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Lavjay Butani

Publications and source records attributed to Lavjay Butani.

At least 19 recordsLinked to original sources
PubMed2006

End-stage renal disease as the presenting manifestation of renal systemic lupus erythematosus.

About one-half of all children with systemic lupus erythematosus have clinical evidence of renal disease at initial presentation, such as proteinuria and acute renal failure. Herein, we report a case of a teenager who presented with end-stage renal disease (ESRD) of uncertain etiology, and who was subsequently determined to have lupus. The purpose of this report is to make health-care professionals aware of this unusual presentation of renal lupus, which has never been reported before. Children presenting in ESRD should be worked-up for autoimmune diseases since the discovery of such a disease process may impact future decision-making, especially with respect to subsequent renal transplantation.

Adolescent↗
PubMed2006

Children presenting with end-stage renal disease of unexplained etiology: implications for disease recurrence after transplantation.

With longer graft and patient survival, recurrent disease is becoming recognized as an increasingly important contributor to long-term graft loss in renal transplant recipients. However, patients may present for the first time in end-stage renal disease (ESRD) leading to uncertainty as to their underlying diagnosis and the risk of recurrence. The purpose of this study was to describe the features of children who presented for the first time in ESRD and to determine the predictive value of investigations in differentiating diseases with and without a recurrence risk. From 7/99 to 11/04, 13 children presented to our center in ESRD. Their median age was 13.3 yr; 77% were male. The majority were hypertensive (77%) and oligoanuric (69%). All had proteinuria (median urine protein to creatinine ratio [Up/c] 7.0), and 92% had microhematuria. Only seven had small kidneys on ultrasound. All children underwent a serologic work-up and six (46%) were biopsied. Of the 13 children, seven had a glomerular disease; in five the diagnosis was made on biopsy, in one on serologic testing and one by family history. Of the remaining six children, three had non-glomerular diseases: obstructive uropathy in one and primary hyperoxaluria type 1 in two, and 3 had an unknown disease. When patients with glomerular diseases were compared with those with non-glomerular diseases, the two predictors for glomerular disease were a lower serum albumin (p = 0.004) and a higher serum bicarbonate level (p = 0.01). Comparing patients with and without a risk of recurrence, there were no differences between the two groups in any of their demographic, clinical, or biochemical parameters by analysis of variance (including serum albumin or proteinuria). In summary, the vast majority of children presenting in ESRD have hematuria and proteinuria, even with non-glomerular diseases. The significant overlap in clinical features between patients with and without a risk of recurrence emphasizes the need for all children presenting in ESRD to be evaluated extensively so that disease recurrence after transplantation can be anticipated or even prevented.

Adolescent↗
PubMed2005

Angiotensin blockade in children with chronic glomerulonephritis and heavy proteinuria.

Patients with chronic proteinuric nephropathies are at high risk of developing progressive renal insufficiency. There are limited controlled data on the efficacy of potentially toxic immunosuppressive therapies for many of these diseases such as immunoglobulin A nephropathy and idiopathic membranoproliferative glomerulonephritis. This limitation has not deterred healthcare providers from using such agents based on anecdotal experience. We report our experience taking care of three children with heavy proteinuria from chronic glomerular diseases. All were treated with a combination of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers without concomitant immunosuppression. All went into complete remission soon after starting therapy, allowing corticosteroid avoidance. The purpose of this report is to make healthcare professionals more aware of the potential success that can be achieved with this relatively nontoxic drug regimen. Larger controlled clinical trials using this strategy are needed to better evaluate the efficacy and safety of this approach in children with glomerular diseases.

Angiotensin II Type 1 Receptor Blockers↗
PubMed2005

Immunoglobulin E-mediated reactions to corticosteroids.

Allergic reactions to steroids are rare, but are becoming more commonly recognized by clinicians. Although many reactions are thought to be IgE mediated, most are adverse drug reactions caused by nonallergic mechanisms, some related to additives in the steroid preparation being administered. Provocative challenge with the suspected offending agent, serologic tests for allergen-specific IgE, and skin testing are the most reliable methods for assessing hypersensitivity to steroids, although none is infallible. Therapeutic options for patients with documented steroid allergy include the use of alternative steroid preparations, desensitization, and, possibly, the use of monoclonal anti-IgE antibodies.

Adrenal Cortex Hormones↗
PubMed2005

Cardiac tamponade in a pediatric renal transplant recipient on sirolimus therapy.

Because of its lack of nephrotoxicity, the use of sirolimus, as an immunosuppressive agent, has increased considerably in solid-organ transplant (Tx) recipients. With its increased use, Tx professionals are encountering a variety of previously unreported side-effects such as angioedema and interstitial pneumonitis. We describe here the case of a pediatric renal Tx recipient who, while receiving sirolimus, developed a large pericardial effusion requiring pericardiocentesis. An extensive workup for an infectious etiology was performed; the only positive result was isolation of adenovirus type 2 from the patient's stool specimen. Following sirolimus dose reduction this child's effusion stabilized and has not recurred. The purpose of this report is to advise health-care professionals caring for Tx recipients about this potentially life-threatening complication associated with sirolimus. The role of adenovirus, if any, in contributing to the development of our patient's pericardial effusion is discussed herein.

Cardiac Tamponade↗
PubMed2005

Prospective monitoring of lipid profiles in children receiving pravastatin preemptively after renal transplantation.

Hyperlipidemia is common after renal transplantation (Tx) and contributes to the increased cardiovascular morbidity seen in the post-transplant period. Limited data are available on the utility of the statins in children after renal Tx. This 12-month prospective study was undertaken to determine the efficacy of pravastatin in reducing dyslipidemia after renal Tx in children and to determine predictors of dyslipidemia after Tx. From August 2001 to April 2004, all 17 newly transplanted pediatric renal transplant recipients at our center were preemptively treated with pravastatin from the immediate post-transplant period. Fasting lipid profiles were obtained at 1, 3, 6 and 12 months after Tx. Trends in the lipid profile were analyzed using the repeated measures general linear model (GLM). A historical cohort of pediatric renal-transplant recipients not treated with pravastatin was used as the control population. The mixed effects GLM was used for multivariable logistic regression analyses to determine the independent effect of age, pretransplant cholesterol (Chol), body mass index (BMI), creatinine clearance (CrCl), and corticosteroid and tacrolimus doses on the development of dyslipidemia. The mean age of the children at Tx was 8.7 yr. The GLM analysis showed that with time, there was a significant decline in the total Chol, serum triglyceride (TG), LDL and also HDL-Chol (p-value <0.05 for each). Compared with the controls, the mean serum Chol was lower at all time points post-transplant in the treated patients. However, despite treatment, the prevalence of hypercholesterolemia increased from 31% pretransplant to 53% at 1-month, but declined thereafter to 6% at 3 and 6 months and 0% at 1 yr. Multivariable regression analyses showed the prednisone dose, pretransplant Chol and age to be the most important risk factors for the development of dyslipidemia. No child developed complications related to therapy. In summary, pravastatin is safe in the post-transplant period in children and reduces serum Chol, LDL-Chol and TG. An unexpected finding in our study was the decline in HDL-Chol after Tx. Whether the preemptive use of the statins will result in lower cardiovascular morbidity, especially considering the concomitant reduction in HDL-Chol remains to be determined.

Body Mass Index↗
PubMed2005

Assessment of pretransplant inflammation in pediatric renal allograft recipients.

Pretransplant (Tx) inflammation is linked to adverse outcomes in adult Tx recipients but no such data exist for children. Our study evaluated the predictive value of three pre-Tx inflammatory markers: serum C-reactive protein (CRP), Neopterin (Neo) and interleukin (IL) 12, in determining outcome. Pre-Tx serum on 51 children (mean age 11 years) transplanted between 1985 and 2000 was analyzed. Data on other variables were abstracted from patient records. Primary end-points were graft survival and acute rejection (AR). Kaplan-Meier and log-rank statistics compared endpoints in patients at different quartiles for each marker. Cox regression analysis was used to determine the independent effect of the markers on the end-points. The mean CRP, Neo, and IL-12 were 1.3 mg/l, 1.78 ng/ml, and 123 pg/ml, respectively. The mean CRP, Neo, and IL-12 were not different between the patients with and without AR or graft loss (P > 0.4 for all). Neither rejection-free survival nor graft survival was affected by CRP, Neo, or IL-12 quartiles. Cox-regression analysis demonstrated no predictive value of any marker on outcome. Unlike adults, a single pre-Tx determination of inflammatory markers was not predictive of AR or graft loss in children, indicating that the pathogenesis of AR may be different in children.

Adolescent↗
PubMed2004

Investigation of pediatric renal transplant recipients with heavy proteinuria after sirolimus rescue.

BACKGROUND: Little is known about the incidence and evolution of proteinuria as a complication of sirolimus rescue in children. This study describes pediatric renal transplant (Tx) recipients who were treated with sirolimus and who developed heavy proteinuria. Risk factors for the development of proteinuria and its time course are explored. METHODS: Data at various time points after sirolimus introduction were abstracted from the records of children treated at the author's center. The repeated measures general linear model and the Student's paired t test were used to analyze changes in laboratory values over time. RESULTS: Of the 13 children on sirolimus, 12 developed heavy proteinuria after a mean interval of 1 month. The mean urine protein (Up)-to-creatinine (c) ratio increased from 1.1 to a peak value of 3.9 (P=0.003) at 4.6 months after the start of sirolimus. Although not statistically significant, children on no calcineurin inhibitor (CNI) had a greater increase in the Up/c than those on low-dose CNI. At last follow-up, with the use of angiotensin receptor blockers (ARB), the Up/c declined to 2.2. No predictors could be identified for the development of proteinuria. CONCLUSIONS: Heavy proteinuria is common after the use of sirolimus as rescue therapy in children with renal Tx. Whether this is attributable to a toxic effect of the sirolimus itself or to lower CNI exposure is uncertain. Early detection of proteinuria is important to enable prompt intervention. Most children have a reduction in their Up/c with the use of ARB and can therefore be continued on sirolimus.

Adolescent↗
PubMed2004

Spontaneous epidural hemorrhage in chronic renal failure. A case report and review.

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 temperoparietal 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.

Adolescent↗
PubMed2004

End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome.

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's TMA facilitated institution of anticoagulation. Our patient'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.

Antiphospholipid Syndrome↗
PubMed2004

Idiopathic hypercalciuria in children--how valid are the existing diagnostic criteria?

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.

Calcium↗
PubMed2004

Bilateral infundibulopelvic stenosis without renal insufficiency: is surgery necessary?

The following case describes the clinical course of a patient with bilateral infundibulopelvic stenosis from her initial presentation at age 2 through the age of 14 years. This condition is associated with hypoplasia of segments of the upper collecting system and is characterized by dilated calyces that drain through stenotic infundibulae. Our patient is unique in that, although her renal function has slowly deteriorated with time, she has a persistently non-obstructive pattern on dynamic imaging studies. Only a minority of patients reported in the literature with this condition progress to renal insufficiency or failure. Although some patients have undergone surgery for presumed obstruction, surgical intervention has no proven benefit. Patients are at risk for hyperfiltration injury and should be followed for the development of hypertension, proteinuria and renal insufficiency. Without evidence of obstruction, medical management remains the cornerstone of treatment.

Child, Preschool↗
PubMed2003

Thrombotic complications in childhood-onset idiopathic membranous nephropathy.

While the most common clinical feature of nephrotic syndrome is generalized edema, patients are at risk of developing other problems, such as bacterial infections, electrolyte abnormalities, and venous thromboses. Adults with membranous nephropathy appear to be at the greatest risk for developing thromboses, especially renal vein thrombosis. However, the same is not true for children with membranous nephropathy. A review of pediatric membranous nephropathy stated that renal vein thrombosis is unrecorded in childhood-onset membranous nephropathy. We present our experience in managing two children with idiopathic membranous nephropathy who developed venous thromboses. To our knowledge, this is the first report of pediatric patients with membranous nephropathy to develop a thromboembolic complication without evidence of predisposing factors or coagulation abnormalities. This report emphasizes the need for appropriate evaluation of patients with membranous nephropathy who develop signs and symptoms suggestive of arterial or venous occlusion in order to avoid missing this potentially life-threatening medical complication.

Adolescent↗