Focal segmental glomerulosclerosis with and without nephrotic syndrome in children

Yoshikawa, Norishige; Ito, Hiroshi; Akamatsu, Ryo; Matsuyama, S; Hasegawa, Osamu; Nakahara, C; Matsuo, Tamotsu

doi:10.1016/s0022-3476(86)80574-1

Cited by 50 publications

(27 citation statements)

References 8 publications

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“…Data from these studies have shown that the mean age at presentation ranges from 5 years [7] to 12 years [28]. Most studies reported a mean age of 6-7 years at baseline [3,29]. Studies with younger age at presentation had more favourable outcomes [11,30].…”

Section: Discussionmentioning

confidence: 99%

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

et al. 2010

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Primary focal segmental glomerulosclerosis (FSGS) in children accounts for 7-20% of all cases of idiopathic nephrotic syndrome (NS). To evaluate the clinical course of children with primary FSGS, the records of 72 patients with biopsy-proven FSGS admitted between 1995 and 2008 were retrospectively analysed. Forty-eight patients were male (66.7%). The mean age at presentation was 76.5 +/- 54 (range 12-192) months. The mean duration of follow-up was 76.3 +/- 42 (range 9-156) months. Seventeen patients (23.6%) presented with gross haematuria at initial presentation, 15 (20.8%) presented with hypertension and 10 patients (14%) presented with chronic renal insufficiency (CRI). The initial response to oral prednisolone was steroid resistance in 63 patients (87.5%) and steroid dependence in 4 patients (5%), while 5 patients (6.9%) were biopsied from the start because of atypical presentation. According to pathological classification, there were the tip variant (2%), collapsing variant (6%), perihilar variant (7%) and NOS (not otherwise specified; 85%). At the last clinical visit, 12 patients (16.7%) were in complete remission and 11 (15.3%) had progressed to CRI. Renal survival rates at 5 and 10 years were 93% and 68%, respectively. In conclusion, in this study we had a low percentage of FSGS, and fewer patients presenting with gross haematuria and hypertension compared with previous reports. In addition, the short-term overall renal survival seems to be better in our cohort.

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Section: Discussionmentioning

confidence: 99%

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

et al. 2010

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“…In contrast, the persistence of nephrotic syndrome portends a reserved prognosis (15,30). Since spontaneous remission of focal segmental glomerulosclerosis is rare (7,(12)(13)(14) and an improved prognosis is associated with the remission of nephrotic syndrome, it is in the group of patients with steroid-resistant focal segmental glomerulosclerosis that cytotoxic drugs have been used in combination with prednisone.…”

Section: Discussionmentioning

confidence: 99%

“…Prednisone is the initial treatment and its therapeutic response seems to be the best predictor of outcome. Since the persistence of nephrotic syndrome is associated with a reserved prognosis and spontaneous remission is rare (7,(12)(13)(14)(15), the treatment of prednisone-resistant patients is controversial. Immunosuppressive agents in combination with steroids have been used in such patients with variable remission rates, which R. Martinelli et al…”

Section: Introductionmentioning

confidence: 99%

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis

Martinelli

Pereira

Silva

et al. 2004

Braz J Med Biol Res

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Prednisone is the initial treatment of primary focal segmental glomerulosclerosis. However, when immunosuppressive agents in combination with steroids are used in the treatment of prednisone-dependent and prednisone-resistant patients the remission rate is variable. We report a long-term trial using cyclophosphamide (2.0 to 3.0 mg/kg body weight for 12 weeks) in combination with prednisone (1.0 to 2.0 mg/kg body weight), as compared with prednisone alone for the treatment of prednisone-resistant and frequently relapsing nephrotic syndrome and focal segmental glomerulosclerosis. Fifty-four patients (34 males and 20 females) with a diagnosis of idiopathic nephrotic syndrome and focal segmental glomerulosclerosis, followed-up for an average of 86.1 ± 82.4 months, were evaluated. Complete remission occurred in 20.4% and partial remission in 14.8% of the patients treated with steroids and in 26.7 and 20.0% of the patients treated with cyclophosphamide + prednisone, respectively. Of the 24 prednisoneresistant patients treated with steroids in combination with cyclophosphamide, 33.3% obtained a complete/partial response. At the time of final evaluation, 25% of the patients treated with prednisone and 10.0% of those treated with prednisone in combination with cyclophosphamide had reached end-stage renal disease. Persistent nephrotic syndrome and progressive renal insufficiency were more frequently observed among the patients treated with prednisone alone (50.0 vs 33.3% and 33.3 vs 16.7%, respectively). The treatments were well tolerated and no patient experienced adverse reactions requiring discontinuation of medications. Although open-label and non-randomized, the present trial showed that cyclophosphamide is a reasonable choice for the treatment of primary focal segmental glomerulosclerosis and prednisone-resistant nephrotic syndrome.

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“…In particular, the site of the FSGS lesion within the glomerulus has been alleged to have signifi cance in children [17,18] and adults [19] with FSGS, those with a lesion adjacent to the hilus showing a worse prognosis than those with a 'tip' lesion at the opposite pole of the glomerulus. An immediate problem is that to identify precisely the site of the FSGS lesion, extensive serial sectioning must be undertaken, which is expensive and time consuming.…”

Section: Focal Segmental Glomerulosclerosis (Fsgs)mentioning

confidence: 99%

Treatment of Primary Glomerulonephritis Using Immunosuppressive Agents

Js¹

1989

Am J Nephrol

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Focal segmental glomerulosclerosis with and without nephrotic syndrome in children

Cited by 50 publications

References 8 publications

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis

Treatment of Primary Glomerulonephritis Using Immunosuppressive Agents

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