23 results on '"Pankaj Hari"'
Search Results
2. Short-term safety and efficacy of escalating doses of atorvastatin for dyslipidemia in children with predialysis chronic kidney disease stage 2–5
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Punitha Lakxmi Ramesh, Priyanka Khandelwal, R. Lakshmy, Aditi Sinha, Arvind Bagga, and Pankaj Hari
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Nephrology ,Pediatrics, Perinatology and Child Health - Published
- 2023
3. Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection
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Priyanka Khandelwal, Sudarsan Krishnasamy, Srinivasavaradan Govindarajan, Manish Kumar, Binata Marik, Aditi Sinha, Pankaj Hari, and Arvind Bagga
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Adolescent ,SARS-CoV-2 ,Coronavirus 19 ,Factor H ,COVID-19 ,Recurrence ,Renal Dialysis ,Nephrology ,Child, Preschool ,Complement Factor H ,Pediatrics, Perinatology and Child Health ,Humans ,Original Article ,Complement factor H related protein ,Thrombotic microangiopathy ,Child ,Alternative complement pathway ,Atypical Hemolytic Uremic Syndrome ,Autoantibodies - Abstract
Background The pathogenesis of autoantibody generation in anti-factor H (FH) antibody associated atypical hemolytic uremic syndrome (aHUS) is unknown and is perhaps triggered by an infectious or environmental agent. We observed an unusual increase of patients with anti-FH antibody associated aHUS coinciding with the second pandemic wave in New Delhi and suspected that SARS-CoV-2 infection might be a potential trigger. Methods We screened for SARS-CoV-2 infection using reverse transcriptase polymerase chain reaction (RT-PCR) and serology in 13 consecutive patients with anti-FH antibody associated aHUS during the past year in New Delhi. Results We report 5 patients, 4–13 years old, who presented with a febrile illness without respiratory symptoms during the second pandemic wave. Of these, 3 patients presented with a relapse 25–85 months following the initial episode of aHUS. SARS-CoV-2 was detected by RT-PCR in 1 patient and by serology in 4 patients (median titer 47.1 cut-off index). Patients had high titers of anti-FH antibodies (median 2,300 AU/ml). Genetic studies, done in 3 of the 5 patients, showed homozygous CFHR1 deletion without other significant genetic abnormalities. Specific management comprised plasma exchanges and oral prednisolone, combined with either cyclophosphamide or mycophenolate mofetil. At median follow-up of 3.3 months, the estimated glomerular filtration rate in 4 patients ranged from 62 to 110 ml/min/1.73 m2; one patient was dialysis-dependent. Conclusion Increased vigilance is required during the pandemic, especially in patients with anti-FH associated aHUS, who might relapse despite quiescent disease for a prolonged period. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information. Supplementary Information The online version contains supplementary material available at 10.1007/s00467-021-05390-4.
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- 2022
4. Variants in complement genes are uncommon in patients with anti-factor H autoantibody-associated atypical hemolytic uremic syndrome
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Priyanka Khandelwal, Aditi Joshi, Aradhana Mathur, Mamta Puraswani, Bahadur Singh Gurjar, Aditi Sinha, Pankaj Hari, Mohammed Faruq, and Arvind Bagga
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Nephrology ,Pediatrics, Perinatology and Child Health - Abstract
Coexisting genetic variants in patients with anti-factor H (FH)-associated atypical hemolytic uremic syndrome (aHUS) have implications for therapy. We estimated the prevalence of complement genetic variants in children with anti-FH aHUS from a prospective nationwide cohort and determined if significant genetic variants impact long-term kidney outcomes.Of 436 patients in the database, 77 consecutive patients, 21 with a relapse and 9 with kidney failure and/or death were included. Targeted sequencing, using a 27-gene panel including CFH, CFI, CFB, C3, CD46, PLG, DGKE, and THBD and multiplex ligation-dependent probe amplification of CFH-CFHR region, was performed. The adverse outcome was eGFR 30 ml/min/1.73 mPatients had high anti-FH titers 5670 (2177-13,545) AU/ml, relapsing course (42.1%), and adverse outcomes (19.6%). Variants, chiefly of unknown significance, were found in 7 (6.5%; 95% CI 3.1-13.2%); a pathogenic variant was found in one patient. Homozygous deletion of CFHR1 was present in 91.6% compared to 9.8% in 184 healthy controls. Plasma exchanges and immunosuppression showed a trend of improving outcomes, independent of genetic defects (HR 0.32; P = 0.070). Meta-analysis of 18 studies (384 patients) showed that the pooled prevalence of pathogenic and likely pathogenic variants was 3% (95% CI 0-8%). Of 37 total variants in the meta-analysis, 7 (18.9%) each were pathogenic and likely pathogenic; others were variants of unknown significance.Significant variants in complement regulatory genes are rare in patients with anti-FH-associated aHUS. Irrespective of genetic defects, plasma exchanges and immunosuppression showed a statistical trend to improved outcomes. A higher resolution version of the Graphical abstract is available as Supplementary information.
- Published
- 2023
5. SARS-CoV-2 infection in children with chronic kidney disease
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Sudarsan Krishnasamy, Rajni Gaind, Swarnim Swarnim, Kanika Kapoor, Megha Brijwal, Shobha Sharma, Mukta Mantan, Pankaj Hari, Kirtisudha Mishra, Arvind Bagga, Manish Kumar, Priyanka Khandelwal, and Aditi Sinha
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Nephrology ,medicine.medical_specialty ,Nephrotic Syndrome - Relapse ,medicine.medical_treatment ,Nephrotic syndrome ,urologic and male genital diseases ,Letter to the Editors ,Renal Dialysis ,Risk Factors ,Interquartile range ,Internal medicine ,Humans ,Medicine ,Hospital Mortality ,Renal Insufficiency, Chronic ,Child ,Children ,Dialysis ,Retrospective Studies ,SARS-CoV-2 ,business.industry ,Acute kidney injury ,COVID-19 ,Odds ratio ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Original Article ,Kidney replacement therapy ,business ,Kidney disease - Abstract
Background Information on the course of SARS-CoV-2 infection in children with chronic kidney disease (CKD) is limited. Methods We retrospectively reviewed the presentation and outcomes of SARS-CoV-2 infection in patients with CKD followed at any of the four pediatric nephrology centers in New Delhi from April 2020 to June 2021. Outcomes, including cardiopulmonary and renal complications, were reported in relation to underlying disease category and illness severity at presentation. Results Underlying illness in 88 patients included nephrotic syndrome (50%), other CKD stages 1–4 (18.2%), CKD 5D (17%), and CKD 5T (14.8%). Thirty-two of 61 patients with symptomatic COVID-19 and 9/27 asymptomatic patients were admitted for median 10 (interquartile range 7–15) days. Seventeen (19.3%) patients developed moderate or severe COVID-19. Systemic complications, observed in 30 (34.1%), included acute kidney injury (AKI, 34.2%), COVID-19 pneumonia (15.9%), unrelated pulmonary disease (2.3%), and shock (4.5%). Nineteen (21.6%) had severe complications (AKI stage 2–3, encephalopathy, respiratory failure, shock). Eight (11%) of twelve (16.4%) patients with severe AKI required dialysis. Three (3.4%) patients, two with steroid-resistant nephrotic syndrome in relapse and one with CKD 1–4, died due to respiratory failure. Univariate logistic regression indicated that patients presenting with nephrotic syndrome in relapse or moderate to severe COVID-19 were at risk of AKI (respective odds ratio, 95%CI: 3.62, 1.01–12.99; 4.58, 1.06–19.86) and/or severe complications (respective odds ratio, 95%CI: 5.92, 1.99–17.66; 61.2, 6.99–536.01). Conclusions Children with CKD presenting with moderate-to-severe COVID-19 or in nephrotic syndrome relapse are at risk of severe complications, including severe AKI and mortality. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information Supplementary Information The online version contains supplementary material available at 10.1007/s00467-021-05218-1.
- Published
- 2021
6. Importance of clinical practice guidelines to practicing pediatric nephrologists and IPNA survey
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Pankaj Hari, Koichi Nakanishi, Dieter Haffner, Paula A. Coccia, Olivia Boyer, Arvind Bagga, Melvin Bonilla-Felix, Hong Xu, Khalid Alhasan, Giovanni Montini, Susan Samuel, Ali Duzova, and Ill So Ha
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medicine.medical_specialty ,Attitude of Health Personnel ,business.industry ,030232 urology & nephrology ,Guideline ,030204 cardiovascular system & hematology ,Pediatrics ,Scientific evidence ,Nephrologists ,Clinical Practice ,03 medical and health sciences ,Cross-Sectional Studies ,0302 clinical medicine ,Trustworthiness ,Nephrology ,Surveys and Questionnaires ,Family medicine ,Practice Guidelines as Topic ,Pediatrics, Perinatology and Child Health ,Humans ,Medicine ,business ,Regional differences ,Pediatric population - Abstract
Clinical practice guidelines (CPGs) are systematically developed statements backed by scientific evidence to assist practitioners in management in clinical practice. An international cross-sectional survey was conducted by the IPNA to examine the perceptions of pediatric nephrologists on guidelines and their usage and to identify important diseases for future clinical practice guidelines (CPGs). The survey found that the majority of pediatric nephrologists find CPGs useful in clinical practice and admitted to using them most of the time. Developing CPGs is challenging and there are standards available to develop trustworthy guidelines. While evidence-based global guidelines are ideal, pediatric nephrologists expressed the desire that they address regional differences. Most respondents (89.2%) to the survey agreed that adult guidelines did not cover the pediatric perspective adequately and 71.4% opined that consensus-based pediatric guidelines can be developed when evidence for the pediatric population is lacking. The development of high-quality practice guidelines requires substantial resources and may not be feasible in resource-poor countries. Adaptation of an existing guideline has been suggested as an alternative and the ADAPTE collaboration provides a systematic approach to adapting guidelines. Several diseases where pediatric guidelines are needed as a priority including IgA and C3 glomerulopathy were identified in the survey. Implementation of guideline-based care is challenging and the survey found that lack of availability of guidelines (43%) and resources (22.8%) are important reasons for poor implementation in lower-middle and low-income countries. Perceived complexity of guidelines, physician attitudes, and lack of training also contribute to non-adherence to guidelines.
- Published
- 2021
7. Phenotypic variability in distal acidification defects associated with WDR72 mutations
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Pankaj Hari, Priyanka Khandelwal, Vijay Prakash Mathur, Arvind Bagga, Aditi Sinha, Thenral S Geetha, Mahesh, Sandhya Nair, and Sumantra Raut
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Vacuolar Proton-Translocating ATPases ,medicine.medical_specialty ,030232 urology & nephrology ,030204 cardiovascular system & hematology ,Gastroenterology ,Renal tubular acidosis ,03 medical and health sciences ,0302 clinical medicine ,Distal renal tubular acidosis ,Polyuria ,Furosemide ,Internal medicine ,medicine ,Humans ,Amelogenesis imperfecta ,Hypercalciuria ,business.industry ,Proteins ,Fanconi syndrome ,Forkhead Transcription Factors ,Metabolic acidosis ,Acidosis, Renal Tubular ,Hydrogen-Ion Concentration ,medicine.disease ,Bicarbonates ,Biological Variation, Population ,Nephrology ,Fludrocortisone ,Mutation ,Pediatrics, Perinatology and Child Health ,Nephrocalcinosis ,medicine.symptom ,Acidosis ,business - Abstract
Distal renal tubular acidosis (RTA) is typically caused by defects in ATP6V0A4, ATP6V1B1, and SLC4A1, accounting for 60–80% of patients. Genes recently implicated include FOXI1, ATP6V1C2, and WDR72, of which WDR72 is associated with dental enamel defects. We describe 4 patients, from three unrelated consanguineous families, with RTA and amelogenesis imperfecta. Distal tubular acidification was evaluated by furosemide-fludrocortisone test, urine-to-blood PCO2 gradient and fractional excretion of bicarbonate. Exome sequencing was performed using a panel of genes implicated in human disease. Patients had polyuria, hypokalemia, hypercalciuria, and nephrocalcinosis, but metabolic acidosis varied in severity. Although all patients acidified urine to pH
- Published
- 2020
8. Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis
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Geetika Singh, Pankaj Hari, S. P. Bhardwaj, Priyanka Khandelwal, Aditi Sinha, and Arvind Bagga
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Nephrology ,medicine.medical_specialty ,Cyclophosphamide ,Glomerulonephritis, Membranoproliferative ,C3 Glomerulonephritis ,030232 urology & nephrology ,030204 cardiovascular system & hematology ,Kidney ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Glomerulopathy ,Internal medicine ,Membranoproliferative glomerulonephritis ,Humans ,Medicine ,Dense Deposit Disease ,Rapidly progressive glomerulonephritis ,Child ,Retrospective Studies ,business.industry ,medicine.disease ,Calcineurin ,Pediatrics, Perinatology and Child Health ,business ,medicine.drug - Abstract
Data on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in children are limited.In this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged18-years with C3 glomerulopathy and IC-MPGN. Initial immunosuppression comprised prednisolone, mycophenolate mofetil (n = 51), tacrolimus (n = 11), and/or IV cyclophosphamide (n = 20). Clinicopathological features, response to therapy, and adverse outcome (eGFRA total of 92 patients were classified as DDD (n = 48, 52.2%), C3GN (n = 26, 28.3%), and IC-MPGN (n = 18, 19.6%) by immunohistochemistry and electron microscopy; 8 patients with DDD were misclassified as IC-MPGN on immunofluorescence. At last follow-up (median 4.3 years), complete or partial remission occurred in 28.5, 36.1, and 16.7% patients with DDD, C3GN, and IC-MPGN, respectively. Serum albumin at onset 2.5 g/dL (HR = 0.29, P = 0.005) and persistently low serum C3 (HR = 0.34, P = 0.02) were associated with lack of remission. The 5-year kidney survival was 62.6, 85.5, and 88.5% in patients with DDD, C3GN, and IC-MPGN, respectively (log-rank, P = 0.006). Presentation as rapidly progressive GN (HR = 11.2, P 0.001), age 10 years at onset (HR = 4.0, P = 0.004), and DDD (HR = 4.2, P = 0.02) were independently associated with adverse outcome; achieving remission was protective (HR = 0.04; P 0.001).Outcome in patients with C3 glomerulopathy and IC-MPGN was unsatisfactory, and only a small proportion of patients achieved complete or partial remission. Patients with DDD were more likely to present with rapidly progressive GN and were at higher risk of adverse outcomes, including kidney failure.
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- 2020
9. Hemolytic uremic syndrome in a developing country: Consensus guidelines
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Ranjeet Thergaonkar, Anil Vasudevan, Sidharth Kumar Sethi, Marie-Agnès Dragon-Durey, Arvind Bagga, Kirtisudha Mishra, Saroj Kumar Patnaik, Aditi Sinha, Pankaj Hari, Jyoti Sharma, and Priyanka Khandelwal
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Nephrology ,medicine.medical_specialty ,Consensus ,Thrombotic microangiopathy ,Consensus Development Conferences as Topic ,030232 urology & nephrology ,India ,Developing country ,030204 cardiovascular system & hematology ,urologic and male genital diseases ,03 medical and health sciences ,0302 clinical medicine ,hemic and lymphatic diseases ,Internal medicine ,medicine ,Humans ,Plasma therapy ,Intensive care medicine ,Developing Countries ,Plasma Exchange ,Shiga-Toxigenic Escherichia coli ,business.industry ,Acute kidney injury ,Eculizumab ,medicine.disease ,Hemolytic-Uremic Syndrome ,Practice Guidelines as Topic ,Pediatrics, Perinatology and Child Health ,Research studies ,Differential diagnosis ,business ,medicine.drug - Abstract
Hemolytic uremic syndrome (HUS) is a leading cause of acute kidney injury in children. Although international guidelines emphasize comprehensive evaluation and treatment with eculizumab, access to diagnostic and therapeutic facilities is limited in most developing countries. The burden of Shiga toxin-associated HUS in India is unclear; school-going children show high prevalence of anti-factor H (FH) antibodies. The aim of the consensus meeting was to formulate guidelines for the diagnosis and management of HUS in children, specific to the needs of the country. Four workgroups performed literature review and graded research studies addressing (i) investigations, biopsy, genetics, and differential diagnosis; (ii) Shiga toxin, pneumococcal, and infection-associated HUS; (iii) atypical HUS; and (iv) complement blockade. Consensus statements developed by the workgroups were discussed during a consensus meeting in March 2017. An algorithm for classification and evaluation was developed. The management of Shiga toxin-associated HUS is supportive; prompt plasma exchanges (PEX) is the chief therapy in patients with atypical HUS. Experts recommend that patients with anti-FH-associated HUS be managed with a combination of PEX and immunosuppressive medications. Indications for eculizumab include incomplete remission with plasma therapy, life-threatening features, complications of PEX or vascular access, inherited defects in complement regulation, and recurrence of HUS in allografts. Priorities for capacity building in regional and national laboratories are highlighted. Limited diagnostic capabilities and lack of access to eculizumab prevent the implementation of international guidelines for HUS in most developing countries. We propose practice guidelines for India, which will perhaps be applicable to other developing countries.
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- 2019
10. Effect of atorvastatin on dyslipidemia and carotid intima-media thickness in children with refractory nephrotic syndrome: a randomized controlled trial
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Pankaj Hari, Amit Kumar Satpathy, Ramakrishnan Lakshmy, Ranjeet Thergaonkar, Priyanka Khandelwal, Arvind Bagga, Aditi Sinha, and Smriti Hari
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Male ,medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,Atorvastatin ,030232 urology & nephrology ,Serum albumin ,Serum Albumin, Human ,030204 cardiovascular system & hematology ,Placebo ,Carotid Intima-Media Thickness ,Gastroenterology ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Double-Blind Method ,Internal medicine ,Hyperlipidemia ,Humans ,Medicine ,Prospective Studies ,cardiovascular diseases ,Child ,Dyslipidemias ,Creatinine ,biology ,business.industry ,Cholesterol, LDL ,Atherosclerosis ,medicine.disease ,Carotid Arteries ,Treatment Outcome ,Intima-media thickness ,chemistry ,Nephrology ,Pediatrics, Perinatology and Child Health ,biology.protein ,Female ,lipids (amino acids, peptides, and proteins) ,Hydroxymethylglutaryl-CoA Reductase Inhibitors ,business ,Nephrotic syndrome ,Dyslipidemia ,medicine.drug - Abstract
Dyslipidemia is an important cardiovascular risk factor in steroid-resistant nephrotic syndrome (SRNS). Efficacy of statins for treatment of hyperlipidemia in children with SRNS is unclear. This prospective, randomized, double-blind, placebo-controlled, parallel-group clinical trial enrolled 30 patients with SRNS, aged 5–18 years, with serum low-density lipoprotein cholesterol (LDL-C) levels between 130 and 300 mg/dl, to receive a fixed dose of atorvastatin (n = 15, 10 mg/d) or placebo (n = 15) by block randomization in a 1:1 ratio. Primary outcome was change in serum LDL-C at 12 months. Change in levels of other lipid fractions, carotid intima-media thickness (cIMT), flow-mediated dilation (FMD) of the brachial artery, and adverse events were also evaluated. At the end of 12 months, atorvastatin was not superior to placebo in reducing plasma LDL-C levels, median percentage reduction 15.8% and 9.5% respectively, in atorvastatin and placebo arms (n = 14 in each; P = 0.40). Apolipoprotein B levels significantly declined with atorvastatin in modified intention-to-treat analysis (P = 0.01) but not in the per-protocol analysis. There was no significant effect on other lipid fractions, cIMT and FMD. Adverse events were similar between groups. Change in serum albumin was negatively associated with change in serum LDL-C, very low-density lipoprotein cholesterol, total cholesterol, triglyceride, and apolipoprotein B (P
- Published
- 2018
11. Dyslipidemia, carotid intima-media thickness and endothelial dysfunction in children with chronic kidney disease
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Aditi Sinha, Pankaj Hari, Smriti Hari, Arvind Bagga, Vijaya Murugan, Priyanka Khandelwal, and Ramakrishnan Lakshmy
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Male ,Nephrology ,medicine.medical_specialty ,030232 urology & nephrology ,Renal function ,030204 cardiovascular system & hematology ,Carotid Intima-Media Thickness ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine.artery ,Prevalence ,Humans ,Medicine ,Vascular Diseases ,cardiovascular diseases ,Renal Insufficiency, Chronic ,Brachial artery ,Endothelial dysfunction ,Child ,Dyslipidemias ,medicine.diagnostic_test ,business.industry ,medicine.disease ,Vasodilation ,Cross-Sectional Studies ,Intima-media thickness ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology ,Female ,Endothelium, Vascular ,business ,Lipid profile ,Dyslipidemia ,Kidney disease - Abstract
Chronic kidney disease (CKD) predisposes to accelerated atherosclerosis that is measured by carotid artery intima-media thickness (cIMT) and brachial artery flow-mediated dilation (FMD). Information on the association of these parameters with dyslipidemia in pre-dialysis pediatric CKD is limited. Eighty patients aged 9.9 ± 3.2 years, with estimated glomerular filtration rate of 38.8 ± 10.8 ml/1.73 m2/min, and 42 pediatric controls underwent cross-sectional analysis of lipid profile, cIMT, and brachial artery FMD. Significant differences in these parameters between patients and controls were analyzed using Student’s t test. Predictors of cIMT and dyslipidemia were assessed using linear and logistic regression respectively. Patients had elevated blood levels of triglyceride and of total and LDL cholesterol than controls (P ≤ 0.001); 73.8 % were dyslipidemic. Mean cIMT was higher (0.421 ± 0.054 mm vs 0.388 ± 0.036 mm, P = 0.001) and brachial artery FMD was reduced (10.6 ± 4.9 % vs 18.9 ± 4.1 %, P
- Published
- 2016
12. Incomplete penetrance of CD46 mutation causing familial atypical hemolytic uremic syndrome
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Pankaj Hari, Priyanka Khandelwal, Hae Il Cheong, Divya Bhatia, Arvind Bagga, and Aditi Sinha
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Male ,viruses ,Penetrance ,medicine.disease_cause ,Asymptomatic ,Membrane Cofactor Protein ,Atypical hemolytic uremic syndrome ,medicine ,Humans ,Sibling ,Child ,Index case ,Atypical Hemolytic Uremic Syndrome ,Genetics ,Mutation ,Familial Atypical Hemolytic Uremic Syndrome ,CD46 ,business.industry ,Siblings ,Sequence Analysis, DNA ,Flow Cytometry ,medicine.disease ,female genital diseases and pregnancy complications ,Nephrology ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,medicine.symptom ,business - Abstract
Hemolytic uremic syndrome (HUS) secondary to homozygous mutations in CD46 is uncommon. While heterozygous individuals may remain asymptomatic, homozygous mutations with severely depleted CD46 surface expression without disease manifestation is rare.We report on two siblings with features suggestive of hemolytic uremic syndrome. Estimation of CD46 expression by flow cytometry and gene sequencing were performed in members of this family.Three siblings, two of whom were symptomatic, had markedly decreased (10%) cell surface expression of CD46 and homozygous splice site mutation (IVS2 + 2 T G) in the CD46 gene; the other 10-year-old sibling was asymptomatic. The illness was preceded by dengue shock syndrome in the index case. Both parents and two other siblings were heterozygous for this CD46 mutation.Homozygous IVS2 + 2 T G mutation in CD46 gene, similar to heterozygous mutation, may be clinically silent at least during childhood. The role of antecedent infections in triggering the disease requires further examination.
- Published
- 2015
13. Effect of plasma exchange and immunosuppressive medications on antibody titers and outcome in anti-complement factor H antibody-associated hemolytic uremic syndrome
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Pankaj Hari, Savita Saini, Marie-Agnes Dragon Durey, Aditi Sinha, Priyanka Khandelwal, Arvind Bagga, and Aarti Gupta
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Male ,Nephrology ,medicine.medical_specialty ,Thrombotic microangiopathy ,medicine.medical_treatment ,Internal medicine ,medicine ,Humans ,Child ,Autoantibodies ,Retrospective Studies ,Plasma Exchange ,biology ,business.industry ,Acute kidney injury ,Antibody titer ,Immunosuppression ,medicine.disease ,Combined Modality Therapy ,Treatment Outcome ,Complement Factor H ,Factor H ,Hemolytic-Uremic Syndrome ,Pediatrics, Perinatology and Child Health ,Immunology ,biology.protein ,Female ,Rituximab ,Antibody ,business ,Immunosuppressive Agents ,medicine.drug - Abstract
Anti-complement factor H (anti-CFH) antibody-associated hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury in Indian children. While management comprises plasma exchange and immunosuppression, information on the impact on serial antibody titers and outcomes is limited.This retrospective study included 45 patients with anti-CFH-associated HUS who were followed for ≥12 months. Following the initial plasma exchange sessions, patients received prednisolone and either intravenous (IV) cyclophosphamide (n = 31) or IV rituximab (n = 14), followed by maintenance immunosuppression.The median anti-CFH antibody titers fell from 3,215.5 [interquartile range (IQR) 1,977.9-8,453.9 to 414.6 (IQR 251.6-1,368.2) AU/ml with plasma exchange therapy (P 0.0001), and the decline was similar with three, five, or seven plasma exchange sessions (P = 0.08). Serial anti-CFH titers were similar in patients receiving IV cyclophosphamide- and rituximab-based regimens during the 12-month follow-up (P = 0.63). Renal outcomes and relapse frequencies at the 15.4-month follow-up were comparable. Seven patients relapsed 6.5 (IQR 2.2-12.3) months from treatment onset. Patients with relapse had higher antibody titers during remission (P = 0.017). Titers of ≥1,300 AU/ml at 6 months predicted subsequent relapses.Our patients with anti-CFH antibody-associated HUS showed a significant fall in antibody titers following daily plasma exchange sessions. Therapy with cyclophosphamide- or rituximab-based regimens was associated with similar outcomes and a comparable decline in antibody titers.
- Published
- 2014
14. Antibiotic prophylaxis in the management of vesicoureteric reflux: a randomized double-blind placebo-controlled trial
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Aditi Sinha, Smriti Hari, Ravindra Mohan Pandey, Arvind Bagga, Arti Kapil, Rajesh Kumar, and Pankaj Hari
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Male ,medicine.medical_specialty ,Anti-Infective Agents, Urinary ,Urology ,Placebo-controlled study ,urologic and male genital diseases ,Placebo ,Chemoprevention ,law.invention ,Double-Blind Method ,Randomized controlled trial ,law ,Internal medicine ,Trimethoprim, Sulfamethoxazole Drug Combination ,medicine ,Humans ,Antibiotic prophylaxis ,Child ,DMSA scan ,Vesico-Ureteral Reflux ,business.industry ,Sulfamethoxazole ,Hazard ratio ,Infant ,bacterial infections and mycoses ,Trimethoprim ,female genital diseases and pregnancy complications ,Nephrology ,Child, Preschool ,Urinary Tract Infections ,Pediatrics, Perinatology and Child Health ,Female ,business ,medicine.drug - Abstract
The benefits of long-term low-dose antibiotics in preventing urinary tract infection (UTI) and renal damage in children with primary vesicoureteric reflux (VUR) are unclear. Children aged between 1 and 12 years with VUR grade I–IV and a microbiologically proven UTI were randomized into two groups to receive either antibiotic prophylaxis [2 mg/kg trimethoprim + sulfamethoxazole (TMP-SMX)] daily or placebo, respectively, for 12 months. Primary outcome was microbiologically confirmed symptomatic UTI. Intention-to-treat analysis using time-to-event data was performed. A total of 93 children (66.7 % boys) with a median age of 4.6 years were enrolled in this study; VUR grade III–IV was present in 73.1 % of these children. At least one symptomatic UTI occurred in ten (21.3 %) patients receiving antibiotic prophylaxis and in three (6.5 %) patients receiving placebo [hazard ratio in antibiotic group 3.9; 95 % confidence interval (CI) 1– 14; log rank test P = 0.02). Compared to the group receiving placebo, the antibiotic group had a 14.8 % increased risk for developing UTI (95 % CI 1–28; P = 0.03). Of the total number of episodes of UTI, 58.3 % of those in the antibiotic group were caused by TMP-SMX-resistant bacteria compared to 20 % in the placebo group (P = 0.15). A renal scan at 12 months revealed that six of 37 (16.2 %) patients in the antibiotic group and seven of 43 (16.3 %) patients in the placebo group had new or worsening of pre-existing scar. Long-term antibiotic prophylaxis with TMP-SMX is associated with increased risk of symptomatic UTI compared to placebo in children with grade I–IV VUR.
- Published
- 2014
15. Vitamin D insufficiency and effect of cholecalciferol in children with chronic kidney disease
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Puneet Mahajan, Arvind Bagga, Pankaj Hari, Ashima Gulati, Smriti Hari, and Nandita Gupta
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Male ,Nephrology ,Vitamin ,medicine.medical_specialty ,Time Factors ,Adolescent ,India ,Renal function ,Parathyroid hormone ,Gastroenterology ,chemistry.chemical_compound ,Internal medicine ,Prevalence ,medicine ,Vitamin D and neurology ,Humans ,Prospective Studies ,Child ,Calcifediol ,Cholecalciferol ,Hyperparathyroidism ,business.industry ,Vitamins ,Vitamin D Deficiency ,medicine.disease ,Treatment Outcome ,Endocrinology ,chemistry ,Parathyroid Hormone ,Child, Preschool ,Chronic Disease ,Pediatrics, Perinatology and Child Health ,Female ,Hyperparathyroidism, Secondary ,Kidney Diseases ,business ,Biomarkers ,Kidney disease - Abstract
Vitamin D insufficiency is common in patients with chronic kidney disease (CKD) and may contribute to mineral bone disease. In a prospective interventional study, we estimated the prevalence of vitamin D insufficiency (serum 25-hydroxyvitamin D3 [25OHD]30 ng/ml), and examined the effect of high-dose (600,000 IU) cholecalciferol supplementation after 6 weeks on serum 25OHD and parathyroid hormone (PTH) levels in children with CKD stages 2-4. Forty-two children (86% boys) with a mean age of 7.7 ± 3.8 (range 2--5) years were studied. Thirty-seven children (82.1%) had vitamin D insufficiency; 18 (42.8%) had 25OHD16 ng/ml. The median 25OHD increased significantly from 16.7 (95% CI 11.3, 19.8) to 46.2 (34.5, 44.6) ng/ml in patients with vitamin D insufficiency (P0.001). The median PTH decreased significantly from 51.3 (95% CI 46.7, 71.5) to 37.1 (29.0, 54.6) pg/ml (P = 0.003). Nineteen patients (47.5%) had30% reduction in the PTH after supplementation. Serum calcium, phosphorus, and estimated GFR did not change significantly. We conclude that vitamin D insufficiency is highly prevalent in children with CKD stages 2-4. High-dose cholecalciferol is safe and effective in correcting vitamin D insufficiency and results in a significant reduction in PTH levels in vitamin D-insufficient children.
- Published
- 2010
16. Vitamin A responsive night blindness in Dent’s disease
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Sidharth Kumar Sethi, Pankaj Hari, Arvind Bagga, Madhulika Kabra, and Michael Ludwig
- Subjects
Male ,medicine.medical_specialty ,Hypophosphatemia ,Hypercalciuria ,urologic and male genital diseases ,Nyctalopia ,Renal tubular dysfunction ,Polyuria ,Chloride Channels ,Night Blindness ,Internal medicine ,medicine ,Humans ,Vitamin A ,Dent's disease ,biology ,business.industry ,CLCN5 ,Infant ,Syndrome ,medicine.disease ,female genital diseases and pregnancy complications ,Proteinuria ,Endocrinology ,Nephrology ,Child, Preschool ,Mutation ,Pediatrics, Perinatology and Child Health ,biology.protein ,medicine.symptom ,Nephrocalcinosis ,business ,Polydipsia ,Thirst ,Rickets - Abstract
Dent's disease is an X-linked renal tubular disorder characterized by low molecular weight proteinuria, hypercalciuria and nephrocalcinosis or nephrolithiasis. The disease is caused by mutations in a renal chloride channel gene, CLCN5. We report on three boys, of Indian origin, with Dent's disease that presented at an early age (1-4 years), with polyuria, polydipsia, salt craving, recurrent vitamin A-responsive night blindness, hypophosphataemic rickets, hypercalciuria and low molecular weight proteinuria. All these patients were found to have novel mutations in the CLCN5 gene.
- Published
- 2009
17. Efficacy of zinc supplements in reducing relapses in steroid-sensitive nephrotic syndrome
- Author
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Shinjini Bhatnagar, Pankaj Hari, Shina Menon, Arvind Bagga, Sasi Arun, and Savita Saini
- Subjects
Male ,Nephrology ,medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,Prednisolone ,Placebo ,Gastroenterology ,law.invention ,Double-Blind Method ,Randomized controlled trial ,Recurrence ,law ,Internal medicine ,medicine ,Humans ,Child ,Glucocorticoids ,Gastrointestinal tract ,business.industry ,Infant ,medicine.disease ,Zinc ,Dietary Reference Intake ,Child, Preschool ,Dietary Supplements ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,business ,Nephrotic syndrome ,Zinc Supplements ,medicine.drug - Abstract
Relapses in steroid-sensitive nephrotic syndrome (SSNS) often follow infections of the respiratory or gastrointestinal tract. Based on data that zinc supplements reduce the risk of infections, we examined the efficacy of such supplements in reducing relapse rates in these patients. Eighty-one patients with SSNS (1-16 years old) were stratified into frequent (n = 52) and infrequent (n = 29) relapsers and randomized to receive 12-months of therapy with the recommended dietary allowance of zinc (10 mg/day) (n = 40) or placebo (n = 41). Patients with frequent relapses also received long-term, alternate-day prednisolone. Subjects receiving zinc showed a 20% lower frequency of relapses, with 44.7% of the patients having sustained remission compared to 27.5% in the placebo group (P > 0.05). Patients with frequent relapses receiving zinc showed a 28% reduction in relapse rates and a significantly higher likelihood of sustained remission (P = 0.02). Findings from this double blind, randomized study suggest that zinc supplementation results in trends towards remission and reduced relapses, especially in patients with frequent relapses. Prospective, adequately powered studies are required for confirmation of these findings.
- Published
- 2009
18. Randomized cross-over trial comparing albumin and frusemide infusions in nephrotic syndrome
- Author
-
Pankaj Hari, Rajmohan Dharmaraj, and Arvind Bagga
- Subjects
medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,medicine.medical_treatment ,Natriuresis ,Urine ,Gastroenterology ,Furosemide ,Internal medicine ,Weight Loss ,medicine ,Edema ,Humans ,Hypoalbuminemia ,Child ,Diuretics ,Infusions, Intravenous ,Serum Albumin ,Osmole ,Cross-Over Studies ,business.industry ,medicine.disease ,Crossover study ,Free water clearance ,Treatment Outcome ,Nephrology ,Child, Preschool ,Anesthesia ,Pediatrics, Perinatology and Child Health ,Urine osmolality ,Drug Therapy, Combination ,Diuretic ,business ,Nephrotic syndrome - Abstract
The contribution of hypoalbuminemia to impaired diuretic responsiveness can be overcome by administering larger doses of loop diuretics. However, the clinical efficacy of the combination of loop-acting diuretics with human albumin remains controversial. In the study reported here, 16 children with nephrotic syndrome and refractory edema were randomized in a cross-over trial to receive either the combination of 20% human albumin and frusemide infusion (HA+FU infusion group) or frusemide infusion alone (FU infusion group). At the end of study, median urine volume was 3.27 [95% confidence interval (CI) 2.04-4.50] ml/kg per hour in the HA+FU infusion group and 1.33 (95% CI 0.79-1.88) ml/kg per hour in the FU infusion group (P = 0.01); the median daily sodium excretion was 58 (95% CI 30-366) mEq and 30 (95% CI 10-122) mEq (P = 0.08), respectively The changes in other variables included weight loss [HA+FU 5.2% (95% CI 3.1-8.8); FU 0.8% (95% CI -1.9 to 4.1); P = 0.006]; urine osmolality [HA+FU 315 (95% CI 220-426) mOsm/kg; FU 368 (95% CI 318-446) mOsm/kg; P = 0.13]; osmolal clearance [HA+FU 1600 (95% CI 916-4140) ml/day; FU 880 (95% CI 510-2105) ml/day; P = 0.01; free water clearance [HA+FU -190 (95% CI -960 to 280) ml/day; FU -162 (95% CI -446 to -70) ml/day; P = 0.18]. The findings from this study suggest that the co-administration of albumin and frusemide infusions is more effective than the administration of frusemide infusion alone in inducing diuresis and natriuresis in patients with nephrotic syndrome.
- Published
- 2009
19. Bacteriuria and urinary tract infections in malnourished children
- Author
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Pankaj Hari, Arti Kapil, Arvind Bagga, R. N. Srivastava, M. K. Bhan, Partha Tripathi, and Vishal Jatana
- Subjects
Diarrhea ,Male ,medicine.medical_specialty ,Bacteriuria ,Fever ,Urinalysis ,Urinary system ,Population ,India ,Blood Sedimentation ,Urine ,urologic and male genital diseases ,Protein-Energy Malnutrition ,Asymptomatic ,Sex Factors ,Risk Factors ,Internal medicine ,medicine ,Humans ,Prospective Studies ,education ,Vesico-Ureteral Reflux ,education.field_of_study ,medicine.diagnostic_test ,business.industry ,Incidence (epidemiology) ,Malnutrition ,Infant ,nutritional and metabolic diseases ,bacterial infections and mycoses ,medicine.disease ,female genital diseases and pregnancy complications ,Anti-Bacterial Agents ,Blood Cell Count ,Surgery ,C-Reactive Protein ,Nephrology ,Case-Control Studies ,Child, Preschool ,Urinary Tract Infections ,Pediatrics, Perinatology and Child Health ,Female ,medicine.symptom ,business ,Acute-Phase Proteins - Abstract
We prospectively examined the incidence of bacteriuria in malnourished patients between 6 months and 5 years of age. For each patient, a normally nourished control matched for age, sex, and presence of fever and diarrhea was included. Of 112 patients (65 boys), 55 had moderate and 57 had severe malnutrition; 43 had diarrhea and 35 had fever. Clean-catch and suprapubic urine specimens were examined microscopically and cultured. Significant bacteriuria was found in 17 (15.2%) malnourished and 2 (1.8%) control subjects ( P
- Published
- 2003
20. Prolonged versus standard prednisolone therapy for initial episode of nephrotic syndrome
- Author
-
Avrind Bagga, Rajendra N. Srivastava, and Pankaj Hari
- Subjects
medicine.medical_specialty ,Nephrotic Syndrome ,Time Factors ,Subsequent Relapse ,medicine.drug_class ,medicine.medical_treatment ,law.invention ,Randomized controlled trial ,Recurrence ,law ,medicine ,Humans ,Child ,Glucocorticoids ,First episode ,Chemotherapy ,business.industry ,Infant ,medicine.disease ,Surgery ,Treatment Outcome ,Nephrology ,Child, Preschool ,Anesthesia ,Pediatrics, Perinatology and Child Health ,Prednisolone ,Prednisone ,Corticosteroid ,business ,Nephrotic syndrome ,Follow-Up Studies ,medicine.drug ,Kidney disease - Abstract
We have examined, in a prospective randomized controlled trial, the effect of 8- and 16-week initial steroid treatment on the course of idiopathic nephrotic syndrome (INS). Patients with a first episode of INS were randomized to receive standard 8-week prednisolone (2 mg/kg daily for 4 weeks, then 1.5 mg/kg on alternate days for 4 weeks) or prolonged 16-week prednisolone treatment (2 and 1.5 mg/kg daily each for 4 weeks, then 1.5 and 1 mg/kg on alternate days each for 4 weeks). Relapses were treated with prednisolone, 2 mg/kg daily for 2 weeks, then 1.5 mg/kg on alternate days for 4 weeks. Of 45 patients, 23 received standard therapy and 22 prolonged therapy. The mean duration of follow-up was 29.2 and 27.3 months in the standard and prolonged treatment groups, respectively. The time to first relapse was longer in the prolonged treatment (mean 222.2 days, median 120.0 days) than the standard group (mean 134.3 days, median 96.5 days). The percentage of patients with no relapse at 6 and 12 months after prednisolone withdrawal was 40.9% and 27.3% in the prolonged treatment and 21.7% and 8.7% in the standard groups, respectively. The inability to show statistically significant differences between the two groups was probably related to the small number of patients studied. Prolonged therapy did not affect the subsequent relapse rates and proportion of patients with frequent relapses and steroid dependence. The mean dose of prednisolone received, for the initial episode and relapses during the next year, was higher and associated with significant steroid toxicity in the prolonged treatment group. Our findings suggest that 16-week prednisolone treatment for the initial episode of INS may delay occurrence of the first relapse, but results in significant side effects. Prolongation of initial therapy may be useful in developing countries where frequent infections often induce early relapses.
- Published
- 1999
21. Elevated FGF-23 and parathormone in linear nevus sebaceous syndrome with resistant rickets
- Author
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Sidharth Kumar Sethi, Arvind Bagga, and Pankaj Hari
- Subjects
Fibroblast growth factor 23 ,medicine.medical_specialty ,Creatinine ,Adenoma ,business.industry ,Renal function ,Rickets ,medicine.disease ,Hypophosphatemic Rickets ,chemistry.chemical_compound ,Endocrinology ,chemistry ,Nephrology ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Nevus ,business ,Hypophosphatemia - Abstract
Sirs, Linear nevus sebaceous syndrome (LNSS) is a neuroectodermal disorder, which may be associated with hypophosphatemic rickets in childhood. The pathogenesis of hypophosphatemia is unclear and has been attributed to increased levels of fibroblast growth factor-23 (FGF-23) [1]. We report a patient with LNSS, hypophosphatemic rickets; and high blood levels of FGF-23, immunoglobulin (Ig) E, and parathormone (PTH). A 5-year-old boy, son a non-consanguineous marriage, was referred for increasing skin lesions on face and arms since birth and lower limb deformities. The child was developmentally normal for age. He had clinical and radiological features of rickets and multiple, linear, hyperpigmented plaques following the morphological pattern of Blaschko lines over the right side of his scalp, face, ear, and arms. Investigations showed normal blood levels of creatinine, calcium, and 25-hydroxyvitamin D; low phosphate (1.8–2.0 mg/dl); and raised alkaline phosphatase and parathormone (PTH). There was evidence of phosphate wasting, with low tubular maximum for phosphate reabsorption per glomerular filtration rate (TmP/GFR) (Table 1). Contrast-enhanced computed tomography (CT) of the head was normal. Following a diagnosis of LNSS with hypophosphatemic rickets, he was treated with phosphate supplements at a dose of 750–1,000 mg and alpha calcidiol 0.25 μg every day. The patient reported recently with poor compliance with medications, increasing bony deformities, and enlarging cutaneous nevi. Investigations showed similar blood findings, with low levels of phosphate and TmP/GFR and markedly raised PTH (Table 1). Serum IgE level was 179 IU/ml (normal
- Published
- 2010
22. Phrenic nerve palsy: a rare complication of indwelling subclavian vein catheter
- Author
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Sandeep Aggarwal, Pankaj Hari, S.N. Mehta, and Arvind Bagga
- Subjects
medicine.medical_specialty ,Diaphragmatic breathing ,Subclavian Vein ,Catheters, Indwelling ,Renal Dialysis ,medicine ,Paralysis ,Humans ,Phrenic nerve ,Palsy ,business.industry ,Peripheral Nervous System Diseases ,medicine.disease ,Surgery ,Phrenic Nerve ,Catheter ,Nephrology ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Female ,Radiography, Thoracic ,medicine.symptom ,business ,Complication ,Subclavian vein ,Kidney disease - Abstract
The use of central venous catheters as access for hemodialysis has become common in children with end-stage renal disease. Phrenic nerve palsy is an unusual complication of this procedure. We report a case of delayed right diaphragmatic palsy due to phrenic nerve damage resulting from an indwelling right subclavian catheter in a 3-year-old child.
- Published
- 2000
23. Neonatal renal failure due to obstructive candidal bezoars
- Author
-
Rajendra N. Srivastava, Anand Srivastava, Pankaj Hari, and Arun Gupta
- Subjects
Male ,Nephrology ,medicine.medical_specialty ,Chemotherapy ,Respiratory distress ,business.industry ,medicine.medical_treatment ,Urinary system ,Candidiasis ,Infant ,Acute Kidney Injury ,medicine.disease ,Surgery ,Amphotericin B ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Humans ,Bezoar ,business ,Complication ,Mycosis ,medicine.drug - Abstract
Acute renal failure (ARF) developed in a 7-week-old infant due to bilateral candidal bezoars (fungal balls) causing obstruction at the pelviureteric junction. The baby was born at term with an appropriate birthweight, and had been treated with broad-spectrum antibiotics for respiratory distress and septicemia during the 1st week of life. Recovery from ARF followed renal decompression with bilateral nephrostomy tube placement and parenteral administration of amphotericin B and 5-flucytosine.
- Published
- 1997
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