Idiopathic nephrotic syndrome in children

Noone, Damien; Iijima, Kazumoto; Parekh, Rulan S.

doi:10.1016/s0140-6736(18)30536-1

Cited by 424 publications

(400 citation statements)

References 117 publications

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“…Nephrotic syndrome (NS) is one of the most common causes of glomerular disease in childhood with an estimated incidence of 1-7 per 100,000 children [1,2]. NS is characterized by the triad of proteinuria (> 40 mg/m 2 /h), hypoalbuminemia (< 2.5 g/dL), and edema [2]. Different etiologies may lead to NS.…”

Section: Introductionmentioning

confidence: 99%

Nephrotic syndrome in a dish: recent developments in modeling in vitro

et al. 2019

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Nephrotic syndrome is a heterogeneous disease, and one of the most frequent glomerular disorders among children. Depending on the etiology, it may result in end-stage renal disease and the need for renal replacement therapy. A dysfunctional glomerular filtration barrier, comprising of endothelial cells, the glomerular basement membrane and podocytes, characterizes nephrotic syndrome. Podocytes are often the primary target cells in the pathogenesis, in which not only the podocyte function but also their crosstalk with other glomerular cell types can be disturbed due to a myriad of factors. The pathophysiology of nephrotic syndrome is highly complex and studying molecular mechanisms in vitro requires state-of-the-art cell-based models resembling the in vivo situation and preferably a fully functional glomerular filtration barrier. Current advances in stem cell biology and microfluidic platforms have heralded a new era of three-dimensional (3D) cultures that might have the potential to recapitulate the glomerular filtration barrier in vitro. Here, we highlight the molecular basis of nephrotic syndrome and discuss requirements to accurately study nephrotic syndrome in vitro, including an overview of specific podocyte markers, cutting-edge stem cell organoids, and the implementation of microfluidic platforms. The development of (patho) physiologically relevant glomerular models will accelerate the identification of molecular targets involved in nephrotic syndrome and may be the harbinger of a new era of therapeutic avenues.

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Section: Introductionmentioning

confidence: 99%

Nephrotic syndrome in a dish: recent developments in modeling in vitro

et al. 2019

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“…Although up to approximately 85% of paediatric NS patients are reportedly sensitive to steroids, most cases relapse, with approximately half becoming frequent relapsers or steroid‐dependent. Frequent relapsers are defined as at least two relapses over a 6‐month or more than three over a 12‐month period, and steroid‐dependent NS is defined as relapses during treatment or within 2 weeks of stopping corticosteroids …”

Section: Introductionmentioning

confidence: 99%

“…Frequent relapsers are defined as at least two relapses over a 6-month or more than three over a 12-month period, and steroid-dependent NS is defined as relapses during treatment or within 2 weeks of stopping corticosteroids. [6][7][8] To date, the literature has described the long-term renal prognosis of this population with childhood-onset idiopathic NS. However, the non-renal outcomes have not been studied as much.…”

Section: Introductionmentioning

confidence: 99%

Review on long‐term non‐renal complications of childhood nephrotic syndrome

et al. 2019

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Background Nephrotic syndrome (NS) is the most common glomerular childhood disease. A body of literature has described the long‐term renal prognosis of childhood‐onset idiopathic NS. However, the nonrenal outcomes have not been studied as much. Aim We aimed to discuss the long‐term non‐renal outcomes of childhood NS, highlighting studies with a follow‐up period of more than 10 years. Results We reviewed the literature and found that a number of immunosuppressive agents have stopped inflammation, stabilised the podocyte cytoskeleton and reduce proteinuria. However, prolonged treatment has frequently been associated with a high risk of renal and non‐renal complications in patients with a complicated disease course, defined as frequent relapses or steroid dependency. Non‐renal complications may include impaired longitudinal growth and pubertal development, undesirable fertility outcomes, ocular complications, bone mineral diseases and potential malignancies. Conclusion This review discusses and summarises the non‐renal outcomes of idiopathic childhood NS.

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“…The prevalence of idiopathic nephrotic syndrome (NS) is about 1.15 to 16.9 per of 100000 children that depends on race and geographical location. The root cause of this disease is unknown, but its pathogenesis includes various factors such as immunity, systemic features, and structure abnormalities (1)(2)(3)(4). The histopathology of idiopathic NS included minimal change nephrotic syndrome (MCNS), mesangial proliferative glomerulonephritis (MesPGN), and focal segmental glomerulosclerosis (FSGS) (5).…”

Section: Introductionmentioning

confidence: 99%

Serum IgG Level and IgG/IgM Ratio on Admission Predict Steroid-Resistant Response in Vietnamese Children with Idiopathic Nephrotic Syndrome

et al. 2019

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Objectives:The current study aimed at evaluating the predictive value of serum IgG and IgG/IgM ratio measured on admission for steroid-resistant response after eight weeks of treatment in pediatric patients with idiopathic nephrotic syndrome (NS). Methods: The current cross sectional study was conducted on 69 children, including 41 patients with idiopathic NS and 28 healthy subjects as controls. Serum IgA, IgG, and IgM levels were measured in all subjects using immunoturbidimetric method. Results: The median serum IgA, IgG, and IgM levels were 1.15, 2.23, and 1.7 g/L, respectively. Serum IgA and IgG levels in patients were lower than controls (P < 0.001). In addition, 46.3% of children had steroid-resistant nephrotic syndrome (SRNS). IgG had a positive predictive value for SRNS (area under the curve (AUC) = 0.923, P < 0.001). With the cutoff point of 2.04 g/L, this test had the sensitivity and specificity of 89.5% and 95.5%, respectively. The IgG/IgM ratio also had a positive predictive value for SRNS (AUC = 0.892, P < 0.001). With the cutoff point of 1.64 g/L, this test had the sensitivity of and specificity of 89.5% and 81.8%, respectively. Conclusions: Serum IgG level and IgG/IgM ratio can be considered as predictive markers for steroid resistance in children first diagnosed with idiopathic NS.

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Idiopathic nephrotic syndrome in children

Cited by 424 publications

References 117 publications

Nephrotic syndrome in a dish: recent developments in modeling in vitro

Nephrotic syndrome in a dish: recent developments in modeling in vitro

Review on long‐term non‐renal complications of childhood nephrotic syndrome

Serum IgG Level and IgG/IgM Ratio on Admission Predict Steroid-Resistant Response in Vietnamese Children with Idiopathic Nephrotic Syndrome

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