Rasheed Gbadegesin, William E.
PDF Childhood nephrotic syndrome NS 3 is associated with an increase in the permeability across the glomerular filtration barrier due to processes that affect the dynamics and permselectivity of the glomerular filtration barrier.
The incidence of idiopathic NS has been reported to be 2 to 7 cases per children 1and the reported prevalence is 16 in children 1. Severe complications include bacterial infections due to immunoglobulin losses and thromboembolisms related to a relative increase in the hematocrit 1.
Children with NS have been classified into different categories. Patients with primary NS, in which a systemic disease cannot be identified, include children with idiopathic NS absence of glomerular inflammation on renal biopsy and children with primary glomerulonephritis.
Secondary NS is related to a systemic disease.
Less commonly, NS is caused by an inherited renal disease congenital or infantile NS. This form occurs in children younger than 1 year and is associated with a poor outcome 2. Primary idiopathic NS may be associated with at least 3 different histologic abnormalities: Recently, circulating soluble urokinase plasminogen activator receptor has been shown to cause FSGS 4.
Congenital or infantile NS is caused by mutations in podocyte or slit diaphragm proteins [e. Idiopathic NS has been identified as the most prominent form of childhood NS. Children with idiopathic NS present with the typical clinical features of NS, as well as histologic signs of MCD diffuse foot process effacement on electron microscopy and minimal changes on light microscopyFSGS, or mesangial proliferation.
It is debated whether these 3 disorders are separate entities or represent different severities of a single disease process 6. A majority of children display the characteristic histologic findings of MCD 1.
NS in steroid-responsive children is usually related to MCD. The risk of progression to chronic kidney disease is low for these patients. The prognosis for patients with steroid-resistant NS is substantially worse. The histologic variant and the response to steroid treatment is associated with ethnicity 17.
In particular, steroid resistance is more likely to be associated with an African or Hispanic ethnicity 7. Currently, the concentrations of urinary protein, serum lipids, and serum albumin are the only diagnostic biomarkers of NS. Establishing a definitive diagnosis relies on the results of a renal biopsy procedure, which can be associated with severe side effects because of its invasive nature.
Recently, microRNAs miRNAs have been drawing attention as their roles as powerful regulators of gene expression have become known. They thus serve as powerful novel therapeutics for many diseases 8. The overriding importance of these molecules for pathophysiological events through modulation of disease-specific signal-transduction pathways has been demonstrated in numerous studies 9.
Until recently, miRNAs were believed to act merely as intracellular modulators of genetic information. In last few years, however, several studies have revealed the remarkable stability of extracellular miRNAs circulating in the blood or excreted in the urine and underscored their key importance as biomarkers of certain diseases 10 Intriguingly, circulating miRNAs may also modulate cellular transcriptional events by transferring genetic information from a donor cell to a recipient cell Endothelial apoptotic bodies released upon cell damage and subsequent apoptosis induction may also transport circulating miRNAs In the current issue of Clinical Chemistry, Luo et al.
They included patients between the ages of 1 and 14 years in their study. The primary exclusion criteria were secondary NS and congenital NS. Twenty-four patients did not respond to steroid treatment.
An initial large analysis of miRNA gene expression was performed with RNA isolated and pooled from serum samples obtained from 33 NS patients and 30 controls. This initial screen revealed deregulation of several miRNAs. Analysis of an independent confirmation cohort of patients and 79 matched controls validated these results for several deregulated miRNAs, including miRa-5p, miR—3p, miR, miR, and miRb.
Moreover, these miRNAs were shown to have prognostic importance for this patient cohort. To assess the response to treatment, Luo et al. After 4 to 8 weeks of steroid therapy, the serum concentrations of all 5 selected miRNAs had declined significantly.
Finally, the investigators evaluated the excretion characteristics of these selected miRNAs in NS patients.Patients with nephrotic syndrome have changes in the concentrations of circulating clotting factors, which may be responsible for an increased risk of thrombus formation including renal vein thrombosis, inferior vena cava thrombosis and pulmonary emboli.
HIV patients are at risk for developing various renal diseases caused by HIV infection, viral hepatitis, drug abuse, antiretroviral drugs, and dehydration.
HIV-associated nephropathy. Patients with idiopathic nephrotic syndrome are initially treated with corticosteroids. Steroid-responsiveness is of greater prognostic use than renal histology. Nephrotic patients are at significantly increased risk of thrombosis, We searched the PubMed on-line database for all Englishlanguage papers on nephrotic syndrome published.
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Acute Respiratory Distress Syndrome, or ARDS, is a condition that causes fluid to leak into your lungs, blocking oxygen from getting to your organs.. It is serious, sometimes life-threatening, and. Introduction It is known that glucocorticoids (GCs) therapy is the treatment of choice for patients with idiopathic nephrotic syndrome (INS); however some patients fail to respond to the treatment even when given high-dose GCs.