Management and outcome of steroid-resistant nephrotic syndrome in children.

INTRODUCTION: Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. MATERIALS AND METHODS: We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 +/- 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. RESULTS: Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively (P = .03). CONCLUSIONS: We showed that patients with late steroid resistance had better response to immunosuppressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD.

Short-term and long-term outcome of hemolytic uremic syndrome in Iranian children.

BACKGROUND: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure during infancy. Many symptoms and clinical features have been proposed as prognostic factors for HUS in the short and long term, while the results of different studies have often been controversial. The aim of this study was to evaluate short-term and long-term outcomes of HUS in Iranian children. METHODS: Medical records of all 92 children suffering from HUS admitted to the pediatrics nephrology ward at Ali-Asghar Children Hospital in Tehran, Iran, from 1990 to 2004, were retrospectively reviewed. RESULTS: Out of 92 children, mortality was observed in 18 patients (19.6%) during the acute phase of the disease. Significant correlation between mortality and seizures, coma and hypertension in the acute phase was found (p<0.05). No association was observed between type of treatment and mortality (p>0.05). In the long-term, the presence of hypertension in the acute phase of the disease (p=0.023; relative risk [RR] = 3.89; 95% confidence interval [95% CI], 1.01-13.76), hypertension at discharge time (p<0.001; RR=10; 95% CI, 2.44-40.91) and need for dialysis (p=0.021; RR=1.38; 95% CI, 1.13-1.70) were shown to be significant risk factors for future hypertension in HUS patients. CONCLUSION: Central nervous system involvement is associated with mortality in the acute phase of HUS, whereas the severity of disease in the admission phase is related to occurrence of hypertension in future.

Prognosis of acute renal failure in children.

UNLABELLED: Acute renal failure (ARF) is the acute loss of renal function over a period of hours or days. Given the poor prognosis of ARF among children, there is some urgency to identifying more effective prognostic indicators for detecting disease onset. Such indicators would help provide the means of selecting patients who would benefit the most from early aggressive treatment. In this study we assessed the etiologic and prognostic indicators of ARF, including several risk factors such as sepsis, respiratory distress, age, among others, in 300 children who were admitted to the Ali Asghar Children's Hospital, Tehran, Iran, from 1990 to 2003. Statistical analysis was performed using multiple regression and chi-square methods, and a score to determine the prognosis of ARF in children was developed. RESULT: Based on the results of this study the three common causes of ARF are acute tubular necrosis (ATN, 38%), acute glumerulonephritis (24%) and hemolytic uremic syndrome (24.1%). The overall mortality rate among our patients was 24.7%, with the highest risk group being those patients suffering from ischemic ATN. In addition, the correlation (p<0.0005) between the etiology and mortality rate was particularly high in patients with ischemic ATN. Mortality was also high (68%) in children younger than 2 years. Multiple regression models revealed that among those factors that significantly differed between the survivors and nonsurvivors, only the necessity of dialysis (p<0.0005), the use of mechanical ventilation (p=0.05) and disseminated intravascular coagulation (p=0.038) can be regarded as independent determinants of ARF prognosis in children.