1.A Case Report of Pachydermoperiostosis by Multidisciplinary Diagnosis and Treatment
Jie ZHANG ; Yan ZHANG ; Li HUO ; Ke LYU ; Tao WANG ; Ze'nan XIA ; Xiao LONG ; Kexin XU ; Nan WU ; Bo YANG ; Weibo XIA ; Rongrong HU ; Limeng CHEN ; Ji LI ; Xia HONG ; Yan ZHANG ; Yagang ZUO
JOURNAL OF RARE DISEASES 2025;4(1):75-82
A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in
4.Exploring Immune Mechanism of Alveolar Epithelial Homeostasis in Idiopathic Pulmonary Fibrosis Based on Principle of "Spleen being in Charge of Defensive Function"
Jie CHEN ; Lijian PANG ; Ningzi ZANG ; Jingyu WANG ; Siyu LI ; Yuanyu LIANG ; XU XINZHU ; Ping LEI ; Xiaodong LYU
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(9):259-264
Idiopathic pulmonary fibrosis (IPF) can be classified as pulmonary collateral disease,and its pathogenesis is mainly characterized by the loss of Qi meridian nourishment,the loss of Yin meridian nourishment,and the formation of blood stasis in the blood vessels. Qi Yin deficiency is the pathological basis that runs through IPF,and obstruction of meridians and collaterals is a key element in the development of the disease. The dysfunction of "spleen being in charge of the defensive function" is closely related to the formation of the pathological pattern of "lung deficiency and collateral stasis" in IPF. The term "spleen being in charge of the defensive function" originated from the Yellow Emperor's Inner Canon. If the spleen is healthy,the Qi will be filled with vitality. Positive energy is stored inside,evil cannot be dried up. Its concept is quite similar to the immune defense function in modern medicine. If the principle of "spleen being in charge of the defensive function" is lost,the key structure and function of the IPF alveolar epithelial barrier may be abnormal,and it can interact with various innate immune cells to promote inflammation and fibrosis processes. Therefore,this article explains the imbalance of immune homeostasis in IPF alveolar epithelium from two aspects:the barrier function of alveolar epithelial cells(AECs) and their interaction with innate immune cells. And based on the theory of "spleen being in charge of the defensive function",using traditional Chinese medicine for strengthening the spleen and nourishing Qi to treat IPF from the perspective of the spleen. This not only strengthens the scientific connotation of "spleen being in charge of the defensive function" in the pathogenesis of IPF,but also provides new research directions and ideas for its future clinical prevention and treatment.
6.Subchronic exposure to benzoapyrene results in lung tissue cell damage caused by ferroptosis in mice
Chaoli ZHOU ; Shihan DING ; Hui HE ; Zhirui MA ; Jie CHEN ; Xingdi GUO ; Yi LYU ; Jinping ZHENG
Journal of Environmental and Occupational Medicine 2025;42(8):971-977
Background Exposure to benzo[a]pyrene (BaP) may impair lung function through various mechanisms; however, it remains uncertain whether BaP induces ferroptosis in lung tissue cells, resulting in lung function impairment. Objective To investigate the ferroptosis of lung tissue cells triggered by subchronic BaP exposure in mice and its correlation with lung injury, and to explore the function of ferroptosis in BaP-induced lung tissue damage. Method Seventy-two healthy 3-weeks-old male C57BL/6J mice were acclimatized for 1 week and then randomly divided into six groups: control group (corn oil 10 mL·kg−1), low-dose BaP group (2.5 mg·kg−1), medium-dose BaP group (5 mg·kg−1), high-dose BaP group (10 mg·kg−1), BaP+ferrostatin-1 (Fer-1) group (10 mg·kg−1+1 mg·kg−1), and Fer-1 group (1 mg·kg−1), with 12 mice each group. Corn oil and BaP were administered via gavage every other day, followed by an intraperitoneal injection of Fer-1 the subsequent day, throughout a period of 90 d. Whole-body plethysmography was applied to detect lung function; hematoxylin-eosin staining (HE) and Masson staining were used to observe lung tissue injury and fibrosis; microscopy of alveolar epithelial cells was conducted to reveal mitochondrial morphology; biochemical assays were used to measure the content of tissue iron, malondialdehyde (MDA), and glutathione (GSH), as well as the activity of glutathione peroxidase (GSH-Px); Western blotting and real-time quantitative PCR (RT-qPCR) analyses were performed to reveal the protein and mRNA expression of ferroptosis markers. Results Compared to the control group, the high-dose BaP group showed a significant increase in expiration time (Te) (P<0.01), and a significant decrease in ratio rate of achieving peak expiratory flow (Rpef), tidal volume (TVb), peak inspiratory flow (PIF), minute volume (MVb), and peak expiratory flow (PEF) (P<0.05 or 0.01). Based on the results of HE and Masson staining, partial destruction of alveolar structures, thickening of alveolar walls, infiltration of inflammatory cells, significant thickening of tracheal walls and a large deposition of collagen fibers in lung tissue were observed in the medium- and high-dose BaP groups. By microscopy, the alveolar epithelial cells exposed to low-dose BaP showed condensed chromatin, and the mitochondria exposed to medium and high-dose BaP showed wrinkles, increased mitochondrial membrane density, and diminished mitochondrial cristae. Compared to the control group, in the medium- and high-dose BaP groups, the lung tissue iron content and the expression levels of ACSL4 protein and mRNA significantly elevated (P<0.01 or 0.05), while the mRNA expression level of SLC7A11 significantly decreased (P<0.05); in the high-dose BaP group, the MDA content, COX2 protein, and PTGS2 mRNA expression levels significantly increased (P<0.05 or 0.01), GSH content and GSH-Px activity, GPX4 protein and mRNA expression levels, and the expression level of SLC7A11 protein significantly decreased (P<0.01 or 0.05). The ferroptosis inhibitor Fer-1 markedly reversed respiratory function, morphology, mitochondrial alterations, and the aforementioned ferroptosis-related biochemical indicators. Conclusion Subchronic exposure to BaP can induce ferroptosis in mice lung tissue cells, resulting in compromised lung function.
7.Effects of Compound Dihuang Granules on α-syn,VAPB,PTPIP51 on substantia nigra of 6-hydroxydopamine-induced Parkinson's disease model rats with Yin deficiency and wind syndrome
Guang-Jie SUN ; Xing-Ling WANG ; Zi-Wei LYU ; Jian-Cheng HE ; Jian-Qing LIANG
Chinese Pharmacological Bulletin 2024;40(6):1179-1186
Aim To observe the effects of Compound Dihuang Granules on α-syn,VAPB and PTPIP51 in the substantia nigra of Parkinson's disease(PD)rats with Yin deficiency and wind syndrome,and to explore the possible mechanisms of their actions.Methods The 6-hydroxydopamine-induced PD model of rats was constructed.The model rats were randomly divided into the model group,madopar group,CLD group,CMD group and CHD group,while the NC group did not re-ceive any treatment and the SO group was injected with ascorbic acid,with 13 rats in each group.The neurobe-havioral changes of the rats were observed,and the ex-pressions of α-syn,VAPB and PTPIP51 in the sub-stantia nigra were detected by Western blot,RT-PCR and Immunohistochemistry;the histopathological and morphological changes of the substantia nigra tissue were observed by HE staining,the changes of mito-chondrial ultrastructure in the substantia nigra cells were observed by transmission electron microscopy,and the changes of ATP content in substantia nigra tis-sue in each group were detected by ELISA.Results Compared with the NC and SO groups,rats in the model group showed that the number of rotational cir-cles and pole-climbing time increased,the expression of α-syn increased,the expression of VAPB and PTPIP 5 1 decreased,the number of neuronal cells decreased,the neuronal cells became crumpling,and mitochondrial swelling,disappearance of the mitochon-drial cristae,a larger distance between the ER-mito-chondrial contacts were observed;the ATP content de-creased.Compared with the model group,rats in Mado-par group and CLD group,CMD group and CHD group showed that the number of rotational circles and pole-climbing time decreased,the expression of α-syn de-creased,the expression of VAPB and PTPIP51 in-creased,the degree of neuronal damage was reduced,the morphology of mitochondria was improved and the content of ATP increased,showing the change of the difference in quantitative efficacy;the relative efficacy of Madopar group and CHD group was better,and there was no statistically significant difference.Con-clusions Compound Dihuang granules attenuate the behavioral symptoms in PD rats and may play a thera-peutic role in PD by down-regulating the expression ofα-syn,up-regulating the expression of PTPIP51 and VAPB and improving mitochondrial function.
8.Evaluation of the efficacy of Huoxue Tongluo recipe iontophoresis combined with butyphthalide sequential therapy in hemiplegia patients with cerebral ischemic stroke
Hongli ZHANG ; Peng LI ; Jie HU ; Xianglong LYU
China Pharmacist 2024;27(5):788-795
Objective To explore the efficacy and safety of the Huoxue Tongluo recipe iontophoresis(HTRI)combined with butylphthalide sequential therapy(BST)in patients with cerebral ischemic stroke(CIS)hemiplegia.Methods 87 patients with CIS hemiplegia treated in Chuzhou Chinese and Western Medicine Hospital affiliated to Anhui University of Chinese Medicine from January 2020 to January 2023 were retrospectively selected as study subjects,and were divided into the BST group(BST treatment)and the combination group(BST in combination with HTRI treatment)according to the treatment regimens.The main observation indicators were total treatment response rate and incidence of adverse reactions after 4 weeks of treatment;the secondary observation indicators were cerebral artery flow velocity[anterior cerebral artery(ACA),middle cerebral artery(MCA)and posterior cerebral artery(PCA)],neurological function[National Institutes of Health Stroke Scale(NIHSS)score,Chinese Stroke Scale(CSS)score]and limb function[Fugl-Meyer Motor Function Scale(FMA)score,Barthel index(BI)score].Results A total of 87 patients were included,including 43 patients in the BST group and 44 patients in the combination group.The total response rate in the combination group was significantly higher than that in the BST group(P<0.05),and no adverse reactions were observed in both groups.Before treatment,there were no significant differences in cerebral artery blood flow velocity,nerve function and limb function between the two groups(P>0.05).After treatment,the flow velocity of ACA,PCA and MCA,and the scores of FMA and BI were significantly higher than those before treatment(P<0.05),while the scores of NIHSS and CSS decreased significantly(P<0.05).Conclusion Compared with BST therapy,HTRI combined with BST has a more significant effect in CIS patients with hemiplegia,with no increase in the incidence of adverse reactions,and has higher safety.
9.TIPS combined with catheter-directed thrombolysis and percutaneous transhepatic portal vein catheterization thrombolysis for acute non-cirrhotic non-neoplastic portal vein thrombosis
Jie JI ; Bifei WU ; Lele YAN ; Penghua LYU ; Weizhong ZHOU ; Fu'an WANG
Chinese Journal of Interventional Imaging and Therapy 2024;21(10):583-587
Objective To comparatively observe the value of TIPS combined with catheter-directed thrombolysis and percutaneous transhepatic portal vein catheterization thrombolysis for acute non-cirrhotic non-neoplastic portal vein thrombosis(PVT).Methods Twenty-five patients with acute non-cirrhotic non-neoplastic PVT were retrospectively enrolled and clustered into TIPS group(n=17,underwent TIPS combined with catheter-directed thrombolysis)and liver puncture group(n=8,underwent percutaneous transhepatic portal vein catheterization thrombolysis)according to the access of thrombolysis.The technical success rate,duration of catheter-directed thrombolysis,complications within 7 days,as well as portal vein patency 3 months after treatment,Child-Pugh grading of liver function and occurrence of hepatic encephalopathy(HE)were recorded and compared between groups.Results The technical success rates were both 100%in 2 groups.There was no significant difference of the duration of catheter-directed thrombolysis between groups(P>0.05).The thrombolytic effect in TIPS group was better than that in liver puncture group(P<0.05).No significant difference of the occurrence of bleeding within 7 days was found between groups(P>0.05).After 3 months'follow-up,the degree of portal vein patency in TIPS group was higher than that in liver puncture group(P<0.05).No significant difference of Child-Pugh grading of liver function nor occurrence of HE was found between groups(both P>0.05).Conclusion Both TIPS combined with catheter-directed thrombolysis and percutaneous transhepatic portal vein catheterization thrombolysis were effective for treating acute non-cirrhotic non-neoplastic PVT,and the thrombolytic effect of the former was better than the latter.
10.Status quo and influencing factors of self-regulating fatigue in aged patients undergoing maintenance hemodialysis
Jie SUN ; Xiaohan LU ; Jie SONG ; Kaili LYU ; Kexin PU
Chinese Journal of Modern Nursing 2024;30(6):758-763
Objective:To understand the status quo of self-regulating fatigue (SRF) in aged patients undergoing maintenance hemodialysis (MHD) and analyze its influencing factors.Methods:This study is a cross-sectional study. From January to June 2022, 310 aged patients undergoing MHD from the blood purification centers of three ClassⅢ Grade A hospitals in Jinan, Shandong Province, were selected by convenience sampling. Various questionnaires, including the general information questionnaire, Mini-Mental State Examination (MMSE), Self-Regulatory Fatigue Scale (SRF-S), Medical Coping Modes Questionnaire (MCMQ), General Self-Efficacy Scale (GSES), and 5-Item Geriatric Depression Scale (GDS-5), were used to investigate and analyze the factors influencing self-regulation of fatigue in aged patients undergoing MHD through univariate and multiple stepwise regression analysis.Results:The SRF-S score for the aged patients undergoing MHD was (42.26±7.86). The results of the multiple factor analysis showed that family average monthly income, depression, coping style, and self-efficacy were factors influencing SRF in these patients ( P<0.05) . Conclusions:It is urgent to pay attention to the current status of self-regulation of fatigue in aged patients undergoing MHD and take effective measures to improve their self-efficacy, promote positive coping strategies, alleviate depression, and reduce their level of SRF.

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