Article
Clinical Neurology
Woo Jin Lee, Han Sang Lee, Do Yong Kim, Hye Sung Lee, Jangsup Moon, Kyung Il Park, Sang Kun Lee, Kon Chu, Soon Tae Lee
Summary: This institutional cohort study examines the clinical characteristics, disease course, effectiveness of immunotherapy, and prognostic factors in patients with seronegative autoimmune encephalitis. Combination immunotherapy with steroids, immunoglobulin, rituximab, and tocilizumab was associated with improved outcomes.
Review
Virology
Chih-Cheng Lai, Chi-Kuei Hsu, Shun-Chung Hsueh, Muh-Yong Yen, Wen-Chien Ko, Po-Ren Hsueh
Summary: Following the emergence of multisystem inflammatory syndrome in children (MIS-C), a similar clinical scenario called multisystem inflammatory syndrome in adults (MIS-A) has been observed. Although the prevalence of MIS-A is low and likely underdiagnosed, it can be associated with high mortality. MIS-A is a multisystem disease that affects the cardiovascular, respiratory, gastrointestinal, dermatologic, hematologic, and neurologic systems. The diagnosis of MIS-A requires clinical manifestations, laboratory evidence of inflammation, and SARS-CoV-2 infection. The appropriate treatment for MIS-A is still uncertain, but anti-inflammatory agents like intravenous immunoglobulin and corticosteroids are commonly used. Further research is needed to determine the true prevalence, pathogenesis, and effective treatment for MIS-A.
JOURNAL OF MEDICAL VIROLOGY
(2023)
Article
Immunology
Lingbo He, Shuyan Yao, Ruoxi Zhang, Menghan Liu, Zhengjie Hua, Heshan Zou, Zhao Wang, Yini Wang
Summary: This study described the clinical characteristics and outcomes of adult macrophage activation syndrome (MAS) patients. The results showed that MAS typically occurs within 2 months after the onset of autoimmune disease in adults. The etoposide-based regimen is effective and tolerable for adult MAS.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Woo-Jin Lee, Young Nam Kwon, Boram Kim, Jangsup Moon, Kyung-Il Park, Kon Chu, Jung-Joon Sung, Sang Kun Lee, Sung-Min Kim, Soon-Tae Lee
Summary: This study investigated the clinical characteristics and outcomes of myelin oligodendrocyte glycoprotein (MOG) antibody-associated autoimmune encephalitis (MOGAE) in adult patients. The results showed that the outcomes varied according to the three phenotypes in MOGAE. Short immunotherapy maintenance was associated with relapse, and brain atrophy and dual antibodies of NMDAR and MOG were associated with poor outcomes.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Review
Clinical Neurology
Robin Stinissen, Eldar Tukanov, Martin Wyckmans, Jeroen Kerstens, Barbara Willekens
Summary: This review provides an overview of patient characteristics, disease course, and treatment patterns in Susac syndrome (SuS) since 2013. It highlights the variability in treatment approaches and suggests the establishment of registries to collect data for more evidence-based therapeutic approaches.
JOURNAL OF NEUROLOGY
(2023)
Review
Medicine, General & Internal
Jin Zhang, Cheng Li, Xiaorong Han, Zhongbo Chen, Binay Kumar Adhikari, Yinghui Wang, Yonggang Wang, Jian Sun
Summary: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by vascular thrombosis and pregnancy complications. Digestive system involvement in APS may result from autoimmune or thrombotic mechanisms, with the liver being the most commonly affected organ. This review discusses the clinical manifestations of APS in the digestive system, along with the epidemiology, pathophysiology, and treatment strategies for these patients.
ANNALS OF MEDICINE
(2021)
Review
Medicine, General & Internal
Gopala K. Rangan, Nilofar Dorani, Miranda M. Zhang, Lara Abu-Zarour, Ho Ching Lau, Alexandra Munt, Ashley N. Chandra, Sayanthooran Saravanabavan, Anna Rangan, Jennifer Q. J. Zhang, Martin Howell, Annette T. Y. Wong
Summary: This study reviewed case reports and studies from 1946 to 2019 on excess water intake and hyponatraemia. The results showed that water intoxication can lead to significant health risks and mortality, with treatment mainly consisting of supportive care and saline solutions.
Article
Medicine, General & Internal
Sophia Lionaki, Evangelos Mantios, Ioanna Tsoumbou, Smaragdi Marinaki, George Makris, George Liapis, Chrysovalantis Vergandis, Ioannis Boletis
Summary: The study found that nearly 46% of adult MCD patients experienced at least one relapse, although renal progression was rare. Younger age of onset was identified as an independent risk factor for relapse in adult MCD patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Dentistry, Oral Surgery & Medicine
Moon-Jong Kim, Jihoon Kim, Hong-Seop Kho
Summary: The study found that clonazepam therapy in combination with appropriate medications was effective for managing patients with burning mouth syndrome. The initial intensity of oral symptoms and psychological status were significantly associated with treatment outcomes.
Article
Medicine, General & Internal
Chu-Sheng Lin, Wei-Ju Lee, Shih-Yi Lin, Hui-Ping Lin, Ran-Chou Chen, Chi-Hung Lin, Liang-Kung Chen
Summary: This study aimed to explore the subtypes of metabolic syndrome and their associations with new onset of diabetes, cardiovascular disease, and major adverse cardiovascular events (MACE). The findings suggest that the risks vary among different subtypes of metabolic syndrome.
FRONTIERS IN MEDICINE
(2022)
Article
Medicine, General & Internal
Halil Yildiz, Diego Castanares-Zapatero, Philippe d'Abadie, Sarah Bailly, Jean Cyr Yombi
Summary: This study examined the clinical and biological characteristics of adult HLH patients in Belgium, revealing that malignancy and infection were the most common triggers. Risk factors for mortality included age, male sex, malignancy, and DIC, with age being the only significant factor in multivariate analysis.
INTERNATIONAL JOURNAL OF GENERAL MEDICINE
(2022)
Article
Clinical Neurology
Rong Yan, Yu Mao, Huiyang Zeng, Qian Liu, Hanqiu Jiang, Jingting Peng, Qingling Yang, Shilei Cui, Lei Liu, Yanjun Guo, Jiawei Wang
Summary: This study reviewed the neuro-ophthalmic findings and outcomes in 5 patients with CRMP5-ON, and found that it often presented with optic disc edema, diffuse defects of the central field, and complications such as vitreitis, retinitis, or uveitis. MRI examination showed optic nerve or perineural optic nerve enhancement, which was associated with tumors. Cancer screening and intervention were crucial for prognosis.
FRONTIERS IN NEUROLOGY
(2023)
Article
Genetics & Heredity
Dong Wang, Xi-Hua Chen, Ang Wei, Chun-Ju Zhou, Xue Zhang, Hong-Hao Ma, Hong-Yun Lian, Li Zhang, Qing Zhang, Xiao-Tong Huang, Chan-Juan Wang, Ying Yang, Wei Liu, Tian-You Wang, Zhi-Gang Li, Lei Cui, Rui Zhang
Summary: This study retrospectively described the clinical-biological characteristics of 28 pediatric LCH patients with MAS-HLH and analyzed the treatment outcomes of second-line chemotherapy and targeted therapy for BRAF-V600E-positive patients. The study found that the BRAF inhibitor dabrafenib provides a promising treatment option for LCH patients with MAS-HLH.
ORPHANET JOURNAL OF RARE DISEASES
(2022)
Article
Oncology
Chunjiang Wang, Wei Sun, Zuojun Li, Tian Wu, Weijin Fang
Summary: This study retrospectively analyzed the literature related to pembrolizumab-induced HLH. The results showed that the most common symptoms were fever, splenomegaly, and hepatomegaly. Laboratory examination revealed abnormalities in anemia, leukopenia, thrombocytopenia, hypertriglyceridemia, and hypofibrinogenemia. Bone marrow biopsy showed hemophagocytosis. After treatment with steroids, etoposide, intravenous immunoglobulin, cytokine blocking, and immunosuppression, the majority of patients showed improvement or recovery, but some patients still died.
INVESTIGATIONAL NEW DRUGS
(2023)
Article
Hematology
Mahasweta Gooptu, Haesook T. Kim, Eric Jacobsen, David C. Fisher, Ann LaCasce, Vincent T. Ho, Corey S. Cutler, John Koreth, Robert J. Soiffer, Joseph H. Antin, Nancy Berliner, Sarah Nikiforow
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that can be fatal if not diagnosed and treated early. Allogeneic hematopoietic stem cell transplantation (HSCT) is a potential curative option for primary and relapsed/refractory HLH cases. This study analyzed the outcomes of adult HLH patients who underwent HSCT with a reduced intensity platform and early administration of alemtuzumab at a cancer institute. The results showed high overall survival and progression-free survival rates, suggesting that HSCT with a reduced intensity regimen can effectively treat HLH in adults.