Steroids steroid-resistant nephrotic syndrome

Cyclosporin is usually given orally, although absorption is often unpredictable. The intravenous route is usually restricted to patients who cannot take the drug orally, because of the risk of anaphylactic reactions. Other uses of cyclosporin include psoriasis and severe, active rheumatoid arthritis when these do not respond to conventional treatment, and steroid-resistant nephrotic syndrome. 

Recently, mutation of the NPHS2 gene (encoding the protein podocin) has been found in nearly 30% of patients with sporadic steroid-resistant nephrotic syndrome (K6). It is possible that a substantial portion of steroid-resistant patients with FSGS may have sporadic mutations of several different genes for (yet-unrecognized) podocyte proteins. 

K6. Karle, S. M., Uetz, B., Ronner, V., Glaeser, L., Hildebrandt, F., and Fuchshuber, A., Novel mutations in NPHS2 detected in both familial and sporadic steroid-resistant nephrotic syndrome. J. Am. Soc. Nephrol. 13, 388-393 (2002). 

Tenbrock K, Muller-Berghans J, Fnchshnber A, Michalk D, Querfeld U. Levamisole treatment in steroid-sensitive and steroid-resistant nephrotic syndrome. Pediatr Nephrol 1998 12(6) 459-62. 

Shibasaki T, Koyama A, Hishida A, Muso E, Osawa G, Yamabe H, Shiiki H, Makino H, Sato H, Ishikawa I, Maeda K, Tomita K, Arakawa M, Ishida M, Sato M, Nagase M, Kashihara N, Yorioka N, Koike T, Saito T, Harada T, Mitarai T, Sugisaki T, Nagasawa T, Tomino Y, Nojima Y, Kobayashi Y, Sakai O. A randomized open-label comparative study of conventional therapy versus mizoribine onlay therapy in patients with steroid-resistant nephrotic syndrome (postmarketing survey). Clin Exp Nephrol 2004 8(2) 117-26. 

Antignac C Molecular basis of steroid-resistant nephrotic syndrome. Nefrologia 2005 25 SuppI 2 25-8. 

Boute N et al (2000) NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat Genet 24(4) 349-354... 

In 11 children with nephrotic syndrome, of whom five were glucocorticoid-sensitive, six glucocorticoid-resistant, and all resistant to other immunosuppressive drugs, levamisole 2.5 mg/kg was given every 48 hours for up to 18 months (7). Two patients were also given ciclosporin. All the patients in the steroid-sensitive group but none in the steroid-resistant group reacted favorably to levamisole, with disappearance of protein from the urine. There... 

Sharma RK, Sahu KM, Gulati S, Gupta A. Pefloxacin in steroid dependent and resistant idiopathic nephrotic syndrome. J Nephrol 2000 13(4) 271. ... 

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