An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome.

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival. Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

Comparison of renal growth in breast fed and artificial fed infants: a cross-sectional study.

INTRODUCTION: Renal growth in infancy determines renal function in adulthood and can easily be assessed via infant renal volume. Renal growth is influenced by many endogenous and exogenous factors among which nutrition is of prime importance. Worldwide, infants get their nutrition either from breast milk or formula, both of which have controversial roles in kidney growth and development. METHODS: A cross-sectional study was done on healthy infants in the Pediatric Nephrology Department of Mayo Hospital, Lahore. These infants were either breastfed or artificially fed and their kidney volumes were noted to determine any significant difference in kidney size. Both informed and written consent was taken before data collection and the data was analyzed using SPSS version 26. RESULTS: Out of 80 infants included in our study, 55% were male and 45% were female. The mean age was 8.9 months and the mean weight was 7.6 kg. The mean total kidney volume was 45.38 cm3 and the mean relative kidney volume was 6.12 cm3/kg. No statistical difference in relative renal volume was found between breastfed and artificially fed infants. CONCLUSION: The present study aimed to compare the renal volume and thus renal growth in breastfed versus formula-fed infants. No statistical significance was found in relative renal volume between breastfed and artificially fed infants.

Multicenter study on the genetics of glomerular diseases among southeast and south Asians: Deciphering Diversities - Renal Asian Genetics Network (DRAGoN).

Multinational studies have reported monogenic etiologies in 25%-30% of children with steroid-resistant nephrotic syndrome. Such large studies are lacking in Asia. We established Deciphering Diversities: Renal Asian Genetics Network (DRAGoN) and aimed to describe the genetic and clinical spectrums in Asians. We prospectively studied a cohort of 183 probands with suspected genetic glomerulopathies from South and Southeast Asia, of whom 17% had positive family history. Using multi-gene panel sequencing, we detected pathogenic variants in 26 (14%) probands, of whom one-third had COL4A4 or COL4A5 variants (n = 9, 5%). Of those with COL4A5 defects, only 25% had features suggestive of Alport syndrome. Besides traditional predictors for genetic disease (positive family history and extrarenal malformations), we identified novel predictors, namely older age (6.2 vs. 2.4 years; p = 0.001), hematuria (OR 5.6; 95% CI 2.1-14.8; p < 0.001), and proteinuria in the absence of nephrotic syndrome (OR 4.6; 95% CI 1.8-11.8; p = 0.001) at first manifestation. Among patients who first presented with proteinuria without nephrotic syndrome, the genetic diagnostic rates were >60% when a second risk factor (positive family history or extrarenal manifestation) co-existed. The genetic spectrum of glomerulopathies appears different in Asia. Collagen IV genes may be included in sequencing panels even when suggestive clinical features are absent.

COVID-19 in children treated with immunosuppressive medication for kidney diseases.

BACKGROUND: Children are recognised as at lower risk of severe COVID-19 compared with adults, but the impact of immunosuppression is yet to be determined. This study aims to describe the clinical course of COVID-19 in children with kidney disease taking immunosuppressive medication and to assess disease severity. METHODS: Cross-sectional study hosted by the European Rare Kidney Disease Reference Network and supported by the European, Asian and International paediatric nephrology societies. Anonymised data were submitted online for any child (age <20 years) with COVID-19 taking immunosuppressive medication for a kidney condition. Study recruited for 16 weeks from 15 March 2020 to 05 July 2020. The primary outcome was severity of COVID-19. RESULTS: 113 children were reported in this study from 30 different countries. Median age: 13 years (49% male). Main underlying reasons for immunosuppressive therapy: kidney transplant (47%), nephrotic syndrome (27%), systemic lupus erythematosus (10%). Immunosuppressive medications used include: glucocorticoids (76%), mycophenolate mofetil (MMF) (54%), tacrolimus/ciclosporine A (58%), rituximab/ofatumumab (11%). 78% required no respiratory support during COVID-19 illness, 5% required bi-level positive airway pressure or ventilation. Four children died; all deaths reported were from low-income countries with associated comorbidities. There was no significant difference in severity of COVID-19 based on gender, dialysis status, underlying kidney condition, and type or number of immunosuppressive medications. CONCLUSIONS: This global study shows most children with a kidney disease taking immunosuppressive medication have mild disease with SARS-CoV-2 infection. We therefore suggest that children on immunosuppressive therapy should not be more strictly isolated than children who are not on immunosuppressive therapy.

Psittacosis: Rare Respiratory Problem in Children.

Psittacosis is a rare disease particularly in children with usual presentation of respiratory and constitutional symptoms. The cases may remain undiagnosed or diagnosis may be delayed because of lack of awareness among the paediatricians and physicians. Early diagnosis is very important as this is potentially curable and preventable disease. An interesting case of psittacosis is being reported here, which has been treated successfully with azithromycin.

Effectiveness of nebulized N-acetylcysteine solution in children with acute bronchiolitis.

OBJECTIVE: To evaluate the effectiveness of nebulized mucolytic therapy (N-acetylcysteine) in treatment of children with acute bronchiolitis. STUDY DESIGN: Prospective randomized, controlled study. PLACE AND DURATION OF STUDY: Paediatric Medical Unit of the Children's Hospital and the Institute of Child Health, Lahore, from November 2011 to April 2012. METHODOLOGY: All the patients aged 2 months to 24 months, with the diagnosis of acute viral bronchiolitis were enrolled in the study. A predesigned proforma was completed after taking parental consent. All eligible patients were randomly assigned to one of the two groups: group-1 received N-acetylcysteine in nebulized form, and group-2 received salbutamol as a nebulized aerosol. Both groups were then compared regarding their clinical improvement and duration of stay in the hospital. The data obtained was analyzed on the statistical software SPSS version 16. Descriptive statistics were obtained by frequencies and percentages. Chi-square test was applied to find p-value. RESULTS: A total of 100 patients met the inclusion criteria. The mean age was 3 months. On the first day of treatment, the mean clinical severity score at baseline were 5.38 ± 2.62 in group-1 and 4.68 ± 2.2 in group-2. At day 3 and 5, it was 2.9 ± 1.48 and 3.30 ± 1.77 in group-1 and 0.88 ± 1.08 and 1.90 ± 1.32 in group-2 respectively. The clinical severity score after N-acetylcysteine inhalation therapy was better in group-1 on the third but more so on fifth day after hospital admission. The mean duration of hospitalization was 4.67 ± 2.2 days for the whole population and it differed between the 2 groups: 4.36 ± 1.66 days in group-1 versus 4.98 ± 2.6 days in group-2. CONCLUSION: On the basis of improvement in clinical severity score and early discharge from the hospital, N-acetylcysteine was found to be an effective therapy in acute bronchiolitis.

Caffey's disease in an infant.

Caffey's disease is a self limited disorder of infantile age group. It is synonymous with 'infantile cortical hyperostosis' and 'Caffey's-Silver syndrome'.It is characterized by fever, irritability, bone pain and characteristic bony changes. It has no definite etiology. This report describes a 4 months old infant presenting with fever, irritability and soft tissue swellings. Investigations led to the diagnosis of Caffey's disease. Symptomatic treatment was given to good clinical response.

Intralobar pulmonary sequestration masquerading as congenital lobar emphysema.

Intrapulmonary sequestrations are quite uncommon in pediatric age group. The preoperative diagnosis of pulmonary sequestration is not possible in most of the cases. A 2-year-old boy presented with recurrent episodes of chest infections and respiratory distress. A preoperative diagnosis of congenital lobar emphysema was made on the basis of chest radiograph and computed tomography scan. At operation, an intralobar pulmonary sequestration was found. The sequestration cyst was excised with uneventful recovery.