Advance in application of rituximab in refractory minimal change disease / 中国医师杂志

Minimally degenerative nephropathy is one of the common types of primary nephrotic syndrome, and it is currently believed that B lymphocytes are closely related to its pathogenesis. Patients with refractory small degenerative kidney disease require treatment with glucocorticoids combined with immunosuppressant. Rituximab is a monoclonal antibody that consumes B cells. Its use in the treatment of patients with refractory microdegenerative kidney disease can reduce recurrence rate, prolong remission period, and reduce hormone exposure. However, there is no consensus on the treatment plan and adverse reaction response measures, and multicenter, prospective, and large-scale research answers are still needed. This article summarizes the latest progress of rituximab in the treatment of refractory minimal degenerative kidney disease, hoping to provide assistance for the development of clinical treatment strategies.

Síndrome nefrótico por enfermedad de cambios mínimos asociado a enfermedad de Graves y uso de metimazol

Introduction: Graves' disease causes kidney injury through a series of multiple mechanisms, including the treatment for this condition. Nephrotic syndrome due to minimal change disease (MCD) is an unusual form of such kidney injury; the association between methimazole use and MCD is also rare. Case presentation: A 36-year-old woman with a history of Graves' disease in use of methimazole for several months, who presents edematous syndrome due to nephrotic syndrome associated with a KDIGO stage 3 acute kidney injury. Thionamide-induced hypothyroidism and the need of thyroid hormone replacement therapy was evidenced at the time of consultation. Based on a renal biopsy, the patient was diagnosed with MCD, her condition worsened as she experienced oliguria and hypervolemia, therefore, renal replacement therapy with hemodialysis was temporarily required. Methimazole administration was suspended, and treatment consisting of prednisolone administration and levothyroxine supplementation was started, achieving hemodialysis suspension, gradual improvement of proteinuria until remission and a full-maintained recovery of renal clearance. Radioiodine therapy was implemented as definitive treatment for Graves' disease, obtaining a successful outcome. Conclusions: Graves' disease and methimazole use are possible causes of minimal change disease; systemic corticosteroid therapy is a possible management. However, further basic, clinical and epidemiological research on this subject is required.

Introducción: La enfermedad de Graves genera daño renal por múltiples mecanismos, incluyendo sus tratamientos. El síndrome nefrótico por enfermedad de cambios mínimos, es una forma inusual de dicho daño renal en la enfermedad de Graves; la asociación de esta condición renal con el metimazol también es anómalo. Presentación del caso: Una mujer de 36 años con antecedente de enfermedad de Graves en uso de metimazol hacía meses, quien debutó con síndrome edematoso por síndrome nefrótico asociado a una lesión renal aguda KDIGO etapa 3. Se evidenció hipotiroidismo inducido por la tionamida con necesidad de suplencia al momento de la consulta. Asimismo, se realizó biopsia renal que concluyó en enfermedad de cambios mínimos. La paciente progresó a oliguria con hipervolemia sin respuesta a diurético del asa por lo que requirió terapia de reemplazo renal con hemodiálisis de forma transitoria. Se retiró el metimazol, se dio manejo con prednisolona y con suplencia tiroidea, lográndose el retiro de la hemodiálisis con mejoría gradual de la proteinuria hasta la remisión y recuperación de la depuración renal de forma plena y mantenida. Se dio manejo definitivo a la enfermedad de Graves con yodo radioactivo con éxito terapéutico. Discusión y conclusiones: La enfermedad de Graves y el metimazol son causas posibles de enfermedad de cambios mínimos; el tratamiento con corticoide sistémico se postula como una posible estrategia de manejo. Se requiere más investigación básica, clínica y epidemiológica en el tema.

Acute Myeloid Leukemia in a Geriatric Patient with a History of Minimal Change Disease

Background: The increase in the incidence of malignancies globally, and the increase in the usage frequency and types of new anti-cancer drugs, have made onconephrology more important in our clinical practice. Paraneoplastic glomerulonephritis constitutes an important part of this approach as well. Purpose: The association of AML-nephrotic syndrome is relatively less defined in the literature compared to other hematological malignancies. Case presentation: In this article, we present a case of acute myelocytic leukemia in a patient who was diagnosed with minimal change disease many years ago. Discussion and Conclusion: Hematological malignancies-MCD association, is one of the best described examples of paraneoplastic glomerulonephritis. We know that cancer can be clinically diagnosed years after the detection of renal disease in paraneoplastic glomerulonephritis. In this case; rationality of follow-up, not only during the diagnosis of glomerulonephritis but also periodically in the long term, especially in clinical situations such as MCD that occur in geriatric patients, should be discussed.

Introducción: El aumento en la incidencia de neoplasias malignas a nivel mundial, y el aumento en la frecuencia de uso y tipos de nuevos medicamentos contra el cáncer, han hecho que la onconefrología sea más importante en nuestra práctica clínica. Asimismo, la glomerulonefritis paraneoplásica también constituye una parte importante de este enfoque. Propósito: La asociación de LMA-síndrome nefrótico está relativamente menos definida en la literatura a comparación de otras neoplasias malignas hematológicas. Presentación del caso: En este artículo presentamos un caso de leucemia mielocítica aguda en un paciente al que se le diagnosticó enfermedad de cambios mínimos hace años. Discusión y Conclusión: La asociación de neoplasias hematológicas malignas-MCD, es uno de los ejemplos mejor descritos de glomerulonefritis paraneoplásica. Sabemos que el cáncer puede diagnosticarse clínicamente años después de la detección de la enfermedad renal en la glomerulonefritis paraneoplásica. En este caso, debe discutirse la racionalidad del seguimiento, no solo durante el diagnóstico de glomerulonefritis, sino también periódicamente a largo plazo especialmente en situaciones clínicas como la ECM que se presenta en pacientes geriátricos.

Hiperinfección por Strongyloides stercolaris de aparición temprana después del tratamiento corticosteroideo de un síndrome nefrótico / Hyperinfection on Strongyloides stercolaris of early appearance after corticosteroid treatment of a nephrotic syndrome

Strongyloides stercolaris es un parásito endémico de áreas tropicales que infecta a su huésped a través de la penetración transcutánea de sus larvas filariformes. Generalmente, la infección por S. stercolaris transcurre de forma asintomática; pero en un porcentaje de pacientes inmunodeprimidos puede acontecer el síndrome de hiperinfección con diversas manifestaciones. La asociación de S. stercolaris con la afectación glomerular es inusual. Se reporta el caso de una paciente de 42 años, que acudió a Urgencias por disnea y edemas. Los síntomas y hallazgos en los exámenes de laboratorio fueron compatibles con síndrome nefrótico. La paciente ingresó en Nefrología para recibir tratamiento corticoesteroideo intravenoso, así como la realización de una biopsia renal. Tras 10 días de tratamiento presentó mejoría clínica y se dio el alta a la espera de los resultados anatomopatológicos. Diez días más tarde acudió nuevamente por dolor abdominal intenso, deposiciones diarreicas y persistencia de la disnea. La paciente se diagnosticó con síndrome de hiperinfección por S. stercolaris secundario al tratamiento inmunosupresor recibido. Los síntomas se resolvieron con ivermectina y albendazol. Los resultados de la biopsia revelaron hallazgos compatibles con glomerulonefritis por cambios mínimos secundarios a la infección por S. stercolaris. Si bien la glomerulopatía suele manifestarse entre el primer y segundo mes de tratamiento inmunosupresor, en el presente trabajo se observó una aparición precoz de los síntomas respecto al resto de casos, que se manifestó en el día 21 de tratamiento corticoesteroideo(AU)

Strongyloides stercolaris is an endemic threadworm from tropical areas that infects its host through transcutaneous penetration of its filariform larvae. Generally, S. stercolaris infection is asymptomatic. However, in a percentage of immunosuppressed patients, the hyperinfection syndrome may occur, presenting constitutional symptoms, gastrointestinal, pulmonary and, sometimes, central nervous system symptoms. The association between S. stercolaris and glomerular damage is unusual. We report the case of a 42-year-old Bolivian patient, living in Spain, that came to the emergency room due to edema in lower limbs and dyspnea. Symptoms and laboratory test results were compatible with nephrotic syndrome, so she was admitted to Nephrology to receive treatment with intravenous corticosteroids, as well as a renal biopsy. After 10 days of treatment, she presented clinical improvement, so she was discharged waiting the anatomopathological results. Ten days later, she was readmitted due to severe abdominal pain, diarrhea, and persistent dyspnea. The patient was diagnosed with hyperinfection syndrome due to S. stercolaris secondary to the immunosuppressive treatment received. Symptoms resolved with ivermectin and albendazole. Biopsy results revealed findings consistent with minimal change disease secondary to S. stercolaris infection. Although glomerulopathy usually appears between the first and second month after immunosuppressive treatment, in the present study, an early appearance of symptoms was observed compared to the rest of the cases, appearing on day 21 of corticosteroid treatment(AU)

Enfermedad de cambios mínimos como glomerulopatía paraneoplásica en un paciente con tumor sólido / Minimal Change Disease as a paraneoplastic glomerulopathy in a patient with solid tumor

Resumen La enfermedad de cambios mínimos (ECM) es la principal causa de síndrome nefrótico en niños y una causa poco común en adultos, asociada usualmente a causas secundarias, predominantemente de origen hematológico. Es poco frecuente que este tipo de glomerulopatía se diagnostique de manera sincrónica en pacientes con tumores sólidos. Presentamos el caso de un paciente de 35 años con diagnostico reciente de cáncer de recto quien ingresa con un cuadro compatible con síndrome nefrótico, a quien se le diagnostica, mediante una biopsia renal, una enfermedad de cambios mínimos, con mejoría parcial del cuadro posterior al manejo quirúrgico de la patología neoplásica. Consideramos que este caso puede ser útil en el enfoque diagnóstico y el manejo de pacientes que presenten tumores sólidos de origen gastrointestinal con síndrome nefrótico concomitante.

Abstract Minimal change disease is the main cause of nephrotic syndrome in children and a rare cause in adults, usually associated with secondary causes, predominantly hematological. This type of glomerulopathy is rarely diagnosed synchronously in patients with solid tumors. We present the case of a 35-year-old patient with a recent diagnosis of rectal cancer who is admitted with a condition compatible with nephrotic syndrome, diagnosed with minimal change disease in the renal biopsy, and with partial improvement in the condition after surgical management of neoplastic pathology. We consider that this case may be useful in the diagnostic approach and the management of patients with solid tumors of gastrointestinal origin with concomitant nephrotic syndrome.

Síndrome nefrótico congénito debutando con enfermedad de cambios mínimos / Congenital nephrotic syndrome debuting with disease of minimal changes

Resumen El síndrome nefrótico es la glomerulopatía primaria más frecuente en pediatría; es una patología que se caracteriza por la presencia de proteinuria, hipoalbuminemia, edema e hipercolesterolemia. Se habla de un neonato con antecedentes de prematuridad y hospitalización al nacer, posterior a múltiples infecciones recurrentes con evolución tórpida durante estancia hospitalaria y edema generalizado, de quien se sospecha tener síndrome nefrótico congénito, apoyándose en resultados paraclínicos positivos y una biopsia renal que reporte enfermedad de cambios mínimos. El síndrome nefrótico congénito es una entidad poco frecuente; sin embargo, presenta alta morbimortalidad, así como diversas formas de presentación clínica e histológica, y su manejo suele ser difícil dada la baja respuesta a corticoides. La presencia del síndrome nefrótico congénito es inusual, no obstante, presenta alta mortalidad y deja muchas secuelas, siendo la enfermedad renal crónica la más temida. Así que resulta importante sospechar en recién nacidos con infecciones recurrentes asociadas a presencia de edema generalizado para iniciar un manejo precoz que pueda ayudar a evitar consecuencias y mejorar la calidad de vida del paciente y su familia.

Abstract Nephrotic syndrome is the most common primary glomerulopathy in pediatrics; it is a pathology characterized by the presence of proteinuria, hypoalbuminemia, edema, and hypercholesterolemia. There is talk of a neonate with a history of prematurity and hospitalization at birth, after multiple infections recurrent with torpid evolution during hospital stay and edema generalized, suspected of having congenital nephrotic syndrome, relying on positive paraclinical results and a renal biopsy that reports minimal change disease. Congenital nephrotic syndrome is a rare entity; However, presents high morbidity and mortality, as well as various forms of clinical and histological, and its management is usually difficult given the low response to corticosteroids. The presence of congenital nephrotic syndrome is unusual; however, it presents high mortality and leaves many sequelae, with chronic kidney disease being the most feared. So, it is important to suspect in newborns with infections recurrent associated with the presence of generalized edema to initiate management early that can help prevent consequences and improve the quality of life of the patient and his family. .

Enfermedad de cambios mínimos como manifestación inicial de COVID-19 en paciente latinoamericano con trasplante renal / Minimal change disease as an initial clinical feature of COVID-19 infection in a Latin-American kidney transplant patient

RESUMEN COVID-19 tiene un espectro amplio de manifestaciones clínicas, se ha descrito lesión renal aguda con evidencia de necrosis tubular aguda y diversos tipos de podocitopatías. Se presenta el caso clínico de un paciente con trasplante renal y COVID-19 que debuta con lesión renal aguda y síndrome nefrótico. Proteína C-reactiva para síndrome respiratorio agudo severo 2 en hisopado nasal es positiva y en tejido renal es negativa. Biopsia renal reporta enfermedad de cambios mínimos. A la fecha es el primer reporte de esta podocitopatía asociada a COVID-19 en paciente con trasplante renal.

ABSTRACT COVID-19 disease is a systemic infection associated with renal damage leading to acute kidney injury with acute tubular necrosis and several types of podocytopathies. We report a clinical case in a kidney transplant patient diagnosed with COVID-19 disease, acute kidney injury and nephrotic syndrome. SARS-CoV 2 PCR results in nasal swab was positive and kidney was negative. Renal histopathologic findings described minimal change disease. So far, this case is the first clinical report of minimal change disease associated to COVID-19 in a kidney transplant patient.

Complications and renal biopsy profile in childhood steroid resistant nephrotic syndrome

Background: Nephrotic syndrome is a notable chronic disease in children. The objective of this study was to study the complications and renal biopsy profile in childhood steroid resistant nephrotic syndrome.Methods: Retrospective observation study done in Sri Ramachandra Medical College and Hospital, Department of Paediatrics, Chennai. Inclusion criteria was children aged 1-12 years diagnosed with steroid resistant nephrotic syndrome defined as absence of remission despite therapy with daily prednisolone at a dose of 2mg/kg/day for 4 weeks. Remission defined as urine albumin nil/trace in 3 consecutive early morning samples. Children less than 1 year of age, children with renal transplant and incomplete records were excluded. Period of study January 2013- December 2015. Informed consent was obtained and 75 cases who fulfilled the study criteria were included in this study. Variables assessed were incidence of hypertension (both at onset of disease and later during the course of disease), incidence of urinary tract infection and its microbiology, associated co-morbidities, complications of nephrotic syndrome and renal biopsy profile.Results: Incidence of hypertension at onset of disease was 13.3% and later during the course of the disease was 48%. Most common infection was UTI (28%) and the most common organism isolated in urine culture was E-coli. Incidence of other co-morbidities like asthma, atopy was 17.3%. No case had evidence of end stage renal disease. 60% of cases had undergone renal biopsy and minimal change disease was the most common biopsy finding.Conclusions: Hypertension and UTI remain important complications in nephrotic syndrome and hence all such children should be continued to be monitored for these complications. Minimal change disease (MCD) was the most common renal biopsy finding.

Renal biopsy in children with nephrotic syndrome: a study of histopathological pattern

Background: Idiopathic nephrotic syndrome (INS) is a common childhood renal disease characterized by a remitting and relapsing course, associated with different histopathological subtypes. The true incidence of various histopathological subtypes of NS remains under estimated owing to the diversity in indication criteria for performing renal biopsies in pediatric population.Methods: This was a cross-sectional observational study in children with nephrotic syndrome at a tertiary health care centre. Total 22 children, with nephrotic syndrome, who underwent renal biopsy procedure during a period of one year, were enrolled for the study. Indications of renal biopsy were noted, and the histopathology reports were studied in detail.Results: In this study group, the most common indication for renal biopsy was 'Atypical age (> 8years) of diagnosis in 45.5% (10/22) patients, followed by 22.7% (5/22) in 'Children presenting with hypertension and hematuria'.The most common histopathological finding in these children was mesangial proliferative glomerulonephritis in 45.5% (10/22) patients followed by IgA nephropathy with mesangial proliferation in 22.72% (5/22) and minimal change disease in only 13.6% (3/22).Conclusions: This study highlights the occurrence of non-MCD as the common cause of INS in the children and denotes the significance of performing renal biopsies in children with INS for better prognostication.

Circulating Permeability Factors in Idiopathic Nephrotic Syndrome

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

Correlation between nephropathy score and hormone threapy in minimal change disease / 临床与实验病理学杂志

Purpose To observe the clinical characteristics, expression of C4d and the morphology of podocyte lesions in steroid-sensitive minimal change disease (SS-MCD) ,steroidresistant minimal change disease (SR-MCD) and early focal segmental glomerulosclerosis (E-FSGS) ,as well as to analyze their differences among the three groups,and provide a novel method for effective evaluation the therapeutic effects of steroid and diagnosis of SR-MCD. Methods To study the clinical data from 24 cases of SS-MCD,30 cases of SR-MCD and 25 cases of E-FSGS as control,and all the biopsies were examined by light microscopy,immunohistochemistry and transmission electron microscopy. Meanwhile,the clinical characteristics,the morphology of podocyte lesion and the expression of C4d were observed. Results The average score of podocyte lesion of SR-MCD was higher than that of SS-MCD,but lower than that of E-FSGS (P< 0. 05) . C4d positive average score of SS-MCD was lower than that of both SR-MCD and E-FSGS (P < 0. 05) ,but there was no significant difference between SR-MCD and E-FSGS (P > 0. 05) . The sum of the average score of podocyte lesion and C4d positive average score of SS-MCD was lower than that of SRMCD and E-FSGS (P < 0. 01) ,however,there was also no significant difference between SR-MCD and E-FSGS(P > 0. 05) . The scores of IgM,C3d and C1q were not significantly different among the three groups. The area under the receiver operating curve (ROC) of the C4d positive score,podocyte lesion score and the sum of the two were 0. 753,0. 658 and 0. 803,respectively, and there was no significant difference between them and the optimal cutoff values were 3,1. 5,and 4. 5 points,respectively. Conclusions The C4d positive score,podocyte lesion score and the sum of the two scores of MCD (the last one is named for MCD nephropathy score in our study) can be used for evaluating the therapeutic effects of steroid and identification of SR-MCD,most especially MCD nephropathy score. The optimal cut-off values of the three kinds of scores are 3,1. 5,and 4. 5 points,respectively. When the values are exceeded,the clinicians should be reminded to follow-up and take appropriate treatment measures to patients.

Primary Focal Segmental Glomerulossclerosis and Minimal Change Disease as one Spectrum of Disease

@#Minimal Change Disease (MCD) and Focal Segmental Glomerulosclerosis (FSGS) are common causes of nephrotic syndrome. These two conditions are similar in their presentations but differentiated via their histopathological features and responsiveness to corticosteroids. There are ongoing debates whether MCD and FSGS are at the same spectrum of disease rather than separate entities. FSGS has been postulated to be the severe end of the spectrum of MCD. We have reported a case that has primary FSGS after years of poorly controlled MCD, which supports both conditions are the same spectrum of disease.

Clinicopathological features and prognosis of minimal change nephropathy with IgA deposition in children / 中华实用儿科临床杂志

Objective To analyze the clinicopathological features and prognosis of minimal change nephropa-thy with IgA deposition(MCD-IgA)in children.Methods The clinical and pathological data of 10 cases in Chil-dren's Hospital of Nanjing Medical University from January 2010 to December 2015 with MCD-IgA were retrospective-ly analyzed,and 24 cases of minimal change nephrotic syndrome(MCD-NS)and 21 cases of IgA nephropathy clini-cally manifested with nephrotic syndrome(NS-IgAN)were selected as controls.Results (1)Clinical manifesta-tions:there were no significant differences in age,gender,incidence of hematuria,level of 24 hours urine protein,serum albumin and cholesterol levels and elevated serum IgA ratio in MCD-IgA compared with MCD-NS group.Compared with MCD-IgA and MCD-NS,NS -IgAN group showed older age of onset[(8.6 ± 2.1)years vs.(4.8 ± 2.4) years,(4.0 ± 1.6)years],higher level of serum albumin[(22.8 ± 4.3)g/L vs.(19.0 ± 1.9)g/L,(16.8 ± 3.0) g/L],and lower level of serum total cholesterol[(7.9 ± 1.9)mmol/L vs.(9.9 ± 2.7)mmol/L,(9.8 ± 2.1)mmol/L], and all the differences were significant(all P<0.05).NS-IgAN group was all associated with gross hematuria.(2) Pathology:the light microscope lesions in MCD-IgA and MCD-NS group were mild,but it was usually associated with severe histologic lesions in NS-IgAN,such as endocapillary proliferation,segmental sclerosis,crescent formation,tuft necrosis and chronic tubulointerstitial lesions;in MCD -NS group,immunofluorescence was negative. In MCD -IgA group,IgA deposition intensity was weak(less than + +),and 3 cases(30.0%)were accompanied with C3deposi-tion.In NS-IgAN group,IgA deposition intensity was stronger(more than + + +),and most of the cases were accom-panied with C3and other immunoglobulins deposition.Under electron microscope,both MCD-IgA and MCD-NS showed wide foot process effacement,and a small amount of mesangial electron dense deposit was detected in 9 cases of MCD-IgA.In NS-IgAN group,large amount of electron dense deposit was found in the mesangial region,and only 8 cases (38.0%)showed more than 50% of foot process effacement.(3)Prognosis:in MCD-IgA group,9 patients were ster-oid-dependent or frequently relapsed,1 case showed steroid-resistance,6 patients required additional agents.Except 1 case lost,with an average of(61.5 ± 28.8)months were followed up,8 patients achieved complete remission;In MCD-NS group,20 cases were steroid-dependent or frequently relapsed,4 cases were steroid-resistant,23 cases re-quired additional immunosuppressive agents.Followed up for an average of(36.4 ± 12.5)months,22 cases(91.7%) achieved complete remission;In NS-IgAN group,all cases were steroid-resistant and combined with cyclophospha-mide treatment;followed up for an average of(38.6 ± 15.2)months,19 cases(90.5%)achieved complete remission. Conclusions The clinical manifestations and prognosis of MCD-IgA were similar to MCD-NS,but the clinical and pathological findings of MCD-IgA were different from those of NS-IgAN.It is deduced that the nature of MCD-IgA is still a MCD,and that the IgA deposition may be nonspecific.

The association of WISP-1 expression in IgA nephropathy patients with renal interstitial fibrosis / 中华肾脏病杂志

Objective To investigate the relationship between the expression of Wnt induced secreted protein-1 (WISP-1) and the fibrosis of renal biopsy tissue in IgA nephropathy (IgAN) patients.Methods Fifty-three patients firstly diagnosed as IgA nephropathy by renal biopsy were included and classified according to Oxford and Lee's classification.Sixteen patients with MCD entered the fibrosis negative control group,and fourteen healthy adults entered the normal control group.The expression of WISP-1 in renal tissues and serum of all subjects were detected by immunohistochemistry and ELISA respectively.Results Immunohistochemistry results showed that WISP-1 was not expressed in MCD patients and normal human kidney tissues,which was abundantly deposited in renal tissue of patients with focal proliferative IgAN with renal interstitial fibrosis.The serum level of WISP-1 in IgAN patients was significantly higher than that in normal subjects (P=0.015) and MCD patients (P=0.030).In the subgroup analysis of IgAN renal fibrosis,the serum concentration of WISP-1 of fibrosis grade between 0-10％ (F1 group) and fibrosis ＞ 25％ (F3 group) were significantly higher than that in the normal group and the MCD group (all P ＜ 0.05).There was no significant difference between F2 group (10％ ＜ fibrosis≤25％) and normal group or MCD group (P ＞ 0.05).Conclusions The expression of WISP-1 in serum and renal tissue of renal interstitial fibrosis IgAN patients is higher than that of normal and MCD patients without renal fibrosis,and the IgAN patients' serum level of WISP-1 is significantly increased in fibrosis lower score group.The expressions of WISP-1 in serum and renal tissue are related to the occurrence of IgAN renal interstitial fibrosis,in which WISP-1 may play an important role as an early precursor factor in the pathogenesis of IgAN renal interstitial fibrosis.

The association of WISP-1 expression in IgA nephropathy patients with renal interstitial fibrosis / 中华肾脏病杂志

Objective To investigate the relationship between the expression of Wnt induced secreted protein-1 (WISP-1) and the fibrosis of renal biopsy tissue in IgA nephropathy (IgAN) patients.Methods Fifty-three patients firstly diagnosed as IgA nephropathy by renal biopsy were included and classified according to Oxford and Lee's classification.Sixteen patients with MCD entered the fibrosis negative control group,and fourteen healthy adults entered the normal control group.The expression of WISP-1 in renal tissues and serum of all subjects were detected by immunohistochemistry and ELISA respectively.Results Immunohistochemistry results showed that WISP-1 was not expressed in MCD patients and normal human kidney tissues,which was abundantly deposited in renal tissue of patients with focal proliferative IgAN with renal interstitial fibrosis.The serum level of WISP-1 in IgAN patients was significantly higher than that in normal subjects (P=0.015) and MCD patients (P=0.030).In the subgroup analysis of IgAN renal fibrosis,the serum concentration of WISP-1 of fibrosis grade between 0-10％ (F1 group) and fibrosis ＞ 25％ (F3 group) were significantly higher than that in the normal group and the MCD group (all P ＜ 0.05).There was no significant difference between F2 group (10％ ＜ fibrosis≤25％) and normal group or MCD group (P ＞ 0.05).Conclusions The expression of WISP-1 in serum and renal tissue of renal interstitial fibrosis IgAN patients is higher than that of normal and MCD patients without renal fibrosis,and the IgAN patients' serum level of WISP-1 is significantly increased in fibrosis lower score group.The expressions of WISP-1 in serum and renal tissue are related to the occurrence of IgAN renal interstitial fibrosis,in which WISP-1 may play an important role as an early precursor factor in the pathogenesis of IgAN renal interstitial fibrosis.

A Case of Steroid Resistant Minimal Change Disease Associated with Portal Vein Thrombosis Treated by Combined Immunosuppressive Agents / 고신대학교의과대학학술지

Minimal change disease (MCD) is a common cause of nephrotic syndrome and relatively well responds with steroid treatment. However, nearly half of patients with MCD experience recurrence of nephrotic syndrome. Thromboembolic events including renal vein thrombosis may occur in patients with MCD, but portal vein thrombosis rarely occurs. We experienced a case of frequent relapse/steroid dependent MCD with nephrotic syndrome progressed to steroid resistance associated with portal vein thrombosis. This patient showed complete remission of MCD and resolution of portal vein thrombosis after treatment with corticosteroid, cyclosporine, mycophenolate mofetil, and anticoagulant.

Children with Steroid-resistant Nephrotic Syndrome: Long-term Outcomes of Sequential Steroid Therapy / 生物医学与环境科学（英文）

<p><b>OBJECTIVE</b>This study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome (SRNS), who received methylprednisolone pulse therapy (MPT)-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events.</p><p><b>METHODS</b>We conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed.</p><p><b>RESULTS</b>Sixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy. Thirty-five (55.6%) achieved complete or partial remission, 19 (30.2%) of whom were in remission even after treatment cessation at last review. The mean time to initial remission after MPT was 24.3±13.1 days. Forty-nine children (77.8%) experienced relapses, of whom 31 (49.2%) demonstrated a frequent relapsing course. Adverse effects relevant to MPT were generally mild and infrequent. Five patients (7.9%) complained of vomiting or nausea during MPT infusion; 25 (39.7%) experienced excessive weight gain and developed an obvious Cushingoid appearance; and 26 (41.3%) had poor growth associated with long-term steroid use. Twenty-eight patients (44.4%) failed to respond to MPT, of whom 21 (33.3%) achieved complete or partial remission with immunosuppressive agents.</p><p><b>CONCLUSION</b>MPT-based sequential steroid therapy appears to be a safe and effective method for inducing rapid remission in childhood SRNS. Further clinical studies are needed to comprehensively evaluate this therapy.</p>

Pattern of Glomerular Disease in Patients with Nephrotic Syndrome- A Single Centre South Indian Study.

Background: The prevalence of primary and secondary glomerular diseases presenting as nephrotic syndrome (NS) varies according to the demography, renal biopsy practice and geographic location. To determine the morphological patterns of glomerular lesions in renal biopsies from patients manifesting with NS, in our local (South Indian) population. Methods: The study was conducted in a tertiary care hospital in South India between 2008 and 2014 on adults and children presenting with NS. Renal biopsies were performed in all patients and subjected to light microscopic and immunofluorescence studies. Results: A total of 264 cases of NS were identified of which 80.7% were adults and 19.3% were children. The mean age for the adults was 42.2 years with a male: female ratio of 1.6:1 and the mean age for children was 12.1 with a male: female ratio of 1.8:1. The most common cause of NS in adults was minimal change disease (MCD) (42.7%) followed by membranous nephropathy (MN) (24.4%) and focal segmental glomerulo-sclerosis (FSGS) (17.8%). In children MCD (88%) was the single most common cause of NS followed by FSGS (5.9%) and MN (3.9%). Compared to MCD and MN, a higher incidence of microscopic haematuria and renal insufficiency was present in FSGS. Conclusion: A wide range of primary and secondary glomerular disorders can present as NS, the prevalence of which varies according to the geographic area and demography. Though the frequencies of MCD, MN, and FSGS in our study were different from other studies conducted in India and other countries, these three glomerular disorders are the three major causes of NS in many geographic areas across the world.

Rifampicin-Induced Minimal Change Disease Is Improved after Cessation of Rifampicin without Steroid Therapy

There are several reports to demonstrate that rifampicin, a major anti-tuberculosis agent, is associated with some adverse renal effects, with a few cases of rifampicin-induced minimal change disease (MCD). In the present case, a 68-year-old female presented with nausea, vomiting, foamy urine, general weakness and edema. She had been taking rifampicin for 4 weeks due to pleural tuberculosis. The patient had no proteinuria before the anti-tuberculosis agents were started, but urine tests upon admission showed heavy proteinuria with a 24-h urinary protein of 9.2 g/day, and serum creatinine, albumin, and total cholesterol levels were 1.36 mg/dL, 2.40 g/dL, and 283 mg/dL, respectively. MCD was diagnosed, and the patient achieved complete remission after cessation of rifampicin without undergoing steroid therapy.

Reversible Acute Renal Failure in a Elderly Patient with Minimal Change Disease: A Case Report.

Introduction: Minimal change disease is a histopathological lesion of the kidneys most commonly associated with nephrotic syndrome. Three pathophysiological mechanisms have been proposed to explain this syndrome: Nephrosarca (severe edema of the kidney), presence of acute tubular necrosis and decreased of glomerular filtration rate. Presentation of Case: We present a 69years old patient with minimal change disease presented with severe renal dysfunction, dyspnoea, oedema of the lower extremities and weight gain of 10kg the month prior to admission. Renal function did not improve despite excess fluid removal with hemodialysis. Renal biopsy did not show significant interstitial Oedema but showed signs of tubular damage and mild atherosclerosis. Renal function returned with remission of proteinuria following administration of corticosteroid therapy. Conclusion: Our case does not support the nephrosarca hypothesis but the presence of acute tubular necrosis and decreased of glomerular filtration rate theories cannot be excluded.