Objective. To identify the features of the development of hormone dependence in children with hormone-sensitive nephrotic syndrome at the onset of the disease.Characteristics of children and research methods. The authors carried out a retro- and prospective study with age assessment at the onset of the disease and first relapse in 118 children with hormone-sensitive nephrotic syndrome at the onset with a rarely and often recurrent course.Results. The authors revealed a rarely and often recurrent course in 118 children with hormone-sensitive nephrotic syndrome at the onset of the disease, respectively, in 51 and 49% of cases with the development of hormone dependence (64%) and without (36%). They found statistically significant differences: the age of children at the onset of nephrotic syndrome, who developed hormone dependence and who did not develop, was 3.4 [2.2; 4.8] and 4.2 [3.0; 5.6] years accordingly (p<0.05); the age of children with nephrotic syndrome who developed hormone dependence and who did not develop hormone dependence by first relapse, was 3.8 [2.6; 5.1] and 5.7 [4.0; 7.4] years accordingly (p<0.001). The duration of the period until the first relapse after the onset in children with nephrotic syndrome, who developed hormone dependence and who did not develop hormone dependence, was 4 [4; 5] and 11 [7; 17] months, respectively (p<0.00001). Hormone dependence was revealed in 75 and 47% patients, respectively (p<0.05) among children with nephrotic syndrome who had the first relapse before 5 years of age and after 5 years of age.Conclusion. The age of children at the onset and the first relapse of nephrotic syndrome, which developed hormone dependence, is significantly less than those without hormone dependence. Children with nephrotic syndrome who have had their first relapse before 5 years of age were more likely to become hormone-dependent.

BACKGROUND. The problem of steroid-sensitive nephrotic syndrome is the developing of steroid dependency and toxicity.THE AIM: Evaluate duration of remission of steroid-dependent nephrotic syndrome (NS) after cyclosporine and mycophenolate sodium therapy in children with or without clinical manifestation of allergy.PATIENTS AND METHODS. Follow-up study with analysis of onset, clinical course and treatment includes 47 children ((31 boys (66 %) и 16 girls (34 %)) with steroid-dependent NS, 34 (72,3 %) had clinical manifestation of allergy, 13 (27,7 %) didn’t have clinical manifestation of allergy. The efficiency of therapy with cyclosporine in 16 patients and mycophenolate sodium in 27 patients with clinical manifestation of allergy is estimated in comparative study by analysis of 6, 12, 24 month remission rate after treatment. Median duration of remission of NS during 2 years after treatment is estimated. Out of 27 children 8 (29,6 %) had mycophenolate sodium treatment after cyclosporine and took part in both groups.RESULTS. Statistically significant differences in 6, 12, 24 month remission rates after cyclosporine and mycophenolate sodium treatment in children with clinical manifestation of allergy are established. Remission of NS during 6 months after mycophenolate sodium treatment was in 81,5 % (in 22 from 27 patients) unlike of that after cyclosporine – in 40 % (in 6 from 15 patients) in children with clinical manifestation of allergy (р<0,05). Remission of NS during 12 months after mycophenolate sodium treatment was in 55,6  % (in 15 from 27 patients) unlike of that after cyclosporine – 13,3 % (in 2 from 15 patients) (р<0,05) in children with clinical manifestation of allergy. Remission of NS during 24 months after mycophenolate sodium treatment was in 37 % (in 10 from 27 patients) unlike of that after cyclosporine – 6,7 % (in 1 from 15 patients) (р<0,05) in children with clinical manifestation of allergy. Median duration of remission during 2 years after treatment with cyclosporine and mycophenolate sodium in children with clinical manifestation of allergy was 7,0 [2,0-11,0] and 17,0 [6,0-24,0] months, retrospectively, (р<0,05).CONCLUSION. Remission of steroid-dependent NS during 6 months after therapy with mycophenolate sodium and cyclosporine in children was in 81,5 % and 40,0 % respectively, in children with clinical manifestation of allergy. Remission of steroid-dependent NS during 12 months after therapy with mycophenolate sodium and cyclosporine in children was in 55,6 % and 13,3 % respectively, in children with clinical manifestation of allergy. Remission of steroid-dependent NS during 24 months after therapy with mycophenolate sodium and cyclosporine in children was in 37 % and 6,7 % respectively, in children with clinical manifestation of allergy. Median duration of remission during 2 years after treatment with cyclosporine and mycophenolate sodium in children with clinical manifestation of allergy was 7,0 [2,0-11,0] and 17,0 [6,0-24,0] months, retrospectively. As the result of comparative study duration of remission of steroid-dependent NS in children with clinical manifestation of allergy was statistically significantly longer in children after therapy with mycophenolate sodium.

AIM. Evaluation in comparative study the efficiency of first choice cytostatic therapy with calcineurin inhibitor cyclosporine A and nucleotide synthesis inhibitormycophenolatemofetil (MMF)/mycophenolate sodium in children with relapsing and frequently relapsing steroid-dependent and steroid-sensitive nephrotic syndrome (NS) with steroid toxicity.PATIENTS AND METHODS. Follow-up study with analysis of onset, clinical course and treatment includes 48 children ((29 boys (60 %) и 19 girls (40 %)) with relapsing and frequently relapsing NS, developedsteroid dependence and/or steroid toxicity.The efficiency of first choice cytostatic therapy with calcineurin inhibitor cyclosporine Ain 17 patients and nucleotide synthesis inhibitormycophenolatemofetil (MMF)/mycophenolate sodium in 31 patients is estimated in comparative study by analysis of 6 month remission rate and one year remission rate after treatment.RESULTS. Statistically significant differences in 6 month and one year remission rate after first choice cytostatic therapy with MMF/ mycophenolate sodium and cyclosporine in children are established. Remission of NS during 6 months after MMF/ mycophenolate sodium treatment was in 67,7 % (in 21 from 31 patients) unlike of that after cyclosporine – in 29,4 % (in 5 from 17 patients) (р<0,05). Remission of NS during one year after MMF/ mycophenolate sodium treatment was in 58,1 % (in 18 from 31 patients) unlike of that after cyclosporine – 23,5 % (in 4 from 17 patients) (р<0,05). Cyclosporine toxicity was diagnosed in 5 from 17patients: increased creatinine (1),arterial hypertension (3), gingival hyperplasia (3) in treatment more than 12 months with reverse development after cancel. Side-effects after nucleotide synthesis inhibitor therapy was dignosed only in 1 from 31 patients (3,2 %) – lymphopenic crisis.CONCLUSION. Remission of relapsing and frequently relapsing steroid-dependent and steroid-sensitive with steroid toxicity NS during 6 months after first choice cytostatic therapy with MMF/ mycophenolate sodium and cyclosporine in children was in 67,7 % and 29,4 % respectively, during one year in 58,1 % and 23,5 % respectively. As the result of comparative study remission during 6 months and one year was statistically significant more often in children after first choice cytostatic therapy with MMF/ mycophenolate sodium.