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Most children diagnosed with nephrotic syndrome show favourable response to corticosteroid therapy, nonetheless 30% of patients have frequent relapses or a steroid-dependent course of disease. Cyclophosphamide, cyclosporin A or MMF are being used in treatment of steroid-dependent nephrotic syndrome in search of a drug with highest long-term effectiveness and least amount of side effects., Aim: The aim of study was to assess of the efficacy of MMF as the first choice immunosuppressive drug in children with nephrotic syndrome after determining a steroid-dependency., Materials and Methods: 23 children with steroid-dependent nephrotic syndrome were enrolled in the study. Mean age at disease onset was 3.8 years. Mean disease duration time before introducing MMF was 21.3 months. Mean treatment time with MMF was 23.6 months. Patients previously treated with immunosuppressive drug, except for prednisone were excluded from the study., Results: Per year of treatment with MMF 56,5% of patients had not more than 1 relapse of the disease, 5 patients had more than 1, but less than 2 relapses. After the mean time of 23.6 months MMF treatment was discontinued in 15 patients (62,5%). 11 patients (48%) from that group significantly benefited from treatment in the form of no further relapses, defer of steroid-dependence or the possibility to reduce the dose of corticosteroids to minimal., Conclusions: MMF has advantage over cyclophosphamide and calcineurin inhibitors in reference to side effect profile, especially glomerular filtration markers, hypertension and frequent drug dependency. Treatment with MMF is effective in maintaining long-term remission and enables the reduction of cumulative steroid dose. Regarding nearly 50% of patients with benefits after MMF treatment and good treatment tolerance, it seems justified to introduce MMF as the first choice immunosuppressive drug in patients with steroiddependent nephrotic syndrome., (© 2018 MEDPRESS.)

Within recent years the number of children with focal segmental glomerulonephritis (FSGS) has increased. A significant progress in defining of molecular basis of the disease has been made. Gene mutations for nephrin, podocin, WT1, alpha-actinin 4 cause the damage of filtration barrier of glomerulus and proteinuria in consequence. A girl (S.G.) became ill at the age of 3.5, suffering form steroid-resistant nephritic syndrome (SRNS) with microscopic hematuria. The renal biopsy showed FSGS accompanied by a complete diffuse effacement of podocyte food processes. Despite intensive and regular immunosuppressive therapy, remission was not achieved. In the control renal biopsy performed a year after cyclosporin A had been applied, 50% of globally sclerosed glomeruli as well as some features of post-cyclosporin damage were found. The girl required renal replacement therapy at the age of 10.5. Dialyzed at the adult dialysis centre she died at the age of 11.5. A boy S.P. was diagnosed with SRNS when he was 11.5 years old. The renal biopsy was performed after one month of treatment and showed mesangial proliferation and diffuse effacement of podocyte food processes. After chlorambucil treatment remission was not achieved, and after methylprednisolon pulse therapy only the reduction of proteinuria was achieved. In a control renal biopsy 10 out of 13 glomeruli were globally sclerosed. At the age of 17 the patient showed chronic renal failure with a fast progression of the disease. In September 2000 the boy started renal replacement therapy, an in June 2001 he received a renal transplant without the recurrence of FGS. In 2001 a heterozygous mutation (A284V) in gene NPHS2 was found in both of the siblings. Within the confines of the clinical project ESCAPE Trial another genetic examination was performed. In the boy one missense mutation on one allele (A284V) and the R229Q polymorphism on the other allele were found. In this family the father is bear. ing the A284V mutation and the mother the R229Q variant. These results prove that this disease is due to alterations of the podocin gene in the described family.

Renovascular hypertension (RVH) accounts for 5-25% cases of secondary hypertension in children. It is the second most frequent cause of hypertension (second only to aortic coarctation), potentially treatable, provided that early diagnosis is made and no permanent organic lesions develop. No clinical symptoms are observed in more than 60% children with renovascular hypertension; hypertension itself is diagnosed incidentally, in routine checkups. The patient is often hospitalised only after organic complications. The study aim was to verify the value of Doppler examination in the diagnosis of renovascular hypertension in children. In 2001, four out of 18 patients of the Nephrology Department examined due to hypertension were diagnosed with renovascular hypertension. In three of them, examination demonstrated aneurysmatic widening of renal artery with multiple stenoses, renal artery trunk stenosis, significant stenosis in abdominal aorta and in both renal arteries. In another case, stenosis of an additional renal artery was observed. All diagnoses were made with Doppler ultrasound and confirmed by angiography. Colour and spectral Doppler ultrasound examination is a non-invasive and valuable method of diagnosing hypertension in children.