Tehran University Medical Journal

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Background: Cyclosporin A (CsA) is now commonly used in the management of children with steroid-dependent and steroid resistant nephoitic syndrome. It has been reported to be effective in maintaining remission in 70-100 percent of patients with SDNS but somewhat SRNS 0-100 percent. The aim of this study was to evaluate the efficacy of long-term (CsA) in children with refractory nephrotic syndrome (RNS) and steroid dependent nephrotic syndrome (SDNS).

Materials and Methods: The long-term effect of (CsA) in 91 Iranian children aged 3 months to 11 years (54 with RNS and 37 with SDNS) was assessed between 1984 and 1999. Eighty of 91 children received renal biopsy prior to introduction of (CsA), and the other 11 patients had not consent for kidney biopsy. If the patients did not show remission aftre receiving 3-6 months of (CsA), the medication was discontinued.

Results: All patient were treated with (CsA) in combination with low dose alternate day prednisolone. In children with RNS and SDNS, therapy with (CsA) induced, remission in 25 of 54 (46.2 percent) and 27 of 37 (73 percent) respectively (P<0.02). Of the 32 patients with minimal change disease (MCD), 23 (72 percent) responded to therapy, compared with 4 of 18 (22 percent) with focal segmental glomerulosclerosis (FSGS) (P<0.005). Twenty-four (48 percent) of 50 who entered complete remission, had relapse 1-12 months after cessation of (CsA). The duration between the onset of nephrotic syndrome (NS) and administration of (CsA) and sexuality of patients had no effect in result of treatment. Side effects occurred in 25 patients (27.4 percent). No patients exhibited raised transaminases, 8 (8.7 percent) of the children developed hirsutism, 7 (7.6 percent) hypertension, 7 (7.6 percent) gingival hyperplasia, (2.2 percent) neurological toxicity and 1 (1 percent) increase in serum creatinine.

Conclusion: Our findings suggest that (CsA) can be used to induce a complete remission in a significant proportion of patients with RNS and SDNS, and patients with SDNS have areasonable potential for remission than children with RNS. Resistant to steroid and cyclophosphamid.

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Background: Urinary tract infection is a common bacterial infection in children and may lead to irreversible renal damage. TC 99-m Dimercaptosuccinic acid renal scintigraphy is the most sensitive method for diagnosing acute pyelonephritis. We designed a study to evaluate the ability of DMSA scan and ultrasonography to detect renal paranchymal lesion.

Materials and Methods: 62 children 1 month to 12 years of age with the first episode of acute pyelonephritis were prospectively studied with DMSA scan and ultrasonography during acute phase of infection. A Voiding Cystourethrogram was performed in 60 children when urine culture became negative. Children with renal paranchymal changes were older at the time of acute pyelonephritis (P=0.04) but no difference was found between the groups with regard to levels of CRP, ESR (P>0.05).

Results: Changes on the DMSA scan were found in 106 (85.5 percent) kidneys of 62 children but ultrasonography showed renal changes in 19.4 percent (sensitivity=20 percent, specificity=83 percent) (Kappa=0.06). Vesicoureteric reflux was found in 14 children (23.3 percent) but 83 percent of the affected kidneys were drained by non-refluxing ureters.

Conclusion: It is concluded that DMSA scan is more sensitive than ultrasonography in detecting renal paranchymal changes in acute pyelonephritis and we found out that renal paranchymal changes after acute pyelonephritis is common, even in those without VUR.

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Early diagnosis, treatment, investigation and follow up of children with urinary tract infection (UTI) are needed to minimize renal scarring. The aims of this study were 1) to evaluate the ability of DMSA scintigraphy, ultrasound and biological parameters in detecting renal parenchymal involvement in children with acute pyelonephritis (APN) 2) to assess the relation between renal parenchymal changes and creatinine clearance 3) to determine the incidence of renal scarring after APN.
Materials and Methods: We prospectively studied 54 children (median age 4.02± 3.41 range 1 month to 12 years) with first time symptomaticUTI. All patients had DMSA scan and ultrasonography within 5 days of admission. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cell (WBC), was measured at the time of infection, and voiding cystourethrography was performed in all children within 10 days. When scintigraphy showed renal parenchymal changes, repeat DMSA scan was done at least 3 months after initial infection.
Results: Changes on the DMSA scan were found in 93/108 (85.5 percent) renal units in 54 children during acute pyelonephritis. Among 42 children who had underwent repeat scintigraphy , renal scars were found in 9 of 16 (56.25 percent ) renal units in 8 infants under 1 year ,23 of 32 (71.87 percent) in 16 children aged 1-5 years, and 33 of 36(91 percent ) in 18 patients older than 5 years. Ultrasonography showed renal changes in 20 of 108 (18.5 percent) kidneys. Reflux was seen in 21 of 108 (19.44 percent ) renal units. The sensitivity of ESR, CRP, WBC, and ultrasonography was 78.5 percent , 64.5 percent , 69.9 percent , 18.5 percent respectively, and the specificity of them was 40 percent, 33.3 percent, 13.3 percent,
80 percent respectively. There was a positive correlation between renal parenchymal involvement and creatinine clearance level (p<0.001).
We found no difference between groups with or without scars with respect to levels of ESR, CRP, and WBC.
Conclusion: The present study suggest that DMSA scan may be a more reliable method of investigation than ultrasonography and biological parameters for identifying children at risk of permanent renal lesion. Additionally we found positive correlation between renal parenchymal change and creatinine clearance level. In order to detect persistent changes, it is suggested that DMSA scintigraphy should be performed at least three months after UTI.

 

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The nephrotic syndrome is the most common chronic renal disease of childhood.
Materials and Methods: In this study the clinical course, risk factors for relapse and the predictors of long-term outcome of 502 patients (median age 5 years)with primary nephrotic syndrome were followed for an average of 60 months (3.5 to 240 months) from 1981 to 2000.
Results: Among the 502 patients 5 (1%) achieved spontaneous remission and 313 children were initial responder. One hundred eighty four patients received at least 1 kidney biopsy (78 prior and 106 after initiation of treatment). Of 104 children with frequently relapsing steroid sensitive and steroid dependent nephrotic syndrome, levamisole induced prolong remission in 33 ( 31.7%) of patients. Cyclophosphamid and cyclosporine A induced prolong remission in 49 (50%) of 98 and 28 (41.3%) of 68 patients respectively. At the time of the final clinical evaluation, 73 patients (14.5%) were on remission 301 (59.9%) had relapsing 43 (8.6%) had persistent nephrotic syndrome 33 (6.6%) of patients evolving to end-stage renal disease (ESRD) and 6 (1.2%) of them with chronic renal failure died (infection and cardio respiratory were the cause of death in 5 and 1 patient respectively). Young age (1-5 y) at onset of disease and atopy were identified as an independent risk factors for relapse (P0.05). Patients with steroid dependent nephrotic syndrome (SDNS) or MCNS had better response to cyclophosphamide or cyclosporin than children with steroid resistance nephrotic syndrome (SRNS) or FSGS (P0.05). Persistent proteinuria, hypertension, microscopic or macroscopic hematuia, glucosuria were associated with progression to chronic renal failure (PO.05).
Conclusion: Steroid dependency and histopathology of MCNS in patients with nephrotic syndrome were significantly associated with good long-term prognosis. In contrast persistent proteinuria, histopatholoy of FSGS, hypertension, macroscopic or microscopic hematuria, and glucosoria were significantly correlated with unfavorable long-term outcome. Additionally our study showed a positive correlation between young age and atopy with higher rate of relapse.

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Background: Despite several years of intensive investigation, relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is poor for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. As newer treatment modalities become available for patients with severe lupus nephritis, it become increasingly important to identify patients at risk for renal failure. The aim of this study was to evaluate the clinical course, histopathology, serologic features and prognostic significance of some parameters, to identify the risk factors for renal failure and mortality in children with lupus nephritis.

Materials and Methods: Retrospectively 30 children under 16 years of age with lupus nephritis from 1989 to 1999 were studied. All patients received renal biopsy and follow-up biopsies were performed in 3 children. Lupus nephritis was classified by the World Health Organization (WHO) criteria. The clinical and serologic parameters at the time of renal biopsy were recorded.

Results: All children underwent renal biopsy within 1 year of disease onset. There were 1 (3.3%) patients with class II, 5 (16.7%) with class III, 21 (%70) with class IV, and 3 (%10) with class V nephritis based on initial biopsy according to the WHO classification. The mean follow-up time was 60 months. Follow-up biopsies were histologically stationary in 2 patients and progressive in one. The overall renal and patient 5- year survival rates were 46.66% (14/30) and 93.33 %( 2/30) respectively. They were 47.61% (10/21) and 95.21 %( 20/21), respectively, of patients with class IV proliferative glomerulonephritis. Children with renal pathology (class V in the WHO classification system) at initial biopsy, were at high risk for renal failure 66.66% (2/3) or morality %33.33 (1/3) despite aggressive treatment. The results revealed that those with persistent hypertension, anemia, and decreased creatinine clearance rate, nephrotic proteinuria, at initial biopsy were more prone to develop renal failure (P<0.01).

Conclusion: The prognosis of children with class IV nephritis in our study was better than reported in other series in recent years. However, those with class V disease, persistent hypertension, anemia, low creatinine clearance and nephrotic proteinuria at the time of diagnosis are at increased risk for renal failure. The improved results may be due to initial histological classification, better supportive care and selective use of aggressive therapy such as methylprednisolone pulse therapy and intravenous cyclophosphamide for those with high risk factors.