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Genetic forms of nephrotic syndrome are typically resistant to steroid and other immunosuppressive treatment. [4] Goals of therapy are to control urinary protein loss and swelling, provide good nutrition to allow the child to grow, and prevent complications. [1] Early and aggressive treatment is required to control the disorder.
Childhood-onset nephrotic syndrome differs from the adult nephrotic syndrome in that the former often has a single common cause that typically responds well to steroid treatment. In adults, there are numerous potential causes, which makes an early kidney biopsy necessary to determine the correct diagnosis and treatment plan. [4]
The prognosis for nephrotic syndrome under treatment is generally good although this depends on the underlying cause, the age of the person and their response to treatment. It is usually good in children, because minimal change disease responds very well to steroids and does not cause chronic kidney failure .
Presentation with nephrotic syndrome can resolve with treatment, but can also progress. [10] Patients can become resistant to steroids or specific immunosuppressive agents, in which case it may be necessary to use different immunosuppressive agents. [2]
Minimal change disease (MCD), also known as lipoid nephrosis or nil disease, among others, is a disease affecting the kidneys which causes nephrotic syndrome. [1] Nephrotic syndrome leads to the loss of significant amounts of protein to the urine (proteinuria), which causes the widespread edema (soft tissue swelling) and impaired kidney function commonly experienced by those affected by the ...
It is a promising treatment of renal diseases with proteinuria, in particular patients with resistance or partial response to rituximab. [10] A single low-dose infusion of Obinutuzumab, found to be effective and safe in inducing prolonged remission in children with steroid-dependent or frequently relapsing nephrotic syndrome.
Because females do not have all the features of the condition (e.g. gonadal dysgenesis), females are usually given the diagnosis of isolated nephrotic syndrome Medline ref. Frasier syndrome in some infants may therefore go unrecognized until the affected child presents with signs of renal impairment and further testing is undertaken to evaluate ...
A meta-analysis of four randomized controlled trials comparing treatments of membranous nephropathy showed that regimes comprising chlorambucil or cyclophosphamide, either alone or with steroids, were more effective than symptomatic treatment or treatment with steroids alone in inducing remission of the nephrotic syndrome. [citation needed]