Volume 76 (2011), No. 3/2011(September)
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Original
Cytokines in acute kidney injury (AKI)
D.W. Lee, S. Faubel and C.L. Edelstein
Page No. 165
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (165-173)
Cytokines in acute kidney injury (AKI)
D.W. Lee, S. Faubel and C.L. Edelstein
Division of Renal Diseases and Hypertension, University of Colorado at Denver, Aurora, CO, USA
In acute kidney injury (AKI), many cytokines are released by leukocytes and renal tubular cells in the injured kidney and are important components of both the initiation and extension of inflammation. Cytokines are 1) produced by the kidney and mediate AKI, 2) produced by the kidney, released into the blood or urine and serve as biomarkers of AKI, and 3) produced by the kidney or other organs in AKI and mediate or protect against distant organ injury. Further understanding of the role of cytokines in AKI may result in therapeutic approaches like cytokine inhibition that may reduce the degree of kidney injury itself, as well as deleterious effects of kidney injury on other organs.Correspondence to:
C.L. Edelstein
Division of Renal
Diseases and Hypertension
Univ. of Colorado at Denver, Box C281
12700 East, 19 th Ave
Aurora, CO 80262, USA
Email: [email protected]
Original
Serum cystatin c levels in normal pregnancy
R. Obrenovic, D. Petrovic, N. Majkic-Singh, J. Trbojevic-Stankovic and B. Stojimirovic
Page No. 174
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (174-179)
Serum cystatin c levels in normal pregnancy
R. Obrenovic1, D. Petrovic2, N. Majkic-Singh1, J. Trbojevic-Stankovic3 and B. Stojimirovic4
1Institute for Medical Biochemistry, Clinical Center of Serbia, Belgrade, 2Clinic of Urology and Nephrology, Clinical Center Kragujevac, Kragujevac, 3Clinic of Urology, Clinical-Hospital Center Dr Dragisa Misovic, and 4Institute of Urology and Nephrology, Clinical Center of Serbia, Belgrade, Serbia
Aim: The aim of this study was to determine the levels of cystatin C, creatinine and creatinine clearance in different trimesters of uncomplicated pregnancy in women with normal kidney function. Subjects and methods: A total of 109 pregnant women were included: group 1 - 38 women (average age 29.63 ± 4.3 y) in the first trimester, Group 2 – 32 women (average age 33.56 ± 5.95 y) in the second trimester and Group 3 – 39 pregnant women (average age 30.1 ± 6.95 y) in the third trimester. Serum cystatin C was determined by the PENIA method (Particle-Enhanced Nephelometric Immuno-Assay), using Behring tests (Behring Diagnostics GmbH, Marburg, Germany). Results were statistically analyzed using the ANOVA. Results: A statistically significant increase in serum cystatin C level was found in the third trimester of pregnancy (0.69 ± 0.16 mg/l vs. 0.78 ± 0.26 mg/l vs. 1.21 ± 0.30 mg/l). Conclusion: It appears that cystatin C is not a reliable marker of kidney function in pregnancy and that its increase is connected with a combination of several factors, including endotheliasis, hormonal influence and glomerular filtration rate (GFR) alterations.Correspondence to:
J. Trbojevic-Stankovic, MD, PhD
Svetozara Markovica 52A
11000 Belgrade, Serbia
Email: [email protected]
Original
Kidney biopsy in patients with diabetes mellitus
D.G. Haider, S. Peric, A. Friedl, V. Fuhrmann, M. Wolzt, W.H. Hörl and A. Soleiman
Page No. 180
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (180-185)
Kidney biopsy in patients with diabetes mellitus
D.G. Haider1, S. Peric1, A. Friedl1, V. Fuhrmann2, M. Wolzt3, W.H. Hörl1 and A. Soleiman4
1Division of Nephrology and Dialysis, 2Division of Hepatology and Gastroenterology, Department of Medicine III, 3Department of Clinical Pharmacology, and 4Department of Clinical Pathology, Medical University of Vienna, Austria
Background: The clinical consequences of the results obtained by kidney biopsy in patients with diabetes mellitus Type 1 or Type 2 have been controversial. Our study was conducted to assess clinical symptoms and histological diagnoses in patients with diabetes mellitus Type 1 and Type 2 undergoing kidney biopsy. Design, setting and patients: Observational study. The study included data from 567 consecutive renal biopsies of patients with diabetes mellitus Type 1 or 2 and chronic kidney disease (CKD) examined by standard histopathological procedures. The main outcome measures were incidence of diabetic nephropathy (DN) and glomerulonephritis (GN), predictors for the presence of both DN or GN. Results: Approximately 70% of patients with diabetes mellitus Type 1 or 2 and evidence for CKD had DN. Glomerular diseases present in approximately 30% of patients with diabetes were predominantly immune complex GN and secondary focal glomerulosclerosis, followed by IgA-GN, which was associated with microhematuria (p = 0.01) and hypertension (p = 0.04). Only a minority had membranous GN, which was associated with nephrotic syndrome (p = 0.004). Progressive CKD predicted the presence of GN in diabetes mellitus Type 2 (r = –0.98; p = 0.02). Conclusion: GN is not uncommon in patients with diabetes and evidence for CKD. Kidney biopsy should therefore be considered in patients with diabetes and progressive CKD.Correspondence to:
D.G. Haider, MD
Division of Nephrology and Dialysis
Department of Medicine III
Medical University of Vienna
Währinger Gürtel 18 – 20
1090 Vienna, Austria
Email: [email protected]
Original
Long-term prognosis for Chinese adult patients with acute postinfectious glomerulonephritis
C. Luo, Z. Tang, D. Chen and Z. Liu
Page No. 186
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (186-194)
Long-term prognosis for Chinese adult patients with acute postinfectious glomerulonephritis
C. Luo, Z. Tang, D. Chen and Z. Liu
Research Institute of Nephrology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China
Aims: The long-term prognosis for adults with acute postinfectious glomerulonephritis (APIGN) is under debate. This study was designed to investigate the long-term renal outcome of adult APIGN. Materials and methods: A total of 64 adults with APIGN were identified and divided into two groups: acute poststreptococcal glomerulonephritis (APSGN) group and acute nonstreptococcal postinfectious glomerulonephritis (nonstreptococcal APIGN) group. For the purpose of outcome analysis, 49 patients with a follow-up of ≥ 12 months were included. Univariate and multivariate analyses were performed for prognostic factors. Results: Nephrotic syndrome was more frequently seen in nonstreptococcal APIGN patients. At the time of biopsy, the mean proteinuria of the two groups was 1.82 ± 2.14 and 4.21 ± 4.39 g/d, respectively (p = 0.005). There was no significant difference between the two groups on microscopy. Among the 49 patients with ≥ 12 months of follow-up, the complete remission rate was 85.7%, and the incidence of chronic renal failure and endstage renal disease (ESRD) was 6.1% and 4.1%, respectively. The complete remission rate of the APSGN group was higher than that of the nonstreptococcal APIGN group. Univariate analysis showed that the prognostic factors were age, underlying disease, proteinuria, tubular damage and C1q deposits. In multivariate analysis, underlying disease was the only significant and independent inverse correlate of complete remission. Conclusions: The long-term prognosis for young Chinese adults with APIGN is optimistic if there is no underlying disease. The renal outcome for patients with APSGN is better than for patients with nonstreptococcal APIGN.Correspondence to:
Prof. Zheng Tang
Research Institute of Nephrology
Jinling Hospital
Nanjing University School of Medicine
305 East Zhongshan Road
Nanjing 210002, China
Email: [email protected]
Original
Prevalence of abnormal in vitro closure time using the Platelet Function Analyzer-100 in chronic kidney disease patients and analysis of associated factors
H.J. Kim, K.M. Kim, T.Y. Koo, H.S. Chi, S.H. Kim and S.B. Kim
Page No. 195
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (195-200)
Prevalence of abnormal in vitro closure time using the Platelet Function Analyzer-100 in chronic kidney disease patients and analysis of associated factors
H.J. Kim1, K.M. Kim1, T.Y. Koo1, H.S. Chi2, S.H. Kim3 and S.B. Kim1
1Division of Nephrology, 2Department of Laboratory Medicine, Asan Medical Center, University of Ulsan, Seoul, and 3Division of Nephrology, Gang Neung Asan Hospital, Gang Neung, Korea
Aim: Platelet Function Analyzer- 100 evaluates platelet function by determining time to occlusion of an aperture in a membrane coated with collagen and epinephrine (CEPI) or collagen and ADP (CADP) during the flow of citrated whole blood. We sought to determine prevalence of abnormal in vitro closure time (CT) in chronic kidney disease (CKD) patients and to analyze associated factors. Materials and methods: CEPI-CT (normal, 82 – 182 sec in Korean), CADP-CT (normal, 62 – 109 sec), CBC, serum creatinine (Cr) and blood urea nitrogen (BUN) were measured in CKD patients, 30 with Stage I, 36 with Stage II, 30 with Stage III, 56 with Stage IV, 283 with Stage V (79 with pre-dialysis Stage V, 130 on chronic hemodialysis (CHD), and 74 on chronic peritoneal dialysis (CPD)). Estimated glomerular filtration rate (eGFR) was calculated with a MDRD equation. Results: Abnormal CEPI-CT and CADP-CT occurred in < 15% of Stage I – III, 20% of Stage IV, and 41% and 54%, respectively, of Stage V patients. There were no differences in prevalence of abnormal CEPI-CT and CADP-CT among predialysis Stage V, CHD and CPD patients. CEPI-CT and CADP-CT were correlated with BUN, Cr and platelet counts in predialysis patients, and with platelet counts in dialysis patients, and CEPI-CT was correlated with BUN, Cr in CPD patients. Neither, however, was correlated with age, gender, hemoglobin or hematocrit. Conclusion: Prevalence of abnormal in vitro CT increases as stage worsens in CKD patients. In vitro CT is correlated with BUN, Cr and platelet counts in predialysis and total CKD patients.Correspondence to:
S.B. Kim, MD
Department of Internal Medicine
Asan Medical Center
388-1 Pungnap-2 dong
Songpa-gu, Seoul, 138-736, Korea
Email: [email protected]
Original
Clinical signifi cance of FGF-23 measurement in dialysis patients
F. Jongbloed, A. Galassi, M. Cozzolino, R. Zietse, G. Chiarelli, D. Cusi, D. Brancaccio and M. Gallieni
Page No. 201
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (201-209)
Clinical signifi cance of FGF-23 measurement in dialysis patients
F. Jongbloed1,2, A. Galassi3, M. Cozzolino1,4, R. Zietse2, G. Chiarelli1, D. Cusi1,4, D. Brancaccio4 and M. Gallieni5
1Renal Division, S. Paolo Hospital, Milan, Italy, 2Department of Nephrology, Erasmus Medical Center, Rotterdam, The Netherlands, 3Renal Division, Desio – Vimercate Hospital, Desio, and 4DMCO Polo San Paolo, University of Milano School of Medicine, and 5Nephrology and Dialysis Unit, San Carlo Borromeo Hospital, Milan, Italy
Aims: Considering the growing relevance of fibroblast growth factor-23 (FGF-23) in the pathogenesis of chronic kidney disease bone and mineral disorder (CKD-MBD), an analysis was performed to determine the relative importance of C-terminal (cFGF-23) and intact (iFGF-23) assays in assessing CKD-MBD status in the first place and the relationship between FGF-23 and mortality as a secondary aim. Methods: In 77 patients (15 peritoneal dialysis and 62 hemodialysis), levels of calcium, phosphate, parathyroid hormone (PTH), 25-hydroxyvitamin- D (25D), 1,25D, FGF-23 (C-terminal and intact molecule) were measured and their correlations were analyzed. The relationship between FGF-23 levels and patient survival was also analyzed. Results: A significant correlation was found between cFGF-23 and 1,25D, PTH and 25D while iFGF-23 was significantly correlated with phosphate, 25D and PTH. PTH and 1,25D were independent predictors of cFGF-23, while for iFGF-23 independent predictors were phosphate and 25D. No significant relationship was found between FGF-23 and mortality. Conclusions: C-terminal or intact FGF-23 levels are weakly correlated and thus not clearly indicative of FGF-23 effects on PTH, P and vitamin D metabolism in dialysis patients. Assays for cFGF-23 and iFGF-23 showed a good correlation, but the intact molecule was not superior in defining interactions with CKD-MBD molecules. Measuring FGF-23 on a regular basis with the current assays in CKD and dialysis patients does not yet seem clinically useful.Correspondence to:
F. Jongbloed
Medical Student
Hofdijk 377
3032 CG Rotterdam, The Netherlands
Email: [email protected]
Original
Hemoglobin level variability in hemodialysis patients treated with epoetin-β during 1 year
M. Kessler, P. Landais, P. Bataille, L. Yver, S. Koné, S. Kraemer, G. Brillet and E. Canivet
Page No. 210
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (210-217)
Hemoglobin level variability in hemodialysis patients treated with epoetin-β during 1 year
M. Kessler1, P. Landais2, P. Bataille3, L. Yver4, S. Koné5, S. Kraemer6, G. Brillet7 and E. Canivet8
1Division of Nephrology, University Hospital of Nancy, Nancy, 2DIM, Necker Hospital, Paris, 3Division of Nephrology, Duchenne Hospital, Boulogne/mer, 4Division of Nephrology, Angouleme Hospital, 5Roche, Neuilly sur Seine, 6Lincoln, Boulogne-Billancourt, 7Division of Nephrology, Chateauroux, Hospital, and 8ARPDD, Reims, France
Aim: This analysis was performed to assess the prevalence and the factors associated with hemoglobin (Hb) variability during treatment with erythropoiesis-stimulating agents (ESA) in France. Methods: Hb variability was evaluated in a subgroup of hemodialysis (HD) patients of the French cohort DiaNE. Eligible patients had received epoetin-β at least 6 months before entering DiaNE, 12 months during DiaNE and had no missing monthly Hb measurements. Up and down excursions (Hb variations > 1.5 g/dl with duration > 8 weeks) were assessed. Results: Of the 499 patients evaluated in this analysis, 295 (59%) had Hb levels inside the target range of 11 – 13 g/dl at baseline. The number of patients with constantly stable Hb level inside the target range decreased from baseline to 27.5% at 6 months and 10.8% at 12 months. More than 70% of patients experienced Hb variability. The number of excursions was 1.7 ± 0.8 per patient/year. The amplitude of up excursions was 2.8 ± 1.0 g/ dl with a duration of 14.7 ± 4.7 weeks. The amplitude of down excursions was 2.6 ± 0.9 g/dl with a duration of 14.5 ± 4.6 weeks. The main factors associated with Hb variability were number of epoetin-β dose changes, adverse events and iron therapy changes. Conclusion: Hb variability is frequent in French ESA-treated HD patients and closely related to practices. Further efforts are needed to improve anemia management.Correspondence to:
M. Kessler
Hôpitaux Brabois Adultes
CHU de Nancy
allée du Morvan
54511 Vandoeuvre-lès-Nancy, France
Email: [email protected]
Original
The effect of calcineurin inhibitors on endothelial and platelet function in renal transplant patients
G. Sahın, O.M. Akay, C. Bal, A.U. Yalcın and Z. Gulbas
Page No. 218
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (218-225)
The effect of calcineurin inhibitors on endothelial and platelet function in renal transplant patients
G. Sahın1, O.M. Akay2, C. Bal3, A.U. Yalcın1 and Z. Gulbas2
1Nephrology, 2Hematology, and 3Biostatistics Departments of Eskisehir Osmangazi University Medical School, Eskisehir, Turkey
Background/aim: Posttransplant cardiovascular mortality is still an important problem in renal transplant patients. In addition to conventional coronary risk factors, coagulation abnormalities play a key role in the hypercoagulable state observed in transplanted patients. Though renal transplantation eliminates cardiovascular disease risk factors by restoring renal function, it introduces new cardiovascular risks derived, in part from immunsupressive medications. We aimed to assess the effect of calcineurin inhibitors on endothelial function, platelet activation and aggregation in renal transplant patients. Methods: 62 renal transplant were studied. Staging was performed according to immunosuppression regimen. Group 1 (n = 37) were treated with cyclosporine/mycophenolate mofetil/methylprednisolone and Group 2 (n = 25) were treated with tacrolimus/mycophenolate mofetil/methylprednisolone. The control group consisted of 16 healthy subjects (Group 3). Hematological and biochemical tests, asymmetric dimethyl arginine (ADMA), sP-selectin levels and platelet aggregation tests were studied. Results: ADMA levels were higher in Group1 and statistically significant differences were observed compared with those of Group 2 and Group 3 (p < 0.05). Platelet aggregation values induced by all agonists (Adenosine diphosphate (ADP), epinephrine, ristocetin, collagen) were lower in Group 1 than Group 2 and Group 3, but the difference did not reach statistical significance (p > 0.05). There was a negative correlation between cyclosporine level and platelet aggregation values induced by ADP (r = –0.43, p < 0.01), ristocetin (r = –0.40, p < 0.05), epinephrine (r = –0.41, p < 0.05), and collagen (r = –0.43, p < 0.01). sP-selectin levels were appreciably higher in Group 1 and statistically significant differences were observed compared with those of Group 2 (p < 0.05) and Group 3 (p < 0.01). Conclusion: The results of our study suggest that CsA induces platelet activation without inducing platelet aggregation. Endothelial dysfunction due to vascular endothelial damage reflected by increases in ADMA values may increase the tendency for thrombotic events in patients who had undergone renal transplantation.Correspondence to:
G. Sahin, MD
Eskisehir Osmangazi University Medical School
Nephrology Department
26050, Eskisehir, Turkey
Email: [email protected]; [email protected]
Original
Comparative study of percutaneous nephrostomy using catheters with and without locking strings
M.-T. Chuang, C.-H. Lu, Y.-S. Tsai, H.-M. Tsai, T.-N. Kuo and Y.-S. Liu
Page No. 226
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (226-232)
Comparative study of percutaneous nephrostomy using catheters with and without locking strings
M.-T. Chuang, C.-H. Lu, Y.-S. Tsai, H.-M. Tsai, T.-N. Kuo and Y.-S. Liu
Department of Diagnostic Radiology, National Cheng-Kung University Hospital, Tainan, Taiwan
Background: Although it is generally felt that a catheter with a locking string can achieve better fixation and thus prevent catheter displacement, no formal study has ever substantiated this. Methods: We retrospectively reviewed the charts from 80 patients (mean age of 64.6 ± 14.76 y) who underwent percutaneous nephrostomy (PCN) over a 1-year period. Results: Most patients had catheters without locking strings and only 17 patients (21.3%) had catheters with locking strings. The median duration of catheter placement was 29 days (interquartile range 14 – 57 d). There were no significant differences in patients’ characteristics or catheter outcomes between catheters with and catheters without locking strings (p > 0.05). In addition, no significant difference in the catheter 90-day survival between catheter types was found (log rank test, p = 0.638). On univariate analysis, tumor as an indication for PCN (p = 0.018), obstruction (p = 0.021) and displacement (p = 0.007) were associated with reduced catheter survival. The multivariate analysis indicated that tumor as an indication for PCN (HR: 0.28, 95% CI: 0.13 – 0.63, p = 0.002), obstruction (HR: 0.25, 95% CI: 0.08 – 0.77, p = 0.015) and catheter displacement (HR: 0.09, 95% CI: 0.03 – 0.31, p < 0.001) were independent hazard factors for reduced catheter 90-day survival. Conclusion: No significant difference in either complication rate or 90-day survival was found between catheters with or without locking strings. These findings may prove helpful to the clinician in deciding the type of catheter to use during PCN.Correspondence to:
Y.-S. Liu
Department of Diagnostic Radiology
National Cheng-Kung University Hospital
No. 138 Sheng Li Road
Tainan, Taiwan 704, R.O.C.
Email: [email protected]
Original
Cinacalcet reduces plasma intact parathyroid hormone, serum phosphate and calcium levels in patients with secondary hyperparathyroidism irrespective of its severity
J.M. Frazão, P. Messa, G.J. Mellotte, H. Geiger, E.C. Hagen, L.D. Quarles, P.G. Kerr, A. Baños, B. Dehmel and P. Urena
Page No. 233
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011 (233-243)
Cinacalcet reduces plasma intact parathyroid hormone, serum phosphate and calcium levels in patients with secondary hyperparathyroidism irrespective of its severity
J.M. Frazão1, P. Messa2, G.J. Mellotte3, H. Geiger4, E.C. Hagen5, L.D. Quarles6, P.G. Kerr7, A. Baños8, B. Dehmel8 and P. Urena9
1Nephrology Research and Development Unit and School of Medicine, Porto University, Porto, Portugal, 2Nephrology Division – CROFF Policlinico di Milano, Italy, 3Adelaide and Meath Hospital, Dublin, Ireland, 4Johann-Wolfgang-Goethe-University, Frankfurt, Germany, 5Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands., 6Department of Internal Medicine, Division of Nephrology, University of Tennessee Health Science Center, Memphis, TN, USA, 7Monash Medical Centre and Monash University, Clayton, Victoria, Australia, 8Amgen Europe GmBH, Zug, Switzerland and 9Clinique du Landy, Saint Ouen, France
Aims: To evaluate the relationship between the severity of secondary hyperparathyroidism (SHPT) – defined in terms of baseline plasma intact parathyroid hormone (iPTH) level – and the magnitude of response to cinacalcet. Materials and methods: In this post hoc analysis, data were pooled from three randomized, placebo-controlled trials in which dialysis patients with iPTH ≥ 300 pg/ml were dose-titrated with cinacalcet or placebo in addition to conventional treatment to achieve iPTH ≤ 250 pg/ml. In 953 patients analyzed (cinacalcet, 545; placebo, 408), baseline iPTH levels were categorized in 100 pg/ml intervals (300 - ≥ 1,000 pg/ml), and the impact of baseline iPTH on changes in iPTH, phosphate (P), calcium (Ca) and calcium– phosphate product (Ca × P) was evaluated. Results: Cinacalcet reduced iPTH (47% reduction), P (9%), Ca (7%), and Ca × P (15%) across all subgroups. For patients receiving cinacalcet, the mean percentage reduction from baseline in iPTH varied from 35 to 55%, being consistently decreased across the severity subgroups. The mean absolute change in iPTH was more pronounced in patients with higher baseline iPTH levels, particularly in the ≥ 1,000 pg/ml subgroup vs. the other subgroups. However, as baseline iPTH levels increased, iPTH ≤ 250 pg/ml was achieved in fewer patients. A trend towards greater absolute change from baseline was observed for P in patients with more severe disease (iPTH ≥ 800 pg/ml) treated with cinacalcet compared with patients with less severe disease (iPTH 300 – < 800 pg/ml). Conclusions: Cinacalcet lowers plasma iPTH and serum P, Ca and Ca × P levels in dialysis patients with SHPT, regardless of disease severity. Patients with more severe disease experienced greater reductions in PTH and P, but fewer achieved iPTH ≤ 250 pg/ml by the efficacy assessment phase. Use of cinacalcet when baseline PTH is lower may result in more stable control of SHPT and help to control bone and mineral alterations.Correspondence to:
J.M. Frazão, MD, PhD
Nephrology Department
Hospital de S João and School of Medicine
University of Porto, Porto, Portugal
Email: [email protected]
Nephrology Education
WT1 mutations may be a cause of severe renal failure due to nephroblastomatosis in Wilms’ tumor patients
S. Santín, G. Fraga, P. Ruíz, N. Pardo, M. Torrent, T. Martí, J. Ballarín, E. Ars and R. Torra
Page No. 244
Abstract
Clin i cal Nephrology, Vol. 76 – No. 3/2011 (244-249)
WT1 mutations may be a cause of severe renal failure due to nephroblastomatosis in Wilms’ tumor patients
S. Santín1*, G. Fraga2*, P. Ruíz1, N. Pardo3, M. Torrent3, T. Martí4, J. Ballarín5, E. Ars1 and R. Torra5
1Molecular Biology Laboratory, Fundació Puigvert, Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, 2Pediatric Nephrology Department, Hospital de la Santa Creu, San Pau, 3Pediatric Oncology Department, Hospital de la Santa Creu i Sant Pau, 4Radiology Department, Fundació Puigvert, Universitat Autònoma de Barcelona, and 5Nephrology Department, Fundació Puigvert, Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain
*These authors contributed equally to this work.
Wilms’ tumor suppressor gene (WT1) encodes a transcription factor required for normal development of the genitourinary system. Germline WT1 mutations have been described in a wide spectrum of pathological conditions, including kidney diseases, genital abnormalities and Wilms’ tumor. Here we report a 4-year-old male patient who presented with bilateral cryptorchidism, Wilms’ tumor, nephroblastomatosis and renal failure without nephrotic proteinuria. Sequence analysis of the WT1 gene demonstrated a constitutional heterozygous nonsense mutation in exon 7, which leads to a truncation of the WT1 protein at the zinc-finger 1. In the DNA of the tumor, we observed the same mutation in homo/hemizygosity. Given the requirement of WT1 for normal development, the WT1 mutation is likely to be responsible for the nephroblastomatosis and, in consequence, for the severe renal failure observed in our patient. This finding extends the spectrum of kidney diseases related to WT1 mutations and points to the need to screen for this gene in children with genitourinary abnormalities and Wilms’ tumor because of the associated risk of nephroblastomatosis and renal failure in those carrying WT1 mutations.Correspondence to:
Dr. R. Torra
Hereditary Kidney Diseases
Department of Nephrology
Fundació Puigvert, Cartagena 340-350
Barcelona 08025, Spain
Email: [email protected]
Nephrology Education
A case of renal infarction associated with elevated factor VIII level
O. Asif Siddiqui, A. Al-Absi and A. Showkat
Page No. 250
Abstract
Clin i cal Nephrology, Vol. 76 – No. 3/2011 (250-255)
A case of renal infarction associated with elevated factor VIII level
O. Asif Siddiqui, A. Al-Absi and A. Showkat
Division of Nephrology, University of Tennessee Health Science Center, Memphis, TN, USA
Elevated factor VIII level has recently been shown to be associated with increased risk of thrombosis. We report here a case of renal infarction in association with elevated factor VIII level. The patient presented with a three-day history of flank pain. Laboratory studies on presentation showed an elevated serum creatinine concentration and microscopic hematuria. He was found to have bilateral pulmonary emboli and left common femoral vein thrombosis; imaging studies showed evidence of renal arterial thrombosis with infarction. Hypercoagulability assessment showed an elevated factor VIII level. He was treated with heparin and warfarin with significant improvement in his renal function. Consideration should be given to measurement of factor VIII level as a part of the workup of unexplained thrombo-embolic events.Correspondence to:
A. Showkat, MD
956 Court Avenue, Suite 224
Memphis, TN 38163, USA
Email: [email protected]
Letter to the Editor
The incidence of fatal kidney biopsy
C.-C. Huang, C.-C. Kuo and Y.-M. Chen
Page No. 256
Abstract
Clinical Nephrology, Vol. 76 – No. 3/2011
The incidence of fatal kidney biopsy
C.-C. Huang1*, C.-C. Kuo2* and Y.-M. Chen3
1Department of Medical Imaging, Far Eastern Memorial Hospital, Taipei, 2Department of Internal Medicine, National Taiwan University Hospital Yun-Lin Branch, Yun-Lin, and 3Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
Correspondence to:
Y.-M. Chen, MD
Department of Internal Medicine, National Taiwan University Hospital, 7 Chung-Shan South Road, Tai pei, Taiwan
Email: [email protected]
