Kidney Disease In Childhood Research Articles

Percutaneous Ultrasound-Guided Renal Biopsy in Greek Children: 15 Years of Experience at a Single Center.

Percutaneous ultrasound-guided renal biopsy (PRB) is a key element for diagnosis and management of several renal pathologies. We aimed to lay out the experience of our pediatric nephrology unit performing PRBs. The rationale and findings of these biopsies, safety issues and considerations of the extracted data are going to be analyzed. A retrospective study was conducted from 2008 to 2023 based on the review of the medical records of pediatric patients who underwent PRBs. In total, 216 kidney biopsies in 206 patients were performed: 115 (53.2%) during the 2008-2015 period and 101 (46.8%) during the 2016-2023 period. The most frequent clinical indication for PRBs was nephritic syndrome followed by nephrotic syndrome, observed in 84 (40.8%) and 72 (34.9%) patients, respectively. The predominant diagnosis was minimal change disease (MCD) (23.3%), followed by focal segmental glomerulosclerosis (FSGS) (15%) equal to lupus nephritis (LN) (15%), and immunoglobulin A nepropathy (10.2%). Minor complications, such as subcapsular hematomas were observed in approximately 15% of patients while no therapeutic intervention was needed. This report is the first review of pathohistological data covering a pediatric population over a 15-year period in Greece and one of the largest in southeastern Europe, especially in the Balkan region. The main indication for a PRB was nephritic syndrome; however, MCD was the main histological diagnosis. This study emphasis the fact that PRBs constitute a safe and reliable method of diagnostic approach to kidney diseases in childhood and offers important information on therapeutic approaches as well as the prognosis of these patients.

Novel mutation in XPNPEP3 in a patient with heart failure without nephronophthisis-like nephropathy (NPHPL1): case report and literature review

Background X-prolyl aminopeptidase 3(XPNPEP3) mutations are known to cause nephronophthisis-like nephropathy-1 (NPHPL1), a rare autosomal-recessive kidney disease characterized by progressive kidney failure and cystic kidney disease in childhood. The full phenotypic spectrum associated with mutations in XPNPEP3 is not fully elucidated.Case presentationA 13-year-old Chinese female patient with intellectual disability presented with a 2-year history of convulsions and fatigue, with a recent episode of swelling, breathlessness, and nocturnal dyspnea lasting 10 days. The patient was diagnosed with heart failure and kidney failure. Whole exome sequencing revealed a homozygous c.970–2 A > G mutation in XPNPEP3 associated with severe cardiac dysfunction and neurological symptoms, including epilepsy and intellectual disability. Notably, kidney ultrasound did not reveal the typical changes of NPHPL1, and kidney failure was hypothesized to be secondary to cardiac dysfunction rather than primary kidney pathology.ConclusionsThis case suggests the possible association of additional phenotypic features associated with XPNPEP3 mutations, emphasizing the need for further investigation into the heterogeneous clinical presentations associated with XPNPEP3 mutations. The findings highlight the importance of considering alternative phenotypes in patients with genetic mutations traditionally associated with specific diseases. Segregation and functional analyses are necessary to determine causality between the c.970–2 A > G XPNPEP3 mutation and disease.

Distinguishing Features of Childhood Renal Dysplasia

Abstract Background Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. Methods All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK). Results A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5–240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2–173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5–208) months, including 12 renal transplant patients. Conclusions As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

Pattern of venous thromboembolism in childhood idiopathic nephrotic syndrome

ABSTRACT Introduction Nephrotic syndrome (NS) is one of the most common childhood kidney diseases. Thromboembolic (TE) events are among the most serious complications of NS in children, with a reported prevalence of 1.8–5%. Venous thromboembolism (VTE) is the predominant type. Most cases of TE are clinically symptomatic, but some may be subclinical, thus, their true incidence may be underestimated. Studies on TE in childhood onset NS are few and varied, this prompted us to conduct the present study. Objective To study the pattern of thromboembolism in childhood INS. Patients and Methods This descriptive retrospective study included 225 pediatric patients with idiopathic NS (11 with TE events and 214 with no TE events) regularly following up in Alexandria University Children’s Hospital, Egypt from 2013 to 2023. Diagnosis of thromboembolism was based on clinical diagnosis and appropriate diagnostic imaging. Results The frequency of TE among our cohort was 4.9%.The median age of onset of the first TE event was 5.5 years. Most of the TE events (63.6%) occurred during the 1st 6 months of NS diagnosis. The total number of TE events was 15 events, and the most common site was cerebral sino-venous thrombosis (CSVT). The triglyceride levels were significantly higher in the TE event group. The median urinary protein/creatinine ratio was slightly higher in the TE event group. In the TE event group, 4 patients (36.4%) were steroid-resistant and only one of the 11 patients died. Conclusion In conclusion, TE events are not uncommon among INS children and most of the events occurred early during the disease. There was no significant relationship between the degree of proteinuria, serum albumin level, and the occurrence of TE events, while the level of triglycerides was significantly higher in patients who developed TE events.

Expression of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) Candidate Genes EDA2R, PCDH9, and TRAF7 in Normal Human Kidney Development and CAKUT.

Approximately half of the cases of chronic kidney disease (CKD) in childhood are caused by congenital anomalies of the kidney and urinary tract (CAKUT). Specific genes were identified as having significant importance in regard to the underlying genetic factors responsible for the CAKUT phenotype, and in our research, we focused on analyzing and comparing the expression levels of ectodysplasin A2 receptor (EDA2R), protocadherin9 (PCDH9), and TNF receptor-associated factor 7 (TRAF7) proteins in the cortex and medulla of healthy control kidneys during developmental phases 2, 3, and 4. We also performed an analysis of the area percentages of the mentioned proteins in the cortical and medullary sections of healthy embryonic and fetal kidneys compared to those affected by CAKUT, including duplex kidneys (DK), horseshoe kidneys (HK), hypoplastic kidneys (HYP), and dysplastic kidneys (DYS). We found that the CAKUT candidate gene proteins EDA2R, PCDH9, and TRAF7 are all expressed during normal human kidney development stages. In DYS, the expression of EDA2R was higher than in normal kidneys, likely due to EDA2R's role in apoptosis, which was upregulated in specific cases and could possibly contribute to the formation of DYS. The expression of PCDH9 was lower in HK, which can be attributed to the possible role of PCDH9 in cell migration suppression. Decreased PCDH9 expression is linked to increased cell migration, potentially contributing to the development of HK. The level of TRAF7 expression was reduced in all examined kidney disorders compared to normal kidneys, suggesting that this reduction might be attributed to the crucial role of TRAF7 in the formation of endothelium and ciliogenesis, both of which are essential for normal kidney development. Further research is required to ascertain the function of these proteins in both the typical development of the kidney and in CAKUT.

#2412 Monoallelic NEK8-related polycystic kidney disease in an Irish ADPKD cohort

Abstract Background and Aims Autosomal dominant polycystic kidney disease (ADPKD), commonly caused by pathogenic variants in PKD1 and PKD2 genes, leads to enlarged cystic kidneys and typically present in adulthood where ∼70% of affected patients progress to kidney failure by their 7th decade of life. Recently, new genes have been reported that appear to explain some of the previously unresolved ADPKD-like cases. An example is the NEK8 gene, where monoallelic variants are reported to cause early-onset ADPKD with progressive phenotype. We are reporting 4 Irish families with monoallelic NEK8 variants. Method In-depth clinical and radiological evaluations were conducted on 250 families with polycystic kidney disease referred to the Irish Kidney Gene Project. All patients underwent genomic testing using gene-panel and exome Next Generation Sequencing (NGS), while multiple ligation probe amplification analysis of PKD1 or PKD2 was utilized in genetically unresolved families. The American College of Medical Genetics and Genomics guidelines were applied to assess variant pathogenicity. Results To date, 471 individuals representing 250 distinct families (F) have been studied, including 105 (42%) families with two or more individuals (326 individuals). 55.2% have reached end-stage kidney failure, with an average age of 49.1 ± 14.3 years. We identified pathogenic / likely pathogenic variants in 76% (190/250) of families, with variants in PKD1 accounting for 76.8% (146/190) and PKD2 accounting for 15.3% (29/190) of families, respectively. In familial cases with non-PKD1/PKD2 variants, we identified pathogenic variants in a small proportion of families with ADPKD-like phenotypes: IFT140 variants (n = 4), ALG5 (n = 2), 17q12 microdeletion (n = 2) and DNAJB11, ALG8, and ALG9 variants (n = 1 each). Upon thorough review of all genetically unresolved PKD families, 4 (6.7%) families were identified with monoallelic protein-kinase domain NEK8 variants [NM_178170.3]: p.R45W (in 2 families) and two other missense variants: p.N69D and p.R140L, none of which contained a second NEK8 variant despite extensive analysis. Autosomal dominant segregation was confirmed in two parent-child pairs, whereas the remaining two index patients reported no family history of kidney disease. The median age at the initial presentation was 12 years (IQR: 2.5–27.3), with a median follow-up period of 22.5 years at the last evaluation. The phenotype of all patients was consistent with polycystic kidney disease with the absence of liver and pancreatic cysts. All patients were diagnosed with hypertension at an average age of 15.3 ± 12.2 years. Cerebral aneurysms were detected in two patients (F1: at age 61 years and F4: at age 54 years), while F3 was reported to have childhood-onset frontal bossing, prominence cerebrospinal fluid, and large arachnoid cysts. Approximately 67% [4/6] patients progressed to end-stage kidney failure by the 3rd decade of life. Compared to families with PKD1/PKD2 variants, families with NEK8 variants were characterized by earlier average age at initial presentation (14.5 versus 29.9 years; p 0.01) and progression to end-stage kidney failure (15.5 versus 49.6 years; p < 0.0001). Table 1 summarizes the clinical and genetic characteristics of the reported families. Conclusion We provide further evidence that monoallelic NEK8 variants cause early presentation and progressive ADPKD-like phenotype. Sequencing for monoallelic NEK8 variants in patients with childhood and early-adulthood cystic kidney disease can inform renal prognosis, treatment planning, familial screening and reproductive choices.

Insights into Nephrotic Syndrome in Pediarics: Case Series Analysis

Nephrotic syndrome (NS) is one of the most common childhood kidney diseases. NS can affect children of any age from infancy to adolescence and predominantly occurs in the age group of 1–6 years. It is an illness caused by idiopathic diseases like minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS), membrane proliferative Glomerulonephritis, membranous Glomerulonephritis and is characterized by increased permeability across the glomerular filtration barrier. NS is classified as primary, secondary, and congenital. It consists of four clinical features like severe proteinuria, edema, hyperlipidaemia, hypoalbuminemia. The main cause of nephrotic syndrome are diseases associated with drugs and rarely genetic disorders. The pathogenesis of the disease affects various biological functions linked to loss of proteins negatively, which results in systemic complications which may be disease and drug-associated complications. Using drugs to treat NS leads to several complications which include improper growth, metabolism, behavior change inpatient. Itis a chronic relapsing disease for most of the steroid response drugs for treatment of nephrotic syndrome. Glucocorticoids, immunosuppressants and biological agents are used for the treatment of the diseases. In this case series, we presented a case series of nephrotic syndrome in pediatrics. Total 4 patients were taken into considerations. The patients came with a chief complaints of swelling of face, foamy urine, abdominal pain, dark coloured urine with decreased urine output, joint pains. The laboratory investigations for all the 4 patients showed that there were decrease in albumin levels (hypoalbuminemia), decrease in serum creatinine levels and protein in urine, although kidney biopsy couldn’t be perform but the diagnosis was made based on clinical and laboratory findings. The patients were mainly treated with prednisolone, pantoprazole, enalapril, nifedipine, amoxiclav, and even albumin transfusion were done. NS was successfully managed and fortunately the patients recovered.

Key stakeholders' perspectives on the development of an early dietary phosphate self-management strategy for children and young people with chronic kidney disease stages 1-3: A modified Delphi consensus process.

An early dietary phosphate intervention (EPI) can provide vital medical benefits supporting self-management of chronic kidney disease (CKD) in childhood. To utilise expert consensus to provide early modelling for an EPI to guide clinical practice across a paediatric renal network. Forty-eight statements across six domains were constructed following a systematic review and semi-structured interviews with children and young people (CYP), parents and healthcare professionals (HCP). A three-round online Delphi survey with parents and paediatric renal multi-disciplinary healthcare experts was undertaken. Twenty-one experts agreed on 56 statements over three Delphi rounds. Statements were accepted in all six domains: definition of an EPI (2), rationale (12), intended users (3), delivery (14) (when [1], where [3], who [2], how [8]), other considerations (16) and potential concerns (9). Consensus was reached on a definition and a set of guiding principles, providing some early modelling for implementation and future research on the development of an EPI strategy for CYP with CKD.

Gestational Exposure to Maternal Systemic Glucocorticoids and Childhood Risk of CKD

Background & ObjectivesThe potential effects of antenatal glucocorticoid exposure on the health of children are unclear. We examined the association of gestational exposure to maternal systemic glucocorticoids (SG) and the risk of developing chronic kidney disease (CKD) in childhood. Study designRetrospective cohort study. Setting & ParticipantsNewborns cared for at the largest healthcare delivery system in Taiwan between 2004 and 2018. ExposureMaternal prescriptions for SG between the last menstrual period and birth as a proxy for gestational exposure. OutcomeIncidence of childhood CKD, including congenital anomalies of the kidney and urinary tract (CAKUT) and other kidney diseases (non-CAKUT) over 10 years. Analytical ApproachCox proportional hazards models with stabilized inverse probability of treatment weighting and robust sandwich estimator were used to estimate the average association between SG and incident CKD after adjustment for offspring characteristics (aHR). ResultsAmong 23,363 singleton-born children, gestational SG exposure was significantly associated with a higher risk of childhood CKD (aHR, 1.69 [95% CI, 1.01–2.84]). Stratified analyses showed stronger associations between SG and childhood CKD within the strata of birth <37 weeks gestational age (aHR, 2.38 [95% CI, 1.19–4.78]), male sex (aHR, 1.89 [95% CI, 1.00–3.55]), gestational exposure in the second trimester (aHR, 6.70 [95% CI, 2.17–20.64]), and total dose >24 mg hydrocortisone equivalent (aHR, 1.91 [95% CI, 1.05–3.47). LimitationsStudy was limited to the Taiwan healthcare delivery system and childhood CKD events through age 10 years. ConclusionsThe findings of this study suggest that gestational exposure to systemic glucocorticoids is associated with the occurrence of kidney disease in childhood. If these findings are confirmed, they may inform clinicians who are considering prescribing SG during pregnancy.

Sex differences in fetal kidney reprogramming: the case in the renin-angiotensin system.

During the early stages of the development of the living multiorgan systems, genome modifications other than sequence variation occur that guide cell differentiation and organogenesis. These modifications are known to operate as a fetal programming code during this period, and recent research indicates that there are some tissue-specific codes in organogenesis whose effects may persist after birth until adulthood. Consequently, the events that disrupt the pre-established epigenetic pattern could induce shifts in organ physiology, with implications on health from birth or later in adult life. Chronic kidney disease (CKD) is one of the main causes of mortality worldwide; its etiology is multifactorial, but diabetes, obesity, and hypertension are the main causes of CKD in adults, although there are other risk factors that are mainly associated with an individual's lifestyle. Recent studies suggest that fetal reprogramming in the developing kidney could be implicated in the susceptibility to kidney disease in both childhood and adulthood. Some epigenetic modifications, such as genome methylation status, dysregulation of miRNA, and histone coding alterations in genes related to the regulation of the renin-angiotensin axis, a common denominator in CKD, may have originated during fetal development. This review focuses on epigenetic changes during nephrogenesis and their repercussions on kidney health and disease. In addition, the focus is on the influence of environmental factors during pregnancy, such as maternal metabolic diseases and dietary and metabolic conditions, as well as some sex differences in fetal kidney reprogramming during which dysregulation of the renin-angiotensin system is involved.

ASSESSMENT OF INFLUENZA VACCINATION STATUS IN CHILDREN WITH NEPHROTIC SYNDROME

Background: Nephrotic syndrome (NS) is a common kidney disease in childhood. Children with this condition are at higher risk of developing vaccine-preventable diseases. Prednisolone remains the mainstay for the treatment of NS. Giving influenza vaccination to NS children will significantly lower the risk of NS relapse during the post-vaccination period. Aims and Objectives: To assess the influenza vaccination status in children with nephrotic syndrome. Materials and Methods: Parents/guardians of 69 pediatric patients (&lt;18 years old) diagnosed with NS from February 2022 to January 2023 were surveyed to evaluate influenza immunization status and parental knowledge regarding flu vaccination recommendations. Results:Among 69 patients included in the study, 7 children (10.1%) were vaccinated with inactivated influenza vaccine. Among the 62 parents of unvaccinated children, 21 (33.9%) had concerns about the safety of influenza vaccination, 18 (29%) had concerns about the cost of such vaccine, 17 (27.4%) were not aware of the proper recommendations and 6 (9.7%) had been told by their neighbors and relatives not to vaccinate with such vaccine. Conclusions:Physicians must be aware that proper information oninfluenza vaccination recommendations must be provided to family members along with the safety and efficacy profile of the vaccine for successful influenza vaccination in childhood nephrotic syndrome.

A Rare Cause of Hypergonadotropic Hypogonadism: Transaldolase Deficiency in Two Siblings.

Transaldolase deficiency is a rare inborn autosomal recessive disorder caused by biallelic mutations in the TALDO1 gene. It is characterized by intrauterine growth restriction, dysmorphism, abnormal skin, cytopenia, hepatosplenomegaly, liver cirrhosis, endocrine problems, renal and cardiac abnormalities. We present two siblings of Turkish origin with early-onset form of transaldolase deficiency and hypergonadotropic hypogonadism in both sexes. The girl (index) was followed-up with cryptogenic cirrhosis, leukopenia and thrombocytopenia, skin abnormalities, congenital heart defects, hypercalciuria, nephrolithiasis, proteinuria, chronic kidney disease throughout childhood. She developed hypergonadotropic hypogonadism in adolescence period. Whole exome sequencing due to the multisystemic involvement revealed a previously described homozygous inframe deletion in TALDO1 gene. Her brother was born as a small for gestational age baby and was also followed-up with cryptogenic cirrhosis since his infancy, together with cytopenia, congenital heart defects, bilateral cryptorchidism, short stature, hypercalciuria, proteinuria and chronic kidney disease in childhood. He presented with testicular microlithiasis and hypergonadotropic hypogonadism in adolescence. Sanger sequencing of TALDO1 gene confirmed the presence of the same homozygous deletion with his sister. The mother was found to be a heterozygous carrier for this deletion. We describe two patients with multisystemic involvement since neonatal period who presented with an additional hypergonadotropic hypogonadism in adolescence. The diagnosis of transaldolase deficiency should be kept in mind for these patients, and they must be evaluated for gonadal functions especially during puberty.

Study on correlation of urine specific gravity with other laboratory parameters of urine in children with Nephrotic syndrome

Nephrotic syndrome is a frequent childhood kidney disease in which proteinuria is a significant characteristic. Proteinuria can be a benign condition as well as indicates severe underlying renal or systemic diseases. The variation in the prevalence of proteinuria may occur based on the definition used or the evaluation time of the test. Proteinuria is present in up to 10% of routine urine testing in school-aged children, although this decreases to 0.1% with repeated testing. The study aimed to analyze the correlation of urinary specific gravity with other laboratory parameters of urine in children with nephrotic syndrome. Methods: This cross-sectional study was conducted in the Department of Pediatric Nephrology (NIKDU), Dhaka from October 2019 to June 2021, and a total of 153 patients with nephrotic syndrome were enrolled after taking written consent. Urine samples were collected for both 24 hours and spot for estimation of urinary protein, urinary creatinine, dipstick protein, and specific gravity by dipstick method. Spot urinary Protein Creatinine Ratio (PCR) and 24 hours urinary protein were also estimated. Results: Among 153 study subjects maximum (44.4%) were 2-5 years old followed by 58 (37.9%) who were 5-10 years and 27 (17.6%) were &gt;10 years old. Boys (61.4%) were more predominant than girls (38.6%). Mean 24 hours urinary volume was 1097.78±662.63 ml, 24 hours urinary creatinine was 32.17±23.70 mg/dl, 24 hours urinary total protein was 851.42±123.68 mg/dl, 24 hours urinary total protein (UTP) was 7.52±7.65 gm/m2/24 hours urinary specific gravity 1.020±0.02, spot urinary protein 989.49±1136.73 mg/dl, spot urinary creatinine was46.09±43.73 mg/dl, spot urinary PCR was 21.87±18.24 mg/mg and spot urinary specific gravity was 1.020±0.01. The Scatter diagram showed a significant positive correlation of 24 hours urinary specific gravity with 24 hours urinary protein (r=0.169, p=0.037). There was a significant positive correlation of 24 hours specific gravity with 24 hours urinary creatinine (r=0.380, p=&lt;0.001). There was a significant positive correlation of spot urinary protein with spot urinary specific gravity as well (r=0.206, p=0.011). Spot urinary specific gravity showed a significant positive correlation with spot urinary creatinine (r=0.355, p=&lt;0.001). There was a significant negative correlation of spot urinary specific gravity with spot urinary PCR (r= -305, p=&lt;0.001). Conclusion: It can be concluded that urinary specific gravity had a substantial correlation with other laboratory parameters of urine and can be used as an important diagnostic tool for diagnosing proteinuria in children with nephrotic syndrome

Feasibility and the Utility of Therapeutic Play in Children with Nephrotic Syndrome in a Hospital Setting in India: An Exploratory Study

Background Nephrotic Syndrome NS is one of the most common kidney diseases in childhood with physical and psychosocial consequences. Objectives To assess the feasibility of therapeutic play and its benefits in children with nephrotic syndrome.Method This hospital-based psychological intervention was carried out in a hospital setting in South India. The sample consisted of five children between 3 and 9 years diagnosed with NS for at least six months. They were recruited from the Department of Pediatric Nephrology. Play sessions ranged from 2-6 depending on the childrenrsquos hospital stay. Feedback was taken from each parent at the end of the session. All play sessions were videotaped and transcribed. The data obtained were analyzed qualitatively and common play themes were identified.Results Children played spontaneously and expressed immense interest while playing. They appeared cheerful and curious. No signs of sadness worry or fear were seen. Play served as a distraction in times of physical distress.Conclusion Play is essential to childrenrsquos lives and can be utilized in pediatric care settings to improve quality of life and outcomes.

Burden of Paediatric Kidney Diseases in a Tertiary Care Hospital in Harare, Zimbabwe.

The pattern of paediatric kidney diseases across different regions is influenced by genetic, racial, and environmental differences. The aim of this study was to review the current spectrum and outcome of childhood kidney diseases at Parirenyatwa Group of Hospitals and highlight the challenges of care. Retrospective observational study. Data on all children below 16 years of age hospitalised for any kidney disease over an 8-month period (1 January-31 August 2022) were retrieved and retrospectively analysed. Kidney diseases were categorised as per standard definitions. Kidney disease accounted for 2.2% (n = 50) of all 2264 admissions in the paediatric unit, with males constituting 60% (n = 30). Age ranged from 2 weeks to 13 years (mean 5.5 ± 3.5 years) with 58.0% being under 5 years. The commonest diagnoses in the unit were acute kidney injury (AKI) (n = 16, 32%) nephrotic syndrome (n = 16, 32%), hypertension (n = 12, 24%) and end stage kidney disease (ESKD) (n = 11, 22%) with some children presenting with more than 1 diagnosis. Only 3 out of 11 children with ESKD and 3 out of 8 children with AKI who required dialysis could be offered dialysis due to limited resources. Overall mortality rate was 32% (16/50): 5 children with AKI, 2 with nephrotic syndrome and normal kidney function, 8 with ESKD and 1 with Fanconi syndrome. Childhood kidney disease contributes significantly to hospitalisations at our institution with highest mortality among children with ESKD. The study highlighted the need for provision of essential drugs and kidney replacement therapy for children with kidney disease at our institution.

Psychosocial Functioning and Health-Related Quality of Life in Children with Nephrotic Syndrome: Preliminary Findings

Background: Nephrotic syndrome (NS) is one of the most commonly diagnosed kidney diseases in childhood, with a high relapse rate. Aim: This study was designed to assess emotional and behavioural problems, temperament, family environment, and evaluate the health-related quality of life in children with NS. Methods: This cross-sectional study was conducted at a tertiary care hospital in South India. A purposive sampling technique was used. A sample of 32 children between ages 6 and 12 years with NS—steroid-sensitive (infrequent relapsers, frequent relapsers, or those with steroid-dependent) and steroid-resistant—for more than 6 months duration and managed by the Pediatric Nephrology department were recruited. The control group of 30 healthy children was matched for age and gender. The assessment was conducted using the Strengths and Difficulties Questionnaire (SDQ), Pediatric Quality of Life Inventory™ (PedsQL 4.0), Malhotra Temperament Schedule, and Family Environment Scale. Data obtained was examined using descriptive and inferential statistics. Results: On the SDQ, 34.4% of children with NS indicated a total score within the clinical range. Children with NS had an overall lower score on the QOL scale ( P &lt; .003). It was found that the SRNS subtype had poor scores on SDQ ( P &lt; .023) and QOL ( P &lt; .017). The temperament of children with NS did not differ from those of healthy children. The families with NS scored lower on organization ( P &lt; .042) and control ( P &lt; .006) subscales. Conclusion: Children with NS have a risk of emotional and behavioural problems. It appears that children with NS have a poor quality of life. Families of children with NS were less organized and low in control. Findings indicate the need for routine psychological evaluation and intervention as they will impact the course and outcome of NS.

EP27.15: Findings and evolution of a patient diagnosed with autosomal recessive polycystic kidney disease in childhood during prenatal period

EP27.15: Findings and evolution of a patient diagnosed with autosomal recessive polycystic kidney disease in childhood during prenatal period

Kidney biopsy diagnosis in childhood in the Norwegian Kidney Biopsy Registry and the long-term risk of kidney replacement therapy: a 25-year follow-up

BackgroundThere is scarce information on biopsy-verified kidney disease in childhood and its progression to chronic kidney disease stage 5 (CKD 5). This study aims to review biopsy findings in children, and to investigate risk of kidney replacement therapy (KRT).MethodsWe conducted a retrospective long-term follow-up study of children included in the Norwegian Kidney Biopsy Registry (NKBR) and in the Norwegian Renal Registry (NRR) from 1988 to 2021.ResultsIn total, 575 children with a median (interquartile range, IQR) age of 10.7 (6.1 to 14.1) years were included, and median follow-up time (IQR) after kidney biopsy was 14.3 (range 8.9 to 21.6) years. The most common biopsy diagnoses were minimal change disease (MCD; n = 92), IgA vasculitis nephritis (IgAVN; n = 76), IgA nephropathy (n = 63), and focal and segmental glomerulosclerosis (FSGS; n = 47). In total, 118 (20.5%) of the biopsied children reached CKD 5, median (IQR) time to KRT 2.3 years (7 months to 8.4 years). Most frequently, nephronophthisis (NPHP; n = 16), FSGS (n = 30), IgA nephropathy (n = 9), and membranoproliferative glomerulonephritis (MPGN; n = 9) led to KRT.ConclusionsThe risk of KRT after a kidney biopsy diagnosis is highly dependent on the diagnosis. None of the children with MCD commenced KRT, while 63.8% with FSGS and 100% with NPHP reached KRT. Combining data from kidney biopsy registries with registries on KRT allows for detailed information concerning the risk for later CKD 5 after biopsy-verified kidney disease in childhood.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information

Disease-Associated Systemic Complications in Childhood Nephrotic Syndrome: A Systematic Review.

IntroductionNephrotic syndrome (NS) is one of the most common childhood kidney diseases. During the active phase, the disease pathogenesis affects various biological functions linked to loss of proteins negatively, which can result in systemic complications. Complications of childhood NS are divided into two categories: disease-associated complications and drug-associated complications. However, complications in pediatric patients with NS, especially disease-associated complications are still limited. Although reported in the literature, information is not comprehensive and needs to be updated. This study aimed to systematically assess systemic complications in children with NS, especially disease-associated complications, to better understand how they impact outcomes.MethodsWe conducted a systematic search of several databases: BioMed Central Pediatrics, PubMed, Google Scholar, the National Library of Medicine, Cochrane Library, CINAHL/EBSCO, British Medical Journal, Science Direct, Scopus, and Elsevier’s ClinicalKey. We followed the PRISMA guidelines to plan, conduct, and report this review. We used the Joanna Briggs Institute’s critical appraisal tools for assuring the quality of the journal articles that were chosen.ResultsEleven articles concerning complications in childhood NS were analyzed. Systemic disease-associated complications in covered were cardiovascular complications, infections, thyroid-hormone complication, kidney complications, and oral health complications.ConclusionNS is marked by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia, which can result in systemic disease-associated complications. Cardiovascular complications, infections, thyroid-hormone complications, kidney complications, and oral health complications are the main systemic complications in childhood NS. It is essential that health-care providers prevent these complications for proper maintenance of patients’ health.

Pediatric Patients Undergoing a Fontan Operation or with a High RACHS-1 Score Require Monitoring for Chronic Kidney Disease in Early Childhood.

Although the number of pediatric patients with long-term survival following cardiac surgery is increasing, concerns regarding chronic kidney disease (CKD) after surgery are growing. We examined the frequency of and risk factors for pediatric CKD development in patients with congenital heart disease (CHD) at least 2years after cardiac surgery. This was a cross-sectional study of 147 patients who underwent open-heart surgery for CHD at Kagoshima University Hospital from April 2010 to March 2017. Data on demographics, acute kidney injury after cardiac surgery, cyanotic heart disease, Fontan circulation, medications in the perioperative period, and Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) category were recorded. CKD was defined using the current classification system described in the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative and assessed during early childhood within 2-3years of cardiac surgery. Statistical analyses were performed using SPSS Statistics for Windows version 25.0. We consecutively enrolled 147 patients, of whom 22 (15.0%) had CKD, all with stage-2 severity. Among patients with CKD, a higher proportion underwent Fontan surgery (P < 0.001), a higher proportion had cyanotic heart disease (P = 0.009), and the RACHS-1 category was high (P = 0.003). Patients with CKD appeared more frequently than patients without CKD in RACHS-1 categories 3, 5, and 6. It is essential to evaluate renal function longitudinally and monitor for CKD, given that patients who underwent Fontan surgery or complicated surgery in infancy have a high rate of developing postoperative CKD in early childhood.