Publications by authors named "Bilal Aoun"

30 Publications

  • Page 1 of 1

Hemodiafiltration maintains a sustained improvement in blood pressure compared to conventional hemodialysis in children-the HDF, heart and height (3H) study.

Pediatr Nephrol 2021 Feb 24. Epub 2021 Feb 24.

University College London Great Ormond Street Hospital for Children and Institute of Child Health, London, UK.

Background: Hypertension is prevalent in children on dialysis and associated with cardiovascular disease. We studied the blood pressure (BP) trends and the evolution of BP over 1 year in children on conventional hemodialysis (HD) vs. hemodiafiltration (HDF).

Methods: This is a post hoc analysis of the "3H - HDF-Hearts-Height" dataset, a multicenter, parallel-arm observational study. Seventy-eight children on HD and 55 on HDF who had three 24-h ambulatory BP monitoring (ABPM) measures over 1 year were included. Mean arterial pressure (MAP) was calculated and hypertension defined as 24-h MAP standard deviation score (SDS) ≥95th percentile.

Results: Poor agreement between pre-dialysis systolic BP-SDS and 24-h MAP was found (mean difference - 0.6; 95% limits of agreement -4.9-3.8). At baseline, 82% on HD and 44% on HDF were hypertensive, with uncontrolled hypertension in 88% vs. 25% respectively; p < 0.001. At 12 months, children on HDF had consistently lower MAP-SDS compared to those on HD (p < 0.001). Over 1-year follow-up, the HD group had mean MAP-SDS increase of +0.98 (95%CI 0.77-1.20; p < 0.0001), whereas the HDF group had a non-significant increase of +0.15 (95%CI -0.10-0.40; p = 0.23). Significant predictors of MAP-SDS were dialysis modality (β = +0.83 [95%CI +0.51 - +1.15] HD vs. HDF, p < 0.0001) and higher inter-dialytic-weight-gain (IDWG)% (β = 0.13 [95%CI 0.06-0.19]; p = 0.0003).

Conclusions: Children on HD had a significant and sustained increase in BP over 1 year compared to a stable BP in those on HDF, despite an equivalent dialysis dose. Higher IDWG% was associated with higher 24-h MAP-SDS in both groups.
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http://dx.doi.org/10.1007/s00467-021-04930-2DOI Listing
February 2021

Kidney and Metabolic Phenotypes in Glycogen Storage Disease Type-I Patients.

Front Pediatr 2020 11;8:591. Epub 2020 Sep 11.

Inherited Metabolic Diseases Program, Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

A retrospective chart review of 32 GSD- I patients, followed at the American University of Beirut Medical Center, between 2007 and 2018 was conducted. Diagnosis was confirmed by enzymatic and/or genetic studies. Clinical presentation, growth, and kidney outcome were assessed. All patients were evaluated for body mass index, blood parameters of metabolic control including uric acid, alanine, lactic acid, and triglycerides in blood. Kidney evaluation included creatinine clearance, microalbuminuria, citraturia, and calciuria as well as urine microalbumin/creatinine ratio. Almost one third of GSD-I patients developed microalbuminuria. This was detected below 7 months of age in 36% of patients who required early treatment with ACEI with significant reduction in albuminuria. Kidney stones were present in 6% and were associated with hypercalciuria and hypocitraturia. Poor metabolic control reflected by hyperuricemia, lactic acidosis, and hyperalaninemia were noted only in patients who developed microalbuminuria. Glomerular injury may appear in early infancy in poorly controlled patients. Adequate metabolic control and ACEI therapy may improve kidney outcome in GSD I patients. Plasma alanine appears to be a promising and reliable marker reflecting metabolic control in GSD-I patients.
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http://dx.doi.org/10.3389/fped.2020.00591DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7518374PMC
September 2020

Infrequent tacrolimus-induced nephrotoxicity in French patients with steroid-dependent nephrotic syndrome.

Pediatr Nephrol 2019 12 13;34(12):2605-2608. Epub 2019 Sep 13.

Department of Pediatric Nephrology, Armand Trousseau Hospital, APHP, 26 Avenue du Docteur Arnold Netter, 75012, Paris, France.

Background: Chronic nephrotoxicity with potentially irreversible lesions is a major concern regarding calcineurin inhibitor (CNI) treatment in children with severe forms of idiopathic nephrotic syndrome (INS).

Case-diagnosis/treatment: We retrospectively included all children on CNI for steroid-dependent INS with a duration of CNI treatment of more than 1 year. Only patients in whom CNI could not be replaced by mycophenolate mofetil were included. All included patients underwent a kidney biopsy. All results were expressed as median and range. Twenty-one children (6 girls) were included. Age at disease onset was 49 (29-66) months and treatment duration on CNI was 30 (20-45) months. Age at kidney biopsy was 108 (78-170) months. Number of relapses was 7 (3-9) since disease onset. Serum creatinine level was transiently and moderately increased in two patients. Kidney biopsy revealed minimal change disease in 20/21 patients and focal segmental glomerulosclerosis in 1/21. Evidence for chronic CNI nephrotoxicity was found in one patient revealed by arteriolar hyalinosis and fibrosis in 50% of glomeruli.

Conclusions: CNI-induced chronic nephrotoxicity was infrequent. In patients who require long-term and/or high-dose CNI treatment, kidney biopsies might be useful to exclude chronic CNI-induced lesions.
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http://dx.doi.org/10.1007/s00467-019-04343-2DOI Listing
December 2019

Penile hair strangulation: A prodigious cause of pediatric agitation.

Clin Case Rep 2019 Jul 17;7(7):1452-1453. Epub 2019 May 17.

Division of Pediatric Nephrology Armand Trousseau University Hospital (APHP) Paris France.

Penile hair strangulation is secondary to a hair tourniquet effect. Albeit a rare presentation in pediatric emergency units, penile hair strangulation must be cautiously noted in any penile pain and edema, to avoid inevitable damage to external genitalia, including penile necrosis, urethrocutaneous fistula, and even amputation.
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http://dx.doi.org/10.1002/ccr3.2198DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6637350PMC
July 2019

Norovirus: a novel etiologic agent in hemolytic uremic syndrome in an infant.

BMC Nephrol 2019 07 5;20(1):247. Epub 2019 Jul 5.

Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, P.O.Box 11-0236/E29, Beirut, Lebanon.

Background: Hemolytic uremic syndrome is a rare thrombotic microangiopathy usually seen in infants and children below the age of 5 years. It usually follows a bout of bloody diarrhea caused by Shiga toxin producing E coli and is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. We report the first case of hemolytic uremic syndrome in an infant following Norovirus gastroenteritis.

Case Presentation: A nine-month-old male infant, was admitted with an 8-day history of watery, non-bloody diarrhea, vomiting and decreased oral intake. Physical exam revealed normal blood pressure, pallor and generalized edema. Laboratory findings were significant for microangiopathic hemolytic anemia, thrombocytopenia and azotemia. Stool studies with Multiplex Qualitative reverse transcriptase PCR were positive for Norovirus GI/G II. His clinical course was unusually severe, complicated by oligoanuria and worsening uremia requiring peritoneal dialysis but with eventual complete recovery.

Conclusions: To our knowledge this is the first case of Norovirus associated HUS in an infant. Given the ubiquity of this virus as a major cause of diarrhea, together with the increased availability of Multiplex Qualitative PCR in reference laboratories, it is quite possible that we shall be seeing more such cases in the future.
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http://dx.doi.org/10.1186/s12882-019-1427-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6612073PMC
July 2019

Practice patterns in ordering a voiding cystourethrogram for pediatric patients among different specialties in a Middle Eastern tertiary care center.

Urol Ann 2019 Apr-Jun;11(2):168-170

Division of Urology, American University of Beirut-Medical Center, Beirut, Lebanon.

Introduction: Voiding cystourethrogram (VCUG) is a very popular test performed to evaluate genitourinary tract anomalies. Nevertheless, this test can be overused and can lead to unnecessary patient discomfort, radiation exposure, and cost. We sought to study the practice patterns in ordering a VCUG in a Middle Eastern tertiary care center.

Methods: Over a period of 3 years, a retrospective analysis of all VCUG images done for pediatric patients in a single center was made. Further clinical details were extracted from the electronic health records. The specialty of an ordering physician and the reported indication for the procedure were noted. Indications for VCUG were recorded based on the AAP 2011 guidelines, NICE guidelines 2007, and ACR 2011 guidelines. Based on these criteria, patients were analyzed.

Results: A total of 92 VCUGs were evaluated. Of all VCUGs done, pediatricians ordered the most VCUGs (50/92), followed by pediatric infectious disease (16/92), pediatric nephrology (9/92), pediatric urology (7/92), adult urology (5/92), pediatric surgery (3/92), obstetrician-gynecologist (1/92), and emergency medicine (1/92). Properly indicated VCUGs were 50% by general pediatrics, 55% by pediatric infectious disease, 45% by pediatric nephrology, 40% by adult urology, 33% by pediatric surgery, and 100% by pediatric urology.

Conclusion: VCUG is utilized differently by different specialties. In some centers, adult specialties may order a pediatric VCUG. General pediatricians order VCUG the most with a tendency for misuse in up to 50%. Pediatric urology is not the most ordering specialty of VCUG; however, it utilizes it most appropriately. The noted practice patterns may be improved with awareness of the indications and limitations of the study and with proper referral.
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http://dx.doi.org/10.4103/UA.UA_124_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6476207PMC
May 2019

Effects of Hemodiafiltration versus Conventional Hemodialysis in Children with ESKD: The HDF, Heart and Height Study.

J Am Soc Nephrol 2019 04 7;30(4):678-691. Epub 2019 Mar 7.

Nephrology Unit, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

Background: Hypertension and cardiovascular disease are common in children undergoing dialysis. Studies suggest that hemodiafiltration (HDF) may reduce cardiovascular mortality in adults, but data for children are scarce.

Methods: The HDF, Heart and Height study is a nonrandomized observational study comparing outcomes on conventional hemodialysis (HD) versus postdilution online HDF in children. Primary outcome measures were annualized changes in carotid intima-media thickness (cIMT) SD score and height SD score.

Results: We enrolled 190 children from 28 centers; 78 on HD and 55 on HDF completed 1-year follow-up. The groups were comparable for age, dialysis vintage, access type, dialysis frequency, blood flow, and residual renal function. At 1 year, cIMT SD score increased significantly in children on HD but remained static in the HDF cohort. On propensity score analysis, HD was associated with a +0.47 higher annualized cIMT SD score compared with HDF. Height SD score increased in HDF but remained static in HD. Mean arterial pressure SD score increased with HD only. Factors associated with higher cIMT and mean arterial pressure SD-scores were HD group, higher ultrafiltration rate, and higher 2-microglobulin. The HDF cohort had lower 2-microglobulin, parathyroid hormone, and high-sensitivity C-reactive protein at 1 year; fewer headaches, dizziness, or cramps; and shorter postdialysis recovery time.

Conclusions: HDF is associated with a lack of progression in vascular measures versus progression with HD, as well as an increase in height not seen in the HD cohort. Patient-related outcomes improved among children on HDF correlating with improved BP control and clearances. Confirmation through randomized trials is required.
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http://dx.doi.org/10.1681/ASN.2018100990DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6442347PMC
April 2019

Effect of haemodiafiltration vs conventional haemodialysis on growth and cardiovascular outcomes in children - the HDF, heart and height (3H) study.

BMC Nephrol 2018 08 10;19(1):199. Epub 2018 Aug 10.

Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

Background: Cardiovascular disease is prevalent in children on dialysis and accounts for almost 30% of all deaths. Randomised trials in adults suggest that haemodiafiltration (HDF) with high convection volumes is associated with reduced cardiovascular mortality compared to high-flux haemodialysis (HD); however paediatric data are scarce. We designed the haemodiafiltration, heart and height (3H) study to test the hypothesis that children on HDF have an improved cardiovascular risk profile, growth and nutritional status and quality of life, compared to those on conventional HD. We performed a non-randomised parallel-arm intervention study within the International Paediatric Haemodialysis Network Registry comparing children on HDF and conventional HD to determine annualised change in cardiovascular end-points and growth. Here we present the 3H study design and baseline characteristics of the study population.

Methods: 190 children were screened and 177 (106 on HD and 71 on HDF) recruited from 28 centres in 10 countries. There was no difference in age, underlying diagnosis, comorbidities, previous dialysis therapy, dialysis vintage, residual renal function, type of vascular access or blood flow between HD and HDF groups. High flux dialysers were used in 63% of HD patients and ultra-pure water was available in 52%. HDF patients achieved a median convection volume of 13.3 L/m; this was associated with the blood flow rate only ((p = 0.0004, r = 0.42) and independent of access type (p = 0.38).

Discussion: This is the largest study on dialysis outcomes in children that involves deep phenotyping across a wide range of cardiovascular, anthropometric, nutritional and health-related quality of life measures, to test the hypothesis that HDF leads to improved cardiovascular and growth outcomes compared to conventional HD.

Trial Registration: ClinicalTrials.gov: NCT02063776 . The trial was prospectively registered on the 14 Feb 2014.
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http://dx.doi.org/10.1186/s12882-018-0998-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6086045PMC
August 2018

Distinctive vasculopathy with systemic involvement due to levamisole long-term therapy: a case report.

J Med Case Rep 2018 Jul 16;12(1):209. Epub 2018 Jul 16.

Hepatobiliary and Transplant Unit, Department of Surgery, Al-Amiri Hospital, Kuwait City, Kuwait.

Background: Levamisole belongs to the antihelminthic class of drugs that are sometimes administered to patients with frequently relapsing or steroid-dependent nephrotic syndrome, owing to its steroid-sparing effects. Neutropenia and skin lesions, compatible with vasculitis, have been reported as drug complications, but they are rarely associated with any systemic involvement.

Case Presentation: We report a case of a 9-year-old Arab boy with steroid-dependent nephrotic syndrome who was treated with levamisole after his third relapse. The drug was initially well tolerated, but mild isolated neutropenia occurred 6 months after levamisole administration. This was followed by cutaneous vasculitis of both ears and the left cheek. The patient also developed hepatosplenomegaly and anemia. Levamisole was discontinued, and his disease remained in remission. All the systemic manifestations disappeared gradually over the course of 1 month. The patient remained in remission until 1 year after levamisole withdrawal, when clinical nephrosis recurred.

Conclusions: Despite levamisole's being a useful drug for maintaining remission in steroid-dependent nephrotic syndrome, patients on long-term levamisole therapy should be monitored closely to prevent serious complications that can easily be resolved by simple drug withdrawal.
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http://dx.doi.org/10.1186/s13256-018-1728-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6047121PMC
July 2018

Pamidronate Rescue Therapy for Hypercalcemia in a Child With Williams Syndrome.

Front Endocrinol (Lausanne) 2018 14;9:240. Epub 2018 May 14.

Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

A 15-month-old male infant diagnosed with Williams Syndrome (WS) was admitted with severe hypercalcemia and nephrocalcinosis. Intravenous hydration and furosemide failed to yield an appreciable and sustainable fall in serum calcium, while the injection of pamidronate achieved a significant decrease in serum calcium in a short period of time. This bisphosphonate could be considered as a second-line treatment for refractory hypercalcemia in WS.
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http://dx.doi.org/10.3389/fendo.2018.00240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5968380PMC
May 2018

Ureteropelvic Junction Obstruction and Parathyroid Adenoma: Coincidence or Link?

Case Rep Nephrol 2017 17;2017:9852912. Epub 2017 Oct 17.

Pediatric Nephrology, Armand Trousseau Hospital, APHP, Paris, France.

Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of upper urinary tract obstruction in children. It is generally diagnosed in the routine work-up during antenatal period and is characterized by spontaneous recovery. It can be associated with urolithiasis; hence further investigation should be carried out. We report the case of a 15-year-old boy, who is known to have right UPJO, presented with right renal colic and discovered to have bilateral kidney stones. Further studies showed primary hyperparathyroidism and genetic analysis revealed a CDC73 mutation (initially HRPT2). We believe that association of UPJO and PHPT is a rare coincidence that can be linked. Careful work-up of children with UPJO and urolithiasis is recommended to exclude an underlying metabolic disease. Surgical correction can be evitable as treatment of the primary cause can lead to complete dissolution of kidney stones and improvement of the medical condition.
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http://dx.doi.org/10.1155/2017/9852912DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5664328PMC
October 2017

Clinical outcomes in children with Henoch-Schönlein purpura nephritis without crescents.

Pediatr Nephrol 2017 Jul 15;32(7):1193-1199. Epub 2017 Feb 15.

Pediatric Nephrology, Armand Trousseau Hospital, APHP, 26 Avenue du Docteur Netter, Paris, 75012, France.

Background: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis.

Methods: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files. The primary endpoint was proteinuria remission defined as a proteinuria <200 mg/L.

Results: Ninety-two children were included in the study with a median follow-up of 36 (6-120) months; 28% had nephrotic syndrome, 31% proteinuria >3 g/L, 52% proteinuria between 1 and 3 g/L, and 18% proteinuria <1 g/L. Forty-seven percent of patients received orally treatment with steroids alone, 37% received methylprednisolone pulses followed by steroids orally, 18% received no steroids. Although 85% reached remission during follow-up, 12% did not maintain complete remission over time so that only 75% remained in complete remission by the end of the follow-up. Univariate analysis found a higher likelihood of remission in patients with higher proteinuria at disease onset (p = 0.009). This trend was not found in the multivariate analysis after adjusting for treatments, as patients with higher proteinuria were most often treated with steroids.

Conclusion: Our study shows that one fourth of patients with HSPN class 2 remain proteinuric and thus carry the risk of developing chronic kidney disease over the long term. This finding, together with the better outcome of patients treated with steroids, is in favor of using high-dose steroids orally or IV in these patients.
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http://dx.doi.org/10.1007/s00467-017-3604-9DOI Listing
July 2017

Chronic peritoneal dialysis in Lebanese children of families with low socioeconomic status.

Perit Dial Int 2015 Jan-Feb;35(1):93-6

Armand-Trousseau University Hospital APHP, Department of Pediatric Nephrology, Paris, France Rafic Hariri University Hospital Department of Pediatrics, Beirut, Lebanon American University Hospital Department of Pediatrics, Beirut, Lebanon Rafic Hariri University Hospital Department of general surgery, Beirut, Lebanon Center for Pediatric and Adolescent Medicine University of Heidelberg, Heidelberg, Germany DHU-I2B Inflammation-Immunopathologie-Biothérapie Université Pierre et Marie Curie Paris, France.

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http://dx.doi.org/10.3747/pdi.2013.00342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4335934PMC
November 2015

Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort.

Clin J Am Soc Nephrol 2015 Apr 29;10(4):592-600. Epub 2015 Jan 29.

Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.

Background And Objectives: Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome.

Design, Setting, Participants, & Measurements: Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal.

Results: Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%-16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%-45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the histopathologic disease type was strongly predictive of intensified immunosuppressive therapy responsiveness. Post-transplant disease recurrence was noted in 25.8% of patients without compared with 4.5% (n=4) of patients with a genetic diagnosis.

Conclusions: The PodoNet cohort may serve as a source of reference for future clinical and genetic research in this rare but significant kidney disease.
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http://dx.doi.org/10.2215/CJN.06260614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4386250PMC
April 2015

Even mild cases of paediatric Henoch-Schönlein purpura nephritis show significant long-term proteinuria.

Acta Paediatr 2015 Aug 29;104(8):843-8. Epub 2014 Jul 29.

Department of Paediatric Nephrology, Armand-Trousseau Hospital, APHP, Paris, France.

Aim: Henoch-Schonlein purpura (HSP) is a common cause of paediatric renal disease in children, representing 10-15% of paediatric glomerulonephritis. This study examined the long-term outcome of biopsy-proven HSP nephritis to identify correlations between disease development and treatment.

Methods: Patients from three French centres were retrospectively analysed.

Results: We followed up 142 patients aged from 2 to 10.5 years with HSP nephritis, graded according to the International Study Group of Kidney Disease in Childhood classification. Mean (±SD) age at presentation was 7.6 ± 2.8 years. Nephrotic range proteinuria was present in 28% of patients with Grade II lesions, 60% with Grade III and 90% with Grade IV. Significant proteinuria (>0.5 g/L) was found in nine of 48 patients 3 years after renal biopsy, eight of 25 patients after 5 years and three of 14 patients after 10 years. There was no correlation between the proteinuria risk at 3, 5 or 10 years and the initial histological lesion or treatment modality. Treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEi/ARB) was linked to lower proteinuria, especially if it was started precociously.

Conclusion: Even mild forms of HSP nephritis risk significant long-term proteinuria. Very early introduction of ACEi/ARB may improve the long-term outcome independent of histological lesions.
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http://dx.doi.org/10.1111/apa.12723DOI Listing
August 2015

Immunoadsorption: a new strategy to induce remission in membranous lupus nephritis.

Case Rep Nephrol Urol 2014 Jan 19;4(1):37-41. Epub 2014 Mar 19.

Department of Pediatric Nephrology, APHP, DHU i2B Inflammation-Immunopathology-Biotherapy, Armand-Trousseau Hospital, Paris, France.

We report the case of an 11-year-old previously healthy girl who presented for microscopic hematuria and nephrotic proteinuria with normal renal function, which persisted after 6 months of steroids, angiotensin-converting enzyme inhibitors (ACEi)/angiotensin receptor blockers, hydroxychloroquine, mycophenolic acid and a low-salt diet. A serum investigation suggested lupus nephritis and a renal biopsy, performed 2 weeks after the first proteinuria detection, revealed membranous lupus nephritis. We decided to perform ten sessions of daily immunoadsorption. Proteinuria decreased significantly over these ten sessions from 8 to 0.12 g/l. After the tenth immunoadsorption session, the patient received the first rituximab (RTX) infusion leading to complete B-cell depletion. The patient was maintained on ACEi associated with mycophenolic acid and hydroxychloroquine. Three RTX reinjections were performed when CD19-positive cells reappeared in peripheral blood. Despite complete B-cell recovery and positive anti-dsDNA-Ab, the patient remained in complete remission 18 months after the initial diagnosis with negative proteinuria and a normal renal function.
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http://dx.doi.org/10.1159/000361014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4000301PMC
January 2014

Rapid and sustained recovery of renal function with transient placement of an intrauretral nephrostomy catheter in an infant with ureteropelvic junction obstruction and acute renal failure.

J Med Liban 2014 Jan-Mar;62(1):54-6

Ureteropelvic junction obstruction (UPJO) is a common, congenital urinary malformation in the pediatric age group. In most cases the diagnosis is made antenataly and resolves spontaneously. Postnatal diagnosis is made when symptoms of urinary tract infection or abdominal pain occur. We report a six-month-old girl with single kidney and known vesicoureteral reflux grade IV presenting with severe acute renal failure (ARF), requiring acute peritoneal dialysis (PD).After diagnosis of decompensated UPJO, a nephrostomy was performed, and renal function restored within seven days. UPJO was subsequently treated by open pyeloplasty. To our knowledge, this is the first case of UPJO requiring PD due to severe renal failure in a child. Children with UPJO and major morbidity of the contralateral kidney are at risk of renal failure and should therefore be followed carefully to prevent serious complications.
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http://dx.doi.org/10.12816/0002628DOI Listing
April 2014

Does rituximab induce hypogammaglobulinemia in patients with pediatric idiopathic nephrotic syndrome?

Pediatr Nephrol 2013 Mar 5;28(3):447-51. Epub 2012 Dec 5.

Department of Pediatric Nephrology, Armand Trousseau Hospital, AP-HP 75012, Paris, France.

Background: Rituximab (RTX) is a promising strategy for treating steroid-dependent idiopathic nephrotic syndrome (SDNS). RTX induces profound B-cell depletion, suggesting hypogammaglobulinemia as a potential side effect after long-term treatment.

Patients And Methods: We analyzed immunoglobulin G (IgG) levels in 12 pediatric patients on RTX with a B-cell depletion of a minimum of 3 months and compared the results to 16 patients on orally administered immunosuppressive drugs, such as mycophenolate mofetil (MMF) and/or cyclosporine A (CyA). All patients were in stable remission of SDNS at the time of IgG analysis.

Results: IgG levels in the RTX group before RTX introduction were 6.2 ± 1.0 g/L and were not significantly different from the MMF/CyA group (8.2 ± 2.5 g/L). In the MMF/CyA group, five patients had at least one episode of hypogammaglobulinemia. In two of them, this episode was prolonged (>3 months), and only one required IgG supplementation. In the RTX group, eight patients had decreased IgG levels before RTX infusion. After RTX, hypogammaglobulinemia persisted in seven among those eight patients. No decreased IgG plasma levels were noted in patients with normal baseline IgG levels before RTX treatment.

Conclusion: RTX does not seem to directly induce decreased IgG levels in patients with SDNS, but it seems to prolong a preexisting low IgG levels.
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http://dx.doi.org/10.1007/s00467-012-2361-zDOI Listing
March 2013

Protocol biopsies in pediatric renal transplant recipients on cyclosporine versus tacrolimus-based immunosuppression.

Pediatr Nephrol 2013 Mar 31;28(3):493-8. Epub 2012 Oct 31.

Service de Néphrologie et Dialyses Pédiatrique, Hôpital Armand Trousseau, Avenue du Docteur Netter, 75012 Paris, France.

Background: Protocol biopsies can detect subclinical rejection and early signs of calcineurin inhibitor-induced nephrotoxicity.

Methods: In a prospective study, protocol biopsies 3 and 12 months after transplant in transplanted children from two centers were studied. One center used cyclosporine (CsA)-based immunosuppression and the other center used tacrolimus. Patients were on CsA (n = 26, group 1) or on tacrolimus (n = 10, group 2). Patients received basiliximab induction, mycophenolate mofetil, and prednisone.

Results: In patients on CsA, 26 kidney biopsies were performed during the 6 months after transplantation. Eighteen protocol biopsies were performed at 3 months post transplant; 13 were normal and five showed rejection (two borderline and three Banff II rejections). Eight biopsies were motivated by an increase of serum creatinine; four were normal and four revealed signs of acute rejection (two borderline and two Banff II). Twelve protocol biopsies were performed after 12 months; all were normal. For patients on tacrolimus (n = 10), ten protocol transplant biopsies were performed at 3 months post-transplant; none showed signs of rejection. No biopsy was performed for an increase of serum creatinine. There were no differences in patient age, number of human leukocyteantigen (HLA) incompatibilities, or other patient characteristics.

Conclusions: Patients on tacrolimus had less acute rejection episodes detected on protocol biopsies 3 months after transplant. Protocol biopsies seem to play an important role in the detection of subclinical rejection in patients on CsA.
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http://dx.doi.org/10.1007/s00467-012-2330-6DOI Listing
March 2013

Do vitamin D plasma levels impact vaccine response in children with idiopathic nephrotic syndrome?

Pediatr Nephrol 2012 Nov 16;27(11):2161-2162. Epub 2012 Aug 16.

Service de Néphrologie et Dialyses Pédiatrique, Hôpital Armand Trousseau, Assistance Publique-Hôpitaux de Paris, Avenue du Docteur Netter, 75012, Paris, France.

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http://dx.doi.org/10.1007/s00467-012-2273-yDOI Listing
November 2012

Dipstick urine analysis screening among asymptomatic school children.

N Am J Med Sci 2011 Apr;3(4):179-84

Department of Pediatrics, Makassed General Hospital, Beirut, Lebanon.

Background: Mass urinary screening is a useful tool to identify children with asymptomatic progressive renal diseases. A dipstick urinalysis screening was conducted to detect such prevalence and to set up a more effective screening program for children.

Patients And Methods: A cross sectional study was carried out in seven nurseries and primary schools in different regions of Lebanon (Beirut, North Lebanon, and Valley of Bekaa) between February 2010 and March 2010. Eight hundred seventy asymptomatic children were enrolled in this study. First morning mid steam urine samples were obtained from students and were tested by dipstick method. Children with abnormal findings were re-tested after fifteen days.

Results: Twenty five (2.9%) children had urinary abnormalities at the first screening; Eighteen (72%) of them still had abnormal results at the second screening. Among all the students, hematuria was the most common abnormality found with a prevalence of 1.5%, followed by nitrituria (0.45%), combined hematuria and nitrituria (0.45%) and proteinuria (0.1%). Urinary abnormalities were more common in females than in males. With respect to age, most positive results were detected at 6 years of age. Hematuria and proteinuria were mainly present in the North of Lebanon.

Conclusion: Asymptomatic urinary abnormalities might be detected by urine screening program at school age. Further work-up should be offered to define the exact etiology of any abnormal finding and to determine whether early detection of renal disorders in childhood will lead to effective interventions and reduction in the number of individuals who develop end-stage renal disease.
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http://dx.doi.org/10.4297/najms.2011.3179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3336909PMC
April 2011

A patient with acute urinary retention of unknown origin.

Pediatr Nephrol 2012 Mar 10;27(3):385-8. Epub 2011 Aug 10.

Department of Pediatric Nephrology, Rafic Hariri University Hospital, Beirut, Lebanon.

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http://dx.doi.org/10.1007/s00467-011-1953-3DOI Listing
March 2012

Acute tubulointerstitial nephritis.

Pediatr Nephrol 2012 Jul 3;27(7):1051-7. Epub 2011 Jun 3.

Department of Pediatric Nephrology, Armand Trousseau Hospital (APHP), University Pierre & Marie Curie, Paris 6, 26, Avenue du Docteur Arnold Netter, 75012, Paris, France.

Acute tubulointerstitial nephritis (TIN) is a frequent cause of acute renal failure, characterised by the presence of inflammatory cell infiltrate in the interstitium of the kidney. Immuno-allergic reaction to certain medications, mainly non-steroidal anti-inflammatory drugs and antibiotics are by far the most important etiology for TIN today, but other situations such as infections, toxins, and vasculitis are known to induce TIN. Incidence of TIN is increasing, probably due to prescription habits and NSAID overuse, representing 3-7% of acute kidney injury in biopsies in children. Avoidance of the causal substance and rapid steroid therapy are hallmarks for patient care, but spontaneous initial recovery is very frequent and the general prognosis seems satisfactory. However, development of chronic TIN, without response to steroid or other immunosuppressive treatment, is possible. As the largest part of TIN is secondary to certain drugs, clear indications in particular for NSAID or antibiotics should be respected to reduce the number of TIN cases.
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http://dx.doi.org/10.1007/s00467-011-1915-9DOI Listing
July 2012

Effect of vitamin E coated dialyzers on anticoagulation requirement in hemodialyzed children.

Saudi J Kidney Dis Transpl 2010 May;21(3):466-70

Department of Pediatric Nephrology, Armand Trousseau Hospital, APHP, University Pierre et Marie Curie, Paris, France.

Unlabelled: As hemodialysis (HD) requires extracorporal blood flow and the need for anticoagulation, we evaluated the effect of vitamin E coated membranes (VIE) on the requirement of low molecular weight heparin (LMWH) in pediatric HD patients.

Patients And Methods: seven children and adolescents on regular hemodialysis were started on VIE and their LMWH dose was decreased every week. In order to monitor the requirement of LMWH we used a coagulation score to evaluate coagulation in the dialyzer, air trap and blood line. Other classical parameters (hemoglobin, erythropoietin dose, inflammatory markers) were monitored weekly while the patients were on VIE dialyzers. LMWH dose during the 1st week was 110 IU/kg +/- 18 (defined as 100%), in the 2nd week the dose was 77 IU/kg +/- 12 (70%), in the 3rd week the dose was 33 IU/kg +/- 5 (30%), and in the 4th week anticoagulation could be stopped in one patient, in the other six patients further decrease was impossible given the increase of the clotting score. There was no increase in clotting score during week one and two. During week three (while on 30% of the initial LMWH dose) six patients showed mild to moderate clotting phenomena: mild coagulation phenomena in three patients and moderate clotting phenomena in three others. One patient did not show any clotting phenomena in week three and LMWH was totally stopped. In conclusion, use of VIE dialyzers may help to reduce the requirement of anticoagulation in pediatric HD patients reducing bleeding problems and simplify hemostasis after HD sessions.
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May 2010

Aortic stiffness in ESRD children before and after renal transplantation.

Pediatr Nephrol 2010 Jul 30;25(7):1331-6. Epub 2010 Mar 30.

Department of Pediatric Nephrology, Armand-Trousseau Hospital, AP-HP, 26, Avenue Arnold Netter, 75012, Paris, France.

Cardiovascular complications are the main cause of death in end-stage renal failure in adult patients, but those complications start in childhood. Renal transplantation (RT) seems to reduce or even reverse certain abnormalities seen in dialyzed patients. Since RT seems to correct metabolic abnormalities that play a role in cardiovascular disease, aortic pulse wave velocity (APWV) was used to evaluate aortic stiffness before and after RT. We included 15 children on chronic hemodialysis (HD), aged 11.1 +/- 4.8 years and dialysis duration was 12.9 +/- 7.4 months. APWV was performed every 6 months before RT and 6 months after. There was no significant difference in APWV (6.1 +/- 1.3 m/s vs 6.5 +/- 1.4 m/s) and augmentation index (AI) on HD and 6 months after RT. APWV pre-transplant was not correlated with time on HD, but increased with age (p = 0.016). No correlation between APWV pre-/post-transplant and other HD parameters or calcineurin inhibitor exposure were found. Only graft function was inversely correlated with APWV post-transplant (p = 0.02). In conclusion, aortic stiffness seems to remain stable before and 6 months after pediatric RT. Graft function was inversely correlated with APWV. Differences in vessel structure among children of the same age group and increase in aortic stiffness with age may jeopardize data interpretation.
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http://dx.doi.org/10.1007/s00467-010-1509-yDOI Listing
July 2010

L-carnitine supplementation and EPO requirement in children on chronic hemodialysis.

Pediatr Nephrol 2010 Mar 7;25(3):557-60. Epub 2009 Aug 7.

Department of Pediatric Nephrology, Armand Trousseau Hospital, APHP, University Pierre et Marie Curie, 75012, Paris, France.

L-carnitine supplementation has been the subject of heated discussion in the context of the treatment of pediatric hemodialysis patients. The aim of this study was to analyze the effect of intravenous L-carnitine supplementation on the erythropoetin (EPO) requirement in six pediatric hemodialysis patients. All patients were on intravenous L-carnitine (2.5 g per session for patients >30 kg and 1 g for those <30 kg) for 9 months. The EPO dose was adapted monthly to maintain a target hemoglobin (Hb) level of 11-13 g/dl. Prior to the initiation of L-carnitine supplementation, the EPO requirement was 1.15 +/- 0.22 (range 0.37-1.75) microg/kg darbepoetin alpha. Free carnitine (FC) levels were measured before (40.4 +/- 4.9 micromol/l), immediately after the 9-month L-carnitine supplementation period (378.5 +/- 77.3 micromol/l), and 4 months after withdrawal of L-carnitine (95.6 +/- 4.0 micromol/l). After 9 months, the EPO dose was 0.47 +/- 0.10 microg/kg (p < 0.002). The Hb levels increased from 12.2 +/- 0.97 to 14.0 +/- 0.54 g/dl (p < 0.05) within the first 2 months, and the EPO dose was then decreased in a stepwise manner. In conclusion, following intravenous carnitine supplementation, FC levels were higher and persisted longer than expected. This rise was associated with increased Hb levels and decreased EPO requirement. Since controls were missing for this study, prospective long-term multi-center studies on a large number of patients are required to provide solid answers to the controversial question of L-carnitine supplementation in hemodialyzed children.
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http://dx.doi.org/10.1007/s00467-009-1276-9DOI Listing
March 2010

Therapy-resistant anaemia in congenital nephrotic syndrome of the Finnish type--implication of EPO, transferrin and transcobalamin losses.

Nephrol Dial Transplant 2009 Apr 18;24(4):1338-40. Epub 2009 Jan 18.

Department of Pediatric Nephrology, Armand Trousseau Hospital, 26 Avenue du Docteur Arnold Netter, 75012 Paris, France.

Congenital nephrotic syndrome of the Finnish type (CNF) is due to NPHS1 mutation and is responsible for a variety of urinary protein losses. We report the case of a 4-month-old girl with a particularly severe form (proteinuria approximately 150 g/l) of CNF. She developed severe non-regenerative anaemia requiring bi-monthly blood transfusions despite daily EPO (600 UI/kg) and iron supplementation. Epoetin pharmacokinetics revealed a urinary loss of 27% of the given dose within the first 24 h after IV injection. However, plasma levels remained increased after 24 h (228 UI/l). Plasma transferrin and transcobalamin levels were undetectable. Atransferrinaemia and atranscobalaminaemia seem to be responsible for disturbed erythropoiesis.
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http://dx.doi.org/10.1093/ndt/gfn762DOI Listing
April 2009