Clinical analysis of 10 cases of multi-center tumor necrosis factor receptor-associated periodic syndrome / 中华儿科杂志

Objective: To summarize the clinical characteristics of tumour necrosis factor receptor-associated periodic syndrome (TRAPS) in children. Methods: The clinical manifestations, laboratory tests, genetic testing and follow-up of 10 children with TRAPS from May 2011 to May 2021 in 6 hospitals in China were retrospectively analyzed. Results: Among the 10 patients with TRAPS, including 8 boys and 2 girls. The age of onset was 2 (1, 5) years, the age of diagnosis was (8±4) years, and the time from onset to diagnosis was 3 (1, 7) years. A total of 7 types of TNFRSF1A gene variants were detected, including 5 paternal variations, 1 maternal variation and 4 de novo variations. Six children had a family history of related diseases. Clinical manifestations included recurrent fever in 10 cases, rash in 4 cases, abdominal pain in 6 cases, joint involvement in 6 cases, periorbital edema in 1 case, and myalgia in 4 cases. Two patients had hematological system involvement. The erythrocyte sedimentation rate and C-reactive protein were significantly increased in 10 cases. All patients were negative for autoantibodies. In the course of treatment, 5 cases were treated with glucocorticoids, 7 cases with immunosuppressants, and 7 cases with biological agents. Conclusions: TRAPS is clinically characterized by recurrent fever accompanied by joint, gastrointestinal, skin, and muscle involvement. Inflammatory markers are elevated, and autoantibodies are mostly negative. Treatment mainly involves glucocorticoids, immunosuppressants, and biological agents.

Short-term waterlogging-induced autophagy in root cells of wheat can inhibit programmed cell death.

Autophagy is a pathway for the degradation of cytoplasmic components in eukaryotes. In wheat, the mechanism by which autophagy regulates programmed cell death (PCD) is unknown. Here, we demonstrated that short-term waterlogging-induced autophagy inhibited PCD in root cells of wheat. The waterlogging-tolerant wheat cultivar Huamai 8 and the waterlogging-sensitive wheat cultivar Huamai 9 were used as experimental materials, and their roots were waterlogged for 0-48 h. Waterlogging stress increased the number of autophagic structures, the expression levels of autophagy-related genes (TaATG), and the occurrence of PCD in root cells. PCD manifested as morphological changes in the cell nucleus, significant enhancement of DNA laddering bands, and increases in caspase-like protease activity and the expression levels of metacaspase genes. The autophagy promoter rapamycin (RAPA) reduced PCD levels, whereas the autophagy inhibitor 3-methyladenine (3-MA) enhanced them. The expression levels of TaATG genes and the number of autophagic structures were lower in cortex cells than in stele cells, but the levels of PCD were higher in cortex cells. The number of autophagic structures was greater in Huamai 8 than in Huamai 9, but the levels of PCD were lower. In summary, our results showed that short-term waterlogging induced autophagy which could inhibit PCD. Mechanisms of response to waterlogging stress differed between cortex and stele cells and between two wheat cultivars of contrasting waterlogging tolerance.

Mutual regulation of ROS accumulation and cell autophagy in wheat roots under hypoxia stress.

Here, we explored the mutual regulation of radical oxygen species (ROS) and autophagy in wheat (Triticum aestivum L.) roots under hypoxia stress. We also analyzed differences between the responses of the stele and the cortex in the two wheat cultivars Huamai 8 (waterlogging-tolerant) and Huamai 9 (waterlogging-sensitive) to hypoxia stress. In situ detection and ultracytochemical localization analysis in wheat roots showed that hypoxia stress caused greater increases in ROS levels and the expression levels of alternative oxidase (AOX) and antioxidant enzymes in the stele than in the cortex. The analysis of exogenous ROS addition and the inhibition of its production revealed the pivotal roles played by ROS in autophagy. Moreover, transmission electron microscopy and qRT-PCR analysis indicated that the stele had a higher level of autophagy than the cortex and that the two wheat cultivars primarily differed in the type and number of autophagosomes. Additional research revealed that autophagy could remove excess ROS, as pre-treatment with the autophagy inhibitor 3-methyladenine increased ROS levels in roots and the addition of the autophagy inducer rapamycin reduced root ROS levels. In conclusion, hypoxia stress induced ROS accumulation in wheat roots where ROS acted as an autophagy signal. Furthermore, higher levels of autophagy and antioxidant enzyme expression in the stele facilitated the elimination of oxidative damage caused by excessive ROS and thereby increased cell survival; in the cortex, a large number of cells died and formed aerenchyma.

Investigation of Relationship Between Spleen and Alzheimer' s Disease Based on Glucose, Lipid and Energy Metabolisms / 中国实验方剂学杂志

Alzheimer' s disease (AD) is a chronic neurodegenerative disease, which is seriously affecting people' s lives. Until now, the pathogenesis of AD is still unknown which result in its worldwide difficulties in prevention and treatment. Some studies have shown that AD might be a metabolic disease associated with glucose, lipid and energy metabolisms. Traditional Chinese medicine (TCM) believes that the spleen is acquired foundation and the origination of Qi and blood. The function of spleen is not only closely related to the metabolism of substance and energy, but also related to the aging of human body. In this article, we summarized and analyzed the interrelationship of metabolism, AD and spleen, as well as the effect of spleen-invigorating prescription on AD. The purpose of the paper is to analyze the possible mechanism of TCM treating AD from the perspective of regulating metabolism, explore the potential value of spleen-strengthening TCM in the treatment or prevention of AD, and provide new ideas for exploring the drug development and TCM therapy of AD.

The release of cytochrome c and the regulation of the programmed cell death progress in the endosperm of winter wheat (Triticum aestivum L.) under waterlogging.

It has been shown in mammalian systems that the mitochondria can play a key role in the regulation of apoptosis by releasing intermembrane proteins (such as cytochrome c) into the cytosol. Cytochrome c released from the mitochondria to the cytoplasm activates proteolytic enzyme cascades, leading to specific nuclear DNA degradation and cell death. This pathway is considered to be one of the important regulatory mechanisms of apoptosis. Previous studies have shown that endosperm cell development in wheat undergoes specialized programmed cell death (PCD) and that waterlogging stress accelerates the PCD process; however, little is known regarding the associated molecular mechanism. In this study, changes in mitochondrial structure, the release of cytochrome c, and gene expression were studied in the endosperm cells of the wheat (Triticum aestivum L.) cultivar "huamai 8" during PCD under different waterlogging durations. The results showed that waterlogging aggravated the degradation of mitochondrial structure, increased the mitochondrial permeability transition (MPT), and decreased mitochondrial transmembrane potential (ΔΨm), resulting in the advancement of the endosperm PCD process. In situ localization and western blotting of cytochrome c indicated that with the development of the endosperm cell, cytochrome c was gradually released from the mitochondria to the cytoplasm, and waterlogging stress led to an advancement and increase in the release of cytochrome c. In addition, waterlogging stress resulted in the increased expression of the voltage-dependent anion channel (VDAC) and adenine nucleotide translocator (ANT), suggesting that the mitochondrial permeability transition pore (MPTP) may be involved in endosperm PCD under waterlogging stress. The MPTP inhibitor cyclosporine A effectively suppressed cell death and cytochrome c release during wheat endosperm PCD. Our results indicate that the mitochondria play important roles in the PCD of endosperm cells and that the increase in mitochondrial damage and corresponding release of cytochrome c may be one of the major causes of endosperm PCD advancement under waterlogging.

Effects of different extraction methods on composition and antibacterial activity of volatile oil from Forsythiae Fructus, Schinzonepetae Herba, and Menthae Haplocalycis Herba / 中草药

Objective To compare the chemical composition and antibacterial activity of the volatile oils from Forsythiae Fructus (FF), Schizonepetae Herba (SH), Menthae Haplocalycis Herba (MH), and their compatibility, and to explore the influence of different extraction methods on components and antibacterial activity of volatile oil. Methods GC-MS was employed to analyze the composition of the volatile oil from FF, SH, and MH extracted separately, and their mixture extracted, as well as physical mixing volatile oil of FF, SH, and MH extracted separately. The diameter of the inhibition zone and the minimum inhibitory concentration (MIC) were measured using the paper agar diffusion method and micro dilution method. The antibacterial activities of volatile oil on four common pathogens of respiratory tract were evaluated. Results When FF, SH, and MH were mixed and extracted, the main obtained components and contents of volatile oil changed. Compared to separately extracted volatile oil, eight new components emerged, seven components from FF and seven components from SH disappeared in FF and SH mixture extracted volatile oil. In FF and MH mixture extracted volatile oil, compared with separately extracted, six components from FF and eight components from MH disappeared, and eight new components emerged. As to SH and MH mixture extracted volatile oil, four components from SH and seven components from MH disappeared, and seven new components emerged. In the three herbs mixture extracted volatile oil, six components from FF, four components from SH, and two components from MH disappeared, one commom component of FF and MH, two common components of SH and MH disappeared, however, nine new components emerged. The content of pulegone significantly decreased in the three herbs mixture extracted volatile oil compared with that in separately extracted. The antibacterial experiments showed that the antibacterial effects of the volatile oils obtained by different extraction methods were different. The antibacterial effects of volatile oil extracted separately and their physical mixing volatile oil on Escherichia coli, Staphylococcus aureus, Pseudomonas aeruginosa, and Candida albicans were better than that from mixture extracted. Conclusion Volatile oil is an important effective component of Chinese patent medicines with relieving exterior syndrome function. Different extraction methods have a certain influence on the yield and composition of volatile oil, which ultimately affects the efficacy of drugs. Therefore, we should pay more attention to extraction method and process of volatile oil in these prescriptions.

Reactive oxygen species regulate programmed cell death progress of endosperm in winter wheat (Triticum aestivum L.) under waterlogging.

Previous studies have proved that waterlogging stress accelerates the programmed cell death (PCD) progress of wheat endosperm cells. A highly waterlogging-tolerant wheat cultivar Hua 8 and a waterlogging susceptible wheat cultivar Hua 9 were treated with different waterlogging durations, and then, dynamic changes of reactive oxygen species (ROS), gene expressions, and activities of antioxidant enzymes in endosperm cells were detected. The accumulation of ROS increased considerably after 7 days of waterlogging treatment (7 DWT) and 12 DWT in both cultivars compared with control group (under non-waterlogged conditions), culminated at 12 DAF (days after flowering) and reduced hereafter. Waterlogging resulted in a great increase of H2O2 and O2 (-) in plasma membranes, cell walls, mitochondrias, and intercellular spaces with ultracytochemical localization. Moreover, the deformation and rupture of cytomembranes as well as the swelling and distortion of mitochondria were obvious. Under waterlogging treatment conditions, catalase (CAT) gene expression increased in endosperm of Hua 8 but activity decreased. In addition, Mn superoxide dismutase (MnSOD) gene expression and superoxide dismutase (SOD) activity increased. Compared with Hua 8, both CAT, MnSOD gene expressions and CAT, SOD activities decreased in Hua 9. Moreover, ascorbic acid and mannitol relieve the intensifying of PCD processes in Hua 8 endosperm cells induced by waterlogging. These results indicate that ROS have important roles in the PCD of endosperm cells, the changes both CAT, MnSOD gene expressions and CAT, SOD activities directly affected the accumulation of ROS in two different wheat cultivars under waterlogging, ultimately led to the PCD acceleration of endosperm.

Pathological diagnosis and ultrastructure features of primary pulmonary cryptococcosis: a study of 27 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate diagnostic histopathology and ultrastructure features of primary pulmonary cryptococcosis (PC).</p><p><b>METHODS</b>Clinical data and pathologic findings of 27 cases of PC were retrospectively reviewed, light and electron microscopic evaluations and histochemistry stain studies were performed.</p><p><b>RESULTS</b>The specimens consisted of 2 fine-needle aspiration lung biopsies and 25 cases of open lung biopsies. Cryptococcosis granuloma formation was identifiable by histopathological examination in 25 of 27 cases, with gum-like lesion and fungi in the remaining 2 cases. The detection rates of cryptococcus neoformans (CN) by mucicarmine (MC), periodic acid-Schiff (PAS), alcian blue (AB) and Grocott methenamine-silver (GMS) were 87.0% (20/23), 100% (27/27), 66.7% (18/27), and 100% (23/23) respectively. Under the electron microscope, most CN had a simple structure with a few organelles. The capsule was seen in all organisms. A percentage of the organisms showed nuclei, nucleoli, mitochondria and vacuoles. The detection rate of CN by EM was 91.7% (11/12).</p><p><b>CONCLUSIONS</b>The clinical manifestation and imaging of PC are nonspecific for PC. Lung biopsy is the major diagnostic modality. The detection rate by electron microscopy was quite high. Therefore, a correct diagnosis of pulmonary cyrptococcosis should rely on the combination of histopathological evaluation, histochemistry staining and/or electron microscopic examination.</p>