Treatment with Cyclophosphamide for steroid-resistant nephrotic syndrome in children.

The management of patients with steroid-resistant nephrotic syndrome remains difficult. We repport our experience with Cyclophosphamide therapy, in an attempt to compare the results between an oral protocol and two i.v. protocols. The complete and sustained general remission rate was 43.1%, which confirms the efficacy of the treatment, especially for children with minimal change nephrotic syndrome. For the i.v. administration we recommend only 6 month of therapy, due to severe side-effects in longer courses.

Nephrotic syndrome from the genetic point of view.

Nephrotic syndrome, an entity described at the beginning of the 20th century, has still an unclear pathophysiology, genetic and immunologic factors being incriminated. Here we report three cases of nephrotic syndrome with genetic involvement. The article presented tremendous progress that have been made over the past few years in enhancing our understanding of the slit-diaphragm and podocyte biology and genetics involvement in nephrotic syndrome.

[The TB infection in children with chronic renal diseases]. / Infectia TBC la copiii cu boli renale cronice.

UNLABELLED: The incidence of tuberculosis in the general population has risen in Romania over the past years. It is, therefore, presumable that this incidence is higher in the immuno-compromised hosts than in the general population. METHODS: We analysed the tuberculosis incidence in children with a chronic renal disease, the favourable factors, and also the tuberculosis impact on the evolution of the renal disease. The tracing period was of 12 years, the lot being composed of 168 patients: 96 with primitive or secondary chronic renal diseases who were treated with immunosuppressive agents and 72 with ESRD, undergoing chronic dialysis. The study traced: the main disease, the immunosuppressive therapy received at the time of the TB diagnosis, the age of the main disease at the time of the TB diagnosis and its evolution stage, the proper framing within the disease type. RESULTS: There were 22 cases of tuberculosis diagnosed (22.03%), 2 ending in deaths. 10 were TB-infected (positive tuberculin skin test-TST) and 12 had TB-disease (5 cases of pulmonary and 6 of extra-pulmonary TB). The period between the diagnosing of the renal disease and that of the TB was, in average, around 6 months (between 0-48 months). Contact with other ill persons existed in only one case. Out of the cases diagnosed with TB, immunosuppression was present as a favouring factor in 9 cases (75%)--2 cases with ESRD and 7 cases with immunosuppressive therapy for the active renal disease. 4 cases with cortico-resistant nephritic syndrome (3 cases with mesangioproliferative glomerulonephritis and one case which did not undergo biopsy) responded to the immunosuppressive treatment after the tuberculostatic treatment. CONCLUSION: The tracing of the TB infection is compulsory in the groups of patients presenting risks, even in the absence of suggestive epidemiologic data. Tuberculosis induces resistance to the immunosuppressive therapy, the remission being dependent on the efficiency of the tuberculostatic treatment.

[Remission of primary vesicoureteral reflux in children with medical management]. / Remisiunea refluxului vezico-ureteral primar sub tratament medical la copil.

We studied the spontaneous resolution rate in children with primary vesicoureteral reflux (VUR) and the interference of some specific factors. We reviewed the records of 110 children (14 days - 16 years) admitted in the 4th Pediatric Clinic Iasi with primary VUR, between 1994 - 2003, which had exclusive medical management and minimum one follow-up cystogram. We used Kaplan-Meier curves to analyze the resolution rate of VUR during the follow-up in relation with initial grade, age at diagnosis, gender, recurrent urinary tract infections (UTIs) and the presence of renal scarring. The remission rate for all the cases was 70%, with differences based on the reflux grade: 100% for grade I and 45% for grade IV. The cases diagnosed during infancy recovered faster than those diagnosed after this period. The presence of renal scarring and breakthrough UTIs increased the remission time. In conclusion the majority of patients with VUR resolved during medical management, the remission rate being influenced by the reflux grade, presence of renal scarring and breakthrough UTIs.

[A possible prognosis score in corticosteroid-resistant nephrotic syndrome]. / Un posibil scor prognostic în sindromul nefrotic corticorezistent.

Chronic glomerular nephropathies in children are marked by an often unfavourable evolution, so that the establishing of a prognosis at the time of the diagnosis is both a professional and a moral duty for the pediatric nephrologists. The estimation of the current practice renal survival prognosis in children with chronic glomerular nephropathies, by using clinical and laboratory elements in different histological forms of primitive chronic glomerulonephritis (CGN), with a minimum period of observation of one year. We analyzed parameters that may intervene in the duration of renal survival: type of CGN, age at the debut of the illness, histological scores of activity and chronicity, the presence of tubular atrophy lesions and that of interstitial fibrosis, renal failure (RF) installment time, in cases with normal renal function at the beginning, the time until the initiation of dialysis in cases with ESRF, respectively. The statistic analysis of data has been carried out with Epi soft (Fischer test). The results have been as follows: unfavourable evolution has been taken into consideration in the cases which have presented fixed nitric retention or which required the initiation of dialysis. The initiation of dialysis was necessary in 19 cases (76%), out of which 11 (44%) having associated between 4 and 6 of the considered risk factors. If the histological type (SFGS, DGS, MPGN) is added to the obtained score, the accuracy of the estimation increases to 89%. In conclusion, the usage of prognosis scores composed of current elements of diagnosis that have proven to have statistical significance, as far as the renal survival prognosis is concerned, may allow the invoking of a medium-term prognosis in the evolution of children with CGN.

[Treatment outcome using prednisone in corticosteroid-responsive primary nephrotic syndrome in children]. / Rezultatele tratamentului cu prednison in sindromul nefrotic primitiv cortico-sensibil la copil.

In children, the nephrotic syndrome is usually corticoid-responsive; approximately 70% of patients experience relapses, frequently triggered by infections. Our paper presents the results obtained using a 4 month prednisone regimen. This retrospective study included 83 children afflicted with nephrotic syndrome over a 10 year span. We analyzed: age at diagnosis, boys/girls ratio, response to corticoid treatment - after one month of prednisone and at the completion of the treatment course, number of relapses and their frequency, complications of prednisone treatment. The median age at diagnosis was 4.8 years, males predominating M:F = 1.5:1. Complete response after 4 weeks of prednisone therapy was noted in 98.79% of cases. We had 116 episodes of relapses during the first year of follow-up, occurring in 67.4% of children (27.9% were frequent relapsers, 11.62% subsequently became corticoid-dependent). Late relapses, after the first year, occurred in 32.55% of cases. We noted mostly mild adverse effects of the prednisone treatment: occurrence of infections during therapy (16.27%), cushingoid facies (37.2%), hirsutism (4.6%), high blood pressure (4.65%), stretch marks (2.32%). In conclusion, the 4 month prednisone treatment regimen is efficient in inducing and maintaining a remission. The incidence of relapses is 32.55%, comparable to the figure cited in larger studies. Serious adverse effects are significantly lower with this regimen compared to other corticoid treatment schemes. Key wo

[Acute renal failure in children. Study of 35 patients]. / Insuficienta renala acuta la copil. Studiu pe 35 de cazuri.

The medical care of children with acute renal failure and the necessity of the substituting the renal functions has dramatically modified over the past 15 years. The study has been conducted retrospectively on 35 children diagnosed with acute renal failure (ARF), for analyzing the etiological spectrum, the evolutionary patterns and the influence factors of the ARF evolution. The following parameters have been taken into consideration: ARF etiology, hTA/HTA, oliguria, level of serum creatinine, the type of treatment (renal substitution by hemodialysis, peritoneal dialysis, or conservative treatment). The ARF etiology is dominated by the sepsis (31.4%) and by the hemolytic and uremic syndrome (17.1%). The treatment applied was conservative in 48.5% of the cases; 51.50% of the patients were in critical state and required extrarenal substitution by hemodialysis--42.8%--and peritoneal dialysis--8.5%. Global mortality is reduced (22.8%), but in the dialysed patients it is of 44.44%. The main death causes were: severe hepatic failure, oncological diseases, severe neurological damage and hemodynamic damages. In conclusion, the ARF prognosis in children is influenced by the comorbidity states. Dialysis has improved the therapeutic results in the currently presented lot, the death causes being extrarenal.

[Acute renal failure in newborn. Case presentation]. / Insuficienta renala acuta la nou-nascut. Caz clinic.

Acute renal failure in newborn occurs in 6-8%. Using a systematic approach, physicians can determine the cause of acute renal failure in most patients. Renal cortical necrosis is an uncommon cause of acute renal failure, secondary to perinatal asphyxia or severe anemia. We report our first experience in peritoneal dialysis for a newborn with renal injury due to severe bleeding through the umbilical cord.

[Current aspects of hemolytic uremic syndrome in children]. / Aspecte actuale ale sindromului hemolitic si uremic la copil.

The authors are approaching the problem of hemolytic uremic syndrome, a common cause of acute renal failure in children. This review present an update about the pathophysiology of typical hemolytic uremic syndrome, useful for understanding the clinical picture and the base for some modern therapeutical models. Concerning the evolution, the authors underline the importance of identifying the risk factors for acute phase, the extrarenal manifestations being considered of vital risk. The atypical hemolytic uremic syndrome still has some uncertainties; the article try to make a synthesis of ethiopathogeneity, clinical manifestations, evolution and therapeutical modern approaches.