[The Lesch-Nyhan syndrome]. / Les-Nijanov (Lesch-Nyhan) sindrom.

Deficiency of hypoxanthine phosphoribosyltransferase (HPRT) has a broad spectrum of clinical manifestations, from the complete enzyme defect, the Lesch-Nyhan syndrome with severe neurological deficiency to the partial defect associated only with uric acid overproduction and its consequences. We present a 5-year old boy with Lesch-Nyhan syndrome. He came to our hospital because of abdominal pain, vomiting and gross haematuria. At the age of 8 months he was categorized as a "cerebral palsy" patient due to involuntary movements and high degree of spastically and tonic spasms. He remained incapable of sitting or standing alone. The patient's brother and two uncles were also categorized as "cerebral palsy" cases and died at the age of 8-14 years. Clinical examination revealed hyperuricaemia and hyperuricosuria, radiolucent renal and urinary bladder stones. HPRT enzyme activity was totally absent, while adenine phosphoribosyl transferase activity was increased compared to control. The patient was treated with allopurinol, urinary alkalization, low-purine diet and adequate hydration while he was in hospital. However, his parents refused further treatment and follow-up. The most important issue is whether the healthy sisters of the patients are heterozygotes for HPRT deficiency. This DNA analysis is now in progress.

[Continuous ambulatory measurement of blood pressure in children--personal experience]. / Kontinuisano ambulatorno merenje krvnog pritiska kod dece--nasa iskustva.

UNLABELLED: Ambulatory blood pressure monitoring (ABPM) during normal daily activities and during night, when the patient is asleep, is a new method of measuring blood pressure (BP) in children, used for better diagnosis and treatment of hypertension. Compared to casual BP measurements, it documents normal daily BP variations, BP during sleep, the influence of emotional and physical stress on BP and is a better predictor of hypertension associated with end-organ damage. However, the experience in ABPM in children is still limited. In our country ABPM has been used since recently, and first results are referred to children with end-stage renal failure. SUBJECTS AND METHODS: ABPM was performed in two groups of children: group A consisted of 61 children, aged 14.3 +/- 2.9 (mean +/- SD) yrs in whom intermittent outpatient BP measurements (for at least 3 months) suggested the diagnosis of hypertension (according to the data of Second Task Force); group B consisted of 52 patients (pts), aged 12.8 +/- 4.6 yr with renal disease. Four pts from group A (6.6%) and 20 pts from group B (38.5%) received antihypertensive therapy (captopril, nifedipine, furosemide and propranolol ). All children from group A and half of the children from group B had normal renal function. Eighteen pts from group B were on chronic haemodialysis (34.6%). Blood pressure was recorded during a 24-hour period except in haemodialyzed pts (48 h) (Table 1). Results of BP measurements are presented as the mean values of BP during a 24-hour period, during normal daily activities and during sleep. We used the age- and gender-appropriate 95th percentile from the Task Force Study as the daytime upper-limit of normal and 10% lower for the upper-limit at night. According to BP load (the percentage of BPs exceeding the upper limits of normal for age), children were assumed to have mild-to-moderate hypertension (BP load between 20% and 40%) or severe hypertension (BP load more than 40%). The success of antihypertensive therapy was evaluated after 1-3 months in 11 pts (twice in 10 pts and three times in one pt). RESULTS: In group A 39.4% of pts were normotensive and 36.1% were without antihypertensive therapy, 58.4% of normotensive and 40.5% of hypertensive pts had blunted circadian BP rhythm (nocturnal BP reduction of less than 10% of diurnal values) (Graph. 1). In group B 38.5% of pts were normotensive and 27% were without antihypertensive therapy. In the group of normotensive pts alteration of circadian BP rhythm was found in 40% of pts with normal renal function, 80% of pts with chronic renal failure and in 100% of pts with terminal renal failure, while in the hypertensive group, altered circadian BP rhythm had 68%, 100% and 92% of pts, respectively (Graph 2). Mild-to-moderate hypertension had 54% of hypertensive pts from group A and 37.5% of hypertensive pts from group B. Severe hypertension was more frequent in group B (62.5%) comparing to group A (46%). The effectiveness of antihypertensive therapy was assessed in 11 pts. In 69.2% of pts BP became normal or was significantly decreased, in 23.1% of pts BP was not changed and 7.7% of pts had higher values of BP. DISCUSSION: ABPM is very useful for diagnosing white coat hypertension. Like other authors, we have pointed out that more than one third of pts who were hypertensive according to usual BP measurements had normal 24-hour BP and we classified them as white coat hypertensives. More than a half of the pts had blunted circadian BP rhythm, and as it is not certain whether they will become hypertensive in adulthood they should be periodically controlled. There are several proofs that results of ABPM have a better correlation with hypertensive end-organ damage; therefore ABPM is used for assessing the severity of hypertension. In our former work, we showed excellent correlation of BP with left ventricular mass index in children with end-stage renal failure. (ABSTRACT TRUNCATED)

[Serum osteocalcin in children with chronic renal insufficiency]. / Osteokalcin u serumu kod dece s hronicnom insuficijencijom bubrega.

UNLABELLED: The research of the bone metabolism has undergone a long evolution which began with the use of radioisotopes in calcium kinetic studies and went through the determination of several humoral parameters like alkaline phosphatase (ALP), hydroxyproline and intact immunoreactive parathyroid hormone (iPTH) and finally to the assay of a new serum and urinary parameters of bone metabolism, like osteocalcine (OC) and procollagen and collagen metabolites. The X-ray study of the skeleton, densitometric techniques, computerized tomography, scintigraphy and NMR are used for visualization of bone changes, but bone biopsy and histomorphometry provide the most precise evaluation [1]. Disorders of bone and mineral metabolism in children with chronic renal failure (CRF) are an almost regular occurrence; so early discovery and treatment of these changes are very important [2]. The aim of this study was to measure the serum OC level in children with CRF and terminal renal failure (TRF), treated with chronic haemodialysis, and to evaluate the significance of OC compared to other humoral parameters of renal osteodistrophy, such as ALP and iPTH. MATERIALS AND METHODS: We studied the fasting levels of OC in three different groups of children: group A consisted of 18 patients with TRF; group B consisted of 12 patients at different stages of CRF, and group C consisted of 32 healthy children, all of the approximately same age. Clinical characteristics of the examinded children are presented in Table 1. Of 30 patients, 26 were treated with calcium carbonate and 21 with vitamin D analogues. None were treated with aluminium hydroxide. Additional parameters included serum calcium, phosphate, ALP and body height, while serum concentrations of iPTH and ionized serum calcium were measured only in group A. Serum OC was measured by radioimmunoassay using OSTK PR RIA (CIS), while ELISA-PTH (CIS) radioimmunoassay was used to determine iPTH plasma levels. Statistical analyses were performed using Kolmogorov-Smirnov test to confirm normal distribution, the Pearson and Spearman rank sum test for correlation between variables of interest, while analysis of variance was used to compare the findings. RESULTS: Serum OC levels were significantly different in all groups (p < 0.01); they were three times higher in group A than in group C. Similar increase was noticed in plasma iPTH, assuming that "normal" uremic iPTH was raised up to threefold above normal range (between 10 and 60 pg/ml) [2]. However, the total serum ALP activity was not sensitive as OC and iPTH, since ALP increases were less as compared to them. OC was age related only in group A (p < 0.01), with a positive correlation between OC and duration of haemodialysis (p < 0.05), as well as between OC and serum phosphate (p < 0.05), but there was no correlation between OC and growth retardation (expressed by SDS), bone age and current therapy for renal osteodistrophy. A direct correlation between OC and ALP was found only in healthy children (p < 0.01), while in groups A and B it was remarkable, although not statistically significant (p = 0.08) (Graphs 1, 2, 3). In group A, ALP and iPTH were directly correlated (p < 0.001), but the correlation of OC with iPTH was less significant (p = 0.06). In patients with CRF no correlation was found between glomerular filtration rate and OC. DISCUSSION: OC is a bone-derived noncollagenous protein of low molecular weight (about 5800 D), containing residues of the vitamin K dependent amino acid gamma-carboxyglutamic acid and is synthesized by osteoblasts and odontoblasts. Calcitriol is a potent stimulator of OC synthesis, acting at the transcriptional level and increasing mRNA severalfold. OC is found mainly in bone, but nanomolar concentrations circulate in the blood. Its serum levels are an expression of the bone formation process and are age related (higher in the neonatal and adolescent period). ABSTRACT TRUNCATED.

[Kidney transplantation in children]. / Transplantacija bubrega kod dece.

During the last 14 years, since the opening of the Center for Childrens' Hemodialysis the University Children's Hospital of Belgrade, 174 children with terminal renal failure were treated by chronic hemodialysis. Of these, only 35 patients (20.1%), 14 girls and 21 boys, mean age 12.6 +/- 4.9 years, were transplanted. The average waiting time for transplantation was 24.6 months (range 0-120 months). Twenty-five patients were transplanted with paternal kidney, the others were cadaveric transplantations. More than half of transplantations were done abroad. Of 35 patients, 20 were under follow-up for over 6 months (45.7 +/- 7.2 range 6 to 102 months). Five year patient survival was 94.1% and the graft survival over the same period was 76.4%. 45.7 +/- 7.2 months after transplantation the outcome was the following: 11 patients (55%) had GFR over 60 ml/min/1.73 m2, 5 patients (25%) had GFR from 25 to 60 ml/min/1.73 m2, 2 patients (10%) returned to chronic hemodialysis, 1 patient was retransplanted and 1 patient died. All patients with GFR of over 60 ml/min/1.73 m2 are well psychosocially rehabilitated, 62.4% are normotensive without antihypertensive therapy, and only 9.9% are growth retarded.

[Cystic kidney disease--genetics, pathogenesis and clinical aspects in children]. / Cisticke bolesti bubrega--genetika, patogeneza i klinicka slika u decjem dobu.

This paper reviews recent studies producing insight into genetics and cellular abnormalities causing kidney cysts, their growth and development. Clinical features of various cystic kidney diseases in our patients are described. Special attention has been paid to those rarely reported in our literature. Important discovery concerns location of the gene for autosomal dominant polycystic kidney disease (ADPKD) 1 and 2 on the short arms of chromosome 16 and 4 respectively, as well as for autosomal recessive polycystic kidney disease (ARPKD) on chromosome 6 and for juvenile nephronophtisis on the short arm of chromosome 2. Two basic abnormalities necessary for cyst formation are increased: epithelial cell proliferation and altered fluid transport. Mitogenic action of epidermal growth factor (EGF) is significantly increased and EGF receptors have been demonstrated on apical as well as on basal surface of cyst lining epithelium. TGF-beta shows marked loos of inhibitory activity with regard to EGF. Cystic epithelium has altered polarity; Na-K-ATP-ase is located exclusively on the apical cell membrane. Tubular basement membrane shows alteration in structural components. Complex medullar cystic disease--nephronophtisis, complex as well as the hepatorenal complex of nephoronophtisis--congenital hepatic fibrosis are emphasized in this paper. The later has proved to be rather frequent in our population. We described a distinctive variant of hepato-renal disorder in 4 patients and reviewed 5 similar patients in the literature. The main characteristics are progressive tubulointerstitial nephritis and cholestatic liver disease. We strongly suggest that this variant represents a new syndrome (Popovic-Rolovic M, Kostic M, Sindic M. et al Progressive tubulointerstitialnephritis and chronic cholestatic liver disease.

[Cystic tumefaction in the minor pelvis--the agenesis-dysplasia syndrome (case report)]. / Cisticka tumefakcija u maloj karlici--sindrom agenezije-displazije (prikaz bolesnika)]

UNLABELLED: Ectopic kidney often shows signs of parenchyma maldifferentiation. Multicystic or dysplastic kidney is usually associated with congenital urogenital and skeletal system anomalies. In the Unilateral form of the agenesia-dysplasis syndrome, the kidney, if it is present, is small dysplastic and usually cystically changed. Ipsilateral uterus horn or vaginal agenesia are the most frequently associated anomalies. Case report. A six years old girl was clinically examined due to recurrent urinary tract infections. On ultrasound, the left kidney was enlarged, while the right kidney was absent in normal position. Cystic mass 4x5 cm was seen in the pelvis. The right kidney was not seen on IVP. CT scan showed a cystic formation in the pelvis, described as cystically changed dysplastic kidney. Pelviceal mass was extirpated. Exploration of the genital system revealed vaginal arch blinded in the hypoplastic right uterus horn. On pathohystology tumefaction corresponded to the dysplastic kidney. IN CONCLUSION: unilateral renal aplasia or dysplasia may indicate genital anomalies having in mind the hereditary character of unilateral form of the agenesia-dysplasia syndrome therefore, it could be helpful in prenatal diagnosis of cystic pelvic mass of fetus in families with this syndrome.

[Urinary bladder dysfunction and vesicoureteral reflux in patients with enuresis]. / Disfunkcija mokracne besike i vezikoureterni refluks kod bolesnika s enurezom.

We present preliminary results of prospective study on 38 children (aged 4-15 y.) with enuresis. The aim of the study was to document the association between bladder disfunction and urinary tract abnormalities. Enuresis was more common in girls (71%). Twenty one children (44.7%) suffered from secondary, and 17 (55.3%) from primary enuresis. High incidence of enuresis (31.6%) or voiding dysfunction, renal lithiasis, constipation of other kidney disease (42%) were disclosed among family members of children with enuresis. Urodynamic studies revealed bladder dysfunction in 84.0% of children with enuresis mostly in the form of dyssingeric (45.5%) or unstable/hyperactive bladder (45.5%). In 9% of patients bladder dysfunction as in the form of inadequate bladder with small and poor detrusor contractions often associated with large residue. Ultrasonography revealed dilatation of pyelocaliceal system in 13.2%, and vesicoureteral reflux was confirmed in 10.5% of patients. We stress out high incidence of bladder dysfunction among children with enuresis as well as among their family members.

[Vesicoureteral reflux as a familial disease]. / Vezikoureterni refluks kao porodicna bolest.

Vesicoureteral reflux and reflux nephropathy are causes of end-stage renal failure in 43 percent of our patients on haemodialysis. Aiming at early discovering of vesicoureteral reflux and preventing reflux nephropathy, we started an investigation of the familial character or the primary vesicoureteral reflux in the families of 44 our patients with the diagnosed anomaly. Investigations which consist of urinalysis, urine culture and ultrasound of kidneys have so far been carried out for siblings of our patients in ten families. Complete investigations, including voiding cystourethrogram where indicated, have been carried out in five families. Familial vesicoureteral reflux ascertained in three families.

[The effect of hemodialysis on endothelin-1 plasma levels in children with terminal renal insufficiency]. / Uticaj hemodijalize na nivo endotelina-jedan u plazmi dece s terminalnom insuficijencijom bubrega.

We analysed the effect of hemodialysis (HD) on endothelin (ET) plasma level in children with terminal renal failure. Twenty-five patients (pts) and 27 healthy children as controls were included in the study. There was no difference in ET plasma levels in pts before HD (1.97 +/- 1.45) and controls (2.08 +/- 1.47), but ET increased in plasma of pts after HD (4.10 +/- 3.66). To estimate the relationship of volume depletion or depuration on ET plasma level changes in pts during HD, ET was measured at three time points of HD (before ultrafiltration-UF, after UF, and after HD without fluid removal) in 10 pts. ET level was not significantly changed after UF (1.93 +/- 2.25 vs 1.71 +/- 1.50; ns), but was significantly increased after depuration (4.46 +/- 3.56; p < 0.05). There was no correlation between ET and blood pressure in controls and pts in either period of testing, neither with plasma renin activity, left ventricular mass index, and body weight changes during HD.

[Continuous blood pressure monitoring over a 24-hour period in children with terminal renal failure treated with hemodialysis]. / Kontinuisano pracenje krvnog pritiska u toku 24 sata kod dece s terminalnom insuficijencijom bubrega, lecenih hronicnim hemodijalizama.

Recent evidence suggests that circadian blood pressure changes are common in patients with impaired renal function and has excellent correlation with end-organ damage. The aim of this paper was to: 1) evaluate if children with end-stage renal failure have altered circadian blood pressure rhythm; 2) assess whether pre- or postdialytic blood pressure is representative for the average interdialytic blood pressure; 3) assess whether pre- or postdialytic blood pressure is representative for the average interdialytic blood pressure; 3) determine influence of pre-, post and interdialytic blood pressure. Ambulatory blood pressure monitoring was performed in two groups of patients: group A-13 children with end-stage renal failure, aged 15.15 +/- 5.58 years, on chronic haemodialysis from 2 to 156 (mean 45.3) months, 4 of whom were hypertensive and 9 normotensive; group B-19 children with chronic hypertension (essential or renal hypertension) aged 15.28 +/- 2.27 years. 84.62% of children from group A and 31.58% from group B (p = 0.0037) had blunted circadian blood pressure rhythm (a nocturnal reduction of blood pressure is less than 10% of daytime values). Pre- and postdialytic systolic, diastolic and mean arterial blood pressure did not differ significantly and were in correlation with interdialytic blood pressure (r = 0.9; p < 0.01). Pre-, post- and interdialytic blood pressures correlated well with left ventricular mass index (r = 0.6; p < 0.05), but were not in correlation with the degree of hypervolemia (p < 0.05).

[The influence of protein and phosphorous restricted diet supplemented with essential aminoacids (EAA) and their alpha-keto-analogues (KAA) on nutritional status of children with chronic renal failure (CRF)]. / Uticaj normoenergetske, neselekcione niskoproteinske dijete na stanje uhranjenosti dece s hronicnom insuficijencijom bubrega.

The purpose of the study was to establish the influence of protein restricted diet supplemented with EAA/KAA on nutritional status of children with CRF. Seven childredn (four girls and three boys), aged from 7 years and eight months to 14 years and two months, with glomerular filtration rate of 42.6-9.2 ml/min/1.73 m2 and proteinuria of 0.025-1.125 g/24 h, who were on conservative treatment and who lived at home, were observed for six months. In prescribing energy value and protein intake WHO recommendations were used for healthly children (age for height). Through food 80-90% of energy and 80-85% of protein needs were provided. The remaining energy and proteins were provided by glucodextrin and EAA/KAA supplements respectevely. Average daily phosphorous intake amounted to about 550-800 mg. The obtained results showed that stunting and kwashiorkor like aminoacid disbalance were the prominent nutritional problems. This dietary regimen had a beneficial effect on all studied parameters. especially on height standard deviation score which increased from 1.71 +/- 2.6 to 1.5 +/- 4.3 (t = 2.809, p < 0.05); total essential/nonessential aminoacid score increased from 0.38 +/- 0.2 to 0.56 +/- 0.2 (t = 2.763, p < 0.05). The ratio between plasma concentration of four nonessential (glycine+serinet glutamine+taurine) to four essential (leucine+isoleucine+valine+methionine) amino acids decreased from 3.82 +/- 1.2 to 2.7 +/- 0.4 (t = 2.528, p < 0.05). Lymphocyte count increased from 1.809 x 10(9) +/- 0.268 x 10(9)/l to 2.314 x 10(9) +/- 0.922 x 10(9)/l (t = 2.431, p < 0.05). No significant changes were found in the values of relative body weight, arm circumpherence, triceps skinfold thickness, total plasma protein, albumin, transferine, complement C3 and plasma valin/glycin and phenylalanine/thyrosine ratio. It should be noted that the significant changes were not found in the results of these latter anthropometric biochemical parameters in relation to their primary almost insignificant values.

[Determination of the level of urinary tract infection in children by antibodies coating bacteria from urinary sediment]. / Otkrivanje mesta infekcije u urinarnom sistemu kod dece pomocu oblazucih antitela na bakterijama u sedimentu urina.

The article deals with the evaluation of the significance of surface antibodies in urinary sediment bacteria by direct immunofluorescence in children with urinary tract infection caused by Escherichia coli (E. coli), so as of determination of location of the infection. Sixty five children were studied, aged from 1 to 16.5 years. Based on the recognized criteria in literature, all patients were classified into three groups: chronic pyelonephritis (CP)--10 patients, acute pyelonephritis (AP)--34 patients, and lower urinary tract infections (LUTI)--21 patients. On the basis of the analysis of the relationship among the three groups of patients in relation to the absence or various presence rates (up to 5%, 6-10% and 11-15% respectively) of fluorescent antibodies in urine sediment, and using the Fischer's test of absolute probability, we obtained a highly significant difference (p = 0.00006) only between the groups AP and LUTI. This difference caused the presence of fluorescence in the AP group and its absence in the LUTI group. By adding the positive fluorescence findings we found that it was present in 80% of CP patients, in 91.17% of AP patients, and in 14.28% of LUTI patients. chi2 test showed a highly significant difference (chi2 = 34.79; p < 0.01), which confirmed that bacterial fluorescence was most often present in the urine of AP patients and absent in LUTI patients. Bacterial fluorescence was significantly more frequent in CP patients in relation to LUTI patients. These results indicate that the method for detecting surface antibodies in urine bacteria, aimed at determinig the location of infection, is more reliable and noninvasive as compared to other methods.

[Clinical significance of serotypization of Escherichia coli isolated in children urinary tract]. / Klinicki znacaj serotipizacije bakterija Escherichia coli izolovanih kod dece s infekcijom urinarnog sistema.

The paper deals with the study of 65 children, aged from 1 to 16,5 years (7 years and 5 months +/- 2 years and 7 months), with urinary tract infection caused by Escherichia coli (E. coli). All patients were classified into three groups according to accepted criteria from literature (clinical features, urographic characteristics, immunological analyses and laboratory signs of inflammatory reaction: (1) 10 patients with chronic pyelonephritis (CP); (2) 34 patients with acute pyelonephritis (AP), and (3) 21 patients with lower urinary tract infection (LUTI). Using 7 antisera antigen preparations O1, O2, 04, O7, O11, O15 and O18, 46 (70.8%) isolated strains were serotyped, while 19 (29.2%) isolated strains of E. coli remained unserotyped. None of the patients showed the presence of more than one serotype in urine. Due to the small number of cases in some groups and low number of serotype strains within the groups, break-down of serotype by patient groups was not done. The prevalent serogroup was O7 found in 22 (47.8%) patients, then O11 in 9 (19.6%), O1 in 4 (8.7%), O15 in 4 (8.7%), O4 in 3 (6.5%), O2 in 2 (4.3%) and O18 in 2 (4.3%) patients. Serogroup O7 was present in all three groups of patients. Statistical analysis showed that the incidence of serogroup O7 was not significantly different (p > 0.05); this suggests that a specific serogroup does not cause only one type of infection. Having in mind that all existing antisera were not used, the possibility of intra-hospital infection provoked by O7 serogroup should be taken into consideration.

Progressive tubulointerstitial nephritis and chronic cholestatic liver disease.

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenal involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.

[Emergencies in arterial hypertension in children]. / Hitna stanja arterijske hipertenzije kod dece.

From March 1982 to March 1992 at the Nephrology Department of Belgrade University Children's Hospital 25 patients (16 females and 9 males, aged from 1.5 to 14 years) were treated for hypertensive emergency. Twenty patients had chronic severe hypertension of whom in 19 it was of renal origin. Five patients had acute hypertension during acute poststreptococcal glomerulonephritis. In 15 patients hypertensive emergency was manifested by neurologic disorders, while in the other patients signs of cardiac failure prevailed. Deterioration of renal function was observed in 9 patients and 2 had sec. haemolytic-uremic syndrome. Three patients died during hypertensive crisis, and the others were successfully treated. The fact that in 50% of patients chronic hypertension could not be revealed until the occurrence of hypertensive emergency, suggests the need of its early detection during systemic check-up.

[IgA nephropathy in children with various clinico-histologic features and therapy]. / Nefropatija IgA kod dece raznolikost klinicko-histoloske slike i lecenje.

Clinical and hystological features of IgA nephropathy were evaluated in 17 children (age range 6 to 14 years). Hematuria was present in all patients (macroscopic hematuria in 10 patients, microscopic hematuria in 7). Mild to moderate proteinuria was present in 10, nephrotic syndrome in two cases. The majority of renal biopsy showed normal picture of mild mesangial proliferation, with crescents in only one patient. Transient impairment of renal function developed in two patients. One of patients progressed to chronic renal failure. Four patients were treated (two with persistent proteinuria, one with nephrotic syndrome, one with nephritic syndrome). Corticosteroid treatment resulted in aggravation of proteinuria in two patients with persistent proteinuria. Patient with nephrotic syndrome demonstrated only diminution of proteinuria during different treatments: corticosteroids, ciclophosphamid and Ciclosporin A. Ciclophosphamid was beneficial in patient with nephritic syndrome.

[Levels of immunoreactive atrial natriuretic peptide in the blood of children on hemodialysis therapy]. / Koncentracija imunoreaktivnog atrijumskog natriuretskog peptida u plazmi dece lecene hemodijalizom.

Expansion of extracellular fluid volume,--increasing right atrial pressure,--appears to be a major stimulus for the rise in plasma atrial natriuretic peptide (ANP). End-stage renal disease (ESRD) is associated with significant changes of several hormone systems. Recent studies have shown that plasma ANP concentration is significantly increased in patients with ESRD and that the concentration of the hormone declines following haemodialysis (HD). In patients with ESRD treated by HD "dry weight" for each individual patient is the body weight at which the patients has normal hydration status. The aim of the present study was to determine changes of plasma concentrations of ANP during haemodialysis. We also attempted to check whether the baseline levels of ANP were related to weight loss during dialysis. Eleven children (7 females, 4 males) participated in the study. The patients age related from 8 to 20 years. Plasma concentrations were markedly elevated (p < 0.01) before haemodialysis (251 ae 119) pg/ml) in comparison with control values (61 ae 23 pg/ml), but remained above normal range in all except 4 cases. During HD body weight was reduced in all but one patient. This patient was infused isotonic fluid during HD and did not show weight loss. The four patients who had normal plasma ANP levels at the end of treatment appeared to be in better overall condition than the other patients. In summary, plasma ANP measurements may be of value in establishing the volume status in patients with ESRD. Plasma ANP levels after HD may help to adjust the right dry body weight for each patient.

[Use of erythropoietin in children with terminal renal insufficiency treated with chronic hemodialysis]. / Primena eritropoetina kod dece s terminalnom bubreznom insuficijencijom lecene hronicnom dijalizom.

Nine pts, aged 4-20 years (mean 12) with chronic uremic anaemia (mean Hb 5.8 g/dl, range 5.0-7.0 g/dl) on regular thrice-weekly haemodialysis, were treated with human recombinant erythropoietin (rh-uEPO) for a mean of 28.11 weeks (range 4-48). To attain a target Hb concentration (9.6-11.2 g/dl) RH-uEPO (Cilag) was administered i.v. after each dialysis in increasing doses within the range (51-300 U/kg/week). All pts were treated with persantine. Five pts needed iron supplementation. All pts showed increased Hb concentration and none of 6 previously transfusion dependent pts needed further transfusions after the first week of rH-uEPO. Pretreatment long-term dialyzed, polytransfused and iron overloaded pts, even when treated with lower doses of rH-EPO, responded better, reaching target Hb level from the 8th- to the 16th week. Three of 4 highly sensitized pts on rH-EPO treatment had a significant decrease of cytotoxic antibody titre, and 2 were successfully transplanted. rH-EPO also significantly improved the life quality in all pts. No pts developed any serious side affects. There was a transient increase in BP (2 pts) and transaminase with eosinophilia (4 pts).