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Epidemiologic Features of the First Flare of Ne-phrotic syndrome in Children in Bamako Pedi-atric Hospitals

Open Journal of Pediatrics 2022年第2期12卷 389-395页

作者： Djeneba Konaté Lala Ndrayni Sidibé Amadou Touré Adama Fomba Abdoul Karim Doumbia Karamoko Sacko Aminata Doumbia Oumar Coulibaly Mohamed Elmouloud Cissé Pierre Togo Adama Dembélé Awa Konaré Belco Maiga Harber Balilé Fatoumata Léonie Françoise Diakité Fousseyni Traoré Hawa Gouro Diall Ibrahima Ahamadou Guédiouma Dembélé Abdoul Aziz Diakité Fatoumata Dicko Traoré Boubacar Togo Department of Pediatrics CHU Gabriel Touré Bamako Mali

Introduction: The most frequent glomerular pathology in children, nephrotic syndrome (NS) is a cosmopolitan affection. It is an immunological disease and certain environmental factors, in particular viral infections and allergens, influence its occurrence. The climatic and environmental differences coupled with the frequency of infectious pathologies signing the African particularity and the absence of data in Mali on this affection, motivated us to carry out this work. Objective: To study the epidemiological aspects of nephrotic syndrome in hospitalized children aged 0 - 15 years. Patients and Methods: This was a descriptive prospective study from January 1 to December 31, 2021 in the Pediatrics Department of the University Teaching Hospital (UTH) Gabriel Touré, anational pediatric reference service in Mali. Diagnostic confirmation was provided by biology. Results: In one year, 120 children were hospitalized for nephrotic syndrome with a sex ratio of 2.75. Male exclusivity was observed at the ages of one year, eight years and 14 years and a reversal at 13 years (sex-ratio = 0.33). The median age was 6 years for an average age of 6.4 years ± 2.4 years with extremes ranging from one year to 14 years. The first attack of nephrotic syndrome occurred outside the classic age (>10 years) in 16.67%. nephrotic syndrome is observed throughout the year with two peaks in summer (July to September) and winter (December to February). Many ethnic groups were affected by this condition with a high frequency among ethnic groups where endogamy is common. All patients were melanoderms. No cases of NS were recorded in Caucasian children from Northern Mali. The patients came from Bamako in 47.5% of cases. Two brothers from the same siblings had had their first attack of nephrotic syndrome the same year, ten months apart. Conclusion: nephrotic syndrome in pediatric hospitals of Bamako occurs at a median age of six years all along the year with a summer and winter peaks. It is more obser

关键词： Epidemiology nephrotic syndrome Child University Teaching Hospital Ga-briel Toure

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Coexistence of the Hypersecretion of Catecholamine,Adrenal Cortical Nodular Hyperplasia,and nephrotic-Range Proteinuria of Focal Segmental Scleronephrosis:Is It Fat-Induced Hypertension?

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International Journal of Clinical Medicine 2011年第3期2卷 206-211页

作者： Khanh vinh quoc Luong Lan Thi Hoang Nguyen Si van Nguyen Ninh T.Nguyen Vietnamese American Research Foundation WestminsterUSA Department of Pathology/Garden Grove Medical Center Garden GroveUSA Department of Surgery/UCI Medical Center OrangeUSA

Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncontrolled hypertension. Laboratory examination suggested hypersecretion of catecholamines by the left adrenal glands in both patients and primary aldosteronism in one of them. A computed tomography scan revealed small nodules on left adrenal gland in both patients. Patients underwent laparoscopic left adrenalectomy. After surgery, blood pressure was normalized and proteinuria was resolved. Most interestingly, prominent adipocytes infiltrated were detected in the adrenal cortex and were associated with the presence of lymphocytes, which suggested that adipocytes might have a role in the pathogenesis of these diseases in our patients.

关键词： Pheochromocytoma Aldosteronism Adipocyte nephrotic syndrome Focal Segmental Scleronephrosis Adrenal Cortical Hyperplasia

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Patterns of Primary Focal Segmental Glomerulosclerosis (FSGS) in Adults Treated in a Dakar Single Center: About 58 Cases

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Open Journal of Nephrology 2020年第4期10卷 265-273页

作者： A. Dieng M. A. Ba A. Sy M. Ndongo B. Ndiaye M. S. Diawara M. Faye Mo Faye B. Ba N. Keita S. Diagne C. Ouanekpone A. T. Lemrabott M. M. Cissé E. F. Ka A. Niang Department of Nephrology Dialysis and Kidney Transplant in Hospital Aristide Le Dantec Dakar Senegal Department of Nephrology and Dialysis in Regional Hospital Thies Senegal

Introduction: The evolution of primary FSGS is often marked by the occurrence of relapse and corticosteroid resistance and the therapeutic options are numerous and have limited effectiveness. The objective of our study was to assess our practice in this lesion. Patients and Methods: We carried out a retrospective study of patients treated for primary FSGS the period January 1, 2010 to September 30, 2018. The clinical pathological, therapeutic and evolutive characteristics were studied. Results: Fifty-eight patients were included in the study. The average age was 30.74 ± 11.35 years and the sex ratio (M/F) was 2.41. Edema was found in 86.2% and hypertension in 37.9%. The average creatinine was 20.17 ± 16.06 mg/l and the average GFR according to MDRD was 82.43 ± 69.06 ml/min/1.73 m2. The average albumin level was 15.11 ± 5.78 g/l and the 24-hour proteinuria was 7.8 ± 3.79 g/24 h. nephrotic syndrome was the main indication for renal biopsy in 84.48% and the classic form of FSGS was found in 90.9%. The average initial corticosteroid dose was 62.68 ± 10.04 mg/d and the average duration of regression was 11.78 ± 7.40 months. Forty-five patients (77.6%) were corticosensitive (27.6% complete remission and 50% partial remission). Corticosteroid resistance was observed in 19% and corticosteroid dependence in 11.1%. The proportion of relapse was 33.3% within an average of 15.4 ± 9.1 months. Cyclosporine was no longer prescribed as a second-line treatment in 8 patients. Infectious complications were more found in 19%. Two patients had progressed to ESRD and we noted 2 death cases. The male gender was correlated with the occurrence of a relapse. However, the impact of certain factors such as hypertension, proteinuria, hematuria and GFR level has not been demonstrated. Conclusion: The evolution of primary FSGS is unpredictable, often marked by relapses, hence the interest in identifying factors associated with th

关键词： FSGS nephrotic syndrome Corticosteroid Resistance

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Three-Month-Induction Therapy with Prednisone and Mycophenolate Followed by Maintenance Therapy with Mycophenolate Alone for 2 Years: An Effective and Safe Autoimmune Treatment for Triggering Factors Adults with Acute Non-Crescentic Nephritis Associated with Henoch-Schönlein Purpura

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Open Journal of Nephrology 2022年第1期12卷 154-161页

作者： Kamel El-Reshaid Shaikha Al-Bader John Madda Department of Medicine Faculty of Medicine Kuwait University Kuwait City Kuwait Department of Medicine Nephrology Unit Amiri Hospital Ministry of Health Kuwait City Kuwait Department of Histopathology Amiri Hospital Kuwait City Kuwait

Background: Henoch-Schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe nephrotic/nephritic flares associated with non-crescentic nephritis in adult patients. Patients and methods: The regimen consisted of an initial induction phase of 3-month Prednisone and Mycophenolate followed by a maintenance phase of Mycophenolate alone for 2 years. Results: They were satisfactory with complete remission in 5 of 7 patients and partial in 2. Creatinine clearance was normalized in patients with complete remission and remained stable in the partially-responsive ones. Conclusion: Our study has shown the short- and long-term safety and efficacy of such autoimmune regimen directed towards the autoimmune triggering factors in severe forms of non-crescentic HSP.

关键词： Acute Henoch-Schönlein Purpura Treatment nephrotic syndrome Nephritis Mycophenolate Mofetil

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Lenvatinib-induced multiorgan adverse events in Hurthle cell thyroid cancer: A case report

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World Journal of Clinical Oncology 2021年第4期12卷 272-281页

作者： Muhammad Imran Butt Abdulmohsen Mohammed Khalid Bakhsh Quaid Johar Nadri Department of Medicine King Faisal Specialist HospitalResearch CentreAl Faisal UniversityRiyadh 12713Saudi Arabia

BACKGROUND The management of metastatic progressive radioiodine-resistant differentiated thyroid cancer remains challenging for *** availability of tyrosine kinase inhibitors(TKIs),sorafenib and lenvatinib,within the last decade has expanded treatment options;however,these lead to significant adverse effects,which may curtail their *** SUMMARY We report the case of a 47-year-old female with Hurthle cell thyroid cancer who underwent total thyroidectomy followed by radioiodine *** followup,she developed noniodine-avid renal and pulmonary *** respect to her pre-existing diabetes,hypertension,and polycystic kidney disease,the tumor board decided against performing renal metastasectomy because of the risk of future renal decline requiring *** were treated using sorafenib,which provided stability followed by progression within a *** switched to lenvatinib,which led to disease ***,the patient experienced severe adverse effects,including cardiomyopathy,bicytopenia,renal impairment,and the rarely reported nephrotic *** metastasis is a rare manifestation of Hurthle cell thyroid cancer with only two reported cases in *** report the experience of our first case of renal metastasis and its treatment with *** case serves as a reminder of the adverse drug reactions associated with TKI *** We advocate close monitoring of patients’hematological and renal profiles as well as their cardiac status using an echocardiogram.

关键词： Hurthle cell thyroid cancer Lenvatinib nephrotic syndrome Vascular endothelial growth factor Protein kinase inhibitors Kidney neoplasms/secondary neoplasm Case report

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Effect of Astragalus-Angelica Mixture on Lipoprotein Lipase and Lecithin-Cholesterol Acyltransferase of nephrotic Rats

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Chinese Journal of Integrative Medicine 2000年第1期6卷 35-37页

作者：李宁军李惊子辛岗许远洪健美王海燕

Objective: To investigate the mechanism of Astragalus-Angelica Mixture (AAM) ’s effects on lipid metabolism disturbance in nephrotic ***: To examine the effects of AAM on serum albumin, lipid levels, and activities of lipoprotein lipase (LPL) and lecithin-cholesterol acyltransferase (LCAT), which are key enzymes for metabolism of lipid in immune-induced nephrotic hyperlipidemia rats and exogenous hyperlipidemia ***: In nephrotic rats, serum albumin was reduced, lipid increased significantly, LPL activity decreased markedly and the LCAT activity was relatively insufficient. Activities of LPL and LCAT increased obviously in AAM treated nephrotic rats. There were no change of activities of LPL and LCAT in exogenous hyperlipidemia rats and AAM showed no effect on the activities of these two ***: The effect of AAM on regulating lipid metabolism might be due to enhance the clearance of both triglyceride-rich and cholesterol-rich apoB containing lipoprotein by improving the activities of LPL and LCAT.

关键词： Astragalus-Angelica Mixture nephrotic syndrome lipoprotein lipase lecithin-cholesterol acyltransferase hyperlipidemia

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Reversible Chronic Interstitial Nephritis Induced by Tacrolimus —Tacrolimus Chronic Interstitial Nephritis

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Open Journal of Nephrology 2023年第1期13卷 1-5页

作者： Kamel El-Reshaid Shaikha Al-Bader Department of Medicine Faculty of Medicine Kuwait University Kuwait City Kuwait Department of Medicine Nephrology Unit Amiri Hospital Ministry of Health Kuwait City Kuwait

Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent arteriolar vasculopathy and irreversible chronic interstitial fibrosis associated with CNI nephrotoxicity. In this case report we describe a patient, with minimal change disease, that had developed chronic and progressive renal disease while receiving therapeutic dose of Tacrolimus. His serum creatinine had reached 537 umol/L and his nephrotic state worsened. Kidney biopsy showed chronic interstitial nephritis. Tacrolimus was discontinued and he was treated with 1 mg/kg prednisone in addition to Mycophenolate mofetil (MMF) 1 g twice daily. By the 2nd month;serum creatinine returned to normal and by the 3rd month serum albumin too. After 1 month of therapy;the dose of Prednisone was tapered down gradually till 5 mg daily by the end of 3rd month. Moreover, the dose of MMF was reduced to 500 mg X2 by the end of 3rd month. After 2 years of follow up;he remained stable and without relapse of NS or renal failure. In conclusion, reversible renal disease, due to chronic interstitial nephritis can be induced by CNI which is amenable to treatment with Prednisone and MMF.

关键词： Calcineurin Inhibitors Mycophenolate Mofetil Tacrolimus Interstitial Nephritis Minimal Change Disease nephrotic syndrome

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When neurological symptoms hides a nephrologic pathology and a cardiac abnormality

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Open Journal of Internal Medicine 2011年第1期1卷 4-5页

作者： C.Muller B.Faller F.Sellal F.Chantrel Nephrology department Civil HospitalColmarFrance Neurology department Civil HospitalColmarFrance

We bring report the case of a young woman who presents with neurological symptoms, and to which the practised investigations led to the discovery in chain of unexpected pathologies. These neurological disorders were revealing of the picture associating a nephrotic syndrome and a patent foramen ovale. This foramen was the major route of a rain of emboles ring peripheral favored by the nephrotic syndrome towards the cerebral hemispheres.

关键词： Edema Hypercoagulable State Patent Foramen Ovale nephrotic syndrome Stroke Venous Thrombosis

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Structural basis of highly specific interaction between Nephrin and MAGI1 in slit diaphragm assembly and signaling

Structural basis of highly specific interaction between Neph...

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中国生物化学与分子生物学会第十二届全国会员代表大会暨2018年全国学术会议

作者： Zhuangfeng Weng Yuan Shang Zeyang Ji Fei Ye Lin Lin Rongguang Zhang Jinwei Zhu State Key Laboratory of Molecular Biology CAS Center for Excellence in Molecular Cell Science Shanghai Institute of Biochemistry and Cell Biology Chinese Academy of Sciences University of Chinese Academy of Sciences Division of Life Science State Key Laboratory of Molecular Neuroscience Hong Kong University of Science and Technology

Slit diaphragm(SD) is a specialized adhesion junction between the opposing podocytes,establishing the final filtration barrier to prevent passage of proteins from the capillary lumen into the urinary *** is the key structural and signaling adhesion molecule prominently expressed in *** contains an evolutionally conserved atypical PDZ-binding motif(PBM),which has been reported to bind to a verity of proteins in *** mutations in the gene lacking the intact PBM have been associated with glomerular ***,the molecular basis of Nephrin-PBM-mediated protein complexes is still ***,we systematically investigated the interactions between Nephrin-PBM and PDZ domain-containing proteins in SD and showed that Nephrin-PBM specifically binds to MAGI1,but not *** complex structure of MAGI1-PDZ3/Nephrin-PBM reveals that the Gly at-3 position of Nephrin-PBM is the determining feature for MAGI1-PDZ3 recognition,which is in sharp contrast with the typical PDZ/PBM binding mode.A single mutation within MAGI2 results in a gain-of-function mutation for Nephrin-PBM ***,based on our structural analysis,we developed a highly efficient inhibitory peptide capable of specifically blocking the Nephrin/MAGI interaction,which may serve as a useful tool for investigating the functions of the interaction in *** study also provide possible explanations for the diseases caused by the mutations of NPHS1.

关键词： nephrin nephrotic syndrome glomerular filtration barrier cell adhesion

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Epidemiology,clinical presentation,treatment,and follow-up of mercury poisoning in a hospital in China:a retrospective analysis

Epidemiology,clinical presentation,treatment,and follow-up o...

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2018全国中毒救治首都论坛-暨第十届全国中毒及危重症救治学术研讨会

作者： Sun Yawei Long Jianhai PengXiaobo Bai Lili Liu Sushun Lin Guodong Zhang Chengcheng Li Jiawei Ding Lei QiuZewu Poisoning Treatment Department 307 Hospital of People's Liberation Army of China People's Liberation Army No.520 Hospital Department of General Surgery the SeconedXiangya HospitalCentral South University

*** are no reports of the incidence of mercury poisoning in a large population in *** study investigated the epidemiology,clinical manifestations,and treatment outcomes of Chinese patients with mercury *** for 288 mercury poisoning patients at our hospital were collected from July 2012 to September 2017,including sex,age,admission time,blood mercury content,24-h urinary protein levels,electromyography(EMG) findings,renal biopsy,and *** characteristics were evaluated by statistical and correlation *** poisoning due to occupational exposure was more common in males in the 20-to 50 year-old age group,while female patients aged 31-50 years were more likely to experience cosmetics-related *** poisoning due to inappropriate use of Chinese folk remedies(CFRs) was observed at all ages regardless of *** most common symptoms were neurological(50.3%),renal(16.4%),and respiratory(8.0%).Of the 136 patients without complications for whom follow-up data were available,100 patients showed normal serum mercury concentration upon reexamination range 6 to 12 months after *** the 13 patients with EMG-confirmed neurological injury,10 showed full recovery after 38.5 ±8.03 *** the 18 patients with nephrotic syndrome that had successful follow-up visits,15 had normal urine protein and serum albumin levels and renal function after 22.67±10.26 ***-up visits revealed that various complications of mercury poisoning(nephrotic syndrome and peripheral nerve injury) can be alleviated by sodium dimercaptosulfonate *** of skin-lightening cosmetic products,CFP surveillance,and prevention and control of occupational contact must be improved to decrease the incidence of mercury poisoning in China.

关键词： Mercury poisoning Epidemiology Electromyogram nephrotic syndrome Prognosis

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