Volume 79 (2013), No. 6/2013(June)
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Original
A randomized trial of intensified vs. standard dosing for enteric-coated mycophenolate sodium in de novo kidney transplant recipients: results at 1 year
Wolfgang Arns, Claudia Sommerer, Petra Glander, Toofan Ariatabar, Martina Porstner, Christoph May, Eva-Maria Paulus, Maria Shipkova, Wolfgang Fischer, Lutz Liefeldt, Ruth Hackenberg, Peter Schemmer, Sophie Domhan, Martin Zeier and Klemens Budde
Page No. 421
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (421-431)
A randomized trial of intensified vs. standard dosing for enteric-coated mycophenolate sodium in de novo kidney transplant recipients: results at 1 year
Wolfgang Arns1, Claudia Sommerer2, Petra Glander3, Toofan Ariatabar1, Martina Porstner4, Christoph May4, Eva-Maria Paulus4, Maria Shipkova5, Wolfgang Fischer4, Lutz Liefeldt3, Ruth Hackenberg1, Peter Schemmer6, Sophie Domhan2, Martin Zeier2 and Klemens Budde3
1Department of Nephrology, Medical Clinic Köln-Merheim, Cologne, 2Department of Nephrology, Heidelberg University Hospital, Heidelberg, 3Department of Nephrology, Charité University Hospital Berlin, Berlin, 4Novartis Pharma GmbH, Nuremberg, 5Department of Clinical Chemistry and Laboratory Medicine, Stuttgart, and 6Department of General, Visceral and Transplant Surgery, University of Heidelberg, Germany
In a 6-month prospective, openlabel, multicenter study, 128 de novo kidney transplant patients receiving cyclosporine (CsA) and steroids were randomized to an intensified regimen of enteric-coated mycophenolate sodium (EC-MPS) or to a standard EC-MPS regimen to Week 6 posttransplant, after which the regimen was identical. In a follow-up study to Month 12 post-transplant (49 intensified regimen, 52 standard regimen), the reduced rate of BPAR observed at Month 6 (intensified regimen 3.2%, standard regimen 16.9%, p = 0.016) was maintained at Month 12 (4.8% vs. 18.5%, p = 0.026). Estimated GFR (Cockcroft-Gault) at Month 12 was comparable in the intensified group (mean (SD) 54.8 (22.9) ml/min) vs. the standard group (mean (SD) 57.5 (23.6) ml/min, p = 0.83). The incidence of adverse events and serious adverse events at Month 12 was similar in both treatment groups, although adverse events with a suspected relation to study drug were reported in 69.8% and 50.8% of patients in the intensified and standard regimen groups, respectively (p = 0.032). Infections and hematological parameters were similar between groups. In conclusion, an early regimen of intensified EC-MPS with CsA and steroids achieves a low rate of BPAR over the first year after kidney transplantation with similar renal function to a standard regimen, and without a clinically relevant impact on safety.Correspondence to:
Dr. Wolfgang Arns
Department of Nephrology
Medical Clinic Köln-Merheim
Ostmerheimer Str. 200
51109 Cologne, Germany
Email: [email protected]
Original
Treatment of childhood nephrotic syndrome with long-term, low-dose tacrolimus
Margret E. Bock, Richard A. Cohn and Farah N. Ali
Page No. 432
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (432-438)
Treatment of childhood nephrotic syndrome with long-term, low-dose tacrolimus
Margret E. Bock, Richard A. Cohn and Farah N. Ali
Division of Kidney Diseases, Ann and Robert H. Lurie Children’s Hospital of Chicago; Northwestern University, Feinberg School of Medicine, Chicago, IL, USA
Aim: Children with steroidresistant (SR) and steroid-dependent (SD) nephrotic syndrome (NS) pose a treatment challenge. Literature on the use of tacrolimus (TAC), a calcineurin inhibitor, for maintenance treatment of NS is sparse. We aimed to evaluate the efficacy and safety of low-dose, long-term TAC for inducing and sustaining remission in children with SD/SR NS. Methods: Data from patients treated at our center from 1999 to 2009 were analyzed. Results: 40 patients with NS were treated with TAC for 3 – 80-month periods (median 25.2 months). Diagnoses included focal segmental glomerulosclerosis (FSGS) (60%), IgM nephropathy (15%), minimal change disease (20%) and membrano-proliferative glomerulonephritis (MPGN) (5%). 58% of patients had been previously treated with alternate agents. After 1, 2, and 3 years on TAC, complete remission was achieved in 26%, 48%, and 29% of patients; complete or partial remission was achieved in 85%, 100%, and 86%, respectively (p < 0.05). Median time to remission was 41 days (range: 10 – 270 days). FSGS and SR diseases were associated with lower likelihood of remission (p < 0.05). Remission was equally likely in both treatment naïve patients and those who had received prior second-line agents. Conclusion: Our results demonstrate that TAC treatment for children with SR/SD NS is associated with high rates of sustained remission, even when prior second-line agents failed.Correspondence to:
Margret E. Bock, MD
Division of Kidney Diseases
Ann and Robert H. Lurie Children’s Hospital of Chicago
Northwestern University
Feinberg School of Medicine
225 E Chicago Ave, Box 37, Chicago, IL 60618, USA
Email: [email protected]
Original
Use of enteric coated mycophenolate sodium in adult resistant nephrotic syndrome
Miguel Angel Nadal, Bruno Lococo, Maria Bernarda Fazzini, Gabriela Gonzalez, Alcira Beatriz Otero and Ana Valeria Malvar
Page No. 439
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (439-444)
Use of enteric coated mycophenolate sodium in adult resistant nephrotic syndrome
Miguel Angel Nadal1, Bruno Lococo2, Maria Bernarda Fazzini2, Gabriela Gonzalez1, Alcira Beatriz Otero3 and Ana Valeria Malvar2
1Daomi Institute, 2Hospital Fernandez, and 3Novartis Argentina SA, Buenos Aires, Argentina
Introduction: Treatment of patients with resistant/relapsed adult nephrotic syndrome (RNS) caused by glomerulopathies has no consensus therapy. Objectives: This is a retrospective analysis (RA), performed on 55 patients with RNS treated during one year with enteric coated sodium mycophenolate (EC-MPS) and reduced corticosteroids doses. Material and methods: Inclusion criteria for this RA were: patients aged ≥ 18 years old, diagnosed with RNS with histologically proven glomerulopathy who had received standard therapy with enalapril and/or losartan and 10 mg per day or 20 mg of prednisone every other day. NS was defined with the following criteria: proteinuria > 3.5 g/day, serum albumin ≤ 3 g/dl, hypercholesterolemia and edema. Treatment consisted of oral EC-MPS in 360 mg tablets, 720 mg bid, together with prednisone 10 mg daily or 20 mg every other day. Effectiveness was assessed as the rate of response in the cohort: complete, partial or absent. Complete response patients: 24 hours proteinuria < 300 mg/day, partial response patients: proteinuria > 300 mg/day and < than 3 g/day, all the rest were considered as non responders. Results: response was achieved in 40/55 (73%) of patients, 24 (44%) with complete response and 16 (29%) with partial response. No EC-MPS discontinuation has been observed due to adverse events, except for one case of transient interruption of medication for 2 weeks. Conclusion: EC-MPS as single therapy with minimal doses of corticosteroids as in this RA could be an effective alternative in the treatment of patients with RNS.Correspondence to:
Dr. Miguel Angel Nadal
Daomi Institute
Lavalle 4094, C1190ABB, Buenos Aires, Argentina
Email: [email protected]
Original
Long-term outcome and prognostic factors of idiopathic membranous nephropathy in the Chinese population
Ke Zuo, Yan Wu, Shi-Jun Li, Feng Xu, Cai-Hong Zeng and Zhi-Hong Liu
Page No. 445
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (445-453)
Long-term outcome and prognostic factors of idiopathic membranous nephropathy in the Chinese population
Ke Zuo, Yan Wu, Shi-Jun Li, Feng Xu, Cai-Hong Zeng and Zhi-Hong Liu
Research Institute of Nephrology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China
Background: Idiopathic membranous nephropathy (IMN) is a representative form of nephrotic syndrome in China. Although IMN is thought to run a more benign course in Asian patients than in the Caucasian population, there has been no persuasive study to determine the long-term prognosis and risk factors for IMN in the Chinese population. Methods: A retrospective chart review. All patients admitted to Nanjing Institution of Nephrology from January 1985 to December 2007 with biopsy-proven IMN were enrolled. The primary outcome was the renal survival rate and risk factors at renal biopsy. Result: A total of 217 patients were included in the study, and the overall renal survival rates were 96.9%, 93.5%, and 86.6% at 5, 10, and 15 years after renal biopsy, respectively. When the clinical features at biopsy were evaluated, patients with hypertension (p = 0.023), decreased eGFR (p < 0.001), nephrotic-range proteinuria (p = 0.047), elevated urinary NAG (p = 0.045) and RBP (p = 0.007) had a worse prognosis. Cox multivariate analysis showed that decreased eGFR and chronic tubulointerstitial lesion were independent risk factors for ESRF (end-stage renal failure). Conclusion: IMN is a disease with a comparatively good prognosis in the Chinese population, with a renal survival rate of more than 90% at 10 years after renal biopsy. Decreased eGFR at biopsy and chronic tubulointerstitial lesion are independent risk factors of ESRF. Partial or complete remission of proteinuria improved the prognosis.Correspondence to:
Zhi-Hong Liu, MD
Professor of Medicine
Research Institute of Nephrology
Jinling Hospital, University School of Medicine
Nanjing 210002, China
Email: [email protected]
Original
Assessment of genetic risk factors for thromboembolic complications in adults with idiopathic nephrotic syndrome
Melisa Sahin, Sultan Ozkurt, Nevbahar Akcar Degirmenci, Ahmet Musmul, Gokhan Temiz and Mehmet Soydan
Page No. 454
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (454-462)
Assessment of genetic risk factors for thromboembolic complications in adults with idiopathic nephrotic syndrome
Melisa Sahin, Sultan Ozkurt, Nevbahar Akcar Degirmenci, Ahmet Musmul, Gokhan Temiz and Mehmet Soydan
Division of Nephrology, Faculty of Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey
Aims: Nephrotic syndrome (NS) may occur with acquired hypercoagulability, however, the fact that it is accompanied by an underlying hereditary thrombophilia, especially combined hereditary thrombophilia would lead to thrombotic events. In this study, we aimed to evaluate the contribution of genetic thrombophilia to development of thrombotic events in adult patients with NS. Material and methods: Factor V Leiden (FVL), prothrombin, and methylenetetrahydrofolate reductase (MTHFR) gene mutation were studied in 51 newly diagnosed idiopathic NS patients and age- and gender-matched 20 healthy control subjects included in the study. Renal vein Doppler ultrasound was conducted in order to investigate the prevalence of subclinical renal vein thrombosis. Results: Of 51 patients, 6 (11.8%) were established to have thromboembolic (TE) complications at the time of diagnosis (4 symptomatic, 2 subclinical), and no recurring thrombotic episode was observed. Genetic mutation was established in all patients that were found to have TE complications. Acquired hypercoagulability factors were similar in patients without and with TE complication. Conclusions: The coexistence of inherited thrombophilia in NS may facilitate thromboembolic complications. If the cause of thrombosis cannot be explained by the usual factors attributed to the occurrence of thrombosis in NS, screening for the other factors, such as FVL, MTHFR, and prothrombin gene mutation, may be beneficial.Correspondence to:
Sultan Ozkurt, MD
Division of Nephrology
Faculty of Medicine
Eskisehir Osmangazi University
Eskisehir, Turkey
Email: [email protected]
Original
Direct hemoperfusion with a polymyxin B column versus vasopressin for gram negative septic shock: a matched cohort study of the effect on survival
Naoki Sawa, Yoshibumi Ubara, Keiichi Sumida, Rikako Hiramatsu, Eiko Hasegawa, Masayuki Yamanouchi, Junichi Hoshino, Tatsuya Suwabe, Naoyuki Uchida, Atsushi Wake, Shuichi Taniguchi and Kenmei Takaichi
Page No. 463
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (463-470)
Direct hemoperfusion with a polymyxin B column versus vasopressin for gram negative septic shock: a matched cohort study of the effect on survival
Naoki Sawa1, Yoshibumi Ubara3, Keiichi Sumida3, Rikako Hiramatsu1, Eiko Hasegawa1, Masayuki Yamanouchi1, Junichi Hoshino3, Tatsuya Suwabe3, Naoyuki Uchida2, Atsushi Wake2, Shuichi Taniguchi2 and Kenmei Takaichi1
1Department of Nephrology, 2Department of Hematology, Toranomon Hospital, Tokyo, Japan, and 3Department of Nephrology, Toranomon Hospital Kajigaya, Kawasaki, Japan
Background: Vasopressin and direct hemoperfusion with an immobilized polymyxin B column (PMX) have emerged recently as treatments for septic shock. This study assessed the impact of these two treatments on the survival of patients with septic shock. Methods: A retrospective, matched cohort study was performed to compare patients who had septic shock and were treated with PMX or vasopressin. The primary endpoint was the 90-day mortality rate. 30 patients receiving PMX were matched to 30 treated with vasopressin. Results: The 90-day survival rate was significantly higher in the vasopressin group than the PMX group (83% vs. 53%; p = 0.008). In the PMX group, the subgroup with gastrointestinal surgery had a significantly higher survival rate than the subgroup without surgery (76.9% vs. 52.9%; p = 0.01). Cox multivariate analysis showed that vasopressin therapy (versus PMX; HR = 0.27; p < 0.01). The estimated hazard ratio for the SOFA score was 1.44 (p < 0.01), and total pressor dosage an baseline was 1.26 (p = 0.01). Conclusion: We found a significant reduction of the mortality rate in septic shock patients who received vasopressin compared with PMX. In the PMX group, the subgroup with gastrointestinal surgery had a significantly higher survival rate than the subgroup without such surgery.Correspondence to:
Naoki Sawa, MD
Nephrology Center, Toranomon Hospital
2-2-2, Toranomon, Minatoku, 105-0087, Tokyo, Japan
Email: [email protected]
Original
In vitro phosphate-binding ability of calcium-based agents is augmented by co-administration of activated charcoal
Meng Jia, Xu-yang Cheng and Li Zuo
Page No. 471
Abstract
In vitro phosphate-binding ability of calcium-based agents is augmented by co-administration of activated charcoal
Meng Jia1,2,3, Xu-yang Cheng1,2,3 and Li Zuo1,2,3
1Renal Division, Department of Medicine, Peking University First Hospital, 2Peking University Institute of Nephrology and 3Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China
Background: Calcium carbonate is widely used as a phosphate binder in patients on maintenance hemodialysis. An unwanted side effect of calcium carbonate is hypercalcemia and vascular calcification. Oral activated charcoal (AC) is a non-selective and highly effective adsorbent. We hypothesized that AC augments the phosphate binding capacity of calcium-based agents. We performed an in vitro study to test this hypothesis. Methods: Simulated gastric fluid and intestinal fluid were prepared with a phosphate concentration of 10 mmol/l. Different dosages of calcium chloride (0.083 g, 0.167 g, and 0.250 g), AC (0.15 g, 0.30 g, and 0.45 g) or a combination of both were added to either gastric or intestinal fluid for phosphate binding. After a reaction time of 2 hours, phosphate concentrations in the supernatant were measured, and absolute reduction and percent reduction of phosphate were calculated. The phosphate-binding abilities of calcium chloride, AC, and a combination of both were compared. Results: In simulated intestinal fluid there was no significant difference in the percent reduction of phosphate concentrations among the different calcium chloride concentration groups (28.90 ± 2.04 vs. 33.33 ± 3.90 vs. 31.86 ± 5.23) and there was still no significant difference in phosphate concentrations among the different AC groups (3.33 ± 0.08 vs. 3.26 ± 0.01 vs. 3.36 ± 0.11). In simulated gastric fluid phosphate concentrations at each of the time points (before the reaction, 1 hour after calcium chloride was added, and 2 hours after AC was added) were not significantly different. In simulated intestinal fluid the percent decrease in phosphate concentration in the calcium chloride + AC group was significantly higher than that in the calcium chloride group (48.23 ± 5.55 vs. 30.72 ± 6.11). Conclusions: AC alone had no phosphate-binding ability in either gastric or intestinal fluid. The phosphate-binding ability of calcium chloride was improved by AC in intestinal fluid.Correspondence to:
Li Zuo, MD
Renal Division, Peking University First Affiliated Hospital
Institute of Nephrology, Peking University
8 Xishiku Street, Xichen District
Beijing, 100034, China
Email: [email protected]
Original
Malnutrition-inflammation score predicts long-term mortality in Chinese PD patients
Ting He, Xin An, Hai-Ping Mao, Xin Wei, Jie-Hui Chen, Na Guo, Xiao Yang, Zhi-Bin Li, Xue-Qing Yu and Zhi-Jian Li
Page No. 477
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (477-483)
Malnutrition-inflammation score predicts long-term mortality in Chinese PD patients
Ting He1,2*, Xin An1,2,3*, Hai-Ping Mao1,2, Xin Wei1,2, Jie-Hui Chen1,2, Na Guo1,2, Xiao Yang1,2, Zhi-Bin Li1,2,4, Xue-Qing Yu1,2 and Zhi-Jian Li1,2
1Department of Nephrology, The First Affiliated Hospital, 2Key laboratory of the Ministry of Education, 3Department of Medical Oncology, Cancer Center, and 4Epidemiology and Clinical Research Unit, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
*These authors contributed equally to this work.
Background: Malnutrition–Inflammation Score (MIS) has proved to predict the prospective mortality in maintenance hemodialysis (MHD) patients. However, its value of long-term mortality predictability in peritoneal dialysis (PD) patients has not been adequately studied. Methods: A total of 155 chronic stable PD patients from November 2005 to December 2006 were enrolled. At baseline, the MIS, Subjective Global Assessment (SGA), as well as clinical, laboratory, and anthropometric parameters were recorded. All patients were followed until October 2009 to evaluate mortality as a primary outcome. Results: The MIS correlated very well with SGA and other nutrition and inflammation markers. Patients with a higher MIS had a worse survival rate compared to those with lower MIS. After adjusting for potential confounding factors, one unit increase of MIS was associated with a 1.27-fold greater death risk (hazard ratio: 1.27, 95% confidence interval: 1.19 – 1.36; p < 0.001). MIS had a superior mortality predictability compared with SGA. Moreover, univariate and multivariate analyses denoted MIS, age, dialysis vintage, and comorbidities as independent predictors of total mortality. Conclusion: MIS is a promising marker for malnutrition inflammation assessment and an independent predictor of long-term mortality in Chinese PD patients.Correspondence to:
Zhi-Jian Li, MD, PhD
58 Zhongshan 2nd Road
Guangzhou, 510080 China
Email: [email protected]
Nephrology Education
Different phenotypes of HNF1β deletion mutants in familial multicystic dysplastic kidneys
Masafumi Hasui, Kazunari Kaneko, Shoji Tsuji, Yuka Isozaki, Takahisa Kimata, Yoshimi Nozu, Kandai Nozu and Kazumoto Iijima
Page No. 484
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (484-487)
Different phenotypes of HNF1β deletion mutants in familial multicystic dysplastic kidneys
Masafumi Hasui1, Kazunari Kaneko1, Shoji Tsuji1, Yuka Isozaki1, Takahisa Kimata1, Yoshimi Nozu2, Kandai Nozu2 and Kazumoto Iijima2
1Department of Pediatrics, Kansai Medical University, Osaka and 2Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
Multicystic dysplastic kidney (MCDK) is one of the most common congenital abnormalities of the kidney and urinary tract (CAKUT), although its pathophysiology remains unknown. Familial occurrence of MCDK suggests that mutations in genes associated with nephrogenesis are involved in the pathogenesis in at least some cases. Hepatocyte nuclear factor 1β (HNF1β) is a member of the homeodomain-containing super family of transcription factors, and is known to regulate tissue-specific gene expression in a number of organs including the kidneys, pancreas and liver. It has been recently postulated to be associated with CAKUT, including MCDK. We recently encountered a family with a deletion mutant of HNF1β, of which the 2nd son, the proband, developed bilateral MCDK resulting in renal loss of function in infancy while the 1st son developed unilateral MCDK. Their father has two normal kidneys. This family confirmed that mutations in the HNF1β gene are strongly associated with the development of MCDK. Furthermore, the fact that no clear phenotype-genotype correlation exists suggests that gene(s) other than HNF1β are also involved in nephrogenesis and the development of MCDK.Correspondence to:
Kazunari Kaneko, MD
Department of Pediatrics
Kansai Medical University
2-3-1, Shin-machi, Hirakata-shi
Osaka 573-1191, Japan
Email: [email protected]
Nephrology Education
Membranous glomerulonephritis associated with splenic marginal zone lymphoma mimicking multiple myeloma
Gyl E.B. Silva, Roberto S. Costa, Fernando Chahud, Osvaldo M. Vieira Neto, Miguel Moyses-Neto, Elen A Romão and Marcio Dantas
Page No. 488
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (488-493)
Membranous glomerulonephritis associated with splenic marginal zone lymphoma mimicking multiple myeloma
Gyl E.B. Silva1, Roberto S. Costa1, Fernando Chahud2, Osvaldo M. Vieira Neto3, Miguel Moyses-Neto3, Elen A Romão3 and Marcio Dantas3
1Renal Pathology and 2Hematopathology Divisions of the Department of Pathology, 3Division of Nephrology, Faculty of Medicine of Ribeirão Preto, University of São Paulo (USP), São Paulo, Brazil
Glomerulonephritis may complicate the course of a wide variety of malignant diseases. However, there are relatively few reports of membranous glomerulonephritis (MGN) in patients with non-Hodgkin lymphoma (NHL). We describe for the first time a case of MGN associated with splenic marginal zone lymphoma with extreme plasmacytic differentiation and bone marrow infiltration mimicking multiple myeloma. We also reviewed the literature and summarize the clinical-pathological findings and the mechanisms involved in NHL-induced MGN. Our current case highlights the importance of a quick and correct diagnosis of the underlying disease and the value of a thorough physical examination. Clinicians should be aware of the possibility of an underlying hematologic malignancy in such cases, particularly in elderly patients with renal biopsy that shows the presence of atypical histology.Correspondence to:
R.S. Costa, PhD
Department of Pathology
Faculty of Medicine of Ribeirão Preto, USP
Av. Bandeirantes 3900, CEP 14049-900, SP, Brazil
Email: [email protected]
Nephrology Education
Proliferative glomerulonephritis with monoclonal IgG deposits in a patient with autoimmune hemolytic anemia
Takashi Fujiwara, Atsushi Komatsuda, Hiroshi Ohtani, Masaru Togashi, Ken-ichi Sawada and Hideki Wakui
Page No. 494
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (494-498)
Proliferative glomerulonephritis with monoclonal IgG deposits in a patient with autoimmune hemolytic anemia
Takashi Fujiwara1, Atsushi Komatsuda1, Hiroshi Ohtani2, Masaru Togashi1, Ken-ichi Sawada1 and Hideki Wakui1
1Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, and 2Department of Nephrology and Dialysis, Akita Kumiai General Hospital, Akita, Japan
A 25-year-old woman was admitted because of proteinuria. A renal biopsy showed mesangial/endocapillary proliferative glomerulonephritis with IgG2-κ deposits. Electron microscopy showed immune complex-type deposits. She also had Coombs-positive hemolytic anemia, anticardiolipin antibodies, and antinuclear antibodies. Middle-dose steroid therapy led to improvement of proteinuria and hemolytic anemia. Six years later, she developed crescentic glomerulonephritis with IgG2-κ deposits during pregnancy. Middle-dose steroid therapy improved renal dysfunction. This is an exceptional case of proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID), a recently described rare dysproteinemia-related glomerulonephritis, associated with autoimmune disease. This case also suggests that crescentic glomerulonephritis can be superimposed on PGNMID.Correspondence to:
Atsushi Komatsuda, MD
Department of Hematology, Nephrology, and Rheumatology
Akita University Graduate School of Medicine
1-1-1 Hondo, Akita City, Akita 010-8543, Japan
Email: [email protected]
Nephrology Education
A patient with hemodialysis-related hyperammonemic encephalopathy: a delayed presentation of congenital arterioportal fistulas
Shang-Feng Yang, Hsiou-Shan Tseng, Hui-Chun Huang, I-Fang Hsin, Yen-Hung Yao and Jinn-Yang Chen
Page No. 499
Abstract
Clinical Nephrology, Vol. 79 – No. 6/2013 (499-503)
A patient with hemodialysis-related hyperammonemic encephalopathy: a delayed presentation of congenital arterioportal fistulas
Shang-Feng Yang1, Hsiou-Shan Tseng2,5, Hui-Chun Huang3,5, I-Fang Hsin3, Yen-Hung Yao4 and Jinn-Yang Chen1,5
1Division of Nephrology, Department of Medicine, 2Department of Radiology, 3Division of Gastroenterology, Department of Medicine, Taipei Veterans General Hospital, Taipei, 4Division of Nephrology, Department of Medicine, National Yang-Ming University Hospital, Yilan, and 5School of Medicine, National Yang-Ming University, Taipei, Taiwan
We present a 32-year-old woman who developed hyperammonemic encephalopathy during hemodialysis. She was rather well before 2009 when receiving peritoneal dialysis due to chronic interstitial nephritis. Due to a refractory peritonitis, the treatment was shifted to hemodialysis in January 2009. About 1 year later, she was found with consciousness disturbance during hemodialysis then admitted to the hospital because of hyperammonemia (165 μg/dl). During hospitalization, the patient’s abdominal Doppler sonography showed a hepatofugal flow in the portal trunk while the hepatic artery angiography demonstrated multiple intrahepatic arterioportal fistulas. Her general condition was improved after the treatment of lactulose and hepatic artery embolization. With the occurrence of arterioportal fistulas induced portal hypertension, we speculated that the portal-systemic shunt was enhanced during hemodialysis because of venous hypotension which then resulted in the transient hyperammonemia. To the best of our knowledge, this is the first patient who developed hemodialysis- related hyperammonemic encephalopathy due to multiple arterioportal fistulas.Correspondence to:
Jinn-Yang Chen, MD, PhD
Division of Nephrology, Department of Medicine
Taipei Veterans General Hospital
No. 201, Sec. 2, Shih-Pai Road, Taipei 112, Taiwan
Email: [email protected]
