Volume 80 (2013), No. 5/2013(November)
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Original
Higher fibroblast growth factor-23 concentrations associate with left ventricular systolic dysfunction in dialysis patients
Shailendra Sharma, Jacob Joseph, Michel Chonchol, James S. Kaufman, Alfred K. Cheung, Zahi Rafeq, Gerard Smits, Jessica Kendrick, and the HOST Investigators
Page No. 313
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (313-321)
Higher fibroblast growth factor-23 concentrations associate with left ventricular systolic dysfunction in dialysis patients
Shailendra Sharma1, Jacob Joseph2,3, Michel Chonchol1, James S. Kaufman2, Alfred K. Cheung4,5, Zahi Rafeq5, Gerard Smits1, Jessica Kendrick1,7, and the HOST Investigators
1Division of Renal Diseases and Hypertension, University of Colorado Denver, Aurora, CO, 2Department of Medicine, Veterans Affairs Boston Healthcare System and Boston University School of Medicine, 3Harvard Medical School, Boston, MA, 4Medical Service, Veterans Affairs Salt Lake City Healthcare System, 5Division of Nephrology & Hypertension, University of Utah, Salt Lake City, UT, 6Department of Medicine, University of Massachusetts Medical School, Worcester, MA, and 7Denver Health Medical Center, Denver, CO, USA
Aims: The concentration of fibroblast growth factor 23 (FGF-23) is elevated in patients on dialysis. FGF receptors have been implicated in the pathogenesis of left ventricular (LV) hypertrophy. The objective of this study was to examine the associations between high plasma FGF-23 concentration and LV systolic dysfunction. Methods: We tested the hypothesis that high plasma FGF-23 concentration is associated with LV dysfunction in 110 chronic dialysis patients from the Homocysteine study who had paired echocardiograms performed for clinical indications. C-terminal FGF-23 concentrations were measured in stored plasma samples. Multivariate regression analyses were performed to evaluate the association of FGF-23 concentration with LV dysfunction. Results: Participants had a mean age of 60 ± 11 years. Median FGF-23 level and mean ejection fraction (EF) at baseline were 4,632 (1,384 – 14,997) RU/ml and 50 ± 13%, respectively. Median follow-up time was 1.9 years. Higher FGF-23 concentration was directly associated with decreases in EF during follow-up. After adjustment for demographics, baseline EF, hypertension, diabetes, cardiovascular disease, body mass index, systolic blood pressure, hemoglobin and markers of mineral metabolism, participants with FGF-23 in the highest tertile had an 8% decrease in EF compared to participants in the lowest tertile (β –8.0, 95% CI –15.5 to –0.53; p = 0.04). When FGF-23 was evaluated as a continuous variable, for every log10 increase in FGF-23, EF decreased during follow-up by 6.5% (β –6.5, 95% CI –11.3 to –1.73; p = 0.01). Conclusion: In conclusion, higher FGF-23 concentration is independently associated with LV systolic dysfunction in chronic dialysis patients.Correspondence to:
Jessica Kendrick MD
Assistant Professor Division of Renal Diseases and Hypertension
University of Colorado Denver
Denver Health Medical Center
660 Bannock St, Mail Code 4000, Denver, CO 80204, USA
Email: [email protected]
Original
Serum osteoprotegerin level is associated with degree of arteriovenous fistula stenosis in patients with hemodialysis
Hye-Ryoun Kim, Hyang-Kyung Kim and Dong-Jin Oh
Page No. 322
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (322-327)
Serum osteoprotegerin level is associated with degree of arteriovenous fistula stenosis in patients with hemodialysis
Hye-Ryoun Kim1, Hyang-Kyung Kim2 and Dong-Jin Oh3
1Department of Laboratory Medicine, 2Department of Surgery, College of Medicine Chung-Ang University, Seoul, and 3Department of Internal Medicine, Myongji Hospital, Goyang, Korea
Aims: Vascular access dysfunction caused by stenosis is a major complication for hemodialysis (HD) patients. However, the relevant humoral mediators of arteriovenous (AV) access stenosis are still under investigation. The aim of the present study was to evaluate the association between serum fetuin-A, osteoprotegerin (OPG), and heat shock protein (Hsp) 70 concentration with arteriovenous fistula (AVF) patency in HD patients. Methods: Serum fetuin-A, OPG and Hsp70 concentrations were measured in 64 HD patients. Using these data, we studied the association of fetuin-A, OPG and Hsp70 with the degree of AVF stenosis using ultrasonography in HD patients. Results: The degree of AVF stenosis was positively correlated with OPG levels (r = 0.411, p = 0.001) and OPG/fetuin-A ratio (r = 0.375, p = 0.002). The levels of OPG and OPG/fetuin-A ratio were statistically higher in the group of AVF stenosis ≥ 11.5%. Stepwise linear regression analyses independently predicted the degree of AVF stenosis on the logarithmic function of the OPG (β = 8.775, standard error = 0.493, p = 0.034) and OPG/fetuin-A ratio (β = 10.325, standard error = 3.247, p = 0.018). Conclusions: Our data demonstrated that OPG and the OPG/fetuin-A ratio correlated with the degree of AVF stenosis in HD patients. In addition, OPG and the OPG/fetuin-A ratio were the most important predictors of the degree of AVF stenosis in HD patients.Correspondence to:
Dong-Jin Oh, MD
Department of Internal Medicine
Myongji Hospital
55 Hwasu-ro 14beon-gil, Deokyang-gu
Goyang-si, Gyeonggi-do 412-826, Korea
Email: [email protected]
Original
Pain resulting from arteriovenous fistulae: prevalence and impact
Emma Aitken, Angus McLellan, Julie Glen, Mick Serpell, Robert Mactier and Marc Clancy
Page No. 328
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (328-333)
Pain resulting from arteriovenous fistulae: prevalence and impact
Emma Aitken1, Angus McLellan2, Julie Glen1, Mick Serpell3, Robert Mactier4 and Marc Clancy1
1Department of Renal Surgery, Western Infirmary, 2Medical School, University of Glasgow, 3Department of Anaesthetics and Pain Medicine, Gartnavel General Hospital, and 4Department of Nephrology, Western Infirmary, Glasgow, UK
Introduction: The burden of pain from cannulation of arteriovenous fistulae (AVF) and the impact it has on quality of life is poorly described in the literature. Methodology: A pain score questionnaire was employed for all patients in the West of Scotland dialyzing via AVF (n = 461). Pain was assessed using visual analogue score (VAS) and McGill pain score. Patients with severe pain (VAS > 5) were compared to those with minimal pain. Results: The questionnaire as completed by 97.5% of the patients. Median VAS on cannulation was 3 (IQR 0.5 – 4.5). Of those who had completed the questionnaire, 24.4% had severe pain on cannulation and 3.2% experienced severe chronic pain. 53 patients (11.3%) cut a dialysis session short due to pain. Of the patients with severe chronic pain, 46.7% had a physical complication affecting their AVF (e.g., venous stenosis, pseudoaneurysm). Following treatment of the problem, pain improved in 71.4% and resolved completely in 14.3%. Brachiobasilic AVF was associated with a higher incidence of severe pain than either brachiocephalic or radiocephalic AVF (50%, 23.3% and 24.4% respectively; p = 0.03). There was a trend towards more severe pain with rope-ladder cannulation (27.7%) compared to button-hole cannulation (18.2%); however, this difference did not reach statistical significance (p = 0.09). Conclusions: Pain from AVF is poorly recognized and an under-reported problem. While severe pain resulting in the avoidance of dialysis is rare, it can lead to significant difficulties and ultimate abandonment of AVF. Pain is often suggestive of an underlying anatomical problem.Correspondence to:
Emma Aitken, MBChB, MRCS
Clinical Research Fellow
Department of Renal Surgery, Western Infirmary
Glasgow, G11 6DT, UK
Email: [email protected]
Original
Reduced hospitalizations in severe, refractory congestive heart failure with peritoneal dialysis: A consecutive case series
Jacques Rizkallah, Manish M. Sood, Martina Reslerova, Francisco Cordova, Amrit Malik, Chris Sathianathan, Estrellita Estrella-Holder and Shelley Zieroth
Page No. 334
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (334-341)
Reduced hospitalizations in severe, refractory congestive heart failure with peritoneal dialysis: A consecutive case series
Jacques Rizkallah1, Manish M. Sood2, Martina Reslerova2, Francisco Cordova1, Amrit Malik1, Chris Sathianathan2, Estrellita Estrella-Holder1 and Shelley Zieroth1
1Section of Cardiology, and 2Section of Nephrology, Department of Medicine, St. Boniface Hospital and University of Manitoba, Winnipeg, Manitoba, Canada
Background: Peritoneal dialysis (PD) for long-term management of diuretic resistant volume overload in heart failure (HF) may provide potential benefit with few adverse consequences. We examined the impact of PD on clinical status hospitalizations, and complications of therapy in severe end-stage HF. Methods: A consecutive case series of 10 transplant ineligible patients receiving PD solely for HF volume management between 2007 and 2011 was evaluated with clinical data reviewed pre- and post-PD initiation. Results: The mean ejection fraction (EF) pre-PD was 24.5 ± 6.0% with the majority of patients having NYHA class IIIB symptoms and moderate-severe right ventricular dysfunction. 9/10 patients were Stage 3 chronic kidney disease (CKD) or worse. After PD initiation, average weight loss was almost 7 kg (p = 0.016) with improvement in diuretic response, peripheral edema, and functional class. There was a significant decrease in re-hospitalization from an average of 3.2 ± 2.5 to 0.1 ± 0.3 admissions per patient (p = 0.007) and reduced average length of stay from 37 ± 36.7 to 0.78 ± 2.3 days (p = 0.019). Summary: Objective criteriabased institution of PD for the treatment of diuretic refractory severe-end-stage HF was well tolerated and demonstrated favorable outcomes; these included improved clinical status, reduced hospitalizations and length of stay, with very few and easily treatable PDrelated complications. PD appears to be a viable option in refractory, end-stage congestive heart failure (CHF).Correspondence to:
Dr. Shelley Zieroth
Y3016-409 Tache Avenue
Winnipeg, Manitoba R2H2A6, Canada
Email: [email protected]
Original
The influence of socioeconomic factors on depression in maintenance hemodialysis patients and their caregivers
Siren Sezer, Mehtap Erkmen Uyar, Zeynep Bal, Emre Tutal and F. Nurhan Ozdemir Acar
Page No. 342
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (342-348)
The influence of socioeconomic factors on depression in maintenance hemodialysis patients and their caregivers
Siren Sezer, Mehtap Erkmen Uyar, Zeynep Bal, Emre Tutal and F. Nurhan Ozdemir Acar
Baskent University Medical School, Department of Nephrology, Ankara, Turkey
Aim:A Depression has a strong impact on the quality of life of patients receiving maintenance hemodialysis (MHD). The objective of this study was to analyze the factors influencing the depression scores of MHD patients and their primary caregivers. Methods: 141 patients (54 female, aged 53.6 ± 14.2 years) who had been on MHD for at least 3 months were included. Age, gender, etiology of renal disease, duration of dialysis, marital and employment status, and income and living status of patients were recorded from patient charts and through face-to-face interviews. Disability was assessed by a selfadministered questionnaire. Additionally, 40 of the 141 patients (28%) primary caregivers were interviewed face-to-face. All patients and 40 primary caregivers were evaluated for the presence of depressive symptoms by Beck Depression Inventory (BDI). Results: Moderate to severe depressive symptoms were found in 36.2% of the study group. Divorced patients were significantly more depressed (p < 0.02) than married or single patients. Patients living with and being cared for by their nuclear family (father, mother, and/or children) were less depressed than patients being cared for by other relatives (p < 0.009). Monthly income was negatively correlated with both patients’ and primary caregivers’ BDI score (p < 0.005). Patients with high disability scores were more depressed than nondisabled patients (p < 0.08). Primary caregivers’ BDI scores were positively correlated with the patients’ BDI scores (p < 0.0001). Binary logistic regression analysis revealed that higher depression scores were significantly associated with more than 3 children under guardianship (p < 0.01) and higher disability scores (p < 0.023). Conclusion: In this present study, we observed that economically, socially, and physically self-sufficient MHD patients had lower depression rates. The treatment of depression and social and psychological support for both the patient and the family members are essential for better medical status among MHD patients.Correspondence to:
Mehtap Erkmen Uyar, MD
Baskent University Medical School
Department of Nephrology
Kivrimli Cad. No: 55/14, 06010 Etlik/ Ankara, Turkey
Email: [email protected]
Original
Urinary KIM-1, IL-18 and Cys-c as early predictive biomarkers in gadolinium-based contrast-induced nephropathy in the elderly patients
Shao-Bin Duan, Gai-Ling Liu, Zhen-Qiu Yu and Peng Pan
Page No. 349
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (349-354)
Urinary KIM-1, IL-18 and Cys-c as early predictive biomarkers in gadolinium-based contrast-induced nephropathy in the elderly patients
Shao-Bin Duan, Gai-Ling Liu, Zhen-Qiu Yu and Peng Pan
Institute of Nephrology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, P.R.China
Background/Aims: This study was designed to investigate whether urinary kidney injury molecule-1 (KIM-1), interleukin-18 (IL-18) and cystatin C (Cys-C) are early predictive biomarkers for gadolinium-based contrast-induced nephropathy (Gd-CIN) in the elderly patients undergoing gadolinium-enhanced magnetic resonance imaging (MRI). Methods: 60 elderly patients undergoing enhanced MRI using gadolinium-based contrast media were enrolled. Urine samples were collected before and 24 hours and 48 hours after the procedure, and KIM-1, IL-18 and Cys-C levels were measured by using an ELLSA kit respectively. Serum samples before and 24 hours and 48 hours after the procedure were also collected, and creatinine was measured by automatic biochemical analyzer. Results: Gd-CIN was diagnosed in 8 of 60 (13.3%) patients. At 24 hours after MRI with gadolinium administration in the Gd-CIN group, the urinary KIM-1, IL-18 and Cys-C were significantly increased. Logistic regression analysis showed that urinary KIM-1 and IL-18 at 24 hours after gadolinium injection were independent predictive markers of Gd-CIN. The predictable time of acute kidney injury (AKI) onset determined by urinary KIM-1, IL-18 and Cys-C was 24 hours earlier than by serum creatinine. Conclusions: Urinary KIM-1, IL-18 and Cys-C could be early predictive biomarkers of Gd-CIN in the elderly patients, which showed a good performance in early diagnosis of Gd-CIN as compared with serum creatinine.Correspondence to:
Shao-Bin Duan
Institute of Nephrology
The Second Xiangya Hospital
Central South University
Changsha 410011, Hunan, P.R. China
Email: [email protected]
Original
Does enteral protein intake affect renal glomerular and tubular functions in very low birth weight infants?
Hayriye Gozde Kanmaz, Banu Mutlu, Omer Erdeve, Fuat Emre Canpolat, Serife Suna Oguz, Nurdan Uras and Ugur Dilmen
Page No. 355
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (355-360)
Does enteral protein intake affect renal glomerular and tubular functions in very low birth weight infants?
Hayriye Gozde Kanmaz1, Banu Mutlu2, Omer Erdeve1, Fuat Emre Canpolat1, Serife Suna Oguz1, Nurdan Uras1 and Ugur Dilmen1,2
1Zekai Tahir Burak Maternity Teaching Hospital, Department of Neonatology and 2Yildirim Beyazit University, Department of Pediatrics, Ankara, Turkey
Objectives and aim: Very low birth weight infants require 3 – 4 g/kg/day protein intake to provide satisfactory postnata growth rates and neurodevelopmental outcomes. However, they have fewer functional nephrons, thereby increasing vulnerability for impaired renal functions. The aim of this study was to investigate the effect of different amounts of enteral protein intake during the fortification of human milk on renal glomerular and tubular functions. Material and methods: Preterm infants were randomized into three groups in terms of their daily protein intake: standard fortification (3 g/kg/d), moderate fortification (3.3 g/kg/d), and aggressive fortification (3.6 g/kg/d). Serum urea, creatinine (Cr), Cystatin C (Cys-C) and urinary β2 microglobulin (β2M) levels were assessed and compared between groups. Results: Serum urea, Cr, Cys-C and urinary β2M levels were similar in all three groups, both on discharge and postnatal Day 14 (p > 0.05). Mean Cr and β2M levels were significantly lower on discharge (p < 0.05), while Cys-C levels did not differ in time (p > 0.05). Conclusion: Enteral protein intake up to 3.6 g/kg/d did not alter the tubular and glomerular functions in very preterm infants. However, the long-term renal effects in these infants maintained on a high protein intake remain unknown and should be addressed in future studies.Correspondence to:
H. Gozde Kanmaz, MD
Zekai Tahir Burak Maternity Teaching Hospital
Department of Neonatology, Talatpasa Bulvari
Samanpazari 06230, Turkey
Email: [email protected]
Original
Association of Anthropometric indexes with chronic kidney disease in a Chinese population
Shanying Chen, Bide Wu, Xinyu Liu, Youming Chen, Yongqiang Li, Mi Li, Yan Liang1, Xiaofei Shao, Harry Holthöfer and Hequn Zou
Page No. 361
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (361-369)
Association of Anthropometric indexes with chronic kidney disease in a Chinese population
Shanying Chen1,2*, Bide Wu2*, Xinyu Liu1, Youming Chen3, Yongqiang Li1, Mi Li4, Yan Liang1, Xiaofei Shao1, Harry Holthöfer5 and Hequn Zou1
1Department of Nephrology, the Third Affiliated Hospital of Southern Medical University, Guangzhou, 2Department of Nephrology, Zhangzhou Municipal Affiliated Hospital of Fujian Medical University, Zhangzhou, 3Clinical laboratory, the Third Affiliated Hospital of Southern, Medical University, Guangzhou, 4Blood purification center, No. 5 Affiliated Hospital of Sun Yat-sen University, Zhuhai, China, and 5National Centre for Sensor Research/BioAnalytical Sciences, Dublin City University, Ireland
Objective: Obesity is associated with an increased risk of chronic kidney disease (CKD), but the best anthropometric obesity measure remains controversial. This study aimed to examine the associations of anthropometric indexes with CKD risk and which anthropometric index is a better predictor of CKD. Methods: Data was drawn from a cross-sectional study in China. We used four anthropometric indexes: body mass index (BMI), waist circumference (WC), waist-tohip ratio (WHR), and waist-to-height ratio (WHtR). CKD was defined as estimated glomerular filtration rate (eGFR) < 60 ml/ min/1.73 m2 or urinary albumin to creatinine ratio (ACR) ≥ 30 mg/g. Logistic regressions were used for the analyses. Results: 1,834 participants were included in the analyses. After adjusting for potential confounders, BMI, WC and WHtR were significantly associated with CKD in men and women. The respective odd ratios for BMI (every SD increment), WC (every SD increment), and WHtR (every SD increment) were 1.46, 1.40, and 1.45 in men as well as 1.21, 1.31, and 1.38 in women. After adjusting for potential confounders, WHR was associated with CKD in women but not men. In women, the associations of WC, WHR and WHtR with CKD was independent of other MetS components. No difference in WHtR was observed between men and women. Conclusion: Anthropometric indexes are associated with CKD. The associations of anthropometric indexes with CKD are independent of other MetS components in women but not men. In women, central obesity indexes are better than BMI for predicting of CKD.
*Both authors contributed equally.Correspondence to:
Hequn Zou, MD
Department of Nephrology
The Third Affiliated Hospital of
University of Southern Medical University
183# Zhongshan Dadao, Tianhe district
Guangzhou, 510000 P. R. China
Email: [email protected]
Nephrology Education
Vascular access malfunction: towards a more genecentric view
Dimitrios Kirmizis, Dimitrios Chatzidimitriou, Fani Chatzopoulou, Georgios Tzimagiorgis, Nikolaos Malisiovas and Aikaterini Papagianni
Page No. 370
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (370-376)
Vascular access malfunction: towards a more genecentric view
Dimitrios Kirmizis1,2, Dimitrios Chatzidimitriou3, Fani Chatzopoulou3, Georgios Tzimagiorgis4, Nikolaos Malisiovas3 and Aikaterini Papagianni5
1Medical School, Aristotle University, Thessaloniki, 2Alpha-Nephrodynamic Dialysis Unit, Serres, 3Laboratory of Microbiology, 4Laboratory of Biological Chemistry, Medical School, and 5Department of Nephrology, Hippokration General Hospital, Aristotle University, Thessaloniki, Greece
A well-functioning vascular access is the cornerstone for an optimal hemodialysis treatment and an issue of major importance for the outcome of patients on chronic hemodialysis. Over the last few years reports supporting the aspect that mechanisms involved in vascular access malfunction are genetically controlled have been published. Triggered by two cases reported herein, we present a comprehensive review of the literature on an evolving field of particular significance to patients on hemodialysis.Correspondence to:
Dr. Dimitrios Kirmizis, MD, MSc, PhD
Medical School, Aristotle University
Perikleous 10, 55134, Thessaloniki, Greece
Email: [email protected]
Nephrology Education
A novel CLCN5 mutation in a boy with asymptomatic proteinuria and focal global glomerulosclerosis
Mary Rose Valina, Christopher P. Larsen, Sherry Kanosky, Sharon F. Suchy, Linda S. Nield and Ali Mirza Onder
Page No. 377
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (377-384)
A novel CLCN5 mutation in a boy with asymptomatic proteinuria and focal global glomerulosclerosis
Mary Rose Valina1, Christopher P. Larsen2, Sherry Kanosky3, Sharon F. Suchy4, Linda S. Nield1 and Ali Mirza Onder3
1Department of Pediatrics, West Virginia University, School of Medicine, Morgantown, WV, 2Nephropathology Associates, Little Rock, AR, 3Department of Pediatrics, Division of Pediatric Nephrology, West Virginia University, School of Medicine, Morgantown, WV and 4GeneDx, Gaithersburg, MD, USA
School of Medicine, Morgantown, WV and GeneDx, Gaithersburg, MD, USA
Dent disease is an X-linked proximal tubulopathy that typically presents with hypercalciuria, low-molecular-weight proteinuria and slow progression to endstage renal disease. We report the case of a 5-year-old boy who presented with asymptomatic nephrotic range proteinuria and was later diagnosed with Dent disease. Absence of specific glomerular pathology in the first kidney biopsy led to erroneous treatment for presumably unsampled primary focal segmental glomerulosclerosis. Aggressive angiotensin blockade and immunosuppression resulted in significant side effects with marginal benefit. The continued nonspecific findings after a second kidney biopsy 2 years later led to the suspicion of a congenital tubulopathy. We detected a novel CLCN5 gene mutation, c.1396G > C, that creates a G466R missense change in the ClC-5 protein. Dent disease should be considered in the differential diagnosis of asymptomatic proteinuria for male patients. Profiling proteinuria in these patients by spot urine albumin/creatinine ratio may give the first clue to a tubulopathy. Determining the extent to which the clinical work-up should proceed for females with Dent phenotype or asymptomatic proteinuria remains to be a challenging clinical dilemma.Correspondence to:
Ali Mirza Onder, MD
West Virginia University/Health Sciences Center
Division of Pediatric Nephrology
P.O. Box 9214, Morgantown, WV 26506-9214, USA
Email: [email protected]
Nephrology Education
Treating desmopressin-induced hyponatremia: a case using hypertonic saline
Shayan Shirazian, Nicole Ali and Steven Fishbane
Page No. 385
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (385-387)
Treating desmopressin-induced hyponatremia: a case using hypertonic saline
Shayan Shirazian, Nicole Ali and Steven Fishbane
Winthrop University Hospital, Division of Nephrology and Hypertension, Mineola, NY, USA
Desmopressin acetate (DDAVP), a medication used in the treatment of bleeding and polyuric disorders, has the potential to cause hyponatremia when free water is not appropriately restricted with its use. This free water retention is reversible when DDAVP is discontinued. We report a case of symptomatic DDAVP-induced hyponatremia in which discontinuation of DDAVP led to a rapid increase of serum sodium. In order to prevent rapid free water excretion, DDAVP and hypertonic saline were used concurrently. With close monitoring, this can be an effective treatment strategy in patients with DDAVP-induced hyponatremia.Correspondence to:
Shayan Shirazian MD
200 Old Country Road Suite 135
Mineola, NY 11501, USA
Email: [email protected]
Nephrology Education
Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis
Rafael T. Krmar, Mathias Kågebrand, Magnus E. A. Hansson, Stella Edström Halling, Kajsa Åsling-Monemi, Maria Herthelius, Ulla Holtbäck, Marta Christensson, Annika Wernerson and Annette Bruchfeld
Page No. 388
Abstract
Clinical Nephrology, Vol. 80 – No. 5/2013 (388-394)
Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis
Rafael T. Krmar1, Mathias Kågebrand1, Magnus E.A. Hansson1, Stella Edström Halling1, Kajsa Åsling-Monemi1, Maria Herthelius1, Ulla Holtbäck1, Marta Christensson2, Annika Wernerson3 and Annette Bruchfeld4
1Department for Clinical Science, Intervention and Technology, Division of Pediatrics, 2Division of Clinical Immunology, Department of Laboratory Medicine, 3Department of Pathology and 4Department for Clinical Science, Intervention and Technology, Division of Renal Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
Pauci-immune renal limited vasculitis (RLV) is a rare and aggressive autoimmune disease. We retrospectively analyzed the renal outcome of 6 children with biopsy proven RLV. Median age at diagnosis was 10.6 years (range 7.1 – 14.5) and the median follow-up was 4.4 years (range 2.3 – 6.6). At diagnosis, 5 patients were given induction therapy (methylprednisolone + cyclophosphamide pulses) followed by maintenance treatment (prednisolone + azathioprine) while 1 patient received maintenance treatment only. After induction, 4 patients either retained or recovered normal renal function, and 1 patient, in whom short-term plasma exchange was prescribed to try to rescue her renal function, became free from dialysis. Repeated biopsy showed no disease activity; however, renal scarring was evident in all renal specimens. At last follow-up, 2 patients had normal renal function, 3 patients had mild renal insufficiency, and 1 patient had advanced renal failure. In addition, 5 patients were treated for hypertension. Our case series suggests that an initial favorable response to immunosuppressive therapy might not necessarily prevent the occurrence of renal scarring and highlights the importance of regular follow-up.Correspondence to:
Rafael T. Krmar, MD, PhD
Karolinska Institutet, Department for Clinical Science
Intervention and Technology, Division of Pediatrics
Karolinska University Hospital
Huddinge, S-141 86 Stockholm, Sweden
Email: [email protected]
