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REVIEW ARTICLES
Nutritional challenges across the spectrum of chronic kidney disease
Kristen Sgambat, Kaushalendra Amatya, Asha Moudgil
January-June 2019, 2(1):2-15
DOI:10.4103/AJPN.AJPN_2_19  
Maintenance of optimal nutrition plays a key role in the management of children with chronic kidney disease (CKD) across different stages of CKD, during dialysis and following transplantation. Malnutrition, both under- and over-nutrition, is widely prevalent and negatively impacts short- and long-term outcomes. It leads to growth retardation, increased risk of hospitalization and infections, poor cognition, and decreased quality of life. Understanding of the pathogenesis of malnutrition is crucial for its proper management. This review discusses the definition, prevalence, pathogenesis, and management of malnutrition in different stages of CKD.
  6,698 653 -
Vesicoureteral reflux and recurrent urinary tract infections
Jitendra Meena, Pankaj Hari
July-December 2019, 2(2):61-70
DOI:10.4103/AJPN.AJPN_26_19  
Vesicoureteral reflux (VUR) refers to backflow of urine from the bladder into the upper urinary tract. Children with high-grade VUR are at risk of recurrent urinary tract infections (UTIs). It is commonly associated with bladder–bowel dysfunction, necessitating treatment because it further increases the risk of recurrent UTI and results in delayed resolution of VUR. Almost one-third of patients with VUR diagnosed following UTI show renal scarring. It is debated whether scarring in VUR is entirely acquired following UTI or due to dysplasia. The efficacy of antibiotic prophylaxis has been variable for the prevention of UTI in children with VUR. A meta-analysis suggests that it might be of some benefit in preventing UTI, but not renal scarring and at cost of bacterial resistance. Due to questionable efficacy and potential risk, use of antibiotic prophylaxis should be based on risk stratification rather than mere presence of reflux. In view of lack of significant improvement in long-term outcomes with current available interventions, there is ongoing debate on the aggressiveness of algorithms for diagnosing VUR following UTI. Long-term complications of VUR include proteinuria, hypertension, and end-stage renal disease. As opposed to previous assumption, the risk of end-stage renal failure and hypertension is fairly small with scarring following UTI.
  5,615 565 -
BRIEF REPORTS
Pediatric acute poststreptococcal glomerulonephritis: A single-center experience
Sherif Mohamed El-Desoky, Lujain K I. Al-Sulimani, Manar T O. Alkhatieb, Khalid A Alhasan, Amr S Albanna, Jameela A Kari
January-June 2019, 2(1):36-40
DOI:10.4103/AJPN.AJPN_6_19  
We evaluated the frequency, severity, and outcome of children with acute poststreptococcal glomerulonephritis (PSGN) by retrospectively reviewing case records of all children diagnosed with PSGN between 2007 and 2015. The diagnosis of PSGN was based on the evidence of recent streptococcal infection, including positive streptococcal culture from the skin or throat, high or rising antistreptolysin O titer (>333 IU/ml), or high anti-deoxyribonuclease B level (>200 IU/ml). In addition, low serum complement 3 level and/or kidney biopsy results were considered. Children with evidence of chronic kidney disease (CKD) were excluded from the study. Sixty-six children (50 boys) with age 8.4 ± 3.4 years were included in the study. Ninety percent of the children had proteinuria and 79% had impaired glomerular filtration rate at presentation. One child had rapidly progressive glomerulonephritis. At 1-year follow-up, 10% of the children had renal impairment, 25% had hypertension, and 40% had proteinuria. As a considerable percentage of patients with PSGN in this series had CKD at 1-year follow-up, careful prolonged follow-up of such patients is advisable.
  4,527 688 -
ORIGINAL ARTICLES
Renal angina index in the prediction of acute kidney injury in critically ill children
Suma Sundararaju, Aditi Sinha, Pankaj Hari, Rakesh Lodha, Arvind Bagga
January-June 2019, 2(1):25-30
DOI:10.4103/AJPN.AJPN_8_19  
Background: Renal Angina Index (RAI) was recently proposed as a tool to identify patients at high risk for severe acute kidney injury (AKI) by integrating baseline, contextual, and clinical evidence of renal injury and to optimize biomarker utility in intensive care units. Methods: In this unicentric prospective observational study, we estimated RAI at admission in 285 critically ill patients, aged 1 month to 18 years, and evaluated the utility of renal angina (RAI ≥8) in identifying patients with severe or any AKI on day 3 and day 7. The relationship between RAI and need for renal replacement therapy (RRT) and duration of mechanical ventilation and hospital stay was also examined. Results: Renal angina was present in 49.4% of 285 patients. Severe AKI, observed in 29 (10.2%) patients on day 3 and 13.2% of 144 patients followed to day 7, had an incidence of 1.1 (0.8–1.6) episodes per 100 person-days. Thirty-six (12.6%) patients required RRT. RAI satisfactorily identified patients at risk of severe AKI on days 3 (area under the curve [AUC]: 0.82; 95% confidence interval [CI]: 0.73–0.90) and 7 (AUC: 0.73; 95% CI: 0.62–0.84), was more useful than Pediatric Index of Mortality in such discrimination, and correlated with duration of mechanical ventilation and hospital stay. However, RAI thresholds ≥12 or ≥20 had higher specificity, Youden index, and positive predictive value than that of RAI ≥8 in discriminating severe AKI on day 3 or 7 and distinguished between patients with and without need for RRT. Conclusions: RAI usefully predicts the development of subsequent severe AKI on days 3 and 7, and is associated with duration of mechanical ventilation and hospital stay. A higher RAI threshold (≥12 or ≥20) is more discriminatory than RAI ≥8.
  4,428 655 -
REVIEW ARTICLES
Next-generation sequencing-based genetic diagnosis of steroid-resistant nephrotic syndrome: Benefits and challenges
Varsha Chottusing Pardeshi, Ambily Narikot, Anil Vasudevan
January-June 2019, 2(1):16-24
DOI:10.4103/AJPN.AJPN_9_19  
Steroid-resistant nephrotic syndrome (SRNS) is the second-most common cause of chronic kidney disease in children and in those requiring kidney transplantation. The disease shows significant heterogeneity in its age at onset and clinical course. The discovery of mutations in NPHS1, the gene encoding nephrin that is a key component of the podocyte slit diaphragm, in a subset of children with congenital NS, led to identification of a distinct subgroup of patients of SRNS that has an underlying genetic etiology. Subsequently, mutations in over 53 podocyte genes have been implicated in monogenic forms of SRNS with no clear genotype-phenotype correlations. The large number of genes implicated in SRNS, phenotypic variability, and lack of information about frequency of mutations in these genes, makes the use of genetic testing in the management of children with SRNS challenging in terms of decisions on who to test, which genes to screen, and how to use the information obtained from testing in the clinical setting. Given the genetic heterogeneity and phenotypic variability, Sanger sequencing is not a feasible approach for routine testing. Next-generation sequencing (NGS) technology is emerging as the preferred method to screen multiple genes in genetically heterogeneous diseases like SRNS. Such high-throughput sequencing method permits rapid and cost-effective simultaneous screening of large number of individuals and genes. However, the high throughput combined with significant phenotypic and genetic variability of monogenic SRNS poses unique challenges for clinicians in the interpretation of genetic result. This review provides an overview of utility of genetic testing with focus on NGS-based genetic testing and the challenges in the interpretation of genetic results in clinical settings.
  4,039 448 -
Therapies for steroid-sensitive nephrotic syndrome
RS Thalgahagoda, A H. H M. Jayaweera, UI Karunadasa, AS Abeyagunawardena
July-December 2018, 1(2):56-61
DOI:10.4103/AJPN.AJPN_35_18  
Nephrotic syndrome (NS), a common childhood kidney disease, is associated with significant morbidity and mortality due to disease complications. Most patients who respond to corticosteroids show a relapsing course that requires repeated courses of therapy, and frequent relapses or steroid dependence are common. Most children with steroid-sensitive relapses show minimal change disease upon biopsy. Focal segmental glomerulosclerosis is the predominant histology in patients with steroid-resistant NS where renal biopsy is recommended, and a complicated disease course is anticipated. Patients with frequent relapses are at risk of severe infections, thrombosis, and hypovolemia and receive repeated and prolonged courses of prednisolone that often result in corticosteroid toxicity. These challenges have led to the use of numerous corticosteroid-sparing agents or regimens to reduce the risk of relapses as well as cumulative corticosteroid burden. This review discusses therapy-related aspects of steroid-sensitive NS and compares different regimens of corticosteroid and other immunosuppressive medications that are used in managing this condition.
  3,514 441 -
ORIGINAL ARTICLES
Pediatric kidney transplantation: Experience over two decades
Jitendra Meena, Aditi Sinha, Pankaj Hari, Amit K Dinda, Priyanka Khandelwal, Sanjeev Goswami, Rajendra N Srivastava, Sandeep Guleria, Ashima Gulati, Virinder K Bansal, Arvind Bagga
January-June 2018, 1(1):22-28
DOI:10.4103/AJPN.AJPN_9_18  
Introduction: Information on outcomes of pediatric renal transplantation in developing countries is limited to few single-center reports with small numbers. Methods: Medical records of patients who underwent kidney transplantation at a single tertiary care center were retrospectively reviewed for information on recipient and donor characteristics, immunosuppression, posttransplantation complications, anthropometry and graft and patient survival. Allograft and patient survival were estimated using Kaplan–Meier survival analysis; risk factors for acute and chronic rejection (AR and CR) and patient or allograft loss were examined using Cox regression. Results: During 1995–2017, 116 patients (84% boys) received 120 kidney allografts at the age of 13 ± 3.8 years, chiefly from live-related donors (76%). During median (range) 4.5 (0.8–19.5) years of follow-up, AR and CR were seen in 24% and 15.5% allografts. Ten (8.8%) patients died, chiefly due to septicemia, while 26 (21.7%) allografts were lost, most often to CR. Severe infections included septicemia (13%), urinary (23%) or respiratory tract (9%) infections, cytomegalovirus (11%), and chronic viral hepatitis (7%). At 1, 3, 5, 10 and 15 years, patient survival was estimated at 95.5%, 94.4%, 91.7%, 89.2% and 79.3%, respectively, while allograft survival was 92.2%, 87.2%, 85.6%, 77.6% and 77.6%, respectively. Final height, at −2.2 ± 1.3 standard deviation scores, was significantly improved from baseline (P = 0.001). Conclusions: Pediatric kidney transplantation in developing countries is associated with similar patient and allograft survival as compared to developed regions. While severe infections cause serious morbidity and mortality, rates of immunological complication are similar to developed regions. While patients show significant height gain, final stature is subnormal.
  3,423 366 -
REVIEW ARTICLE
Challenges in managing end stage kidney disease in children
Ali Asghar Anwar Lanewala
January-June 2018, 1(1):3-7
DOI:10.4103/AJPN.AJPN_2_18  
Treatment of children with end-stage kidney disease (ESKD) poses various challenges. While these have been studied extensively in affluent countries, data from developing regions are scant. In most rich countries, state-of-art management and financial and social support are provided by the government or available through insurance, and prognosis for even young children is improving. In contrast, most low-income countries, either lack in facilities for renal replacement or these are not afforded by the vast majority, and a large number of patients succumb for lack of treatment. In rare settings where such treatment is provided free of cost, the inability to meet costs of related expenses such as transport and accommodation result in families discontinuing management for most patients. Studies should define the extent and determinants of these concerns for children with ESKD in developing countries.
  3,027 367 -
ORIGINAL ARTICLES
Kidney length in healthy term neonates
Nandini Malshe, Priscilla Joshi, Lina Ajay Ranchhod, Neel Tapryal, Jyoti Sharma
January-June 2018, 1(1):8-11
DOI:10.4103/AJPN.AJPN_10_18  
Introduction: There is a lack of data on kidney size in Indian neonates, their determinants, and how the sizes compare with neonatal kidney sizes reported elsewhere. Methods: This cross-sectional study of renal size on ultrasonography was conducted in 765 consecutive healthy term neonates over 1 year at a single center. Multivariable regression analysis was used to examine the relationship of kidney length and volume with neonatal anthropometric variables and maternal characteristics. Information on kidney size was compared with published reports. Results: The mean ± standard deviation for the length of the right and left kidney were 38.6 ± 2.2 mm and 38.9 ± 2.2 mm, respectively, while the estimated kidney volume of the right and left kidney was 7.7 ± 1.7 ml and 8.3 ± 1.9 ml, respectively. Birth weight and neonatal length were independent predictors of renal length. Kidney length and volume in the present study were significantly lower as compared to that reported in the studies from outside south Asia. Conclusions: Kidney length in Indian term neonates depends on neonatal weight and length and is smaller in comparison to neonates from outside south Asia.
  2,942 348 -
Incidence and determinants of acute kidney injury in patients with nephrotic syndrome
Sunil Kushwah, Menka Yadav, Pankaj Hari, Jitendra Meena, Aditi Sinha, Arvind Bagga
July-December 2019, 2(2):75-81
DOI:10.4103/AJPN.AJPN_25_19  
Background: Retrospective studies from developed countries indicate an increasing prevalence of acute kidney injury (AKI) in nephrotic syndrome. Prospective information on incidence and determinants of AKI in nephrotic syndrome from developing countries is limited. Methods: This prospective study enrolled consecutive patients with nephrotic syndrome, 1 month–18 years old, admitted to a single tertiary care center. Patients were evaluated daily for the development of AKI using Kidney Disease Improving Global Outcomes serum creatinine criteria until day 14 or discharge, along with clinical and biochemical information to determine associated risk factors. Estimated glomerular filtration rate (eGFR) was reassessed at 3 months' follow-up. Results: Of 115 patients (72.2% boys) enrolled at median (interquartile range) age 64 (36–111) months, 25 (21.7%) developed AKI. The incidence density of AKI was 3.3 (2.2, 4.8) episodes per 100 person-days. Stage 3 AKI comprised 64% of cases. Steroid-resistant illness, hypoalbuminemia, and low baseline eGFR were independently associated with the occurrence of AKI. AKI recovered completely or partially in 48% and 20% cases, respectively; 20% of patients remained dialysis-dependent and 12% of patients died. Patients with AKI had significantly longer hospital stay, and lower median eGFR at 3-month follow-up, than those without AKI. Conclusions: AKI affects 21.7% of patients admitted with nephrotic syndrome, is predominantly severe, and is associated with adverse outcomes in one-third cases, prolonged hospital stay, and reduced eGFR at discharge and short-term follow-up. Steroid-resistant nephrotic syndrome, hypoalbuminemia, and low eGFR at admission were independently associated with the occurrence of AKI.
  2,878 410 -
Acute kidney injury in children treated with aminoglycosides
Mukta Mantan, Priyanka Jindal, Smita Kaushik
January-June 2018, 1(1):17-21
DOI:10.4103/AJPN.AJPN_16_18  
Introduction: The etiology of acute kidney injury (AKI) in hospitalized children is multifactorial. While nephrotoxic antibiotics, such as aminoglycosides, cause AKI, the prevalence and severity of AKI in this context are uncertain. Methods: This prospective observational study enrolled consecutive patients, from 1 month to 12-year-old, admitted to pediatric wards at an academic tertiary care center between March 2013 and March 2014. Included patients had normal renal function at baseline and were treated with aminoglycosides for more than 7 days. Serum creatinine was monitored serially for the development of AKI by pediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), and AKI network (AKIN) classifications. Data, presented as percentage or mean ± standard deviation, were compared using appropriate tests. Results: Of 100 patients (68% boys) treated with aminoglycosides for 11.4 ± 4.5 days, chiefly for pneumonia, febrile neutropenia and abscesses, 97% received amikacin and 3% received gentamicin, chiefly (84%) in multiple doses. AKI was observed in 46% and 62% of cases using AKIN and pRIFLE criteria, respectively. Patients with AKI were younger than those without AKI (P = 0.04) and received multiple dosing more often than once-daily regimen (P = 0.07). Conclusions: AKI develops in a significant proportion of hemodynamically stable patients treated with aminoglycosides for 7 days or longer. Young age and multiple daily doses are potential risk factors for AKI in patients treated with aminoglycosides.
  2,771 370 -
STUDY PROTOCOL
Determining the optimal dose of cholecalciferol supplementation in children with chronic kidney disease (C3 Trial): Design of an open-label multicenter randomized controlled trial
Arpana Aprameya Iyengar, Nivedita Kamath, V Hamsa, Susan Uthup, Jyoti Sharma, Jyoti Singhal, Sudha Ekambaram, Rukshana Shroff
July-December 2018, 1(2):67-73
DOI:10.4103/AJPN.AJPN_34_18  
Introduction: 25-hydroxyvitamin D (25OHD) deficiency is common in children with chronic kidney disease (CKD) and can affect bone mineralization and cardiovascular morbidity. It is important to treat 25OHD deficiency appropriately in a manner that ensures not only replenishing stores but also sustaining adequate 25OHD levels without causing toxicity. The present study was planned to determine the appropriate dosing regimen for oral cholecalciferol that achieves and maintains normal 25OHD levels in children with CKD stage 2–4 and to assess the effect of various dosing regimens on bone biomarkers, secondary hyperparathyroidism, and vitamin D toxicity. Methods: We present the design of an open-label, multicenter randomized controlled trial conducted across four pediatric nephrology centers in India. Children in CKD stages 2–4 with 25OHD levels <30 ng/ml will be randomized to one of three therapy regimens for oral cholecalciferol (3000 IU daily, 25,000 IU weekly, or 100,000 IU monthly) given for 3 months, allowing an equivalent cumulative cholecalciferol dose in all arms over this intensive replacement therapy phase. After 3 months, patients with 25OHD levels ≥30 ng/ml will continue on maintenance therapy, administered at 1000 IU cholecalciferol orally daily for 9 months. Outcomes include the median change in the level of 25OHD from baseline to the end of intensive phase; proportions of children in each limb that attain and maintain normal 25OHD levels after intensive replacement and maintenance treatment; the change in levels of bone biomarkers and the incidence of adverse effects with each therapy regimes. Conclusion: The study design of a multicenter randomized controlled trial in children with CKD is described. Trial Registration: Clinical Trials Registry of India; www.ctri.nic.in; CTRI/2015/11/010180.
  2,815 320 2
ORIGINAL ARTICLE
Nationwide pediatric renal biopsy audit by the Indian Society of Pediatric Nephrology
Rajiv Sinha, Nimisha Arora, Manpreet Kaur, Arpana Iyengar, Pankaj Hari, Abhijeet Saha
July-December 2018, 1(2):62-66
DOI:10.4103/AJPN.AJPN_25_18  
Objective: The survey was conducted to identify current renal biopsy practices in India and compare them with the British Association of Pediatric Nephrology (BAPN, 2015) standards. Methods: A 53-question survey questionnaire was sent to 48 centers across the country by electronic mail. Questions included were related to the number of biopsies performed, indications, prerequisites and procedure of biopsy, monitoring, and complications. The results were compared against the BAPN 2015 standards. Results: Thirty (62.5%) out of 48 centers responded to the questionnaire. Real-time ultrasound was the favored method at 24 (80%) centers. Most (80%) of the biopsies were performed by nephrologists alone. The biopsy was usually (80%) an inpatient procedure with overnight hospitalization; 20% of the centers performed it as a day-care procedure. The 18-gauge needle was preferred by 60% of the centers. Biopsy was achieved with three or fewer passes in 93% of the centers. Almost half (47%) of the centers considered 10 or more glomeruli on light microscopy as adequate to reach a diagnosis. The rates of gross hematuria were <5% in 80% of the centers surveyed. Death following biopsy was reported by two centers. Conclusion: Majority of the centers surveyed across India achieve BAPN standards in most parameters. Such audit of practices against the standards for kidney biopsy enables comparison between units as well as for monitoring of individual center's performance over time.
  2,679 352 -
ORIGINAL ARTICLES
Mycophenolate mofetil for maintenance of remission in children with steroid- and calcineurin inhibitor- dependent nephrotic syndrome: A prospective, randomized multicenter trial
Eun Mi Yang, Eujin Park, Hyun Jin Choi, Hyesun Hyun, Yong-hoon Park, Kyung Hee Han, Hyewon Park, Seong Heon Kim, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
July-December 2019, 2(2):82-87
DOI:10.4103/AJPN.AJPN_10_19  
Background: Mycophenolate mofetil (MMF), a nonsteroidal immunosuppressive agent, has been used as a therapeutic option in various immune-mediated glomerulonephritis. However, controlled studies that examine the efficacy of MMF monotherapy in steroid- and calcineurin inhibitor (CNI)-dependent nephrotic syndrome (NS) are lacking. Aim: This study assessed the efficacy and safety of MMF monotherapy in children with steroid- and CNI-dependent NS. Methods: In this prospective, randomized, open-label, multicenter pilot study, we evaluated the efficacy and safety of MMF after CNI and corticosteroid therapy in children with steroid-dependent NS. We compared the duration and timing of sustained remission between patients managed with or without MMF and monitored for adverse effects of MMF. Statistical Analysis Used: The data were analyzed using standard statistical tests on the modified intention-to-treat population. Results: The baseline characteristics between the two groups did not differ significantly. There was neither difference in the duration of the sustained remission between patients in the MMF and control groups (4.2 ± 3.5 months vs. 3.8 ± 3.4 months, respectively; P = 0.772) nor in the proportion in sustained remission after 12 months (P = 0.936). Patients received MMF did not experience significant toxicity; chiefly, self-remitting gastrointestinal adverse events were observed. Conclusions: Therapy with MMF is safe but ineffective in influencing rates of remission in patients with NS dependent on steroids and CNIs.
  2,635 313 -
BRIEF REPORTS
Respiratory tract infection and diarrhea as risk factors for relapsing nephrotic syndrome
Sudung O Pardede, Achmad Rafli, Jonathan Odilo, Yoshua Billy Lukito
January-June 2018, 1(1):38-40
DOI:10.4103/AJPN.AJPN_1_18  
While infections are known to precipitate disease relapses in childhood nephrotic syndrome, it is unclear whether diarrheal illnesses carry the same risk for relapses as acute respiratory tract infections. This paired case–control study included age- and sex-matched episodes of relapses (cases) and remission (controls). Records were reviewed retrospectively for the presence of acute respiratory tract infections and diarrhea in the two weeks preceding the clinic visit. McNemar test was used to examine associations between these infections and relapse. In 17 patients with 38 paired episodes of relapses and remission, the odds ratio for relapse compared to remission, associated with acute respiratory tract infections, was 3.25 (95% confidence interval: 1.06, 9.97; P = 0.03), while that for acute diarrhea was 3.5 (95% confidence interval: 0.73, 16.85; P = 0.10). Acute respiratory tract infections are a risk factor for relapses in pediatric nephrotic syndrome, while acute diarrhea does not predispose to disease relapses.
  2,555 315 -
ORIGINAL ARTICLES
Association between polymorphisms in genes regulating Vitamin A metabolism and kidney size in Indian Newborns
Ambili Narikot, Varsha Chotu Pardeshi, Annes Siji, Arun George, Anil Vasudevan
January-June 2018, 1(1):12-16
DOI:10.4103/AJPN.AJPN_6_18  
Introduction: Previous studies suggest that maternal serum vitamin A levels and polymorphisms in genes regulating vitamin A metabolism may impact fetal kidney development. Information on these genetic variations is limited to Caucasian population. Methods: This prospective observational single center study included newborns of 349 pregnant women enrolled at 8 weeks of gestation. Kidney volumes were measured at birth by ultrasound and cord blood was tested for single nucleotide polymorphisms (SNPs) in genes encoding for aldehyde dehydrogenase 1 family member A2rs7169289, cellular retinoic acid binding protein-2rs12724719, receptor tyrosine kinasers1800860, retinol-binding proteinrs11187540, and the Vitamin A receptor stimulated by retinoic acid 6rs17852249. Results: The total (left + right) kidney volume, unadjusted and adjusted for body surface area at birth, was 21.5 ± 4.6 ml and 107.3 ± 20.7 ml/m2, respectively. Single nucleotide polymorphisms (SNPs) in genes encoding for proteins regulating vitamin A metabolism were not associated with kidney volume at birth. Conclusions: Polymorphisms in genes involved in vitamin A metabolism are not associated with kidney size in Indian newborns.
  2,459 323 -
Endothelial dysfunction in children with frequently relapsing and steroid-resistant nephrotic syndrome
Anuja Bhatia, Abhijeet Saha, Bobbity Deepthi, Parul Goyal, Ashish Datt Upadhyay, Nand Kishore Dubey
January-June 2020, 3(1):4-9
DOI:10.4103/AJPN.AJPN_28_19  
Background: Impaired endothelial function is a precursor of the atherosclerotic process leading to cardiovascular adverse events. This study evaluated endothelial dysfunction using endothelial markers in children with steroid-resistant NS (SRNS) and frequently relapsing or steroid-dependent NS (FRNS/SDNS). Methods: This was a cross-sectional study with short-term follow up. Thirty-five patients with nephrotic syndrome (NS), aged 1–18 years, including 19 with frequent relapses or steroid-dependent NS (FRNS/SDNS) and 16 with steroid resistant NS (SRNS), and 19 age- and gender-matched controls, were enrolled for the study. Soluble thrombomodulin (sTM), plasminogen activator inhibitor 1 (PAI-1), and von Willebrand factor (vWF) levels were measured in patients with FRNS/SDNS in relapse and after 6 months of steroid-induced remission, at diagnosis in SRNS, and in controls. Results: Levels of vWF, PAI-1, and sTM were significantly higher than controls in patients with active NS (FRNS/SDNS in relapse or SRNS; P < 0.0001). Patients with FRNS/SDNS had significantly elevated vWF levels, compared to controls, even after 6 months of corticosteroid therapy. Levels of vWF were significantly higher for patients with recently diagnosed SRNS than relapse of FRNS/SDNS (P < 0.0001). Conclusion: Children with FRNS/SDNS and SRNS have significant endothelial dysfunction in all stages of disease in varying severity. It is unclear whether persistently elevated biomarkers of endothelial dysfunction contribute to future atherosclerotic events in patients with NS.
  2,515 253 -
REVIEW ARTICLES
Epidemiology of acute kidney injury in critically ill children living in the Kingdom of Saudi Arabia
Jameela Abdulaziz Kari
July-December 2018, 1(2):52-55
DOI:10.4103/AJPN.AJPN_37_18  
Acute kidney injury (AKI) is very common in children admitted to pediatric intensive care and affected children are at increased risk of morbidity and mortality. The epidemiologic characteristics of children with AKI have not been well described in children living in the Kingdom of Saudi Arabia (KSA). This review of the epidemiology of AKI in critically ill children in KSA shows that AKI is common in this population and is chiefly attributed to sepsis, other infections and postcardiac surgery. The occurrence of AKI is linked to increased mortality and length of hospital stay. The severity of AKI correlates with increased inhospital mortality as well as risk of mortality after discharge. A considerable proportion of survivors develop evidence of chronic kidney disease. Cystatin C and urinary neutrophil gelatinase-associated lipocalin are useful in enabling early diagnosis of AKI in critically ill children.
  2,461 298 -
BRIEF REPORTS
Hypertension with metabolic alkalosis
Aakanksha Sharma, Priyanka Khandelwal, Aditi Sinha, Sanjeev Kumar, Pankaj Hari, Arvind Bagga
July-December 2018, 1(2):90-92
DOI:10.4103/AJPN.AJPN_20_18  
Severe hypertension in children is chiefly renal parenchymal or renovascular in origin. Renovascular hypertension is usually symptomatic and rarely presents with renal tubular dysfunction. We describe a 2-year-old child with polyuria, failure to thrive, hyponatremia, hypokalemia, metabolic alkalosis, hypercalciuria, low molecular weight proteinuria, and medullary nephrocalcinosis. Evaluation revealed severe hypertension and discrepant renal sizes. Doppler ultrasonography and digital subtraction angiography showed right main renal artery stenosis. Hypertension and electrolyte abnormalities abated following percutaneous angioplasty. Unilateral renal artery stenosis may manifest with symptoms of renal tubular dysfunction alone. Hypokalemia and metabolic alkalosis must prompt consideration of renovascular hypertension and monogenic causes. Angiography is essential for confirmation of renovascular hypertension and enables angioplasty, the mainstay of management.
  2,523 230 -
REVIEW ARTICLES
Summary of 'Hemolytic uremic syndrome in a developing country: Consensus guidelines'
Sushmita Banerjee, Jyoti Sharma
July-December 2019, 2(2):71-74
DOI:10.4103/AJPN.AJPN_21_19  
Hemolytic uremic syndrome (HUS) is a common cause of acute kidney injury in children and has implications of irreversible renal damage. The management depends on the etiology of HUS. Guidelines for India have been formulated to arrive at the etiology with an algorithmic approach based on the epidemiology and the constraints of facilities available for investigations and therapy in the region. Anti-Factor H antibody-associated illness accounts for over half the cases of atypical HUS (aHUS) in Indian children, and prompt plasma exchange and immunosuppression are recommended to lower antibody levels. Since access to eculizumab is limited, the management of other forms of aHUS relies on plasma therapy. Indications for biopsy and concerns around kidney transplantation are highlighted. A brief comparison with current guidelines from other regions has been made.
  2,198 419 1
BRIEF REPORTS
OHVIRA syndrome in an infant girl
Ruba Sahab, Sherif Mohamed El-Desoky, Ahmed Alsayyad, Mazen Kurdi, Jameela A Kari
July-December 2019, 2(2):88-90
DOI:10.4103/AJPN.AJPN_16_19  
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital urogenital anomaly, which usually presents after menarche. We report a 3-month-old infant who presented with recurrent urinary tract infections (UTI) and had an abdominal lump. Radiological investigations revealed absent right kidney, hydronephrotic left kidney, and a cystic pelvic mass. Exploratory laparotomy revealed hydrometrocolpos which was evacuated. During 1-year follow-up, hydronephrosis decreased, there were no further UTI, and kidney function remained normal. Our case illustrates that OHVIRA syndrome may present in early infancy with UTI, renal anomalies, and pelvic mass.
  2,278 239 -
Renal involvement in a child with Donnai-Barrow syndrome
Gurinder Kumar, Manika Chaudhry, Khalid Mohamed Mansour Mohamed Faris, Omar Al Masri
July-December 2018, 1(2):93-95
DOI:10.4103/AJPN.AJPN_22_18  
Donnai-Barrow or facio-oculo-acoustico-renal (DB/FOAR) syndrome is characterized by typical craniofacial features, ocular findings, sensorineural hearing loss and agenesis of the corpus callosum along with varying degree of intellectual disability. Renal involvement in the form of low molecular weight proteinuria is commonly reported. We report a case of an 8-year-old girl with DB/FOAR syndrome which was genetically confirmed to have a novel frameshift mutation, c.13139del in exon 72 of LRP2, the gene encoding low density lipoprotein receptor related protein 2 precursor, megalin. The child had chronic kidney disease (CKD) and significant proteinuria with focal segmental glomerulosclerosis on renal biopsy. Our case highlights presentation in childhood with this rare syndrome, with significant renal involvement as nephrotic range proteinuria and CKD. Children with DB/FOAR syndrome need close follow up with nephrologist.
  2,318 196 2
EDITORIAL
From the Editorial Board
Arvind Bagga
January-June 2019, 2(1):1-1
DOI:10.4103/AJPN.AJPN_15_19  
  2,074 355 -
BRIEF REPORTS
Complete renal recovery following delayed therapy with eculizumab in atypical hemolytic uremic syndrome
Saeed M Alzabli, Abdulkarim Al Anazi, Muhammad Amin Ur Rahman, Khawla A Rahim
January-June 2018, 1(1):33-37
DOI:10.4103/AJPN.AJPN_4_18  
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening illness characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). Plasma therapy, previously considered the primary treatment for this condition, is associated with death and permanent kidney injury in over 50% of patients. Eculizumab, a monoclonal antibody that blocks C5, is far more effective and is the current treatment of choice, but it is not widely available. An 18-month-old boy presented with AKI, anemia, and normal platelet count. While managed initially as proliferative antineutrophil cytoplasmic antibody-associated glomerulonephritis, a diagnosis of partial HUS was suggested by low levels of complement C3, complement factor I (CFI) and complement Factor B (CFB), and histological changes suggesting microangiopathy without thrombi. Later, next-generation sequencing indicated homozygous pathogenic mutations in CFI and heterozygous variations in C3 and CFB. Corticosteroid pulses and plasmapheresis were ineffective in resolving the need for dialysis, and hypertension was refractory to polytherapy. Therapy with eculizumab, initiated only after 5 months on dialysis, was associated with rapid increase in urine output, control of hypertension, and slow but complete renal recovery. Dialysis was discontinued after 5 months of eculizumab therapy, and renal function has been within normal range after 33 months of initiating therapy with eculizumab. Our case emphasizes that a diagnosis of aHUS should be considered in the appropriate clinical setting even in the absence of thrombocytopenia. Kidney biopsy and genetic testing are useful in confirming the diagnosis. Therapy with eculizumab in patients with severe AKI may be associated with complete renal recovery even if therapy is delayed by several months.
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EDITORIAL
Pandemic and practice of pediatric nephrology
Arvind Bagga, Aditi Sinha
January-June 2020, 3(1):1-3
DOI:10.4103/AJPN.AJPN_24_20  
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