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Background: This retrospective real-world study used data from two registries, International Pediatric Peritoneal Dialysis Network (IPPN) and International Pediatric Hemodialysis Network (IPHN), to characterize the efficacy and safety of continuous erythropoietin receptor activator (C.E.R.A.) in pediatric patients with chronic kidney disease (CKD) on peritoneal dialysis (PD) or hemodialysis (HD). Methods: IPPN and IPHN collect prospective data (baseline and every 6 months) from pediatric PD and HD centers worldwide. Demographics, clinical characteristics, dialysis information, treatment, laboratory parameters, number and causes of hospitalization events, and deaths were extracted for patients on C.E.R.A. treatment (IPPN: 2007–2021; IPHN: 2013–2021). Results: We analyzed 177 patients on PD (median age 10.6 years) and 52 patients on HD (median age 14.1 years) who had ≥ 1 observation while being treated with C.E.R.A. The median (interquartile range [IQR]) observation time under C.E.R.A. exposure was 6 (0–12.5) and 12 (0–18) months, respectively. Hemoglobin concentrations were stable over time; respective means (standard deviation) at last observation were 10.9 (1.7) g/dL and 10.4 (1.7) g/dL. Respective median (IQR) monthly C.E.R.A. doses at last observation were 3.5 (2.3–5.1) µg/kg, or 95 (62–145) µg/m2 and 2.1 (1.2–3.4) µg/kg, or 63 (40–98) µg/m2. Non-elective hospitalizations occurred in 102 (58%) PD and 32 (62%) HD patients. Seven deaths occurred (19.8 deaths per 1000 observation years). Conclusions: C.E.R.A. was associated with efficient maintenance of hemoglobin concentrations in pediatric patients with CKD on dialysis, and appeared to have a favorable safety profile. The current analysis revealed no safety signals. [ABSTRACT FROM AUTHOR]

Objective The present study aimed to assess the long-term safety and tolerability of valsartan in hypertensive children aged 6-17 years, with or without chronic kidney disease (CKD). Methods This was an 18-month, open-label, multicentre, prospective study conducted in 150 patients with history of hypertension with or without CKD. The primary endpoint was long-term safety and tolerability of valsartan and valsartan-based treatments, assessed in terms of adverse events (AEs), serious AEs, laboratory measurements, estimated glomerular filtration rate (eGFR), urinalysis and electrocardiogram. Results Of 150 enrolled patients, 117 (78%) completed the study. At week 78, a clinically and statistically significant reduction in mean sitting systolic and diastolic blood pressures was observed in all patients (- 14.9 mmHg and - 10.6 mmHg, respectively). Within the first 3 months of treatment, mean urine albumin creatinine ratio decreased in CKD population, which was sustained. A higher percentage of CKD patients had at least one AE compared to non-CKD patients (85.3% vs. 73.3%, respectively). The majority of AEs were mild (50.7%) or moderate (18.7%) in severity. As expected, in patients with underlying CKD, increases in serum potassium, creatinine and blood urea nitrogen were more commonly reported compared to non-CKD patients. A > 25% decrease in Schwartz eGFR was observed in 28.4% of CKD patients and 13.5% of non-CKD patients. Conclusions Valsartan was generally well tolerated, with an AE profile consistent with angiotensin receptor blockers in the overall population and in patients with underlying CKD. Long-term efficacy was maintained and a beneficial effect on proteinuria was observed., Author(s): Randall Lou-Meda [sup.1] , Brigitte Stiller [sup.2] , Zenaida L. Antonio [sup.3] , Ewa Zielinska [sup.4] , Hui-Kim Yap [sup.5] , Hee Gyung Kang [sup.6] , Monique Tan [sup.7] [...]

Author(s): Anil Vasudevan [sup.1] , Kishore Phadke [sup.2 ] , Hui-Kim Yap [sup.3] [sup.4] Author Affiliations: (1) 0000 0004 1770 8558, grid.416432.6, Department of Pediatric Nephrology, St. John's Medical College [...]

Neonatal renal vascular thrombosis is rare but has devastating sequelae. The renal vein is more commonly affected than the renal artery. Most neonates with renal vein thrombosis present with at least one of the three cardinal signs, namely, abdominal mass, macroscopic hematuria and thrombocytopenia, while unilateral renal artery thrombosis presents with transient hypertension. Contrast angiography is the gold standard for diagnosis but because of exposure to radiation and contrast agents, Doppler ultrasound scan is widely used instead. Baseline laboratory tests for platelet count, prothrombin time, activated partial thromboplastin time and fibrinogen concentration are essential before therapy is initiated. Maternal blood is tested for lupus anticoagulant and anticardiolipin antibody. Evaluation for prothrombotic disorders is warranted when thrombosis is clinically significant, recurrent or spontaneous. Management should involve a multidisciplinary team that includes neonatologists, radiologists, pediatric hematologists and nephrologists. In addition to supportive therapy, recent guidelines recommend at least prophylactic heparin therapy in the majority of cases to prevent thrombus extension. Thrombolytic therapy is reserved for bilateral thrombosis compromising kidney function. Long-term sequelae, such as kidney atrophy, systemic hypertension and chronic kidney disease, are common, and follow-up by pediatric nephrologists is recommended for monitoring of kidney function, early detection and management of hypertension and chronic kidney disease., Author(s): Lourdes Paula R. Resontoc[sup.1] , Hui-Kim Yap[sup.1] [sup.2] Author Affiliations: (1) Shaw-NKF-NUH Children's Kidney Center, KTP-National University Children's Medical Institute, National University Health System, Singapore, Singapore (2) Department of [...]

Background: Children with chronic kidney disease (CKD) generally have worse educational and psychosocial outcomes compared with their healthy peers. This can impair their ability to manage their treatment, which in turn can have long-term health consequences through to adulthood. We attempted to capture the experiences of children with CKD and to describe the perspectives of their parents and caregivers on access to educational and psychosocial support. Methods: Children with CKD (n = 34) and their caregivers (n = 62) were sampled via focus groups from pediatric hospitals in Australia, Canada, and the USA. Sixteen focus groups were convened and the transcripts were analyzed thematically. Results: We identified four themes: disruption to self-esteem and identity (emotional turmoil of adolescence, wrestling with the sick self, powerlessness to alleviate child's suffering, balancing normality and protection); disadvantaged by lack of empathy and acceptance (alienated by ignorance, bearing the burden alone); a hidden and inaccessible support system (excluded from formal psychological support, falling behind due to being denied special considerations); and building resilience (finding partners in the journey, moving towards acceptance of the illness, re-establishing childhood). Conclusions: Children with CKD and their caregivers encountered many barriers in accessing psychosocial and educational support and felt extremely disempowered and isolated as a consequence. Improved availability and access to psychosocial and educational interventions are needed to improve the wellbeing and educational advancement of children with CKD. A higher resolution version of the Graphical abstract is available as Supplementary information. [ABSTRACT FROM AUTHOR]

Kidney replacement therapy (KRT) is used to provide supportive therapy for critically ill patients with severe acute kidney injury and various other non-renal indications. Modalities of KRT include continuous KRT (CKRT), intermittent hemodialysis (HD), and sustained low efficiency daily dialysis (SLED). However, circuit clotting is a major complication that has been investigated extensively. Extracorporeal circuit clotting can cause reduction in solute clearances and can cause blood loss, leading to an upsurge in treatment costs and a rise in workload intensity. In this educational review, we discuss the pathophysiology of the clotting cascade within an extracorporeal circuit and the use of various types of anticoagulant methods in various pediatric KRT modalities. [ABSTRACT FROM AUTHOR]

Background Initiating continuous renal replacement therapy (CRRT) in infants exposes them to the dual hemodynamic challenges of high circuit extracorporeal volumes and potential membrane reactions, in the case of acrylonitrile AN69 membranes. The use of the new Prismaflex HF20 membrane in hemodynamically unstable low-body-weight infants on inotropic support has not been reported. Treatment We describe the use of the HF20 (Gambro Lundia AB, Lund, Sweden) membrane in four low-body-weight infants (2.3 to 5.4 kg) with multi-organ dysfunction syndrome who were critically ill in the Pediatric Intensive Care Unit (PICU), hemodynamically unstable, and on inotropes. We were able to achieve target volume loss in all infants without compromising their hemodynamic status. Mean arterial pressures were maintained between 39 and 57 mmHg. The relatively low circuit volume of the HF20 set (60 ml) obviated the need for blood prime in the majority; however, when blood prime was required, there was no adverse reaction with the polyarylethersulfone (PAES) membrane. Solute clearance in these small infants was efficient with correction of metabolic acidosis and electrolyte abnormalities. Excellent circuit lifespan (56.3 [+ or -] 32.3 h) was observed. Conclusions CRRT using the HF20 membrane is safe and hemodynamically well tolerated in high-risk, unstable low-body-weight infants with cardiac dysfunction on multiple inotropes. Keywords Continuous renal replacement therapy * HF20 * Infant * Acute kidney injury, Introduction Critically ill, low-body-weight infants weighing less than 10 kg represent the most technically challenging selection of patients for continuous renal replacement therapy (CRRT). In addition to access issues, the [...]

Chronic peritoneal dialysis (PD) for children in Singapore was instituted in 1988 at the National University Hospital with adult nurses providing dialysis services during the first 10 years. In 1998, a specialist pediatric dialysis nursing team was recruited. This study was conducted to determine the impact of dialysis nursing service on PD-related outcomes during the two nursing periods. Comparing the adult (group 1) and pediatric (group 2) nursing periods, the peritonitis rate was significantly higher in group 1 (RR 1.90; 95%CI 1.27-2.84), and this association did not weaken after adjusting for age, gender, and exit site infections. Exit site infection rate (RR 2.16; 95%CI 1.44-3.23), risk of peritonitis during the first year (RR 3.65; 95%CI 1.68-7.90), and multiple peritonitis attacks (RR 2.45; 95%CI 1.321.55) were higher in group 1. The peritonitis rates for adult patients cared for by the same adult nurses declined sharply from 1.05 episodes per patient-year between 1989 and 1992 to 0.41 episodes per patient-year between 1995 and 1997, however the corresponding pediatric rates did not change (1.48 to 1.06 episodes per patient-year, respectively) until the second era when specialized pediatric nurses were available. In conclusion, establishment of a specialist pediatric dialysis nursing team resulted in significant improvement in infection-related PD outcomes. Keywords Peritoneal dialysis * Nursing service * Peritonitis * Exit site infection * Catheter, Introduction Peritoneal dialysis (PD) is the preferred modality of dialysis in children with end-stage renal disease (ESRD) [1]. Children with chronic illnesses have special needs. The child's physiology, psychosocial make-up, [...]

Byline: Wai Cheung (1), Chang-Li Wei (1), Ching-Ching Seah (1), Stanley C. Jordan (2), Hui-Kim Yap (1) Keywords: Steroid-responsive nephrotic syndrome; Immunoglobulins; Atopy; Th1/Th2 cytokine Abstract: Earlier studies have demonstrated a strong association of steroid-responsive nephrotic syndrome (SRNS), atopy, and elevated serum IgE levels. Interleukin (IL-13) gene expression is significantly increased in children with SRNS in relapse. As interferon (IFN)-I3, IL-13, and IL-4 have regulatory effects on IgE synthesis, we examined the relationship between intracellular cytokine production and serum IgE levels in children with SRNS, in order to further define the reported association with atopy. The median serum IgE levels in nephrotic patients in relapse with (492 U/ml) or without atopy (561 U/ml) were significantly higher than those in remission (221 U/ml, P&lt 0.002 or 90 U/ml, P&lt 0.001, respectively) and non-atopic controls (177 U/ml) (P&lt 0.001). The percentage of CD3+ IL-13-producing cells was significantly higher in nephrotic children in relapse, and correlated with the serum IgE levels during the active phase of the disease (r=0.90, P&lt 0.001). These data suggest that the elevated serum IgE levels during relapses of SRNS were the result of upregulation of IL-13. This probably reflects some common immune activation following various stimuli, rather than a direct association with atopy. Author Affiliation: (1) Department of Pediatrics, National University of Singapore, 5 Lower Kent Ridge Crescent, 119074, Singapore (2) Steven Spielberg Pediatric Research Center, Cedars-Sinai Medical Center, UCLA School of Medicine, Los Angeles, CA 90028, USA Article History: Registration Date: 30/01/2004 Received Date: 04/08/2003 Accepted Date: 20/01/2004 Online Date: 03/04/2004

Byline: Wei-Kin Gong (1), Teng-Hong Tan (1), Pei-Pei Foong (1), Belinda Murugasu (1), Hui-Kim Yap (1) Keywords: KeywordsaAcute peritoneal dialysis; Continuous hemodiafiltration; Mortality Abstract: This study reviewed the 18-year experience of acute dialysis in the pediatric intensive care unit, in order to identify factors that could predict outcome, and to determine whether newer modalities of acute dialysis have influenced this outcome. Sixty-six children (ages 1 day to 19 years) received acute dialysis from May 1980 to April 1998. Factors predicting outcome were analyzed using univariate and Cox regression analysis. Modality of dialysis in the first 15 years was exclusively peritoneal dialysis, with a mortality of 63.9%. However, in the last 3 years, with increasing patient numbers, continuous hemodiafiltration (CHDF) was the modality of choice (56.7%), with a mortality of 73.3%. Univariate analysis showed that age &lt 1 year, coma, acute tubular necrosis, disseminated intravascular coagulopathy, assisted ventilation, and hypotension were associated significantly with poor outcome (P&lt 0.05). Cox regression analysis revealed that mortality was significantly higher in patients on mechanical ventilation (RR 5.96, 95% CI 1.82--19.50), or with age &lt 1 year (RR 2.00, 95% CI 1.08--3.73). In conclusion, despite the increasing use of CHDF over the last 3 years, there was no significant improvement in mortality, probably related to the fact that more critically ill patients were dialyzed. Author Affiliation: (1) Department of Pediatrics, National University of Singapore, Singapore, SG (2) Department of Pediatrics, National University Hospital, Lower Kent Ridge Road, Singapore 119074 Fax: +65-779-7486, SG Article note: Received: 21 March 2000 / Revised: 12 October 2000 / Accepted: 19 October 2000

Byline: Y. S. Lee (1), Hui Kim Yap (2), Bruce A Barshop (3), Yoke Sun Lee (4), Sinniah Rajalingam (4), Kah Yin Loke (2) Keywords: Keywords Mitochondrial disorder; Proximal renal tubular dysfunction; Hypoparathyroidism Abstract: We report a rare presentation of mitochondrial disorder in a child with recurrent carpopedal spasms due to hypocalcemia and hypomagnesemia, secondary to renal proximal tubulopathy and possible hypoparathyroidism. At least two mutant mitochondrial DNA species were identified, and abnormal mitochondria were found in the muscle and renal biopsy specimens. The case illustrates the spectrum and diversity of mitochondrial presentations, arising because of heteroplasmy of mutations and the type of organs affected. Author Affiliation: (1) National University of Singapore, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074. paeleeys@nus.edu.sg, SG (2) Department of Pediatrics, National University of Singapore, Singapore, SG (3) Department of Pediatrics, University of California, San Diego, California, USA, US (4) Department of Pathology, National University of Singapore, Singapore, SG Article note: Received: 31 October 2000 / Revised: 16 April 2001 / Accepted: 17 April 2001

Byline: Hui-Kim Yap (1), Kah-Yin Loke (1), Belinda Murugasu (1), Bee-Wah Lee (1) Keywords: Key words: Growth hormone; Systemic lupus erythematosus Abstract: The effect of growth hormone (GH) on subclinical disease activity in a 15-year-old boy with previously quiescent lupus nephritis and chronic renal failure is described. Institution of supraphysiological doses of GH resulted in a rise in erythrocyte sedimentation rate, decrease in serum complement, rise in anti-DNA antibody titers, and increase in T-cell activation markers, all of which improved following cessation of GH treatment. Author Affiliation: (1) Department of Pediatrics, National University of Singapore, Singapore, SG Article note: Received April 3, 1997 received in revised form September 2, 1997 accepted September 3, 1997

Coronavirus disease 2019 (COVID-19) has rapidly spread not only in China but throughout the world. Children with kidney failure (chronic kidney disease (CKD) stage 5) are at significant risk for COVID-19. In turn, a set of recommendations for the prevention and control of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and COVID-19 in pediatric hemodialysis (HD) centers and in home peritoneal dialysis (PD) settings have been proposed. The recommendations are based on the epidemiological features of the SARS-CoV-2 virus and COVID-19 disease, susceptibility factors, and preventive and control strategies. These recommendations will be updated as new information regarding SARS-CoV-2 and COVID-19 becomes available. [ABSTRACT FROM AUTHOR]

Earlier studies have demonstrated a strong association of steroid-responsive nephrotic syndrome (SRNS), atopy, and elevated serum IgE levels. Interleukin (IL-13) gene expression is significantly increased in children with SRNS in relapse. As interferon (IFN)-γ, IL-13, and IL-4 have regulatory effects on IgE synthesis, we examined the relationship between intracellular cytokine production and serum IgE levels in children with SRNS, in order to further define the reported association with atopy. The median serum IgE levels in nephrotic patients in relapse with (492 U/mI) or without atopy (561 U/mI) were significantly higher than those in remission (221 U/mI, P<0.002 or 90 U/mI, P<0.001, respectively) and nonatopic controls (177 U/mI) (P<0.001). The percentage of CD3+ IL-13-producing cells was significantly higher in nephrotic children in relapse, and correlated with the serum IgE levels during the active phase of the disease (r=0.90, P<0.001). These data suggest that the elevated serum IgE levels during relapses of SRNS were the result of upregulation of IL-13. This probably reflects some common immune activation following various stimuli, rather than a direct association with atopy. [ABSTRACT FROM AUTHOR]

This study compared the pattern of acute glomerulonephritis (AGN), a disease known to be influenced by socioeconomic and environmental factors, in children 12 years and under, for the years 1971 and 1985. All children admitted to the four major paediatric departments with haematuria and at least two of the following (oedema, hypertension or oliguria) had an initial diagnosis of AGN. A sample population from one unit from 1980 to 1984 showed that over 70% of these children had evidence of a post-streptococcal aetiology. In 1971, 411 children were admitted with AGN, as compared with only 58 in 1985. The age-sex-race standardized rates for 1971 and 1985 were 0.632 and 0.023/1,000 children 12 years and under, respectively ( P<0.001). The mean age of presentation was lower in 1971. Over this period, Singapore saw a threefold rise in the gross national product, accompanied by rapid urbanization. On analysis of the housing pattern, only 31% of the children lived in high-rise apartments in 1971, in contrast with 86% in 1985 ( P<0.001). The majority of non-apartment dwellers had homes in runal districts. From an epidemiological perspective, factors which could have led to the highly significant decline in prevalence of AGN in Singapore children included improvement in the socioeconomic status and health care system, and urbanization of the country. [ABSTRACT FROM AUTHOR]