Background Childhood-onset systemic lupus erythematosus (SLE) is more severe than adult-onset disease, including more frequent kidney involvement. This study aimed to investigate baseline clinical features, treatment modalities, short- and long-term renal outcomes of pediatric patients with lupus nephritis (LN). Materials and Methods This study enrolled 53 LN patients out of 102 childhood-onset SLE patients followed at Hacettepe University between 2000-2020. The demographic and clinical data were reviewed retrospectively from the medical charts and electronic records. All SLE patients with renal involvement underwent renal biopsy either at the time of diagnosis or during follow-up. Results The median age at onset of SLE was 13.3 (IQR : 10.4-15.8) years. The median follow-up duration was 43.1 (IQR : 24.3-69.3) months. Of the 102 SLE patients, 53 patients (52%) had lupus nephritis (LN). The most frequent histopathological class was class IV LN (54.7%), followed by class III LN (22.6%). The proportion of patients who achieved either complete or partial remission were 77.3% and 73% at 6 and 12 months, respectively. In the overall LN cohort, 5- and 10-year renal survival rates were 92% and 85.7%, respectively. The remission rate at 6th month was significantly higher in Mycophenolate mofetil (MMF) and Cyclophosphamide (CYC) treated groups than other combination therapies (p = 0.02). Although no difference was found between the CYC and MMF response rates (p = 0.57), in the proliferative LN (Class III and IV), the vast majority of class IV patients (%79) received CYC as induction threapy. There was no difference between the response rates in any treatment regimens at 12th month (p = 0.56). In the multivariate analysis; male gender, requiring dialysis at the time of LN diagnosis, failure to achieve remission at 6th and at 12 th months were found to be associated with poor renal outcome. Conclusion Our study demonstrated that male gender, failure to achieve remission at 6th and at 12 th months, and requiring dialysis at the time of diagnosis were the best predictors of poor renal outcome. Therefore, appropriate and agressive management of pediatric LN is essential to achieve and maintain remisson.
Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder resulting from excessive activation and nonmalignant proliferation of T-lymphocytes and macrophages. Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Reports pertaining to the association between HLH and HUS are rarely published; however, we report on a 4-year-old boy who was diagnosed with both conditions and treated successfully with high-dose steroid and intravenous immunoglobulin. Differentiating HUS from HLH can be challenging because of their clinical similarities. Therefore, prompt diagnosis and immunosuppressive treatment are essential and life-saving to these patients.
Background/aim: The data concerning the effects of desmopressin on water/electrolyte disturbances of children with primary monosymptomatic nocturnal enuresis (PMNE) are limited. In the present study we aimed to evaluate the effect and tolerability of desmopressin on blood and urine electrolytes and osmolality in PMNE.Materials and methods: Thirty-five children with PMNE between the ages of 5 and 15 participated in the study. Patients collected urine during the daytime and acknowledged the night time fluid restriction before starting to use the desmopressin tablets. The medication was taken orally at least 1 h before bedtime. Blood and urine samples were collected before the introduction of the treatment (day 0) and on the third and seventh days of the administration of desmopressin to determine osmolality and electrolyte levels.Results: Thirty-five patients participated in the study. Twenty-one patients (60%) were male and 14 (40%) were female. The mean age was 9.6 ± 2.7 years. There were no significant changes in serum osmolality, urine osmolality, and serum sodium concentration. Mean urine calcium/creatinine ratio was 0.03 ± 0.01 mg/mg at the beginning, 0.06 ± 0.02 mg/mg on the third day, and 0.04 ± 0.01 mg/mg on the seventh day of the study. No significant changes were seen in urine calcium/creatinine ratio before and after treatment. Conclusion:Desmopressin appeared to be a well-tolerated drug and provided a safe and effective treatment for children who were following fluid intake restriction for PMNE.
Background and Aims Urinary system complications after hematopoietic stem cell transplantation (HSCT) cause severe morbidity and mortality. The aim of the study is to investigate the incidence and risk factors of urinary system complications in patients who had HSCT during their childhood. Method Patients who had HSCT between January 2010-2019 with a minimum follow-up period of 6 months were included in the study. Data regarding urinary system complications were collected from the medical records of the patients. pRIFLE and KDIGO classification systems were used for the definition of acute kidney injury (AKI). Results 167 patients (108 males and 59 females) with allogeneic (n=165) and autologous (n=2) HSCT were investigated for renal complications. In cohort, HSCT was performed in 41 patients (%24.6) secondary to malignant diseases and in 126 patients secondary to non-malignant diseases. Hemorrhagic cystitis (HC) developed in 28 patients (16.8%) after HSCT. The mean age of the patients with and without HC was 14.6±5.6 years and 10.5±6 years, respectively (p=0.044). Among patients with HC, 17 had concomitant viral infection. Presence of viral infection, gender, disease group, history conditioning regime, total body irradiation, acute graft-versus-host disease and veno-occlusive disease (VOD) did not have any effect on the development of HC in logistic regression analysis. In cohort, 126 patients (75.4%) developed AKI according to KDIGO classification (stage 1; 55 patients, stage 2; 36 patients, stage 3; 35 patients). The mean period of development of AKI after HSCT was 34±22 days. The risk of AKI (according to KDIGO) was higher in patients who had HSCT secondary to malignant diseases and/or who developed viral infections after HSCT (p=0.034 and p=0.013, respectively). Among patients, 71 patients (%42.5) developed AKI according to pRIFLE classification. The risk of AKI (according to pRIFLE) was higher in patients who had HSCT secondary to malignant diseases, who developed viral infections and/or who developed VOD during follow-up (p=0.043, p=0.001 and p=0.006, respectively). One patient developed thrombotic microangiopathy after HSCT. None of the patients had nephrotic syndrome during follow-up period. Conclusion Patients who had HSCT secondary to malignant disease, viral infections and/or VOD had higher risk of AKI and should be closely monitored.
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