Volume 57, No. 2/2002(February)
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 Clinical Nephrology
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Review
Lupus nephritis: a clinical review for practicing nephrologists
Abstract
G. Contreras, D. Roth, V. Pardo, L.G. Striker and D.R. Schultz
1Dialysis Unit VAMC, 2Division of Nephrology, 3Electron Microscopy VAMC, 4Renal Cell Biology Laboratory and 5Division of Rheumatology and Immunology, Veterans Affairs Medical Center and University of Miami School of Medicine, Miami, FL, USA
The renal manifestations in systemic lupus erythematosus (SLE) are protean and difficult to categorize into clinical syndromes and histologic classes. Lupus nephritis is frequently unrecognized until full-blown nephritic and/or nephrotic syndrome with renal failure emerge. Epidemiologically, approximately one third of SLE patients from unselected populations have renal involvement early during the disease. Most renal abnormalities emerge within the first few years of SLE diagnosis. Currently, most nephrologists agree that an early renal biopsy is worthwhile in those SLE patients with abnormal urinalysis and/or reduced renal function. First, it provides a histologic categorization of the glomerulonephritis as well as an assessment of the degree of activity and chronicity. Second, it provides vital prognostic information. Third, it is beneficial in planning a more rational therapy with or without potentially toxic immunosuppressive agents. Over the last 3 decades, many controlled clinical trials for treatment of lupus nephritis have been completed with a few therapeutic immunosuppressive regimens. Among those agents used, cyclophosphamide and azathioprine provide a reduction of morbidity in those patients afflicted with proliferative forms of lupus glomerulonephritis. A new immunosuppressive agent, mycophenolate mofetil, is being studied for treatment of proliferative forms of lupus glomerulonephritis in a controlled clinical trial at our institution. Immunosuppressive agents and the availability of dialysis and transplantation have improved the survival of patients with lupus nephritis, in particular those with proliferative forms.
Originals
Comparative effects of plasmapheresis and intravenous cyclophosphamide on urinary podocyte excretion in patients with proliferative Lupus nephritis
Abstract
T. Nakamura, C. Ushiyama, M. Hara, S. Osada, K. Ugai, N. Shimada, K. Hayashi, I. Ebihara and H. Koide
1Department of Medicine, Misato Junshin Hospital, Saitama, 2Department of Pediatrics, Yoshida Hospital, Niigata, 3Department of Medicine, National Rehabilitation Center, Saitama, 4Housei Kidney Clinic, and 5Department of Medicine, Koto Hospital, Tokyo, Japan
Intravenous cyclophosphamide (IVC) in combination with steroids is standard therapy for Lupus nephritis. Reduction of autoantibodies and circulating immune complexes can be used in the treatment of autoimmune diseases. The aim of the present study was to compare the effects of IVC pulse therapy and double-filtration plasmapheresis (DFPP) on proteinuria and urinary excretion of podocytes in adult patients with diffuse proliferative Lupus nephritis (DPLN). Twenty patients were
randomly assigned to two groups. Group A (n = 10) was treated with IVC (0.75 – 1.0
g/m2 body surface area) pulse therapy, given as boluses once a month for 6
consecutive months, combined with oral corticosteroid (up to 1 mg/kg/day) administration.Group B (n = 10) was treated with a combination of DFPP (performed 1 – 2 times weekly) and corticosteroid (up to 1 mg/kg/ day). The total average number of treatments was 8.4 and the therapeutic efficacies were evaluated after 6 months. Twenty healthy individuals participated as a control group. Urinary podocytes were examined by immunofluorescence with monoclonal antibodies against podocalyxin. Both Group A and Group B reduced proteinuria (p < 0.001) as well as the number of urinary podocytes (p <0.001). Differences between the 2 treatment outcomes were not statistically significant.
Cyclophosphamide pulse therapy and DFPP may be similarly effective in the treatment of podocyte injury in patients with DPLN.
Originals
Lupus nephritis: a retrospective review of 78 cases from a single center
Abstract
L. Martins1, G. Rocha1, A. Rodrigues1, J. Santos1, C. Vasconcelos2, J. Correia2, F. Farinha2, I. Almeida2, P. Barbosa2 and S. Guimarães1
1Nephrology and 2Medicine Department, Hospital de Santo António, Porto, Portugal
Lupus nephritis (LN) is a frequent and serious manifestation of systemic lupus erythematosus. However, the outcome has
progressively improved in the last 3 decades and this was due to more efficient and early
treatment of LN and comorbid situations. The aim of our study was to analyze our
experience and outcome in LN, to evaluate clinicopathologic and clinicolaboratory
correlations and to search for risk factors for renal and patient survival. Patients and methods: We conducted a retrospective study of 78 patients with biopsy-proven LN. Results:Acute renal failure and subnephrotic proteinuria with microhematuria occurred each one in 39.7% of the patients, nephrotic syndrome in 16.7% and nephritic syndrome in 3.8%. The mean serum creatinine at presentation was 1.45 ± 1.03 mg/dl and the creatinine clearance was 68.2 ± 40.3 ml/min. Class IV LN existed in 71.8%, Class III in 20.5%, Class V in 6.4% and Class VI in 1.3%. The treatment included steroids and cytotoxic agents in 87.5% of the patients with proliferative LN. Hypertension, serum creatinine and acute renal failure at
presentation, as well as significant chronicity on renal biopsy, were significantly correlated with the progression to chronic renal failure in our population. Males were more prone to develop renal flares. 3.8% of the patients died, 9% lost their renal function, 26.9% are in remission, 33.3% still have subnephrotic proteinuria and microhematuria, 7.7% have nephrotic syndrome and 19.2% have chronic renal failure. The mean global follow-up was 102 ± 74 months and 96.2% of the patients survived. The actuarial renal survival was 96.1% in the first year; 89.9% at 5 years; and 83.7% at 10 and 20 years. Conclusion: We can say that hypertension, serum creatinine and acute
renal failure at the onset and significant chronicity on renal biopsy, proved to be risk
factors for chronic renal failure in our study population. Male gender was a risk factor
for renal flares. The achieved global outcome can be considered a good result.
Originals
Minimal change disease in systemic lupus erythematosus
Abstract
G.K. Dube1, G.S. Markowitz1, J. Radhakrishnan2, G.B. Appel2 and V.D. D’Agati1
1Department of Pathology and 2Department of Medicine, Columbia University, New York Presbyterian Hospital, New York, NY, USA
We report the clinical and pathologic findings in 7 patients with systemic lupus erythematosus and minimal change disease. All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria (mean 9.6 g/day), and hypoalbuminemia (mean 1.8 g/dl). In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. In all cases, steroid therapy induced a rapid remission of nephrotic syndrome. Minimal change disease is an underrecognized and readily reversible form of nephrotic syndrome in systemic lupus erythematosus. Because it may occur superimposed on mild mesangial proliferative lupus nephritis, this entity may be misinterpreted as an atypical presentation of lupus nephritis class II. Proper recognition of this entity requires careful integration of the renal biopsy immunofluorescence and electron microscopic findings.
Originals
Renal function in children with sickle cell anemia
Abstract
A. Karabay Bayazit, A. Noyan, B. Aldudak, A. Özel, A. Anarat, Y. Kilinç, I. Sasmaz, E. Gali, R. Anarat and N. Dikmen
1Department of Pediatric Nephrology, 2Department of Pediatrics,
3Department of Pediatric Hematology, Çukurova University School of Medicine, 4Antakya State Hospital, 5Biochemistry Laboratory, Adana Hospital, Baskent University, and 6Department of Biochemistry, Çukurova University School of Medicine, Balcali, Adana, Turkey
Background: Patients with sickle cell anemia have various forms of renal dysfunction. Subjects, materials and methods: The purpose of this study is to demonstrate the abnormalities of HbSS patients’ renal function in childhood. Renal function studies were performed in 55 patients with homozygote sickle cell anemia and compared with 13 healthy children. The blood and timed urine samples were obtained for hematological and biochemical determinations. Results: Mean serum creatinine, sodium, phosphorus and calcium levels were not statistically different between patients and controls. Mean serum potassium and uric acid levels were significantly higher in patients than in controls. Mean tubular phosphate reabsorption (p < 0.001) and fractional excretion of potassium (p < 0.05) were lower in patients than in the control. There were no significant differences in fractional excretion of sodium and uric acid between patients and controls. Patients had significantly higher urine pH and significantly lower specific gravity and osmolality than controls. Also, there were no significant differences in urinary protein/ creatinine, urinary N-acetyl-b-D-glucosaminidase/creatinine and urinary malondialdehyde/creatinine between patients and controls. Conclusion: Thus, significant proximal tubular dysfunction is not a common feature but distal tubular abnormality is the most consistent renal functional derangement of patients with SCA in childhood.
Originals
Calcium-sensing receptor gene polymorphism affects the parathyroid response to moderate hypercalcemic suppression in patients with end-stage renal disease
Abstract
K. Yokoyama, T. Shigematsu, T. Tsukada, S. Hara, A. Yamada, Y. Kawaguchi and T. Hosoya
1The Division of Nephrology and Hypertension, Jikei University School of Medicine, Tokyo, 2Nephrology and Dialysis Unit, Department of Internal Medicine, Sakura National Hospital, Chiba, 3Department of Clinical Physiology, Toranomon Hospital, Tokyo, 4Kidney Center, Toranomon Hospital, Tokyo, Japan
Aim: The basic mechanism of secondary hyperparathyroidism is still unclear, but a change in Ca2+ sensing by parathyroid cells is possibly involved in this uremic complication. A rightward shift of the calcium set-point and an increase of the minimum secretion rate have been found in secondary hyperparathyroidism, indicating abnormal calcium sensing. Methods: We evaluated the effect of calcium sensing receptor (CaR) gene polymorphism (codon G990R) on the response of the parathyroid gland to moderate hypercalcemic suppression in 77 ESRD patients on regular hemodialysis (HD using 2.5 mEq/l Ca2+ dialysate). All patients underwent an HD session with 3.0 mEq/l Ca2+ dialysate to suppress parathyroid hormone (PTH). Then we investigated the effect of CaR gene polymorphism on the parathyroid response to hypercalcemic stimulation. Results: Patients were divided into 3 groups on the basis of genotype (GG = 33 patients (42.9%), GR = 39 patients (50.6%), RR = 5 patients (6.5%)). Baseline intact PTH levels in patients without the R allele were not significantly different from those in patients with the R allele (GG group, 181.4 ± 31.1 pg/ml vs. GR and RR groups, 230 ± 51.2 pg/ml: mean ± SEM). The significant effect of moderate hypercalcemic suppression on the intact PTH level was observed in the GG group (p < 0.01) but not in the GR and RR groups, despite the identical increase in Ca2+. Conclusion: Our results suggest that CaR gene polymorphism (codon G990R) influences the responsiveness of the parathyroid gland to changes of extracellular Ca2+ in ESRD patients. The glands of patients with the GG genotype of the CaR gene may be more sensitive to extracellular Ca2+ changes.
Originals
Reduction in erythropoietin doses by the use of chronic intravenous iron supplementation in iron-replete hemodialysis patients
Abstract
C.-H. Chang, C.-C. Chang and S.-S. Chiang
Department of Internal Medicine, Division of Nephrology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
Background: Iron deficiency is the most common cause of suboptimal response to recombinant human erythropoietin (rHuEPO) in chronic hemodialysis (HD) patients. Iron supply can correct this situation, however, optimal dosage, route of administration, and monitoring of iron status during rHuEPO therapy in maintenance HD patients remains controversial. Methods: We conducted a 12-month intravenous iron substitution trial in 149 iron-replete chronic HD patients receiving subcutaneous rHuEPO therapy. The available iron pool was maintained with 100 mg iron every 2 weeks or 1 month depending on serum ferritin and transferrin saturation levels, the rHuEPO dosage titrated depending on hematocrit (Hct) levels. Results: After 12-month protocol, the Hct increased (28.7 ± 4.1 vs 27.7 ± 2.6, p = 0.003), rHuEPO requirement reduced 25% (46.1 ± 28.9 vs 61.5 ± 67.8 U/kg/week, p = 0.006), serum ferritin increased (1383 ± 727 vs 930 ± 857 ng/ml, p < 0.001), so did the transferrin saturation (36.1 ± 12.7 vs 27.5 ± 12.8%, p < 0.001). The serum albumin decreased slightly but reached statistical significance (4.1 ± 0.48 vs 4.2 ± 0.36 g/dl, p = 0.006), so did the cholesterol levels (166 ± 41 vs 173 ± 38 mg/dl, p = 0.044) and pre-dialysis creatinine (11.3 ± 2.3 vs 11.5 ± 2.4 mg/dl, p = 0.015). Besides, the iPTH levels did not interfere with the rHuEPO dosage reduction and Hct increment in our patients. Conclusion: We conclude that maintaining high levels of serum ferritin and transferrin saturation could further reduce the requirement of rHuEPO in chronic HD patients, but the long-term effect of iron overloading to patients’ nutritional status must be further evaluated in contrast to the economic saving.
Case reports
Renal involvement in sarcoidosis - a report of 6 cases
Abstract
M. Brause, K. Magnusson, S. Degenhardt, U. Helmchen and B. Grabensee
1Department of Nephrology and Rheumatology, Heinrich Heine University, 2Evangelisches Krankenhaus, Düsseldorf and
3Department of Pathology, University Hamburg, Germany
This report concerns 6 patients with renal involvement in sarcoidosis. Two of the patients had no clinical symptoms at all. In 3 patients, no extrarenal organ manifestation was found. All 6 patients had elevated levels of serum creatinine, 2 were hypercalcemic. Five patients manifested with mild proteinuria, but in none of the cases was a nephritic sediment with erythrocytes found. Kidney biopsies in 5 patients showed epitheloid cell granulomatous interstitial nephritis, and 1 patient presented with nephrocalcinosis. All patients were treated with corticosteroids. The serum creatinine levels decreased significantly in 4 patients (> 50% decrease), and slightly in 2 patients, elevated serum calcium levels were normalized. Thus, even in the absence of other organ manifestations, sarcoidosis can be the cause of renal insufficiency, and it responds well to corticosteroid treatment. These patients demonstrate the importance of kidney biopsy in the unexplained deterioration of renal function.
Case reports
Interstitial nephritis, acute renal failure in a patient with non-fulminant hepatitis A infection
Abstract
A.-L. Vaboe, S. Leh and T. Forslund
1Renal Research Group, Institute of Medicine, 2Department of Pathology, Gades Institute, Haukeland University Hospital, Bergen, Norway, and
3Department of Medicine, Central Hospital of Jyväaskylä, Jyväaskylä, Finland
This is the first report from Norway of a patient with interstitial nephritis and renal failure due to non-fulminant hepatitis A virus (HAV) infection. HAV infection was confirmed by positive anti-HAV IgM serology. All tests for other virus infections were negative. At admittance serum creatinine (s-Creat) and blood urea nitrogen (BUN) concentration were 539 mmol/l and 32.6 mmol/l increasing the following days to 890 mmol/l and 39.9 mmol/l, respectively. Nine courses of hemodialysis had to be given. Kidney biopsy specimen showed interstitial edema, lymphocytic cell infiltration and acute tubular injury with normal glomeruli. Examination with immunohistochemistry was negative. In contrast to the findings associated with HBV and HCV infection in which glomerular disease is predominantly found, the HAV infection in our patient was associated with interstitial nephritis and acute tubular necrosis. The prognosis of the renal failure due to HAV infection was good although the recovery was substantially delayed.
Case reports
Type I membranoproliferative glomerulonephritis in an HIV-infected individual without hepatitis C co-infection
Abstract
M. Chidambaram, C.E. Stigant, L.M. Sugar and G.V. Ramesh Prasad
1Department of Medicine, 2Department of Pathology, St. Michael?s Hospital, University of Toronto, Toronto, Ontario, Canada
Type I membranoproliferative glomerulonephritis (MPGN) is an uncommon manifestation of human immunodeficiency virus (HIV)-associated renal disease in patients co-infected with hepatitis C virus (HCV). We describe a case of Type I MPGN in an HIV-positive diabetic man with nephrotic-range proteinuria and renal insufficiency who was not co-infected with HCV. Tubuloreticular inclusions were present but there was no evidence for either cryoglobulinemia or cryoglobulin deposits in the kidney. This finding suggests that Type I MPGN may represent a reaction of the kidney to HIV independent of the effects of HCV co-infection. Clinical suspicion must be maintained for Type I MPGN in all HIV infected patients presenting with significant proteinuria regardless of HCV infection status.
Case reports
Hyperhomocysteinemia, low folate status, homozygous
C677T mutation of the methylene tetrahydrofolate reductase and renal arterial thrombosis
Abstract
G. Queffeulou, C. Michel, F.Vrtovsnik, J.-B. Philit, E. Dupuis and F. Mignon
Department of Nephrology, CHU Bichat-Claude Bernard, Assistance Publique Hopitaux de Paris, Paris, France
We report a renal artery thrombosis in a 42-year-old man. Fasting homocysteinemia was at 23 mmol/l 3 months later and at 33 mmol/l 5 months after the vascular event. A homozygous C677T MTHFR was found with low folate status. Active smoking may also have contributed to the pathogenesis of renal arterial thrombosis. The other causes of thrombophilia were ruled out. Homocysteine lowering treament was started: homocysteine normalized at 10.6 mmol/l. There was no recurrence of vascular event within 18 months. We propose mild or moderate hyperhomocysteinemia triggered by low folate status in patients with homozygous C677T MTHFR as a cause of renal arterial thrombosis.
Case reports
Necrotic skin lesions in a
dialysis patient: a multifactorial entity
Abstract
V. Garrigue1, R. Lorho2, S. Canet1, D. Augias3, M.-F. Servel4, B. Canaud1, B. Albat5, G. Mourad1 and A. Argilés1,2
1Department of Nephrology, University Hospital Lapeyronie, 2Institut de Génétique Humaine – CNRS UPR 1142, 3Department of Dermatology, University Hospital St Eloi, 4Association pour l’Installation à Domicile des Epurations Rénales (AIDER), and 5Department of Cardiovascular Surgery, University Hospital Arnaud de Villeneuve, Montpellier Cedex 5, France
A female dialysis patient with a consistently high serum calcium phosphate product presented with large necrotic skin lesions with ulcers. The clinical course was highly suggestive of calciphylaxis. Parathyroidectomy was followed by the healing of the lesions. New skin lesions appeared following relapse of hyperparathyroidism. Her clinical records included a long past of hypertension, which was the cause of her renal failure. She had a limited walking range and previously had presented bilateral ulcers of vascular origin. This case presents a type of lesion which bears a serious prognosis in dialysis patients. The clinical context and the presentation of the lesions are compatible with multiple etiology: vascular lesions and calciphylaxis. The documented longitudinal follow-up illustrates the importance of treating the different factors known to participate in the appearance of skin lesions in dialysis patients. Particularly, it stresses the benefit of performing parathyroidectomy, even if the parathyroid hormone level is not in the range normally accepted as requiring surgical removal of parathyroid glands.
Case reports
Is continuous veno-venous hemofiltration for acetaminophen-induced acute liver and renal failure worthwhile?
Abstract
R. Agarwal and M.O. Farber
Indiana University & Roudebush VA Medical Center, Indianapolis, USA
CVVH for acetaminophen intoxication?
Abstract. We describe a patient with acute liver and renal failure secondary to acetaminophen and chronic alcohol abuse who was treated aggressively with oral acetylcysteine, continuous renal replacement therapy, glucose and branched-chain amino acid intravenous feeding and ventilatory support. The patient had a predicted mortality of > 95% without liver transplantation however, with intensive ventilatory, renal, and nutritional support he made a complete recovery. We discuss the benefits of aggressive supportive therapy and suggest that continuous renal replacement therapy may allow gentle fluid removal, excellent control of cerebral edema and intravenous feeding that may favorably influence prognosis.
Letters to the Editor
Renal involvement in a patient with idiopathic hypereosinophilic syndrome
Abstract
F. Bulucu, C. Can, V. Inal, Y. Baykal and S. Erik
Letters to the Editor
Focal and segmental glomerulosclerosis and non-Hodgkin?s lymphoma
Abstract
J.M. Calvo Villas, A. Morales Umpierrez, M.J. Ram
