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Abstract

D.C. Cattran, M.M. Wang, G. Appel, A. Matalon and W. Briggs

¹Department of Medicine, University Health Network, University of Toronto, Ontario, Canada, and ²Departments of Medicine, Columbia-Presbyterian Medical Center, New York, NY and ³Johns Hopkins University School of Medicine, Baltimore, MD, USA
Aims: Primary focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease in both children and adults. Our current treatments are suboptimal, and a significant percentage of cases are resistant to current therapy. Patients and methods: We performed an open-label, 6-month trial of the new immunosuppressive agent mycophenolate mofetil (MMF) in 18 biopsy-proven patients resistant to a course of corticosteroids therapy. Seventy-five percent had also failed to respond to a cytotoxic agent and/or a calcineurin inhibitor. Results: A substantial improvement in proteinuria was seen in 44% (8/18) of the patients by 6 months. This was sustained for up to 1 year post treatment in 50% (4/8) of this group. No patient had a complete remission. No deterioration in renal function was observed in any patient over the treatment period, but 3 progressed to chronic kidney failure during follow-up. Adverse effects were mild. Only 1 patient required a dose reduction due to an intercurrent infection. Conclusions: MMF appears safe to use in this group of patients and did lower proteinuria in 44% of this cohort resistant to other forms of treatment. Relapses were common, suggesting more prolonged or combination therapy may be required. More rigorous trials utilizing this medication should be considered to further assess the risk-benefit ratio of treatment with MMF in patients with FSGS.Correspondence to:
Dr. D.C. Cattran University Health Network Toronto General Hospital NCSB11-1256 585 University Avenue Toronto, ON, M5G 2N2, Canada
Email: daniel.cattran@uhn.on.ca

Abstract

H. Tanaka, K. Tsugawa, K. Tsuruga, K. Suzuki, T. Nakahata, E. Ito and S. Waga

¹Department of Pediatrics, Hirosaki University School of Medicine and and ²National Aomori Hospital, Hirosaki, Japan
Aim: The optimal treatment for lupus nephritis in the pubertal age group is still unclear. We therefore retrospectively evaluated the efficacy and safety of mizoribine (MZR), a novel selective inhibitor of inosine monophosphatase dehydrogenase, developed in Japan for the treatment of lupus nephritis in pubertal patients, because MZR has been reported to have relatively less clinically toxicity than conventionally used drugs. Methods: Over the past 12 years, we have treated 20 children with newly diagnosed, biopsy-proven lupus nephritis at our hospital. Of these, 10 patients who received a 2-year course of MZR in combination with prednisolone (PSL) as initial maintenance therapy and who could be observed for at least two years after the commencement of the MZR treatment, were enrolled in this study. MZR was given orally at the dose of 4 – 5 mg/kg per day in two divided doses. Changes in the clinical parameters, such as the urinary protein excretion, serum anti-dsDNA antibody titer, serum hemolytic complement activity (CH50) and serum creatinine, and in the frequency of disease flares defined as persistent worsening of those clinical parameters, were examined pre- and posttreatment for comparison, and the steroid-sparing effect of MZR was analyzed. Results: As induction therapy, all the patients had received high-dose oral PSL or intravenous methylprednisolone pulse therapy. Five female patients who were diagnosed to have proliferative lupus nephritis (WHO class III or IV) were scheduled to receive an 8-week course of oral cyclophosphamide (CPA) combined with high-dose steroids prior to the commencement of MZR, but CPA needed to be discontinued in four of these patients because of clinical drug toxicity. The clinical parameters showed significant improvement after the 2-year treatment with MZR combined with PSL, as compared to the pretreatment values (mean urinary protein excretion: 1.6 ± 1.9 g/day vs.0.1 ± 0.1 g/day, serum CH50 value: 12.6 ± 5.4 U/ml vs. 34.5 ± 7.7 U/ml, serum anti-dsDNA antibody titer: 141.8 ± 128.2 IU/ml vs. 18.5 ± 17.7 IU/ml, respectively (p <0.01)). The serum creatinine remained normal. Although the daily dose of the concomitantly administered PSL to maintain clinical remission could be decreased significantly in all the study participants (39.5 ± 1.6 mg/day vs. 6.8 ± 5.1 mg/day, p < 0.01), two patients developed flares while on treatment, which were successfully treated by transiently increasing the dose of PSL. The MZR therapy could be completed in all of the patients without significant clinical toxicity being reported. At their most recent follow-up (mean 4.3 years), none of the 10 patients had renal insufficiency, and complete remission without any need for further medication had been achieved in two patients. Conclusion: Although this case series is without controls, maintenance therapy with MZR administered in combination with PSL may be beneficial and clinically less toxic in at least a proportion of pubertal patients with lupus nephritis.Correspondence to:
Dr. H. Tanaka
Department of Pediatrics
Hirosaki University
School of Medicine
5 Zaifu-cho
Hirosaki 036-8562, Japan
Email: hirotana@cc.hirosaki-u.ac.jp

Abstract

L. Szönyi¹, M. Dobos¹, B. Vásárhelyi², E. Héninger², T. Vas³, J. Nagy³ and T. Kovács³

¹¹st Department of Pediatrics, Semmelweis University, ²Research Laboratory of Pediatrics and Nephrology, Hungarian Academy of Sciences, Budapest, and ³²nd Department of Medicine and Nephrological Centre, Medical Faculty, University of Pécs, Pécs, Hungary
Background: a1-antitrypsin (AAT) is the main protease inhibitor in the blood. Several different AAT phenotypes exist. The most common variant is the MM phenotype, which is also associated with normal AAT levels. The less common phenotypes with Z and S variants are associated with low AAT levels. AAT deficiency is a risk factor for pulmonary emphysema, liver impairment and some immune-mediated diseases, some of which are also associated with IgA nephropathy (IgAN). In fact, liver impairment resulting from AAT deficiency may directly contribute to renal abnormalities resembling IgAN. Patients and methods: We investigated AAT phenotype and AAT levels in 100 IgAN patients who did not have end-stage liver disease. Fifteen patients in our sample had secondary IgAN. We also tested for the presence of renal deposition of AAT in patients heterozygous for AAT variants as well as in a randomly chosen group of patients with MM phenotype. We checked for any association between AAT phenotype and the progression of IgAN as well as the prevalence of diseases associated with IgAN (i.e. secondary IgAN). Results: Twelve patients in our sample were heterozygous for AAT variants. Phenotypes were MZ in 5 patients, MS in 3, MF in 1, ML in 2 and ME in 1 patient. AAT levels were lower in these 12 patients than in those homozygous for the M variant (1.17 ± 0.46 vs. 1.44 ± 0.34 g/l, p < 0.05). We found renal deposition of AAT in 2 heterozygous patients and in 1 of the 12 patients which were randomly chosen. End-stage renal (ESRF) failure developed in 3 of the 12 heterozygous patients and in 6 of the 88 homozygous patients (p = 0.07) during the follow-up. The prevalence of heterozygosity was significantly higher in patients with secondary IgAN than in those with primary IgAN ((5/15 vs. 7/85; p < 0.02). Conclusions: AAT phenotype is not associated with the risk of primary IgA nephropathy, but might have an impact on disease outcome as well as on the risk of secondary IgAN.Correspondence to:
Dr. B. Vásárhelyi
1st Department of Pediatrics
Semmelweis University
Budapest, Hungary
Bókay u. 53
H-1083 Budapest
Email: vasbar@gyer1.sote.hu

Abstract

M. Wiesmann¹, C.U. Bergmann-Köster², B. Kreft³, M. Stöckelhuber⁴, H.-B. Gehl² and B.M. Stöckelhuber²

¹Department of Neuroradiology, University of Munich, ²Department of Radiology, ³Department of Internal Medicine, Medical University, Lübeck, and ⁴Anatomical Institute, University of Munich, Germany
Aims: We evaluated different techniques of contrast-enhanced phase-inversion ultrasound to visualize renal perfusion in native kidneys and kidney transplants. Materials and methods: Contrast-enhanced phase inversion ultrasound with different levels of mechanical index and frame rate was performed in 20 kidneys of 13 healthy volunteers. In addition, five dysfunctioning kidneys of patients with chronic renal failure, five functionally intact kidney transplants, three kidney transplants with compensated renal failure, and two kidney transplants with acute rejection were studied. Analysis using a software algorithm for time-resolved perfusion imaging was compared to single-image analysis performed by three independent radiologists. Results: Optimal depiction of renal perfusion was achieved only by using a mechanical index, which was high enough to destroy the microbubbles of the contrast agent (burst imaging) combined with a low frame rate (0.5 images/second). Renal cortex and medulla showed a homogeneous enhancement in kidneys of healthy volunteers and functionally intact renal transplants. Dysfunctioning kidneys of patients with chronic renal failure as well as kidney transplants with compensated renal failure or acute rejection showed a significantly reduced level of enhancement. Computer-assisted time-resolved perfusion analysis was not superior to single-image analysis. Conclusion: Renal perfusion patterns of normal and abnormal tissue can be visualized using contrast-enhanced phase-inversion ultrasound imaging.Correspondence to:
Dr. M. Wiesmann
Abteilung für Neuroradiologie
Universitätsklinikum München-Großhadern
Marchioninistraße 15
81377 München, Germany
Email: martin.wiesmann@med.uni-muenchen.de

Abstract

R. Lobão, A.B. Carvalho, L. Cuppari, R. Ventura, M. Lazaretti-Castro, V. Jorgetti, J.G. Vieira, M. Cendoroglo and S.A. Draibe

Nephrology Division, Federal University of São Paulo, Escola Paulista de Medicina, São Paulo, Brazil
Chronic kidney disease (CKD) leads to reduced bone mineral density (BMD) in pre-dialysis and dialysis patients. A few studies have used dual-energy x-ray absorptiometry (DEXA) to assess BMD in pre-dialysis CKD patients and have shown low BMD to be highly prevalent. Until now there have been no studies reporting the histological features of low BMD in pre-dialysis CKD patients. Aim: To determine the prevalence and histological features of low BMD in pre-dialysis CKD patients. Method: Pre-dialysis CKD patients (n = 103, 46 females/57 males), median creatinine clearance of 29 (10 – 78) ml/min/ 1.73 m2, were evaluated using biochemical analysis and DEXA. Bone biopsies were obtained from those with low BMD. Results: Fifty (48.5%) out of the 103 patients had low BMD (LBD group) and 53 (51.5%) had normal BMD (NBD group). The risk for low BMD was increased in those patients with alkaline phosphatase levels above 190 U/l and intact-PTH (iPTH) below 70 pg/ml (p < 0.05). Demographic and biochemical parameters from both groups were comparable, except for lower body mass index (BMI) in LBD subjects (p = 0.04). Women who had been post-menopausal for at least 1 year comprised 65% (13/20) and 50% (13/26) of the LBD and NBD groups, respectively (p = NS). In 40 LBD patients, bone histomorphometry revealed adynamic bone disease (ABD, 52.5%), osteomalacia (OM, 42.5%) and mixed bone disease (MBD, 5%). Trabecular bone volume (BV/TV) was lower in ABD and OM patients. A nearly significant association was found between ABD and iPTH £ 150 pg/ml (p = 0.056), whereas higher values of iPTH were associated with OM. Total alkaline phosphatase £ 190 U/l was significantly associated with ABD, whereas higher values were associated with OM. No correlation was observed between BV/TV and BMD. Conclusion: Low BMD is frequent in pre-dialysis CKD patients, and low turnover bone disease, manifesting as ABD and OM, was the hallmark of this bone loss.Correspondence to:
Dr. S.A. Draibe
Rua Borges Lagoa,
960 4° andar, CEP 04038-002, São Paulo, Brazil
Email: uremia@uol.com.br

Abstract

P. Barré¹, H. Reichel², M.G. Suranyi³ and C. Barth⁴

¹Hemodialysis, Nephrology Division, McGill University Health Center, Royal Victoria Hospital, Quebec, Canada, ²Nephrological Center, Villingen-Schwenningen, Germany, ³Dialysis Services, Liverpool Health Service, Sydney, Australia, ⁴Medical Affairs, Ortho Biotech, Division of Janssen-Cilag, Neuss, Germany
Background: Patients with end-stage renal disease requiring hemodialysis are frequently treated with epoetin alfa (recombinant human erythropoietin, rHuEPO) for anemia. The aim of this study was to determine whether successful management of anemia could be maintained by changing the dosing schedule of epoetin alfa from 2 or 3 times per week to once-weekly administration via not only the subcutaneous (s.c.) but also the intravenous (i.v.) route. Methods: Patients included in the study had hemodialysis for > 12 months, treatment with epoetin for ³ 6 months and adequate iron stores. The study consisted of a pre-study period (12 weeks), Phase I (4 weeks, patients continued pre-study regimen), Phase II (12 weeks, once-weekly i.v. or s.c. regimen with dose adjustments permitted to maintain target hemoglobin (Hb) concentrations) and Phase III (4 weeks, once-weekly i.v. or s.c. regimen without dose adjustments). Results: The study was completed by 203 patients (per-protocol population: i.v. group, n = 115, s.c. group, n = 88). In the majority of patients (69.4% overall: i.v. group, 67.0%, s.c. group, 72.7%), the individual Phase I Hb concentrations were maintained within ± 1.0 g/dl (± 10 g/l) during Phase III. In 79.3% of the patients (i.v. group, 75.7%, s.c. group, 84.1%), a stable Hb concentration (decrease of £ 1 g/dl (£ 10 g/l)) was maintained without statistically significant dose adjustments (82.4 ± 33.8 – 86.8 ± 42.1 IU/kg body weight/week). Hb concentrations decreased from 11.57 ± 0.83 g/dl (115.7 ± 8.3 g/l) in Phase I to 11.39 ± 1.09 g/dl (113.9 ± 10.9 g/l) in Phase III (p < 0.05) in the entire group. The weekly dose of epoetin alfa required to maintain the individual target Hb concentrations changed from 85.1 ± 34.6 IU/kg in Phase I to 92.1 ± 45.1 IU/kg in Phase III in the entire population (p <0.05). Conclusions: With once-weekly administration of epoetin alfa, Hb concentrations can be maintained in the majority of stable hemodialysis patients, and only minimal dose adjustments are required.Correspondence to:
Dr. P. Barré
Nephrology Division, Room R2.38
McGill University Health Center
Royal Victoria Hospital
687 Ave Des Pins West
Montreal, Quebec H3A1A1, Canada
Email: paul.barre@muhc.mcgill.ca

Abstract

C. Nailescu¹, M. Castaneda², M. Del Rio¹ and J.T. Flynn¹

¹Division of Pediatric Nephrology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine of Yeshiva University, Bronx, New York, USA, and ²Hospital Bloom – Children’s Hospital, San Salvador, El Salvador
Aims: Iron supplementation is necessary in children on hemodialysis, but the optimal protocol remains unknown. We studied the effects of changing our unit’s protocol from oral iron with periodic doses of parenteral iron dextran to routine administration of parenteral sodium ferric gluconate on anemia and iron parameters. Methods: We followed seven hemodialysis patients aged 15 – 20 years (mean 17 years). Hemoglobin, hematocrit, serum iron, transferrin saturation, ferritin, erythropoietin dose, total elemental iron dose and total iron cost for the six months prior to the protocol change were compared to the same variables during the six months following the change. Results: There was no statistically significant difference between the doses of parenteral iron between the two protocols; however, the total dose of elemental iron administered in the oral iron plus iron dextran protocol was greater than in the sodium ferric gluconate protocol (19.6 ± 13.1 (mean ± SD) mg/kg/week versus 1.1 ± 0.3 mg/kg/week; p = 0.03). Both protocols had equivalent efficacy with respect to hemoglobin, ferritin and other measures of iron stores. On the other hand, the costs of sodium ferric gluconate were significantly higher than those of oral iron plus intermittent iron dextran (157.75 ± 45.94 $/patient/month versus 52.08 ± 49.88 $/patient/month; p = 0.01). Conclusions: Routine administration of sodium ferric gluconate is equivalent if not superior to use of oral iron plus iron dextran for maintenance of iron stores in adolescents on hemodialysis, but more expensive.Correspondence to:
J.T. Flynn, M.D., M.S.
Division of Pediatric Nephrology
Children’s Hospital at Montefiore
111 East 210th Street
Bronx, NY 10467, USA
Email: jflynn@montefiore.org

Abstract

M. Numata, M. Nakayama, T. Hosoya, C.M. Hoff, C.J. Holmes, M. Schalling, L. Nordfors and B. Lindholm

¹Division of Kidney and Hypertension, Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan, ²Baxter Healthcare Renal Division Scientific Affairs, Baxter Healthcare Corporation, McGaw Park, IL, USA, ³Department of Molecular Medicine, Karolinska Institutet, Stockholm, and ⁴Department of Clinical Science, Divisions of Renal Medicine and Baxter Novum, Huddinge University Hospital, Huddinge, Sweden
Encapsulating peritoneal sclerosis (EPS) is a serious complication of PD. The cause(s) of EPS are unknown but may include peritonitis and long duration of PD treatment. However, EPS may also develop in some patients without a history of peritonitis or with rather short duration of PD therapy. It has been suggested that an increasing peritoneal solute transport rate (PSTR) as a function of time on PD treatment is a risk factor for EPS development after transfer to hemodialysis, and that high PSTR is associated with an increased peritoneal microvessels surface area. Other putative mechanisms might include advanced glycated end products (AGE) and their receptors, RAGE. The purpose of this study was to investigate genetic variations in PD patients developing EPS in comparison to PD patients without EPS. SNPs in genes related to angiogenesis as well as RAGE were analyzed. Twenty patients (M/F: 12/8, mean age at start of PD 42.2 years, mean duration of PD 8.4 years) who were diagnosed as EPS during the period 1982 – 2002 at Jikei University Hospital and a matched control group (n = 20) of nonEPS PD patients were studied. The following 5 SNPs were analyzed: VEGF 936 C/T, ecNOS –786 T/C, 298 Glu/Asp, and RAGE –374 T/A, and –429 T/C. The SNPs were analyzed by the pyrosequencing method. The C allele (T/C and C/C) in the RAGE –429T/C genotype was not found in any of the EPS patients (EPS, T/T: 20/20 (100%), nonEPS, T/T: 15/20 (75%), T/C: 4/20 (20%), C/C: 1/2 0(5%), nonC allele vs C allele, p = 0.013), although every allele was found in other SNPs. We conclude that these preliminary data show that whereas genotypes directly related to angiogenesis did not differ between EPS and nonEPS patients, it is noteworthy that no patients in the EPS group had a C allele in the RAGE –429T/C genotype. This might indicate a possible genetic contribution to the development of EPS that is related to RAGE.Correspondence to:
Dr. M. Numata
Division of Kidney and Hypertension
Department of Internal Medicine
Jikei University School of Medicine
3-19-18, Nishishinbashi
Minato-ku, Tokyo, 105-8471, Japan
Email: mnumata@jikei.ac.jp

Abstract

L. Couzi¹, D. Morel¹, C. Deminière² and P. Merville¹,³

¹Département de Néphrologie, Hôpital Pellegrin,
²Laboratoire d’Anatomo-pathologie, Hôpital Pellegrin, and
³UMR-CNRS ⁵¹⁶⁴, Université Bordeaux II, Bordeaux, France
A 58-year-old man presented with fever and a rapidly progressive glomerulonephritis. An infective endocarditis due to Streptococcus parasanguis was diagnosed. A renal biopsy revealed type III pauci-immune crescentic glomerulonephritis. As first-line therapy, antibiotics were administered alone. Faced to the unsuccessful anti-infective approach, corticosteroid therapy was added as a second-line therapy. Finally, plasmapheresis introduced as the third-line therapy, significantly improved renal function. This case is an original type III rapidly progressive glomerulonephritis, since ANCA were repeatedly found negative. In very few cases, plasmapheresis was successfully used for the treatment of infective endocarditis-induced crescentic glomerulonephritis. The pathophysiology and the potential efficiency of plasmapheresis are discussed.
Correspondence to:
Dr. L. Couzi
Département de Néphrologie
Hôpital Pellegrin
place Amélie Raba Léon
F-33076 Bordeaux, France
Email: lionel.couzi@chu-bordeaux.fr

Abstract

C. Esposito, N. Bellotti, G. Fasoli, A. Foschi, A.R. Plati and A. Dal Canton

Unit of Nephrology, Dialysis and Transplantation, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
Hyperkalemia is a potentially lethal condition to be aware of in the presence of ECG abnormalities especially in patients with reduced renal function. However, ECG abnormalities are not always dependent on the degree of hyperkalemia but may be aggravated by the rapidity of the development of hyperkalemia and by associated electrolyte disorders. We describe 3 patients with renal failure and different ECG changes induced by hyperkalemia. More severe changes were observed when hyperkalemia developed rapidly, but not in presence of electrolyte disorders. Even minor ECG abnormalities must alarm physicians in patients with renal failure since severe hyperkalemia is not always associated with critical ECG changes.

Correspondence to:
Dr. C. Esposito Unit of Nephrology, Dialysis and Transplantation IRCCS Policlinico San Matteo University of Pavia P. le Golgi, 2 I-27100 Pavia, Italy
Email: espositociro56@hotmail.com

Abstract

K. Kasuno, S. Ueda, A. Tanaka, Y. Tanaka-Kasuno and T. Kuwahara

¹Department of Biological Responses, Laboratory of Infection and Prevention, Institute for Virus Research, Kyoto University, Kyoto, ²Department of Nephrology, Saiseikai Nakatsu Hospital, Osaka, and ³Wakayama Medical Center of Japanese Red Cross Society, Wakayama, Japan
Angiomyolipoma (AML), a benign renal tumor, has various clinical forms, and the nature of AML is not sufficiently understood because of few reports of long-term observation. We report a 57-year-old female without tuberous sclerosis who developed multiple pulmonary AML 26 years after a right nephrectomy for renal AML. A computed tomogram demonstrated multifocal round lesions with lipid-like density throughout both lung fields. An open lung biopsy revealed a histological diagnosis of pulmonary AML. An abdominal computed tomogram was also compatible with splenic AML. She has been in good health for 12 years since the lung biopsy over the 38 years since the nephrectomy. This case suggests that multicentric AML can recur at distant organs even after long-term silence in a patient who has a past history of renal AML.

Correspondence to:
Dr. T. Kuwahara
Department of Nephrology
Saiseikai Nakatsu Hospital
2-10-39 Shibata
Kita-ku, Osaka 530-0012, Japan
Email: nkt-hp@nakatsu.saiseikai.or.jp

Abstract

V.-C. Wu, C.-C. Fang, W.-Y. Li, P.-R. Hsueh and T.-S. Chu

¹Department of Internal Medicine, Far Eastern Memorial Hospital, and ²Departments of Internal Medicine, ³Emergency Medicine,
⁴Laboratory Medicine, and ⁵Primary Care Medicine, National Taiwan
University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan
Although the kidney is often involved in disseminated and localized candidiasis, bilateral emphysematous pyelonephritis (EPN) is infrequently reported. Renal papillary necrosis (RPN) caused by fungi is also rare. We describe a patient with bilateral RPN and EPN caused by Candida tropicalis, who suffered from recurrent hematuria, flank pain, acute fulminant renal failure, and obstruction by a sloughed papilla. He was treated successfully with antifungal therapy and percutaneous nephrostomy (PCN). This is the first case report of C. tropicalis-associated EPN and RPN.Correspondence to:
Dr. T.-S. Chu
Department of Internal Medicine
National Taiwan University Hospital
7 Chung-Shan South Road
Taipei, Taiwan
Email: tschu@ha.mc.ntu.edu.tw

Abstract

S. Kapoulas, S. Liakos, G. Karkavelas, D. Grekas and E. Giannoulis

Abstract

C.J. Yen, W.T. Huang, J.J. Huang, F.F. Chen and J.M. Sung

Abstract

M.J. Leikis, I.K. Forbes, L.P. McMahon and G.J. Becker

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Volume 62, No. 6/2004(December)