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World Journal of Clinical Cases 2022年第13期10卷 4131-4136页

作者： Ting-Ting Dan Tai-Xiang Liu Yi-Lu Liao Zong-Ze Li Department of Ophthalmology The Affiliated Hospital of Zunyi Medical UniversityZunyi 563003Guizhou ProvinceChina

BACKGROUND Diffuse lamellar keratitis(DLK)is a complication of laser-assisted in situ keratomileusis(LASIK).This condition can also develop after small-incision lenticule extraction(SMILE)with a distinctive *** report the case involving a female patient with delayed onset DLK accompanied by immunoglobulin A(IgA)*** SUMMARY A 22-year-old woman was referred to our department for DLK and a decline in vision 1 mo after undergoing *** initial examination showed grade 2 DLK in the flap involving the central visual axis of the right *** was immediately administered with a large dose of a topical steroid for 30 ***,the treatment was *** vision deteriorated from 10/20 to 6/20,and DLK gradually worsened from grade 2 to ***,interface washout was performed,after which her vision *** completely disappeared 2 mo after *** months after SMILE,the patient was diagnosed with IgA nephropathy due to a 4-year history of interstitial *** DLK is a typical complication of LASIK but can also develop after *** steroid therapy was ineffective in our patient,and interface washout was *** nephropathy could be one of the factors contributing to the development of delayed DLK after SMILE.

关键词： Diffuse lamellar keratitis Small-incision lenticule extraction immunoglobulin a nephropathy Laser-assisted in situ keratomileusis Case report

Novel COL4A3 synonymous mutation causes Alport syndrome coexistent with immunoglobulin a nephropathy in a woman:A case report

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世界临床病例杂志 2023年第25期11卷 5947-5953页

作者： Yu-Ting Chen Wen-Ze Jiang Ke-Da Lu The First Clinical Medical College Zhejiang Chinese Medical UniversityHangzhou 310053Zhejiang ProvinceChina Department of Nephrology The Third Affiliated Hospital of Zhejiang Chinese Medical UniversityHangzhou 310005Zhejiang ProvinceChina

BACKGROUND Alport syndrome(AS)is an inherited disease of the glomerular basement membrane caused by mutations in genes encodingα3,α4,orα5 chains of type IV *** manifests with hematuria or proteinuria,which is often accompanied by hearing impairments and ocular ***,AS shows mesangial proliferation and sometimes incidental immunoglobulin A(IgA)*** or proteinuria is also a common presentation in patients with IgA nephropathy that makes it difficult to differentially diagnose AS and IgA nephropathy solely based on these clinical and pathological *** SUMMARY Herein,we present the case of a 59-year-old female patient who was admitted to our hospital with persistent microscopic hematuria and occasional proteinuria that had lasted for>2 *** patient had a familial history of renal disease and was diagnosed with autosomal dominant AS(ADAS)and IgA nephropathy based on the findings of renal biopsy as well as genetic testing performed using whole-exome sequencing,which suggested that the patient carried a novel heterozygous variation(c.888G>A:***296Gln)in the COL4A3 gene that enriches the mutation spectrum of *** proband received an angiotensin receptor blocker therapy after a definitive diagnosis was *** one year of therapy,a significant reduction in proteinuria was *** number of microscopic red blood cells per high-power field decreased to one-quarter of the baseline *** function also maintained well during the *** Our case highlights the significance of performing kidney biopsy and genetic testing in the diagnosis of AS and familial IgA nephropathy.

关键词： Alport syndrome immunoglobulin a nephropathy COL4A3 gene Whole-exome sequencing Renal biopsy Case report

Efficacy of Leflunomide, Telmisartan, and Clopidogrel for immunoglobulin a nephropathy： A Randomized Controlled Trial

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Chinese Medical Journal 2016年第16期129卷 1894-1903页

作者： Jie Wu Shu-Wei Duan Xue-Feng Sun Wen-Ge Li Ya-Ping Wang Wen-Hu Liu Jian-Rong Zhang Li-De Lun Xue-Mei Li Chun-Hua Zhou Ji-Jun Li Shu-Wen Liu Yuan-Sheng Xie Guang-Yan Cai Lu Ma Wen Huang Hua Wu Qiang Jia Xiang-Mei Chen Department of Nephrology Chinese People's Liberation Army General Hospital Chinese People's Liberation Army Institute of Nephrology State Key Laboratory of Kidney Diseases National Clinical Research Center for Kidney Diseases Beijing 100853 China Department of Nephrology China-Japan Friendship Hospital Beijing 100029 China Department of Nephrology PLA Army General Hospital Beijing 100700 China Department of Nephrology Beijing Friendship Hospital Capital Medical University Beijing 100050 China Department of Nephrology General Hospital of Chinese People's Armed Police Forces Beijing 100039 China Department of Nephrology Air Force General Hospital of People's Liberation Army Beijing 100142 China Department of Nephrology Peking Union Medical College Hospital Chinese Academy of Medical Sciences Beijing 100730 China Department of Nephrology Navy General Hospital Beijing 100048 China Department of Nephrology First Affiliated Hospital of General Hospital of People's Liberation Army Beijing 100048 China Treatment Center for Kidney Disease No. 281 Hospital of Beijing Military Region Qinhuangdao Hebei 066100 China Department of Nephrology Beijing Tongren Hospital Capital Medical University Beijing 100730 China Department of Nephrology Beijing Hospital Beijing 100730 China Department of Nephrology Xuanwu Hospital Capital Medical University Beijing 100053 China

Background： The efficacy and safety of telmisartan combined with clopidogrel, leflunomide, or both drugs for immunoglobulin a nephropathy （IgAN） are unclear. This study was designed to evaluate the efficacy and safety of telmisartan combined with clopidogrel, leflunomide, or both drugs for IgAN. Methods： It is a multicenter, prospective, double-dummy randomized controlled trial. Primary IgAN patients were recruited in 13 renal units across Beijing, China, from July 2010 to June 2012. After a 4-week telmisartan （80 mg/d） wash-in, 400 patients continuing on 80 mg/d telmisartan were randomly assigned to additionally receive placebo （Group A）, 50 mg/d clopidogrel （Group B）, 20 mg/d leflunomide （Group C）, or 50 mg/d clopidogrel and 20 mg/d leflunomide （Group D）. The 24-week intervention was completed by 360 patients. The primary endpoint was change in 24-h proteinuria at 24 weeks. A linear mixed-effect model was used to analyze the changes at 4, 12, and 24 weeks. Generalized estimating equations were used to evaluate changes in hematuria grade. This trial was registered at the Chinese Clinical Trial Registry. Results： The effects oftelmisartan combined with leflunomide on changes in proteinuria （0.36 [95% confidence interval （CI） 0.18 0.55] g/d, P 〈 0.001）, in serum uric acid （76.96 [95% CI 57.44-96.49] μmol/L, P 〈 0.001）, in serum creatinine （9.49 [95% CI 6.54-12.44]μmol/L, P 〈 0.001）, and in estimated glomerular filtration rate （-6.72 [95% CI-9.46 to -3.98] *** -1. 1.73 m -2, p 〈 0.001） were statistically significant, whereas they were not statistically significant on changes in systolic and diastolic blood pressure and weight （P 〉 0.05）.Telmisartan combined with clopidogrel had no statistical effect on any outcome, and there was no interaction between the interventions. No obvious adverse reactions were observed. Conclusions： Telmisartan combined with leflunomide, not clopidogrel, is safe and effective for decreasing proteinuria in certain IgAN patients.

关键词： Clopidogrel immunoglobulin a nephropathy Leflunomide Randomized Controlled Trial

Autoimmune hepatitis in a patient with immunoglobulin a nephropathy:A case report

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World Journal of Clinical Cases 2020年第17期8卷 3828-3834页

作者： You Hyun Jeon Da Woon Kim So Jeong Lee Young Joo Park Hyo Jin Kim Miyeun Han Il Young Kim Dong Won Lee Sang Heon Song Soo Bong Lee Eun Young Seong Department of Internal Medicine Pusan National University School of MedicineBusan 49241South Korea Department of Pathology Pusan National University HospitalBusan 49241South Korea Division of Gastroenterology Department of Internal MedicineBiomedical Research InstitutePusan National University HospitalBusan 49241South Korea Division of Nephrology Department of Internal MedicineBiomedical Research InstitutePusan National University HospitalBusan 49241South Korea Division of Nephrology Department of Internal MedicinePusan National University Yangsan HospitalYangsan 626-770South Korea

BACKGROUND immunoglobulin a nephropathy(IgAN)is the most commonly encountered glomerular disease in Asian *** has a broad clinical presentation,and it is frequently associated with other *** liver disease is well recognized as the leading cause of secondary ***,cases of IgAN associated with autoimmune hepatitis(AIH)have seldom been *** SUMMARY A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver *** weeks prior,she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with *** profiles were highly positive for antinuclear antibodies,and symptoms related to portal hypertension including ascites and peripheral edema were present.A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis *** immunosuppression with prednisolone and azathioprine,rapid deterioration of liver function led to end-stage liver *** a living-donor liver transplantation,liver function gradually improved,and she had maintained stable liver and kidney function at the six months *** Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with *** encountered an IgAN patient with concurrent progressive liver failure due to AIH.

关键词： immunoglobulin a nephropathy Secondary immunoglobulin a nephropathy Autoimmune hepatitis Liver transplantation Case report

immunoglobulin A glomerulonephropathy:A review

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World Journal of Clinical Cases 2024年第8期12卷 1388-1394页

作者： Mohamad El Labban Salim Surani Department of Internal Medicine Mayo Cliic Health SystemMankatoMN 56001United States Department of Medicine&Pharmacology Texas A&M UniversityCollege StationTX 77843United State

In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical *** comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and ***,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)*** is mostly found among the Asian *** presentation can be variable,from microscopic hematuria to a rapidly progressive *** 50%of patients present with single or recurring episodes of gross *** upper respiratory infection and tonsillitis often precede these *** 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine *** diagnosis relies on having a renal biopsy for pathology and immunofluorescence *** focus on risk stratification and management of *** provide a review of all the landmark studies to *** to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six *** approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease ***-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney *** experts consider proteinuria≥2 g/d to be very high *** with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.

关键词： immunoglobulin a nephropathy Glomerulonephritis Nephritic syndrome Angiotensin-converting enzyme inhibitor Angiotensin receptor blocker Systemic steroids Mycophenolate mofetil

Guben Tongluo Formula Protects LPS-induced Damage in Lamina Propria B Lymphocytes Through TLR4/MyD88/NF-κB Pathway

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Current Medical Science 2022年第5期42卷 991-999页

作者： Qing WU Wei MENG Jiao-jiao SHEN Jia-yuan BAI Luo-bing WANG Ting-yu LIANG Di HUANG Pei-cheng SHEN Department of Nephrology Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghai201203China Department of Clinical Laboratory Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghai201203China Department of Nursing Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghai201203China Department of Pathology Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese MedicineShanghai201203China Key Laboratory of Liver and Kidney Diseases Shanghai201203China Key Laboratory of Liver and Kidney Diseases(Shanghai University of Traditional Chinese Medicine) Ministry of EducationShanghai201203China Shanghai Key Laboratory of Traditional Chinese Clinical Medicine(20DZ2272200) Shanghai201203China

Objective The main pathological feature of immunoglobulin a nephropathy(IgAN),an autoimmune kidney disease,is the deposition of IgA immune complexes,accompanied by mesangial cell proliferation and elevated urine *** Guben Tongluo formula(GTF)is a traditional Chinese medicine prescription,which has predominant protective effects on ***,the therapeutic mechanism of the GTF in IgAN remains *** present study aimed to determine the effects of GTF in treating IgAN via regulating the TLR4/MyD88/NF-κB *** In the present study,lamina propria B lymphocytes were treated with different concentrations of lipopolysaccharide(LPS)(0,1,5,10 and 20 ng/mL).Flow cytometry was used to define positive CD86+CD19+***-8 assay was used to examine cell *** was used to induce TLR4 ***-PCR and Western blotting were used to determine gene *** It was found that the LPS dose-dependently increased the content of IgA and galactose-deficient IgA1(Gd-IgA),the levels of TLR4,Cosmc,MyD88 and phosphorylated(p)-NF-κB,and the ratio of CD86+CD19+and IgA-producing B ***,the TLR4 knockdown reversed the role of *** suggests that TLR4 mediates the effects of LPS on lamina propria B ***,the GTF could dose-dependently counteract the effects of LPS and TLR4 overexpression on lamina propria B lymphocytes through the TLR4/MyD88/NF-κB *** Collectively,these results demonstrate that the GTF can regulate the TLR4/MyD88/NF-κB pathway to treat IgAN model lamina propria B lymphocytes stimulated by LPS.

关键词： Guben Tongluo formula immunoglobulin a nephropathy lamina propria B lymphocytes TLR4/MyD88/NF-κB

Renal biopsy reports in nephritic syndrome:Update

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World Journal of Nephrology 2022年第2期11卷 73-85页

作者： Saeed Taheri Department of Medicine New Lahijan Scientific FoundationLahijan 44158-13166Iran

BACKGROUND Nephritic syndrome(NiS)is a major indicator of serious renal diseases necessitating kidney biopsies for histopathological evaluations,but due to the lack of comprehensive reviews in the literature,the current understanding of the syndrome and its significance is *** To collect all the evidence retrievable from the literature on the diagnoses made on the renal biopsies performed for NiS as the indication to the *** A literature search was conducted to find studies reporting final diagnoses on renal biopsies in NiS *** were pooled and analyzed with stratifications on age and ***-analyzes were performed using Stata *** Overall,26414 NiS patients from the total number of 96738 kidney biopsy diagnoses reported by 47 studies from 23 countries from all continents(except sub-Saharan Africa)were found and *** was the indication for renal biopsy in 21%of the patient populations across the reviewed *** A(IgA)nephropathy was the single most frequent diagnosis in these patients(approximately 38%)followed by lupus nephritis(approximately 8%)and Henoch Schönlein purpura(approximately 7%).IgA nephropathy was the most frequent diagnosis reported for the NiS patients from the East Asia,comprising half of all the cases,and least prevalent in South *** the age subgroups,adult(vs pediatric or elderly)patients were by far the most likely age group to be diagnosed with the IgA nephropathy.A myriad of such regional and age disparities have been found and *** As the indication for renal biopsy,NiS represents a very distinctive epidemiology of final renal disease diagnoses compared to the other major syndromes.

关键词： Renal biopsy Nephritic syndrome immunoglobulin a nephropathy Diagnosis Histopathology Epidemiology

immunoglobulin A vasculitis nephritis:Current understanding of pathogenesis and treatment

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World Journal of Nephrology 2023年第4期12卷 82-92页

作者： Michela Amatruda Nicolina Stefania Carucci Roberto Chimenz Giovanni Conti Pediatric Nephrology and Rheumatology Unit AOU G MartinoUniversity of MessinaMessina 98125Italy

The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal *** and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell *** knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.

关键词： immunoglobulin A vasculitis nephritis immunoglobulin A vasculitis Henoch-Schoenlein purpura immunoglobulin a nephropathy Vasculitis,glomerulonephritis