Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood.

BACKGROUND: The aim of the study was to assess idiopathic nephrotic syndrome (INS) relapse rate, co-morbidities, and social status of adults diagnosed with INS in childhood. MATERIAL AND METHODS: A written questionnaire was sent to 118 adults treated for INS in childhood. In 61 (51.7%) responders (aged 26.0 ± 6.2 years, range 18 - 51.5 years), we used available medical records to evaluate age at the onset of INS, number of INS relapses below 18 years of age, response to corticosteroids (CS), renal biopsy findings, and immunosuppressive treatment as well as questionnaire to evaluate the number and treatment of INS relapses above 18 years of age, co-morbidities, age at menarche, marital status, offspring, educational status, and occupation. RESULTS: In the group of 61 responders, median age at the onset of INS was 3 (range 1.3 - 14.0) years, median number of INS relapses at < 18 years of age was 5 (1 - 20). Steroid-sensitive nephrotic syndrome (SSNS) was diagnosed in 37 (60.7%) patients, steroid-dependent nephrotic syndrome SDNS in 18 (29.5%) patients, and steroid-resistant nephrotic syndrome (SRNS) in 6 (9.8%) patients. Mesangial proliferation was the most common pattern in renal biopsy (35.7%). All patients received CS, 15 were treated with methylprednisolone pulses, 13 with cyclophosphamide, 11 with chlorambucil, 2 with cyclosporine, and 21 with levamisole. All patients achieved remission and had normal renal function at the age of 18. In adulthood, INS relapsed in 10 (16.4%) patients, including 5 (13.5%) patients with SSNS, 4 (22.2%) with SDNS, and 1 (16.7%) with SRNS (p = 0.72). Median number of relapses was 2 (range 1 - 11). Patients with relapses at > 18 years of age had more (p < 0.005) relapses at < 18 years of age. Hypertension was diagnosed in 8 (16.1%), overweight in 14 (23.0%), obesity in 3 (4.9%), and bone fractures in 12 (19.7%) patients. Five patients had height < 3rd percentile, including 4 with INS onset at < 3 years of age. One patient had growth retardation before the treatment. No myocardial infarctions, strokes, severe infections, or malignancies were reported. Mean age at menarche was 12.9 ± 1.4 years, 37 (60.7%) patients were in a steady relationship/ married, 1/18 (5.6%) patients treated with cytostatic agents and 12/43 (24/7%) patients not treated with cytostatic agents had offspring (p < 0.05). Elementary education was reported by 4 (6.6%), secondary education by 32 (52.5%), and higher education by 25 (40.9%) patients, and 34 (55.7%) patients were professionally active. None of the 6 patients with SRNS developed end-stage renal disease. CONCLUSIONS: 1. High number of INS relapses in childhood is a risk factor for recurrences in adulthood. 2. INS relapses in childhood do not preclude active professional life in adulthood.

[Significance of early diagnosis of posterior urethral valves in fetus for further development - own experience]. / Znaczenie wczesnego rozpoznawania zastawek cewki tylnej u plodu dla dalszych losów dziecka- doswiadczenia wlasne.

The incidence of posterior urethral valves is estimated to be from 3:1000 to 8:1000 and this is one of the most common causes of obstruction of urinary tract in boys. About 13-17% of children with posterior urethral valves develop end stage renal failure. We present a  6-month-old boy with late diagnosis of posterior urtehral valves. Antenatal ultrasound investigation of the urinary tract was normal. A small degree of oligohydramnios was found during delivery. At the age of six months the boy was admitted to hospital because of urinary tract infection, hypertension (130/90 mmHg) and acute kidney injury (urea - 46 mg/dL, creatinine - 1.1 mg/dL, GFR - 35.5 mL/min/1.73 m2 ). Bilateral hydronephrosis and megaureters, low-capacity bladder with hypertrophied wall were seen on ultrasound examination. Voiding cystourethrograhy revealed vesicoureteral refluxes (III/V), hypertrophy of the bladder wall with numerous diverticula and dilated posterior urethra. During urethroscopy urethral valves were resected. Increased intravesical pressure (leak point up to 305 cm H2 O) was found on urodynamic test. Renal scintigraphy (99mTc-EC) revealed decreased intake of isotope in the left kidney (5%), and the right kidney intake was 95% ERPF. The patient was qualified for left-sided nephrectomy, which was postponed because of high leak point and high risk of worsening of vesicoureteral reflux to right kidney after nephrectomy. Anticholinergic and α-blocker treatment was started. At the age of 11 months left-side nephrectomy was performed because of recurrent urinary tract infections. After 3.5-year follow-up blood pressure, physical development, kidney function tests, and urinalysis are normal. Additionally to this investigation the significance of early diagnosis including prenatal (PUV) for further development as well as further therapeutic procedure is discussed.

[Vitamin D status in children with renal diseases]. / Zasoby witaminy D u dzieci chorobami nerek.

AIM: Assessment of vitamin D status in children with selected renal diseases based on serum 25OHD3 level taking into consideration type of kidney disease, vitamin D supplementation and season of the year. MATERIAL AND METHODS: Retrospective analysis of 127 children aged from 0.6 to 17.7 years, median 9.5 [24 with risk status of urolithiasis (RSU), 29 with urolithiasis, 15 with glomerulonephritis, 59 with idiopathic nephrotic syndrome (INS)] was performed. Serum concentrations of 25OHD3, 1,25(OH)2 D3, calcium, and phosphorus were measured. The correlation of 25OHD3 with the type of kidney disease, supplementation of vitamin D, seasons, gender, age, and the dose of glucocorticosteroids (in children with glomerulopathies) were analyzed. RESULTS: In all children serum concentration of 25OHD3 from 4.3 to 72.6 ng/mL (median 21.1 ng/mL). The deficiency or insufficiency of vitamin D were observed in 55 (43.3%) analyzed children with kidney diseases. The deficiency or insufficiency of vitamin D were observed in 49.1% children with RSU and urolithiasis, and in 39.2% with glomerulonephritis and INS: in 36% children supplemented with vitamin D and 54% not supplemented (NS). In winter, 25OHD3 serum concentration was significantly higher in children supplemented with vitamin D compared to not supplemented [median 21.5 ng/mL vs 16.5 ng/mL (p<0.05)]. There were no significant differences in serum concentrations of 25OHD3 , 1,25(OH)2 D3 , calcium, phosphorus and calcium x phophorus product depending on type of kidney disease and gender. The significant negative correlation was found between 25OHD3 and patients' age (r=-0.26 p<0.01) and between calcium (r=0.31, p<0.05) and calcium x phosphorus in children supplemented with vitamin D (r=0.28, p<0.05). CONCLUSIONS: Our results suggest the necessity to develop new strategies in vitamin D supplementation in children with kidney diseases. Futher studies shoud also be performed to evaluate their efficiency.

[Efficacy and safety of cyclosporine in the treatment of inflammatory bowel disease in children - a retrospective study]. / Retrospektywna ocena skutczscii bezpieczenstwa leczenia cyklosporyna dzieci z nieswoistym zapaleniem jelit.

AIM: the aim of this study was to report single centre experience with cyclosporine used in treatment of children with inflammatory bowel disease with regard to safety and efficacy. METHODS: retrospective analysis included 23 patients, 21 with ulcerative colitis and 2 with Crohn's disease, aged 2.75 to 18.5 years. They were treated with cyclosporine during the last 5 years. Before cyclosporine therapy was started they received steroids and azathioprine. Cyclosporine treatment was given in severe steroid-resistant exacerbation of the disease (n = 10) or steroid-dependence (n = 13). Cyclosporine dose was set to obtain therapeutic levels (serum concentration > 100 ng/ml and < 200 ng/ml). RESULTS: Cyclosporine treatment was continued up to 2 months in 6 cases, 2 to 6 months in 8 patients and more than 6 months in 9 patients. Complications were reported in 2 patients: hirsutism and gingival hypertrophy. Cyclosporine treatment was stopped in the second case. None of the two patients with Crohn's disease improved during the treatment. Short-term improvement was observed in 11 patients with ulcerative colitis. Long-term recovery (> 6 months) was obtained in 6 cases. In 10 patients with severe exacerbation of ulcerative colitis colectomy was performed, in 4 of them elective surgery was performed when the clinical status improved. CONCLUSION: cyclosporine appears to be a safe and relatively effective treatment of ulcerative colitis in children. Cyclosporine is less effective in maintaining remission and it did not allow to avoid colectomy in severe exacerbation. Still case controlled studies are needed to show the efficacy of this treatment.