Tertiary Pediatric Nephrology Center Research Articles

Urinary Tract Infection and Antimicrobial Resistance Patterns: 5-Year Experience in a Tertiary Pediatric Nephrology Center in the Southwestern Region of Poland.

(1) Background: Urinary tract infections (UTIs) are among the most common infections in the pediatric population. This study aimed to analyze the urine culture results and antimicrobial patterns over the last 5 years in children diagnosed with UTI. (2) Methods: Retrospective analysis of medical records of 242 patients hospitalized in the Pediatric Nephrology Department diagnosed with a UTI in the years 2018-2022. (3) Results: The most common causative agent was E. coli, responsible for 64% of UTIs, followed by Klebsiella spp. (16%), Pseudomonas spp. (6%), Enterobacter spp. (4%), Proteus spp. (4%), and Enterococcus spp. (3%). Non-E. coli UTIs were significantly more frequently observed in patients with congenital anomalies of the kidney and urinary tract or neurogenic bladder and patients receiving antibiotic prophylaxis. For the whole study period, 32% of E. coli were resistant to amoxicillin/clavulanic acid, 23.3% to trimethoprim/sulfamethoxazole, 12.2% to ciprofloxacin, and 4.4% to nitrofurantoin. During 2018-2022, the prevalence of E. coli resistant to amoxicillin/clavulanic acid varied from 16.7% to 41.2%, and resistance to cefuroxime increased four times (from 4% in 2018 to 16.7% in 2022). Starting in 2021, all isolated E. coli strains were classified as susceptible-increased exposure or resistant to cefuroxime. (4) Conclusion: Managing pediatric UTIs remains challenging in clinical practice. The choice of optimal empiric treatment should be considered following local recommendations and individual risk factors assessment and require careful dosage adjustment. Observed changes in antimicrobial resistance indicated the need for frequent updating of local recommendations for the management of pediatric patients with UTIs.

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria.

Paroxysmal cold hemoglobinuria (PCH) is a rare condition in childhood characterized by complement-mediated premature destruction of red blood cells. PCH is associated with intravascular hemolysis causing hemoglobinuria, which may result in acute kidney injury of various severity. We aimed to retrospectively analyze clinical and laboratory features of children with PCH-associated acute kidney injury received at tertiary Pediatric Hematology and Nephrology Center, University Motol Hospital, Prague, Czech Republic during the period 2016 to 2022. We present here 3 children with PCH-associated acute kidney failure requiring renal replacement therapy. We highlight the association of PCH with kidney disease. Renal parameters and urine examination should be regularly tested in all children with PCH.

Yield of diagnostic testing in evaluating etiology and end organ effects of pediatric hypertension.

Current recommendations regarding the utility of diagnostic investigations for pediatric hypertension are based on limited evidence, leading to wide practice variation. The objective of this study was to characterize the cohort of children that may benefit from secondary hypertension workup, and determine the diagnostic yield of investigations. This was a single-center, retrospective cohort study of 169 children aged 1-18years referred between 2000 and 2015, to a tertiary pediatric nephrology center in Canada, for evaluation of hypertension. The number of investigations completed, abnormal findings, and diagnostic findings that helped establish hypertension etiology was determined. 56 children were diagnosed with primary and 72 children with secondary hypertension in the outpatient setting. Secondary hypertension was predominant at all ages except for obese adolescents ≥ 12years. Half of children with traditional risk factors for primary hypertension, including obesity, were diagnosed with secondary hypertension. Kidney ultrasound had the highest yield of diagnostic results (19.8%), with no difference in yield between age groups (P = 0.19). Lipid profile had a high yield of abnormal results (25.4%) as part of cardiovascular risk assessment but was only abnormal in overweight/obese children. Echocardiogram had a high yield for identification of target-organ effects in hypertensive children (33.3%). A simplified secondary hypertension workup should be considered for all hypertensive children and adolescents. High yield investigations include a kidney ultrasound, lipid profile for overweight/obese children, and echocardiograms for assessment of target-organ damage. Further testing could be considered based on results of initial investigations for the most cost-effective management. A higher resolution version of the Graphical abstract is available as Supplementary information.

Schimke immunoosseous dysplasia: an ultra-rare disease. a 20-year case series from the tertiary hospital in the Czech Republic

BackgroundSchimke immunoosseous dysplasia (SIOD) is an ultra-rare inherited disease affecting many organ systems. Spondyloepiphyseal dysplasia, T-cell immunodeficiency and steroid resistant nephrotic syndrome are the main symptoms of this disease.Case presentationWe aimed to characterize the clinical, pathological and genetic features of SIOD patients received at tertiary Pediatric Nephrology Center, University Hospital Motol, Prague, Czech Republic during the period 2001–2021. The mean age at diagnosis was 21 months (range 18–48 months). All patients presented with growth failure, nephropathy and immunodeficiency. Infections and neurologic complications were present in most of the affected children during the course of the disease.ConclusionsAlthough SIOD is a disease characterized by specific features, the individual phenotype may differ. Neurologic signs can severely affect the quality of life; the view on the management of SIOD is not uniform. Currently, new therapeutic methods are required.

Improving acute peritoneal dialysis outcome with use of soft peritoneal dialysis catheter (Cook Mac-Loc Multipurpose Drainage catheter®) among infants < 1500g in a low resource setting.

Despite its utility, uncertainty exists on the feasibility of acute peritoneal dialysis (PD) and optimal PD catheter type for very low birth weight (VLBW < 1500g) and extremely low birth weight (ELBW < 1000g) infants. We hereby report our experience of acute PD among these high-risk infants and compare the outcome between stylet-based rigid catheter (SRC) and Cook Mac-Loc Multipurpose Drainage catheters® (CMMDC). Case notes of infants < 1500g undergoing PD between 2012 and 2021 in a network of five participating neonatal units supported by a tertiary paediatric nephrology centre in Kolkata, India, were retrospectively reviewed. PD was conducted either with SRC or after 2018 with CMMDC. Outcome parameters included complications, survival during PD, and survival to discharge. 24 infants (VLBW: n = 13 and ELBW: n = 11) underwent PD at median age 4.5days (IQR 3-6) with either CMMDC (n = 14) or SRC (n = 10). Significant improvement in biochemical parameters and fluid removal was seen in both ELBW and VLBW infants. CMMDC was associated with significantly fewer PD-related complications 7/14 (50%) vs. 9/10 (90%) (p = 0.04) and higher survival during PD 13/14 (93%) vs. 5/10 (50%) (p = 0.02), without significant difference in survival to hospital discharge 8/14 (57%) vs. 3/10 (30%) (p = 0.25). CMMDC also enabled longer duration of PD, higher ultrafiltration, and better control of acidosis. Consumable cost was higher for CMMDC (USD$60) than SRC (USD$14). In a low resource setting, CMMDC had lower PD complications and superior short-term survival among ELBW/VLBW infants. A higher resolution version of the Graphical abstract is available as Supplementary information.

Percutaneous ultrasound-guided renal biopsies in a paediatric population: comparison of coaxial and non-coaxial techniques using 18-gauge core biopsy needles.

Percutaneous ultrasound-guided biopsy is performed in paediatric patients for evaluation of diffuse renal parenchymal disease. When compared with the non-coaxial technique, the coaxial technique has the advantages of obtaining multiple tissue cores via a single capsular puncture and post-biopsy tract embolisation. To compare the coaxial and non-coaxial techniques of percutaneous ultrasound (US)-guided biopsy of native kidney parenchyma in children and adolescents with renal disease. We retrospectively identified consecutive patients who underwent percutaneous US-guided renal biopsy using an 18-gauge core biopsy needle from July 2019 to July 2021 in a single tertiary paediatric nephrology centre. Focal renal tumour biopsy and transplant kidney biopsy were excluded. The total glomerular yield, specimen adequacy, complication rate and procedural time between the coaxial and non-coaxial groups were compared. There were 34 percutaneous US-guided renal biopsies: 22 using a coaxial technique and 12 using a non-coaxial technique. The total median glomerular yield obtained was higher in the coaxial group (coaxial=37.9; non-coaxial=22.2; P=0.02). No statistically significant difference was noted between specimen adequacy (coaxial=100%; non-coaxial=91.7%; P=0.35). While no statistically significant difference was detected for overall complication rates (coaxial=13.6%; non-coaxial=41.7%; P=0.09), the coaxial group had a lower rate of haemorrhagic complications (coaxial=4.5%; non-coaxial=41.7%; P=0.01). One patient in the non-coaxial group had post-biopsy haemorrhage requiring embolisation. The procedural time was shorter in the coaxial group (coaxial=26.3 ± 7.0min; non-coaxial=51.3 ± 11.5min; P&lt;0.001). Percutaneous US-guided renal biopsy in children using the coaxial technique has significantly higher total glomerular yield, shorter procedural time and fewer haemorrhagic complications, compared to biopsies using the non-coaxial technique.

POS-742 HEALTH RELATED QUALITY OF LIFE (HRQOL) IN PAEDIATRIC CHRONIC KIDNEY DISEASE (CKD) AND CAREGIVER BURDEN (CB) – A PROSPECTIVE MIXED-METHODS STUDY

CKD reduces the HRQoL of affected children and is associated with burden on caregivers. Data on HRQoL and CB in CKD from high income countries may not reflect realities of patients and families in low resource settings. Despite our centre’s expertise in providing paediatric CKD care, families frequently discontinue treatment for their child, resulting in poor patient outcomes and ethical challenges to the healthcare team. There is no data regarding HRQoL and CB for paediatric CKD in India. We aimed to measure HRQoL in children with advanced CKD and assess CB in primary caregivers by quantitative scoring and qualitative analysis of novel factors pertinent to our low resource setting. A prospective study conducted at a stand-alone, low-cost, tertiary paediatric nephrology centre with a large referral base. Patients: All children (1-18 yrs) with CKD (Stage 4,5) on treatment >3 months. Caregivers: The parent/grandparent identified by family as patient’s primary medical caregiver. HRQoL Tool: validated PedsQL™ Version 4.0. Patient scores were compared with published control data mean scores [Indian (87.4 ± 11.1) and international (83.9 ± 12.5)]. PedsQL ESRD Module, Version 3.0 was used and compared with previous reported data (69.2 ± 23.2). HRQoL scores were standardised by minimum-maximum normalization (range 0 – 1). ‘Worse’ HRQoL = standardized score </= 0.5. CB Tool: validated Pediatric Renal Caregiver Burden Scale and compared with previous data (120 ± 16.4). ‘High’ CB = CB scores>our study population mean. Reflexive thematic analysis of qualitative data (in-depth audiotaped interviews) was performed to capture family-prioritized factors affecting HRQoL and CB. Patients: 40 CKD patients (26 male, median age 11 yrs, 30 on dialysis) and caregivers (38 mothers) were included. Families (65% lower middle/upper lower SES) had median income of INR 18000/month. Patient HRQoL mean scores (63.1 ± 16.7) were lower than controls and previous high income country paediatric CKD data (73.72 ± 17.42). The physical domain contributed significantly to worse Qol. ‘Worse’ HRQoL was associated with inability to attend school (65% vs 13%, p = 0.001), anaemia (57% vs 23%, p=0.031), longer duration of illness (55% vs 15%, p=0.008) and dialysis (58% vs 18%, p=0.034) but not with low SES (p>0.5). Mean scores in ESRD module (57.2 ± 13.2) were low and social life (family/peer interaction) was the worst affected domain (39.9 ± 18.2). Low ESRD module scores were associated with school absence but not with modality or duration on dialysis. Primary Caregiver burdenmean (143.9 ± 17.1) was higher than previously reported. ‘High’ CB was associated with CKD Stage 5 vs stage 4 but was similar for HD vs CAPD. Parent CB inversely correlated with child’s HRQoL (r= - 0.58, p=0.001). Qualitative analysis themes generated were “catastrophic healthcare expenditure”, “mental stress and sleep disturbance” and “extreme social isolation” especially in those doing dialysis. Identified themes and illustrative quotes are depicted in figure 1. Fig1: Thematic schema derived from analysis of in-depth caregiver interviews Children with CKD have low HRQoL and their caregivers, high CB. Factors contributing to CB were unique to our low resource settings, and not elucidated by scoring tools alone. We intend to use these findings to improve counseling and provide better social and financial support to families.

High Incidence of COL4A Genetic Variants Among a Cohort of Children With Steroid-Resistant Nephrotic Syndrome From Eastern India

High Incidence of COL4A Genetic Variants Among a Cohort of Children With Steroid-Resistant Nephrotic Syndrome From Eastern India

Genetic atypical hemolytic uremic syndrome in children: a 20-year experience from a tertiary center.

Introduction:Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, which primarily affects preschool-aged children. This study’s aim was to describe the clinical profile, management, and long-term outcome of the genetic aHUS patients admitted to a tertiary care pediatric nephrology center during 20 years.Methods:We performed a retrospective analysis of the clinical records of all aHUS patients younger than 18 years with identified genetic mutations. Data on clinical features, genetic study, therapeutic interventions, and long-term outcomes were reviewed.Results:Five cases of aHUS with an identified genetic mutation were included; all were inaugural cases with the youngest being 4 months old. Complement factor H gene mutation was identified in four patients. Therapeutic plasma exchange was performed for acute management in 4 patients, one of whom also needed acute renal replacement therapy (peritoneal dialysis). All patients went on complete remission, 2 had more than one relapse but only 1 of these progressed to chronic kidney disease during the follow-up period (median (25th-75th percentile), 136 (43.5-200.5) months).Conclusion:In children, the prognosis of renal function seems to be strongly dependent on the genetic background, thus being crucial to perform genetic study in all aHUS cases. In our cohort, 2 patients presented genetic mutations not previously described. Recent innovations on the genetic field leading to the identification of new mutations has lead to a better understanding of aHUS pathogenesis, but further studies, focusing on the genotype-phenotype correlation, with longer follow-up periods, are needed.

Low renal transplantation rates in children with end-stage kidney disease: A study of barriers in a low-resource setting.

After 2 decades as a low-cost transplant centre in India, our rates of kidney transplantation are low compared to the burden of end-stage kidney disease (ESKD). We performed this study to identify possible barriers inhibiting paediatric kidney transplant and to assess the outcomes of paediatric ESKD. A retrospective chart review of ESKD patients (2013 - 2018) at a tertiary paediatric nephrology centre was conducted. Medical/non-medical barriers to transplant were noted. Patient outcomes were classified as "continued treatment," "lost to follow-up (LTFU)" or "died." Of 155 ESKD patients (monthly income 218 USD [146, 365], 94% self-pay), only 30 (19%) were transplanted (28 living donor). Sixty-five (42%) were LTFU, 19 (12%) died, and 71 (46%) continued treatment. LTFU/death was associated with greater travel distance (300km [60, 400] vs 110km [20, 250] km, P<.0001) and lower monthly income (145 USD [101, 290] vs 290 USD [159, 681], P<.0001). Among those who continued treatment, 41 proceeded to transplant evaluation of whom 13 had no living donor and remained waitlisted for 27months (15, 30). The remainder (n=30) did not proceed to transplant due to unresolved medical issues (n=10) or a lack of parental interest in pursuing transplant (n=20). Barriers to transplantation in low-resource setting begin in ESKD. LTFU resulted in withdrawal of care and was associated with low socioeconomic status. Among those who continued treatment, transplant rates were higher but medical challenges and negative attitudes towards transplant and organ donation occurred.

Histopathological Pattern of Difficult Childhood Nephrotic Syndrome in A Tertiary Care Centre, Bangladesh

Background The aim of this study was to know the spectrum of histopathology in children who underwent a renal biopsy for difficult NS in a tertiary care pediatric nephrology center. Method This prospective observational study took place in Pediatric Nephrology department of Bangabandhu Sheikh Mujib Medical University, Dhaka Bangladesh, from January 2011 to July 2018. Patients presented with difficult pattern of nephrotic syndrome and underwent renal biopsy were enrolled in this study. Results Total 140 patients were recruited in this study. Patients with SRNS & nephrotic syndrome with atypical presentation had renal biopsy ; a good number of atypical NS were SDNS. They were grouped into Group A: SRNS, Group B: SDNS, Group C:Nephrotic Syndrome with atypical presentation. Comparison among 3 groups were done. Regarding lab parameters, serum creatinine was raised in 40.6% patients in nephrotic syndrome with atypical presentation and 16.2%in SRNS. In patients with SDNS, MCD (51.3%) was the most common histological pattern followed by MesPGN (33.3%); whereas MesPGN was the commonest histological pattern in SRNS (56.8%) and atypical presentation (54.7%) followed by MCD and FSGS.Most of the patients response to immunosuppressive therapy. In SRNS partial response achieved in 18.9% and CKD developed in 16.2% cases. In comparison, nephrotic syndrome atypical presentation 10.9% patients achieved partial response and 7.8% developed CKD but these are not statistically significant. 5.4% patients of SRNS died. Conclusion Mesangioproliferative glomerulonephritis was the most common histopathological diagnosis in patients with SRNS & nephrotic syndrome atypical presentation in our population. MCD is predominant among SDNS.

Both the rituximab dose and maintenance immunosuppression in steroid-dependent/frequently-relapsing nephrotic syndrome have important effects on outcomes

Rituximab is an effective treatment for steroid-dependent/ frequently-relapsing nephrotic syndrome (SDFRNS) in children. However, the optimal rituximab regimen remains unknown. To help determine this we conducted an international, multicenter retrospective study at 11 tertiary pediatric nephrology centers in Asia, Europe and North America of children 1-18 years of age with complicated SDFRNS receiving rituximab between 2005-2016 for 18 or more months follow-up. The effect of rituximab prescribed at three dosing levels: low (375mg/m2), medium (750mg/m2) and high (1125-1500mg/m2), with or without maintenance immunosuppression (defined as concurrent use of corticosteroids, mycophenolate motile or calcineurin inhibition at first relapse or for at least six months following the rituximab treatment) was examined. Among the 511 children (median age 11.5 year, 67% boys), 191, 208 and 112 received low, medium and high dose rituximab, respectively. Within this total cohort of 511 children, 283 (55%) received maintenance immunosuppression. Renal biopsies were performed in 317 children indicating the predominant histology was minimal change disease (74%). Without maintenance immunosuppression, low-dose rituximab had a shorter relapse-free period and a higher relapse risk (8.5 months) than medium (12.7 months; adjusted hazard ratio, 0.62) and high dose (14.3 months; adjusted hazard ratio, 0.50; all significant). With maintenance immunosuppression, the relapse-free survival in low-dose rituximab (14 months) was similar to medium (10.9 months; adjusted hazard ratio, 1.23) and high dose (12.0 months; adjusted hazard ratio, 0.92; all non-significant). Most adverse events were mild. Thus, children receiving low-dose rituximab without maintenance immunosuppression had the shortest relapse-free survival. Hence, both rituximab dose and maintenance immunosuppression have important effects on the treatment outcomes.

Efficacy and side effects of mycophenolate mofetil therapy in children with steroid dependent nephrotic syndrome in a tertiary paediatric nephrology centre in Sri Lanka

Background: Mycophenolate mofetil (MMF) has been shown to be a well-tolerated drug which could maintain prolonged remission in patients with steroid dependent nephrotic syndrome (SDNS). MMF is known to have a steroid sparing effect. Objectives: To determine the effect of MMF in reducing relapses in Sri Lankan children with SDNS and to ascertain the incidence of side effects of MMF in this population. Method: Children between 1-18 years of age with SDNS who had been treated with MMF for 12 months or more at the paediatric nephrology unit, Teaching Hospital Peradeniya, were selected and reviewed retrospectively. Children who had previously received immunosuppressive therapy other than prednisolone, cyclophosphamide, cyclosporin A, tacrolimus and levamisole and those who were on MMF for indications other than idiopathic nephrotic syndrome were excluded. Children and parents were interviewed and their patient-held health records, including their ‘nephrotic syndrome diary’ were reviewed. Data were collected twice a week using pretested questionnaires, in the nephrotic syndrome clinic for a period of 8 weeks. Results were analysed using SPSS version 23. Results:Thirty five children who met the selection criteria were included. Of them 26 (74.3%) were male and 09 (25.7%) were female. The median age was 9.7 years. Mean number of relapses in the year before starting MMF and the year after starting MMF were 3.5 and 1.8 respectively. The reduction in the relapse rate after being on MMF was significant (p=0.009). Child’s age or gender did not impact the relapse rate (p>0.05). The most common side effect observed was cough (20%) and the least common ones were abdominal pain, dyspnoea and dizziness (3%). Conclusions: In this study MMF reduced relapse rates of children with SDNS, despite some minor side effects.

Vaccination Practices in Pediatric Dialysis Patients Across Europe. A European Pediatric Dialysis Working Group and European Society for Pediatric Nephrology Dialysis Working Group Study

Background: Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. Methods: A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization. The immunization practices and center protocols for monitoring antibody titers after vaccination in dialysis patients were obtained through a questionnaire. Results: All centers included in the study recommended immunization against hepatitis B virus (HBV), diphtheria, tetanus, pertussis, Hemophilus influenzae type b (Hib), poliomyelitis, measles, mumps, rubella (MMR), and streptococcus pneumonia in dialysis patients. In 16 centers, dialysis patients were vaccinated against influenza virus annually. HBV protective antibody titers were measured in 17 centers (during dialysis period in 14 centers, during pre-renal transplantation preparations in 14 centers or in both times in 11 centers). Hepatitis A virus (HAV) was reported to be followed in 13 centers, in 8 centers during dialysis period, and in 11 centers during pre-RTx preparations. MMR and varicella-zoster virus (VZV) protective antibody titers were measured during the dialysis period or before renal transplantation (RTx) in 12 and 15 centers, respectively, and in 6 centers both titers were checked both times. Conclusion: There are variations in vaccination practice across Europe. Children with CKD, those undergoing dialysis, and transplant candidates should receive age-appropriate vaccinations before RTx as well as before the transition to adult nephrology clinics and antibody levels should be monitored to evaluate the immunization status before and after RTx.

Feasibility of sustained low efficiency dialysis in critically sick pediatric patients: A multicentric retrospective study.

Sustained low-efficiency dialysis (SLED) has emerged as a cost effective alternative to Continuous Renal Replacement Therapy in the management of hemodynamically unstable adult patients with acute kidney injury. The objective of the study was to document the SLED practices in these centers, and to look at the feasibility, and tolerability of SLED in critically sick pediatric patients. It was a retrospective record review from January 2010 to June 2016 done in four tertiary pediatric nephrology centers in India. All pediatric patients undergoing SLED in the collaborating centers were included in the study. Basic demographic data, prescription parameters and outcomes of patients were recorded. During the study period a total of 68 children received 211 sessions of SLED. PRISM score at admission in patients was 13.33 ± 9.15. Fifty-seven patients were ventilated (84%). Most of the patients had one or more organ system involved in addition to renal (n = 64; 94%). Heparin free sessions were achievable in 153 sessions (72%). Out of 211 sessions, 148 sessions were on at least one inotrope (70.1%). Overall premature terminations had to be done in 27 sessions (13% of all sessions), out of which 7 sessions had to be terminated due to circuit clotting (3.3%). Intradialytic hypotension or need for inotrope escalation was seen in 31 (15%) sessions but termination of the session for drop in BP was required in only 20 (9%) sessions. SLED is a feasible method of providing renal replacement in critically ill pediatric patients.

Spectrum of renal biopsy finding in idiopathic nephrotic syndrome in children: An 18-month retrospective analysis at a tertiary care pediatric nephrology center in North Karnataka, India

Context: Idiopathic nephrotic syndrome (NS) is the most common glomerular nephropathy in pediatrics, with great variation in patient characteristics in different regions of the world. Difficult childhood NS poses a great diagnostic and management challenge for the treating nephrologists. The histopathological features of NS have been extensively studied by various authors from different regions of the world with a wide variation in the histopathological distribution. We aimed to describe the spectrum of histopathological diagnosis in childhood NS from North Karnataka, India, where there is a high incidence of consanguinity. Aims: The aim of this study is to study the spectrum of histopathology in children who underwent a renal biopsy in our tertiary care pediatric nephrology center. Settings and Design: Retrospective observational study at a tertiary care pediatric nephrology center over 1 year from January 2013 to June 2015. Subjects and Methods: The medical records of all children diagnosed as NS were retrospectively reviewed for demographic data, clinical course, and histopathological diagnosis. Statistical Analysis Used: Descriptive statistics. Results: Twenty-five children underwent biopsy in the study period. Seventeen (68%) were male as compared to 8 (32%) females. Thirteen (52%) of biopsied patients showed minimal change NS, 6 (24%) had focal segmental glomerulosclerosis, and 3 (14%) patients showed IgA nephropathy while 1 patient had mesangioproliferative glomerulonephritis, IgM nephropathy, and Finnish type NS each. Conclusions: Minimal change nephritic syndrome is the major biopsy finding in our part of the country even in steroid-resistant NS. This finding needs to be confirmed with studies with bigger sample size.

Etiology, clinical profile and short-term outcome of acute kidney injury in children at a tertiary care pediatric nephrology center in Pakistan

Background: The reported prevalence rates and etiologies of acute kidney injury (AKI) are quite variable in different regions of the world. The current study was planned to determine the etiology, clinical profile, and short-term outcome of pediatric AKI at our hospital.Methods: A prospective, observational study was carried out from April 2014 to March 2015. All pediatric patients (1 month to ≤15 years) diagnosed as AKI using modified pRIFLE criteria were studied and followed for 3 months to document short-term outcome.Results: AKI was diagnosed in 116 children. The mean age was 7.5 ± 4.4 years and males were predominant (60.3%). At presentation, 83.6% had oliguria/anuria, 37.1% hypertension and 17.2% severe anemia. Etiology included primary renal (74/116; 63.8%), postrenal (28/116; 24.1%) and prerenal (11/116; 9.5%) causes. Postinfectious glomerulonephritis (PIGN) and crescentic glomerulonephritis in primary renal, obstructive urolithiasis in postrenal and sepsis in prerenal, were the most common etiologies. At presentation, 89/116 (76.7%) patients were in pRIFLE Failure category. Regarding outcome, 68 (58.6%) patients recovered, six (5.2%) died, 18 (15.5%) developed chronic kidney disease (CKD) and 22 (19%) end-stage renal disease (ESRD). Comparison of recovered and unrecovered AKI showed that characteristics such as hypertension, severe anemia, edema, volume overload, requirement of mechanical ventilation, initiation of dialysis and need of >5 sessions of dialysis had statistically significant (p <0.05) association with nonrecovery.Conclusion: Glomerulonephritides (PIGN and crescentic) and obstructive urolithiasis are major causes of pediatric AKI at our center. A fairly high percentage of cases recovered and these mainly comprised of PIGN and obstructive urolithiasis.

MYCOPHENOLIC ACID (MYFORTIC) IS AN ALTERNATIVE FOR PAEDIATRIC RENAL TRANSPLANT RECIPIENTS WITH GASTROINTESTINAL SYMPTOMS ON MYCOPHENOLATE MOFETIL

PurposeTo determine the prevalence of mycophenolic acid (Myfortic) use as part of the immunosuppressant regime in our current paediatric renal transplant recipients in a single tertiary paediatric nephrology centre and...

Clinical profile and outcome of renal tubular disorders in children: A single center experience.

Tubular disorders form a significant proportion of pediatric kidney diseases and are an important differential diagnosis of failure to thrive (FTT) in children. Data regarding their outcome is scarce from India. We evaluated the clinical profile of these children and studied the outcome in terms of their growth and renal failure. This is a retrospective longitudinal study of all children with renal tubular disorders attending a tertiary care pediatric nephrology center from 2005 to 2010. Growth and renal outcomes were assessed by Z scores and estimated glomerular filtration rate at diagnosis and. The common disorders encountered were distal renal tubular acidosis (d-RTA) (44%), Bartter-like (Bartter's and Gitelman) syndromes (22%) followed by hereditary Fanconi syndrome (cystinosis and idiopathic Fanconi syndrome) (13%) and few cases of nephrogenic diabetes insipidus, hypophosphatemic rickets and idiopathic hypercalciuria. Male: female ratio was 1.22. The median age at diagnosis was 1.5 (range 0.13-11) years. Growth failure was the presenting feature in 86% of children followed by polyuria (60%) and bone deformities (47%). In 60% of children with hereditary Fanconi syndrome, nephropathic cystinosis was diagnosed, all of whom progressed to stage III chronic kidney disease (CKD) within 3.41 ± 1.42 years. With appropriate therapy, catch-up growth was noted in d-RTA and Bartter syndrome. Renal tubular disorders usually present with FTT. d-RTA is the most common etiology followed by Bartter-like syndrome. Renal function is preserved in all these disorders except for nephropathic cystinosis, who ultimately progressed to CKD. With appropriate and inexpensive therapy, these children do grow well.

Pattern of Renal Diseases in Children Presented to King Abdulaziz University Hospital

The pattern of renal diseases in children presented to the tertiary pediatric nephrology center at King Abdulaziz University Hospital was reviewed respectively over the period of 8 years (20052012). A total of 850 children were seen. Male: female ratio is 2:1. Median (range) age at presentation was 9 (0.01-16) years. Fifty-five percent were Saudi and 45% were from various nationalities. Underlying etiology was congenital or inherited disease in 55.5% of the children. Glomerular diseases were the most common in acquired causes while obstructive uropathy was the most common in congenital causes. Non-neurogenic neurogenic bladder was the most common underlying diagnosis of children with urological abnormalities (28%), followed by posterior urethral valve (26%). Nephrotic syndrome was the most common of glomerular diseases with 31% with steroid resistant nephrotic syndrome. Renal impairment was present in 315 children with low glomerular filtration rate; chronic kidney disease stage 2 in 88, stage 3 in 84, stage 4 in 35 and stage 5 in 108. In conclusion, childhood congenital or inherited renal diseases were the majority. Non-neurogenic neurogenic bladder was the most common urological abnormality and nephrotic syndrome was the most common glomerular disease with high percentage of steroid resistant nephrotic syndrome. A considerable percentage presented with advanced chronic kidney disease.