Volume 75 (2011), No. 4/2011(April)
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Review
C4d-negative antibody-mediated rejection in renal allografts: evidence for its existence and effect on graft survival
M. Haas
Page No. 271
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (271-278)
C4d-negative antibody-mediated rejection in renal allografts: evidence for its existence and effect on graft survival
M. Haas
The use of C4d staining as a tissue marker for humoral immunity has served an important role in allowing pathologists to more accurately diagnose acute antibody-mediated rejection (AMR) in renal and other allograft biopsies, and also to recognize the contribution of humoral immunity to lesions of chronic renal allograft rejection, including transplant glomerulopathy. However, while C4d remains a specifi c marker of a humoral response, recent evidence indicates that a considerable fraction of renal allograft biopsies showing antibody-mediated injury are C4d-negative, even by immunofl uorescence. This review summarizes the current evidence supporting the existence of C4d-negative AMR, as well as evidence that this entity may, if untreated, lead to the development of scarring within the graft, transplant glomerulopathy and even graft loss.Correspondence to:
M. Haas, MD, PhD
Department of Pathology and Laboratory Medicine
Cedars-Sinai Medical Center
8700 Beverly Blvd., Room 8742
Los Angeles, CA 90048, USA
Email: [email protected]
Review
Imaging patients with kidney disease in the era of NSF: can it be done safely?
V. Manjunath and M.A. Perazella
Page No. 279
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (279-285)
Imaging patients with kidney disease in the era of NSF: can it be done safely?
V. Manjunath and M.A. Perazella
Section of Nephrology, Department of Medicine, Yale University School of Medicine, New Haven, CT, USA
Nephrogenic systemic fibrosis (NSF) was first recognized as a complication of gadolinium-based contrast (GBC) exposure in 2006 and confirmed subsequently by numerous investigators. Early information on the toxicity of free gadolinium (Gd3+) and the pharmacokinetics of this agent in patients with underlying kidney disease were likely unrecognized subtle clues to its potential for adverse effects in humans. Since the recognition of NSF as a complication of GBC exposure, our approach to imaging in patients with kidney disease has been altered. As these patients require various forms of imaging to diagnose associated conditions, we must utilize an evidence-based approach to imaging options. It is our responsibility to identify patients at high risk to suffer this complication and choose between non-GBC imaging modalities, radiocontrast-based imaging modalities, and GBC imaging modalities based on risk:benefit assessment. The benefits of rapid, accurate diagnosis must be weighed against risks associated with CT scan with radiocontrast (radiation exposure, allergic contrast reactions, acute kidney injury), GBC imaging (development of NSF), and missed diagnoses due to use of suboptimal imaging modalities in an effort to avoid radiocontrast and GBC agent exposure.Correspondence to:
Prof. M.A. Perazella, MD, FASN
Section of Nephrology, Department of Medicine
Yale University School of Medicine
Boardman Building 114
330 Cedar Street
New Haven, CT 06520-8029, USA
Email: [email protected]
Review
Vitamin D supplementation in CKD
K.J. Martin and E.A. González
Page No. 286
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (286-293)
Vitamin D supplementation in CKD
K.J. Martin and E.A. González
Division of Nephrology, Saint Louis University, St. Louis, MO, USA
Vitamin D deficiency is common in chronic kidney disease and is associated with increased morbidity and mortality. In addition, vitamin D deficiency in renal disease is associated with serious comorbidities including secondary hyperparathyroidism and cardiovascular disease. Several therapeutic options are available to correct vitamin D deficiency, including nutritional vitamin D, pro-hormones and active hormones. In this review we discuss clinical findings related to the effectiveness of these therapies in chronic kidney disease.Correspondence to:
K.J. Martin, MB, BCh, FACP
Professor of Internal Medicine, Director
Division of Nephrology, Saint Louis University
3635 Vista Ave, St. Louis, MO 63110, USA
Email: [email protected]
Original
Poorer graft survival in ethnic minorities: results from a multi-centre UK study of kidney transplant outcomes
J.F. Medcalf, P.A. Andrews, J. Bankart, C. Bradley, S. Carr, J. Feehally, P. Harden, J. Marsh, C. Newstead and J. Thompson
Page No. 294
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (294-301)
Poorer graft survival in ethnic minorities: results from a multi-centre UK study of kidney transplant outcomes
J.F. Medcalf1, P.A. Andrews2, J. Bankart3, C. Bradley4, S. Carr1, J. Feehally1, P. Harden5, J. Marsh2, C. Newstead6 and J. Thompson7
1John Walls Renal Unit, Leicester, 2St Helier Hospital, Carshalton, 3Department of Health Sciences, University of Leicester, Leicester, 4Royal Holloway, University of London, Egham, 5Churchill Hospital, Oxford, 6St James’s Hospital, Leeds, and 7Department of Health Sciences, University of Leicester, Leicester, UK
Background: The STEPP group was established to investigate factors that affect long-term transplant outcomes including quality of life and other patient-reported outcomes between different transplant centers and patients. Methods: Data were collected for 2,650 patients whose first renal transplant took place between 1992 and 2003 in five UK centers. Univariable and multivariable survival analyses were performed using eleven candidate explanatory variables. Results: Graft survival was worse in Black (B) patients (HR B v W 1.57 95% CI 1.10, 2.24), and in South Asian (A) patients (HR A v W 1.39 95% CI 1.03, 1.85) compared to Whites (W) after adjusting for other factors including HLA mismatch, and time on dialysis. Time spent on dialysis pre-transplantation was non-linearly associated with patient, but not death-censored graft survival. Losing a functioning graft was a strong predictor of patient death. One site had both the best graft and the worst patient survival. Conclusions: Differences in patient and graft survival between ethnic groups cannot be explained by currently recognized factors. These, and the complex balance between optimum patient and graft survival which differs between sites in this study require further investigation.Correspondence to:
Dr. J. Medcalf
John Walls Renal Unit
Leicester General Hospital
Gwendolen Road, Leicester, LE5 4PW, UK
Email: [email protected]
Original
MESNA (sodium 2-mercaptoethanesulfonate) for prevention of contrast medium-induced nephrotoxicity – controlled trial
U. Ludwig, M.K. Riedel, M. Backes, A. Imhof, R. Muche and F. Keller
Page No. 302
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (302-308)
MESNA (sodium 2-mercaptoethanesulfonate) for prevention of contrast medium-induced nephrotoxicity – controlled trial
U. Ludwig1, M.K. Riedel2, M. Backes3, A. Imhof2, R. Muche4 and F. Keller1
1Nephrology Division, Clinic for Internal Medicine I, 2Cardiology Department, Clinic for Internal Medicine II, Center of Internal Medicine, University Hospital Ulm, Ulm, 3Radiology Department, Robert-Bosch Hospital, Stuttgart, and 4Institute of Biometrics, University of Ulm, Ulm, Germany
Background: The purpose of this study was to examine the efficacy of sodium 2-mercaptoethanesulfonate (MESNA), a reactive oxygen scavenger, in at-risk patients given radiographic contrast agents. Contrast-induced nephropathy (CIN) is a common complication of radiographic procedures; reactive oxygen species (ROS) could play a key role. Methods: We conducted a randomized, double-blinded, placebo-controlled trial in 100 patients with stable serum creatinine levels >= 150 µmol/l. They received an infusion of either 1,600 mg of MESNA (n = 51) or placebo (n = 49) plus 0.9% saline prior to and after contrast administration. CIN was defined as a >= 25% increase in serum creatinine after 48 h compared to baseline. Results: CIN occurred in 7 patients in the placebo group and none in the MESNA group (p = 0.005). The adjusted odds ratio for CIN was 0.17 (95% confidence interval 0.03 – 0.80, p = 0.026) in the MESNA group compared to the placebo group. Cystatin C concentrations decreased slightly in the MESNA group but increased in the control group (p < 0.05). Conclusion: MESNA plus volume expansion before and during contrast exposure was effective in this single-center study for preventing CIN compared to volume expansion alone.Correspondence to:
U. Ludwig, MD
Nephrology Division, Internal Medicine I
University Hospital of Ulm
Albert Einstein Allee 23, 89081 Ulm, Germany
Email: [email protected]
Original
The PTH (1-84)/non-PTH (1-84) ratio is a risk factor for cardiovascular events in hemodialysis patients
E. Zitt, A.H. Kirsch, M. Haueis, A. Strasak, U. Neyer, G. Mayer and A.R. Rosenkranz
Page No. 309
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (309-318)
The PTH (1-84)/non-PTH (1-84) ratio is a risk factor for cardiovascular events in hemodialysis patients
E. Zitt1,2,3, A.H. Kirsch1, M. Haueis1, A. Strasak4, U. Neyer2,3, G. Mayer1 and A.R. Rosenkranz1
1Department of Internal Medicine IV, Nephrology and Hypertension, Innsbruck Medical University, 2Department of Nephrology and Dialysis, Academic Teaching Hospital Feldkirch, 3Vorarlberg Institute for Vascular Investigation and Treatment (VIVIT), Feldkirch, and 4Department for Medical Statistics, Innsbruck Medical University, Innsbruck, Austria
Background: We hypothesized that the PTH (1-84)/non-PTH (1-84) ratio (PTH ratio) might help to assess cardiovascular risk in hemodialysis patients. Methods: In this prospective cohort study 70 prevalent hemodialysis patients were followed up to 4 years. The PTH ratio was determined at baseline. Primary outcomes were cardiovascular events (CVE) and all-cause mortality. Cumulative event-free survival was compared between patients with a ratio < 1 and those with a ratio > 1. The risk-association of the PTH ratio with CVE was examined using an adjusted Multiple Cox Proportional Hazards model. Results: A PTH ratio > 1 was found in 34 patients (49%). During follow-up 26 patients suffered a CVE. Patients with a CVE showed a higher ratio than patients with event-free survival (p = 0.033). In patients with a ratio > 1 a significantly higher number of CVE occurred (53 vs. 22%; p = 0.013), and these patients showed a significantly shorter event-free survival (p = 0.032). In an adjusted Cox-proportional hazards model a higher PTH ratio was found to be independently associated with an elevated risk for CVE (HR = 3.2; 95% CI 1.06 – 13.63; p = 0.04). Conclusions: A higher PTH (1-84)/non-PTH (1-84) ratio is associated with an increased risk for CVE in hemodialysis patients and might therefore be useful for cardiovascular risk estimation in this population.Correspondence to:
Dr. A.R. Rosenkranz
Universitätsklink für Innere Medizin IV
Nephrologie und Hypertensiologie
Medizinische Universität Innsbruck
Anichstrasse 35, 6020 Innsbruck, Austria
Email: [email protected]
Original
Education for dialysis patients lowers long-term phosphate levels and maintains health-related quality of life
A. Gardulf, M. Pålsson and U. Nicolay on behalf of the Swedish Renal Nurse group
Page No. 319
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (319-327)
Education for dialysis patients lowers long-term phosphate levels and maintains health-related quality of life
A. Gardulf1, M. Pålsson2 and U. Nicolay1 on behalf of the Swedish Renal Nurse Group1
1The Department of Laboratory Medicine, Section of Clinical Immunology, Karolinska Institute at Karolinska University Hospital, and 2Genzyme AB, Stockholm, Sweden
Aims: This study aims at biological, knowledgeable, behavioral and health-related quality of life effects of self-dosing of phosphate binders before and after an education program in patients with CKD. Material and methods: The following 12-month study included 43 patients with CKD Stage 4 – 5 and plasma phosphate levels repeatedly above recommended maximum level. Patients underwent a structured educational program regarding calcium and phosphate balance, food intake and phosphate binders comprising a 60-min session, 3 – 5 times during a 2-month period (intervention). Patients’ knowledge on the topics included in the program was assessed before and after the program as well as phosphate levels. Results: The mean plasma phosphate level had decreased significantly directly after the intervention (1.87 mmol/l, p < 0.05) and remained low over time; 1.78 mmol/l at 12 months follow-up (p < 0.001). The patients’ self-reported knowledge regarding food intake, calcium/phosphate balance and self-dosing of phosphate binders increased significantly after instruction (p < 0.001). Conclusion: A structured education program focusing on calcium and phosphate balance and self-dosing of phosphate binders significantly increased the patients’ self-reported knowledge regarding these issues which in turn led to changes in behaviors reflected in decreased long-term plasma phosphate levels.Correspondence to:
Dr. A. Gardulf
The Department of Laboratory Medicine
Section of Clinical Immunology, F79
Karolinska Institute at Karolinska University Hospital
Huddinge, 141 86 Stockholm, Sweden
Email: [email protected]
Original
C282Y hemochromatosis gene mutation and iron parameters in dialysis patients
A.M. Fernández Rodríguez, L.H. Gascón, I. García La Orden, J.P. Santos, R.G. Samper, R.M. Letosa, L. Palop Cubillo, J.C. Rodríguez Pérez and J.C. Rodríguez-Gallego
Page No. 328
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (328-335)
C282Y hemochromatosis gene mutation and iron parameters in dialysis patients
A.M. Fernández Rodríguez1, L.H. Gascón2, I. García La Orden2, J.P. Santos1, R.G. Samper2, R.M. Letosa1, L. Palop Cubillo2, J.C. Rodríguez Pérez2 and J.C. Rodríguez-Gallego3
1Nephrology Service, Hospital Ramón y Cajal, Madrid, 2Nephrology Service, Hospital Doctor Negrín, and 3Immunology Service, Hospital Doctor Negrín, Las Palmas de Gran Canaria, Spain
Background: Hereditary hemochromatosis is an autosomal recessive condition causing excessive intestinal iron absorption related to C282Y hemochromatosis mutation gene. Dialysis patients receive intravenous iron supplements as treatment for anemia. The gene mutation frequency and its influence on iron deposits and intravenous iron response are unknown in these patients. Study design: Prospective observational. Setting and participants: 290 dialysis patients in Gran Canaria, Spain. Outcomes and measurements: The C282Y hemochromatosis mutation gene was studied. Other active players in iron metabolism have not been included in this study. Red cell parameters, serum iron, transferrin and ferritin concentrations were measured every 2 months for 2 years. Results: No differences in allelic and genotypic frequencies between dialysis patients and the general population were detected. Baseline clinical or analytical parameters were similar in C282Y +/– and C282Y –/– patients. Among those who did not need intravenous iron treatment, C282Y+/– patients maintained constant serum ferritin (302.1 ± 216.7 vs. 319.5 ± 300.5 µg/l after 4 months), whereas C282Y–/– patients showed decreased levels during the same period (306.7 ± 212.2 vs. 221.6 ± 167.8 µg/l, p < 0.001). After 4 months of parenteral iron, serum ferritin increased more intensely in C282Y +/– patients than in C282Y –/– patients (934.2 ± 195.8 vs. 658.7 ± 259.9 µg/l, p < 0.001). A multivariance analysis identified the C282Y allele as the most important factor that explains this difference. Conclusions: Heterozygosity for the C282Y allele of the hemochromatosis mutation gene could be associated with differences in iron parameters in dialysis patients.Correspondence to:
A.M. Fernández Rodríguez
Servicio de Nefrología
Hospital Ramón y Cajal
Carretera de Colmenar Km 9,1
28034 Madrid, Spain
Email: [email protected]
Original
Parenteral vitamin B12 in macrocytic hemodialysis patients reduced MMA levels but did not change mean red cell volume or hemoglobin
V. Su, K. Shalansky, J. Jastrzebski, A. Martyn, G. Li, C.K. Yeung, F. Snyder and N. Zalunardo
Page No. 336
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (336-345)
Parenteral vitamin B12 in macrocytic hemodialysis patients reduced MMA levels but did not change mean red cell volume or hemoglobin
V.C.H. Su1, K. Shalansky1, J. Jastrzebski2, A. Martyn2, G. Li2, C.K. Yeung2, F. Snyder3 and N. Zalunardo2
1Vancouver General Hospital Pharmaceutical Sciences CSU, 2Department of Medicine, University of British Columbia, Vancouver, and 3Departments of Medical Genetics, Biochemistry and Molecular Biology, University of Calgary, Alberta, Canada
Aims: Unexplained macrocytic anemia was common in our hemodialysis (HD) unit. Vitamin B12 requirements may be higher in HD patients; therefore, patients may be deficient despite “normal” serum levels. We studied vitamin B12 status and the effect of parenteral vitamin B12 administration in macrocytic HD patients. A normocytic group was included for comparison. Materials and methods: Prospective cohort study of 62 HD patients (34 macrocytic, 28 normocytic) from November 2008 to March 2009. Patients were on stable doses of darbepoetin and iron replete. Vitamin B12 1,000 µg IV was given once weekly for 4 weeks and follow-up was 12 weeks. Methylmalonic acid (MMA) level was used as an indicator of vitamin B12 status. MCV and hemoglobin were also examined for an effect of B12 administration. Results: At baseline: all patients had serum B12 levels > 200 pmol/l; 97% had serum folate levels > 55 nmol/l; there was no difference in serum B12 levels between groups (504 vs. 571 pmol/l, p = 0.18); MMA was higher in the macrocytic group (0.56 vs. 0.48 µmol/l, p = 0.048) and hemoglobin (Hg) was lower (119 vs. 125 g/l, p = 0.03); median darbepoetin dose was equivalent (20 µg/week). Following IV vitamin B12, the macrocytic group had a greater and more sustained reduction in MMA (–0.064 vs. –0.0066 µmol/l/wk, p = 0.004). There was no improvement in hemoglobin (Hg), reticulocyte count or MCV in either group. Median darbepoetin dose was unchanged. Conclusions: IV vitamin B12 led to a sustained decline in MMA levels in macrocytic patients, suggesting functional vitamin B12 deficiency at baseline. However, there were no significant changes in Hg or darbepoetin dose.Correspondence to:
Dr. N. Zalunardo
Clinical Assistant Professor
Division of Nephrology
Gordon and Leslie Diamond Health Care Centre
5th Floor, 2775 Laurel St
Vancouver, BC, Canada
Email: [email protected]
Original
Factors associated with silent cerebral microbleeds in hemodialysis patients
T. Naganuma, Y. Takemoto, T. Yamasaki, H. Shima, T. Shoji, E. Ishimura, Y. Nishizawa, M. Morino, M. Okamura and T. Nakatani
Page No. 346
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (346-355)
Factors associated with silent cerebral microbleeds in hemodialysis patients
T. Naganuma1, Y. Takemoto1, T. Yamasaki1, H. Shima2, T. Shoji2, E. Ishimura2, Y. Nishizawa2, M. Morino3, M. Okamura4 and T. Nakatani1
1Department of Urology, 2Department of Nephrology, 3Department of Neurosurgery, Osaka City University Graduate School of Medicine, and 4Department of Nephrology, Ohno Memorial Hospital, Osaka, Japan
Background: The recent development of gradient-echo T2*-weighted magnetic resonance imaging (MRI) has enabled the highly accurate detection of prior cerebral microbleeds (CMBs), which might indicate a higher risk of future intracerebral hemorrhage (ICH) and be a marker of cerebral small-vessel disease in the general population. The present study investigated the clinical factors associated with the presence of CMBs in hemodialysis (HD) patients. Methods: Cranial MRI, including T2*-weighted MRI, was performed on 179 HD patients without symptomatic cerebrovascular disease and 58 healthy control subjects, and we investigated the prevalence of CMBs and clinical factors associated with the presence of CMBs. We also investigated the relationship between CMBs and other cerebral small-vessel diseases. Results: The prevalence of CMBs was significantly higher in the HD patients than in the healthy subjects (45 patients (25.1%) vs. none in the healthy controls (0%), p < 0.0001). Multiple logistic regression analysis showed that independent and significant factors associated with the presence of CMBs were age, systolic blood pressure, diastolic blood pressure and pulse pressure. Moreover, the presence of CMBs correlated significantly with the presence of lacunar infarcts, periventricular hyperintensity and deep and subcortical white matter hyperintensity. Conclusions: These findings indicated a high prevalence of CMBs among HD patients, and that older age and high blood pressure were strong factors associated with the presence of CMBs. Moreover, CMBs were closely associated with other cerebral small-vessel diseases.Correspondence to:
T. Naganuma, MD
Department of Urology
Osaka City University Medical School
1-4-3 Asahi-machi, Abeno-ku
Osaka, 545-8585, Japan
Email: [email protected]
Original
The association of the Friesinger score and estimated glomerular filtration rate in an urban South Asian patient population
F. Javed, G.N. Nadkarni, S.A. Khan, M.S. Sabharwal, N. Annapureddy, A. Benjo, E.F. Aziz and E. Herzog
Page No. 356
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (356-361)
The association of the Friesinger score and estimated glomerular filtration rate in an urban South Asian patient population
F. Javed1, G.N. Nadkarni1, S.A. Khan2, M.S. Sabharwal1, N. Annapureddy1,A. Benjo1, E.F. Aziz1 and E. Herzog1
1St. Luke’s-Roosevelt Hospital Center, University Hospital of Columbia University College of Physician and Surgeons, New York, NY, and 2Department of Medicine/Pediatrics, Wayne State University, Detroit, MI, USA
Aim: The correlation between kidney function and coronary artery disease (CAD) severity as assessed by an angiographic score has not yet been studied in the South Asian population. We sought to estimate the association by performing a single-center, cross-sectional study. Patients and methods: The estimated glomerular filtration rate (eGFR) calculated by the CKD-EPI equation and the Friesinger score to quantify the severity of CAD were the primary endpoints in patients undergoing coronary angiograms. Results: The mean eGFR was significantly lower in participants with a Friesinger score of > 5 compared to participants with a score of < 5 (73 vs. 86 ml/min/1.73 m2 by MDRD). In univariate analysis, an eGFR of < 55 ml/min/1.73 m2 was associated with a 9.5-fold increased odds of a higher Friesinger score compared to an eGFR >= 55 ml/min/1.73 m2 (p = 0.043), which was unchanged in multivariate analysis. In multivariate analysis, a 10 ml/min/1.73 m2 decrease in eGFR was associated with a 1.63-fold increased odds of a higher score (95% CI 1·10 – 2.37, p = 0.042). Traditional risk factors such as a history of previous CAD, hypertension, and dyslipidemia remained predictors of a higher Friesinger score. Conclusion: Our study demonstrates that kidney function as assessed by eGFR is a significant independent predictor of severity of CAD as determined by the Friesinger score.Correspondence to:
F. Javed, FSc, MD, MRCP
St. Luke’s Roosevelt Hospital Center
Columbia University College of Physicians and Surgeons
1111 Amsterdam Ave, New York, NY 10025, USA
Email: [email protected], [email protected]
Nephrology Education
Familial collapsing focal segmental glomerulosclerosis
V. Liakopoulos, A. Huerta, S. Cohen, M.R. Pollak, R.A. Sirota, K. Superdock and G.B. Appel
Page No. 362
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (362-368)
Familial collapsing focal segmental glomerulosclerosis
V. Liakopoulos4, A. Huerta1, S. Cohen1, M.R. Pollak5, R.A. Sirota2, K. Superdock3 and G.B. Appel1
1Departments of Medicine and Division of Nephrology of Columbia University College of Physicians and Surgeons, New York, NY, 2Abington Memorial Hospital, Abington, 3Lankenau Hospital, Wynnewood, PA, USA, 4University of Thessaloniki, Thessaloniki, Greece and 5Harvard Medical School, Boston, MA, USA
The majority of patients with non-HIV-related collapsing focal segmental glomerular sclerosis (FSGS) have idiopathic disease. Only a few genetic forms associated with rare syndromes have been described in families. Here we report two families with multiple members who have collapsing FSGS with no clear associated secondary etiology. Genetic analysis revealed a defect in the TRPC6 gene in one family, but excluded all known common inherited podocyte defects in the other family. The course and response to treatment differed dramatically among members of the same family.Correspondence to:
Dr. G.B. Appel
Professor of Clinical Medicine
Columbia U. College of Physicians and Surgeons
622 West 168th Street, PH 4124
New York, NY 10032, USA
Email: [email protected]
Nephrology Education
An adolescent with marked hyperimmuno-globulinemia E showing minimal change nephrotic syndrome and a STAT3 gene mutation
K. Miyazaki, T. Miyazawa, K. Sugimoto, S. Fujita, H. Yanagida, M. Okada and T. Takemura
Page No. 369
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (369-373)
An adolescent with marked hyperimmuno-globulinemia E showing minimal change nephrotic syndrome and a STAT3 gene mutation
K. Miyazaki, T. Miyazawa, K. Sugimoto, S. Fujita, H. Yanagida, M. Okada and T. Takemura
Department of Pediatrics, Kinki University School of Medicine, Osaka, Japan
We encountered a patient with marked hyperimmunoglobulinemia E who had a mutation of the signal transducer and activator of transcription 3 gene (STAT3) and developed minimal change nephrotic syndrome (MCNS). From early infancy, the patient showed repeated episodes of refractory chronic eczema accompanied by impetigo vulgaris with cicatrization, as well as otitis media. Serum IgE was markedly increased (from 4,000 to 25,000 IU/ml). The nephrotic syndrome (NS) frequently relapsed, and was alternately responsive and resistant to corticosteroids. The STAT3 mutation was heterozygous, located in exon 23 of the transactivation domain and causing A744V substitution. Presently treated with mycophenolate mofetil, the patient has less frequent MCNS recurrences. Increases in circulating Th2 cytokines and IgE combined with suppression of the Th1 cytokine interferon-gamma caused by the STAT3 abnormality, presumably caused MCNS by altering the Th1/Th2 balance among T-lymphocytes. To our knowledge, this is the first report of type I hyper-IgE syndrome (HIES) showing a STAT3 gene mutation and MCNS.Correspondence to:
T. Takemura, MD, PhD
Department of Pediatrics
Kinki University School of Medicine
377-2 Ohno Higasi, Osaka-Sayama-shi
Osaka 589-8511, Japan
Email: [email protected]
Nephrology Education
Cryoglobulinemic membranoproliferative glomerulonephritis: beyond conventional therapy
G. Colucci, C. Manno, G. Grandaliano and F.P. Schena
Page No. 374
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (374-379)
Cryoglobulinemic membranoproliferative glomerulonephritis: beyond conventional therapy
G. Colucci*, C. Manno*, G. Grandaliano and F.P. Schena
Renal, Dialysis and Transplant Unit, Department of Emergency and Organ Transplant, University of Bari, Bari, Italy
*The Authors contributed equally
Membranoproliferative glomerulonephritis associated with Type II cryoglobulinemia is the predominant type of HCV-related glomerulonephritis. Immunosuppressive and anti-viral therapy is alternately used to treat it, but the results are not always satisfactory or lasting. In this paper we report 3 cases of cryoglobulinemic membranoproliferative glomerulonephritis, treated with different and personalized therapeutic approaches by using conventional therapy and new drugs such as mycophenolate mofetil and rituximab. Our case series report emphasizes the importance of choosing the treatment for each patient, taking into account many factors: age, severity of liver and renal involvement, extra-renal manifestations, any previous treatment, contraindications or adverse events and last but not least the balance between immunosuppression and virus activity.Correspondence to:
F.P. Schena, MD, FASN
Professor of Nephrology
Renal, Dialysis and Transplant Unit
Department of Emergency and Organ Transplant
University of Bari, Policlinico
Piazza G. Cesare 11, 70124 Bari, Italy
Email: [email protected]
Nephrology Education
Cyclosporin A in adult patients with Henoch-Schönlein purpura nephritis and nephrotic syndrome; 5 case reports
P. Kalliakmani, E. Benou and D.S. Goumenos
Page No. 380
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (380-383)
Cyclosporin A in adult patients with Henoch-Schönlein purpura nephritis and nephrotic syndrome; 5 case reports
P. Kalliakmani, E. Benou and D.S. Goumenos
Department of Internal Medicine-Nephrology, University Hospital, Patras, Greece
Henoch-Schönlein purpura (HSP) is usually followed by mild renal involvement, but heavy proteinuria may also occur. Limited experience with cyclosporin A in children shows reduction of proteinuria. In this report, the use of cyclosporin A in 5 adult HSP patients with nephrotic-range proteinuria is described. Cyclosporin A in combination with prednisolone was given in 3 patients with HSP nephritis and nephrotic syndrome after a course of other immunosuppressive drugs and in 2 patients as initial treatment. All patients showed complete or partial remission of nephrotic syndrome with cyclosporin A and preserved stable renal function over a follow-up period of 5 years. We conclude that the combination of cyclosporin A with corticosteroids is effective in inducing remission of nephrotic syndrome in adult patients with HSP nephritis.Correspondence to:
Prof. D.S. Goumenos
Internal Medicine-Nephrology
University Hospital of Patras
26500 Patras, Greece
Email: [email protected]
Nephrology Education
A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections
C. Sakoda, T. Kusaba, T. Adachi, K. Sonomura, T. Kimura, M. Nakayama, N. Kishimoto, H. Nakagawa, M. Okigaki, T. Hatta, H. Matsubara and Y. Mori
Page No. 384
Abstract
Clinical Nephrology, Vol. 75 – No. 4/2011 (384-388)
A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections
C. Sakoda1*, T. Kusaba1*, T. Adachi2, K. Sonomura1, T. Kimura1, M. Nakayama1, N. Kishimoto1, H. Nakagawa1, M. Okigaki1, T. Hatta2, H. Matsubara1 and Y. Mori1
1Division of Cardiology and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, and 2Division of Nephrology, Omihachiman Community Medical Center, Shiga, Japan
A 62-year-old female was admitted to our hospital for investigation of acute progressive renal insufficiency and a systemic inflammatory reaction, despite treatment with several antibiotics. Laboratory data revealed severe renal insufficiency and positive titers for the myeloperoxidase anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies. The deterioration of her general status did not allow us to perform the renal biopsy. Although corticosteroid therapy, hemodialysis, and plasma exchange were concomitantly initiated, pulmonary hemorrhage occurred several days after admission. Mechanical ventilation support was provided and continuous hemodiafiltration was carried out, following which the respiratory failure improved immediately. However, she developed clinical depression and suicidal behavior under the intensive therapy. Therefore, plasma exchange was discontinued and corticosteroid was tapered as quickly as possible. Four months after admission, platelet transfusion and short-term mechanical ventilation support improved the pulmonary hemorrhage; however, her mental status deteriorated despite psychiatric consultation and treatment with a tranquilizer. Thereafter, severe and serious systemic infection due to various pathogens including Staphylococcus aureus, Cytomegalovirus, Pneumocystis jiroveci, Pseudomonas aeruginosa, and Bacteroides recurred, and she died from systemic invasive aspergillosis (IA). We suspected severe immunosuppression caused by various factors, such as predonisolone administration, chronic renal failure on maintenance hemodialysis, depression, and malnutrition due to chronic inflammation and granulocytopenia as a side effect of ganciclovir. When treating rapidly progressive glomerulonephritis, immunosuppressive status should be carefully monitored regarding not only the dosage of therapeutic regimen but also the mental health status and nutrition of the patient.Correspondence to:
C. Sakoda, MD
Division of Cardiology and Nephrology
Department of Medicine
Kyoto Prefectural University of Medicine
465 Kajii-cho, Kawaramachi-Hirokoji
Kamigyo-ku Kyoto 602-8566, Japan
Email: [email protected]
Letter to the Editor
Angiomyolipoma manifested as accessory kidney mass
Y.B. Jeong, H.J. Kim, K.P. Kang, W. Kim, S.K. Park and S. Lee
Page No. 389
Abstract
Angiomyolipoma manifested as accessory kidney mass
Y.B. Jeong, H.J. Kim, K.P. Kang, W. Kim, S.K. Park and S. Lee
