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Abstract

M. Ibernon, R. Poveda, A. Vidaller, O. Bestard and J.M. Grinyó

Departments of ¹Nephrology and ²Medicine, Hospital Universitari de Bellvitge, Barcelona, Spain
Background: Standard therapy with corticosteroids and cyclophosphamide followed by azathioprine has improved renal and patient survival in renal vasculitis. However, this regimen is associated with high toxicity. Mycophenolate mofetil (MMF), a less toxic immunosuppressive drug, has been proposed as a therapeutic alternative. Methods: We report 12 patients (4 males, 8 females, aged 65.6 ± 12.1 years) with anti-MPO renal vasculitis who were switched from standard therapy to MMF because of drug-related adverse effects: leukopenia, toxic hepatitis, nausea, hair loss or appearance of carcinoma. MMF was introduced at a dose of 500 mg/8 h, after 83 ± 56 days under standard therapy. Results: After 354 ± 195 days of MMF therapy, all patients maintained clinical remission. Mean values of serum anti-MPO, disease activity markers and serum creatinine decreased when these values were compared from pre-therapy to the time of switching to MMF, and then to the end of the study anti-MPO: 204 ± 144 U, 54 ± 85 U and 12 ± 5 U. Serum-reactive C protein 97 ± 82 mg/l, 13 ± 10 mg/l and 4 ± 2 mg/l. Erythrocyte sedimentation rate 88 ± 40, 41 ± 28 and 26 ± 15 mm. Serum creatinine 415 ± 238, 202 ± 93 and 169 ± 104 mmol/l. In one case there was a relapse of vasculitis under MMF and a low dose of prednisone after 9 months of therapy. Side effects were herpes infection in four cases and chickenpox in one. Neither leukopenia nor anemia was observed. Conclusions: These results indicate that MMF could be an alternative therapy for anti-MPO renal vasculitis associated with cyclophosphamide or azathioprine-related toxicity.Correspondence to:
M.I. Vilaró, MD; Nephrology Department, Hospital Universitari de Bellvitge, Feixa Llarga s/n. L’Hospitalet del Llobregat 08907, Barcelona, Spain
Email: 35055miv@comb.es

Abstract

Y. Nakayama, T. Inoue, Y. Kohda, H. Inoue, Y. Izumi, K. Tomita and H. Nonoguchi

Department of Nephrology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
Aims: Recently, we reported the effectiveness of PAC therapy, a combination therapy with prostaglandin (PG) and angiotensin-converting enzyme inhibitor (ACE-I), as a new tool for the prevention of chronic kidney disease. In the current study, we continually treated these patients with or without PG and analyzed the survival rate of renal function by Kaplan-Meier method and Cox regression analysis. Material and methods: 52 patients (serum creatinine 2.9 ± 1.9 mg/dl) were followed-up for 48 months. 26 patients continued to receive ACE-I monotherapy and the remaining 26 patients were treated by PAC therapy. Primary end-point was defined as a decrease in 1/Cr by 0.2 (dl/mg), initiation of renal replacement therapy or death. Results: At the end of the study, PAC therapy significantly reduced the risk for the decline in renal function compared to ACE-I monotherapy by 54%. Survival time was longer in PAC group (21.7 ± 2.2 and 35.1 ± 3.9 months, in ACE-I monotherapy and PAC therapy, p < 0.05). Cox regression analysis indicated that age, sex and blood pressure except urinary protein excretion did not relate to the risk reduction by PAC therapy. Conclusion: PAC therapy was proved to reduce the progression of end-stage renal failure.

Correspondence to:
Y. Nakayama, MD; Department of Nephrology, Graduate School of Medical Sciences, 1-1-1 Honjo, Kumamoto, 860-8556, Japan
Email: ynakaya@kumamoto-u.ac.jp

Abstract

M. Mengel, O. Bock, M. Priess, H. Haller, H. Kreipe and W. Gwinner

¹Institute for Pathology and ²Department of Nephrology, Medizinische Hochschule Hannover, Germany
Aim: Better understanding of early onset of interstitial fibrosis and tubular atrophy (IF/TA), as the morphological surrogate of renal allograft deterioration might improve outcome after renal transplantation. Material and methods: We quantified mRNA expression of 3 profibrotic (transforming growth factor-β (TGF-β), tissue transglutaminase (tTG), tissue inhibitor of matrix metalloproteases (TIMP-1)) and 1 antifibrotic (matrix metalloprotease-2 (MMP-2)) molecule in protocol biopsies from renal allografts. From 107 transplants, two sequential protocol biopsies (6 weeks and 6 months) were analyzed. We evaluated a control group showing no IF/TA in both biopsies (n = 65) and a IF/TA group developing IF/TA at 6 months (n = 42). Expression data were correlated with clinical and histological risk factors for IF/TA and allograft function. Results: The expression of the genes correlated strongly with each other, particularly the profibrotic genes and in patients who developed IF/TA. Analyzing protocol biopsies from stable grafts, not all patients in both groups showed increased gene expression. In patients with increased gene expression a significantly higher tTG expression (matrix stabilization) at 6 weeks and a significantly lower MMP-2 expression (failure in matrix degradation) at 6 months were observed in the IFTA group compared to controls. Multivariate logistic regression revealed donor age positively and TIMP-1 expression at 6 weeks inversely correlated with IF/TA at 6 months. Conclusions: We conclude that a disturbance in the equilibrium of pro- and antifibrotic pathways is decisive for early onset of IF/TA in renal allografts: insufficient degradation of exaggerated matrix production apparently changes the balance in the direction of IF/TA.Correspondence to:
Dr. M. Mengel; Institute for Pathology, Medizinische Hochschule Hannover Carl-Neuberg-Straße 1, 30625 Hannover, Germany
Email: Mengel.michael@mh-hannover.de

Abstract

G.H.M. Piotto, M.C.M. Moraes, D.M.A.C. Malheiros, L.B. Saldanha and V.H.K. Koch

¹Medical School, University of São Paulo, ²Pathology Department, Renal Pathology Unit and ³Pediatric Nephrology Unit, Children’s Institute, University of São Paulo General Hospital, São Paulo, Brazil
Introduction: Pediatric percutaneous renal biopsy (Bx) is a routine procedure in pediatric nephrology to obtain renal tissues for histological study. We evaluated the safety, efficacy, indications and renal findings of this procedure at a tertiary care pediatric university hospital and compared our findings with the literature. Methods: Retrospective study based on medical records from January 1993 to June 2006. Results: In the study period, 305 Bx were performed in 262 patients, 127 (48.5%) male, aged 9.8 ± 4.2 years. A 16-gauge needle was utilized in 56/305 Bx, an 18-gauge needle in 252/305 Bx (82.6%). 56.1% Bx were performed under sedation plus local anesthesia, 43.9% under general anesthesia. The number of punctures per Bx was 3.1 ± 1.3. Minor complications occurred in 8.6% procedures. The 16-gauge needle caused a higher frequency of renal hematomas (p = 0.05). The number of glomeruli per puncture was >= 5 in 96.7% and >= 7 in 92%. Glomeruli number per puncture and frequency of complications were not different according to the type of anesthesia used. A renal pathology diagnosis was achieved in 93.1% Bx. The main indications of Bx were nephrotic syndrome (NS), lupus nephritis (LN) and hematuria (HE). The diagnosis of minimal change disease (MCD) (61.3%), class V (35.6%) and IgA nephropathy (26.3%) predominated in NS, LN and HE patients, respectively. Conclusion: Pediatric real-time ultrasound-guided percutaneous renal biopsy was safe and effective. The main clinical indications for Bx were NS and LN, the predominant renal pathology diagnoses were MCD and class V LN.Correspondence to:
Dr. V.H.K. Koch; 91 Mangabeiras Road, Apartment 81, São Paulo, SP 01233-010, Brazil
Email: vkoch@terra.com.br

Abstract

M. Leidig, R. Bambauer, E.J. Kirchertz, T. Szabó, R. Handrock, D. Leinung, M. Baier and R.E. Schmieder

¹Department of Medicine IV/Nephrology and Hypertension, University of Erlangen-Nürnberg, ²Center for Nephrology, Homburg/Saar, ³Center for Dialysis, Bad Muender, Germany, ⁴B. Braun Avitum Dialysis Center, Kistarcsa, Hungary, ⁵Novartis Pharma GmbH, Clinical Development, Nürnberg and ⁶Novartis Pharma GmbH, Department of Biometrics, Nürnberg Germany
Background: End stage renal disease (ESRD) patients mainly die of cardiovascular disease, and hypertension is regarded as the major risk factor. Valsartan is an angiotensin receptor blocker (ARB) with a well-established efficacy and safety profile in hypertensive patients, but with relatively few data in patients on hemodialysis (HD). The aim of this 2 × 5-week, open-label, multicenter, randomized cross-over study was to investigate whether valsartan (Val) 80 mg is as effective, safe and well-tolerated as irbesartan (Irb) 150 mg in patients with arterial hypertension on long-term hemodialysis. Methods: After a wash-out of previous ARBs for 1 week, 67 patients (ITT) on long-term hemodialysis, between 18 and 80 years, with mean supine systolic blood pressure (MSupSBP) >= 140 mmHg and < 180 mmHg were randomized to either Val 40 or Irb 75 for 1 week with forced titration to Val 80 or Irb 150 for another 4 weeks. After a second wash-out period of 1 week, patients were switched from Val to Irb or vice versa for another 5 weeks (1 week low-dose, 4 weeks target dose). The primary objective was non-inferiority of Val versus Irb on predialytic MSupSBP. Secondary objectives were predialytic MSupDBP, adverse events (AEs), laboratory abnormalities, hypotension during and after dialysis and quality of life. BP values are given as mean ± SD. Results: Baseline BP values were 158 ± 11 / 78 ± 13 mmHg (Val) and 161 ± 13 / 83 ± 10 mmHg (Irb). The predialytic MSupSBP and MSupDBP after 4 weeks of treatment were similar in both treatment groups (Val 150 ± 19 / 79 ± 13 mmHg; Irb 151 ± 16 / 78 ± 14 mmHg). Most of the reported AEs were mild to moderate. The percentage of AEs considered by the investigator to be possibly drug-related was similar between both groups: 15.4% in the valsartan group and 20.4% in the irbesartan group. The most common AEs were nausea, muscle spasms and nasopharyngitis. Eight SAEs occurred, four in each treatment group (all not drug-related), including one death (cardiovascular insufficiency) in the Irb group. Laboratory changes were similar in both groups and not clinically relevant. The number of patients with symptomatic hypotension was similar during (9% each) as well as after dialysis (1.3% each). The quality of life data (SF-36) were comparable for each category. Conclusions: Valsartan 80 mg is as effective, safe and well tolerated as irbesartan 150 mg in hypertensive patients on chronic hemodialysis.

Correspondence to:
Dr. M. Leidig; Department of Medicine, Klinikum Nürnberg Süd, Breslauer Straße 201, 90471 Nürnberg, Germany
Email: Michael.Leidig@klinikum-nuernberg.de

Abstract

T. Doi, T. Masaki, N. Shiraki, T. Kawai and N. Yorioka

¹Department of Nephrology, ²Department of Advanced Nephrology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan
A 24-year-old woman with focal segmental glomerulosclerosis was referred to our hospital for treatment of nephrotic syndrome. Though she experienced partial remission following treatment with prednisone and cyclosporine, she had a relapse of nephrotic syndrome when her prednisone was tapered off to 30 mg/day. The prednisone could not be tapered to less than 30 mg/day due to repeated relapses. After introduction of oral mizoribine (MZR) pulse therapy, the patient’s prednisone was tapered to 15 mg/day and she had no signs of relapse for more than 1 year. This case suggests that oral MZR pulse therapy is a good therapeutic option for patients with steroid-dependent nephrotic syndrome.

Correspondence to:
Prof. Dr. Noriaki Yorioka; Department of Advanced Nephrology, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
Email: nyorioka@hiroshima-u.ac.jp

Abstract

A. Omokawa, H. Wakui, S. Okuyama, M. Togashi, H. Ohtani, A. Komatsuda, R. Ichinohasama and K. Sawada

¹Third Department of Internal Medicine, Akita University School of Medicine, Akita, ²Department of Hematopathology, Tohoku University School of Medicine, Sendai, Japan
A 63-year-old man with systemic lupus erythematosus developed tubular proteinuria. All subclasses of serum IgG increased, and the largest IgG subclass increase was IgG4. A renal biopsy showed lupus nephritis (Class II) with severe tubulointerstitial nephritis (so-called predominant tubulointerstitial lupus nephritis, an unusual form of lupus nephritis). Immunofluorescence microscopy revealed positive granular staining for IgG, C3 and C1q in the mesangium and peritubular interstitium, and along the tubular basement membranes (TBM). Electron microscopy also showed electron-dense deposits in the mesangium and TBM. Immunophenotyping of interstitial infiltrating cells disclosed a predominance of T cells. CD8-positive cytotoxic T cells infiltrated the peritubular interstitium, and some of these cells infiltrated the tubules. B cell-rich lymphoid follicles were also observed. IgG subclass analyses showed glomerular IgG1, IgG2 and IgG4 deposition, positive staining of IgG4 in the peritubular interstitium and along the TBM, and abundant IgG1-, IgG3- and IgG4-positive plasma cells in the interstitium. The patient responded well to moderate-dose steroid therapy. This is the first report of immunophenotyping of interstitial infiltrates in predominant tubulointerstitial lupus nephritis. The results suggest CD8-positive cytotoxic T cell-mediated tubular injury. Furthermore, immune complexes containing IgG4 might be one of etiologic factors.Correspondence to:
H. Wakui, MD; Third Department of Internal Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita City, Akita 010-8543, Japan
Email: Correspondence to: H. Wakui, MD; Third Department of Internal Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita City, Akita 010-8543, Japan Email: wakui@med.akita-u.ac.jp

Abstract

R. Torra, F. Algaba, E. Ars, S. Santin, P. Fernández-Llama and J. Ballarin

Nephrology Department, Molecular Biology Laboratory, Puigvert Foundation, Barcelona, Spain
Fabry disease is an X-linked recessive inborn error of glycosphingolipid metabolism caused by the deficient activity of the lysosomal enzyme, a-galactosidase A. Enzyme replacement therapy (ERT) for this disorder has been available in Europe since 2001. However, its effect on advanced renal failure remains controversial. We report the case of a patient whose decline in renal function was reduced by the administration of ERT (agalsidase-a). This reduction was more pronounced after doubling the dose of the enzyme. The rate of deterioration of eGFR went from 6.3 ml/min/year prior to the start of ERT (0.2 mg/kg) to 2 ml/min/year (0.4 mg/kg). To our knowledge, this is the first reported case of a patient with moderately impaired renal function treated with high doses of ERT and follow-up of 6 years. The data shown here suggest that ERT may have a very positive impact on renal function even in advanced stages. The role of proteinuria and its control seem to have a clear responsibility for this favorable outcome.Correspondence to:
Dr. R. Torra; Foundacio Puigvert, Calle Cartagena 340-350, 08025 Barcelona, Spain
Email: rtorra@fundacio-puigvert.es

Abstract

I. Pela, M. Bigozzi and B. Bianchi

¹Department of Pediatrics, Pediatric Nephrology Unit and ²Department of Neuro-ophthalmologic Sciences, Audiology Unit, University of Florence, Italy
Pendred syndrome is a recessive autosomal disorder characterized by thyroid goiter and sensorineural hearing loss. The Pendred syndrome gene (SLC26A4) encodes a new anion exchanger named pendrin which mediates iodide transport by thyrocytes and regulates ion and fluid transport by the endolymphatic sac epithelium. Pendrin defects result in inner ear malformations, with enlargement of the endolymphatic sac and duct in association with a large vestibular aqueduct. Furthermore, patients may develop endolymphatic hydrops requiring diuretic therapy, mainly in the form of thiazides. Pendrin could also account for apical Cl–/ HCO3– exchange at level of intercalated cells of the cortical collecting duct in the kidneys, however, humans with Pendred syndrome have no symptoms attributable to renal pendrin abnormalities in basal conditions. We report the case of a child with Pendred syndrome and intercurrent endolymphatic hydrops, who developed profound hypokalemia and severe hypochloremic metabolic alkalosis (potassium 1.7, chloride 70, sodium 129, HCO3 43.8, base excess +17.8 mmol/l, pH 7.52) following thiazide therapy. In subjects with Pendred syndrome thiazide therapy seems to provoke more severe Cl– and extracellular volume depletion. A possible explanation could be the defective action of the disrupted pendrin, which exacerbates the effects of the inhibition of Cl– reabsorption mediated by the thiazide-sensitive NaCl cotransporter (SLC12A3).

Correspondence to:
I. Pela, MD; Pediatric Nephrology Unit, Department of Pediatrics, University of Florence, Via L. Giordano 13, 50132 Florence, Italy
Email: ivana.pela@unifi.it

Abstract

J. Serratrice, B. Dussol, N. Ené, A. Benyamine, Y. Berland and P.J. Weiller

¹Service de Médecine Interne, CHU Timone and ²Centre de Néphrologie et de Transplantation Rénale, CHU Conception, Marseille Cedex ⁵, France
We report on a 79-year-old woman having hemophagocytosis and capillary hyperpermeability syndrome who presented with anuric prerenal acute renal failure. The patient eventually died of a hypovolemic shock. Post-mortem biopsies evidenced a highly aggressive B cell intravascular lymphoma without amyloidosis. Physicians should be aware of the risk of anuric prerenal acute renal failure in the course of lymphoma-associated hemophagocytic syndrome.Correspondence to:
Prof. B. Dussol; Centre de Néphrologie et de Transplantation Rénale, Hôpital de la Conception, 147 Bd Baille 13385 Marseille Cedex 5, France
Email: bdussol@ap-hm.fr

Abstract

M. Abe and K. Matsumoto

Division of Nephrology, Hypertension and Endocrinology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan
We report perforation of a small intestine diverticulum in a maintenance hemodialysis patient. The patient was a 57-year-old male. He was undergoing continuous ambulatory peritoneal dialysis (CAPD) for chronic glomerulonephritis (CGN) since 1984. In 1992, his dialysis therapy was changed from CAPD to hemodialysis (HD) because of peritonitis. On September 29, 2007, he developed abdominal pain, and on October 2, 2007, he complained of more severe pain and muscular tenderness and was positive for Blumberg’s sign. He was admitted in emergency. Abdominal CT scan revealed some free air in the endoperitoneum. Emergency laparotomy was performed following the diagnosis of panperitonitis due to intestinal perforation. We found a perforation measuring 1 mm in diameter at the actinal side approximately 10 cm from the Bauhin valve, however, no other abnormalities were detected in the abdominal cavity. Partial bowel resection was performed, and a segment approximately 50 cm in length, including the perforated lesion, was excised. The postoperative course was uneventful, and he was discharged on October 17, 2007. Pathological findings of the resected specimen revealed a perforation of diverticulum with inflammatory changes. Such perforation of small intestine diverticulum in a hemodialysis patient has rarely been reported in the literature, and we emphasize that prompt laparotomy is necessary for the prevention of severe complications.Correspondence to:
M. Abe, MD; Division of Nephrology, Hypertension and Endocrinology, Department of Medicine, Nihon University School of Medicine, 30-1, Oyaguchi-Kamimachi, Itabashi-ku, Tokyo, 173-8610, Japan
Email: mabe@med.nihon-u.ac.jp

Abstract

C.-C. Liang, J.-T. Fang, K.-H. Chen, C.-C. Hung, T.-L. Hwang, J.-Y. Huang

Department of Nephrology, Chang Gung Memorial Hospital, Chang Gung University School of Medicine, Taoyuan, Taiwan
In continuous ambulatory peritoneal dialysis (CAPD)-related cases of fungal peritonitis, Candida parapsilosis (C. parapsilosis) has become as common as Candida albicans (C. albicans) in fungal isolates. This report describes a 74-year-old male CAPD patient who received bypass surgery for coronary artery disease, followed by an episode of bacterial peritonitis. The peritonitis was successfully treated with intraperitoneal antibiotics. However, C. parapsilosis peritonitis with concomitant pancreatitis and infected pseudocysts occurred one month later. Despite surgical drainage and intravenous administration of fluconazole, fungal peritonitis persisted. Finally, he died of nosocomial pneumonia. This case demonstrates the poor outcome of C. parapsilosis peritonitis, suggesting a more aggressive treatment in peritoneal dialysis patients.Correspondence to:
J.-Y. Huang, MD; Department of Nephrology, Chang Gung Memorial Hospital, 5. Fu-Hsing Street, Keui Shan Hsiang, Taoyuan Hsien, Taiwan
Email: healthwu@yahoo.com.tw

Abstract

K.P. Katopodis, M. Mitsis, E.G. Triantou, M. Fatouros and K.C. Siamopoulos

Abstract

M. Abe, K. Okada and K. Matsumoto

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Volume 69, No. 6/2008(June)