The 15th CONGRESS of the INTERNATIONAL ...

Pediatr Nephrol (2010) 25:1779–2004 DOI 10.1007/s00467-010-1577-z

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Pediatr Nephrol (2010) 25:1779–2004

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The 15th Congress of the International Pediatric Nephrology Association Executive Congress Organizers Congress President Frederick J. Kaskel, MD, PhD Albert Einstein College of Medicine Bronx, NY, USA

IPNA Secretary General Isidro B. Salusky, MD University of California, Los Angeles Los Angeles, CA, USA

Honorary Chairman Ira Greifer, MD Albert Einstein College of Medicine Bronx, NY, USA

Co-Chair Richard Fine, MD Stony Brook University Stony Brook, NY, USA

Scientific Committee Chair H. William Schnaper, MD Northwestern University Chicago, IL, USA

Co-Chair Mary B. Leonard, MD Children’s Hospital of Philadelphia Philadelphia, PA, USA

Co-Chair Lisa M. Guay-Woodford, MD University of Alabama at Birmingham Birmingham, AL, USA

Committee Chairs Local Organizing Committee Chair Lisa M. Satlin, MD Mount Sinai School of Medicine New York, NY, USA

Developmental Committee Chair Barbara A. Fivush, MD Johns Hopkins University Baltimore, MD, USA

Awards and Scholarship Committee Chair Sandra L. Watkins, MD Seattle Children’s Hospital, University of Washington Seattle, WA, USA

IPNA Congress Headquarters 15000 Commerce Parkway, Suite C Mt. Laurel, NJ 08054 USA Telephone: 856-439-0500 Fax: 856-439-0525 Website: www.ipna2010.org

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International Scientific Committee Robert Kleta (United Kingdom) Vera Koch (Brazil) Daniel Landau (Israel) Craig B. Langman (United States) Mary Leonard (United States) Robert H.K. Mak (United States) Doug Matsell (Canada) Tej K. Matoo (United States) Mignon McCulloch (South Africa) Ruth McDonald (United States) Sevgi Mir (Turkey) Mark Mitsnefes (United States) Marva M. Moxey-Mims (United States) Victoria F. Norwood (United States) Nelson Orta-Sibu (Venezuela) Anthony A. Portale (United States) Lesley Rees (United Kingdom) Gyorgy Reusz (Hungary) Moin A. Saleem (United Kingdom) Cheryl Sanchez (United States) Fernando Santos (Spain) Lisa M. Satlin (United States) Franz Schaefer (Germany) Morris Schoeneman (United States) Tomas Seeman (Czechoslovakia) Oguz Söyemezoglu (Turkey) Burkhard Toenshoff (Germany) Howard Trachtman (United States) Alexy Tsygin (Russia) Alda Tufro (United States) Tivadar Tulassay, Hungary Sandor Turi (Hungary) William Van’t Hoff (United Kingdom) V. Matti Vehaskari (United States) Bradley A. Warady (United States) Alan Watson (United Kingdom) Robert J. Wyatt (United States) Hui-Kim Yap (Singapore) Gaston Zilleruelo (United States)

Steven R. Alexander (United States) Uri Alon (United States) Ellis Avner (United States) Arvind Bagga (India) Aysin Bakkaloglu (Turkey) Carl M. Bates (United States) Michel G. Baum (United States) John J. Bissler (United States) Melvin Bonilla (Puerto Rico) David M. Briscoe (United States) Necla Buyan (Turkey) Pierre Cochat (France) Prasad Devarajan (United States) Jie Ding (China) Allison Eddy (United States) Alberto Edefont (Italy) Felicia Eke (Nigeria) Michel Fischbach (France) Barbara Fivush (United States) Joseph T. Flynn (United States) John Foreman (United States) Aaron L. Friedman (United States) Susan L. Furth (United States) Debbie Gipson (United States) Stuart Goldstein (United States) Paul R. Goodyer (Canada) Lisa Guay-Woodford (United States) Friedhelm Hildebrandt (United States) Elizabeth Hodson (Australia) Masataka Honda (Japan) Bernd Hoppe (Germany) Peter Hoyer (Germany) Ikeuni Ichikawa (Japan) Takashi Igarashi (Japan) Julie Ingelfinger (United States) Shouji Kagami (Japan) Clifford Kashtan (United States) Kanwal Kher (United States) Doris Kinuthia (Kenya) 2


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IPNA 2010 Abstract Reviewers IPNA would like to thank those involved in the 2010 abstract review and selection process. Abstract Program Chair Mary B. Leonard (United States)

Helena F. Jardim (Portugal) Gunter Klaus (Germany) Daniel Landau (Israel) Marc Lande (United States) Mary B. Leonard (United States) Alvaro D. Madrid (Spain) Susan Massengill (United States) Doug Matsell (Canada) Tej Mattoo (United States) Mara Medeiros (Mexico) Mark Mitsnefes (United States) Giovanni Montini (Italy) Michael Moritz (United States) Daryl Okamura (United States) Rulan Parekh (Canada) Madhura Pradhan (United States) Lesley Rees (United Kingdom) Jaime Manuel Restrepo (Colombia) Laura Espinosa Roman (Spain) Cheryl Sanchez (United States) Turi Sandor (Hungary) Minnie Sarwal (United States) Douglas Silverstein (United States) Jodi Smith (United States) Michael Somers (United States) Howard Trachtman (United States) Albertus J. van der Heijden (The Netherlands) Enrico Verrina (Italy) Shefali Vyas (United States) Alan Watson (United Kingdom) Donald J. Weaver (United States) Katherine Wesseling-Perry (United States) Robert Woroniecki (United States) Elke Wuhl (Germany) Ikuyo Yamaguchi (United States) Kim Yap (Singapore) Israel Zelikovic (Israel)

Abstract Review Chairs Leonard Feld (United States) Rosanna Coppo (Italy) Robert Mak (United States) Kevin Meyers (United States) Craig Wong (United States) Fernando Santos (Spain) Abstract Reviewers Laura F. Alconcher (Argentina) Uri Alon (United States) Arvind Bagga (India) Ashraf Bakr (Egypt) Carl M. Bates (United States) Rajendra Bhimma (South Africa) John Joseph Bissler (United States) Detlef Bockenhauer (United Kingdom) Alberto A. Caldas-Afonso (Portugal) Francisco J. Cano (Chile) Gianni Celsi (Sweden) James CM Chan (United States) Robert L. Chevalier (United States) Annabelle Chua (United States) Vikas Dharnidharka (United States) Jie Ding (China) Alberto C. Edefonti (Italy) Mesiha Ekim (Turkey) Barbara Fivush (United States) Beth Foster (Canada) Yaacov Frischberg (Israel) Sue Furth (United States) Eduardo Garin (United States) Luciana Ghio (Italy) Jens Goebel (United States) Beatrce Goilav (United States) Larry Adam Greenbaum (United States) Dieter Haffner (Germany) Friedhelm Hildebrandt (United States) Julie R. Ingelfinger (United States) Khalid Ismaili (Belgium)

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Schedule of Activities August 29 – September 2, 2010 Hilton New York New York, NY, USA Poster Viewing Hours

On-Site Registration Hours Friday, August 27 Saturday, August 28 Sunday, August 29 Monday, August 30 Tuesday, August 31 Wednesday, September 1 Thursday, September 2

Sunday, August 29 Monday, August 30 Tuesday, August 31 Wednesday, September 1

4:00 PM – 6:00 PM 6:30 AM – 6:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 12:00 PM

Poster Session (Presenters in Attendance)

Speaker Ready Room Hours Friday, August 27 Saturday, August 28 Sunday, August 29 Monday, August 30 Tuesday, August 31 Wednesday, September 1 Thursday, September 2

6:30 PM – 7:30 PM 9:00 AM – 5:30 PM 9:00 AM – 5:30 PM 9:00 AM – 5:30 PM

4:00 PM – 6:00 PM 6:30 AM – 6:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 7:00 PM 6:30 AM – 12:00 PM

Exhibit Hours

Poster Session I Sunday, August 29 Monday, August 30

6:30 PM – 7:30 PM 12:15 PM – 1:00 PM

Poster Session II Tuesday, August 31

12:15 PM – 1:00 PM

Poster Session III Wednesday, September 1

12:15 PM – 1:00 PM

Congress Opening Ceremony

Sunday, August 29 6:30 PM – 7:30 PM Opening of Exhibits, Opening Reception Monday, August 30 9:00 AM – 3:45 PM Tuesday, August 31 9:00 AM – 3:45 PM Wednesday, September 1 9:00 AM – 3:45 PM

Sunday, August 29

5:00 PM – 6:30 PM

Congress Reception Wednesday, September 1

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7:00 PM – 10:00 PM


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Awards Trainee Travel Scholarship Awards Yonca Acikgoz Abstract #321

Ashima Gulati Abstract #396

Qian Shen Abstract #880

Banu Aykanat Abstract #589

Kaan Gulleroglu Abstract #121

Diamant Shtiza Abstract #156

Nora Banki Abstract #193

Anna Kaczmarek Abstract #373

Magdalena Silska Abstract #443

Surabhi Choudhary Abstract #563

Ina Kazyra Abstract #61

Aditi Sinha Abstract #678

Elif Comak Abstract #105

Agata Korzeniecka-Kozerska Abstract #381

Vesna Stojanovic Abstract #903

Orsolya Cseprekal Abstract #798

Dusan Kostic Abstract #734

Qiang Sun Abstract #327

Melina Chã d´Oliveira Abstract #29

Flavia Leao Abstract #407

Katarzyna Taranta-Janusz Abstract #683

Martin Cuk Abstract #353

Aleksandra Mazo Abstract #389

Prayong Vachvanichsanong Abstract #457

Cagla Dogan Abstract #354

Md. Ariful Haque Mollik Abstract #298

Lucy Yaguo Ide Abstract #255

Mohammad Hossein Fallahzadeh Abstract #360

A Ohri Abstract #746

Onder Yavascan Abstract #769

Augustina Okpere Abstract #643

Ilona Zagozdzon Abstract #771

Iva Palčić Abstract #617

Mona Zahrane Abstract #257

Cintia Rogow Abstract #751

Yihui Zhai Abstract #717

Krisztina Rusai Abstract #181

Xin Zhang Abstract #265

Andrea Fekete Abstract #111 Quancheng Feng Abstract #648 Yolanda Fuentes Abstract #544 Paul Joseph Galutira Abstract #401 Na Guan Abstract #366

Joana Santos Abstract #439

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Non-Trainee Travel Scholarship Awards Asiri Abeyagunawardena Abstract #640

Wen-Yan Huang Abstract #935

Ertug Toroslu Abstract #760

Adebowale Ademola Abstract #330

Augustina Jankauskiene Abstract #813

Vu Tru Abstract #687

Indira Agarwal Abstract #188

Dafina Kuzmanovska Abstract #405

Alexey Tsygin Abstract #453

Aamir Al Mosawi Abstract #335

Maria del Carmen Laso Abstract #641

Sandor Turi Abstract #250

Laura Alconcher Abstract #843

Ming-Lee Lee Abstract #737

Anna Wasilewska Abstract #691

Sampson Antwi Abstract #849

Ali Reza Merrikhi Abstract #297

Alev Yilmaz Abstract #262

Csaba Bereczki Abstract #794

Israel Odetunde Abstract #869

Igor Zorin Abstract #391

Wattana Chartapisak Abstract #24

Saroj Patnaik Abstract #234

A Delucchi Abstract #106

Maria-Goretti Penido Abstract #570

Osman Donmez Abstract #206

Zvonimir Puretic Abstract #507

Ana Rose Dy Abstract #357

Jose Reyes Abstract #899

Ayah Elmaghrabi Abstract #805

Emilija Sahpazova Abstract #752

Ahmed El-Refaey Abstract #730

Paulina Salas Abstract #515

Fatemeh Ghane Sharbaf Abstract #117

Ashot Sarkissian Abstract #441

Wei Guo Abstract #934

Ana Cristina Simoes eSilva Abstract #875

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Trainee Research Awards Best Clinical Thurid Ahlenstiel Abstract #555 Best Translational J Jackson Abstract #467 Best Basic Science Eriko Tanaka Abstract #9

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Support* IPNA would like to thank the following companies for the generous support of the Congress: Alexion Pharmaceuticals Amgen Astellas Baxter Bristol Myers Squibb Cytochroma Genetech Genzyme Oceana IPNA would like to thank the following non-profit organizations for the generous support of the Congress: Alport Syndrome Foundation, Inc. Cystinosis Research Network International Pediatric Hypertension Association Kidney and Urology Foundation of America, Inc. The Nephcure Foundation Oxalosis and Hyperoxaluria Foundation PKD Foundation *All names listed are as of the date of printing.

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PRE CONGRESS PROGRAMMING

All sessions will take place at the Hilton New York Hotel unless otherwise stated.

CONGRESS PROGRAMMING

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The 15th CONGRESS of the INTERNATIONAL PEDIATRIC NEPHROLOGY ASSOCIATION ABSTRACTS

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GLOMERULAR DISEASE

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Abstract# 3

Glomerular Disease

(O-3) Oxidative Stress and Aberrantly Glycosylated IgA1 as Risk Factors for IgA Nephropathy R. Camilla,1 H. Suzuki,2 V. Daprà,1 E. Loiacono,1 L Peruzzi,1 A. Amore,1 G.M. Ghiggeri,3 G. Mazzucco,4 F. Scolari,5 A.G.G. Gharavi,6 G. Appel,6 J. Novak,2 B.A. Julian,2 S. Troyanov,7 R. Coppo.1 1 Nephrology Dial Transplant, R Margh Hosp, Turin, Italy; 2Microbiology Medicine, Un of Alabama at Birmingham, Birmingham, AL, United States; 3 Nephrology Lab, Gaslini Inst, Genoa, Italy; 4Biom Science Oncology, Un, Turin, Italy; 5Nephrology, Montichiari Hosp, Brescia, Italy; 6Medicine, Columbia Un, New York, NY, United States; 7Nephrology, Hop Sacré Coeur, Montreal, QC, Canada.

Abstract# 1 (O-1) Cytochrome P450 2B1 (CYP2B1) Mediates Complement-Induced Sublytic Injury to Glomerular Epithelial Cells (GEC) R. Baliga, N. Tian, I. Arany. Pediatrics, University of Mississippi Medical Center, Jackson, MS, United States. Objectives: CYP2B1 is an important source for reactive oxygen metabolites (ROM) mediated injury in Passive Heymann Nephritis (PHN) (JASN, 18:295A, 2007). Effacement of the foot processes and disorganization of the cytoskeleton are characteristic features leading to proteinuria.The current study was to determine whether CYP2B1 mediates anti-Fx1A-induced ROM production and collapse of the actin cytoskeleton in GEC. Methods: GEC that overexpress the CYP2B1 gene were transfected with scrambled or CYP2B1-specific siRNA prior to treatment with anti-Fx1A followed by incubation with heat-inactivated (HIS) or normal human serum (NS). ROM production, LDH release, Ethidium homodimer (EtD) staining (to quantitate membrane permeabilization) and disorganization of the actin filament was determined. Results: NS(complement) significantly increased ROM production, LDH release, EtD staining(A)and collapse of the actin cytoskeleton (B-C) with loss of lamellopodia ( arrows in B-C). Knockdown of the CYP2B1 gene attenuated ROM production, LDH release and EtD staining (A) as well as preserved the actin filament and lamellopodia (D). EtD staining (arrow in E) was accompanied by dissolution of the actin stress fiber network.

Objectives: Aberrantly glycosylated IgA1 is thought to be involved in pathogenesis of IgA nephropathy (IgAN) and a role for oxidative stress has been suggested. Aim of the study was to evaluate these markers with respect to clinical activity and progression of IgAN. Methods: We measured levels of galactose-deficient IgA1 (total levels,Gd-IgA1 and % of IgA, %HAA), advanced oxidation protein products (AOPPs) and albumin free SH groups (SH-Alb) in 292 IgAN patients (67 children) and 69 controls (HC) and correlated with clinical data. Results: Gd-IgA1 (U/ml) %HAA AOPPs (µmol/L) SH-Alb (AU) *p 0.05; The positive rates of Food and inhaled allergens in two groups were 40%, 33.33%, no difference between the two indicators, P> 0.05. Conclusions: 1,It seems that boy has more chance to relapse than girl in AP ; 2, The proportion of non-infectious incentive was risen in recurrent AP; 3,there were no diffrences in MP infection, streptococcal infection, allergic factors in AP at stage of early-onset and recurrent.

Gene expression I II III IV V VI Nephrin copy/µg RNA 10.9 ± 0.5 9.9 ± 0.6* 10.7 ± 0.4 10.3 ± 0.5 10.3 ± 0.5 10.2 ± 0.95 Podocin copy/µg RNA 10.8 ± 0.7 10.8 ± 0.8 10.7 ± 0.4 10.4 ± 0.2 10.6 ± 0.4 10.9 ± 0.8 ANOVA p = 0.03

Conclusions: CsA and Tac treatment did not improve proteinuria in PAN induced NS animals. The gene expression of nephrin was reduced in PAN induced NS. The treatment with calcineurin inhibtors in PAN induced NS increases the expression of nephrin. Podocin gene expression was not modified by the PAN induced model neither by the CI treatment. DISCLOSURE: Medeiros, M.: Consultant, Novartis, Mexico.

Abstract# 47 Efficacy of Prednisone –Tacrolimus vs. Prednisone – Cyclosporine in Steroid-Resistant Nephrotic Syndrome S. Valverde, A.M. Hernandez, L. Velasquez, B. Romero, A. Mendoza, G. Ramon, M. Medeiros. Hospital Infantil de México Federico Gómez, Mexico, Mexico. Objectives: To determine if the treatment with Prednisone (PDN) and Tacrolimus (FK) in pediatric patients with steroid resistant nephrotic syndrome (SRNS) for 12 months is superior to PDN and Cyclosporine(CyA) to induce and maintain remission. Methods: A comparative, randomized clinical trial was conducted in children with SRNS. Group I receive PDN+CsA, Group II PDN+FK for 12 months. Blood and urine samples were drawn monthly for serum creatinine, total protein albumin, lipids, calcineurin inhibitor levels and proteinuria. Results: 17 patients were included. Response rate is depicted in Table 1.

Abstract# 50 Intravenous Cyclophosphamide for Lupus Nephritis in Children L. Huynh Thoai, D. Nguyen thi Ngoc. Nephrology, Children’s Hospital 1, Ho Chi Minh, Viet Nam. Objectives: This study evaluates the clinical efficacy of CYC IV to achieve remission in children with severe lupus nephritis Methods: prospective descriptive study. Patients SLE who had 4 /11 ARA, biopsy proven lupus nephritis with either diffuse proliferative glomerulonephritis (WHO class IV) or focal proliferative glomerulonephritis (WHO class III) and who had received no immunosuppressive agent other than oral steroids were enrolled in the study. Results: 47 pateints were diagnosed lupus nephritis WHO III, IV (8 WHO III, 38 WHO IV). 1 did not attend follow-up, non died. 46 patients completed 6 months therapy with CYC. 41 (89%) girls, 4 (11%) boys. The mean age was 12 ± 2.5 years (range 8–16 years). At the time of diagnosed: 16 (34.7%) of patients were hypertention (blood pressure ≥ 140/90 mmHg), 2 (4%) macro hematuria, 6 (13%) renal failure (serum creatinine ≥ 1.2mg/dl). Clinical significance of lupus nephritis: 17 (37%) nephrotic syndrome (proteinuria > 50mg/kg/day or proteinuria/creatininuria > 200 mg/mmol), 1 (2%) nephritis (hematuria or cellular

Response to treatment Group I CyA+PDN (n=10 ) 10 (100%) 6 (60%) 4 (40%) 8 (80%)

Remission (n,%) Partial Complete Hypertension Time to achieve remission 24 ± 18 (weeks mean ± SD) *Chi square p 30µg albumin/mg creatinine was considered positive for micro albuminuria. These were measured at baseline and at 4 weeks. Renal ultrasound, CD4, clinical staging of HIV and treatment with HAART were done in all the patients and assessed. Results: Prevalence rate of HIVAN was 31.6%, out of which 3.1% had abnormal ultrasound findings. There was a significant correlation (r = -0.22, p = 0.03 ) between CD4 count and HIVAN. There was no correlation between HIVAN and duration on HAART ( r = -0.10, p = 0.31) and no association between HIVAN and clinical staging of HIV ( p=0.14). Conclusions: Prevalence of childhood HIVAN in Nigeria is high. Screening for urine microalbuminuria is essential for the early diagnosis of HIVAN and prevention of progression to CKD. Identified risk factors were low CD4 count and late clinical stages of HIV infection.

Abstract# 54 Effect of Huaiqihuangkeli on Proteinuria and Podocyte-Associated Molecules in Rats with IgA Nephropathy H.-y. Lu, X. Jiang, L.-z. Chen, Y. Mo, S.-M. Chen. Pedaitrics, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China. Objectives: To observe the effect of huaiqihuangkeli on proteinuria in rats with IgAN and changes of expression and distribution of nephrin, podocin, CD2AP and desmin, and to explore the mechanism of proteinuria of IgAN and try to find the approache to the therapy for IgAN. Methods: SD rats were divided into control group, model group, and huaiqihuang group. IgAN model was induced by the method of BSA+CCl4+LPS. The IgA intensity was examined by direct immunofluorescence. The protein expression and distribution of nephrin, podocin and desmin were examined by indirect immunofluorescence. The mRNA expression of nephrin, podocin, CD2AP and desmin were examined by RT-PCR. Results: (1) Hematuria and proteinuria in huaiqihuang group was less than model group. (2) Expression of nephrin, podocin and desmin in model were higer than control group. In huaiqihuang group, nephrin was higher than model group. Podocin had no difference from that in model group, but was still higher than control group. Desmin was lower than model group, but higher than that group. Abnormal discontinuous distribution of nephrin and podocin in huaiqihuang group was slighter than model group. (4) There was no difference of mRNA of nephrin, podocin and CD2AP between control and model group, however, desmin mRNA in model group was higher than control group. In huaiqihuang group, mRNA of nephrin, podocin and CD2AP expressed higher than that in model group, but desmin mRNA has no difference from that in model group. Conclusions: Huaiqihuangkeli is good for decreasing urinary protein by the way of regulating the expression and distribution of nephrin, podocin, CD2AP and desmin.

Abstract# 52 Efficacy and Safety of Tacrolimus in Children with Refractory Nephrotic Syndrome X.Y. Jiang, R.H. Lin, L.Z. Chen, Y.H. Ling, Y. Mo. Pediatrics, Sun Yat-sen University, Guangzhou, China. Objectives: To observe the efficacy and safety of a 12-24 months course of tacrolimus(FK506) therapy in children with refractory NS. Methods: Of the 16 patients enrolled in our study, 2 were SDNS, 10 were SRNS and 4 were FRNS. All patients initially received prednisone 2 mg/(kg·d). Fifteen of 16 patients were nonrespnsive to other therapies included CsA, CTX and mycophenolate mofetil(n=4). All these patients received a combined FK506 [(0.10∼0.15) mg/(kg·d)] / prednisone treatment. The dose of FK506 was adjusted to maintain a blood level of (5.0∼10.0) µg/L during the initial 10 months of treatment, then tapered off gradually until the total course of the treatment was 12-24 months. 24 hours urine protein and the blood level of Scr, BUN, Alb, ALT, Ccr, Chol, Glu, PLT were measured before and after FK506 treatment. Side effects of FK506 were observed at the same time. Results: Remission was achieved in 15(complete remission 12, partial remission 3) of 16 patients (93.8%). The remission rate was 100% in simple type NS, while 87.5% in nephritis type NS. And the remission rates in FRNS, SDNS and SRNS groups were 100%, 100% and 90.0%, respectively. In MCD group and MsPGN group, the remission rates were both 100%, while 75.0% in FSGS group. Two cases(12.5%) relapsed when FK506 was tapering. One patient had gastrointestinal tract reaction and anorexia,while another had insomnia during the combined FK506/prednisone treatment. Conclusions: FK506 was effective and well-tolerated in children with refractory NS, especially to NS children who were FRNS or had MCD. Besides, Our study also suggested that FK506 were effective for NS in children who were nonrespnsive to CsA and CTX.

Abstract# 55 Effect of Huangqi on Proteinuria and Podocyte-Associated Molecules in Rats with IgA Nephropathy H.-y. Lu, X.-Y. Jiang, L.-z. Chen, Y. Mo, S.-m. Chen. Pedaitrics, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China. Objectives: To observe the effect of huangqi on proteinuria in rats with IgAN and changes of expression and distribution of nephrin, podocin, CD2AP and desmin, and to explore the mechanism of proteinuria of IgAN and try to find the approache to the therapy for IgAN. Methods: SD rats were divided into control group, model group, and huangqi group. IgAN model was induced by the method of BSA+CCl4+LPS. The IgA intensity was examined by direct immunofluorescence. The protein expression and distribution of nephrin, podocin and desmin were examined by indirect immunofluorescence. The mRNA expression of nephrin, podocin, CD2AP and desmin were examined by RT-PCR. Results: (1) Hematuria and proteinuria in huangqi group was less than in model group. (2) Nephrin, podocin and desmin in model were higer than control group. In huangqi group, nephrin had no difference from that in model group, but was higher than control group. Podocin was lower than model group, but had no difference from that in control group. Desmin was higher than model group and control group. Abnormal discontinuous distribution of nephrin and podocin in huangqi group was slighter than model group. (3) There was no difference of mRNA expression of nephrin, podocin and CD2AP between control group and model group, however, desmin mRNA in model group was higher than control group. In huangqi group, mRNA of nephrin, podocin and CD2AP expressed higher than model group, but desmin mRNA has no significant difference from that in model group. Conclusions: Huangqi is good for decreasing urinary protein by the way of regulating the expression and distribution of nephrin, podocin, CD2AP and desmin.

Abstract# 53 Effects of Prednisone and Benazepril on Proteinuria and PodocyteAssociated Molecules in Rats with IgA Nephropathy H.-y. Lu, X.-Y. Jiang, L.-z. Chen, Y. Mo, S.-m. Chen. Pedaitrics, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China. Objectives: To observe the effects of prednisone and benazepril on proteinuria in rats with IgAN and changes of expression and distribution of nephrin, podocin, CD2AP and desmin, and to explore the mechanism of proteinuria of IgAN and try to find the approaches to the therapy for IgAN. Methods: SD rats were divided into control group, model group, prednisone group and benazepril group. The IgA intensity was examined by direct immunofluorescence. Expression and distribution of nephrin, podocin and desmin were examined by indirect immunofluorescence. The mRNA expression of nephrin, podocin, CD2AP and desmin were examined by RT-PCR.

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Monday, August 30

casts, hypertention, ± renal failure), 11 (24%) nephotic – nephritis syndrome, 17 (37%) abnormal urinalysis. After 6 times CYC IV: 3(6.5%) non response, 43 (93.5%) response: 25 (54.3%) partial remission, 18 (39.1%) complete remission. Conclusions: CYC IV seems to be effective in the treatment of severe lupus nephritis with minor side-effects. However, the long-term outcomes and sideeffects of CYC need to be followed up.

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Abstract# 56 Improved IgA Nephropathy Model H.-y. Lu, X.-Y. Jiang, L.-z. Chen, Y. Mo, S.-m. Chen. Depatment of Pedaitrics, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China. Objectives: To improve the method of constructing IgA nephropathy (IgAN) model and seek a more reliable and stable method. Methods: SD rats (n=96) were divided into control group (n=48) and model group (n=48). Reforming method: the dose of oral immunogen bovine serum albumin (BSA) increased one time (400mg/kg every other day), lasting 8 weeks; the method of injecting carbon tetrachloride (CCl4) was subcutaneous injection instead of intraperitoneal injection, dose of which was one third of content to induce hepatic fibrosis (0.1mL), weekly, continuing 9 weeks; combined with lipopolysaccharide (LPS) 0.05mg through tail vein once in the 6th week. Urine, serum and renal tissue samples were collected at the end of the 2nd, 4th, 6th, 8th, 10th and 12th week. Results: Hematuria and proteinuria appeared at the end of the 6th week in model group. Count of urinary red cells and urinary protein continued increasing till the end of the 12th week. There were no significant differences of ALT, AST, ALB, TP, CHOL, TG, TBIL, Cr, BUN and CK-MB between model group and control group(all P>0.05). Under LM, renal histopathologic changes became obvious after the end of the 8th week in model group, that was moderate to severe mesangial proliferation. Under EM, foot processes fusion appeared at the end of 4th week in model group, that was more and more severe till the end of the 12th week. Immunofluorescence showed intensity of IgA deposition in glomeruli in model group was ++∼+++. Conclusions: Rat IgAN model is successfully established by the improved method of BSA+CCl4+LPS, whose clinical index and pathology are similar to the human IgAN.

Age Sex/Eth Pre-Bx syst.symp Time

Pre-Bx renal symp

Time

16 7 14 9 16

M/AA M/AA F/AA F/Chin F/AA

NephrSynd, ↑BP,↓GFR NephrSynd,↓GFR Uprot NephrSynd Uprot,↓GFR

2 wks 1 wk 4 wks 1 wk 1 wk

C3-4/ Antibod ↓/⊕ ↓⊕ N/⊕ ↓/⊕ N/-

5 4 2 5 4

17

F/His

6 mo

NephrSynd

5 wks

↓/⊕

5

19 15 17 13 18

M/His F/AA F/AA F/AA M/AA

6 mo 6 wks 2 wks 6 mo 8 days

NephrSynd,↓GFR NephrSynd NephrSynd,↑BP NephrSynd,↓GFR,↑BP NephrSynd,↓GFR,↑BP

2 wks 2 wks 2 wks 1 wk 3 days

↓/⊕ ↓/⊕ ↓/⊕ ↓/⊕ ↓/⊕

4 5 4 4 4

Abd pain Fever,↑lymph Arthr,↓WBC ↓Plat,rash Myalg,fever Arthr,RVT,lung thrombi Arthr,rash,fever Edema Malaise Abd pain,vomit Diarrh,fever

6 mo 10 mo 4 yr 6 yr 10 mo

Class

Conclusions: Lupus nephritis can be preceded by a protean assortment of systemic symptoms lasting from several months to years. During this time, renal dysfunction may already be present and its detection could be renal preserving. A high index of suspicion for nephritis should be considered when children present with prolonged imprecise symptoms that could be lupus.

Abstract# 59 Descriptive Study of Biopsy Proven IgA and Henoch Schonlein Purpura (HSP) Nephropathy in Two Government Hospitals in Johannesburg, South Africa J.G. Mitchell,1 U.K. Kala,1 D. Hahn.2 1 Paediatrics, University of the Witwatersrand and Chris Hani Baragwanath Hospital, Johannesburg, Gauteng, South Africa; 2Paediatrics, University of the Witwatersrand and Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, Gauteng, South Africa. Objectives: Determine if IgA and Henoch Schonlein Purpura(HSP) nephropathies occur less frequently in South African Black Children and the disease progression is worse. Methods: A retrospective review of biopsy proven IgA or HSP nephropathy presenting at two paediatric nephrological services in two academic hospitals namely Chris Hani Baragwanath and Charlotte Maxeke Johannesburg Academic Hospitals in Johannesburg from January 1985 to December 2008 in children ≤ 16 years. Results: Total of 1835 renal biopsies of which 51 (3%) confirmed HSP or IgA nephropathy. 1 excluded from analysis due to inadequate records. Average age of presentation was 9.5 years. All children well nourished. M:F ratio 2.2:1. HSP group n=17(34%) and IgA group n=33 (66%).Racial breakdown, Black n=28 (56%), Caucasian n=15(30%), Asian n=4(8%) and Mixed races n=3(9%). Average calculated GFR (Swartz formula) was 100ml/min/1.73m2.Commonest presenting symptom was haematuria macroscopic >macroscopic.50% had nephrotic range proteinuria. 22% (11) presented in acute renal failure 10 (91%) of which were IgA. Histological grading, 26% grade 1, 28% grade 2, 28% grade 3, 12% grade 4 and 6% grade5. Average follow up of 3 years.5 children dialysed, 2 in ESRF received transplants, 3 demised. Various treatment regimes used. Conclusions: Neither race nor sex indicated poorer prognosis. No difference in outcome of IgA and HSP nephritis. No correlation between presenting symptoms and outcome based on disease activity.

Abstract# 57 Changes and Significance of Podocyte-Associated Molecules in Rats with IgA Nephropathy H.-y. Lu, X.-Y. Jiang, L.-z. Chen, Y. Mo, S.-m. Chen. Depatment of Pedaitrics, The First Affiliated Hospital of Sun Yatsen University, Guangzhou, Guangdong, China. Objectives: To observe the expression and distribution of nephrin, podocin, CD2AP and desmin in rats with IgAN, and to explore the mechanism of proteinuria of IgAN. Methods: SD rats were divided into control group and model group. IgAN model was induced by the method of BSA+CCl4+LPS. Expression and distribution of nephrin, podocin and desmin were examined by indirect immunofluorescence. The mRNA expression of nephrin, podocin, CD2AP and desmin were examined by RT-PCR. Results: (1) In model group, proteinuria increased at the 6th week and persisted up to the 12th week. (2) Podocin and nephrin increased from the 2nd and 4th week individually to the 8th week, then decreased, but higher than control group at the 12th week. Podocin and nephrin staining gradually shifted from a linear-like pattern along the capillary loop of glomeruli to a discontinuous patch or gobbet pattern. Expression of desmin increased at the 2nd week and elevated up to the 12th week. (3) Nephrin, podocin and CD2AP mRNA were up-regulated at the 2nd, 2nd and 4th week individually, which persisted increasing to the 8th week, and thereafter returned to control level at the 12th week. Desmin mRNA increased at the 8th week, then recovered again, but it was still higher than control group at the 12th week. (4) Nephrin, podocin and mRNA, CD2AP mRNA and desmin mRNA were all positively correlated with urinary protein. Conclusions: Changes of expression and abnormality of distribution of podocyteassociated molecules may be the cause of proteinuria of IgAN. Change of podocin may be the trigger of proteinuria, and change of CD2AP may be caused by the change of nephrin and podocin.

Abstract# 60 5% vs 20% Albumin in Nephrotic Syndrome M. Kanitkar, A. Garg, V. Venkateshwar. Department of Pediatrics, Armed Forces Medical College, Pune, India. Objectives: Compare efficacy of 5% Vs 20% albumin for edema in nephrotic syndrome. Methods: 24 nephrotics 15 to144 months with anasarca randomised to two groups; Group A(n=14) received 20% albumin followed by 5% and group B(n=10) vice versa with a wash out period of 48 hours.Baseline and post therapy vitals, fluid intake, urine output and biochemistry noted. Frusemide given midway to all. Data tabulated for 20% Group 1(n=24) and 5% Group2(n=24). Paired ‘t’ test used for efficacy of either 5% or 20% and unpaired ‘t’ test to compare difference between the two. The effect of sequential use of 5% and 20 % was analysed for Groups A and B. Results: Baseline and post transfusion FeNa was 0.7±0.72%, 0.73±0.76% and 1.46±1.1%,1.73±2.3% in groups 1 & 2.Urine output and change in weight in the two groups depicted and the effect of sequential use of albumin.

Abstract# 58 The Protean Nature of Childhood SLE May Cause a Delay in the Diagnosis of Lupus Nephritis R. Jodorkovsky, K. Bromberg. Pediatrics, The Brooklyn Hospital Center, Brooklyn, NY, United States. Objectives: Lupus nephritis occurs in 80% of childhood-onset SLE. Systemic symptoms of lupus are non-specific. Given non-specific symptoms clinicans may not look for renal dysfunction and possibly delay the treatment of lupus nephritis. Methods: Demographics, pre-renal biopsy systemic and renal presenting symptoms, their duration, and pathology diagnosis were reviewed in all patients diagnosed with lupus nephritis from 2005-2009. Results: There were 11 new cases. The diagnosis was confirmed between 5 months to 6 years after the onset of systemic symptoms in the majority of cases. During this time, several children did not appear to have had renal testing. Renal pathology was severe in most cases.

Table 1 Effect of Albumin Infusion (n=24) Variables Group1 (5%Albumin) Group2 (20%Albumin) ‘t’ 2.25±2.12 3.68±3.84 1.6 ∆%Wt loss 1.66±0.95 .47 ∆Urine output(ml/kg/hr) 1.52±1.11 Table 2 Effect of sequential use of albumin Gp A Vs Gp B* Variable ∆Urine output treatment effect ∆Urine output periodic effect ∆Wt loss treatment effect ∆Wt loss periodic effect

t value -1.768 2.876 -2.5318 1.8895

p value 0.0909 .0088 0.01 .07

*Gp A(14)- 5% followed by 20% albumin Gp B(10)- 20% followed by 5% albumin

26

p value .12 .64

GLOMERULAR DISEASE

1807 Methods: We reviewed the medical records of five children with unexplained mental retardation and nephrotic syndrome. Results: All patients showed microcephary with diffuse cortical atrophy. Four of the five had infantile spasms. Three developed nephrotic syndrome before two years of age and died from renal failure at early ages(cases 1, 2 and 3). The other two children developed nephrotic syndrome after four years of age and remain alive without renal dysfunction. Nephrotic syndrome was steroid-resistant in all cases. Renal pathology on light microscopy showed diffuse mesangeal hypercellularity (cases 1 and 2), FSGS (case 3), MPGN like with mesangiolysis (case 4) and extracapillary glomerulonephritis (case 5). All patients had effacement of foot processes and two had irregular thickness of the glomerular basement membranes (cases 1 and 4). Conclusions: The three patients with early onset nephrotic syndrome were clinically diagnosed with typical GMS while the other two patients may have had a milder form. Some podocyte proteins involved in the formation of cellular processes and signal transduction are known to be expressed in the kidneys and brain. It is possible that some unknown mutations in podocyte proteins are responsible for the proteinuria and brain anomalies seen in GMS.

Abstract# 61 Mycophenolate Mofetil (MMF) Treatment in Paediatric Onset Systemic Lupus Erythematosus (SLE) I. Kazyra, C. Pilkington, S.D. Marks, K. Tullus. Paediatrics, 2nd Children’s Hospital, Belarus State Medical University, Minsk, Belarus; Paediatric Nephrology, Great Ormond Street Hospital, London, United Kingdom. Objectives: We present our safety and efficacy data on the use of MMF treatment in children with SLE and lupus nephritis (LN). Methods: 31 children and adolescents, aged 5-22 (median 16) years with SLE were treated with MMF at GOSH, London, UK. 22/31 patients had biopsyproven LN. Treatment outcome was monitored according the British Isles Lupus Assessment Group (BILAG) index, blood and urine parameters. Group 1 were commenced on MMF induction and/or maintenance therapy (n=15) and Group 2 were converted from azathioprine (AZA) due to inadequate disease control (n=16). Results: 77% of all (67% group 1 and 88% group 2) patients experienced an improvement in BILAG at 12 months after initiation of MMF treatment (p < 0.05). Group 1 with low C3, C4 increased their C3 (from 0.53 to 1.15) and C4 (0.08 to 0.17g/l) levels significantly (p