Volume 62, No. 3/2004(September)
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 Clinical Nephrology
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Originals
Pediatric IgA nephropathy: clinical features at presentation and outcome for African-Americans and Caucasians
Abstract
K.K. Lau, L.W. Gaber, N.M. Delos Santos, K.A. Fisher, S.J. Grimes and R.J. Wyat
1Children’s Foundation Research Center at the Le Bonheur Children’s Medical Center, Departments of 2Pediatrics and 3Pathology, University of Tennessee Health Science Center, Memphis, TN, USA
Aim: To determine the disease severity at onset and outcome for African-American and Caucasian pediatric patients with IgA nephropathy diagnosed at the Le Bonheur Children’s Medical Center since 1990. Design/Methods: The study population included all patients diagnosed with IgA nephropathy at the Le Bonheur Children’s Medical Center from January 1990 through February 2004. All were below age 18 at biopsy. Clinical features assessed at diagnosis were age, gender, presence of hypertension, history of macroscopic hematuria, degree of proteinuria, severity of renal histology and pattern for immunofluorescent reactants. Statistics: Student’s t-test was used to compare age at biopsy and length of follow-up between the 2 groups. Fisher’s exact test was used to compare features at presentation and patterns of immunofluorescence. Kidney survival was predicted by the Kaplan-Meier method. Results: Forty-seven patients (17 African-American, 29 Caucasian) were studied. Clinical features at diagnosis and pattern for all immunofluorescent reactants did not differ significantly between the 2 groups. Mesangial deposition of C1q occurred in 4/17 African-Americans as compared to 1/27 Caucasians (p = 0.06). Four patients (2 African-Americans, 2 Caucasians) progressed to end-stage renal disease. Predicted kidney survival was 96% (94% in African-Americans and 97% in Caucasians) at 1 year and 91% (94% in African-Americans and 89% in Caucasians) at 5 years from diagnosis. Mean time from diagnosis to end-stage renal disease or last follow-up was 3.3 years (3.8 for African-Americans, 3.0 for Caucasians). Macroscopic hematuria occurred prior to diagnosis for 90% of the Caucasian as compared to 61% of the African-American patients (p = 0.03). Urinalysis was normal at last follow-up visit for 24% of African-American patients and 32% of Caucasian patients. Conclusion: In a relatively small sample from a single center, except for the difference in macroscopic hematuria, clinical features at diagnosis and outcome of IgA nephropathy appear similar for African-American and Caucasian pediatric patients.Correspondence to:
Dr. R.J. Wyatt
Division of Pediatric Nephrology
Room 301 West Patient Tower
Children’s Foundation Research Center
50 N. Dunlap
Memphis, TN 38103, USA
Email: rwyatt@utmem.edu
Originals
Close relationship of plasminogen activator inhibitor-1 4G/5G polymorphism and progression of IgA nephropathy
Abstract
H. Suzuki, Y. Sakuma, Y. Kanesaki, M. Eiro, K. Asahi, H. Sanada, K. Watanabe, T. Katoh and T. Watanabe
Departments of Internal Medicine III and 2Pathology, Fukushima Medical University School of Medicine, Fukushima, Japan
Background: The plasminogen activator inhibitor-1 (PAI-1) 4G/4G genotype influences the development of diabetic nephropathy and lupus nephritis. However, the association of the PAI-1 4G/4G genotype and IgA nephropathy (IgAN) has not been investigated. Subjects and methods: The PAI-1 and ACE polymorphisms were examined in 270 healthy volunteers and 202 biopsy-proven IgAN patients, including 117 untreated IgAN patients who had an annual health check, allowing an estimation of the time of onset of overt proteinuria and/or hematuria. The relationship between the gene polymorphisms and the pathogenesis of IgAN were examined in 202 IgAN patients and the relationship between the gene polymorphisms clinical and pathohistological findings were examined in 117 untreated IgAN patients cross-sectionally at the time of renal biopsy. Results: 202 IgAN patients and 117 untreated IgAN patients did not have different frequencies in PAI-1 4G/5G (4G/4G : 4G/5 : 5G/5G = 82 : 90 : 30, 45 : 55 :17) and ACE I/D (DD : ID : II = 41 : 82 : 79, 21 : 54 : 42) gene polymorphisms compared with 270 healthy volunteers(4G/4G : 4G/5 : 5G/5G = 99 : 124 : 47, DD : ID : II = 53 : 106 : 111). However, IgAN with 4G/4G had significantly more advanced histological changes than IgAN with 4G/5G or 5G/5G both in glomerular and tubulointerstitial findings (p < 0.0005). The disease duration in IgAN with 4G/4G was shorter than in IgAN with 4G/5G + 5G/5G (6.22 ± 6.38 and 8.80 ± 9.79 years, respectively, p < 0.05). Creatinine clearance (Ccr) in IgAN with 4G/4G was significantly lower than IgAN with 4G/5G or 5G/5G (72.3 ± 26.5 and 82.4 ± 22.8 ml/min, respectively, p < 0.05). The mean urinary protein excretion in IgAN with 4G/4G was significantly more than in IgAN with 4G/5G or 5G/5G (1.10 ± 1.48 and 0.70 ± 1.01 g/day, respectively, p < 0.05). There was no difference between IgAN with the DD ACE genotype and IgAN with ID + II genotypes in either the clinical or histopathological findings. Conclusion: PAI-1 polymorphism is not associated with genesis of IgA nephropathy, but may be a risk factor for the progression of IgA nephropathy in Japanese.Correspondence to:
Dr. T. Katoh
Department of Internal Medicine III
Fukushima Medical University School of Medicine
Hikarigaoka, Fukushima, 960-1295 Japan
Email: t-katoh@fmu.ac.jp
Originals
A novel splice site mutation of the thiazide- sensitive NaCl cotransporter gene in a Japanese patient with Gitelman syndrome
Abstract
K. Iida, M. Hanafusa, I. Maekawa, T. Kudo, K. Takahashi, S. Yoshioka, M. Kishimoto, G. Iguchi, T. Tsukamoto, Y. Okimura, H. Kaji and K. Chihara
1Department of Clinical Molecular Medicine, Division of Endocrinology/Metabolism, Neurology and Hematology/Oncology, Kobe University Graduate School of Medicine, Kobe, 2Department of Basic Allied Medicine, Kobe University School of Medicine, Kobe and 3Department of Physiology and Biochemistry, College of Nursing Art and Science, Hyogo, Akashi, Japan
Gitelman syndrome (GS, MIM 263800) is an inherited disorder characterized by metabolic alkalosis with hypokalemia, hypomagnesemia, and hypocalciuria. The genetic abnormalities causing GS are known to lie in the thiazide-sensitive NaCl cotransporter (TSC), which is expressed in the distal tubule of the kidney. The TSC gene, located at chromosome 16, consists of 26 exons and encodes the protein containing 12 putative transmembrane domains with long intracellular amino and carboxy termini. Most of the abnormalities identified in GS were missense mutations, distributed throughout the TSC gene without a hot spot. A 42-year-old Japanese man was introduced for close examination of hypokalemia. In renal clearance studies using furosemide or thiazide, chloride clearance was increased after furosemide but not after thiazide administration. Furthermore, the distal fractional chloride reabsorption was dramatically decreased by furosemide but not thiazide administration, suggesting a defect in the distal tubule. We then analyzed the TSC gene to confirm the diagnosis of GS, and identified a novel G to T mutation at the acceptor splice site preceding exon 14, resulting in disruption of a conventional 3?AG consensus splice site. Abnormal splicing by this mutation is predicted to cause the formation of truncated TSC with a partial deletion of the transmembrane domain, which will loose the function of transporter. In conclusion, we have identified a unique novel splice site mutation of the TSC gene in GS. The predicted structure of this mutant TSC can conceivably cause an impairment of the transporter activity and thereby be responsible for the development of GS in our patient.
Correspondence to:
Dr. K. Iida
Division of Endocrinology/ Metabolism
Neurology and Hematology/Oncology
Department of Clinical Molecular Medicine
Kobe University Graduate School of Medicine
7-5-1, Kusunoki-cho, Chuo-ku
Kobe, 650-0017, Japan
Email: iidak2@aol.com
Originals
Sepsis and SOFA score: related outcome for critically ill renal patients
Abstract
N. Carbonell1, M. Blasco1, J. Ferreres1, J. Blanquer1, R. García-Ramón2, A. Mesejo1 and A. Miguel2
1Intensive Care Unit, and 2Nephrology Unit, Hospital Clínico Universitario, Valencia, Spain
Aims: To evaluate the influence of sepsis in critically ill patients with acute renal failure (ARF), and to analyze the value of the sequential organ failure assessment (SOFA) score for assessing the morbidity and related mortality of these patients. Material and methods: A prospective observational study developed in a medical intensive care unit (ICU) of a tertiary care university hospital. Data were collected from January 1, 2001 – July 31, 2002. The inclusion criterion was either a creatinine plasma level ³ 2 mg/dl on ICU admission or increases ³ 30% from its initial value. Sepsis was evaluated at the time of study inclusion, and patients were distributed into 2 groups (septic and nonseptic patients). Results: Two hundred patients with ARF were prospectively enrolled in the study (91 (45.5%) septic and 109 (54.5%) nonseptic patients). Median age was 68 years in septic patients and 72 in nonseptic ones while the percentage of males in both groups was 66% vs 69%, respectively. Septic patients showed more organ failures and more respiratory, cardiovascular and coagulation failures at the time of study admission as well as a worse mean SOFA score during the first 4 days after inclusion (p < 0.01). Mortality rate at the ICU was significantly higher in the septic group when compared to the nonseptic one (55% vs 19.3%, OR = 2.21 (1.65 – 2.97)). Using stepwise logistic regression, acute tubular necrosis and oliguria in septic patients as well as cardiovascular failure (evaluated by SOFA score) in nonseptic patients were identified as independent risk factors for mortality. Conclusions: Septic and nonseptic ICU patients with ARF have an increased risk of ICU mortality depending on the type of organ failure. Although SOFA score does not predict outcome, it is a useful tool to categorize these patients and to describe a sequence of complications in critically ill patients.
Correspondence to:
Dr. N. Carbonell
Hospital Clínico Universitario
Av. Blasco Ibáñez 17
E-46010 Valencia, Spain
Email: edurnecarbonell@yahoo.es
Originals
Efficacy and safety of lanthanum carbonate for reduction of serum phosphorus in patients with chronic renal failure receiving hemodialysis
Abstract
W.F. Finn, M.S. Joy, G. Hladik and the Lanthanum Study Group
Division of Nephrology and Hypertension, Department of Medicine,
University of North Carolina School of Medicine, Chapel Hill, NC, USA
Background: Lanthanum carbonate is a highly effective phosphate binder with significant potential as a treatment for hyperphosphatemia in patients with end-stage renal disease (ESRD). Here, the results of a placebo-controlled, dose-ranging study are presented. Methods: 196 patients (³ 18 years) receiving hemodialysis for at least 6 months entered a 1- to 3-week, single-blind, placebo run-in phase. Of these, 145 patients were randomized to a double-blind phase in which they received placebo or lanthanum carbonate in daily lanthanum doses of 225, 675, 1,350 or 2,250 mg for 6 weeks. Serum levels of phosphorus, calcium and parathyroid hormone, and adverse events were monitored throughout the study. Results: The intent-to-treat analysis (n = 144) showed significant dose-related reductions in serum phosphorus at lanthanum doses of 675, 1,350 and 2,250 mg. After 6 weeks of treatment, phosphorus levels were significantly lower in the lanthanum groups receiving 1,350 mg/day and 2,250 mg/day, compared with the placebo group (respective changes from randomization: –0.95 ± 1.39 mg/dl (–0.31 ± 0.45 mmol/l), –1.13 ± 2.01 mg/dl (–0.36 ± 0.65 mmol/l), 0.75 ± 1.47 mg/dl (0.24 ± 0.47 mmol/l), p < 0.001). Significant reductions in serum phosphorus, compared with placebo, occurred in the lanthanum 1,350 mg/day group from the second week of treatment and in the 2,250 mg/day group from the first week of treatment. Adverse events were mainly gastrointestinal (e.g. nausea and vomiting). Treatment-related adverse events occurred in 39% of patients treated with lanthanum carbonate and 44% of the placebo group. Conclusion: Lanthanum carbonate is an effective and well-tolerated agent for the short-term treatment of hyperphosphatemia in patients with ESRD.Correspondence to:
Dr. W.F. Finn
Division of Nephrology and Hypertension
Department of Medicine
University of North Carolina School of Medicine
345 MacNider Building, CB #7155
Chapel Hill, NC 27599, USA
Email: wffinn@med.unc.edu
Originals
High ratio of 1,25-dihydroxyvitamin D3 to parathyroid hormone in serum of tuberculous patients with end-stage renal disease
Abstract
K. Yonemura, T. Ohtake, H. Matsushima, Y. Fujigaki, and A. Hishida
1Hemodialysis Unit and 4First Department of Medicine, Hamamatsu University School of Medicine, 2Department of Nephrology, Shonan Kamakura General Hospital, Kamakura, and 3Department of Nephrology, Seirei Mikatagahara General Hospital, Hamamatsu, Japan
Aim: Diagnosis of tuberculosis is sometimes difficult because of the low specificity of diagnostic procedures especially in patients on end-stage renal disease (ESRD). As abnormal vitamin D metabolism has been reported in tuberculosis, the aim of the present study was to determine whether serum concentration of 1,25-dihydroxyvitamin D3 (1,25-(OH)2D3) may be a useful diagnostic indicator of tuberculosis in patients with ESRD. Patients and methods: Serum concentrations of 1,25-(OH)2D3, parathyroid hormone (PTH), and calcium were compared in 6 patients with ESRD and active tuberculosis (ESRD-TB group) and 110 patients with ESRD and no tuberculosis (ESRD group). These parameters were compared before and after treatment for tuberculosis in patients of ESRD-TB group. Results: Hypercalcemia was observed in all 6 patients in the ESRD-TB group. Both higher serum concentration of 1,25-(OH)2D3 and lower serum concentration of PTH were observed in the ESRD-TB group relative to the ESRD group, suggesting enhanced extrarenal production of 1,25-(OH)2D3 and suppressed secretion of PTH by hypercalcemia in the ESRD-TB group. However, these parameters could not be used to distinguish the ESRD-TB group from the ESRD group. The ratio of 1,25-(OH)2D3 to PTH in serum was above 0.9 in the ESRD-TB group and below 0.9 in the ESRD group. Antituberculous treatment reduced this ratio to the range observed in the ESRD group. Conclusion: High ratio of 1,25-(OH)2D3 to PTH in serum is noted in active tuberculous patients with ESRD because of enhanced extrarenal production of 1,25-(OH)2D3.Correspondence to:
Dr. K. Yonemura
Hemodialysis Unit
Hamamatsu University School of Medicine
1-20-1 Handayama
Hamamatsu, 431-3192, Japan
Email: katsuh@hama-med.ac.jp
Originals
Evidence-based medical quality management in dialysis - Part I: Routine implementation of QiN, a German quality management system
Abstract
M.P. Stoffel, C. Barth, K.W. Lauterbach, C.A. Baldamus and participating Dialysis Units
1Division of Nephrology, Department of Internal Medicine, University of Cologne, 2KfH Head Office, Neu-Isenburg, and 3Institute for Health Economics and Clinical Epidemiology (IGKE), Cologne, Germany
Increasing medical complexity, centrifugal forces of medical subspecialization and growing economic constraints are the key reasons for the introduction of quality management into routine care processes such as dialysis. Adequate quality assurance and improvement must be implemented in order to supply medical staff, care providers, and patients with the necessary information on critical issues of clinical management of dialysis patients. QiN (Quality in Nephrology), the quality management program of the largest German dialysis provider, is outlined here as a practicable example. The first of 2 parts provides information on the structure, implementation of QiN and achieved clinical improvement in routine care. The second part (quotation) analyzes longitudinal data in order to differentiate whether observed improvements during more than 5 years of QiN can be ascribed to the intervention (application of QiN) or whether they are due to other factors such as generally improved medical knowledge.
Correspondence to:
Dr. M.P. Stoffel
QiN-Gruppe/IGKE
Universität zu Köln
Gleueler Straße 176-178
D-50935 Köln
Email: markusp.stoffel@uni-koeln.de
Originals
Evidence-based medical quality management in dialysis - Part II: Improvement of hemodialysis adequacy
Abstract
M.P. Stoffel, C. Barth, K.W. Lauterbach, C.A. Baldamus and participating Dialysis Units
Case reports
A possible favorable effect of colchicine in IgA nephropathy in a carrier of a MEFV mutation
Abstract
L. Koukoui, A. Blau, J. Kopolovic, M. Pras and
1Department of Medicine F, 2Department of Nephrology and Hypertension, 3Department of Pathology, 4Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
IgA nephropathy is the most common primary glomerulopathy. Currently, no satisfactory treatment is available and as a result, a significant proportion of affected patients progress to end-stage renal disease. We present a patient with IgA nephropathy in whom continuous colchicine treatment induced remission, which has lasted for 22 years. The patient was a carrier of a mutation in the FMF gene (MEFV). This case raises hopes for a better prognosis in at least one subgroup of IgA nephropathy, consisting of patients who happen to be heterozygous carriers of MEFV mutations.Correspondence to:
Dr. A. Livneh
Heller Institute of Medical Research
Sheba Medical Center
Tel-Hashomer, 5262
Israel
Email: alivneh@post.tau.ac.il
Case reports
Widespread crystalline inclusions affecting podocytes, tubular cells and interstitial histiocytes in the myeloma kidney
Abstract
M. Tomioka, K. Ueki, H. Nakahashi, A. Isoda, T. Kuroiwa, Y. Kaneko, K. Hiromura, and Y. Nojima
Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, Gunma, Japan
Numerous crystalline inclusions were observed in glomerular and tubular epithelial cells in a 46-year-old female patient with multiple myeloma and renal dysfunction. On light microscopy, epithelial cells were filled with homogenous materials and were remarkably swollen. Infiltrations of histiocytes with expanded cytoplasm were also seen in the interstitium of the kidney and bone marrow. On electron microscopy, cytoplasmic inclusions had crystalline structure showing rhomboid and oval shapes. Immunofluorescence study revealed that these cells were positive for IgG-k. The combination chemotherapy followed by autologous stem cell transplantation led to a partial resolution of her renal dysfunction, continued by a slight reduction in the number of crystalline-containing podocytes at the second renal biopsy. Crystal inclusions in the kidney are rarely found and cause renal impairment in multiple myeloma.Correspondence to:
Dr. K. Ueki
Department of Medicine and Clinical Science
Gunma University Graduate School of Medicine
3-39-15 Showa-machi
Maebashi, Gunma 371-8511, Japan
Email: kueki@showa.gunma-u.ac.jp
Case reports
Antineutrophil cytoplasmic antibody-associated necrotizing crescentic glomerulonephritis in a patient receiving treatment with etanercept for severe rheumatoid arthritis
Abstract
T.W.R. Doulton, B. Tucker, J. Reardon and N. Velasco
1Department of Nephrology, and 2Department of Rheumatology,
Mayday University Hospital, Croydon, Surrey, UK
Etanercept is a tumor necrosis factor inhibitor used in the treatment of rheumatoid arthritis and, increasingly, in a range of other diseases. We report a case of necrotizing crescentic glomerulonephritis, associated with a positive antineutrophil cytoplasmic antibody, causing acute renal failure in a woman receiving treatment with etanercept for severe rheumatoid arthritis. Our patient was treated with steroids and cyclophosphamide following withdrawal of etanercept, with a good clinical response. Although reports of vasculitis in patients receiving treatment with etanercept are rare, this drug has been shown to up-regulate some aspects of immune function, and the possibility that this agent may precipitate or exacerbate vasculitis in some individuals has to be considered.Correspondence to:
Dr. T. Doulton
Department of Nephrology
Mayday University Hospital
London Road
Croydon, Surrey CR7 7YE, UK
Email: tdoulton@doctors.org.uk
Letters to the Editor
Temporary suprarenal inferior vena cava filter for renal biopsy in a patient with renal vein thrombosis
Abstract
A. Wieland, A. Mehta, A. Greenfield and M. Wolf
Letters to the Editor
The shorter T1/2 of cystatin C explains the earlier change of its serum level compared to serum creatinine
Abstract
P. Sjöström, M. Tidman and I. Jones
Letters to the Editor
Sodium reduces calcium binding to albumin and fibrinogen
Abstract
H. Takahashi, Y. Ando, T. Akimoto, Y. Asano and E. Kusano
