Clinicopathological and imaging differences between pediatric and adult patients with anti-hydroxy-3-methyl-glutaryl-coenzyme A reductase necrotizing myopathy.

We aimed to elucidate the clinicopathological and imaging differences between pediatric and adult patients with anti-hydroxy-3-methyl-glutaryl-coenzyme A reductase (anti-HMGCR) myopathy. A series of 111 patients with anti-HMGCR myopathy were divided into pediatric (33 patients, onset age < 18 years) and adult (78 patients, onset age ≥ 18 years) groups. Clinical, imaging, and pathological characteristics were compared between the groups.

Treatment of refractory immune-mediated necrotizing myopathy with efgartigimod.

Objective: We aimed to explore the efficacy and safety of efgartigimod in patients with refractory immune-mediated necrotizing myopathy (IMNM).

Methods: This open-label pilot observational study included seven patients with refractory IMNM, all of whom received intravenous efgartigimod treatment. The clinical response was assessed after 4 weeks of efgartigimod treatment according to the 2016 American College of Rheumatology-European League Against Rheumatism response criteria for adult idiopathic inflammatory myopathy.

Varicella-zoster virus meningitis with hypoglycorrhachia: A case report.

Background: Varicella-zoster virus (VZV) is a common viral infection, but meningitis is a rare complication of VZV infection. The cerebrospinal fluid glucose of viral meningitis is usually within the normal range, which is different from bacteria, fungi, and cancerous meningitis. This paper reports a case of VZV meningitis with hypoglycorrhachia and the relevant literature was reviewed.

Optimization of the cut-offs in acetylcholine receptor antibodies and diagnostic performance in myasthenia gravis patients.

Objective: This study aims to establish an optimization procedure to define the cut-offs of quantitative assays for acetylcholine receptor antibody (AChRAb), evaluate their diagnostic performance in myasthenia gravis (MG), and explore the association with clinical features.

Methods: Samples from a representative cohort of 77 MG patients, 80 healthy controls (HC) and 80 other autoimmune diseases (OAD) patients were tested using competitive inhibition ELISA and RIA. Raw values (OD and cpm) and processed values (inhibition rate, binding rate and concentration) were used to define the cut-offs with statistical methods, a rough method, and receiver operating characteristic (ROC) curve.

Juvenile Generalized Myasthenia Gravis With AChR and MuSK Antibody Double Positivity: A Case Report With a Review of the Literature.

Myasthenia gravis is an autoimmune disease mediated by B cells and is associated with acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK) antibodies in the postsynaptic membrane at the neuromuscular junction. The presence of both antibodies in the serum of patients with myasthenia gravis has been rarely reported. Case description: A 9-year-old girl was admitted to our hospital with the chief complaints of reduced facial expression for 3 months and unclear speech and choking from drinking water for 2 months.

Noninvasive tools based on immune biomarkers for the diagnosis of central nervous system graft--host disease: Two case reports and a review of the literature.

Background: Central nervous system graft--host disease (CNS-GVHD) is a rare cause of CNS disorders after allogeneic hematopoietic stem cell transplantation. Currently, establishing a diagnosis of CNS-GVHD is challenging because the diagnostic criteria and diagnostic methods are not well defined and many confounding factors need to be ruled out.

Case Summary: Here, we present two patients with CNS-GVHD.

Vitamin D Receptor Polymorphism and Myasthenia Gravis in Chinese Han Population.

Myasthenia gravis (MG) is an autoimmune disease in which antibodies bind to acetylcholine receptors (AChR) or other functional molecules in the postsynaptic membrane at the neuromuscular junction. Vitamin D (VD) has a number of pluripotent effects, which include immune-regulation and bone metabolism. The immunomodulatory actions of 1,25(OH)2D3 are mediated by its binding to a vitamin D receptor (VDR).

Complement C3 polymorphism is associated with the susceptibility of myasthenia gravis in Chinese adult patients.

Complement component 3 (C3) had been proved to be involved in the pathogenesis and exacerbation of both myasthenia gravis (MG) patients and experimental autoimmune myasthenia gravis (EAMG) models. We evaluated the underlying association between five SNPs (rs344555, rs7951, rs3745568, rs366510 and rs163913) in C3 gene and Chinese adult MG patients. Our study consisted of 409 adult MG patients and 487 healthy controls.

Association between CTLA-4 gene polymorphism and myasthenia gravis in a Chinese cohort.

Abnormal CTLA-4 expression is involved in the development of myasthenia gravis (MG), and serum CTLA-4 levels are positively correlated with serum anti-AChR antibody concentration, which might be related with the severity of MG. Polymorphism in CTLA-4 gene is associated with various autoimmune disorders. We investigated the association of polymorphism in CTLA-4 gene with the clinical variables and severity of MG.

IL-4Rα Polymorphism Is Associated With Myasthenia Gravis in Chinese Han Population.

Interleukin-4 (IL-4) is a potent growth and differentiation factor for B cells which play a vital role in the pathogenesis of myasthenia gravis (MG). IL-4 exerts its function by binding to three types of IL-4 receptor (IL-4R) complexes. IL-4Rα is the key component of the IL-4R complex.

Rs3761389 polymorphism in autoimmune regulator (AIRE) gene is associated with susceptibility of myasthenia gravis in Chinese patients.

Polymorphism in autoimmune regulator (AIRE) gene is associated with various autoimmune disorders. Abnormal AIRE expression is associated with the development of myasthenia gravis (MG). We investigated the association of polymorphism in AIRE gene and the clinical features and severity of MG.

Autoantibody profile and clinical characteristics in a cohort of Chinese adult myasthenia gravis patients.

Myasthenia gravis (MG) is an autoimmune disorder with heterogeneity. Antibodies against acetylcholine receptor (AChR), muscle-specific kinase (MuSK), titin and ryanodine receptor (RyR) were examined in 437 adult Chinese MG patients. The AChR, MuSK, titin and RyR antibodies were found in 82.

Precision medicine in myasthenia graves: begin from the data precision.

Myasthenia gravis (MG) is a prototypic autoimmune disease with overt clinical and immunological heterogeneity. The data of MG is far from individually precise now, partially due to the rarity and heterogeneity of this disease. In this review, we provide the basic insights of MG data precision, including onset age, presenting symptoms, generalization, thymus status, pathogenic autoantibodies, muscle involvement, severity and response to treatment based on references and our previous studies.

Association study between IL-17A and IL-17F gene polymorphism and myasthenia gravis in Chinese patients.

Alleles of IL-17A and IL-17F genes were reported to be associated with many inflammatory and autoimmune disorders in Asian patients. Serum level and mRNA of IL-17A in peripheral blood mononuclear cells were reported to be significantly higher in MG patients than in healthy controls. In experimental autoimmune myasthenia gravis (EAMG) animals, IL-17 may have effects on the severity of MG.

Different Eukaryotic Initiation Factor 2Bε Mutations Lead to Various Degrees of Intolerance to the Stress of Endoplasmic Reticulum in Oligodendrocytes.

Background: Vanishing white matter disease (VWM), a human autosomal recessive inherited leukoencephalopathy, is due to mutations in eukaryotic initiation factor 2B (eIF2B). eIF2B is responsible for the initiation of protein synthesis by its guanine nucleotide exchange factor (GEF) activity. Mutations of eIF2B impair GEF activity at different degree.

[An analysis of stroke subtypes and influencing factors in different regions of China].

Objective: To analyze the stroke subtypes and influencing factors in four largest economic regions of China.

Methods: We analyzed the investigation data of QUEST (Quality Evaluation of Stroke Care and Treatment) study conducted in 2006 which included 62 hospitals in a national scale. According to the concept of four economic regions designed by the Development Research Center of the State Council, we performed the univariate and multivariate analysis for the stroke subtypes and its related risk factors in the different economic regions.

[Effects of quercetin on multidrug resistance and expression of related genes in human erythroleukemic K562/a cells].

The study was aimed to investigate the effect of quercetin, flavonoid molecules on reversing leukemia multidrug resistance and its mechanism. K562/A cells were cultured in vitro with different concentrations of quercetin. Cell growth inhibition and adriamycin (ADR) sensitivity were detected by MTT method.

Capture and activation of aerial CO2 by carbamoylation of L-threonine in a Ag(I) supramolecular framework.

An ultrasonic reaction of Ag(2)O, 4,4'-bipyridine (bipy) and (2S, 3R)-3-amino-2-hydroxybutanoic acid (L-Thr) gives an unexpected Ag(I) supramolecular framework, {[Ag(3)(bipy)(3)(cahba)]·HCO(3)·10H(2)O}(n) (1), in which the (2S, 3R)-3-(carboxyamino)-2-hydroxybutanoic acid (H(2)cahba) is a carbamate derivative of L-Thr, obtained via in situ transformation of amino group of L-Thr into carbamate by means of CO(2) uptake.

[Apoptosis-inducing effect of quercetin and kaempferol on human HL-60 cells and its mechanism].

The purpose of this study was to explore the anti-leukemia effect of quercetin and kaempferol and its mechanism. The HL-60 cells were used as the leukemia models. The inhibitory effects of quercetin and kaempferol on growth of HL-60 cells was assessed by MTT assay.