Long-term follow-up of children with steroid-responsive Nephritic Syndrome

Nephrotic syndrome is one of the most remarkable diseases in childhood. The majority of patients have prompt response to corticosteroids. Methods: In this study, we retrospectively evaluate the outcome of patients with steroid-responsive nephritic syndrome. Medical records from January 1996 to September 2006 were reviewed to identify all children with steroid sensitive nephrotic syndrome at the Pediatric Medical Center, Tehran, Iran. Initial steroid therapy was 60 mg/m[2] per day for four weeks. Levamisole, a steroid-sparing agent, was prescribed at a dose of 2.5 mg/kg on alternate days in conjunction with alternate-day prednisolone. If no benefit was observed by three months, levamisole was discontinued and immunosuppressive therapy with cyclophosphamide at a dose of 3 mg/kg daily for 8 weeks, or cyclosporin A at a dose of 3-5 mg/kg was prescribed. Of 745 children with steroid sensitive nephrotic syndrome, 63.1% of patients were male. The most common causes were minimal change disease [98/324, 30.2%] and focal segmental glomerulosclerosis [81/324, 25%]. At presentation, microscopic hematuria was found in 22.6% of the patients. During follow-up, 9.2% had no relapse at any time, while 15.8% were frequent relapsers. The remission period ranged from 3.5 to 168 months. At the last follow-up, 57.6% of the patients were in remission, 37.7% relapsed and 29 children developed chronic renal failure. The outcome of nephrotic syndrome was not associated with age or gender. The end clinical status of patients correlated with duration of remission, number of subsequent relapses and response to cytotoxic agents. Conclusion: Steroid-responsive nephrotic syndrome in children should be followed over a long period, especially patients with early relapse. Relapse was seen in more than 90% of patients. Documentation of histopathology by renal biopsy may be helpful to identify those at increased risk for a poor outcome

Thoracic ectopic kidney with diaphragmatic hernia

Congenital thoracic ectopic kidney is a very rare developmental anomaly and the rarest form of all ectopic kidneys .it is usually asymptomatic and discovered incidentally in routine chest radiography. We report an 18-month old boy with right congenital diaphragmatic hernia with thoracic ectopic kidney and positional respiratory symptoms. Chest X- ray revealed opacity at the base of right lung. Dimercaptosuccinic acid [DMSA] scan showed right thoracic kidney. Hereby we discuss the features of congenital right thoracic ectopic kidney and review the literature in this regard

Clinical outcome of acute poststreptococcal glomerulonephritis [APSGN] in Iranian children

Recently, the prognosis of acute post-streptococcal glomerulonephritis [APSGN] has been reported as improved compared with the results of previous studies. In an attempt to clarify this in Iranian children, we analyzed the clinical course of patients with APSGN. In this retrospective study, a total of 53 children diagnosed as having APSGN according to the presence of hematuria and/or proteinuria, evidence of group A beta-hemolytic streptococcal infection, transient hypocomplementemia and absence of clinical or histological evidence of previous renal disease were studied in our department between March 1986 to September 2002. Fifty-three children, 19 females and 34 males, aged 3-13 years [mean 8.7 years] were enrolled in the study. All children had hematuria, proteinuria and decreased serum complement. There were no patients with renal dysfunction, but one case with nephritic syndrome. Forty-three [81%] had hypertension according to Second Task Force criteria. Blood pressure [BP] was normal in the remaining 10 [19%] patients. Eight children were lost for the follow-up examination. Two patients received renal biopsy. Both biopsies were abnormal showing mild changes with corresponding immunologic findings. Forty-five children were reassessed after an average of 5 months [range 1 month to 66 months]. At the last follow-up all of these children were in good physical health. The BP, serum creatinine, and complement levels were within normal limits. These findings indicated that the prognosis of APSGN during childhood is excellent, when adequately recognized and received supportive measures in the treatment, including control of high blood pressure and chemical imbalance in acute phase of disease. Additionally, according to our results, we could predict a favorable prognosis and reassure the family