Síndrome nefrótica sem causa conhecida.
Introdução
O que você precisa saber de cara
A síndrome nefrótica idiopática é uma condição caracterizada por perda de proteínas pelos rins sem que uma causa primária seja identificada. Os sinais mais comumente percebidos pelas famílias incluem inchaço nos olhos e no corpo, urina com alterações e aumento de lipídios no sangue. O diagnóstico pode envolver exames laboratoriais detalhados e avaliação genética com testes específicos. No Brasil, a condição não consta na lista oficial de PCDTs de doenças raras do Ministério da Saúde, devendo o paciente buscar acompanhamento na rede especializada do SUS.
Síndrome nefrótica sem causa conhecida.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 24 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 72 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
35 genes identificados com associação a esta condição.
Nephrotic syndrome 11
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. NPHS11 is an autosomal recessive, steroid-resistant and progressive form with onset in the first decade of life.
Focal segmental glomerulosclerosis 4
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Nephrotic syndrome 15
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. NPHS15 is an autosomal recessive form with onset in the first months of life. Disease severity is variable. Some patients show rapid progression to end-stage renal failure.
Nephrotic syndrome 17
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS17 is an autosomal recessive, steroid-resistant progressive form with onset in the first decade of life.
Microcephaly 24, primary, autosomal recessive
A form of microcephaly, a disease defined as a head circumference more than 3 standard deviations below the age, sex and ethnically matched mean. Brain weight is markedly reduced and the cerebral cortex is disproportionately small. MCPH24 patients additionally manifest mildly impaired intellectual development, cerebellar vermis hypoplasia, and fifth finger clinodactyly.
Palmoplantar keratoderma and woolly hair
A disorder characterized by abnormal thickening of the skin on the palms and soles, in association with woolly scalp hair. Affected individuals manifest a variable degree of striate palmoplantar keratoderma, generally more severe on the soles. Leukonychia is more pronounced on the fingernails than toenails. Scalp hair, body hair, eyebrows, and eyelashes are sparse. The fifth toes show variable degrees of pseudoainhum, ranging from external rotation to a deep sulcus at the digitoplantar fold, accompanied by a bulbous appearance of the distal toe.
Focal segmental glomerulosclerosis 5
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Focal segmental glomerulosclerosis 2
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Focal segmental glomerulosclerosis 6
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation. FSGS6 is a childhood-onset disorder resulting in nephrotic syndrome, which includes massive proteinuria, hypoalbuminemia, hyperlipidemia, and edema.
Frasier syndrome
Characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant.
Nephrotic syndrome 24
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS24 is an autosomal recessive, slowly progressive form. Most patients eventually develop end-stage renal disease.
Nephrotic syndrome 26
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS26 is an autosomal recessive form characterized by onset of proteinuria in the first months or years of life. Some patients respond to steroids, whereas others show steroid resistance and progression to end-stage renal disease.
Nephrotic syndrome 20
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS20 is an X-linked, steroid-resistant form with onset at birth or in the first years of life in affected males. Death in childhood may occur in absence of renal transplantation. Carrier females may be unaffected or have a mild disease with proteinuria.
Nephrotic syndrome 19
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS19 is an autosomal recessive, steroid-resistant form with onset in the first or second decade of life, resulting in chronic kidney disease.
Focal segmental glomerulosclerosis 8
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Focal segmental glomerulosclerosis 3
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Nephrotic syndrome 18
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS18 is an autosomal recessive, steroid-resistant progressive form with onset in the first decade of life.
Focal segmental glomerulosclerosis 9
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Nephrotic syndrome 21
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form that progresses to end-stage renal failure. NPHS21 is an autosomal recessive, rapidly progressive, steroid-resistant form characterized by onset of kidney dysfunction in the first year of life. Some patients may have variable extra-renal manifestations.
Nephrotic syndrome 1
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.
Papillorenal syndrome
An autosomal dominant disorder characterized by both ocular and renal anomalies, but may also include vesicoureteral reflux, high frequency hearing loss, central nervous system anomalies, and/or genital anomalies. Eye anomalies in this disorder consist of a wide and sometimes excavated dysplastic optic disk with the emergence of the retinal vessels from the periphery of the disk, designated optic nerve coloboma or 'morning glory' anomaly. Associated findings may include a small corneal diameter, retinal coloboma, scleral staphyloma, optic nerve cyst, microphthalmia, and pigmentary macular dysplasia. The kidneys are small and abnormally formed (renal hypodysplasia), and have fewer than the normal number of glomeruli, which are enlarged (oligomeganephronia). These ocular and renal anomalies result in decreased visual acuity and retinal detachment, as well as hypertension, proteinuria, and renal insufficiency that frequently progresses to end-stage renal disease.
Nephrotic syndrome 10
A form of nephrotic syndrome, a renal disease clinically characterized by focal segmental glomerulosclerosis, progressive renal failure, severe proteinuria, hypoalbuminemia, hyperlipidemia and edema. NPHS10 is a steroid-sensitive form characterized by onset in childhood and remission without end-stage kidney disease.
Nephrotic syndrome 13
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.
Coenzyme Q10 deficiency, primary, 6
An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis.
Nephrotic syndrome 2
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. The disorder is resistant to steroid treatment and progresses to end-stage renal failure in the first or second decades. Some patients show later onset of the disorder.
Nephrotic syndrome 9
A form of nephrotic syndrome, a renal disease clinically characterized by progressive renal failure, severe proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show focal segmental glomerulosclerosis.
Nephrotic syndrome 8
A form of nephrotic syndrome, a renal disease clinically characterized by progressive renal failure, severe proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show diffuse mesangial sclerosis, with small glomeruli, hypercellularity, increased extracellular matrix, and contracted/collapsed glomerular tufts surrounded by immature or abnormal podocytes.
Nephrotic syndrome 3
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. Most patients with NPHS3 show diffuse mesangial sclerosis on renal biopsy, which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity, hypertrophy of the podocytes, vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen.
Focal segmental glomerulosclerosis 1
A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and progressive decline in renal function. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.
Nephrotic syndrome 12
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. NPHS12 inheritance is autosomal recessive.
Medicamentos aprovados (FDA)
3 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
128 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 9 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
84 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome nefrótica idiopática
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Com ensaio aberto em 5 países. O ponto verde marca onde há vaga agora.
🟢 Recrutando agora
3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
17 ensaios clínicos encontrados, 6 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 674
Mycophenolate mofetil versus prednisone for the initial treatment of idiopathic steroid-sensitive nephrotic syndrome in children in Germany (INTENT): a multicentre, open-label, randomised, controlled, parallel-group, non-inferiority, phase 3 trial.
Prolonged glucocorticoid therapy is the standard initial treatment for idiopathic nephrotic syndrome in children, but is associated with marked toxic effects. We aimed to assess whether a novel treatment protocol with mycophenolate mofetil is as effective as standard therapy with prednisone, while reducing the burden of glucocorticoid-related side-effects. INTENT was a multicentre, open-label, randomised, controlled, parallel-group, non-inferiority, phase 3 trial done in 37 community, municipal, and university hospitals in Germany. Patients aged 1-10 years with a first episode of steroid-sensitive nephrotic syndrome were randomly assigned (1:1) by a centralised web-based tool to receive either mycophenolate mofetil or prednisone (standard treatment), after remission induced by prednisone or prednisolone at a dose of 60 mg/m2 body surface area (maximum 80 mg/day) within 28 days. Block randomisation (block size of eight) was stratified by age (<7 years or ≥7 years). Mycophenolate mofetil was given at 1200 mg/m2 body surface area per day, twice daily, as a suspension (200 mg/mL) for a total treatment duration of 12 weeks. Prednisone was administered once, twice, or three times daily for 6 weeks at 60 mg/m2 body surface area per day (maximum 80 mg). Thereafter, prednisone was given for a further 6 weeks at 40 mg/m2 body surface area (maximum 60 mg) once daily in the morning on alternate days. The primary endpoint was the occurrence of a treated relapse during the 24-months of follow-up in the modified intention-to-treat population. The non-inferiority margin was 15%. This trial is registered with the European Union Drug Regulating Authorities Clinical Trials database (EudraCT 2014-001991-76) and has been completed. Between Oct 12, 2015, and April 23, 2021, 497 patients were screened for eligibility, 272 of whom were randomly assigned (136 to each group). The modified intention-to-treat population comprised 269 patients, of whom 173 (64%) were boys and 96 (36%) were girls (median age 4·0 years [IQR 2·0-5·0]). Mycophenolate mofetil was non-inferior to prednisone for the primary endpoint of treated relapse (106 [79·1%] of 134 vs 101 [74·8%] of 135; difference 4·3% [90% CIs -4·2 to 12·7]; p=0·019). At the end of the first 12 weeks of treatment, fewer glucocorticoid-related side-effects were observed in the mycophenolate mofetil group than the prednisone group, including arterial hypertension (78 [59·1%] of 132 vs 115 [87·1%] of 132; difference -28·0% [95% CI -37·7 to -17·5]), lower BMI (BMI Z score 0·16 [SD 0·85] vs 1·41 [1·02]; difference -1·24 [-1·47 to -1·02]), and fewer psychological abnormalities (37 [27·8%] of 133 vs 77 [57·9%] of 133; difference -30·1% [-40·9 to -18·4]). More patients in the mycophenolate mofetil group than in the prednisone group developed infections (93 [69·9%] of 133 vs 74 [55·6%] of 133; difference 14·3% [2·7 to 25·5]) and there was no statistically significant difference in the number of patients who developed at least one gastrointestinal disorder (22 [16·5%] of 133 vs 13 [9·8%] of 133; difference 6·8% [-1·5 to 14·8]). Our findings suggest that mycophenolate mofetil is non-inferior to standard prednisone treatment, with reduced glucocorticoid-related toxic effects. These findings could modify the initial standard of care for patients with steroid-sensitive nephrotic syndrome. German Federal Ministry of Education and Research.
Role of renal doppler in children with idiopathic nephrotic syndrome.
Renal Doppler can measure intrarenal vascular resistance and may help determine the degree of intrarenal damage, as well as predict subsequent kidney function impairment. However, its utility in children with an underlying kidney disease such as nephrotic syndrome has not been widely examined. This work aimed to measure serial renal resistive index (RI) in pediatric patients with idiopathic nephrotic syndrome to assess its predictive value for steroid resistance and disease outcome. This prospective cohort study included 60 patients with idiopathic nephrotic syndrome aged 5 to < 18 years. Renal Doppler was performed on all children, and renal RI was measured at diagnosis, after 1, 3, and 6 months of diagnosis. The average interlobar renal RI of the right and left kidneys at diagnosis, during follow-up at 1, 3, and 6 months, was significantly higher in steroid-resistant nephrotic syndrome than in the steroid-sensitive nephrotic syndrome. Meanwhile, the estimated glomerular filtration rate (GFR) was significantly lower in steroid-resistant nephrotic syndrome than in steroid-sensitive nephrotic syndrome after 6 months, with no significant difference at diagnosis, after 1 month, or 3 months. The average interlobar RI at diagnosis can predict steroid resistance at a cutoff > 0.60 with 92.31% sensitivity and 85.32% specificity. Moreover, the average interlobar RI after 3 months of follow-up can predict short disease outcome at a cutoff > 0.63 with 84.62% sensitivity and 82.98% specificity. Renal RI might be an effective non-invasive tool for early prediction and risk stratification of steroid resistance in pediatric patients with idiopathic nephrotic syndrome. Its utility lies in supporting earlier clinical decisions rather than replacing established diagnostic methods.
Endothelial cell-released CD93 contributes to podocyte injury in idiopathic nephrotic syndrome.
Idiopathic nephrotic syndrome (INS) is a podocyte disease triggered by immune-derived factors. Endothelial activation occurs in this context, but whether the activated endothelium contributes to podocyte injury is unknown. We tested the hypothesis that CD93, a protein primarily expressed in the endothelium, is a contributory factor of podocyte injury. We studied 460 patients with INS and 150 with other podocytopathies. CD93 was analyzed in kidney tissue, urine, and serum samples. We tested the efficacy of CD93 blockade in vitro and in vivo and investigated the relationship between soluble CD93 and clinical outcomes in human INS. CD93 was highly expressed by glomerular endothelial cells (GEnCs) in human INS, and INS sera stimulated cultured human GEnCs to release CD93. Mechanistically, soluble CD93 mediated podocyte activation via β1 integrin/FAK signaling in cultured human podocytes. CD93 blockade mitigated the activation of cultured human podocytes and albumin permeability in human GEnC-podocyte cocultures as well as albuminuria, glomerulosclerosis, and podocyte loss in two models of nephrotic syndrome: podocyte-specific transforming growth factor-β1 signaling (PodTgfbr1) mice and adriamycin-treated mice. Cd93 knockout mice showed less proteinuria and glomerulosclerosis, compared with controls, after adriamycin injection. In patients with INS, soluble CD93 was high in urine in ~90% and 50% of patients in relapse and remission, respectively. High urinary CD93 was associated with faster decline in kidney function and slower response to immunosuppression. Soluble and glomerular CD93 was also elevated in other podocytopathies. We conclude that soluble CD93 contributes to podocyte injury.
Factors Influencing Cyclosporine Trough and Two-Hour Post-dose Concentrations in Children with Idiopathic Nephrotic Syndrome.
To determine the cyclosporine trough (C0) and two-hour post-dose concentrations (C2) in children with nephrotic syndrome (NS) and study the factors influencing them. In this ambispective cohort study, children with NS (including frequently relapsing, steroid-dependent and steroid-resistant nephrotic syndrome) on cyclosporine therapy were enrolled. Clinical and laboratory data were recorded. C0 and C2 were estimated using liquid chromatography mass spectrometry. Among 27 patients with a mean (SD) age of 6.4 (4.1) years, the median (Q1, Q3) of C0 and C2 were 114.8 (86.1, 186.3) ng/mL and 661.6 (442.1, 884.6) ng/mL, respectively. At a mean (SD) cyclosporine dose of 4.1 (0.8) mg/kg/day, 12 (44%) patients had C0 above and 4 (15%) below the therapeutic range (80-120 ng/mL). C2 levels were outside the recommended ranges in 17 (63%) children, within range in 7 (26%), and below in 3 (11%). Capsule users had higher C0 than syrup users. Amlodipine therapy was associated with higher C0 levels. Nearly half the children had C0 and C2 levels outside the therapeutic range. Dosage form and amlodipine influenced C0, not C2 concentrations.
Inferior Vena Cava/Aorta Diameter Index for the Evaluation of Intravascular Volume Status in Children with Idiopathic Nephrotic Syndrome.
To correlate the inferior vena cava/aorta diameter (IVC/Ao index) in children with nephrotic syndrome with clinical indicators of volume status, and to compare the IVC/Ao index between children in relapse and in remission. This prospective longitudinal study included children aged 2-18 years presenting with relapsed nephrotic syndrome. Low intravascular volume status was diagnosed if two of the three criteria (tachycardia, hypotension, prolonged capillary filling time) were present. Sonographic measurements of the IVC and aorta diameter were obtained by a single observer at recruitment and after achieving remission. One hundred and three children (73 boys) with a median (Q1, Q3) age 72 (24, 216) months were recruited. 26 (25%) children had low intravascular volume at recruitment. The mean (SD) IVC/Ao index was lower in low volume status compared to normal volume status [0.74 (0.2) vs 0.83 (0.1); P = 0.090]. The correlation between IVC/Ao index and heart rate (r = -0.29, P = 0.003) and mean arterial pressure (r = 0.23, P = 0.018) was weak. IVC/Ao index was not an independent predictor of volume status (OR = 0.04, P = 0.331). The mean (SD) IVC/Ao index increased significantly from when in relapse to remission [0.94 (0.1) vs 1.05 (0.2), respectively; P = 0.001]. The IVC/Ao index was lower in relapse than in remission, and did not predict low intravascular volume status independently.
Publicações recentes
Humoral Immune Response to SARS-CoV-2 in Children with Idiopathic Nephrotic Syndrome: A Cross-Sectional Exploratory Study.
Navigating the methodological, ethical, and operational challenges of Trials within Cohorts (TwiCs): insights from a French pediatric research-based cohort.
Rituximab in a desensitization protocol as a rescue therapy in severe nephrotic syndrome-a case report.
Angiotensin-converting enzyme insertion/deletion gene polymorphism in children with nephrotic syndrome: Genotype-phenotype correlations.
Mycophenolate mofetil versus prednisone for the initial treatment of idiopathic steroid-sensitive nephrotic syndrome in children in Germany (INTENT): a multicentre, open-label, randomised, controlled, parallel-group, non-inferiority, phase 3 trial.
📚 EuropePMC958 artigos no totalmostrando 195
B cell depletion with rituximab in children with steroid-sensitive nephrotic syndrome: does depletion duration change outcome?
Pediatric nephrology (Berlin, Germany)Mycophenolate mofetil versus prednisone for the initial treatment of idiopathic steroid-sensitive nephrotic syndrome in children in Germany (INTENT): a multicentre, open-label, randomised, controlled, parallel-group, non-inferiority, phase 3 trial.
The Lancet. Child & adolescent healthA Retrospective Cohort Analysis of Short-Term Outcomes of the Mycophenolate Mofetil-Based Triple Regimen for the Treatment of Calcineurin Inhibitor-Resistant Childhood Nephrotic Syndrome: A Single-Center Experience.
CureusUnraveling BCR Repertoire Diversity and its Impact on Glucocorticoid Therapy in Pediatric Idiopathic Nephrotic Syndrome.
Journal of clinical immunologyAnti-nephrin autoantibodies in post-transplant recurrent focal segmental glomerulosclerosis: diagnostic advances and future directions.
Clinical and experimental nephrologyEffect of Steroids on Anti-nephrin Autoantibodies and B cells in Minimal Change Disease.
Kidney international reportsVoclosporin ameliorates proteinuria in a model of non-inflammatory glomerular disease.
BMC nephrologySteroid-induced adrenal insufficiency in children with nephrotic syndrome: a systematic review.
Pediatric nephrology (Berlin, Germany)Glomerular Transcriptome Analysis Reveals Endothelial Disturbances in Patients With Idiopathic Nephrotic Syndrome.
Kidney medicineEfficacy and Safety of Therapies for Pediatric Steroid-Resistant Idiopathic Nephrotic Syndrome: A Systematic Review of the Last Decade.
CureusSingle relapse at ≥ 0.5 mg/kg alternate-day prednisone predicts course of childhood nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Role of renal doppler in children with idiopathic nephrotic syndrome.
BMC pediatricsPolygenic risk score from steroid-sensitive nephrotic syndrome GWAS indicates overlapping genetic basis with steroid-resistant cases.
Scientific reportsLevels of circulating kidney injury markers and IL-10 identify non-critically ill patients with COVID-19 at risk of death.
JCI insightA New Hope for Treating Podocytopathies: Emerging Role of Anti-Nephrin Antibody.
Clinical journal of the American Society of Nephrology : CJASNEndothelial cell-released CD93 contributes to podocyte injury in idiopathic nephrotic syndrome.
Science translational medicineAssociation between Anti-Nephrin Antibodies and Podocytopathies: A Systematic Review and Meta-Analysis.
NephronCeliac Disease in Children with Idiopathic Nephrotic Syndrome-A Retrospective Cohort Study.
Journal of clinical medicineDynamic Changes in Oxidative Stress Biomarkers in a Child with Idiopathic Nephrotic Syndrome: A Longitudinal Case Study.
International journal of molecular sciencesBiopsy Morphometrics as Predictors of Treatment Response in Primary Nephrotic Syndrome.
Kidney medicineB-cell activation underpins Rituximab response in focal segmental glomerulosclerosis.
Translational research : the journal of laboratory and clinical medicineProlonged impairment of immunological memory after anti-CD20 treatment in pediatric idiopathic nephrotic syndrome: an extended follow-up.
Frontiers in immunologyTherapeutic trough mycophenolic acid levels positively correlate with omega-3 fatty acids in leukocytes of children with idiopathic nephrotic syndrome. a pilot study.
Frontiers in pharmacologyVitamin D Deficiency in Relapsing Idiopathic Nephrotic Syndrome in Children: Prevalence, Correlates, and Therapeutic Implications.
International journal of endocrinologySerum Podocalyxin Level as a Potential Biomarker for Diagnosis of Nephrotic Syndrome and Prediction of Steroid Response.
Medeniyet medical journalAfucosylated IgG in idiopathic nephrotic syndrome patients with anti-nephrin autoantibodies correlate with disease activity.
Journal of translational autoimmunityIdentification of Potential Focal Segmental Glomerulosclerosis (FSGS) Through Evaluation of Parietal Epithelial Cells Status Changes Using Integrin α3 Immunostaining.
Laboratory investigation; a journal of technical methods and pathologyDaratumumab combined with anti-CD20 therapy in pediatric and adult refractory idiopathic nephrotic syndrome: single-center experience.
Frontiers in immunologyComparative analysis of the bone age of wrist bones in Chinese children with different causes of short stature and central precocious puberty.
Quantitative imaging in medicine and surgerySeroconversion following varicella vaccination in children and adolescents with nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Blood biomarkers of disease activity in pediatric idiopathic nephrotic syndrome: A prospective study.
Clinical nephrologyPneumococcal Vaccine Use and Immunogenicity in Patients with Glomerular Disease: A Scoping Review and Analysis of Healthcare Claims Data.
Glomerular diseasesMachine learning model for predicting severe infection in children with idiopathic nephrotic syndrome: multicenter retrospective study.
Italian journal of pediatricsVitamin D Status and Clinical Outcomes in Pediatric Idiopathic Nephrotic Syndrome: A Prospective Observational Study from North India.
Annals of African medicineUrinary vitamin D binding protein levels in children with idiopathic nephrotic syndrome: a biomarker differentiating steroid sensitive from steroid resistant nephrotic syndrome.
Jornal brasileiro de nefrologiaImmunogenicity of the 23-valent polysaccharide pneumococcal vaccine in children with steroid sensitive nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Obinutuzumab as a therapeutic option for SDNS children resistant to rituximab with anti-RTX antibodies.
Pediatric nephrology (Berlin, Germany)Modelling CubAm function and regulation in proximal tubular cells using iPSC-derived kidney organoids.
Experimental cell researchLatent Intrarenal Renin-Angiotensin-Aldosterone System Activation Could Persist Until Early School-Aged in Children with a History of Low Birth Weight.
Biomarker insightsNovel inflammation biomarkers in adult minimal change disease: predicting steroid-resistance and relapse.
European journal of medical researchEmerging roles of non-coding RNAs in idiopathic nephrotic syndrome: a narrative review.
BMC nephrologyClinical characteristics of children with idiopathic nephrotic syndrome infected with SARS-CoV-2: a single-center retrospective cohort study.
BMC pediatricsDevelopment and external validation of a machine learning-based predictive model for acute kidney injury in hospitalized children with idiopathic nephrotic syndrome.
BMC medical informatics and decision makingMacrophage migration inhibitory factor and angiopoietin-like protein 4 as markers for steroid response in children with idiopathic nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Effectiveness of Mycophenolate Mofetil Trough Level Monitoring in Children with Relapsing Nephrotic Syndrome.
Clinical journal of the American Society of Nephrology : CJASNThymic Stromal Lymphopoietin May Induce Steroid Resistance in Minimal Change Disease.
Nephrology (Carlton, Vic.)Factors Influencing Cyclosporine Trough and Two-Hour Post-dose Concentrations in Children with Idiopathic Nephrotic Syndrome.
Indian pediatricsLong-term outcome of mycophenolate mofetil after a single dose of rituximab in childhood-onset refractory idiopathic nephrotic syndrome.
Clinical and experimental nephrologyCorrelation of Oxidative Stress Biomarkers with Activity of Pediatric Idiopathic Nephrotic Syndrome.
BiomedicinesAnti-Rituximab Antibodies Occurrence and Clinical Outcomes in Patients With Primary Membranous Nephropathy.
Kidney international reportsNon-invasive Diagnosis of Antinephrin-Associated Podocytopathy.
Kidney international reportsSerum Syndecan-1 is Associated With Kidney and Cardiovascular Outcomes in Idiopathic Nephrotic Syndrome.
Kidney international reportsProteinuria and albuminuria prevalence in allergic children.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieImpact of autonomic dysfunction in light chain amyloidosis patient with nephrotic syndrome and cardiac involvement.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisDysregulated Bone Marrow Contributes to Glomerular Injury through Soluble Factors.
Journal of the American Society of Nephrology : JASNCo-localization of IgG with nephrin in immune-mediated idiopathic nephrotic syndrome.
Clinical and experimental nephrologyInferior Vena Cava/Aorta Diameter Index for the Evaluation of Intravascular Volume Status in Children with Idiopathic Nephrotic Syndrome.
Indian pediatricsManagement of Nephrotic Syndrome in Pediatric Patients Treated by Different Steroid Regimens.
Medicina (Kaunas, Lithuania)Podocyte Glucocorticoid Receptor Expression and Treatment Outcome in Idiopathic Nephrotic Syndrome.
Kidney international reportsKidney function and mortality in childhood nephrotic syndrome.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationOptimizing the use of anti-CD20 in childhood idiopathic nephrotic syndrome: an intersection of art and science.
Kidney internationalMachine learning using serial changes in proteinuria during initial steroid therapy to predict treatment response and immunosuppressant use in pediatric idiopathic nephrotic syndrome.
Clinical and experimental nephrologyAutoantibodies Targeting Vinculin Reveal Novel Insight into the Mechanisms of Autoimmune Podocytopathies.
Research (Washington, D.C.)Serum matrix metalloproteinase-9 as a predictor of recurrence in idiopathic nephrotic syndrome.
Clinical and experimental nephrologySingle cell analysis identified IFN signaling activation contributes to the pathogenesis of pediatric steroid-sensitive nephrotic syndrome.
Biomarker researchAssociation between SIRT-1 and SERPINA4 gene polymorphisms and the risk of idiopathic nephrotic syndrome among Egyptian children.
Molecular biology reportsPotential and pitfalls of measuring circulating anti-nephrin autoantibodies in glomerular diseases.
Clinical kidney journalInterventions for idiopathic steroid-resistant nephrotic syndrome in children.
The Cochrane database of systematic reviewsThe efficacy and safety of rituximab monotherapy in the new onset pediatric idiopathic nephrotic syndrome: a randomized controlled clinical trial.
Renal failureAnti-CD20 monoclonal antibodies for idiopathic nephrotic syndrome: Advances, challenges, and future directions.
Pediatric nephrology (Berlin, Germany)Slowly Progressive Type 1 Diabetes following Steroid-Sensitive Relapsing Nephrotic Syndrome in Childhood: A Case Report.
Biomedicine hubUnraveling the Immunogenetic Mechanisms of Childhood Idiopathic Nephrotic Syndrome.
The Journal of pediatricsLong-Term Outcomes in Nephrotic Syndrome by Kidney Biopsy Diagnosis and Proteinuria.
Journal of the American Society of Nephrology : JASNSalivary Interleukin-6 and Interleukin-18 Levels and Their Association with Dental Health in Children with Idiopathic Nephrotic Syndrome.
International journal of molecular sciencesEffects of steroid-resistant nephrotic syndrome serum on AA pathway in podocytes cultured in 3D in vitro glomerular model.
Scientific reportsObinutuzumab and Ofatumumab are More Effective Than Rituximab in the Treatment of Membranous Nephropathy Patients With Anti-Rituximab Antibodies.
Kidney international reports[The highlights of nephrology in 2024].
Nephrologie & therapeutiqueAnti-GAD antibody-negative, anti-IA2 antibody-positive slowly progressive insulin-dependent diabetes mellitus and Graves' disease preceded by childhood-onset minimal change nephrotic syndrome: a case report.
Diabetology internationalTreatment of nephrotic syndrome with anti-CD20 therapies in pregnancy: a case series and review of the literature.
Renal failureComparison of B lymphocyte profile between membranous nephropathy and idiopathic nephrotic syndrome pediatric patients.
Pediatric nephrology (Berlin, Germany)A case of acute appendicitis in a patient with minimal change disease.
CEN case reportsDifferent profiles of fatty acids between leukocytes and whole blood in children with idiopathic nephrotic syndrome.
Lipids in health and diseaseAmino acid variants in the HLA-DQA1 and HLA-DQB1 molecules explain the major association of variants with relapse status in pediatric patients with steroid-sensitive nephrotic syndrome.
Italian journal of pediatricsA digest of the clinical practice guideline for pediatric idiopathic nephrotic syndrome 2020 updated: medical therapy.
Clinical and experimental nephrologyQuality of Life of Children with Idiopathic Nephrotic Syndrome.
Indian journal of nephrologyPersonalized disease recurrence modeling using iPSC-derived podocytes in patients with idiopathic nephrotic syndrome.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationA "Trial within a Cohort" platform for pediatric clinical trials on idiopathic nephrotic syndrome: scope, objectives, and design of the retrospective-prospective cohort PIN'SNP.
Pediatric nephrology (Berlin, Germany)The Role of Cytokines and Chemokines as Biomarkers of Disease Activity in Idiopathic Nephrotic Syndrome in Children.
Current issues in molecular biologyProteomic profiling of kidney biopsies in nephrotic syndrome.
Wellcome open researchDetection of Antinephrin Antibodies in Childhood Idiopathic Nephrotic Syndrome.
Kidney international reportsProatherogenic changes in the quantity and quality of lipoproteins in adults with idiopathic nephrotic syndrome.
Clinica chimica acta; international journal of clinical chemistryBiomarkers to predict or measure steroid resistance in idiopathic nephrotic syndrome: A systematic review.
PloS onePast and future in vitro and in vivo approaches toward circulating factors and biomarkers in idiopathic nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Changes in Serum Immunoglobulin Levels Play as Predictors of Treatment Response and Prognosis in Pediatric Idiopathic Nephrotic Syndrome During the Remission Phase.
Immunity, inflammation and diseaseRetrospective Cohort Analysis of Class II Human Leukocyte Antigen (HLA) Alleles in Children With Steroid-Dependent Nephrotic Syndrome Treated With Cyclophosphamide.
CureusKinetic-pharmacodynamic model to predict post-rituximab B-cell repletion as a predictor of relapse in pediatric idiopathic nephrotic syndrome.
Frontiers in pharmacologyAnalysis of Glomerular Transcriptomes from Nephrotic Patients Suggest APOL1 Risk Variants Impact Parietal Epithelial Cells.
medRxiv : the preprint server for health sciencesRituximab as a first-line therapy in children with new-onset idiopathic nephrotic syndrome.
Clinical kidney journalHeat shock protein 70 levels in children with nephrotic syndrome.
The Turkish journal of pediatricsSuccessful Switch to Obinutuzumab in a Rituximab-Intolerant Child with Difficult-to-Treat Idiopathic Nephrotic Syndrome.
Journal of clinical medicineManagement and Treatment of Primary Membranous Nephropathy With a Positive PLA2R Marker.
CureusIPNA clinical practice recommendations on care of pediatric patients with pre-existing kidney disease during seasonal outbreak of COVID-19.
Pediatric nephrology (Berlin, Germany)Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.
Pediatric nephrology (Berlin, Germany)Adult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan.
World journal of nephrologyStem Cells Derived From Human Deciduous Exfoliated Teeth Ameliorate Adriamycin-induced Nephropathy In Rats By Modulating The Th17/Treg Balance.
Current stem cell research & therapyPublisher Correction: Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey.
Pediatric nephrology (Berlin, Germany)Renal survival and treatment of adult patients with Primary Focal Segmental glomerulosclerosis: A historical cohort study of the National Greek Registry.
PloS oneThe Pathogenesis of Nephrotic Syndrome: A Perspective from B Cells.
Kidney diseases (Basel, Switzerland)MicroRNAs in idiopathic childhood nephrotic syndrome.
Clinical and experimental nephrologyDetailed Pathophysiology of Minimal Change Disease: Insights into Podocyte Dysfunction, Immune Dysregulation, and Genetic Susceptibility.
International journal of molecular sciencesThe autoimmune architecture of childhood idiopathic nephrotic syndrome.
Kidney internationalEfficacy and Safety of Subcutaneous Rituximab in Idiopathic Nephrotic Syndrome.
Kidney international reportsGut dysbiosis as a susceptibility factor in childhood idiopathic nephrotic syndrome.
Pediatrics and neonatologyAssessment of health-related quality of life of children with idiopathic nephrotic syndrome and their caregivers in China.
Health and quality of life outcomesModeling a biofluid-derived extracellular vesicle surface signature to differentiate pediatric idiopathic nephrotic syndrome clinical subgroups.
Scientific reportsLong-Term Outcomes of Rituximab-Treated Adult Patients with Podocytopathies.
Journal of the American Society of Nephrology : JASNRecovery from rituximab-associated persistent hypogammaglobulinaemia in children with nephrotic syndrome.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationAnti-Tumor Necrosis Factor Treatment for Glomerulopathy: Case Report and Review of Literature.
Federal practitioner : for the health care professionals of the VA, DoD, and PHSAutoantibodies Targeting Proteasome Subunit Alpha Type 1 in Autoimmune Podocytopathies.
Journal of the American Society of Nephrology : JASNResolution versus persistence of childhood idiopathic nephrotic syndrome-A population-based study.
Acta paediatrica (Oslo, Norway : 1992)Steroid pulse therapy in idiopathic nephrotic syndrome in the era of modern immunosuppressive treatment-still up to date?
Pediatric nephrology (Berlin, Germany)Exosomes secreted by podocytes regulate the differentiation of Th17/Treg cells in idiopathic nephrotic syndrome.
HeliyonTo biopsy or not to biopsy a teenager with typical idiopathic nephrotic syndrome? Start steroids first.
Pediatric nephrology (Berlin, Germany)To biopsy or not to biopsy a teenager with idiopathic nephrotic syndrome? Biopsy first.
Pediatric nephrology (Berlin, Germany)Risk Factors for Idiopathic Nephrotic Syndrome Relapse in Pediatric Age.
Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologiaTiming of relapse as a key indicator of steroid-sparing requirements in childhood idiopathic nephrotic syndrome.
Journal of nephrologySerum tumour necrosis factor-alpha as a marker of disease activity in children with nephrotic syndrome.
Journal of tropical pediatricsNational Unified Renal Translational Research Enterprise: Idiopathic Nephrotic Syndrome (NURTuRE-INS) study.
Clinical kidney journalNovel mutation patterns in children with steroid-resistant nephrotic syndrome.
Clinical kidney journalPre-transplant anti-nephrin antibodies are specific predictors of recurrent diffuse podocytopathy in the kidney allograft.
Kidney internationalAn Atypical Presentation of Fabry Disease in a Patient With Nephrotic Syndrome: A Case Report.
CureusAssociation between serum albumin and body water using a bioelectrical impedance analyzer: a case report of longitudinal variation in a child with initial idiopathic nephrotic syndrome.
AME case reportsB-cell repertoire and functions in idiopathic nephrotic syndrome: what to learn from single-cell RNA sequencing?
Kidney internationalNephrotic syndrome associated with solid malignancies: a systematic review.
BMC nephrologyRelation between interleukin-13 and annexin-V levels and carotid intima-media thickness in nephrotic syndrome.
Journal of circulating biomarkersPediatric idiopathic nephrotic syndrome during COVID-19 omicron variant pandemic.
Pediatrics international : official journal of the Japan Pediatric SocietyImmunogenicity, Immunological Memory and Monitoring of Disease Activity Following an Anamnestic Immunization With the 13-Valent Pneumococcal Conjugate Vaccine in Children With Idiopathic Nephrotic Syndrome.
Journal of the Pediatric Infectious Diseases SocietyAutoantibodies Targeting Nephrin in Podocytopathies.
The New England journal of medicineSteroid-Resistant Nephrotic Syndrome due to NPHS2 Variants Is Not Associated With Posttransplant Recurrence.
Kidney international reportsProteomic Analysis of Idiopathic Nephrotic Syndrome Triggered by Primary Podocytopathies in Adults: Regulatory Mechanisms and Diagnostic Implications.
Journal of proteome researchA Rare De Novo Mutation in the TRIM8 Gene in a 17-Year-Old Boy with Steroid-Resistant Nephrotic Syndrome: Case Report.
International journal of molecular sciencesPediatric frequent relapsing nephrotic syndrome with multiple cerebral infarctions accompanied by patent foramen ovale and cerebral venous sinus thrombosis: a case report.
BMC nephrologyLevamisole in childhood idiopathic nephrotic syndrome: new promises, and advocacy for global access.
Kidney internationalComprehensive mapping of saliva by multiomics in children with idiopathic nephrotic syndrome.
Nephrology (Carlton, Vic.)A novel flow cytometry panel to identify prognostic markers for steroid-sensitive forms of idiopathic nephrotic syndrome in childhood.
Frontiers in immunologyEffect of proteinuria at relapse on shear wave velocity assessed using ultrasound elastography in children with idiopathic nephrotic syndrome.
Journal of medical ultrasonics (2001)The Role of TNF-α in the Pathogenesis of Idiopathic Nephrotic Syndrome and Its Usefulness as a Marker of the Disease Course.
Journal of clinical medicineSaliva monitoring of prednisolone in children with first onset steroid-sensitive nephrotic syndrome: Is it possible?
British journal of clinical pharmacologyIdiopathic nephrotic syndrome in Syrian children: clinicopathological spectrum, treatment, and outcomes.
Pediatric nephrology (Berlin, Germany)Characterization of glomerular basement membrane components within pediatric glomerular diseases.
Clinical kidney journalMycophenolate Mofetil Versus Prednisone for Induction Therapy in Steroid-Sensitive Idiopathic Nephrotic Syndrome in Children: An Observational Study.
Kidney medicineDupilumab for atopic dermatitis in children with idiopathic nephrotic syndrome treated with cyclosporin: a report of two cases.
The Australasian journal of dermatologySteroid induced ocular complications in idiopathic nephrotic syndrome: a cross sectional single center study.
JPMA. The Journal of the Pakistan Medical AssociationLow Birth Weight is Associated with More Severe Course of Steroid-Sensitive Nephrotic Syndrome in Children, Multicentric Study.
Klinische PadiatrieLipidomic profiles in serum and urine in children with steroid sensitive nephrotic syndrome.
Clinica chimica acta; international journal of clinical chemistryFeasibility of discontinuing immunosuppression in children with idiopathic nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Association between the time of initial relapse and subsequent relapses in patients with childhood-onset idiopathic nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Glucocorticoid- and pioglitazone-induced proteinuria reduction in experimental NS both correlate with glomerular ECM modulation.
iScienceReconsidering the role of the IL-23/IL-17 immune axis in idiopathic nephrotic syndrome pathogenesis.
Clinical kidney journalRepeated implants failure in young patient with idiopathic nephrotic syndrome: a case report with brief review of the literature.
BMC oral healthAtypical Anti-Glomerular Basement Membrane Nephritis: A Case Series From the French Nephropathology Group.
American journal of kidney diseases : the official journal of the National Kidney FoundationRetrospective Cross-Sectional Study Reviewing the Effectiveness of Mycophenolate Mofetil on Saudi Children With Nephrotic Syndrome.
CureusIdiopathic Nephrotic Syndrome in Children in Chad: Epidemiology and Clinical Outcomes.
Journal of clinical medicineTumor necrosis factor alpha gene polymorphism affects the pattern of idiopathic nephrotic syndrome in Kuwaiti Arab children.
Journal of tropical pediatricsOngoing impacts of childhood-onset glomerular diseases during young adulthood.
Pediatric nephrology (Berlin, Germany)Urinary Apolipoprotein A1 and Neutrophil Gelatinase-associated Lipocalin in Children with Idiopathic Nephrotic Syndrome.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaBurdens and Difficulties Experienced by Parental Caregivers of Children and Adolescents with Idiopathic Nephrotic Syndrome in Mainland China: A Qualitative Study [Letter].
Journal of multidisciplinary healthcareBurdens and Difficulties Experienced by Parental Caregivers of Children and Adolescents with Idiopathic Nephrotic Syndrome in Mainland China: A Qualitative Study.
Journal of multidisciplinary healthcareExternal Validation of a Urinary Biomarker Risk Score for the Prediction of Steroid Responsiveness in Adults With Nephrotic Syndrome.
Kidney international reportsRituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Clinical and experimental nephrologyLevamisole Modulation of Podocytes' Actin Cytoskeleton in Nephrotic Syndrome.
BiomedicinesThe extrafollicular B cell response is a hallmark of childhood idiopathic nephrotic syndrome.
Nature communicationsA Unifying Theory for Idiopathic Nephrotic Syndrome Pathogenesis?
Journal of immunology (Baltimore, Md. : 1950)Exploring the significance of interleukin-33/ST2 axis in minimal change disease.
Scientific reportsA Study of Dyslipidemia and Its Clinical Implications in Childhood Nephrotic Syndrome.
CureusRituximab in idiopathic nephrotic syndrome: still waiting for stronger evidences.
Jornal brasileiro de nefrologiaCurrent understanding of the molecular mechanisms of circulating permeability factor in focal segmental glomerulosclerosis.
Frontiers in immunologyVoice of a caregiver: call for action for multidisciplinary teams in the care for children with atypical hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany)COVID-19 outcomes in patients with a history of immune-mediated glomerular diseases.
Frontiers in immunologyComplications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.
CureusCCL22 and Leptin associated with steroid resistance in childhood idiopathic nephrotic syndrome.
Frontiers in pediatricsIntussusception as a complication of idiopathic nephrotic syndrome.
Kidney internationalMembranous Nephropathy After Exposure to Immune Checkpoint Inhibitors.
Kidney international reportsChildhood nephrotic syndrome.
Lancet (London, England)Is kidney biopsy necessary in children with idiopathic nephrotic syndrome?
Acta paediatrica (Oslo, Norway : 1992)The pattern of steroid sensitivity and steroid resistance in childhood idiopathic nephrotic syndrome: A 5-year retrospective observational descriptive study in a South-East Nigerian tertiary hospital.
Nigerian journal of clinical practiceDelayed diagnosis and exacerbation of hyperlipidemia in idiopathic nephrotic syndrome in children during the COVID-19 pandemic.
Clinical and experimental nephrologyNutritional Management of Idiopathic Nephrotic Syndrome in Pediatric Age.
Medical sciences (Basel, Switzerland)Unusual Clinical Presentation of Nephrotic Syndrome With Significant Renal Vein Thrombosis in a Young Male: A Case Report and Literature Review.
CureusLevamisole suppresses activation and proliferation of human T cells by the induction of a p53-dependent DNA damage response.
European journal of immunologyThe Potential Impact of MYH9 (rs3752462) and ELMO1 (rs741301) Genetic Variants on the Risk of Nephrotic Syndrome Incidence.
Biochemical geneticsProteomics of Plasma and Plasma-Treated Podocytes: Application to Focal and Segmental Glomerulosclerosis.
International journal of molecular sciencesA phase I study of autologous mesenchymal stromal cells for severe steroid-dependent nephrotic syndrome.
JCI insightRelationship between H. pylori infection and some other risk factors in the incidence and recurrence of idiopathic nephrotic syndrome in children.
Caspian journal of internal medicineThe Role of PLA2R in Primary Membranous Nephropathy: Do We Still Need a Kidney Biopsy?
GenesAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Síndrome nefrótica idiopática.
É de uma associação que acompanha esta doença? Fale com a gente →
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Síndrome nefrótica idiopática e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Trata-se de uma síndrome nefrótica que ocorre sem uma causa conhecida identificada. Ela é catalogada internacionalmente no Orphanet sob o código ORPHA:357502.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
9 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Síndrome nefrótica idiopática. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/sindrome-nefrotica-idiopatica
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Síndrome nefrótica idiopática
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
