cases. Quantitative PCR of these samples showed no increase in SV40 transcript compared to the negative controls. CONCLUSIONS: SV40 is not associated with the development of MM in Korea.
Asbestos ; DNA ; Humans ; Immunohistochemistry ; Korea ; Mesothelioma ; Pleura ; Poliomyelitis ; Polymerase Chain Reaction ; Polyomavirus ; Real-Time Polymerase Chain Reaction ; Simian virus 40 ; Viruses

BACKGROUND: This study was conducted to analyze, compare, and assess the indications, incidences, and types of chromosomal abnormalities in the amniotic fluid, chorionic villus sampling (CVS), and abortus and to compare these with those previously reported. METHODS: The study subjects included 1,995 cases of amniocentesis and 169 cases of abortus, 20 cases of CVS, 21 cases of cord blood, and 2 cases of cardiac-puncture fluid in the last ten years (June 1999 to May 2009). RESULTS: Among the indications, the maternal serum and triple/quad markers testing positive emerged the highest (57.5%). Other factors those were found were an advanced maternal age (over35) (19.5%) and abnormal ultrasonography findings (8.2%). The frequency of chromosomal abnormality in the amniotic fluid was 4.5%, wherein the numerical abnormality was 3.1% and the structural abnormality was 1.4%. Among the numerical abnormalities, trisomy of chromosome 21 emerged the highest (1.4%). The frequency of the chromosomal abnormality of CVS and abortus was 39.1%, the numerical abnormality was 23.7%, and the order of frequency for trisomy was obtained chromosomes 16, 22, and 21. In the sex ratio of the normal chromosomes, it was 1.1%, but it resulted in 0.5% in CVS. CONCLUSIONS: The results of this chromosomal study on amniotic fluid, CVS, and abortus could serve as useful data regarding the prenatal genetic abnormalities of fetuses and for genetic consultation.
Amniocentesis ; Amniotic Fluid ; Chorionic Villi Sampling ; Chromosome Aberrations ; Chromosomes, Human, Pair 21 ; Cytogenetic Analysis ; Cytogenetics ; Female ; Fetal Blood ; Fetus ; Incidence ; Maternal Age ; Pregnancy ; Sex Ratio ; Trisomy

PURPOSE: Hyperuricemia is a common complication occurring shortly after kidney transplantation. Although increased uric acid level is a risk factor for cardiovascular disease and mortality, the relationship between uric acid level and graft function after transplantation has been a controversial issue. Therefore, we investigated the effects of uric acid on glomerular filtration rate (GFR) and graft survival in the early periods of kidney transplantation. METHODS: Data were collected from 245 patients who underwent kidney transplantation between 2002 and 2004 at Yonsei University Medical Center. Uric acid level and estimated GFR were measured monthly during the first 6 months and then yearly for 3 years. RESULTS: The mean age of the study population was 40.2+/-11.7 years. The proportion of patients with hyperuricemia (uric acid < or =6.8 mg/dL) showed increment during the 3 year follow up. Increased serum uric acid level showed a negative correlation with estimated GFR during the initial 6 months after transplantation (r=-0.026, p<0.05). In Kaplan-Meier analysis, patients with a mean uric acid level higher than 6.8 mg/dL during the first 6 months showed a lower cumulative graft survival during the consecutive 3 years compared to patients with a uric acid level lower than 6.8 mg/dL (HR 1.7, p=0.003). CONCLUSION: Uric acid levels were associated with the changes of GFR in the initial 6 months of kidney transplantation, and the hyperuricemia during the early stages of transplantation might influence the long term graft survival.
Academic Medical Centers ; Cardiovascular Diseases ; Follow-Up Studies ; Glomerular Filtration Rate ; Graft Survival ; Humans ; Hyperuricemia ; Kaplan-Meier Estimate ; Kidney ; Kidney Transplantation ; Risk Factors ; Transplants ; Uric Acid