Pediatric Nephrology Research Articles

The European Rare Kidney Disease Reference Network

Rare kidney diseases encompass awide range of congenital, inherited and acquired conditions. Two million Europeans are affected by rare kidney diseases. The European Rare Kidney Disease Reference Network (ERKNet) aims to improve the clinical management of patients with these diseases. ERKNet encompasses 95highly specialized adult and pediatric nephrology units at 72sites in 24European Union (EU) member states, as well as agroup of patient advocates (European Patient Advocacy Group, ePAG). ERKNet centers care for more than 65,000 rare kidney disease patients and pursues avariety of activities. An online consultation service helps improve the management of complex cases. Expert working groups develop clinical practice guidelines for individual groups of rare kidney diseases. In a3-year postgraduate program, junior physicians at ERKNet centers are trained by experts in the diagnosis and treatment of rare kidney diseases through webinars and case-based eLearning modules. Information brochures and online texts on rare kidney diseases for patients, relatives and the general public are produced and disseminated. Clinical research is supported by aEuropean Registry for Rare Kidney Diseases (ERKReg), which provides important information on demographics and disease progression and facilitates the identification of patient cohorts for therapeutic studies. In addition, the registry provides clinical performance statistics of reference centers and allows benchmarking to promote the harmonization and standardization of care for rare kidney disease patients across Europe.

Hubungan Antara Sindrom Nefrotik Resisten Steroid Dengan Gangguan Pertumbuhan Pada Anak

Background: The incidence of nephrotic syndrome is increasing and has been reported to be the second most common disease found in pediatric nephrology. Most children with nephrotic syndrome tend to be picky eaters and have poor appetite due to malabsorption due to intestinal edema and abdominal pain, combined with enteropathic protein loss which can further impair nutrition. In addition, corticosteroid treatment in patients with nephrotic syndrome also has an important role in growth disorders in children that can have a long-term effect and reduce their quality of life if not detected and not intervened early. Aims : to identify the association between steroid-sensitive nephrotic syndrome and growth retardation in children Methods: This study used a cross-sectional study. The calculation of the sample size used the unpaired categorical analytic formula and purposive sampling method in taking the sample size. The research subjects in this study was 50 patients with nephrotic syndrome. Data analysis was performed using non-parametric statistics testing, Chi-square. Results: The results of the Chi Square test showed a significant value or p value = 0.248 (p> 0.05, so it can be concluded that there is no relationship between steroid-resistant nephrotic syndrome (SNRS) and growth retardation in children. Odds Ratio in this study is 1.962. Conclusion: There is no significant association between steroid resistant nephrotic syndrome and growth disorders in children. Keywords: Nephrotic Syndrome, Growth Retardation

Pathophysiology of Congenital Anomalies of the Kidney and Urinary Tract: A Comprehensive Review

Congenital anomalies of the kidney and urinary tract (CAKUT) represent a broad range of diseases with differing mechanisms, clinical presentations, and prognoses. With an estimated prevalence of between 4 and 60 per 10,000 births, CAKUT represents a sizable number of patients for pediatric and adult nephrologists as therapies have progressed, allowing longer life spans. Many CAKUT disorders are associated with genetic mutations, and with advances in genomic sequencing, these genes are being identified at an increasing rate. Understanding these mutations provides insight into these conditions’ molecular mechanisms and pathophysiology. In this article, we discuss the epidemiology, presentation, and outcomes of CAKUT in addition to our current understanding of genetic and molecular mechanisms in these diseases.

REHABILITATION IN NEPHROLOGY: HISTORY, CURRENT STATE, PROSPECTS

Rehabilitation assistance at healthcare institutions is a comprehensive package of measures designed to enhance the functional abilities of individuals who experience or may potentially experience limitations in their daily functioning within their surrounding environment. Nephrology is a branch of medical science that studies the etiology and pathogenesis of kidney damage, develops and improves diagnostic methods, treatments, and prevention strategies for congenital and acquired primary and secondary kidney damage at all stages of development. Pediatric urological disorders remain a signifi cant concern due to their high prevalence among children. Chronic kidney disease (CKD) is a particularly worrisome complication, as it is characterized by a relapsing nature and the potential for severe complications. The current system of healthcare is regarded as a costly branch of medical services, with expenditures on therapeutic and rehabilitative care (inpatient and outpatient stages) constituting a signifi cant proportion of the overall costs.The article presents the results of an analysis and a summary of the recommendations set forth in international and Ukrainian medicaltechnologicaldocuments based on evidence- based medicine with regard to rehabilitation care in pediatric nephrology. The methodology for establishing a clinical and rehabilitation patient pathway, comprising an individual rehabilitation patient plan, is presented. This pathway considers the most common urinary diseases among children at the hospital district level. The scientifi c research was conducted within the scientifi c focus of the Department of Pediatrics, Neonatology and Perinatal Medicine of Bukovinian State Medical University and the research project entitled «Chronobiological and Adaptive Aspects and Features ofAutonomic Regulation in Pathological Conditions in Children of Diff erent Age Groups.» The registration number is 0122U002245, and the project will run from January 1, 2022, to December 31, 2026.

Care of children with posterior urethral valves after initial endoscopic incision/ablation: what a nephrologist needs to know.

Posterior urethral valves (PUV) are the most common cause of congenital urethral obstruction and are unique in the challenges they pose in management. Endoscopic ablation/incision of the valves is usually offered as the primary treatment of choice. Following this, a range of different clinical patterns are observed, each with varying bladder dysfunction and continence issues and associated with different grades of chronic kidney disease. This review outlines a systematic approach that could help pediatric nephrologists, pediatricians, and pediatric urologists, as well as nursing and allied health specialists, assess these children and develop well-informed management plans. The need for surveillance for bladder dysfunction (incidence approximately 55%), identification of red flags for progression to kidney failure (incidence approximately 20%), and multidisciplinary approach to care are presented, with a focus on reducing long-term morbidity in patients and difficulties for the families. Where possible, an alternative in resource-constrained situations is suggested. We also briefly outline the role of pharmacotherapy, assisted bladder emptying/drainage, and other interventions that have a role in the medium- to long-term management of these patients.

Fluid deresuscitation in critically ill children: comparing perspectives of intensivists and nephrologists.

Fluid accumulation, presently defined as a pathologic state of overhydration/volume overload associated with clinical impact, is common and associated with worse outcomes. At times, deresuscitation, the active removal of fluid via diuretics or ultrafiltration, is necessary. There is no consensus regarding deresuscitation in children admitted to the pediatric intensive care unit. Little is known regarding perceptions and practices among pediatric intensivists and nephrologists regarding fluid provision and deresuscitation. Cross-sectional electronic survey of pediatric nephrologists and intensivists from academic societies in the United States designed to better understand fluid management between disciplines. A clinical vignette was used to characterize the perceptions of optimal timing and method of deresuscitation initiation at four timepoints that correspond to different stages of shock. In total, 179 respondents (140 intensivists, 39 nephrologists) completed the survey. Most 75.4% (135/179) providers believe discussing fluid balance and initiating fluid deresuscitation in pediatric intensive care unit (PICU) patients is "very important". The first clinical vignette time point (corresponding to resuscitation phase of early shock) had the most dissimilarity between intensivists and nephrologists (p = 0.01) with regards to initiation of deresuscitation. However, providers demonstrated increasing agreement in their responses to initiate deresuscitation as the clinical vignette progressed. Compared to intensivists, nephrologists were more likely to choose "dialysis or ultrafiltration" as a deresuscitation method during the optimization [10.3 vs. 2.9% (p = 0.07)], stabilization [18.0% vs. 3.6% (p < 0.01)], and evacuation [48.7% vs. 23.6% (p < 0.01)] phases of shock. Conversely, intensivists were more likely to utilize scheduled diuretics than nephrologists [47.1% vs. 28.2% (p = 0.04)] later on in the patient course. Most physicians believe that discussing fluid balance and deresuscitation is important. Nevertheless, when to initiate deresuscitation and how to accomplish it differed between nephrologist and intensivists. Widely understood and operationalizable definitions, further research, and eventually evidence-based guidelines are needed to help guide care.

The Efficacy and Outcomes of Renal Replacement Therapy in Pediatric Metabolic Disorders.

Background/Objectives: This study aims to evaluate the efficacy and outcomes of renal replacement therapy (RRT) in pediatric patients with metabolic diseases, specifically focusing on the impact of hemodialysis (HD) and peritoneal dialysis (PD) on clinical parameters, toxin reduction, and long-term survival. Methods: This retrospective study included 10 pediatric patients (eight females and two males) treated at a pediatric nephrology department between 2020 and 2023. Patients diagnosed with metabolic disorders, including maple syrup urine disease (MSUD), methylmalonic acidemia (MMA), and glycogen storage disease (GSD), underwent RRT. Clinical data, demographic information, and biochemical parameters were collected and analyzed. Results: Among the patients, 50% were diagnosed with MSUD, 30% with MMA, and 20% with GSD. RRT, including HD and PD, was administered to manage acute metabolic crises. HD was particularly effective in rapidly reducing toxic metabolite levels. Patients treated with HD showed significant reductions in leucine and ammonium levels, with median reductions of 94.5% and 86%, respectively. Overall, 60% of the patients demonstrated long-term survival, highlighting the critical role of RRT in managing metabolic crises. In conclusion, RRT, including HD and PD, is crucial in managing pediatric metabolic disorders by effectively reducing toxic metabolite levels and improving clinical outcomes. Conclusions: The results of this study are consistent with previous research, highlighting the critical role of RRT in the acute management of metabolic crises and supporting its adoption as a standard treatment method.

The diagnostic value of cytokines in chronic glomerulonephritis in children

In modern clinical practice of pediatricians, the problem of timely adequate treatment of chronic glomerulonephritis (CGN) in children is of particular relevance, due to both the high prevalence of the disease and the severity of its course, the complexity of therapy and the ambiguity of the prognosis. Lesions of the urinary system in children are not only common, but also tend to grow, and often at an early age. Deterioration of the environmental background, toxic and allergic effects of drugs lead to damage primarily to the kidneys, which are the eliminating organ. The aim of the study was to study urocytokines to assess the immune response in children with HCG, depending on the association with cytomegalovirus infection. The study included 100 children aged 4-7 years, permanently residing in the Bukhara region of the Republic of Uzbekistan. At the time of the study, the patients were undergoing routine treatment in the Department of Pediatric Nephrology of the Bukhara Regional Children’s Multidisciplinary Medical Center. All children underwent general clinical (general blood test with leukoformula, general urine analysis, biochemical blood test with determination of urea, creatinine and cystatin C, immunological (TNFα, IL-18, MCP-1, IgM and IgG antibodies to cytomegalovirus infection (CMVI) in blood serum, IL-1β and IL-17A in urine) examination methods. Thus, it was found that the concentration of cystatin C in the blood serum was inversely proportional to the glomerular filtration rate in the kidneys – with a decrease in kidney function in sick children, a twofold increase was noted, that is, the accumulation of cystatin C in the blood. An increase in the concentration of IL-18 in serum and IL-1β in urine relative to the control group in CGN was an indicator of the severity of an autoimmune reaction, and a relatively low concentration of cytokines in both blood and urine in CGN with CMVI indicated suppression of the specific antiviral immunity of CMVI. It was found that an increase in serum MCP-1 by 1.4 times in group 1 and 2.9 times in group 2 of CGN with CMVI is an indicator of viral kidney damage. A significantly high concentration of IL-17A in urine in CGN with CMVI indicated local cytokine production in the kidneys and acted as an indicator of the prognosis of the outcome of CGN.

International expert consensus statement on the diagnosis and management of congenital nephrogenic diabetes insipidus (arginine vasopressin resistance).

Congenital nephrogenic diabetes insipidus (NDI; also known as arginine vasopressin resistance) is a rare inherited disorder of water homeostasis, caused by insensitivity of the distal nephron to arginine vasopressin. Consequently, the kidney loses its ability to concentrate urine, which leads to polyuria, polydipsia and the risk of hypertonic dehydration. The diagnosis and management of NDI are very challenging and require an integrated, multidisciplinary approach. Here, we present 36 recommendations for diagnosis, treatment and follow-up in both children and adults, as well as emergency management, genetic counselling and family planning, for patients with NDI. These recommendations were formulated and graded by an international group of experts in NDI from paediatric and adult nephrology, urology and clinical genetics from the European Rare Kidney Disease Reference Network and the European Society of Paediatric Nephrology, as well as patient advocates, and were validated by a voting panel in a Delphi process. The goal of these recommendations is to provide guidance to health care professionals who care for patients with NDI and to patients and their families. In addition, we emphasize the need for further research on different aspects of this potentially life-threatening disorder to support the development of evidence-based guidelines in the future.

Long term outcome of Immunoglobulin M (IgM) Nephropathy of Children in National Institute of Kidney Diseases &amp; Urology (NIKDU), Dhaka, Bangladesh

Background: Immunoglobulin M nephropathy (IgMN) is a new clinicoimmunopathologic entity which present mainly as idiopathic nephrotic syndrome in both children and adults. The clinical manifestation of IgMN are highly variable presenting with isolated haematuria or asymptomatic proteinuria. About 6 to 23 percent of IgMN patient developed end stage kidney disease. Usually treated with immunosuppressive agents (cyclosporine/tacrolimus) along with corticosteroid has response rates up to 50%. In our institute last few years we have observed a surprisingly rise of IgM nephropathy in children. Objective: To see the response of IgMN children treated with tacrolimus or mycophenolate mofetil(MMF) and long term follow up to observe their outcome. Methodology : This was a prospective observational study done in the Department of Paediatric Nephrology, National Institute of Kidney Diseases &amp; Urology, Dhaka, starting from January/2014 to December/2017. The study population consists of 86 IgMN children, age ranges from 2 to 12 years. The study was approved by institutional ethical review committee and informed written consent was taken from every parents. Twenty seven patients(Group-I) were selected purposively and treated with MMF and 59 patients (Group- II) treated with tacrolimus for 2 years. Patients were followed up three monthly onward in a prescribed form including adverse effect of drugs. Clinical outcome data like remission, active disease, active disease with renal impairment and dependency on renal replacement therapy(RRT) were documented for statistical analysis. Results: Of 86 IgMN children most of them were older than &gt;8-12 years in both groups and the highest percentage were clinically diagnosed as frequently relapsing nephrotic syndrome. In Group I 43.48% children remain on remission, 13.04% have active disease, 30.43% are active disease with impaired renal function and 4.35 % went into RRT. In Group II 55.56% children remain on remission, 12.96 % have active disease, 16.67% are active disease with impaired renal function and 5.56% dependent on RRT. Conclusion: From above observations, the overall prognosis of IgMN children is not good. The clinical course and disease outcome did not differ significantly between two groups treated with mycophenolate mofetil and tacrolimus. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 34-38

Baseline characteristics and associated factors for hypertension in children with chronic kidney disease: results from the Korean Cohort Study for Outcome in Patients with Pediatric Chronic Kidney Disease study.

Hypertension is one of the most important complications of chronic kidney disease (CKD) as it exacerbates disease progression in children. The aim of this study is to identify characteristics and factors associated with hypertension in children with CKD. This is a cross-sectional study using baseline data from the 10-year ongoing cohort study named KNOW-PedCKD (Korean Cohort Study for Outcome in Patients with Pediatric Chronic Kidney Disease). We enrolled finally 378 patients aged <18 years at seven major pediatric nephrology centers in Republic of Korea. Blood pressure was measured and samples and clinical data were collected during the patients' annual hospital visits. We found that 30.7% of the patients had hypertension (n = 116); specifically, 16.4% (n = 62) had systolic hypertension, and 22.8% (n = 86) had diastolic hypertension. Multiple logistic regression analysis indicated that older age (odds ratio [OR], 1.13; p < 0.001), female sex (OR, 2.32; p = 0.002), a high left ventricular mass index (OR, 1.05; p < 0.001), and a high urine protein/creatinine ratio (OR, 1.12; p = 0.02) were significant associated factors for systolic or diastolic hypertension. This study analyzed the associated factors for hypertension in children with CKD. Hypertension is associated with various factors, including age, sex, heart status, and proteinuria. Therefore, clinicians should consider these factors during patient evaluations to improve health outcomes.

IPNA consensus definitions for clinical trial outcomes in steroid-resistant nephrotic syndrome.

Assessment of the true impact of therapeutic interventions is a challenge in the absence of universal, standardized definitions for clinical trial endpoints in children with kidney diseases. Steroid-resistant nephrotic syndrome (SRNS) is a difficult kidney disease to treat, with unremitting disease progressing to kidney failure. Currently, available therapies result in suboptimal cure rates. Clinical trials with innovative, targeted treatments will likely be conducted for this disease in the foreseeable future. An international consortium of the IPNA Best Practices and Standards Committee and the Pediatric Nephrology Expert Group of the conect4children (c4c) network developed through consensus, standardized, internationally acceptable definitions for trial outcomes for SRNS. The endpoint definitions were formulated for use with urine protein to creatinine ratios and estimated glomerular filtration rates. Definitions of complete remission, partial remission, non-remission of disease, reduction in proteinuria, kidney disease progression, kidney failure, and composite kidney outcome were refined using an iterative process until a consensus was achieved.

"I won't ever feel normal": experience reported through photovoice by children with chronic kidney disease.

Much of the global chronic kidney disease burden is experienced in low- and middle-income countries. Children living with chronic kidney disease (CKD) face medical and social challenges, and they need support at the individual and family levels. This study aimed to explore children's experiences living with kidney replacement therapy (KRT) who attend the largest pediatric nephrology department in Guatemala. This qualitative study used photovoice and asked children to take pictures that represented what is like to live with CKD. Each child and their caregiver underwent an interview where the photos were used to elicit and facilitate discussion. The interviews were recorded, transcribed, and then analyzed using thematic analysis. Eight children and their mothers participated in the study. Three themes were identified: interactions with the health system, changing and difficult family dynamics, and strains on social interactions. Children face social challenges including self-isolation and alienation. The family dynamics and familial structures often are forced to change, inducing stress. This is all exacerbated by the difficulties that arise in navigating the Guatemalan health system. Photovoice techniques are a feasible way to understand the experiences of children and their families who face CKD. The disease affects all aspects of life and recognizing this while advising and administering care can help provide a comprehensive level of care. Health systems need to make efforts aimed at improving the quality of care as well as the multidisciplinary support available to children and their families.

Pediatric nephrologists' perspectives and clinical practices related to genetic testing and education.

While genetic testing is now more accessible in pediatric nephrology, little is known about the views of pediatric nephrologists regarding genetic testing in clinical settings. An online 41-item survey was developed and distributed via professional listservs to self-identified U.S. licensed pediatric nephrologists from January 22 to May 4, 2021. Pediatric nephrologists had a high referral rate to genetic counseling and agreed on the significant impact of genetic testing on diagnosis, treatment, prognosis, counseling, and kidney transplant planning. Challenges for the utilization of genetic testing among pediatric nephrologists include the need to (1) learn how to counsel patients on the risks and benefits of genetic testing, (2) choose appropriate testing, (3) interpret genetic results, and (4) return those results to patients and families. There exists an opportunity to expand genetic testing education for pediatric nephrologists to assist incorporation of genetic testing into clinical practice.

Risk Factors of Relapse in Nephrotic Syndrome

Background: Nephrotic syndrome (NS) is a kidney disease with high incidence, typically in young children. NS is a disease of relapse, and it is a major problem to manage the cases with relapse. So, it is very important to find out such children who are prone to develop relapse. Hence, this study was carried out to find out the risk factors of relapse in children with NS. Objective: To determine the risk factors of relapse in patients with nephrotic syndrome. Methods: This case-control study was conducted at the Department of Paediatric Nephrology and Department of Paediatrics, Dhaka Medical College Hospital, for 12- months following ethical approval. A total of 100 NS children (age: 2-12 years) were enrolled in this study, among them 50 were cases (NS with relapse) and 50 were controls (initial attack of NS with no relapse within one year of disease onset). After obtaining written informed consent from parents/legal guardian, a detailed history, thorough clinical examination and necessary investigations were carried out in each patient during initial episode of NS. Data analysis was done by SPSS 26.0. Results: Mean age of the cases and controls was6.11±3.01years and 6.96±3.14years, respectively (p=0.169), with an overall boys-girl ration of 1.3:1. Maximum cases (56.0%) had infrequent relapse. Lower age of onset was prevalent among case group than control (31.96±15.15 months vs 46.48±27.76 months; p=0.002). Infection was significantly more prevalent among cases than controls (68.0% vs 38.0%; OR=3.46; p=0.003), UTI (38.0% vs 10.0%; OR; 5.52; p=0.001). Cases had significantly higher history of atopy (52.0% vs 30.0%; OR=2.53; p=0.025). Higher serum cholesterol (441.62±61.46 mg/dL vs 389.12±72.18 mg/dL; &lt;0.001) and lower serum albumin (15.74±2.20 gm/L vs 17.30±2.99 gm/L; p=0.004) were seen in cases than control. Conclusion: Lower age of onset, history of atopy, infection, higher serum cholesterol and lower serum albumin level during initial attack were associated .......

Human Immunodeficiency Virus Associated Neuropathy Perinatally Infected HIV Children In Calabar, Nigeria. Clinical Features, Treatment and Outcome

Background Renal disease is increasing being recognized as a significant cause of morbidity and mortality in HIV positive patients. Human Immunodeficiency virus associated nephropathy (HIVAN) is the most common type of HIV related renal disease and rapidly progress to end stage renal disease(ESRD). There is paucity of report on true prevalence of HIVAN in African. This study was aimed at determining the prevalence of HIVAN, clinical features, treatment and outcome, in Calabar Nigeria. Methods/Subject This was a retrospective review of all renal patients managed by Pediatric nephrology unit of University of Calabar Teaching Hospital from January 2016 to December 2022 enrolled in the Renal register. During the period of study 215 patients had renal diseases out of which 15 had HIVAN. The following information were extracted from the HIVAN patients; Demographics and clinical data as well as mode of transmission, laboratory investigations, renal Ultrasound scan, treatment and Outcome were obtained and analyzed using SPSS version 20. Results There were 215 cases of renal diseases seen during the study period of which 15 had HIVAN giving a prevalence of 6.9% There were 5 males and 10 females giving a ratio of 1:2, age range 69-192months. with a mean age of 127±43.7months All received HAART and had acquired HIV infection through vertical transmission. 8 (53%) of patient were asymptomatic with 6 (40%) presenting with both legs and facial swelling. Nephrotic range proteinuria was a common presentation seen in 40% of the patients and 5(33.3%) had hypertension. Only 4 (33%) had eGFR&lt;60 ml/min/1.73m2 and 2(16%) had ESRD. Two were lost to follow-up and 4 (26%) died with two requiring dialysis. Conclusion HIVAN is common in patients with renal diseases and there is need to monitor patient at initiation of HAART and at risk patients with low CD4+ count and exposure to nephrotoxic HAART.

Supporting Infants with Multicystic Dysplastic Kidney Disease: A Comprehensive Approach.

Multicystic dysplastic kidney (MCDK) is a congenital renal disease characterized by variable-sized noncommunicative cysts, impeding parenchymal development and functionality. Renal capabilities are relative to the functionality of the contralateral kidney and response to management. Unilateral and isolated cases are often asymptomatic with more positive outcomes, while severe bilateral derangements have a high mortality rate. We present a case of left-sided MCDK and right-sided renal dysplasia diagnosed at a nontertiary center. In addition, we offer a review of the epidemiology, epigenetics, and pathophysiology of MCDK. A concise discussion of prenatal, intrapartum, and postnatal renal function surveillance methods is presented to assist neonatal healthcare providers in collaborating with pediatric nephrology and urology specialists.

Alport syndrome: Expanding diagnosis and treatment

Alport syndrome (AS) is the second common monogenic cause of end-stage kidney disease (ESKD) worldwide and is caused by defective type 4 collagen due to pathogenic variants of COL4A3, COL4A4, or COL4A5. Type 4 collagen also exists in the eyes and ears, and thus ocular defects and hearing loss occur in AS. The understanding of AS has expanded over the past two decades due to greater availability of genetic testing and research on genotype-phenotype correlation. Patients previously diagnosed with idiopathic steroid resistant nephrotic syndrome or ESKD of unknown etiology may now be diagnosed as AS if pathogenic COL4A3-5 variants are identified. Some carriers of heterozygous COL4A3-5 variants may now be classified into females with X-linked AS or autosomal dominant AS, if there are typical pathologic changes in the glomerular basement membrane or if there is proteinuria and progression of kidney disease. Lastly, it has been recommended that renin-angiotensin-aldosterone system inhibition be started as soon as possible for selected AS patients for its long-term protective effect against kidney function deterioration. The purpose of this review is to introduce these important concepts to general pediatricians and pediatric nephrologists.

“Chronic Kidney Disease In Children Of A Paediatric Critical Care Nephrology &amp; Dialysis Department: A Study In Bangladesh Shishu (Children) Hospital &amp; Institute, Bangladesh”

Introduction: Chronic Kidney Disease (CKD) for children is a major problem of public health both in poor and developed countries. This study aimed to investigate the diagnosis and the management options of CRF for children. Patients and methods: This study retrospectively evaluated patients who had chronic kidney disease (CKD) in the Paediatric Critical Care &amp; Nephrology Department in Dhaka Shishu Children Hospital, Dhaka, Bangladesh between July 2017 and December 2017. Results: Over 6 month’s period, we diagnosed and managed 41 children with CRF. The estimated incidence of CRF is 4.7 new cases per million-child population per year. Parental consanguinity was found in 16 patients (39%). Family history with kidney disease was noted in 6 cases (14.6%). Malformations of the urinary tract were observed in 24 patients (58.5%). Other causes are divided into hereditary kidney disease in 8 patients (19.5%) predominated by primary hyperoxaluria, in vascular nephropathy who were objectified in 5 patients (12%) whereas glomerulopathy were represented in 3 cases (7.5%). No etiology was found in 1 patient. Over the 6 month’s 22 patients (54%) had renal replacement therapy (RRT). Peritoneal dialysis (PD) was practiced in over then 90% of patients. A passage from peritoneal dialysis to hemodialysis was done in 8 patients. Only four patients had a kidney transplant. The rate of overall mortality in our series was 40% with median of follow-up in 54 months. Conclusion: In Tunisia and in all Low source country children with CRF must be treated by pediatric nephrologists and the pediatric renal transplantation must be developed.

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.