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Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (485-490)

Efficacy of sodium thiosulfate for the treatment for calciphylaxis

L. Noureddine¹, M. Landis¹,², N. Patel¹ and S.M. Moe¹,³

¹Division of Nephrology, Indiana University, University School of Medicine, Indianapolis, IN, ²Department of Dermatology, Mayo Clinic, Jacksonville, FL, and ³Roudebush VAMC, Indianapolis, IN, USA
Background/Aims: Calciphylaxis is a rare condition with dismal prognosis that affects patients with ESRD. Sodium thiosulfate (STS) may play a therapeutic role but its proposed efficacy is based on case reports and thus subject to publication bias. Methods: We identified all patients who received STS for any indication over a 5-year period through pharmacy records of 4 hospitals, retrospectively reviewing medical records for risk factors, laboratory values, the response of skin lesions to STS, and mortality. Results: 14 patients received STS for calciphylaxis over 5 years. Following STS administration, pain decreased in 71% of patients, and 70% had an improvement in their lesions. Those who did not improve or stabilize their skin lesions tended to have more advanced skin lesions, were on renal replacement therapy longer, were more obese and received less total dose of STS. However, despite STS, there was a 71% mortality rate, with 50% of subjects dying within 6 months. Conclusion: We conclude in this study of all subjects who received STS at our Institution that STS is an effective treatment for the pain and skin lesions of calciphylaxis if given in the early stages of disease and for a consistent period of time. However, there is little impact on overall mortality compared to historical published cohorts.Correspondence to:
S.M. Moe, MD
Professor of Medicine and Anatomy and Cell Biology
Division of Nephrology
Vice-Chair for Research
Department of Medicine
Indiana University School of Medicine
1001 W. 10th Street, OPW 526
Indianapolis, IN 46202, USA
Email: smoe@iupui.edu

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (491-496)

The effect of immunosuppressive treatment on arterial stiffness and matrix Gla protein levels in renal transplant recipients

O. Gungor¹, F. Kircelli¹, J.J. Carrero², E. Hur¹, M.S. Demirci¹, G. Asci¹ and H. Toz¹

¹Ege University School of Medicine, Division of Nephrology, Izmir, Turkey, and ²Division of Renal Medicine, Centre for Gender Medicine and Centre for Molecular Medicine, Karolinska Institutet, Sweden
Introduction: Arterial stiffness is a risk marker for cardiovascular events. In this study we aimed to compare the effect on calcineurin inhibitors (CNI) and mammalian Target of Rapamycine inhibitors (mTORi) on arterial stiffness in renal transplant patients. Patients and methods: 81 renal transplant patients under CNI-based or mTORi-based protocol for at least 6 months were included in the study. Arterial stiffness was measured by using the SphygmoCor device (AtCor Medical, Sydney, Australia). Vitamin K-dependent, calcification inhibitor matrix Gla protein (MGP) concentrations were quantified by ELISA methods (Biomedica, Vienna, Austria). Results: 34 patients were on mTORi-based and 47 on CNI-based immunosuppression. Mean age was 37.9 ± 10.8 (18 – 71) years and 45% were female. Age, gender, graft functions and follow-up period of the groups were similar. Augmentation index was 15.2 ± 12.6% in CNI and 18.8 ± 14.0% in mTORi groups (p > 0.05). There was no difference regarding carotid-femoral pulse wave velocity between groups. Arterial stiffness was positively correlated with age, total cholesterol, LDL cholesterol, mean arterial pressure (MAP) and proteinuria. MGP levels were higher in the mTORi group but were not predictors for carotid-femoral pulse wave velocity. Conclusion: Rather than specific immunosuppressive drug effects, conventional risk factors, blood pressure and proteinuria are the most important predictors for arterial stiffness in renal transplant patients.Correspondence to:
O. Gungor, MD
Ege University School of Medicine
Division of Nephrology
35100, Bornova, Izmir, Turkey
Email: ozkan.gungor@yahoo.com

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (497-505)

Epidemiology of hemodialysis vascular access in the United States

E. Lacson Jr.

Fresenius Medical Care-North America, Waltham, MA, USA
Reemergence of the importance of vascular access in the care of the chronic hemodialysis patient has gained prominence due to renewed interest in clinical outcomes and evidence-based interventions. Further fueled by anticipated regulatory changes in the reimbursement for dialysis care in the United States by 2011 and beyond, the drive to improve quality of care for hemodialysis patients has identified vascular access issues as a key contributor to outcomes. Focus has shifted from simply providing any hemodialysis vascular access to a strong preference for the use of native arteriovenous fistulas and subsequently to a need for reducing exposure to central venous catheters. Combined, these goals have forced a reevaluation of the role of arteriovenous grafts. The context and events associated with the evolution of thinking on these issues as well as available data supporting them are discussed. The key leadership role of nephrologists is emphasized along with a summary of problems and proposed solutions.Correspondence to:
E. Lacson Jr., MD, MPH, FACP, FASN
Vice President, Clinical Science, Epidemiology, and Research
Fresenius Medical Care, North America
920 Winter Street
Waltham, MA, 02451-1457, USA
Email: elacsonj@fmc-na.com

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (506-510)

Fabry disease: results of the first UK hemodialysis screening study

E.F. Wallin¹, M.R. Clatworthy¹,² and N.R. Pritchard¹

¹Division of Renal Medicine, University of Cambridge Department of Medicine, and Addenbrooke’s Hospital, and ²Cambridge Institute for Medical Research, University of Cambridge Department of Medicine, Cambridge, UK
Background: Fabry disease is an X-linked lysosomal storage disorder in which deficiency of α-Galactosidase A (α-Gal A), leads to accumulation of glycosphingolipids in the vascular endothelium, kidneys and heart. Males with classical disease present in childhood, however some individuals with low levels of α-Gal A activity present atypically with adult onset renal impairment. Screening studies in patients with established end-stage renal failure (ESRF) suggest that up to 1.5% of patients have sub-normal α-Gal A levels. We used the dried blood spot (DBS) enzyme activity test to screen for undiagnosed Fabry disease in patients with ESRF. Methods: Male hemodialysis patients treated at a single UK center (n = 155) were screened using the DBS assay. In patients with low enzyme activity on DBS, α-Gal A activity was assessed in plasma and leucocytes. Results: 8 of the 155 (5%) patients screened showed low enzyme activity on the DBS assay. Confirmatory testing of plasma and leucocyte α-Gal A activity showed normal activity in all cases tested, indicating a false positive DBS result. Conclusions: This study is the first screening program in UK hemodialysis patients using the DBS test and did not identify any new cases of Fabry disease. In this cohort, the DBS enzyme assay had a false positive rate of 2.6%, emphasizing the need for validation with alternative techniques.Correspondence to:
E. Wallin, MB BChir
C/o Dialysis Unit, Box 118
Addenbrooke’s Hospital
Hills Road, Cambridge, CB2 2QQ, UK
Email: lizwallin@doctors.org.uk

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (511-517)

Combination treatment with corticosteroid, cyclosporine A, and mycophenolate in refractory nephrotic syndrome

H.R. Jang¹*, H.W. Jung²*, Y.-J. Lee³, J.E. Lee¹, W. Huh¹, D.J. Kim¹, H.Y. Oh¹ and Y.-G. Kim¹

¹Nephrology Division, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, ²Nephrology Division, Department of Medicine, Incheonsarang Hospital, Incheon, and ³Nephrology Division, Department of Medicine, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea

*Both authors contributed equally to this work.
Background/aims: Refractory nephrotic syndrome (NS) is problematic because the optimal therapy for this disease is unclear and because persistent NS progresses eventually to end-stage renal disease. We report our experience using a combination of corticosteroid, cyclosporine A (CsA), and mycophenolate mofetil (MMF) to treat 10 refractory NS patients. Methods: Ten refractory NS patients, who showed resistance to corticosteroid and CsA, were treated with triple immunosuppressive therapy. Cyclophosphamide and MMF had been used previously in 6 patients, but had failed to induce remission. Results: Triple immunosuppressive therapy was discontinued after 4 months in 1 patient because of progressive azotemia. Partial remission was achieved in 9 of the 10 patients after 10 months, and remission was maintained during the treatment (urine protein to creatinine ratio, mg/mg, baseline vs. 12th month; 5.7 ± 1.8 vs. 1.4 ± 0.7). Renal function was preserved in these 9 patients (estimated GFR, ml/min/1.73 m², baseline vs. 12th month; 71.4 ± 29.1 vs. 68.9 ± 31.5). Of the 7 patients who discontinued triple immunosuppressive therapy, remission and renal function were maintained in 4 patients. Conclusion: Triple immunosuppressive therapy significantly reduced proteinuria and preserved renal function in refractory NS patients, indicating a promising role of this therapy for refractory NS.Correspondence to:
Y.-G. Kim, MD
Division of Nephrology
Department of Medicine
Samsung Medical Center
Sungkyunkwan University School of Medicine
50 Irwon-dong, Gangnam-gu
135-710, Seoul, Korea
Email: ygkim26@skku.edu

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (518-523)

Risk factors for chronic kidney disease among human immunodeficiency virus-infected patients: A European case control study

A. Di Biagio¹, R. Rosso¹, F. Vitale¹, F. Cardinale², M.P. Sormani³, G. Secondo¹, L. Di Stefano¹ and C. Viscoli¹

¹Department of Infectious Diseases, University of Genoa, San Martino Hospital, ²Department of Clinic Epidemiology, National Institute for Cancer Research, and ³Biostatistics Unit, Dept of Health Science, University of Genoa, Genoa, Italy
Objectives: Renal dysfunction is a common complication in human immunodeficiency virus (HIV)-infected patients and can be attributed to direct viral damage, comorbidities or drug toxicity. The aim of this study was to assess cross-sectional correlates of renal damage in a contemporary European cohort of patients. Methods: We performed a case-control study from our cohort of 750 HIV-infected adults over a period of 5 months. We assessed renal damage by either proteinuria (≥+ on urine dipstick), reduced creatinine clearance (< 60 ml/min) or reduced estimated glomerular filtration rate (eGFR) of < 60 ml/ min/1.73 m². The characteristics of cases and controls were compared in analysis and in multivariate logistic regression models with stepwise selection. Results: Approximately 50% of the screened 106 patients had a qualifying abnormality. Altogether, we identified 55 cases with 110 age- and gender-matched controls. Mean eGFR was 90.7 (4.8) for cases vs. 106.1 (2.3) ml/min/1.73 m² for controls (p = 0.001). Cases had a longer duration of HIV infection, more complex regimen, longer exposure to antiretroviral therapy and a more frequent diagnosis of acquired immune-deficiency syndrome (AIDS) and hepatitis C virus (HCV) infection. In the logistic multivariate model, renal damage remained significantly associated with longer known duration of HIV infection (OR 2.88, 95% CI: 1.28 – 6.46, p = 0.01), AIDS defining condition (OR 1.09 95% CI: 1.03 – 1.16, p = 0.002) female gender (OR 2.01, 95% CI: 0.96 – 4.18, p = 0.06), and HCV infection (OR 2.12, 95% CI: 0.99 – 4.52, p = 0.05). Conclusions: Duration, antiretroviral regimen and coincidental HCV impacted the frequency of renal abnormalities in our patients.Correspondence to:
A. Di Biagio, MD
Dept. Infectious Diseases
University of Genoa, San Martino Hospital
Pad. Specialità Complesse, Piano -2
Largo R. Benzi, 10, 16132 Genoa, Italy
Email: antonio.dibiagio@hsanmartino.liguria.it

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (524-533)

Anemia, inflammation and health-related quality of life in chronic kidney disease patients

Y.M.K. Farag¹*, S.R. Keithi-Reddy¹*, B.V. Mittal¹, S.P. Surana¹, F. Addabbo², M.S. Goligorsky² and A.K. Singh¹

¹Renal Division, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, and ²Department of Medicine and Department of Pharmacology, Renal Research Institute, New York Medical College, Valhalla, NY, USA

*Both the authors have equally contributed to the manuscript and are to acknowledged co-first authors.
Background: There is controversy regarding whether an incremental increase in hemoglobin levels is associated with improvements in health-related quality of life (HRQOL) in chronic kidney disease (CKD) patients treated with erythropoiesis-stimulating agents (ESAs). We hypothesized that HRQOL in anemic CKD patients has a multifactorial etiology, including the effects of anemia and inflammation. Methods: 69 non-dialysis CKD patients over 18 years of age with a mean estimated glomerular filtration rate (eGFR) of 43.7 ± 28.8 ml/min/1.73 m² were divided into anemic and non-anemic cohorts. Kidney disease quality of life (KDQOL) was prospectively recorded using Short Form (SF)-36 components of KDQOL-SF-™ version 1.3 questionnaire. Inflammation was assessed by using a composite of interleukin (IL)-6, IL-8 and tumor necrosis factor (TNF)-α levels in the upper two quartiles. Results: Anemic patients had significantly worse SF-36 components of KDQOL-SF-™ version 1.3, including SF-12 mental component (p = 0.02), role emotional (p = 0.002) and physical function (p = 0.01) compared to patients without anemia. However, in multiple linear regression models, adjusted for GFR, age, gender and inflammatory markers including C-reactive protein (CRP), albumin, ferritin, IL-6, IL-8 and TNF-α, anemia predicted mental components of SF-36 (SF-12 mental component (p = 0.02) and role emotional (p = 0.04)) but not physical components (SF-12 physical component (p > 0.05) and physical function (p > 0.05), supporting the multifactorial nature of reduced HRQOL in anemic patients. Conclusions: Reduced HRQOL in anemic patients is likely related to both anemic and inflammatory status. Prospective studies will be needed to evaluate whether modulating the inflammatory state independent of changes in the hemoglobin concentration improves physical components of HRQOL.Correspondence to:
Dr. A.K. Singh
Renal Division, Brigham and Women’s Hospital
75 Francis Street, Boston, MA 02115, USA
Email: asingh@partners.org

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (534-541)

Impact of short term use of interdialytic 60% ethanol lock solution on tunneled silicone catheter dysfunction

A.-E. Heng¹, M.H. Abdelkader¹, M. Diaconita², A. Nony³, A. Guerraoui⁴, N. Caillot⁵, M. Rince², P. Deteix¹,⁶ and B. Souweine¹,⁶

¹CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Service de Néphrologie Réanimation Médicale, Clermont-Ferrand, ²CHU Limoges, Hôpital Dupuytren, Service de Néphrologie transplantation rénale, Limoges, ³Centre Hospitalier Jacques Cœur, Service de Néphrologie, Bourges, ⁴Centre Hospitalier de Vichy, Service de Néphrologie, Vichy, ⁵NRC Médical, Saint Saturnin, and ⁶Univ Clermont ¹, UFR Médecine, Clermont-Ferrand, France
Introduction: Ethanol lock (EL) is used to reduce catheter infections, but its impact on dialysis catheter dysfunction has not been studied. Methods: We analyzed the rate of dialysis silicone catheter dysfunction after an interdialytic 60% EL in an open prospective controlled non crossover cohort study, with each patient being his or her own control. The study was divided into three consecutive 2-week periods: the pre- and post-intervention periods, in which interdialytic standard locks (SL) were used, and the intervention period, in which one EL was instilled during the first week, and two consecutively in the second week. We analyzed the rate of catheter dysfunction (exchange, use of fibrinolytic, reversing the lines, difficulty in instilling or withdrawing fluid) after EL or SL. Results: In 30 patients, 90 EL were instilled. The rate of catheter dysfunction increased during the EL period (2 – 13%, p < 0.001), and between the two consecutive dialysis sessions before and after EL (p < 0.05). It decreased between the two consecutive dialysis sessions after EL and the following dialysis session after an SL (p < 0.05). No catheter was removed. The urea reduction ratio did not differ in dialysis after an SL and after an EL (0.77 vs. 0.73, p = 0.17). Conclusion: Short term EL is associated with a transient increase in catheter dysfunction, without resulting in catheter removal or decreasing dialysis efficiency.Correspondence to:
A.-E. Heng, MD
CHU Clermont-Ferrand
Hôpital Gabriel Montpied
Service de Néphrologie Réanimation Médicale
Pole REUNNIRH, Clermont-Ferrand, 63003 France
Email: aheng@chu-clermontferrand.fr

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (542-546)

Early-onset of disseminated cryptococcal infection in two renal transplant recipients

I. Castro-Ferreira¹, C. Carvalho¹, L. Coentrão¹, S. Gaião¹, M. Bustorff¹, J. Santos¹, S. Sampaio¹, R. Portugal² and M. Pestana¹

¹Nephrology, Research and Development Unit, Faculty of Medicine, University of Porto, Porto, and ²Department of Pathology, Hospital São João, Porto, Portugal
Cryptococcosis is the third most common invasive fungal infection in organ transplant recipients after candidiasis and aspergillosis. Newly acquired and reactivation of latent infection are the major causes of infection, with typical later-onset and mainly as disseminated infection. The type and intensity of immunosuppression, diabetes mellitus and other co-morbidities as well as uremia seem to be important determinants on clinical presentation and outcome. Moreover, the diagnosis is not always apparent since it usually presents subacutely, as well as mimicking bacterial infections, which may be responsible for a delay in the diagnosis. Thus, a high degree of suspicion and need of invasive procedures for microbiological and histological evaluation are critical for definitive diagnosis and prompt institution of adequate treatment. We report two cases of disseminated cryptococcosis with different presentations and with an early-onset after renal transplantation.Correspondence to:
I. Castro-Ferreira
Avenida Prof. Hernani Monteiro
4202-451 Porto, Portugal
Email: inescastroferreira@sapo.pt

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (547-549)

Giant ureteral stone in a patient with a single functioning kidney: a case report

Y.B. Jeong, J.K. Park, H.J. Kim, Y.G. Kim and M.K. Kim

Department of Urology, Chonbuk National University Medical School and Research Institute of Clinical Medicine, Jeonju, Korea
A 43-year-old man presented with long-standing left flank pain. A plain abdominal radiograph and intravenous urography (IVU) revealed a giant ureteral stone measuring 6.2 × 2.2 cm causing ureteral obstruction. A non-enhanced computerized tomography (CT) scan showed a significantly atrophied right kidney and left hydronephroureterosis with a giant stone. A left transperitoneal laparoscopic ureterolithotomy was performed with excellent results.Correspondence to:
M.K. Kim, MD
Department of Urology
Chonbuk National University Medical School
634-18, Geumam-dong, Dukjin-gu
Jeonju #561-712, Korea
Email: mkkim@chonbuk.ac.kr

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (550-556)

Glomerulonephritis emerging shortly after Puumala hantavirus infection: a report on 7 patients

M. Miettinen¹, S. Mäkelä²,³, M. Haapala⁴, A. Helanterä⁵, H. Helin⁶, T. Vänttinen⁷ and J. Mustonen²,³

¹Department of Internal Medicine, Central Hospital of Jyväskylä, ²Medical School, University of Tampere, ³Department of Internal Medicine, Tampere University Hospital, Tampere, ⁴Department of Internal Medicine, Länsi-Uusimaa Hospital, Helsinki and Uusimaa Health District, Tammisaari, ⁵Department of Internal Medicine, Päijät-Häme Central Hospital, Lahti, ⁶Department of Pathology, Helsinki University Hospital, University of Helsinki, and ⁷Department of Internal Medicine, Mikkeli Central Hospital, Mikkeli, Finland
Aims: Nephropathia epidemica (NE) is mild type of hemorrhagic fever caused by Puumala (PUU) hantavirus. Renal biopsy typically shows acute tubulointerstitial nephritis and complete recovery is the usual outcome. We previously described 5 patients with membranoproliferative glomerulonephritis (MPGN) after acute NE. We now report on 7 more patients who developed biopsy-confirmed glomerulonephritis (GN) during the convalescent phase of NE. Material and methods: We present case histories of 7 patients with nephrotic-range proteinuria concomitant with hematuria after serologically verified NE. Results: Renal biopsy specimens disclosed MPGN in 5 patients, membranous GN (MGN) in 1 and mesangial GN (MesGN) in 1. All patients achieved remission of nephrotic syndrome within a median time of 0.6 years (range 0.5 – 5.5 y). The median follow-up time was 1.7 years (0.7 – 15.6 y). Conclusions: As a rare phenomenon, nephrotic syndrome may emerge during the convalescent phase of acute PUU hantavirus infection. In most cases the prognosis of GN caused by NE seems to be favorable.Correspondence to:
M. Miettinen, MD
Department of Internal Medicine
Central Hospital of Jyväskylä
Keskussairaalantie 19
40620 Jyväskylä, Finland
Email: marja.miettinen@ksshp.fi

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (557-559)

Purple urine bag syndrome: case report and literature review

K.-H. Kang, K.-H. Jeong, S.-K. Baik, W.-Y. Huh, T.-W. Lee, C.-G. Ihm, S.-H. Lee and J.-Y. Moon

Department of Internal Medicine, College of Medicine, Kyung Hee University, Seoul, Republic of Korea
Purple urine bag syndrome (PUBS) is a medical syndrome in which there is purple discoloration of the urine of catheterized patients as well as discoloration of the collecting bag and the associated tubing. This rare condition, which mostly affects women, is generally associated with catheter-associated urinary tract infection, chronic constipation and alkaline urine. PUBS may be caused by sequential chemical reactions involving tryptophan from food in the gastrointestinal tract. The clinical course of PUBS is generally benign, and intensive treatment is not usually needed. We present 3 cases of this unusual and interesting phenomenon and a literature review.Correspondence to:
K.-H. Jeong, MD, PhD
Department of Internal Medicine
College of Medicine
Kyung Hee University
1, Hoegi-dong, Dongdaemun-gu
Seoul, Republic of Korea
Email: aprilhwan@naver.com

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 (560-564)

Massive venous thrombosis of inferior vena cava as primary manifestation of renal Ewing’s sarcoma

D. Rizzo¹*, G. Barone¹*, A. Ruggiero¹, P. Maurizi¹, I.F. Furfaro¹, M. Castagneto² and R. Riccardi¹

¹Pediatric Oncology Division, and ²Surgery Division, Catholic University, “A. Gemelli” Hospital, Rome, Italy
We report an extraordinarily rare case of a 17-year-old male with an extraskeletal Ewing’s sarcoma (ESS) of the kidney and a massive thrombosis involving the inferior vena cava (IVC), from the iliac axis to the right atrium. This onset resembled renal cell carcinoma (RCC), although histological examination revealed it was an extraskeletal Ewing’s sarcoma/peripheral neuro-ectodermal tumor (EES/PNET). EES/PNET should benefit from neoadjuvant chemotherapy to reduce the risk of metastasis and of recurrent disease due to delay in suitable treatment. Therefore, in the presence of a renal mass with tumor extension of IVC, it is reasonable to bear in mind that other tumors, apart from RCC, could occur. In such cases, a US or CT-scan guided biopsy could be useful.Correspondence to:
D. Rizzo, MD
Pediatric Oncology Division
Catholic University
“A. Gemelli” Hospital
Largo “A. Gemelli”, 8 00168, Rome, Italy
Email: rizzo.dany@libero.it

Abstract

Clinical Nephrology, Vol. 75 – No. 6/2011 – Letter to the editor

Re: Four cases of postrenal renal failure induced by renal stones associated with rotavirus infection

Correspondence to:
F. Cachat, MD
Department of Pediatrics
Pediatric Nephrology Unit
University Hospital
1011 Lausanne-CHUV, Switzerland
Email: Francois.Cachat@hopital-riviera.ch

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Volume 75, No. 6/2011(June)