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Current Medical Science 2021年第2期41卷 390-397页

作者： Yue-ying PAN Shu-chang ZHOU Yu-jin WANG Ting-ting ZHU Dan PENG Han-xiong GUAN Department of Radiology Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China Department of Nuclear Medicine Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patientsdiagnosed with IgG4-RD according to the comprehensive diagnostic criteria (CDC) were includedin the retrospective study from May 2012 to March 2019. We collcted data on clinical, laboratory,imaging, histological features and treatment. Totally, 98 patients with IgG4-RD were *** common clinical manifestations included abdominal pain, salivary gland swelling andlymphadenopathy. 51% of the patients had multiple organs involvement. Lymph nodes, pancreasand salivary glands were most commonly involved. Four rare sites including ulna, cerebellum,scalp, and mammary gland were found. The serum IgG4 level was increased by 85.7%. The serumIgG4 level was positively correlated with the number of involved organs, IgG and IgG4/IgG. LowC3 and C4 levels were observed in 37.5% and 12.2% patients respectively, and all patients withkidney involvement had hypocomplementemia. A total of 54 patients underwent tissue biopsies,and 55.6%, 31.5% and 11.1% cases were diagnosed as definite, probable and possible IgG4-RD,respectively. Eighty-eight patients received glucocorticoids (GCs) therapy. Five patients underwentradical surgery to remove the lesion. 73% of them presented a complete or partial remission. IgG4-RD is a systemic fibroinflammatory disease with involvement of multiple organs throughout thebody including some rare sites. Most IgG4-RD patients had increased serum IgG4 levels andpatients with kidney involvement showed bypocomplementermia. GCs therapy is effective. Moreresearch is needed to provide a more reliable basis for the diagnosis and treatment of patients.

关键词： immunoglobulin G4-related disease clinical features Immunoglobulin G4 hypocomplementemia glucocorticoid

Immunoadsorption therapy for Klinefelter syndrome with antiphospholipid syndrome in a patient:A case report

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World Journal of Clinical Cases 2022年第19期10卷 6580-6586页

作者： You Song Yong-Zhen Xiao Cheng Wang Rong Du Department of Rheumatology Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022Hubei ProvinceChina Health Management Center Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022Hubei ProvinceChina

BACKGROUND Klinefelter syndrome(KS) is a genetic disease of male sex chromosome malformations that affects sperm production and reduces testosterone production. It has been reported that there is currently more than 10 cases of KS combined with antiphospholipid syndrome(APS).CASE SUMMARY Here, we describe a 31-year-old male patient with chromosome 47, XXY type, who suffered deep vein thrombosis of the lower limbs accompanied by abnormal antiphospholipid antibody, lupus anticoagulant and factor VⅢ. After treatment with immunoadsorption therapy, glucocorticoids, cyclophosphamide, intravenous immunoglobulin and anticoagulant therapy, the patient showed dramatic symptomatic improvement. During the follow-up, the patient did not develop any new thrombotic *** Immunoadsorption combined with glucocorticoid and cyclophosphamide shock comprehensive treatment has achieved significant results for patients with KS combined with antiphospholipid syndrome.

关键词： Klinefelter syndrome Antiphospholipid syndrome Immunoadsorption glucocorticoid Cyclophosphamide Bone morphogenetic protein receptor type-2 Case report

Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report

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World Journal of Clinical Cases 2021年第1期9卷 267-273页

作者： Jong-Sun Kim Won Ho Choi Kyung-Ann Lee Hyun-Sook Kim Department of Internal Medicine Soonchunhyang University Seoul HospitalSeoul 04401South Korea

BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multi-system fibroinflammatory disorder that can involve any organ,including the salivary glands,pancreas,and biliary *** of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)is similar to that for IgG4-RD,but progression is irreversible in some *** present a case of IgG4-SC in which an immuno-suppressant induced marked clinical and radiologic *** SUMMARY A 63-year-old male presented with a prominent itching sensation and wholebody *** showed obstructive-pattern jaundice,an elevated IgG4 level,and infiltration of a large number of IgG4-positive cells in the ampulla of *** imaging findings of intrahepatic duct(IHD)and common bile duct dilation,an elevated serum IgG4 level,and characteristic histological findings led to diagnosis of IgG4-SC that compatible with the 2019 ACR/EULAR classification *** planned to treat the patient with high-dose glucocorticoid(GC),followed by cyclophosphamide pulse *** treatment with high-dose GC and an immunosuppressant,imaging studies showed that IHD dilatation had completely *** Prompt diagnosis and appropriate treatment of IgG4-SC are *** there is a risk of relapse of IgG4-SC,the GC dose should be gradually reduced,and a maintenance immunosuppressant should be given.

关键词： Immunoglobulin G4-related disease Immunoglobulin G4-related sclerosing cholangitis glucocorticoid Immunosuppressant Case report ACR/EULAR classification criteria

Full recovery from chronic headache and hypopituitarism caused by lymphocytic hypophysitis:A case report

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World Journal of Clinical Cases 2022年第3期10卷 1041-1049页

作者： Mao-Guang Yang Han-Qing Cai Si-Si Wang Lin Liu Chun-Mei Wang Department of Endocrinology The Second Hospital of Jilin UniversityChangchun 130000Jilin ProvinceChina Department of Radiology The First Hospital of Jilin UniversityChangchun 130000Jilin ProvinceChina

BACKGROUND Lymphocytic hypophysitis(LYH)is an important condition to consider in the differential diagnosis of patients with a pituitary *** main clinical manifestations of LYH include headache,symptoms related to sellar compression,hypopituitarism,diabetes insipidus and ***,which is a frequent complaint of patients with LYH,is thought to be related to the occupying effect of the pituitary mass and is rapidly resolved with a good outcome after timely and adequate glucocorticoid treatment or *** SUMMARY Here,we report a patient with LYH whose initial symptom was headache and whose pituitary function assessment showed the presence of secondary hypoadrenalism,central hypothyroidism and hypogonadotropic *** magnetic resonance imaging showed symmetrical enlargement of the pituitary gland with suprasellar extension in a dumbbell shape with significant homogeneous enhancement after gadolinium *** size of the gland was approximately 17.7 mm×14.3 mm×13.8 *** pituitary stalk was thickened without deviation,and there was an elevation of the optimal *** lesion grew bilaterally toward the cavernous sinuses,and the parasternal dural caudal sign was *** patient presented with repeatedly worsening and prolonged headaches three times even though the hypopituitarism had fully resolved after glucocorticoid treatment during this *** This rare headache regression suggests that patients with chronic headaches should also be alerted to the possibility of LYH.

关键词： Lymphocytic hypophysitis Magnetic resonance imaging Hypopituitarism Headache glucocorticoid Case report

Acute-on-chronic Liver Failure in a Patient with Candida Endophthalmitis:A Case Report

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Journal of Clinical and Translational Hepatology 2021年第3期9卷 447-451页

作者： Ying Cao Ying Fan Yanbin Wang Xiyao Liu Wen Xie Center of Liver Diseases Capital Medical University Affiliated to Beijing Ditan HospitalBeijingChina Department of Ophthalmology Capital Medical University Affiliated to Beijing Ditan HospitalBeijingChina

Acute-on-chronic liver failure(ACLF)is a risk factor for fungal *** fungal endophthalmitis is a serious,sight-threatening *** causes include immunocompromised state and intravenous drug use,permitting opportunistic pathogens to reach the eye through the blood *** report a case of Candida endophthalmitis in a 47 year-old woman who was admitted to our hospital with ACLF and poorly controlled *** addition,she was treated with glucocorticoids due to severe *** treatment for ACLF,the patient experienced fever with blurred vision in the left eye and was diagnosed with candidemia,endogenous Candida endophthalmitis in the left eye,and chorioretinitis in the right *** and topical antifungal treatment was administered based on the positive Candida albicans test in intraocular fluid using second-generation *** patient underwent vitrectomy in the left eye and *** was confirmed in vitreous ***-up visit,at 6 weeks after the operation,showed only light perception in the left eye and stable visual acuity in the right *** should be aware of endogenous fungal endophthalmitis in patients with ACLF,especially those with Candida infection,a history of glucocorticoid use,and diabetes.A dilated retinal examination should be performed by an ophthalmologist if ACLF patients develop fever and fungal infection.

关键词： Acute-on-chronic liver failure Candida endophthalmitis Candida albicans glucocorticoid Case report

Diffuse alveolar hemorrhage with histopathologic manifestations of pulmonary capillaritis:Three case reports

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World Journal of Clinical Cases 2020年第12期8卷 2662-2666页

作者： Jun Xie Ying-Yue Zhao Jing Liu Guang-Min Nong Department of Pediatrics the First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina

BACKGROUND Diffuse alveolar hemorrhage(DAH)is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury(mainly capillaries,including arteries and veins),causing pulmonary microcirculation blood to accumulate in the alveolar *** is classified by the histological absence or presence of pulmonary capillaritis(PC)and is rarely reported in the *** SUMMARY This is a report of three girls aged 6-11 years with DAH and *** patients had decreased hemoglobin and one had increased erythrocyte sedimentation ***-resolution computed tomography showed bilateral diffuse pulmonary infiltrate,and diagnosis of PC was confirmed by lung *** test in one case showed granular IgG and a small amount of granular IgA deposit on the alveolar walls,and was negative in the other two cases,describing isolated pauci-immune *** was with glucocorticoid alone or combination with immunosuppressants,and the symptoms resolved in all *** PC is classified as isolated and immune-mediated PC associated with systemic *** can be controlled in most children with glucocorticoid alone or combined with immunosuppressants.

关键词： Diffuse alveolar hemorrhage Pulmonary capillaritis glucocorticoid Immunosuppressant Lung biopsy Case report

High glucose augments stress-induced apoptosis in endothelial cells.

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Journal of Geriatric Cardiology 2009年第2期6卷 102-107页

作者： Wenwen Zhong Yang Liu Hui Tian Department of Geriatric Endocrinology Chinese PLA General Hospital Beijing 100853 China

Hyperglycemia has been identified as one of the important factors involved in the microvascular complications of diabetes, and has been related to increased cardiovascular mortality. Endothelial damage and dysfunction result from diabetes; therefore, the aim of this study was to determine the response of endothelial cells to stressful stimuli, modelled in normal and high glucose concentrations in vitro. EAhy 926 endothelial ceils were cultured in 5 mmol/L or 30 mmol/L glucose conditions for a 24 hour period and oxidative stress was induced by exposure to hydrogen peroxide （HzO2） or tumour necrosis factor- α （TNF- α ）, following which the protective effect of the glucocorticoid dexamethasone was assessed. Apoptosis, necrosis and cell viability were determined using an ELISA for DNA fragmentation, an enzymatic lactate dehydrogenase assay and an MTT assay, respectively. High glucose significantly increased the susceptibility of EAhy 926 cells to apoptosis in the presence of 500 gmol/L H2O2 , above that induced in normal glucose （P〈0.02）. A reduction of H2O2- and TNF- a -induced apoptosis occurred in both high and low glucose after treatment with dexamethasone （P〈0.05）. Conclusion high glucose is effective in significantly augmenting stress caused by H2O2, but not in causing stress alone. These findings suggest a mechanism by which short term hyperglycemia may facilitate and augment endothelial damage.

关键词： Hyperglycemia endothelial cells oxidative stress glucocorticoid

Clinical observations of acute onset of myopic optic neuropathy in a real-world setting

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International Journal of Ophthalmology(English edition) 2021年第3期14卷 461-467页

作者： Li Liao Rui Fang Fang Fang Xiao-Hua Zhu Department of Ophthalmology the Second Xiangya HospitalCentral South UniversityChangsha 410011Hunan ProvinceChina Changsha Aier Eye Hospital Changsha 410015Hunan ProvinceChina Department of Orthopedics the Second Xiangya HospitalCentral South UniversityChangsha 410011Hunan ProvinceChina

AIM: To describe the clinical features of acute myopic onset of optic neuropathy and observe the effects of retrobulbar and systemic glucocorticoid therapy in a realworld ***: A retrospective observational case series included 18 patients with a clinical diagnosis of acute onset of myopic optic neuropathy in a real-world setting. While the patients were using retrobulbar and systemic glucocorticoid therapy, various imaging examination data were analysed, and the clinical features of myopic optic neuropathy were summarized for 6 mo to 2 y. RESULTS: The included group of patients with acute onset of myopic optic neuropathy consisted mostly of females(n=11). The visual field(VF) showed abnormalities in bilateral eyes, including the spread of physiological blind spots, central and paracentral dark spots, and centripetal peripheral VF reduction;but central vision with no subjective changes. The visual evoked potential(VEP) was abnormal in all eyes with vision loss. The best corrected visual acuity(BCVA) was improved from 1.04±0.63 to 0.47±0.57(log MAR) af ter glucocor ticoid treatment(P0.05). There was no recurrence during long-term followup ***: The acute onset of myopic optic neuropathy is characterized by BCVA and VF abnormalities in bilateral eyes. Retrobulbar and systemic glucocorticoid therapy is effective.

关键词： acute onset of myopic optic neuropathy glucocorticoid visual field

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World Journal of Clinical Cases 2023年第7期11卷 1656-1665页

作者： Pei-Hua He Li-Chang Liu Xing-Fu Zhou Jun-Jie Xu Wei-Hong Hong Li-Chun Wang Su-Jun Liu Jia-Hao Zeng The Second Clinical Medical College Guangzhou University of Chinese MedicineGuangzhou 510006Guangdong ProvinceChina Department of Nephrology Zhuhai Hospital of Guangdong Provincial Hospital of Chinese MedicineZhuhai 519015Guangdong ProvinceChina

BACKGROUND IgG4-related disease(IgG4-RD)is a chronic fibrotic disease mediated by immunity recognized by clinicians in recent *** the kidney is involved,it is called IgG4-related kidney disease(IgG4-RKD).IgG4-related tubulointerstitial nephritis(IgG4-TIN)is a representative manifestation of ***4-TIN can cause obstructive nephropathy complicated by retroperitoneal fibrosis(RPF).Cases of IgG4-TIN complicated with RPF are *** are the firstline therapeutic medication for IgG4-RD and can significantly improve renal *** SUMMARY Herein,we report the case of a 56-year-old man with IgG4-RKD complicated with *** patient presented to the hospital with complaints of elevated serum creatinine(Cr),nausea,and *** hospitalization,Cr was 1448.6μmol/L,and serum IgG4 was increased.A total abdominal computed tomography(CT)scan and enhanced CT scan obviously indicated *** this patient had a long course and renal insufficiency,we performed a kidney *** biopsy showed that the renal tubulointerstitium had focal plasma cell infiltration and increased lymphocyte infiltration accompanied by *** combining the biopsy results with immunohistochemistry,it was found that the absolute number of positive IgG4+cells per high power field exceeded 10,and the ratio of IgG4/IgG was over 40%.Finally,the patient was diagnosed with IgG4-TIN complicated with RPF and given glucocorticoids as long-term maintenance therapy,helping him keep out of *** a follow-up of 19 mo,the patient had recovered *** literature on IgG4-RKD and RPF was retrieved from PubMed to characterize the clinical and pathological features and to identify the diagnosis and treatment of *** Our case report demonstrates the clinical characteristics of IgG4-RKD complicated with *** IgG4 is a favorable indicator for *** renal biopsy actively plays a vital role in diagnosis and treatment,even if the

关键词： IgG4-related kidney disease Retroperitoneal fibrosis glucocorticoid Case report