Juvenile dermatomyositis (JDM) is a rare pediatric autoimmune disease. A distinct clinical phenotype is associated with anti-melanoma differentiation-associated gene 5 (anti-MDA5) autoantibodies, which are linked to features such as arthritis, ulcerative skin lesions, and a heightened risk of interstitial lung disease (ILD), including its rapidly progressive form (RP-ILD). Despite increased recognition of this phenotype in East Asian, European, and North American populations, significant gaps remain in understanding its pathogenesis, and no consensus has been reached regarding optimal treatment strategies. Moreover, data on anti-MDA5-associated JDM in African populations are nonexistent. We report the first three documented cases of anti-MDA5-positive JDM with ILD in African children. All patients exhibited characteristic extramuscular manifestations, and all had pulmonary involvement, which was rapidly progressive in two children, one of whom died. The clinical course, diagnostic findings, and treatment strategies are discussed in the context of existing literature. A review of the literature was performed to evaluate the prevalence, clinical presentation, and treatment approaches for RP-ILD in anti-MDA5-associated JDM across different populations. These cases highlight the wide heterogeneity of clinical phenotypes associated with anti-MDA5 autoantibodies in JDM. Given this variability, individualized monitoring and management strategies are essential to optimize outcomes.

BackgroundCritically ill children on peritoneal dialysis (PD) are at an increased risk of raised intra-abdominal pressure (IAP). The aim of this study was to describe IAP and complications with a standard PD fill volume, using conventional PD and continuous flow PD (CFPD) in critically ill children with acute kidney injury. A secondary objective was to compare two IAP measurement techniques, which were direct measurement from the PD catheter via a manometer and indirect measurement via the bladder catheter to a transducer.MethodThis study was a secondary analysis of a previously published randomized controlled crossover trial. Within- and between-group changes in IAP, blood pressure and ventilation parameters were analysed using paired t-tests. Bland-Altman and intraclass correlation tests were used to assess agreement between direct and intravesicular measurement of IAP.ResultsFifteen participants (median (range) age and weight 6.0 (0.2-14) months and 5.8 (2.3-14.0) kg) were included, of which 9 (60%) had raised IAP (>10 mmHg) after filling. No children developed compartment syndrome or other complications. Mean ± standard deviation IAP on conventional versus CFPD was 9.35 ± 2.97 and 11.5 ± 2.96 versus 9.3 ± 3.35 and 11.2 ± 3.62 when measured directly and indirectly, respectively (p > 0.5). Intravesicular measurement of IAP was constantly significantly higher compared to the direct method (p = 0.002); however, there was good correlation (Pearson correlation coefficient 0.7; p < 0.001) and moderate reliability (ICC = 0.6) between the methods.ConclusionIncreased IAP (>10 mmHg) was common, although not associated with serious adverse events, and warrants monitoring in these patients. The pressures measured are different with the two techniques used, although they do correlate well. More studies are needed to define increased IAP depending on the measuring technique and inform IAP measurement practice in children with acute PD.

BACKGROUND:Blood group incompatibility previously represented an obstacle to living related donor (LRD) options; desensitization modalities have expanded LRD options. ABO-incompatible kidney transplants have been successful in adults and pediatric liver transplants, but to date not yet in pediatric kidney transplants in South Africa. CASE REPORT:Patient X is a 5 year old male with end-stage kidney failure due to Posterior Urethral Valves, requiring peritoneal dialysis pre-transplant. His sister was the only suitable LRD. The recipient was blood group A+; donor was blood group B+; HLA match was 5/10, nil HLA donor specific antibodies and negative CDC and Flow crossmatches. The recipients' anti-B titer pre-transplant was a maximum of 1:8. A pre-emptive desensitization dose of Rituximab (375 mg/m2) was administered 4 weeks before transplant. The anti-B titer decreased to 1:2 following the Rituximab (CD19% of 0%). Post-transplant, the Anti-B titers were monitored for the first 10 days, with a maximum titer of 1:8 (immunoadsorbtion therapy available if > 1:16, or if associated graft dysfunction). Immunosuppressant protocol consisted of Basiliximab, Tacrolimus, Azathioprine, and Prednisone. Post-transplant, the creatinine improved to 60-70 μmol/L at Week 1. 7 weeks post-transplant, serum creatinine increased > 10% with an antibody titer of 1:2. Kidney biopsy showed CNI-related toxicity; renal function improved with reduction in Tacrolimus doses. Kidney function remains stable 1-year post-transplant with nil episodes of rejection. CONCLUSION:This is the first ABO-incompatible kidney transplant in a pediatric patient in South Africa and represents an important step in expanding the pool of potential living related donors.

Multicystic dysplastic kidney disease (MCDK) is a congenital kidney anomaly frequently misdiagnosed as hydronephrosis on antenatal ultrasound. Confirmatory nuclear imaging is seldom available in resource-limited settings. The study aimed to assess the diagnostic accuracy of kidney ultrasound (KUB scan) for detecting paediatric MCDK, using Mercaptoacetyltriglycine-3 scan ([99mTc]Tc-MAG3) differential renal function as the reference standard. A retrospective diagnostic accuracy analysis of consecutive children under 13 years with suspected unilateral MCDK, who underwent both KUB and [99mTc]Tc-MAG3 scans within 6–8 weeks of presentation at Red Cross War Memorial Children’s Hospital between January 2014 and December 2023 was done. Diagnosis required characteristic MCDK features on KUB and absent function on [99mTc]Tc-MAG3 scans. Reporting adhered to the STARD guidelines. Of 793 eligible children, the [99mTc]Tc-MAG3 classified 101/101 (100.0

Multicystic dysplastic kidney disease (MCDK) is a notable congenital anomaly of the kidney and urinary tract, with potential risk for chronic kidney disease, yet data from sub-Saharan Africa remain scarce. This study examined the pattern of MCDK, associated contralateral kidney abnormalities, determined the predictors of MCDK involution and assessed short-term outcomes in children followed beyond one year in South Africa. This retrospective study involved children under 13 years of age with suspected unilateral MCDK, confirmed on kidney ultrasound and [99mTc]Tc-MAG3 scans at the Red Cross War Memorial Children’s Hospital between January 1, 2014, and December 31, 2023. Demographic, clinical, and radiologic data were obtained. The Log-rank test and Cox Proportional Hazards regression analyses were used to identify predictors of MCDK involution. Among 1,581 new cases, 98 (6.2

Dialytic sodium removal (DSR) is an important parameter of peritoneal dialysis (PD) adequacy. The aim of this study was to report the DSR of children with acute kidney injury (AKI) on a standard acute PD prescription and to compare it to that of children on continuous flow peritoneal dialysis (CFPD). A secondary analysis of prospectively collected data was performed from a published randomized controlled crossover trial comparing children on conventional PD and CFPD. The conventional PD prescription used: fill volume 20 mL/kg, glucose 2.5

Background: Protein loss and glucose absorption in children on acute peritoneal dialysis (PD) is important to inform dietary prescription, yet data are lacking in this regard. This study was a secondary analysis of a previously published crossover randomised controlled trial, aiming to describe glucose uptake and protein loss into dialysate among children with acute kidney injury (AKI) receiving PD. Methods: This secondary analysis described and compared dialysate albumin loss and glucose absorption in 15 children with AKI receiving PD or continuous flow peritoneal dialysis (CFPD). In addition, correlations between albumin loss, glucose absorption and other patient and dialysis factors were analysed. Results: Median (range) age and weight of participants were 6.0 (0.2–14) months and 5.8 (2.3–14.0) kg, respectively. Patients received approximately 8 h of dialysis on each modality; however, results were extrapolated and expressed per day. The mean ± SD albumin loss on conventional PD and CFPD was 0.3 ± 0.19 g/kg/day and 0.56 ± 0.5 g/kg/day, respectively, and the mean ± SD glucose absorption was 4.67 ± 2.87 g/kg/day and 3.85 ±4.1 g/kg/day, respectively. There was a moderate correlation between ultrafiltration and albumin loss during CFPD only (Pearson’s R = 0.61; p = 0.02). There were no significant differences between PD and CFPD for either glucose absorption or albumin loss; however, the study was not powered for this outcome. Conclusions: Protein losses and glucose absorption in children on PD with AKI are significant and should be considered when prescribing nutritional content. Protein losses on CFPD were twice as high as on conventional PD.

INTRODUCTION:In South Africa, only children considered eligible for transplantation are offered dialysis as bridge to kidney transplantation. Maintenance peritoneal dialysis (PD) is preferred and has several advantages over hemodialysis (HD). While awaiting transplantation, PD may be discontinued due to permanent transfer to HD or death while on PD, of which the occurrence and burden is not known in our setting. We investigated the rate of discontinuation of maintenance PD, and associated factors among children awaiting a kidney transplant under challenging socio-economic circumstances in a low resource setting. METHODS:Single center retrospective analysis of children receiving maintenance PD. Outcomes included the proportion of children who discontinued PD before transplantation, associated factors and timing of discontinuation, and the proportion transplanted. Time to discontinuation or transplantation was displayed using a Kaplan-Meier curve. RESULTS:Sixty-seven children who received maintenance automated PD as initial dialysis modality were identified from the kidney transplant waiting list between January 2009 and December 2018. Complete data was available for 52 of the 67 children. Four children had prior failed kidney transplants. The median age was 11 years (interquartile range 6.0, 13.1). Overall, 17/52 (32.7%) children discontinued PD, with 13 (25%) transfers to HD and 4 deaths (7.7%), whereas 29/52 (55.8%) received a kidney transplant. Three of the deaths were PD related. Six children remained on maintenance PD at the end of the study period. Over a half of our patients discontinued PD by 12 months, and 80% by 30 months. Most PD discontinuations were associated with peritonitis. CONCLUSIONS:The proportion discontinuing PD was high, highlighting the need to optimize measures to improve retention rates, especially through prevention of peritonitis.

Background Dialysis is lifesaving for acute kidney injury (AKI), but access is poor in less resourced settings. A "peritoneal dialysis (PD) first" policy for paediatric AKI is more feasible than haemodialysis in low-resource settings. Methods Retrospective review of modalities and outcomes of children dialysed acutely at Red Cross War Memorial Children's Hospital between 1998 and 2020. Results Of the 593 children with AKI who received dialysis, 463 (78.1%) received PD first. Median age was 9.0 (range 0.03-219.3; IQR 13.0-69.6) months; 57.6% were < 1 year old. Weights ranged from 0.9 to 2.0 kg (median 7.0 kg, IQR 3.0-16.0 kg); 38.6% were < 5 kg. PD was used more in younger children compared to extracorporeal dialysis (ECD), with median ages 6.4 (IQR 0.9-30.4) vs. 73.9 (IQR 17.5-113.9) months, respectively (p = 0.001). PD was performed with Seldinger soft catheters (n = 480/578, 83%), predominantly inserted by paediatricians at the bedside (n = 412/490, 84.1%). Complications occurred in 127/560 (22.7%) children receiving PD. Overall, 314/542 (57.8%) children survived. Survival was significantly lower in neonates (< 1 month old, 47.5%) and infants (1-12 months old, 49.2%) compared with older children (> 1 year old, 70.4%, p < 0.0001). Survival was superior in the ECD (75.4%) than in the PD group (55.6%, p = 0.002). Conclusions "PD First for Paediatric AKI" is a valuable therapeutic approach for children with AKI. It is feasible in low-resourced settings where bedside PD catheter insertion can be safely taught and is an acceptable dialysis modality, especially in settings where children with AKI would otherwise not survive.

Infection-related complications remain the most significant cause for morbidity and technique failure in infants, children and adolescents who receive maintenance peritoneal dialysis (PD). The 2024 update of the Clinical Practice Guideline for the Prevention and Management of Peritoneal Dialysis Associated Infection in Children builds upon previous such guidelines published in 2000 and 2012 and provides comprehensive treatment guidance as recommended by an international group of pediatric PD experts based upon a review of published literature and pediatric PD registry data. The workgroup prioritized updating key clinical issues contained in the 2012 guidelines, in addition to addressing additional questions developed using the PICO format. A variety of new guideline statements, highlighted by those pertaining to antibiotic therapy of peritonitis as a result of the evolution of antibiotic susceptibilities, antibiotic stewardship and clinical registry data, as well as new clinical benchmarks, are included. Recommendations for future research designed to fill important knowledge gaps are also provided.

Background: Kidney transplantation remains the treatment of choice for children with kidney failure (KF). In South Africa, kidney replacement therapy (KRT) is restricted to children eligible for transplantation. This study reports on the implementation of the Paediatric Feasibility Assessment for Transplantation (pFAT) tool, a psychosocial risk score developed in South Africa to support transparent transplant eligibility assessment in a low-resource setting. Methods: Single-center retrospective descriptive analysis of children assessed for KRT using pFAT tool from 2015 to 2021. Results: Using the pFAT form, 88 children (median [range] age 12.0 [1.1 to 19.0] years) were assessed for KRT. Thirty (34.1%) children were not listed for KRT, scoring poorly in all domains, and were referred for supportive palliative care. Fourteen of these 30 children (46.7%) died, with a median survival of 6 months without dialysis. Nine children were reassessed and two were subsequently listed. Residing >300 km from the hospital (p = .009) and having adherence concerns (p = .003) were independently associated with nonlisting. Of the 58 (65.9%) children listed for KRT, 40 (69.0%) were transplanted. One-year patient and graft survival were 97.2% and 88.6%, respectively. Only one of the four grafts lost at 1-year posttransplant was attributed to psychosocial issues. Conclusions: Short-term outcomes among children listed using the pFAT form are good. Among those nonlisted, the pFAT highlights specific psychosocial/socioeconomic barriers, over which most children themselves have no power to change, which should be systemically addressed to permit eligibility of more children and save lives.

Background. Histopathological patterns of childhood primary nephrotic syndrome (PNS) and clinical response to steroids have beenassociated with certain race groups in parts of South Africa. However, there are no recent studies of childhood PNS in Cape Town.Objectives. To describe the demographics, histological subtypes and steroid response of patients with PNS who underwent kidney biopsiesat Red Cross War Memorial Children’s Hospital (RCWMCH) over a 10-year period.Methods. Details of patients with PNS who underwent kidney biopsies in the Paediatric Nephrology Department at RCWMCH between2006 and 2015 were retrospectively recorded.Results. A total of 103 patients were included in the study. Most patients were either of mixed race (42%) or black (36%), with a mean age of6.8 years and a male-to-female ratio of 1.19:1. The most identified histopathological subtype was mesangioproliferative glomerulonephritis(MesPGN; 60% (n/N=62/103)). Of the patients with focal segmental glomerulosclerosis (FSGS), MesPGN and minimal change disease(MCD) 45% (n/N=43/95) were steroid-resistant, and 54% (n/N=51/95) were steroid-sensitive. There was no significant associationbetween any race group and steroid response. Patients with FSGS were more likely to be black, while MCD was more common in mixed-race patients (p=0.04). There was no difference in the likelihood of being mixed race or black between patients with FSGS and MesPGN(p=0.472).Conclusion. MesPGN was the most common histopathological subtype found in our study. There was no significant association betweenrace and steroid response. Patients with FSGS were more likely to be black than mixed race when compared with MCD patients. Race wasnot otherwise significantly associated with any histopathological subtype

Acute kidney injury (AKI) is a frequent complication of children admitted to the paediatric intensive care unit. One key management modality of AKI is the use of diuretics to reduce fluid overload. Aminophylline, a drug that is well known for its use in the treatment of bronchial asthma, is also purported to have diuretic effects on the kidneys. This retrospective cohort study assesses the effect of aminophylline in critically ill children with AKI. A retrospective chart review of children admitted to the paediatric intensive care unit of the Red Cross War Memorial Children’s Hospital (RCWMCH) with AKI who received aminophylline (from 2012 to June 2018) was carried out. Data captured and analyzed included demographics, underlying disease conditions, medications, urine output, fluid balance, and kidney function. Data from thirty-four children were analyzed. Urine output increased from a median of 0.4 mls/kg/hr [IQR: 0.1, 1.1] at six hours prior to aminophylline administration to 0.6 mls/kg/hr [IQR: 0.2, 1.9] at six hours and 1.6 mls/kg/hr [IQR:0.2, 4.2] at twenty-four hours post aminophylline therapy. The median urine output significantly varied across the age groups over the 24-h time period post-aminophylline, with the most response in the neonates. There was no significant change in serum creatinine levels six hours post-aminophylline administration [109(IQR: 77, 227)—125.5(IQR: 82, 200) micromole/l] P-value = 0.135. However, there were significant age-related changes in creatinine levels at six hours post-aminophylline therapy. Aminophylline increases urine output in critically ill children with AKI.

Our previously demonstrated continuous flow peritoneal dialysis (CFPD) technique in children with acute kidney injury (AKI), although effective, was manpower heavy and expensive due to the high-volume pumps required. The aim of this study was to develop and test a novel gravity-driven CFPD technique in children using readily available, inexpensive equipment and to compare this technique to conventional PD. After development and initial in vitro testing, a randomised crossover clinical trial was conducted in 15 children with AKI requiring dialysis. Patients received both conventional PD and CFPD sequentially, in random order. Primary outcomes were measures of feasibility, clearance and ultrafiltration (UF). Secondary outcomes were complications and mass transfer coefficients (MTC). Paired t-tests were used to compare PD and CFPD outcomes. Median (range) age and weight of participants were 6.0 (0.2–14) months and 5.8 (2.3–14.0) kg, respectively. The CFPD system was easily and rapidly assembled. There were no serious adverse events attributed to CFPD. Mean ± SD UF was significantly higher on CFPD compared to conventional PD (4.3 ± 3.15 ml/kg/h vs. 1.04 ± 1.72 ml/kg/h; p < 0.001). Clearances for urea, creatinine and phosphate for children on CFPD were 9.9 ± 3.10 ml/min/1.73 m2, 7.9 ± 3.3 ml/min/1.73 m2 and 5.5 ± 1.5 ml/min/1.73 m2 compared to conventional PD with values of 4.3 ± 1.68 ml/min/1.73 m2, 3.57 ± 1.3 ml/min/1.73 m2 and 2.53 ± 0.85 ml/min/1.73 m2, respectively (all p < 0.001). Gravity-assisted CFPD appears to be a feasible and effective way to augment ultrafiltration and clearances in children with AKI. It can be assembled from readily available non-expensive equipment.