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Bullous systemic lupus erythematosus (BSLE) is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE). It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE. A renal biopsy confirmed the features of class V lupus nephritis. Based on the clinical features and investigations, a diagnosis of BSLE with nephritis was made. She received methylprednisolone pulse therapy and hydroxychloroquine; however, it did not alleviate the vesiculobullous eruption, so treatment with dapsone started and resulted in the dramatic disappearance of the lesions. Interruption of dapsone due to hemolysis did not aggravate the bullous disease. During follow-up, she had multiple flare-ups of disease and nephritis without rebound of bullous lesions. BSLE is a rare presentation of SLE in children. Differentiating it from other skin bullous diseases and SLE with blister is important for the correct management. The unusual presentation of this disease may delay the diagnosis and therefore requires a high index of clinical suspicion.

Background: Chronic kidney disease (CKD) is an overwhelming illness in children. Considering the importance of CKD in pediatric age group, in addition to the racial and ethnic differences in prevalence and etiology of CKD, epidemiologic studies are recommended. The majority of the results regarding the incidence of CKD have been achieved from studies on hospitalized children. Objectives: This study evaluates the incidence and etiology of chronic renal failure (CRF) in children in a tertiary care center in Isfahan, the second large province of Iran. Patients and Methods:A retrospective analysis of medical record data of children less than 19 years old, hospitalized for CKD at St Alzahra hospital, Isfahan, Iran, during the period of November 2001 until December 2011 was made. A total of 268 eligible cases were recruited. Patients were categorized into 5 groups according to glomerular filtration rate (GFR) and KDOQI guideline. Results: Out of 268 patients, 144 patients were male (54%). The mean and median of age was 11.01± 0.39(SD) years and 12.5 years, respectively. Approximately 55% of patients had either systolic and/or diastolic hypertension. The most frequent etiology of CKD was glomerular diseases (34%) followed by reflux nephropathy (16.7%). Most patients (74.8%) had GFR less than 15 ml/min/m², when diagnosed. Anemia was the most prevalent laboratory abnormality (85%). Small-size kidneys were seen in 65.8% of patients. Kidney replacement therapy (either hemoor peritoneal dialysis) was applied for 72.3% of the patients. The transplantation rate average was 7.2 per million children population. The annual incidences of CKD (mostly advanced stages) were 5.52 per million population (PMP) and 16.8 per million children population, respectively. The all cause of mortality rate was 18.4%. Younger age was the only independent predictor of mortality (p=0.005). Conclusions: We concluded that glomerular diseases are the leading cause of CKD in our patients. In addition, the annual incidence of CKD is considerably high.

Implication for health policy/practice/research/medical education:The etiology of acute kidney injury (AKI) varies in different countries. In a retrospective analysis data of 180 children less than 18 years treated for AKI was studied. We concluded that the mortality is still high in AKI. The poor outcome (defined as low GFR) are higher among patients with low levels of first GFR and higher RIFLE score.Please cite this paper as: Gheissari A, Mehrasa P, Merrikhi A, Madihi Y. Acute kidney injury: A pediatric experience over 10 years at a tertiary care center. J Nephropathology. 2012; 1 (2) Results: The male to female ratio was 1.57 to 1. Mean age was 5.28 ± 6.3 (SD) years and the median was 1.8 years. The more frequent age group was children less than 2 years. The mortality rate was 22.2% (40 patients). The mortality was not correlated with age (p= 0.74). Renal replacement therapy was recommended for 62 patients (34.4%). Mean of the first and last glomerular filtration rate (GFR) were 18.33± 1.12 ml/min/1.73 m² and 52.53 ± 2.98 ml/min/1.73 m², respectively. The most common urinary sediment finding in approximately 70% of the patients was either renal epithelial cell or renal cell cast. Increased kidney echogenicity was the most common ultrasound finding (48%). Using ANOVA regression analysis, the etiology of disease was the only predictor of mortality (p=0.0001). Conclusions:We concluded that the mortality is still high in AKI. Furthermore, the poor outcome (defined as low GFR) are higher among patients with low levels of first GFR and higher RIFLE score.