[Evaluation of the efficacy of cyclosporine A treatment in idiopathic nephrotic syndrome in children]. / Ocena skutecznosci cyklosporyny A w idiopatycznym zespole nerczycowym u dzieci.

This study reports the results of cyclosporine A (CsA) treatment in 106 children with idiopathic nephrotic syndrome. All of them had normal renal function. In 66 CsA was added to prednisone because of primary steroid-resistancy. Remission was obtained in 31 (47%) and improvement in 23 (35%). In 12 patients (18%) CsA was ineffective. Best results were obtained in youngest children (< 2 years of age) and those with minimal change disease (78% of remission). Renal function remained normal in all children in remission, while in 30% of those with improvement GFR decreased. In all children with persistent nephrotic syndrome chronic or end stage renal failure developed. 40 children were treated with CsA because of long-lasting steroid-dependency, the lack of effect of alkylating agents and steroid toxicity. In 16 of them prednisone was discontinued and in the rest given in very low doses. The steroid-toxicity effects disappeared or became less prominent in all of them. Appearance of albuminuria with decrease of the CsA dose and/or relapses of nephrotic syndrome during or after discontinuation of CsA treatment were quite frequent. For this reason the treatment course was prolonged, in some patients up to 55 months. This may result in chronic CsA nephrotoxicity. We performed second renal biopsy during remission in 28 children. The morphological signs of nephrotoxicity (grade I according to Habib and Niaudet) were considered in 5 (18%) of them.

[Verification of indications for kidney biopsy in children with steroid-dependent nephrotic syndrome]. / Weryfikacja wskazan do biopsji nerki u dzieci ze sterydozaleznym zespolem nerczycowym.

The aim of this study was to assess if cytostatic treatment of steroidodependent nephrotic syndrome in children should be preceded by renal biopsy. The result of treatment of 75 children with steroidodependent nephrotic syndrome were analysed. They were randomized for treatment with chlorambucil and cyclophosphamide and divided into two groups. Group I includes 32 children without preceding biopsy, group II, 43 children with established histopatologic diagnosis. Remission was achieved by 25 (78%) children in group I (15 (79%) of 19 treated with chlorambucil and 10(77%) of 13 treated with cyclophosphamide) and 38 (88.4%) in group II (25 (96%) of 26 treated with chlorambucil and 13 (76%) of 17 treated with cyclophosphamide). The results of therapy in children of two groups are comparable. This would indicate that a trial of cytostatic treatment can be started without previous renal biopsy.

[Uric acid metabolism abnormalities in IgA nephropathies]. / Zaburzenia metabolizmu kwasu moczowego w nefropatii IgA (doniesienie wstepne).

The possibility of uric acid tract stones forming was analysed in 12 children with IgA nephropathy (group I) and 10 children with other hematuric glomerulopathies (group II). Elevated serum uric acid level and higher urinary excretion was found only in children with IgAN during exacerbation of the disease. Uric acid nephrolithiasis was found only in children with IgA nephropathy. The prophylactic treatment (proper diet, high fluid intake, adjusting urinary pH to 6.5-6.8) was effective in decreasing the number of exacerbations, urinary tract infections and formation of uric acid stones.

[Molecular markers of hemostasis activation in children with nephrotic syndrome]. / Molekularne markery aktywacji hemostazy u dzieci z zespolem nerczycowym.

Vein and arterial thrombosis is a rather rare but potentially life-threatening complication of nephrotic syndrome (n.s.). None of the specific markers of hypercoagulability state in n.s. have been identified. The aim of the study was to estimate plasma parameters of prothrombic state in children with n.s. Ten children aged from 3 to 10 yrs (mean 5.7 +/- 2.5) with recurrence of n.s. and 10 healthy controls matched for age and sex were studied. In all children with n.s. prothrombin fragments F 1+2, D-dimers (D-d), thrombin-antithrombin III complexes (TAT) and whole blood clotting time thrombin time, prothrombin time, plasma fibrinogen and platelet count were determined in the hypovolemic state (before therapy was started), in the normovolemic state (after plasma expander was used) and in the course of anticoagulation treatment (two week dicumarol therapy). In comparison with healthy controls all children with recurrence of n.s. in the hypovolemic state showed a significant (p < 0.05) increase of D-d (910 vs 500 ng/ml), TAT (14.26 vs 2.6 ng/ml), F 1+2 (5.68 vs 0.79 nmol/l), plasma fibrinogen (592 vs 272 mg/dl), platelet count (632 vs 275 x 10(9)/l) and shortening of WBCT (30.0 vs 35 s). Plasma volume expansion produced by dekstran was followed by a moderate decrease of all parameters. Two-week anticoagulant treatment had no impact on estimated haemostasis parameters.

Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children.

A total of 184 children aged, 13 months to 11 years, suffering from their first attack of steroid-responsive nephrotic syndrome were included in a randomized study. They were treated according to three treatment protocols. All children received 1-2 mg of prednisone/kg body weight/day (up to 80 mg daily) for 4 weeks, and thereafter 1 mg/kg body weight/48 h for the next 4 weeks. Treatment was discontinued at this point in 44 children (protocol A); in 68 (protocol B) the dose was reduced by 25% each week, tapering off to 0 at the end of the third month, while in 72 children (protocol C), after the first 2 months of initial treatment the dose was reduced by 25% each month and tapered off to 0 by the end of the sixth month. All patients completed a 2-year follow-up period after withdrawal of prednisone. Treatment results were expressed as: percentage of children relapse-free within the first 6 months and 2 years after withdrawal of treatment, and average number of relapses per patient per year. The best results were obtained in children who had been treated for 6 months; 65.3% of them remained relapse-free within the first 6 months and 50% over the entire 2-year follow-up period; the number of relapses per patient per year in this group was 0.49. The respective values for children treated 2 and 3 months were: 36.4% and 32.4% for the 6-month period; 27.3% and 20.6% for the 2-year period; the numbers of relapses per patient per year were 0.79 and 0.77, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

[Evaluation of the efficacy and safety of monotherapy for significant essential hypertension in adolescents with use of enalapril]. / Ocena skutecznosci i bezpieczenstwa monoterapii znamiennego nadcisnienia tetniczego u mlodziezy przy zastosowaniu enalaprylu.

Clinical evaluation of hypotensive effectiveness and the cardiovascular and side effects of enalapril monotherapy of significant essential hypertension (SEH) in adolescents was performed. The studied group included 30 pts., aged from 12 to 18 years. The mean enalapril dose was 0.22 mg/kg/24 hours. Significant decrease of systolic, diastolic and mean arterial blood pressure was observed. ECHO examination performed after 6 months of therapy demonstrated significant decrease of intraventricular septum thickness, cardiac index and percentage of LV fractional shortening. Only a few minor side effects involving the GI tract were observed during therapy. We conclude that enalapril monotherapy is effective in adolescents with SEH and exerts a beneficial influence on sodium and purine balance and no adverse effect on the lipid profile. It can therefore be safely used in hypertensive patients with hyperuricemia and hypercholesterolemia. It causes regression of LV hypertrophy and improves exercise capacity.

[Successful autotransplantation of a solitary kidney in a 2-year old girl with malignant hypertension caused by critical renal artery stenosis]. / Udana autotransplantacja jedynej nerki u 2-letniej dziewczynki ze zlosliwym nadcisnieniem tetniczym spowodowanym krytycznym zwezeniem tetnicy nerkowej.

A 22-month-old girl with malignant hypertension caused by critical stenosis of the right renal artery resistant to all of pharmacological treatment is described. Left renal function was lost in the course of the hypertensive crisis. The child was successfully treated by autotransplantation of the right kidney.

[Evaluation of the efficacy and tolerance of three antihypertensive agents used as single-drug therapy, nifedipine, prazosin and acebutolol in severe, idiopathic hypertension in adolescents]. / Evaluation de l'efficacité et de la tolérance de trois antihypertenseurs utilisés en monothérapie, nifédipine, prazosin et acébutolol dans l'hypertension idiopathique grave de l'adolescent.

The antihypertensive efficacy of single-drug therapy with nifedipine (N), prazosin (P), or acebutolol (A) and the influence of these agents on coronary risk factors including hypoglycemia, hyperuricemia, and hyperlipidemia, were studied in adolescents with hypertension. Ninety patients (73 girls and 17 boys) aged 14 to 18 years with idiopathic hypertension (IH) were randomized into three groups. Each group received N, P, or A as single-drug therapy for six months. Systolic and diastolic blood pressures fell in all three groups, from 152/90 mmHg to 127/70 mmHg* with N, from 150/90 mmHg to 121/70 mmHg* with P, and from 148/92 mmHg to 122/74 mmHg* mmHg with A. In 17% of cases, N failed to reduce blood pressures below the 90th centiles. Heart rate was not influenced by N or P but decreased from 84 to 75 bpm with A. Although none of the drugs modified serum uric acid levels, fractional uric acid secretion rose with P and A (from 4.1% to 6% with P; and from 4.4% to 6% with A). The lipid profile remained unchanged under N and P, whereas a decrease in serum LDL-cholesterol from 99.6 to 88.8% mg* was seen with A. Fasting serum glucose levels increased from 86.4 to 92.7 mg %* in the group given A. N, P, and A are suitable for single-drug therapy of IH in adolescents; the most appropriate drug should be selected on the basis of medical history.

[Difficulties in the diagnosis of pheochromocytoma in children]. / Trudnosci diagnostyczne w rozpoznawaniu guza chromochlonnego u dzieci.

Pheochromocytoma was the cause of arterial hypertension observed in 0.9% of children treated in 1982-1989. Out of clinical features the most characteristic was sustained hypertension often complicated by the accelerated phase of malignant hypertension and encephalopathy. Sustained tachycardia was also found in all patients. Increased urinary excretion of catecholamines and its metabolites confirmed the diagnosis in all cases. The most sensitive and specific methods for tumor diagnosis were ultrasonography and computer tomography of the adrenals while scintigraphy with meta-iodobenzylguanidine+ labelled with iodine-131 radioisotope gave a high percentage of false negative results.

A single pediatric center experience with 1025 children with hypertension.

Between January 1982 and December 1989 1025 patients aged between one month and 18 years with increased blood pressure were referred for evaluation. Borderline hypertension was found in 389 children; 636 had sustained significant hypertension. In 351 patients, hypertension was secondary to a known disease. Renal parenchymal diseases were present in 68% of patients while renovascular and endocrine disorders were found in 10% and 11%, respectively. Of the 258 children aged less than 15 years, all but six children had known causes of hypertension, while 75% of adolescents had essential hypertension. In the 389 children with borderline hypertension, 65% developed fixed hypertension over a period of 2-3 years.

[Primary hyperaldosteronism suppressed after glucocorticoid administration]. / Pierwotny hiperaldosteronizm hamowany podawaniem glikokortykoidów.

Authors present a case of glucocorticoid suppressible hyperaldosteronism in 18 year old female. Unmeasurable low plasma renin activity and marked increase in aldosterone concentration was established. After administration of dexamethasone, normalization of aldosterone concentration and blood pressure has been observed.

[Renin activity and aldosterone level in the plasma of children with lipoid nephrosis]. / Aktywnosc reninowa i poziom aldosteronu w osoczu dzieci chorych na nerczyce lipidowa.

In 16 children with steroid-responsive nephrotic syndrome (with minimal changes in the glomeruli) the plasma renin activity and aldosterone level were determined during recurrence of the nephrotic syndrome at the stage of oedema increase (FEN alpha 0.25%) and in early period of remission. Plasma renin activity was raised in all cases suggesting presence of hypovolaemia. Since it was not possible to establish a correlation between plasma renin activity and albumin level, as well as between this activity and aldosterone level, and aldosterone level and sodium excretion it may be surmised that the renin-angiotensin-aldosterone system has no decisive role in the pathogenesis of the nephrotic syndrome. The mechanism of oedema development is doubtlessly more complex.

Pheochromocytoma in children: difficulties in diagnosis and localization.

Surgically confirmed pheochromocytoma was the cause of arterial hypertension in 6 out of 668 (0.8%) children with significant hypertension admitted to Child Health Centre in Warsaw. Among clinical features most characteristic was sustained hypertension observed in all patients, often complicated by the accelerated phase of malignant hypertension and encephalopathy. Sustained tachycardia was also found in all patients. Elevated sedimentation rate and electrocardiographic changes were observed in each child while other abnormal laboratory findings such as hyperglycemia, etc. occurred at similar rate as in adults. Increased urinary excretion of catecholamines and their metabolites confirmed the diagnosis. In our study the most sensitive methods for tumor localization were ultrasonography and computed tomography of the adrenals while scintigraphy with iodo-131-metabenzylguanidine gave a high percentage of false negative results. Clinical presentation of pheochromocytomas in children is different than in adults and all pediatric patients with severe hypertension should be screened for this disease.