Oral cyclophosphamide therapy in 100 children with steroid-sensitive nephrotic syndrome: experience from a developing country.

BACKGROUND: Oral cyclophosphamide (CYP) is an important therapeutic agent in treatment of steroid-sensitive nephrotic syndrome having a steroid-dependent (SD) or frequent relapsing (FR) course. This retrospective observational study aimed to determine response to oral CYP and factors associated with positive response in these patients. METHODS: We studied 100 children (male; 75) with FR (19%) and SD (81%) NS treated with CYP in the Pediatric Nephrology clinic. Responders were defined as children in whom steroids were stopped for at least 6 months following CYP and factors affecting response were analysed. Relapse-free survival was estimated by Kaplan-Meier method. RESULTS: Median age at onset of NS was 3 years (IQR 2-5.2). Median age at CYP was 5.7 years (IQR 3.7-7.9). Fifty percent of patients were in the responder group at 6 months of CYP. Relapse-free survival post CYP therapy was 31% at 1 year, 11% at 2 years. Factors predicting good response were age at onset of NS > 3 years with 61.2% response at 6 months (p = 0.028) and older age at CYP initiation (> 5 years) with 61% response (p = 0.008). Multivariate regression analysis showed age at start of CYP > 5 years was an independent factor for good response (p = 0.044, OR = 2.903, CI -1.03 to 8.18). CONCLUSIONS: Judicious selection of patients, especially with age of onset of NS more than 3 years and initiation of CYP after age of 5 years, can predict better response in this group of patents.

Utilization of the renal angina index in PICU of a developing country for prediction of subsequent severe acute kidney injury.

BACKGROUND: Acute kidney injury (AKI) is independently associated with worsened morbidity and increased mortality in the pediatric intensive care unit (PICU). AKI risk score, termed renal angina index (RAI) is used in developed countries to predict subsequent severe AKI. Very few studies have investigated application of RAI in the PICU of a developing country. This prospective observational study aimed to predict severe subsequent AKI in children admitted to PICU using RAI. METHODS: Over 1 year, children admitted to PICU aged 1 month-18 years old, with no previous kidney disease, were included. RAI was assessed from 8 to 12 h of PICU admission (day 0). RAI was calculated from product of the renal risk and renal injury score. Renal angina positivity was defined as RAI ≥ 8. On day 3, serum creatinine was estimated and estimated glomerular filtrration rate (eGFR) calculated. RAI was correlated with presence/absence of subsequent (day 3) severe AKI. RAI positivity was also correlated with duration of PICU stay, need for dialysis, mechanical ventilation, and mortality. RESULTS: RAI positivity was seen in 16.7% cases, of which 36.2% developed AKI at 4 days vs. 2.3% in RAI-negative cases (p < 0.001). Mean duration of PICU stay in the RAI-positive group was 7.19 ± 5.13 days vs. 4.72 ± 2.71 days in the RAI-negative group (p < 0.001). Mortality was seen in 31.9% of RAI-positive cases vs. 2% in RAI-negative cases (p < 0.001). CONCLUSIONS: RAI could be used as a simple and important bedside tool to predict patients at risk of severe AKI.

Validation of the STARZ neonatal acute kidney injury risk stratification score in an independent prospective cohort.

OBJECTIVE: A rapid AKI risk assessment score would allow for improving management and outcomes. STARZ (Sethi, Tibrewal, Agrawal, Raina, waZir) score was developed for acute kidney injury (AKI) risk stratification of critically ill neonates. This is the first independent validation for the novel score outside the original enrolled centres. STUDY DESIGN: 750 neonates were included in the study. The STARZ score was calculated after 12 hours of admission. Neonates admitted in NICU and receiving IV fluids for at least 48 hours were included. RESULTS: A total of 8.8% neonates had AKI in the first 7 days post admission. The duration of hospital stay was significantly higher among neonates with AKI [10.5 (7-19) vs. 7 (5-10) days; p < 0.001]. Mortality risk was 6.4 times higher among those with AKI [8 (12.1%) vs. 13 (1.9%); p < 0.001; RR (95% CI): 6.38 (2.74-14.83)]. In this study, the STARZ neonatal scoring model showed a sensitivity of 89.4% in detecting AKI with a 90.9% specificity and a high negative predictive value of 98.9%. The area under ROC was 0.958 (0.934-0981) - a high discriminative power. CONCLUSIONS: The STARZ score allows for AKI risk stratification, providing opportunity for therapeutic interventions which may improve outcomes in critically ill neonates.

Evaluation of Health-Related Quality of Life in Adult Patients on Hemodialysis.

BACKGROUND: Hemodialysis in end-stage renal disease patients causes disability in different domains of patient's lives, leading to impaired (quality of life [QOL]). Studies measuring the QOL in patients on renal replacement therapy are limited in the Indian scenario. AIMS: To evaluate the QOL in adult patients on maintenance hemodialysis by applying the World Health Organization QOL (WHOQOL)-BREF scale. MATERIAL AND METHODS AND STUDY DESIGN: Health-related QOL was evaluated in 100 adult patients on maintenance haemodialysis for 3 months or more in Dayanand Medical College and Hospital, Ludhiana. The WHOQOL-BREF was applied in these patients and the physical, psychological, social, and environmental health domains were assessed. RESULTS: Most common age group was 31-60 years (56%) with a mean age of 54.44 years, male: 74%. Patients with age more than 60 years had better QOL scores in the social domain which was statistically significant (P = 0.005). Male patients had better scores in all four domains and was significant in the social domain (P = 0.025). Married patients had better QOL scores in social domain. Duration of dialysis had a reverse correlation with QOL scores in physical domain with better scores in dialysis duration of <12 months. Frequency of dialysis did not significantly affect the QOL scores. QOL scores were directly related to the monthly family income in all four domains with the highest income group showing better scores (P < 0.05). CONCLUSION: The present study provided an insight into the factors that affect the QOL in hemodialysis patients. Patients with age >60 years had better QOL scores in the social domain. Female gender, low serum proteins, HD duration of more than 1 year, and low monthly income were found to be associated with impaired QOL domains in patients undergoing maintenance hemodialysis.

Comparison of Clinical Features and Outcome of Dengue Fever and Multisystem Inflammatory Syndrome in Children Associated With COVID-19 (MIS-C).

OBJECTIVE: To identify clinical and laboratory features that differentiate dengue fever patients from MIS-C patients and determine their outcomes. Methods: This comparative cross-sectional study was done at a tertiary care teaching institute. We enrolled all hospitalized children aged 1 month - 18 years and diagnosed with either MIS-C and/or dengue fever according to WHO criteria between June and December, 2020. Clinical and laboratory features and outcomes were recorded on a structured proforma. RESULTS: During the study period 34 cases of MIS-C and 83 cases of Dengue fever were enrolled. Mean age of MIS-C cases (male, 86.3%) was 7.89 (4.61) years. MIS-C with shock was seen in 15 cases (44%), MIS-C without shock in 17 cases (50%) and Kawasaki disease-like presentation in 2 cases (6%). Patients of MIS-C were younger as compared to dengue fever (P=0.002). Abdominal pain and erythematous rash were more common in dengue fever. Of the inflammatory markers, mean C reactive protein was higher in MIS-C patients [100.2 (85.1) vs 16.9 (29.3) mg/dL] (P<0.001). In contrast, serum ferritin levels were higher in dengue fever patients (P=0.03). Mean hospital stay (patient days) was longer in MIS- C compared to dengue fever (8.6 vs 6.5 days; P=0.014). CONCLUSION: Clinical and laboratory features can give important clues to differentiate dengue fever and MIS-C and help initiate specific treatment.

Nephrotic range proteinuria as a presenting feature of classical nephropathic cystinosis.

Patients with renal tubular acidosis (RTA) usually have tubular or low molecular weight proteinuria. The authors present a rare case of a 6-y-old girl with Fanconi syndrome secondary to cystinosis, who at presentation had nephrotic range proteinuria along with rickets and failure to thrive. Although hypoalbuminemia and massive proteinuria are characteristics of nephrotic syndrome, there are other conditions which can present with massive proteinuria.