Scholars@Duke
Shashi Kumar Nagaraj

Shashi Kumar Nagaraj

Professor of Pediatrics
Pediatrics, Nephrology
shashi.nagaraj@duke.edu
2301 Erwin Rd, Durham, NC 27710
2301 Erwin Rd, Durham, NC 27710

Selected Publications

Long-Term Efficacy and Safety of the Long-Acting Complement C5 Inhibitor Ravulizumab for the Treatment of Atypical Hemolytic Uremic Syndrome in Adults

Journal Article Kidney International Reports · June 1, 2021 Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare, complex, multisystem disease of dysregulated complement activity, characterized by progressive thrombotic microangiopathy (TMA), acute kidney injury, and multiorgan dysfunction, which often ... Full text Cite

Tailored use of belatacept in adolescent kidney transplantation.

Journal Article Am J Transplant · March 2020 Adolescent transplant recipients are at risk for nonadherence, development of de novo donor-specific antibody (dnDSA), and allograft loss. Belatacept, a selective T cell costimulatory blocker, is associated with reduced dnDSA, improved renal function, and ... Full text Link to item Cite

SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder.

Journal Article J Clin Invest · January 2, 2020 Inherited optic neuropathies include complex phenotypes, mostly driven by mitochondrial dysfunction. We report an optic atrophy spectrum disorder, including retinal macular dystrophy and kidney insufficiency leading to transplantation, associated with mito ... Full text Link to item Cite

Correction to: Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings : A Midwest Pediatric Nephrology Consortium (MWPNC) study.

Journal Article Pediatr Nephrol · March 2019 The original version of this article unfortunately contained a mistake. The subtitle "A Midwest Pediatric Nephrology Consortium (MWPNC) study" was missing. The correct title including subtitle is given above. ... Full text Link to item Cite

Psychosocial Assessment of Candidates for Transplantation (PACT) Score Identifies High Risk Patients in Pediatric Renal Transplantation.

Journal Article Front Pediatr · 2019 Background: Currently, there is no standardized approach for determining psychosocial readiness in pediatric transplantation. We examined the utility of the Psychosocial Assessment of Candidates for Transplantation (PACT) to identify pediatric kidney trans ... Full text Open Access Link to item Cite

Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings.

Journal Article Pediatr Nephrol · October 2018 BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end-stage kidney disease in children. Recurrence of primary disease following tra ... Full text Link to item Cite

Looking beyond the exome: a phenotype-first approach to molecular diagnostic resolution in rare and undiagnosed diseases.

Journal Article Genet Med · April 2018 PurposeTo describe examples of missed pathogenic variants on whole-exome sequencing (WES) and the importance of deep phenotyping for further diagnostic testing.MethodsGuided by phenotypic information, three children with negative WES underwent targeted sin ... Full text Open Access Link to item Cite

Neutropenic enterocolitis (typhlitis) in a pediatric renal transplant patient. A case report and review of the literature.

Journal Article Pediatr Transplant · September 2017 NE (typhlitis) is a potentially life-threatening disease process characterized by bowel wall edema, ulceration, and hemorrhage in an immunosuppressed patient. We report a 15-year-old boy status post deceased donor renal transplantation who presented with f ... Full text Link to item Cite

Rare variants in tenascin genes in a cohort of children with primary vesicoureteric reflux.

Journal Article Pediatr Nephrol · February 2016 BACKGROUND: Primary vesicoureteral reflux (PVUR) is the most common malformation of the kidney and urinary tract, and reflux nephropathy is a major cause of chronic kidney disease in children. Recently, we reported mutations in the tenascin XB gene (TNXB) ... Full text Link to item Cite

Variability in phenotype induced by the podocin variant R229Q plus a single pathogenic mutation.

Journal Article Clin Kidney J · October 2015 BACKGROUND: Mutations in podocin (NPHS2) are the most common cause of childhood onset autosomal recessive steroid-resistant nephrotic syndrome (SRNS). The disease is characterized by early-onset proteinuria, resistance to immunosuppressive therapy and rapi ... Full text Open Access Link to item Cite

HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome.

Journal Article J Am Soc Nephrol · July 2015 Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome arra ... Full text Link to item Cite

Efficacy of antibiotic prophylaxis in children with vesicoureteral reflux: systematic review and meta-analysis.

Journal Article J Urol · March 2015 PURPOSE: Controversy exists regarding the use of continuous antibiotic prophylaxis vs observation in the management of children with vesicoureteral reflux. The reported effectiveness of continuous antibiotic prophylaxis in children with reflux varies widel ... Full text Link to item Cite

Neurogenic bladder dysfunction presenting as urinary retention in neuronopathic Gaucher disease.

Journal Article JIMD Rep · 2015 Neuronopathic Gaucher disease can present as a continuum of clinical findings, including somatic symptoms of anemia, thrombocytopenia, hepatosplenomegaly, and bone disease as well as neurologic sequelae. There is a spectrum of neurologic symptoms ranging f ... Full text Link to item Cite

A de novo novel missense mutation in AVPR2 with severe nephrogenic diabetes insipidus.

Journal Article NDT Plus · December 2010 We describe a paediatric case of nephrogenic diabetes insipidus (NDI) with a novel mutation in the arginine vasopressin receptor 2 gene (AVPR2) in the absence of a family history of congenital polyuria. The patient, a 5-month-old Caucasian boy, had failure ... Full text Link to item Cite

Management patterns of childhood-onset nephrotic syndrome.

Journal Article Pediatr Nephrol · November 2009 As an initial effort to identify opportunities to improve the management of children with nephrotic syndrome, the goal of this study was to assess the present-day management of children with primary nephrotic syndrome. A web-based survey was designed to as ... Full text Link to item Cite

Potential donor-recipient MYH9 genotype interactions in posttransplant nephrotic syndrome after pediatric kidney transplantation.

Journal Article Am J Transplant · October 2009 Recurrence of focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome is relatively common after kidney transplantation in young recipients whose predialysis course consists of heavy proteinuria, hypertension and subacute loss of kidney function. ... Full text Link to item Cite

Management of childhood onset nephrotic syndrome.

Journal Article Pediatrics · August 2009 The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children prese ... Full text Link to item Cite

Efficacy, safety, and pharmacokinetics of candesartan cilexetil in hypertensive children aged 6 to 17 years.

Journal Article J Clin Hypertens (Greenwich) · October 2008 This 4-week randomized, double blind, placebo-controlled study (N=240), 1-year open label trial (N=233), and single-dose pharmacokinetic study (N=22) evaluated candesartan cilexetil (3 doses) in hypertensive children aged 6 to 17 years. Seventy-one percent ... Full text Link to item Cite

Index of suspicion.

Journal Article Pediatr Rev · April 2007 Full text Link to item Cite

Peritonitis due to Neisseria mucosa in an adolescent receiving peritoneal dialysis.

Journal Article Infection · October 2005 Neisseria mucosa is part of the normal nasopharyngeal flora and rarely pathogenic in humans. Reports of serious infections associated with this pathogen are very unusual. A 17-year-old boy with end-stage renal disease due to IgA nephropathy presented with ... Full text Link to item Cite

Renovascular disease in children and adolescents.

Journal Article J Vasc Surg · June 2005 PURPOSE: This retrospective review describes the surgical management of renovascular disease in 25 consecutive children and adolescents with severe hypertension. METHODS: Patients Full text Link to item Cite

Cranberry use among pediatric nephrology patients.

Journal Article Ambul Pediatr · 2005 INTRODUCTION: Recurrent urinary tract infections are common in children, and the use of complementary therapies is common in other children with recurrent illnesses. However, little is known about the use of cranberry products by children with renal diseas ... Full text Link to item Cite

Kidney and pancreas transplantation at Wake Forest University Baptist Medical Center.

Journal Article Clin Transpl · 2003 More than 1,100 transplants have been performed at WFUBMC, including 60 pediatric transplants and 40 pancreas transplants. The one-year living donor kidney graft survival rate exceeds 90% and the 2 year deceased donor kidney graft survival rate exceeds 80% ... Link to item Cite

Acute renal failure in cystic fibrosis: association with inhaled tobramycin therapy.

Journal Article Pediatr Pulmonol · November 2002 We describe a 20-year-old patient with cystic fibrosis who developed acute nonoliguric renal failure associated with inhaled tobramycin. Clinical evaluation and renal biopsy findings were consistent with aminoglycoside-induced changes. Renal failure due to ... Full text Link to item Cite

Basic fibroblast growth factor among children with diarrhea-associated hemolytic uremic syndrome.

Journal Article J Am Soc Nephrol · March 2002 Diarrhea-associated hemolytic uremic syndrome (D+HUS) is characterized by endothelial injury and activation of inflammatory cytokines. Basic fibroblast growth factor (bFGF) is an angiogenic peptide released in response to vascular damage. The plasma concen ... Full text Link to item Cite

Pediatric biopsy of a single native kidney.

Journal Article Pediatr Nephrol · November 2000 Technological improvements have reduced the frequency of complications in children receiving a percutaneous renal biopsy. No study has systematically compared the safety of open and percutaneous kidney biopsy. Yet many nephrologists consider a single nativ ... Full text Link to item Cite

Molecular cloning of KS, a novel rat gene expressed exclusively in the kidney.

Journal Article Kidney Int · November 1998 BACKGROUND: We aimed to identify genes with kidney specific, developmentally regulated expression. Here we report the cDNA sequence and expression pattern of KS, a novel kidney-specific rat gene. METHODS: A partial cDNA was identified by differential displ ... Full text Link to item Cite

Water intoxication in a patient with the Prader-Willi syndrome treated with desmopressin for nocturnal enuresis.

Journal Article J Urol · February 1997 PURPOSE: We report on a girl with the Prader-Willi syndrome who received desmopressin for nocturnal enuresis, and water intoxication developed after she ingested a large amount of fluid. MATERIALS AND METHODS: The patient received 10 mg. desmopressin at be ... Link to item Cite

A UNIQUE KIDNEY SPECIFIC GENE

Conference JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1995 Link to item Cite

Hyperimmune human IgG or recombinant human granulocyte-macrophage colony-stimulating factor as adjunctive therapy for group B streptococcal sepsis in newborn rats.

Journal Article J Pediatr · May 1993 Group B streptococcus (GBS) continues to cause considerable morbidity and death in newborn infants despite the use of antibiotics. We investigated the use of adjunctive therapies to be used with antibiotics in the treatment of neonatal sepsis, using a neon ... Full text Link to item Cite