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Infections remain the most common cause of hospitalization after kidney transplantation, contributing to significant post-transplant morbidity and mortality. There is a growing body of literature that suggests that immunoglobulins may have a significant protective role against post-transplant infections, although the literature remains sparse, inconsistent, and not well publicized among pediatric nephrologists. Of great concern are data indicating a high prevalence of immunoglobulin abnormalities following transplantation and a possible link between these abnormalities and poorer outcomes. Our educational review focuses on the epidemiology and risk factors for the development of immunoglobulin abnormalities after kidney transplantation, the outcomes in patients with low immunoglobulin levels, and studies evaluating possible interventions to correct these immunoglobulin abnormalities., Author(s): Victoria Dimitriades [sup.1] , Lavjay Butani [sup.2] Author Affiliations: (1) grid.27860.3b, 0000 0004 1936 9684, Division of Pediatric Allergy, Immunology and Rheumatology, Department of Pediatrics, University of California, , [...]

Objective To determine whether pre-transplant body mass index (BMI) affects renal allograft function and survival in pediatric renal transplant recipients. Study design This is a retrospective cohort study using the Organ Procurement and Transplantation Network data from 2000 to 2013 to compare time to total allograft loss (allograft failure or death), prevalence of delayed graft function, prevalence of acute rejection, and estimated glomerular filtration rate (eGFR) post-transplant in pediatric renal transplant recipients categorized by BMI z-score. Results A total of 8804 kidney transplant recipients met our inclusion criteria, and of those, 6% were underweight, 14% were overweight, and 17% were obese pre-transplant. The adjusted hazard ratio (HR) for allograft failure was significantly higher for obese recipients compared to normal weight recipients (HR 1.25, 95% CI 1.1, 1.42); for every 1 point increase in BMI z-score, there was a 7% increased hazard of allograft failure (HR 1.07; 95% CI 1.03-1.1, p < 0.001). The prevalence of delayed graft function and acute rejection increased with higher BMI z-score category; however, this difference did not reach statistical significance. eGFR at 1 and 5 years post-transplant decreased with higher BMI z-score although it was only statistically significant at 1 year. Conclusions Obesity is prevalent in pediatric renal transplant recipients, and obese, but not overweight or underweight, pediatric renal transplant recipients have an increased risk of allograft failure. Implementation of effective obesity interventions in pediatric renal transplant recipients is of critical importance to improve longevity of the renal allograft., Author(s): Erica Winnicki [sup.1] , Madan Dharmar [sup.2] , Daniel J. Tancredi [sup.2] , Stephanie Nguyen [sup.2] , Lavjay Butani [sup.2] Author Affiliations: (Aff1) 0000 0001 2297 6811, grid.266102.1, Department [...]

Background Non-calcium-containing phosphate binders, such as sevelamer preparations, are being increasingly used in patients on dialysis due to their lower association with hypercalcemia and cardiovascular morbidity and mortality. While minor gastrointestinal side effects are quite common with the use of sevelamer, more serious gastrointestinal toxicities have only rarely been reported. Case-diagnosis/treatment We report a pediatric patient on maintenance dialysis receiving sevelamer hydrochloride who developed severe abdominal pain and a high-grade stricture of the sigmoid colon. The patient underwent exploratory laparotomy, resulting in a partial colectomy and colostomy. Histopathologic examination showed colonic mucosal injury and characteristic 'fish-scale'-like sevelamer hydrochloride crystals within the mucosa. Conclusions Whether the sevelamer crystals were causal, contributory or purely incidental remains to be clearly elucidated. However, our case raises sufficient concern to warrant additional investigation into whether there is a causal relationship between sevelamer use and intestinal mucosal injury., Author(s): Joseph Kim[sup.1] , Kristin Olson[sup.1] , Lavjay Butani[sup.1] Author Affiliations: (1) Section of Pediatric Nephrology, Department of Pediatrics , University of California Davis Medical Center, 2516 Stockton Boulevard, 95817, [...]

Abstract Patients with chronic proteinuric nephropathies are at high risk of developing progressive renal insufficiency. here are limited controlled data on the efficacy of potentially toxic immunosuppressive therapies for many [...]

Byline: Kiarash Shahlaie (1), Andrew Fox (1), Lavjay Butani (2), James E. Boggan (1,3) Keywords: Epidural hematoma; Chronic renal failure; Spontaneous; Intracranial Abstract: Spontaneous epidural hemorrhage has been described in a variety of clinical scenarios, including chronic renal failure (CRF). During hemodialysis, patients with CRF rarely develop spinal or cranial epidural hematomas. Such hemorrhages have been attributed to intracranial pressure fluctuations during hemodialysis, heparin administration, uremic-platelet syndrome, or hypertension. Although the exact pathophysiology is not understood, this rare consequence of hemodialysis has been well documented in the literature. Hemorrhage in the absence of hemodialysis therapy in a CRF patient, however, has not been previously reported. We report a 16-year-old boy with no history of trauma who woke in the morning with severe headache and resultant neurological deterioration. He was found to have a large left tempero-parietal epidural hematoma and underwent urgent surgical evacuation. Postoperatively he developed a contralateral extra-axial hematoma that did not require surgical intervention. He recovered completely, with no significant neurological deficit. This unique presentation of spontaneous intracranial epidural hemorrhage in an adolescent not receiving hemodialysis highlights a rare, but serious, complication of CRF. Author Affiliation: (1) Department of Neurological Surgery, Davis Medical Center, University of California, Sacramento, California, USA (2) Department of Pediatrics, Davis Medical Center, University of California, Sacramento, California, USA (3) Department of Neurological Surgery, School of Medicine, University of California, Davis, 4860 Y Street, Suite 3740, Sacramento, CA 95817, USA Article History: Registration Date: 19/05/2004 Received Date: 03/02/2004 Accepted Date: 06/05/2004 Online Date: 05/08/2004

Byline: Lavjay Butani (1), Alok Kalia (2) Keywords: Urinary calcium excretion; Calcium to creatinine ratio; Nephrolithiasis; Osteoporosis; Hematuria; Osteopenia; Methodology Abstract: Idiopathic hypercalciuria is a common metabolic abnormality in children of all ages. There is evidence of an association of idiopathic hypercalciuria with nephrolithiasis, hematuria, and osteoporosis. However, much of this evidence is anecdotal and the precise role of hypercalciuria in the pathogenesis of these conditions is far from clear. Furthermore, the precise definition of idiopathic hypercalciuria has not yet been established. The methodologies for quantitating urinary calcium excretion have also not been standardized, adding another potential confounding factor to the accurate interpretation of urinary calcium excretion. Long-term studies on the natural history of unselected children with idiopathic hypercalciuria are needed to establish the true clinical significance of this condition. The focus of this review is to critically evaluate the methods currently being used to measure urinary calcium excretion in children and to assess the validity of existing criteria for diagnosing idiopathic hypercalciuria. Author Affiliation: (1) Section of Pediatric Nephrology, Department of Pediatrics, University of California Davis Medical Center, 2516 Stockton Boulevard, 3rd Floor, Sacramento, CA 95817, USA (2) Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas, USA Article History: Registration Date: 11/03/2004 Received Date: 25/07/2003 Accepted Date: 12/02/2004 Online Date: 31/03/2004

Byline: Lavjay Butani (1) Keywords: Anti-phospholipid syndrome; Rapidly progressive glomerulonephritis; Chronic renal failure; Thrombotic microangiopathy; Lupus anticoagulant Abstract: Primary anti-phospholipid syndrome (APS) is perceived to be an uncommon disorder, infrequently recognized as a cause of renal disease in childhood. While renal involvement in APS classically manifests as thrombotic events, other renal diseases associated with APS have been reported in adults, including membranous nephropathy and minimal change disease. We report our experience of caring for a child who presented with acute anuric renal failure due to anti-neutrophilic cytoplasmic antibody-negative rapidly progressive glomerulonephritis (RPGN), with concomitant thrombotic microangiopathy (TMA). Recognition of the APS as a cause of the patient&apos;s TMA facilitated institution of anticoagulation. Our patient&apos;s renal failure did not improve and the patient remained dependent on dialysis until he was successfully transplanted. The purpose of our report is to make health-care professionals aware of the previously unreported association of pauci-immune RPGN and APS in children early recognition of APS will allow initiation of anticoagulation to prevent recurrent thromboses and enable successful transplantation. Author Affiliation: (1) Section of Pediatric Nephrology, University of California Davis Medical Center, 2516 Stockton Boulevard, Ticon II, 3rd Floor, Sacramento, CA 95817, USA Article History: Registration Date: 26/03/2004 Received Date: 29/12/2003 Accepted Date: 10/03/2004 Online Date: 06/05/2004

Byline: Lavjay Butani (1), Martin S. Polinsky (1), Bruce A. Kaiser (1), H. J. Baluarte (1) Keywords: Key words: Peritoneal dialysis; Pleural effusion; Hemolytic uremic syndrome Abstract: Hemolytic uremic syndrome (HUS) is a leading cause of acute renal failure (ARF) in children, and one for which treatment with peritoneal dialysis (PD) is often necessary. Between January 1982 and December 1996, 176 children received PD for ARF at St. Christopher&apos;s Hospital for Children 34 (19%) of whom had HUS. Of these 34, 7 (20%) developed pleural effusions (PE) while receiving PD, whereas none of the remaining 142 children with other causes of ARF did so. The mean age of the 7 affected children was 5.2 (range 0.4--17) years none had heart failure or nephrotic syndrome, nor had any of them undergone thoracic surgery. PE were diagnosed by chest radiograph at an interval of 2 (range 1--3) days after starting PD. Thereafter, 4 (57%) patients were successfully maintained on a modified PD prescription 2 others were converted to hemodialysis and 1 to continuous venovenous hemodiafiltration. Although PE are a known complication of PD, none of the patients so treated for non-HUS related ARF developed them. Whether they represent a purely mechanical complication of PD, or are in some way attributable to HUS itself, is not entirely clear. Regardless, when children with HUS require PD, physicians should monitor for the development of this potential complication to minimize the risk of serious respiratory compromise. Author Affiliation: (1) Department of Pediatrics, Section of Pediatric Nephrology, St. Christopher&apos;s Hospital for Children, Erie Avenue at Front Street, Philadelphia, Pennsylvania 19134-1034, USA, US Article note: Received February 19, 1997 received in revised form and accepted May 27, 1998

Sirs, Idiopathic minimal-change nephrotic syndrome (NS) remains one of the commonest causes of NS in childhood, especially in the non-black population [1]. While transient microscopic hematuria has been described in [...]

Byline: Lavjay Butani (1) Keywords: Hemolytic uremic syndrome; Diarrhea; Clostridium difficile; Escherichia coli Author Affiliation: (1) Section of Pediatric Nephrology, University of California Davis Medical Center, 2516 Stockton Boulevard, Sacramento, CA 95817, USA Article History: Registration Date: 05/08/2004 Online Date: 21/10/2004