Effect of octreotide on oxidative stress in the erythrocyte and kidney tissue in adriamycin-induced experimental nephrotic syndrome model / Efeito do octreotide no estresse oxidativo em eritrócitos e no tecido renal no modelo de síndrome nefrótica experimental induzida por adriamicina

Abstract Introduction: Nephrotic syndrome (NS) is one of the reasons of end-stage kidney disease, and elucidating the pathogenesis and offer new treatment options is important. Oxidative stress might trigger pathogenesis systemically or isolated in the kidneys. Octreotide (OCT) has beneficial antioxidant effects. We aimed to investigate the source of oxidative stress and the effect of OCT on experimental NS model. Methods: Twenty-four non-uremic Wistar albino rats were divided into 3 groups. Control group, 2 mL saline intramuscular (im); NS group, adriamycin 5 mg/kg intravenous (iv); NS treatment group, adriamycin 5 mg/kg (iv) and OCT 200 mcg/kg (im) were administered at baseline (Day 0). At the end of 21 days, creatinine and protein levels were measured in 24-hour urine samples. Erythrocyte and renal catalase (CAT) and thiobarbituric acid reactive substance (TBARS) were measured. Renal histology was also evaluated. Results: There was no significant difference among the 3 groups in terms of CAT and TBARS in erythrocytes. Renal CAT level was lowest in NS group, and significantly lower than the control group. In treatment group, CAT level significantly increased compared with NS group. In terms of renal histology, tubular and interstitial evaluations were similar in all groups. Glomerular score was significantly higher in NS group compared with control group and it was significantly decreased in treatment group compared to NS group. Conclusions: Oxidative stress in NS might be due to the decrease in antioxidant protection mechanism in kidney. Octreotide improves antioxidant levels and histology in renal tissue and might be a treatment option.

Resumo Introdução: Síndrome nefrótica (SN) é uma das causas de doença renal em estágio terminal. É importante elucidar a patogênese e oferecer novas opções de tratamento. Estresse oxidativo pode desencadear a patogênese sistemicamente ou isoladamente nos rins. O octreotide (OCT) tem efeitos antioxidantes benéficos. Nosso objetivo foi investigar a fonte de estresse oxidativo e efeito do OCT no modelo experimental de SN. Métodos: Dividimos 24 ratos albinos Wistar não urêmicos em 3 grupos. Grupo controle, 2 mL de solução salina intramuscular (im); grupo SN, adriamicina 5 mg/kg intravenosa (iv); grupo tratamento SN, adriamicina 5 mg/kg (iv) e OCT 200 mcg/kg (im) foram administrados no início do estudo (Dia 0). Aos 21 dias, mediram-se os níveis de creatinina e proteína em amostras de urina de 24 horas. Mediu-se a catalase (CAT) eritrocitária e renal e a substância reativa ao ácido tiobarbitúrico (TBARS). Avaliou-se também histologia renal. Resultados: Não houve diferença significativa entre os três grupos em termos de CAT e TBARS em eritrócitos. O nível de CAT renal foi menor no grupo SN e significativamente menor que no grupo controle. No grupo tratamento, o nível de CAT aumentou significativamente em comparação com o grupo SN. Quanto à histologia renal, as avaliações tubular e intersticial foram semelhantes em todos os grupos. O escore glomerular foi significativamente maior no grupo SN em comparação com o grupo controle e diminuiu significativamente no grupo de tratamento em comparação com o grupo SN. Conclusões: Estresse oxidativo na SN pode ser devido à diminuição do mecanismo de proteção antioxidante nos rins. O octreotide melhora níveis de antioxidantes e histologia do tecido renal e pode ser uma opção de tratamento.

Atypical evolution of collapsing FSGS in extreme elderly: case report / Evolução atípica de GESF Colapsante na velhice extrema: relato de caso

Introduction: Segmental and Focal Glomerulosclerosis (FSGS) is an entity characterized by glomerular damage involving the podocyte as the main target. A variant with a worse prognosis, poor response to corticosteroid therapy, and rapid progression to end-stage renal disease is recognized as collapsing FSGS. Case Report: A 102-year-old man with nephrotic syndrome and acute kidney injury underwent renal biopsy, revealing collapsing FSGS. He achieved an excellent response to immunosuppressive treatment, presenting renal recovery, and was discharged from dialysis treatment. Discussion: Diagnosis and definition of treatment in the elderly are challenging, and each case must be individualized and have functionality and risk assessed in a naturally immunosuppressed population.

Introdução: Glomeruloesclerose Segmentar e Focal (GESF) é uma entidade caracterizada por lesão glomerular que envolve o podócito como principal alvo. Uma variante de pior prognóstico, baixa resposta à corticoterapia, e rápida evolução para doença renal terminal é reconhecida como GESF colapsante. Relato de Caso: Um homem de 102 anos com síndrome nefrótica e lesão renal aguda foi submetido a biópsia renal, revelando GESF colapsante. Ele obteve excelente resposta ao tratamento imunossupressor, apresentando recuperação renal e recebendo alta de terapia renal substitutiva. Discussão: Diagnóstico e definição de tratamento nos idosos são desafiadores, devendo-se individualizar cada caso, avaliar funcionalidade e risco em uma população naturalmente imunossupressa.

Genotype-Phenotype Correlation Analysis of WT1 Gene Variants in Denys-Drash Syndrome and Frasier Syndrome / 罕见病研究

ObjectiveThis study aims to explore the association between different genotypes of WT1 gene variations and the phenotypes of Denys-Drash syndrome (DDS) and Frasier syndrome (FS).MethodsThrough searching and summarizing the case information of WT1 gene variations recorded in NCBI PubMed and CNKI databases from January 1, 1991 to October 31, 2023, we analyzed the association between variation types, occurrence locations, and phenotypes such as progressive renal function impairment, genitourinary developmental abnormalities, nephroblastoma, and gonadal tumors between DDS and FS.ResultsA total of 128 articles, including 304 subjects, were included in this study, and 86 pathogenic variations of the WT1 gene were detected.The distribution characteristics of these variations were as follows: the most common occurrence was in exon 9(24/86, 27.9%) and exon 8 (23/86, 26.7%); the most common variation type was missense mutation(51/86, 59.3%), followed by splice site mutation (13/86, 15.1%).The disease types caused by WT1 gene variations were as follows: DDS had the highest number of cases (174/304, 57.2%), followed by FS (83/304, 27.3%); DDS was mainly caused by missense mutations on exon 9 and exon 8 (143/174, 82.2%), while FS was mainly caused by splice site mutations on intron 9 (76/83, 91.6%).ConclusionsThe missense variants in exon 9 and exon 8 on the WT1 gene mainly resulted in DDS, while the splice variants in intron 9 mainly resulted in FS. Infants and children with progressive renal injury should undergo a comprehensive evaluation of the genitourinary system, and early genetic diagnosis should be established to improve prognosis.

Research Progress of Monogenic Mutation in the Pathogenesis of Steroid-Resistant Nephrotic Syndrome / 罕见病研究

Steroid-resistant nephrotic syndrome (SRNS) is the second cause of chronic kidney disease in children. The SRNS has high risk of rapid progression to end-stage renal disease. With the advancement of high-throughput sequencing technology, more than 70 monogenic mutation having the Mendelian inheritance patterns are identified to be associated with SRNS. Most of these genes are involved in podocyte function. Accurate diagnosis of monogenic mutation in SRNS patients helps with guiding clinical treatment protocols and genetic counseling, avoiding the excessive use of steroids/immunosuppressive therapy, and opening up possibilities for targeted therapies in SRNS patients. In this article, our research team summarizes and generalizes the molecular mechanisms, genetic testing, and specific treatment for the major types of monogenic mutations associated with SRNS.

Blood concentration monitoring of tacrolimus in patients with nephrotic syndrome and establishment of MLP prediction model / 中国药房

OBJECTIVE To investigate the monitoring of tacrolimus blood concentration in patients with nephrotic syndrome （NS），and to establish a prediction model for tacrolimus blood concentration. METHODS Data from 509 concentration monitoring sessions of 166 NS patients using tacrolimus were collected from January 1， 2020 to August 31， 2023 in Zhongshan Hospital Affiliated to Xiamen University. The relationship of efficacy and adverse drug reaction（ADR） with blood concentration was analyzed. A multilayer perceptron （MLP） prediction model was established by using the blood concentration monitoring data of 302 times from 109 NS patients with genetic information， and then verified. RESULTS In terms of efficacy， the median blood concentration of tacrolimus in the non-remission group was 2.20 ng/mL， which was significantly lower than that in the partial remission group （4.00 ng/mL， P＜0.001） and the complete remission group （3.60 ng/mL， P＝0.002）. In terms of ADR， the median blood concentration of tacrolimus in the ADR group was 5.01 ng/mL， which was significantly higher than that in the non-ADR group （3.37 ng/mL） （P＝0.001）. According to the subgroup analysis of the receiver operating characteristic curve， when the blood concentration of tacrolimus was ≥6.65 ng/mL， patients were more likely to develop elevated blood creatinine [area under the curve （AUC） was 0.764， P＜0.001）； when the blood concentration of tacrolimus was ≥6.55 ng/mL， patients were more likely to develop blood glucose （AUC＝0.615， P＝ 0.005）. The established MLP prediction model has a loss function of 0.9， with an average absolute error of 0.279 5 ng/mL between the predicted and measured values. The determination coefficient of the validation scatter plot was 0.984， indicating an excellent predictive performance of the model. CONCLUSION Tacrolimus blood concentration has an impact on both efficacy and ADR in NS patients. The use of the MLP model for predicting blood concentration exhibits high accuracy with minimal error between predicted and measured values. The model can be used as an important tool in clinical individualized medication regimens.

Catéter peritoneal tunelizado para el manejo de ascitis refractaria en paciente con síndrome nefrótico

Introducción: la ascitis refractaria puede ser una complicación frecuente en el síndrome nefrótico (SN), existen casos reportados del uso de un catéter tunelizado de diálisis peritoneal en pacientes con cirrosis o neoplasias abdominales. Se presenta el caso de un paciente con SN en quién se utilizó un catéter para diálisis peritoneal (DP) para manejo de la ascitis refractaria. Objetivo: mostrar que el catéter peritoneal puede ser considerado como una alternativa para el manejo de la ascitis refractaria en pacientes con síndrome nefrótico. Presentación del caso: paciente varón de 19 años, sin antecedentes patológicos, cursó con edema progresivo y alteración de la función renal. Se evidenció síndrome nefrótico con anasarca y evolucionó con empeoramiento de la función renal ingresando a hemodiálisis de soporte. Se realizó biopsia renal: podocitopatía, glomerulopatía colapsante. Se inició tratamiento con corticoterapia, mejorando la función renal hasta suspender la hemodiálisis, pero presentó ascitis refractaria al tratamiento médico, por lo que se realizó paracentesis evacuatoria en reiteradas ocasiones. Se decidió colocación de catéter peritoneal tunelizado para el manejo de la ascitis refractaria. La ascitis fue disminuyendo progresivamente hasta el retiro del catéter peritoneal. Discusión y conclusión: el uso de catéter tunelizado de diálisis peritoneal es una opción de manejo efectiva en casos de síndrome nefrótico con ascitis refractaria.

Introduction: Refractory ascites can be a frequent complication in nephrotic syndrome (NS), there are reported cases of the use of a tunneled peritoneal dialysis catheter in patients with cirrhosis or abdominal neoplasms. The case of a patient with NS is presented in whom used a peritoneal dialysis (PD) catheter to manage refractory ascites. Purpose: To show that the peritoneal catheter can be considered as an alternative for the management of refractory ascites in patients with nephrotic syndrome. Presentation of the case: A 19-year-old male patient, with no pathological history, presented progressive edema and impaired renal function. Nephrotic syndrome with anasarca was evidenced, and it evolved with worsening renal function, entering supportive hemodialysis. Renal biopsy was performed: podocytopathy, collapsing glomerulopathy. Corticosteroid treatment was started, improving renal function until hemodialysis was discontinued, but he presented ascites refractory to medical treatment, for which evacuatory paracentesis was performed repeatedly. It was decided to place a tunneled peritoneal catheter for the management of refractory ascites. Ascites gradually decreased until the peritoneal catheter was removed. Discussion and conclusion: The use of a tunneled peritoneal dialysis catheter is an effective management option in cases of nephrotic syndrome with refractory ascites.

Frecuencia de mutaciones encontradas en niños con enfermedad renal crónica por síndrome nefrótico cortico-resistente / Frequency of mutations found in children with chronic kidney disease due to steroid-resistant nephrotic syndrome

RESUMEN La enfermedad renal crónica en niños puede tener como etiología un grupo cuya causa genética, incluye las anomalías congénitas del riñón y las vías urinarias y las nefropatías hereditarias. Objetivo: Describir la frecuencia de mutaciones en niños con síndrome nefrótico corticoresistente (SNRE). Material y métodos: Estudio multicéntrico, tipo serie de casos, realizado en niños con SNRE, mediante el resultado de secuenciamiento directo de los genes; NPHS1, NPHS2, NPHP1 y WT1. Resultados: Se enrolaron 33 pacientes pediátricos con enfermedad renal crónica, 45,5% mujeres, edad promedio 13±7 años, 78,8% de raza mestiza, 24,2% consanguíneos, 60,6% se encontraban en hemodiálisis, 72,7% presentaban síndrome nefrótico cortico resistente y de ellos 8/24 (33,3%) presentó al menos una mutación en los genes; WT1, NPHS1, NPHP1y NPHS2 siendo la frecuencia de estas mutaciones de 37,5% (3/8), 25% (2/8), 25% (2/8) y 12,5% (1/8), respectivamente. Conclusiones: El 33,3% de los pacientes pediátricos con enfermedad renal crónica con síndrome nefrótico corticoresistente (SNRE) presentaron mutaciones, la más frecuente fue en el gen WT1.

SUMMARY Chronic kidney disease in children may be caused by a group of genetic abnormalities of the kidney, urinary tract and hereditary nephropathies. Objective: To report the frequency of mutations in children with steroid-resistant nephrotic syndrome (SRNS). Methods: A multicentric case series among children with SRNS identified through direct sequencing of NPHS1, NPHS2, NPHP1 and WT1 genes. Results: 33 children were enrolled; 45.5% were females; mean age was 13±7 years; 78.8% were mestizo: 24.2% consanguineous; 60.6% were receiving dialysis: 72.7% had SRNS and 8/24 (33.3%) of them presented at least one mutation to WT1, NPHS1, NPHP1 and NPHS2 genes. Corresponding values for these mutations were 37.5% (3/8), 25% (2/8), 25% (2/8) and 12.5% (1/8), respectively. Conclusions: 33% of pediatric patients with SRNS presented gene mutations, the most frequent of these mutations was WT1.

Glomerular parietal epithelial expression of CD44 in minimal change nephrotic syndrome and primary focal segmental glomerulosclerosis: A clinico-pathological study

Introduction: Minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) are the two common causes of nephrotic syndrome (NS) in both children and adults with overlapping clinical features, but with distinct prognostic and therapeutic implications. The distinction between these relies entirely on histopathology, which can sometimes be difficult. CD44 is expressed by activated parietal epithelial cells, plays a role in matrix deposition and thus in the pathogenesis of FSGS. Aims: To assess the expression of CD44 in MCNS and FSGS and to evaluate its association with the known clinical and histopathological prognostic factors. Materials and Methods: Thirty cases each of MCNS and FSGS were studied. The clinical, laboratory, histopathological, and CD 44 immunohistochemical data were recorded. The findings were analyzed and correlated. A P value of < 0.05 was considered statistically significant. Results: Statistical association was noted between CD44 positivity and serum creatinine (p = 0.031), estimated glomerular filtration rate (p = 0.040), segmental sclerosis (p < 0.001), tubular atrophy (p = 0.027), interstitial fibrosis (p = 0.027), and histological diagnosis (p < 0.001). The sensitivity, specificity, positive predictive, and negative predictive values were 90%, 76.67%, 79.41% and 88.46%, respectively. Conclusions: CD44 immunostain can effectively distinguish MCNS from FSGS. The congruent results of CD44 positivity with known prognostic factors support the possibility of using the CD44 marker as a predictive tool in selecting high-risk patients and offering appropriate therapeutic measures.

Rituximab therapy for childhood onset idiopathic nephrotic syndrome: experience of a Portuguese tertiary center / Terapia com Rituximabe para síndrome nefrótica idiopática de início na infância: experiência de um centro terciário português

ABSTRACT Introduction: Rituximab (RTX) is a therapeutic option in pediatric difficult-to-treat idiopathic nephrotic syndrome (NS). We aimed to assess the efficacy and safety of RTX use in these patients. Method: A retrospective study of all patients with idiopathic NS treated with RTX was conducted in a pediatric nephrology division of a tertiary hospital. Demographic, anthropometric, clinical and analytical data were collected prior to treatment and at 6, 12, and 24 months. Results: Sixteen patients were included (11 males), with a median (25th-75th percentile, P25-P75) age at diagnosis of 2 (2.0-2.8) years. Fifteen were steroid-sensitive and 1 was steroid-resistant and sensitive to cyclosporine. The median age at administration of RTX was 10 (6.3-14.0) years. Throughout a median follow-up time of 2.5 (1.0-3.0) years, 6 (37.5%) patients achieved partial remission and 7 (43.8%) had no relapses and were not taking any immunosuppressants at the 24-month follow-up visit. Regarding complications, 1 patient presented persistent hypogammaglobulinemia. Compared with the 12-month period before RTX, there was a decrease in the median number of relapses at 6 and 12 months [3 (3.0-4.0) vs 0 (0-0.8) and 0.50 (0-1.0), respectively; p = 0.001] and in the daily steroids dose (mg/kg/day) at 6, 12, and 24 months [0.29 (0.15-0.67)vs [0.10 (0.07-0.13); p = 0.001], [0.12 (0.05-0.22); p = 0.005] and [0.07(0.04-0.18); p = 0.021]], respectively. There was also a reduction in the median BMI z score at 24 months [2.11 (0.45-3.70) vs. 2.93 (2.01-3.98); p = 0.049]. Conclusion: Our results confirm the efficacy and safety of RTX use in pediatric idiopathic NS and highlight its' potential cardiometabolic benefits.

Resumo Introdução: Rituximabe (RTX) é uma opção terapêutica na síndrome nefrótica (SN) idiopática pediátrica de difícil tratamento. Visamos avaliar eficácia e segurança do uso de RTX nestes pacientes. Método: Realizou-se estudo retrospectivo de todos os pacientes com SN idiopática tratados com RTX, em uma unidade de nefrologia pediátrica de um hospital terciário. Dados demográficos, antropométricos, clínicos e analíticos foram coletados antes do tratamento e aos 6, 12 e 24 meses. Resultados: Incluímos 16 pacientes (11 do sexo masculino), com idade mediana (percentil 25-75, P25-P75) de 2 (2,0-2,8) anos ao diagnóstico. Quinze eram sensíveis a esteroides, e 1 resistente a esteroides e sensível à ciclosporina.A idade mediana na administração do RTX foi 10 (6,3-14,0) anos. Durante um tempo mediano de acompanhamento de 2,5(1,0-3,0) anos, 6 (37,5%) pacientes alcançaram remissão parcial e 7 (43,8%) não tiveram recidivas e não estavam tomando imunossupressor no acompanhamento aos 24 meses. Quanto às complicações,1 paciente apresentou hipogamaglobulinemia persistente. Comparado ao período de12 meses anterior ao RTX, houve diminuição no número mediano de recidivas em 6 e 12 meses [3 (3,0-4,0) vs 0 (0-0,8) e 0,50 (0-1,0), respectivamente; p = 0,001] e na dose diária de esteroides (mg/kg/dia) aos 6, 12 e 24 meses [0,29 (0,15-0,67) >vs [0,10 (0,07-0,13); p = 0,001], [0,12 (0,05-0,22); p = 0,005] e [0,07 (0,04-0,18); p = 0,021], respectivamente. Houve também redução na mediana do escore z do IMC aos 24 meses [2,11 (0,45-3,70) vs 2,93 (2,01-3,98);p = 0,049]. Conclusões: Nossos resultados confirmam a eficácia e segurança do uso de RTX em SN idiopática pediátrica, destacando seus potenciais benefícios cardiometabólicos.

Outcomes of children with idiopathic steroid resistant nephrotic syndrome: a single centre observational study / Desfechos de crianças com síndrome nefrótica idiopática córtico-resistente: um estudo observacional de centro único

ABSTRACT Introduction: Idiopathic steroid resistant nephrotic syndrome (SRNS) has variable outcomes in children. The primary objective of the present study was to assess the cumulative remission rate and the secondary objectives were to assess factors affecting the remission status, kidney function survival, and adverse effects of medications. Methods: One hundred fourteen patients with SRNS were included. Calcineurin inhibitor-based treatment protocol along with prednisolone and angiotensin-converting enzyme inhibitor were used, and patients were followed over 5 years. Results: Median age was 4.5 years; 53.5% of cases were between 1 to 5 years of age. Sixty-two patients (54.4%) were at initial stage and 52 (45.6%) were at a late SRNS stage. Median eGFRcr was 83.5 mL/min/1.73m2 at presentation. Of the 110 patients, 63 (57.3%) achieved remission [complete remission 30 (27.3%), partial remission 33 (30%)], and 47 (42.7%) had no remission. Kidney function survival was 87.3% and 14 cases (12.7%) had progression to CKD (G3-8, G4-3, G5-1, and G5D-2). Median duration of follow up was 36 months (IQR 24, 60). Age of onset, cyclosporine/tacrolimus, eGFRcr, and histopathology (MCD/FSGS) did not affect remission. Similarly, remission status in addition to age of onset, drug protocol, and histopathology did not significantly affect kidney function during a period of 5 years. Hypertension, cushingoid facies, short stature, cataract, and obesity were observed in 37.7, 29.8, 25.5, 17.5, and 0.7% of cases, respectively. Conclusion: About half of the cases achieved remission. Age of onset of disease, cyclosporine/tacrolimus use, and histopathological lesion neither affected remission status nor short-term kidney function survival in SRNS.

RESUMO Introdução: A síndrome nefrótica idiopática córtico-resistente (SNICR) apresenta desfechos variáveis em crianças. O objetivo principal deste estudo foi avaliar a taxa de remissão cumulativa. Os objetivos secundários foram avaliar fatores que afetam status de remissão, sobrevida da função renal e efeitos adversos de medicamentos. Métodos: Foram incluídos 114 pacientes com SNCR. Utilizou-se protocolo de tratamento baseado em inibidores de calcineurina juntamente com prednisolona e inibidor da enzima conversora de angiotensina. Os pacientes foram acompanhados durante 5 anos. Resultados: A idade mediana foi 4,5 anos; 53,5% dos casos tinham entre 1 e 5 anos. 62 pacientes (54,4%) estavam em estágio inicial; 52 (45,6%) em estágio tardio da SNCR. A TFGecr mediana foi 83,5 mL/min/1,73 m2 na apresentação. Dos 110 pacientes, 63 (57,3%) alcançaram remissão [remissão completa 30 (27,3%), remissão parcial 33 (30%)], e 47 (42,7%) não apresentaram remissão. A sobrevida da função renal foi 87,3%; 14 casos (12,7%) progrediram para DRC (G3-8, G4-3, G5-1, G5D-2). A duração mediana do acompanhamento foi 36 meses (IIQ 24, 60). Idade no início, ciclosporina/tacrolimus, TFGecr e histopatologia (DLM/GESF) não afetaram a remissão. Igualmente, status de remissão, além da idade no início, protocolo de medicamentos e histopatologia não afetaram significativamente a função renal por 5 anos. Observou-se hipertensão, fácies cushingoide, baixa estatura, catarata e obesidade em 37,7; 29,8; 25,5; 17,5; e 0,7% dos casos, respectivamente. Conclusão: Aproximadamente metade dos casos alcançou remissão. Idade no início, uso de ciclosporina/tacrolimus e lesão histopatológica não afetaram o status de remissão nem a sobrevida da função renal a curto prazo na SNICR.

The challenges of the pandemic and the vaccination against covid-19 in pediatric patients with kidney disease / Os desafios da pandemia e a vacinação covid-19 na população pediátrica com doenças renais

ABSTRACT The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.

Resumo A vacina covid-19 confere proteção direta, reduz as taxas de transmissão do vírus e de novas variantes. No Brasil, estão liberadas para a população pediátrica as vacinas Pfizer/BioNTech e a CoronaVac, ambas seguras, eficazes e imunogênicas. Pacientes pediátricos com síndrome nefrótica e covid-19 têm curso clínico regular sem complicações relacionadas ao uso de esteroides ou vacinas. Esses pacientes, com ou sem imunossupressão, não apresentam maior risco de covid-19 grave e o tratamento com esteroides é seguro. Os pacientes com doença renal crônica têm covid-19 mais leve, sem necessidade de hospitalização. A resposta vacinal pode ser reduzida e/ou a duração dos anticorpos pós-vacinação pode ser menor do que na população geral. Entretanto, a vacina covid-19 está recomendada, considerando o risco de exposição. Acredita-se que pacientes com síndrome hemolítico-urêmica teriam maior risco de covid-19 grave. A vacina é recomendada, embora dados específicos sobre segurança e eficácia da vacina covid-19 sejam limitados. Há concordância que os benefícios da imunidade induzida superam quaisquer riscos da imunização. A vacina covid-19 é recomendada para crianças e adolescentes candidatos ao transplante renal ou já transplantados. Esses pacientes têm resposta imunológica reduzida após a vacina, entretanto ela é recomendada porque os benefícios superam qualquer risco dessa vacinação. A recomendação atual no Brasil é a vacina de tecnologia RNA mensageiro. O objetivo deste documento é levar aos nefrologistas pediátricos os conhecimentos mais recentes sobre a vacinação contra contra-19 em crianças com doenças renais.

AL Amyloidosis with Polycythemia, Leucocytosis and Thrombocytosis : A Case Report

A young lady presented to us with clinical and biochemical evidence of Nephrotic Syndrome. Her laboratory investigations also revealed Erythrocytosis, Leucocytosis and Thrombocytosis. A renal biopsy revealed a diagnosis of Amyloidosis which was further characterized as AL amyloidosis with further investigations (kappa chain monoclonal gammopathy). She was started with appropriate therapy and she showed significant decline in her monoclonal Proteins on follow up. Her Erythrocytosis, Leucocytosis and Thrombocytosis also normalized with the decline in the levels of monoclonal light chains. We postulate a link between the monoclonal protein associated growth factors and inflammatory markers which were responsible for this unique association between AL Amyloidosis and tri-lineage hematopoietic cell proliferation.

Proteinuria y síndrome nefrótico / Proteinuria and Nephrotic Syndrome

Introducción: El síndrome nefrótico es una patología que afecta el complejo glomerular del riñón, se caracteriza por una proteinuria mayor 3500 mg/d. De acuerdo a la respuesta de los esteroides se puede clasificar en síndrome nefrótico en esteroide resistente o esteroide sensible. Objetivo: Determinar la relación que existe entre la proteinuria y las variantes del síndrome nefrótico en adultos. Métodos: Se realizó un estudio descriptivo, retrospectivo, tipo serie de casos, con una población de 28 pacientes. Se recolectaron y se procesaron los datos a través del software Epi-Info 7,2TM; la frecuencia simple, la media estadística, prueba t de Student, y el coeficiente de correlación de Pearson. Resultados: En el análisis combinatorio de los fármacos adyuvantes para síndrome nefrótico, el grupo que utilizó antiproteinúricos pero no estatinas, demostró una diferencia estadísticamente significativa entre la proteinuria postratamiento media del grupo de síndrome nefrótico esteroideo resistente (6202 mg/d) vs síndrome nefrótico esteroideo sensible (65,9 mg/d) (valor de p 0,418). Existe una correlación negativa entre los niveles proteinuria postratamiento y el nivel de albúmina sérica postratamiento (r = - 0,7 valor de p < 0,00001). Conclusiones: Se demostró la ausencia de asociación entre la proteinuria inicial y las variantes de síndrome nefrótico esteroide sensible y esteroide resistente (valor de p = 0,8)(AU)

Introduction: Nephrotic syndrome is a pathology that affects the glomerular complex of the kidney, characterized by proteinuria greater than 3500 mg/d. According to the response to steroids, nephrotic syndrome can be classified as steroid-resistant or steroid-sensitive. Objective: To determine the relationship between proteinuria and the variants of the nephrotic syndrome in adults. Methods: A descriptive, retrospective, case series type study was carried out with a population of 28 patients. The data was collected and processed through Epi-Info 7.2TM software; simple frequency, statistical mean, student's t-test, and Pearson's correlation coefficient. Results: The statistically significant difference was obtained in the antiproteinuric and non-statin group, between the mean post-treatment proteinuria of the steroid resistant nephrotic syndrome group (6202 mg/d) in comparison to steroid sensitive nephrotic syndrome (65.9 mg/d) (p value 0.0418). There is negative correlation between post-treatment proteinuria levels and post-treatment serum albumin level (r= -0.7 p value <0.00001). Conclusions: The absence of association between initial proteinuria and steroid-sensitive and steroid-resistant variants of nephrotic syndrome was demonstrated (p value=0.8)(AU)

Distribution of memory B cell subsets in peripheral blood of children with frequently relapsing nephrotic syndrome / 中国当代儿科杂志

OBJECTIVES@#To investigate the change in the distribution of memory B cell subsets in children with frequently relapsing nephrotic syndrome (FRNS) during the course of the disease.@*METHODS@#A total of 35 children with primary nephrotic syndrome (PNS) who attended the Department of Pediatrics of the Affiliated Hospital of Xuzhou Medical University from October 2020 to October 2021 were enrolled as subjects in this prospective study. According to the response to glucocorticoid (GC) therapy and frequency of recurrence, the children were divided into two groups: FRNS (n=20) and non-FRNS (NFRNS; n=15). Fifteen children who underwent physical examination were enrolled as the control group. The change in memory B cells after GC therapy was compared between groups, and its correlation with clinical indicators was analyzed.@*RESULTS@#Before treatment, the FRNS and NFRNS groups had significantly increased percentages of total B cells, total memory B cells, IgD+ memory B cells, and IgE+ memory B cells compared with the control group, and the FRNS group had significantly greater increases than the NFRNS group (P<0.05); the FRNS group had a significantly lower percentage of class-switched memory B cells than the NFRNS and control groups (P<0.05). After treatment, the FRNS and NFRNS groups had significant reductions in the percentages of total B cells, total memory B cells, IgM+IgD+ memory B cells, IgM+ memory B cells, IgE+ memory B cells, IgD+ memory B cells, and IgG+ memory B cells (P<0.05) and a significant increase in the percentage of class-switched memory B cells (P<0.05). The FRNS group had a significantly higher urinary protein quantification than the NFRNS and control groups (P<0.05) and a significantly lower level of albumin than the control group (P<0.05). In the FRNS group, urinary protein quantification was negatively correlated with the percentage of class-switched memory B cells and was positively correlated with the percentage of IgE+ memory B cells (P<0.05).@*CONCLUSIONS@#Abnormal distribution of memory B cell subsets may be observed in children with FRNS, and the percentages of IgE+ memory B cells and class-switched memory B cells can be used as positive and negative correlation factors for predicting recurrence after GC therapy in these children.

Correlation of serum creatinine, blood urea nitrogen and uric acid levels in patients with nephrotic syndrome / 公共卫生与预防医学

Objective To explore the correlation between serum creatinine, blood urea nitrogen content and uric acid level in adult nephrotie syndrome (AS) patients of different ages, in order to provide a theoretical basis for the prevention and control of uric acid metabolism. Methods Individuals of different ages who were diagnosed with nephrotic syndrome from March 2018 to August 2020 in the outpatient department of our hospital were selected as research subjects by stratified random sampling,healthy individuals who underwent physical examination during the same period were selected as controls .The age range of the subjects was 18-55 years old. The biochemical test results of serum creatinine, blood urea nitrogen and uric acid were retrospectively collected from patients of different ages. Pearson correlation analysis of adult serum creatinine, blood urea nitrogen and uric acid levels. Results The levels of creatinine and blood urea nitrogen in healthy adults showed an upward trend at the age of 18-40, and reached a plateau at the age of 40; while the level of uric acid showed an upward trend with age. The levels of serum creatinine, blood urea nitrogen and uric acid in AS patients at different ages were higher than those in healthy controls, and the increase was most pronounced between the ages of 45 and 55. The t-test results showed that the serum creatinine, blood urea nitrogen and uric acid contents of healthy controls were different from those of AS patients except for the 18-22 age group. The main performance was that AS patients had higher measured values than healthy controls. Pearson correlation was used to analyze the correlation between serum creatinine and blood urea nitrogen levels and uric acid in AS patients of different ages. The results showed that the 41-45-year-old patients had the strongest correlation with uric acid, r was 0.584; The patients' blood urea nitrogen level had the strongest correlation with uric acid, with r of 0.373. The age groups with the correlation between serum creatinine, blood urea nitrogen and uric acid content in AS patients were stratified according to gender. There was a significant positive correlation (r>0.45, P0.30, P<0.05). Conclusion The expression level of serum creatinine in 34-45 years old patients with nephrotic syndrome can effectively predict the level of uric acid, and the predictive value of women is higher than that of men.

Increased behavioural problems associated with corticosteroid use in children with nephrotic syndrome: a Southeast Asian perspective

INTRODUCTION@#The study was performed to determine the psychological problems in children with idiopathic nephrotic syndrome (INS) while they were on steroid therapy, as compared to healthy children.@*METHODS@#This prospective cohort study was conducted in a paediatric clinic of a tertiary hospital. Parents of the participants in the INS group and control group (comprising children without chronic illness) completed questionnaires using the Child Behavioural Checklist (CBCL). The CBCL measures a range of age-specific emotional and psychological problems, including internalising and externalising domains. Analyses of the CBCL scores between groups were done using Mann-Whitney U test.@*RESULTS@#A total of 140 children were recruited with an equal number in the INS and control groups. There was a significant difference in the mean total CBCL scores between the INS group and the control group, specifically in the withdrawal, somatic, anxious and aggressiveness subdomains. Similar findings were demonstrated in correlation between total psychological problems and corticosteroid dosage. In the INS group, steroid dose and cushingoid features were found to have a significant positive association with internalising psychological problems.@*CONCLUSION@#Children with INS on corticosteroid treatment showed an increase in internalising and externalising scores, as compared to healthy children.

Effect of intravenous albumin in patients with liver cirrhosis and nephrotic syndrome / 西安交通大学学报(医学版)

【Objective】 To investigate the outcomes of intravenous injection of human albumin (HA) in patients with both liver cirrhosis and nephrotic syndrome. 【Methods】 We retrospectively studied 101 liver cirrhosis patients with ascites and nephrotic syndrome treated in our hospital from January 2018 to November 2021. All the patients received oral diuretic and intravenous albumin therapy. Their baseline characteristics were collected and the changes in serum albumin and creatinine levels before and after treatment were evaluated. The patients with elevated albumin levels after treatment greater than the median value (1.8 g/L) were defined as response group. The rest of the patients were classified as the non-response group. And Logistic regression analysis was used to evaluate the relevant influencing factors for treatment response. 【Results】 All the patients’ symptoms of abdominal distension related to moderate to great ascites were clinically lessened at the end of treatment, and no case of acute kidney injury occurred during the treatment. Of them, 32 patients had repeated hospitalizations within six months after discharge. The serum albumin level was significantly increased after treatment [(26.5±5.9) g/L vs. (29.9±4.9) g/L, P<0.001] and there was no significant difference in serum creatinine before and after treatment [(111.9±118.4)μmol/L vs. (108.5±87.9)μmol/L, P=0.816]. Fifty-three patients were defined as treatment response group. The differences in characteristics including age, sex, etiology of cirrhosis, and proteinuria were not statistically significant. However, the serum creatinine level was significantly lower in the response group than in the non-response group [(84.1±51.2)μmol/L vs. (142.7±158.4)μmol/L, P=0.017\]. A similar trend of difference was observed with respect to urea nitrogen level \[(8.7±5.1)mmol/L vs. (11.8±9.1)mmol/L, P=0.039\]. Multivariate analyses demonstrated that the serum creatinine level was a risk factor for non-response to treatment (hazard ratio=1.025, 95% CI: 1.010-1.049, P=0.037). In addition, the correlation analysis showed that the baseline albumin levels were negatively correlated with hospital stay time (r=-0.340, P=0.001), daily HA usage (r=-0.546, P<0.001), and baseline proteinuria levels (r=-0.654, P<0.001), respectively. 【Conclusion】 Intravenous injection of HA in cirrhotic patients with nephrotic syndrome was safe and effective for the treatment of ascites. Kidney function affects serum albumin levels and response to treatment.

Long-term outcomes and influencing factors of idiopathic nephrotic syndrome in children / 中华肾脏病杂志

Pediatric idiopathic nephrotic syndrome (INS) is characterized by massive albuminuria, hypoproteinemia, edema and hyperlipidemia, with a long course and high probability of relapse and prolongation. Long-term complications caused by long-term usage of hormones and immunosuppressants in children with INS seriously affect their physical and mental health and quality of life. Most children with steroid-sensitive nephrotic syndrome can be cured before adulthood, while some of them relapse in adulthood. Long-term prognosis of children with steroid-resistant nephrotic syndrome is poor. There have been few studies in China followed the long-term outcomes and its related factors of children with INS over 10 years. The paper reviewed the literatures on the long-term outcomes of children with INS, including renal survival, growth, mental health, learning and work, marriage and fertility, disease recurrence and long-term related complications, to explore the factors related to the poor long-term outcomes of children with INS and to assist in clinical decision-making and follow-up management.

Influencing factors of early non-remission of proteinuria in patients with idiopathic membranous nephropathy / 中华肾脏病杂志

Objective:To investigate the influencing factors of non-remission of proteinuria in patients with nephrotic syndrome (NS) and idiopathic membranous nephropathy (IMN).Methods:The study was a retrospective observational study. The clinical data of patients with NS who were diagnosed as IMN by renal biopsy and serum albumin recovered normal after six months of treatment were collected from Beijing Anzhen Hospital, Capital Medical University from June 1, 2010 to January 31, 2022. Patients were divided into proteinuria remission group and non-proteinuria remission group according to whether urinary protein < 3.5 g/24 h and decreased 50% from the onset. The differences of clinical and pathological characteristics between the two groups at baseline were compared. The logistic regression model was used to analyze the influencing factors of non-remission of proteinuria.Results:Ninety-five NS patients with renal pathology of IMN were included in this study, with age of 57(43, 65) years old and 50 males (52.6%). There were 75 patients in the proteinuria remission group and 20 patients in the non-proteinuria remission group. Compared with the proteinuria remission group, the non-proteinuria remission group had higher baseline body mass index [(26.83±4.03) kg/m 2vs. (24.68±3.97) m 2, t=-2.149, P=0.034] and proportion of overweight (85.0% vs. 58.7%, χ2=4.765, P=0.029), and larger waist circumference [88.5(85.3, 101.5) cm vs. 87.0(77.5, 92.0) cm, Z=2.362, P=0.018]. Renal pathological results showed that the proportions of diabetes nephropathy (10.0% vs. 0, P=0.043) and glomerular hypertrophy (45.0% vs. 20.0%, χ2=5.227, P=0.022) were higher, and the average diameter of hypertrophic glomeruli was longer [(197.96±6.37) μm vs. (193.51±8.50) μm, t=2.029, P=0.041] in the proteinuria remission group than those in the non-proteinuria remission group. Multivariate logistic regression analysis results showed that waist circumference was an independent influencing factor of non-proteinuria remission in patients with IMN under waist circumference > 90 cm in men and >85 cm in women ( OR=1.083, 95% CI 1.005-1.168, P=0.037). Conclusion:Abdominal obesity is an independent risk factor of non-remission of proteinuria in NS patients with IMN after early treatment.

A case of podocyte infolding glomerulopathy / 中华肾脏病杂志

Podocyte infolding glomerulopathy (PIG) is a pathologic type of podocyte glomerulopathy reported recently. The characteristic is that the ultrastructure related to podocytes, such as microspheres and microtubules, are folded into the glomerular basement membrane (GBM) under electron microscope. At present, there are few reports about this disease at home and abroad, and most of them are concentrated in Japan. The clinical characteristics and pathogenesis of PIG are still unclear. In this paper, we report a case of clinical manifestations of nephrotic syndrome, renal biopsy indicated PIG, after the treatment of glucocorticoid, hydroxychloroquine and tacrolimus, the patient's clinical symptoms were relieved and urinary protein decreased.