[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.

[Vasoactive peptides, endothelin 1 and neuropeptide Y, and total peripheral vascular resistance in children with essential hypertension]. / Stezenie wazoaktywnych peptydów endoteliny 1 i neuropeptydu Y a calkowity obwodowy opór naczyniowy u dzieci z samoistnym nadcisnieniem tetniczym.

Cardiac function was evaluated in rest and after exercise on a cycloergometer in 20 patients with essential hypertension (EH) aged 14 to 19 years and 12 age-matched healthy volunteers. Selected parameters of ECHO examination and mitral flow were assessed, including SV, CO, CI, %SF, EF, LIVDd, IVSd, LVPWd, LVMI, total peripheral vascular resistance, ESS, velocity of wave E. A and E/A index. In all cases, serum ET1 and NPY levels were measured in rest and after exercise, before and after 6-month enalapril therapy. The ETI serum level in hypertensive patients did not differ from controls, while the NPY level was significantly higher in hypertensives. Exercise did not affect the ETI serum concentration, however, it did increase the NPY level. Enalapril therapy had no effect on serum peptide concentrations. Correlation of the ETI serum level with ECHO parameters, including CO, Cl, SV, LIVDd, LVPWd, %SF, TPRI and wave E velocity, as well as correlation of NPY concentration with LIVDd, LVPWd, LVMI, ESS and wave E and A velocity may suggest that these peptides influence left ventricle function and structure disturbances in children with EH.

[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.

[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.

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.