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  Most popular articles (Since March 23, 2018)

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Challenges in managing end stage kidney disease in children
Ali Asghar Anwar Lanewala
January-June 2018, 1(1):3-7
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.
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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
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.
  1,102 179 -
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
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.
  1,035 173 -
Kidney length in healthy term neonates
Nandini Malshe, Priscilla Joshi, Lina Ajay Ranchhod, Neel Tapryal, Jyoti Sharma
January-June 2018, 1(1):8-11
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.
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Acute kidney injury in children treated with aminoglycosides
Mukta Mantan, Priyanka Jindal, Smita Kaushik
January-June 2018, 1(1):17-21
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.
  996 172 -
A new journal
Arvind Bagga
January-June 2018, 1(1):2-2
  892 177 -
Message from the secretary general, asian pediatric nephrology association
Hui-Kim Yap
January-June 2018, 1(1):1-1
  886 183 -
Therapies for steroid-sensitive nephrotic syndrome
RS Thalgahagoda, A H. H M. Jayaweera, UI Karunadasa, AS Abeyagunawardena
July-December 2018, 1(2):56-61
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.
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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
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.
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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
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.
  709 161 -
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
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.
  708 144 -
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
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.
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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
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.
  734 93 -
Challenge of congenital abnormalities of the kidney and urinary tract
RN Srivastava
July-December 2018, 1(2):49-51
  640 168 -
Reversal of delayed pulmonary hemorrhage with plasmapheresis in antiglomerular basement membrane disease
Vina Tresa, Sabeeta Khatri, Irshad Ali, Seema Hashmi, Ali Asghar Lanewala
January-June 2018, 1(1):29-32
Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV collagen in the GBM, may present with rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. While rare in childhood, pediatric patients usually have both renal and pulmonary involvement. We report a child who presented with crescentic glomerulonephritis and severe renal-limited anti-GBM disease. The diagnosis was aided by findings of characteristic linear IgG deposits on GBM on immunofluorescence on kidney biopsy and high titers of anti-GBM antibody. As patients with renal-limited disease and severe renal failure usually do not recover renal function despite aggressive immunosuppression and plasmapheresis, intensive therapy was withdrawn while continuing maintenance hemodialysis. However, the patient later developed severe alveolar hemorrhage that responded completely to plasmapheresis and intensification of immunosuppression. Sustained serological and pulmonary response was documented, even though renal dysfunction persisted. Our case emphasizes the utility of kidney biopsy and serology in enabling rare diagnosis in patients with rapidly progressive glomerulonephritis and the role of aggressive plasmapheresis in enabling recovery from delayed catastrophic alveolar hemorrhage.
  716 66 -
Nutritional challenges across the spectrum of chronic kidney disease
Kristen Sgambat, Kaushalendra Amatya, Asha Moudgil
January-June 2019, 2(1):2-15
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.
  550 146 -
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
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.
  577 64 -
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
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.
  497 124 -
Journal Scan
Priyanka Khandelwal
January-June 2018, 1(1):45-48
  514 103 -
From the Editorial Board
Arvind Bagga
January-June 2019, 2(1):1-1
  473 132 -
Eculizumab as treatment in dense deposit disease in children
Saeed M Alzabli, Abdulkarim Al Anazi, Hassan Y Faqeehi, Muhammad Amin Ur Rahman, Mohamed E Suliman, Khawla A Rahim
July-December 2018, 1(2):78-83
Background: Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease that occurs secondary to hyperactivity of the alternative complement pathway. Renal biopsy typically reveals electron-dense deposits in the glomerular basement membrane. However, treatment options are limited. Till date, to the best of our knowledge, no evidence exists for specific treatment influencing the disease course. Eculizumab, a monoclonal antibody, has been reported to prevent membrane attack complex formation by binding to C5 and leads to improvement in clinical findings. However, the number of reported cases in which eculizumab was administered, particularly in children, is limited. Subjects and Methods: In this report, we present our experience with three pediatric cases of DDD receiving eculizumab treatment. All three patients were diagnosed with DDD on kidney biopsy. All patients showed acute kidney injury, high blood pressure, proteinuria >1 g/day, and decreased C3 levels. Immunosuppressive therapy, which included high-dose methylprednisolone, prednisolone, mycophenolate mofetil, or plasma exchange (in the third patient), as well as antihypertensive drugs, was administered to all the patients. Eculizumab treatment was initiated early in two patients but was initiated later in the third patient, who underwent dialysis initially. Results: The two patients with early initiation of eculizumab treatment showed significant improvement of proteinuria and renal function within weeks of treatment. C3 levels were normalized in one patient but remained decreased in the other. The third patient showed no response. She ultimately progressed to end-stage renal disease and eventually needed maintenance dialysis. Conclusions: Early initiation of eculizumab was associated with decreased proteinuria and improved renal function in two patients. These findings were in agreement with previous reports on the beneficial effects of eculizumab in DDD patients.
  529 64 -
Kidney disease in cloacal malformations
Majid Malaki
January-June 2018, 1(1):41-42
  501 77 -
Kids' dream choir
Alison Ma
January-June 2018, 1(1):43-44
  502 75 -
Hypertension with metabolic alkalosis
Aakanksha Sharma, Priyanka Khandelwal, Aditi Sinha, Sanjeev Kumar, Pankaj Hari, Arvind Bagga
July-December 2018, 1(2):90-92
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.
  494 74 -
Nephrogenic systemic fibrosis: A rare complication following exposure to gadolinium-based contrast media
Habib Qaiser, Vina Tresa, Sabeeta Khatri, Irshad Bajeer Ali, Ali Lanewala, Seema Hashmi
July-December 2018, 1(2):84-86
Nephrogenic systemic fibrosis (NSF) is a rare complication following exposure to gadolinium-based contrast media. Gadolinium-based contrast agents (GBCAs) are widely used for imaging throughout the world. NSF, formerly known as nephrogenic fibrosing dermopathy, is a rare progressive fibrosing disorder associated with administration of GBCA in patients with severely compromised renal functions. The condition is well reported in adults, but pediatric cases are rarely reported. Out of 1280 cases in the literature of NSF associated with GBCA, only 12 were found in the pediatric age group. We are reporting a pediatric case of a 7-year-old child with chronic kidney disease Stage VD, who developed NSF following magnetic resonance imaging.
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