Steroid-resistant primary focal segmental glomerulosclerosis

The Expert Group emphasized that while all patients with SRNS should initially be referred to a pediatric nephrologist for evaluation, the subsequent care might be collaborative involving the primary pediatrician and the nephrologist. Following the diagnosis of SRNS (lack of remission despite treatment with prednisolone at 2 mg/kg/day for 4 weeks), all patients (with initial or late resistance) should undergo a renal biopsy, before instituting specific treatment. Patients with idiopathic SRNS secondary to minimal change disease or focal segmental glomerulosclerosis should receive similar therapy. Effective regimens include treatment with calcineurin inhibitors (tacrolimus, cyclosporine), intra-venous cyclophosphamide or a combination of pulse corticosteroids with oral cyclophosphamide, and tapering doses of alternate day corticosteroids. Supportive management comprises of, when indicated, therapy with angiotensin converting enzyme inhibitors and statins. It is expected that these guidelines shall enable standardization of care for patients with SRNS in the country.

Elward: We thought with the SAMs there was an opportunity to convey the essential content of the guidelines to primary care physicians. With the SAMs, we could reach a broad audience and use those 60 questions not just to review asthma and its pathology, but also to really target the key guidelines. In addition, we could give them some of the tools that could be used for optimal asthma care, to teach them really how easy it would be to use the Asthma Control Test™ (ACT) or the Asthma Therapy Assessment Questionnaire© (ATAQ), show them the asthma action plan and how it might be filled out, and explain the rationale behind each of the key messages in the context of primary care. We also thought that it was a great opportunity, given that we’re really dealing with front–line clinicians—people who might not go out of their way to go to a conference on asthma guidelines—to reach them and learn what barriers and what challenges they have in their use of the NAEPP guidelines.  

In the initial studies of renal allograft rejection, potentially fatal, severe pulmonary edema occurred in 5% of the initial 107 patients. Fluid overload was present before treatment in all of these cases. It occurred in none of the subsequent 311 patients treated with first-dose volume/weight restrictions. In subsequent trials and in post-marketing experience, severe pulmonary edema has occurred in patients who appeared to be euvolemic. The pathogenesis of pulmonary edema may involve all or some of the following: volume overload; increased pulmonary vascular permeability; and/or reduced left ventricular compliance/contractility. During the first 1 to 3 days of ORTHOCLONE OKT3 therapy, some patients have experienced an acute and transient decline in the glomerular filtration rate (GFR) and diminished urine output with a resulting increase in the level of serum creatinine. Massive release of cytokines appears to lead to reversible renal functional impairment and/or delayed renal allograft function. Similarly, transient elevations in hepatic transaminases have been reported following administration of the first few doses of ORTHOCLONE OKT3.

Steroid-resistant primary focal segmental glomerulosclerosis

steroid-resistant primary focal segmental glomerulosclerosis


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