Steroid-resistant nephrotic syndrome

Glucocorticoid (GC) responsiveness represents a continuous spectrum, with GC-resistant individuals falling at one end of a unimodal distribution. Patients with severe asthma who are poorly responsive to high doses of GCs and are without confounding factors ( table 1 ) have been termed GC-resistant [ 1 ]. A larger subset of patients with asthma that is poorly-controlled despite optimal treatment or who experience worsening of asthma control during GC withdrawal have severe asthma and are considered relatively GC-insensitive [ 1 ]. High doses of GCs usually indicate a daily dose of 1000 microg or more of inhaled fluticasone propionate or 2000 microg or more of triamcinolone, the equivalent ( table 2 ).

When testing, our laboratory analyzes all of the coding DNA in a gene to determine if any disease-causing pathogenic variants are present. By sequencing all of the coding DNA in a gene, instead of just a portion, we are able to offer the most accurate genetic testing available, regardless of your ethnicity. The majority of laboratories are only sequencing a portion of the gene, leaving room for error with missed pathogenic variants, especially when testing a variety of ethnicities. By sequencing the entire gene, NxGen MDx testing eliminates the doubt in a negative result and drastically reduces the residual risk, regardless of ethnicity.

For a subset of patients with skin manifestations of DM, trials of multiple therapies (in combination or sequentially) are required to identify the treatment regimen that produces satisfactory disease control. Because many patients fail to improve adequately with photoprotection, antipruritics, and topical corticosteroids or topical calcineurin inhibitors, the addition of systemic antimalarials or methotrexate is often necessary. Patients who fail to respond to these conventional interventions or who relapse after an initial response have refractory disease and require the initiation of more aggressive therapies. (See "Initial management of cutaneous dermatomyositis in adults", section on 'Treatment' .)

Steroid-resistant nephrotic syndrome

steroid-resistant nephrotic syndrome


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