Volume 67, No. 1/2007(January)
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 Clinical Nephrology
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Originals
Renal-coloboma syndrome: a single nucleotide deletion in the PAX2 gene at Exon 8 is associated with a highly variable phenotype
Abstract
A. Taranta, A. Palma, V. De Luca, A. Romanzo, L. Massella, F. Emma and L. Dello Strologo
1Nephrology and Urology Department and 2Department of Ophthalmology, Bambino Gesu Children's Hospital, Rome, Italy
Background: Renal-coloboma syndrome (RCS) is an autosomal dominant disorder characterized by renal abnormalities and optic nerve defects, caused by heterozygous mutations of the PAX2 gene. This gene encodes for the PAX2 developmental nuclear transcription factor, which is primarily expressed during embryogenesis in kidneys, eyes, ears and in the central nervous system. The aim of the present study was to characterize PAX2 mutations in a renal coloboma syndrome family with a highly variable phenotype. Methods: DNA screening was performed by direct sequencing. Results: Five subjects over three generations presented with renal hypodysplasia or horseshoe kidneys in association with bilateral optic nerve colobomas in four cases. one patient with early-onset renal failure had no detectable eye defects. All five subjects carried a novel PAX2 mutation consisting in a frameshift mutation located in Exon 8 (G911del), which causes premature termination of translation and loss of the PAX2 transactivation domain. Conclusion: This is the first report of a PAX2 mutation located in Exon 8. The variability of clinical symptoms may be explained by the limited disruption of the protein sequence at the transactivation domain.Correspondence to:
L. Dello Strologo, MD
Department of Nephrology and Urology
Division of Nephrology
Bambino Gesu Children's Hospital
Piazza S. Onofrio, 4
00165 Rome, Italy
Email: dellostrologo@opbg.net
Originals
Favorable long-term outcome of nephrotic syndrome after allogeneic hematopoietic stem cell transplantation
Abstract
M.J. Kemper, T. Güngör, J. Halter, U. Schanz and T.J. Neuhaus
1Department of Pediatric Nephrology, University Children’s Hospital Eppendorf, Hamburg, Germany, 2Department of Immunology/Hematology, University Children’s Hospital Zurich, 3Department of Hematology, University Hospital Zurich and 4Department of Pediatric Nephrology, University Children’s Hospital Zürich, Switzerland
Background: The development of nephrotic syndrome (NS) after allogeneic hematopoietic stem cell transplantation (HSCT) is a rare complication with few long-term outcome data. Patients: Clinical course and long-term outcome of three adult patients and one child with NS after HSCT (total number of transplants n = 533) are presented. Results: The median age at onset of NS was 35 years (range 15 – 56), occurring at a median of 17 months (range 11 – 21) after HSCT. Discontinuation of cyclosporine A (CSA) prior to onset of NS was a consistent feature and occurred a median of 6 months (range 2 – 10 months) prior to the development of NS. The histopathological lesion was membranous nephropathy (n = 3) and membranoproliferative glomerulonephritis Type 1 (n = 1). History of acute or concomitant clinically apparent chronic graft versus host disease (GVHD) was present in all cases except the pediatric patient who had abundant DR-activated cytotoxic T cells without evidence of viral reactivation. Long-term immunosuppression for 11 – 36 months with steroids (n = 1), combined steroids and CSA (n = 2) or CSA alone in steroid-refractory NS (n = 1) resulted in sustained remission of the NS in all patients (12 months – 8 years off immunosuppression). Conclusion: NS after HSCT seems to be etiologically related to subclinical or overt chronic GVHD, which flares up after discontinuation of CSA. However, resumption of immunosuppression can reverse NS as well as GVHD and induce favorable sustained long-term remission.Correspondence to:
T. Neuhaus, MD Pediatric Nephrology, Kinderspital Steinwiesstrasse 75 8032 Zurich, Switzerland
Email: Thomas.Neuhaus@kispi.unizh.ch
Originals
Prospective comparison of the effects of maxacalcitol and calcitriol in chronic hemodialysis patients with secondary hyperparathyroidism: a multicenter, randomized crossover study
Abstract
T. Mochizuki, S. Naganuma, Y. Tanaka, Y. Iwamoto, C. Ishiguro, Y. Kawashima, K. Maekawa, A. Suda and T. Akiba for TWMU Renal Osteodystrophy Study Group
TWMU Renal Osteodystrophy Study Group includes the following facilities: Tokyo Women’s Medical University Hospital, Kameda Medical Center, Naganuma Clinic, Shinjyuku Kohsin Clinic, Suda Clinic, Saito Memorial Hospital, Bousei Nishi-Shinjyuku Clinic, Toda Chuo General Hospital, Aiwa Clinic, Koenji Suzuki Clinic, Minamicho Clinic, Seiwa Jin Clinic, Saiseikai Kawaguchi General Hospital, Okubo Watanabe Clinic, Shinobe Clinic, Hidaka Hospital, Tokyo Women’s Medical University, Daini Hospital Tabata Ekimae Clinic
Background: Maxacalcitol is a vitamin D analogue, which is administered intravenously for secondary hyperparathyroidism in dialysis patients as well as calcitriol. However, few dose-comparison clinical studies have been reported for these drugs. The present multicenter, randomized crossover study was conducted to determine the equivalence of maxacalcitol and calcitriol doses. Methods: Subjects comprised 31 patients on chronic hemodialysis with secondary hyperparathyroidism who had not received maxacalcitol or calcitriol in the previous 3 months. Patients were randomly divided into two groups, and maxacalcitol or calcitriol was administered in a crossover design for 12 weeks each. Maxacalcitol and calcitriol doses were adjusted based on serum levels of calcium and intact parathyroid hormone. Results: After the 12-week maxacalcitol/calcitriol administration, there were no significant differences in levels of calcium (maxacalcitol 2.40 ± 0.22 mmol/l (9.6 ± 0.9 mg/dl), calcitriol 2.42 ± 0.25 mmol/l (9.7 ± 1.0 mg/dl), p = 0.71), phosphate (maxacalcitol 1.97 ± 0.42 mmol/l (6.1 ± 1.3 mg/dl), calcitriol 2.00 ± 0.48 mmol/l (6.2 ± 1.5 mg/dl), p = 0.64), intact parathyroid hormone (maxacalcitol 267 ± 169 pg/ml, calcitriol 343 ± 195 pg/ml, p = 0.11) in serum or other bone-metabolic parameters such as serum alkaline phosphatase. The doses of maxacalcitol and calcitriol were 49.3 ± 23.7 µg/month and 9.0 ± 3.8 µg/month, respectively, and maxacalcitol : calcitriol dose ratio was 5.5 : 1. No severe adverse reactions were seen for either maxacalcitol or calcitriol during the study period. Conclusions: Comparable therapeutic efficacy can be obtained in the treatment of secondary hyperparathyroidism using either maxacalcitol or calcitriol at a dose ratio of 5.5 : 1.
Correspondence to:
T. Mochizuki, MD
Department of Nephrology
Kameda Medical Center
929 Higashi-cho, Kamogawa Chiba 296-8602, Japan
Email: tmochizuki@kameda.jp
Originals
Low-calcium dialysate improves mineral metabolism in hemodialysis patients
Abstract
A. Fujimori, M. Yorifuji, M. Sakai, M. Oyama, N. Nakao, M. Tokuyama and M. Fukagawa
1Department of Artificial Kidney, 2Department of Internal Medicine, Konan Hospital, 3Department of Nephrology and Blood Purification Center, Rokko Island Hospital, 4Division of Nephrology and Dialysis Center, Kobe University School of Medicine, Kobe, Japan
Background: The Kidney Disease Outcomes Quality Initiative (K/DOQI) Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease recommend 1.25 mmol/l Ca dialysate for both hemodialysis and peritoneal dialysis, while 1.5 mmol/l Ca dialysate has been used in our dialysis center. Methods: Therefore, we switched the dialysate calcium concentration from 1.5 – 1.25 mmol/l and observed the effects on serum calcium (S-Ca), phosphorus (S-P), 1-84 parathyroid hormone (whole PTH, w-PTH), bone-specific alkaline phosphatase (BAP), and tartrate-resistant acid phosphatase isoform 5b (TRACP-5b) for 6 months in 58 hemodialysis patients. Prescription of active vitamin D (VD) metabolites and Ca carbonate was increased in response to the changes in laboratory data. Results: Decrease of S-Ca was evident at 2 weeks and S-Ca remained low for 6 months. Transient elevation of S-P, which might be caused by stimulated bone resorption, was observed after the switch. In patients with low PTH (w-PTH less than 90 pg/ml before the switch), continuous increase of w-PTH, BAP, and TRACP-5b was observed. This appeared to be a favorable response because the risk of adynamic bone disease was high in this group of patients. On the other hand, acute elevation of the 3 parameters was well-controlled in patients with moderate and high PTH (w-PTH from 90 – 180 pg/ml, w-PTH more than 180 pg/ml, respectively) by increased dosage of active VD. Conclusion: These results demonstrate that 1.25 mmol/l Ca dialysate improved mineral metabolism by lowering S-Ca and releasing oversuppression of PTH. Our data also suggest that appropriate use of active VD could prevent acute rise of PTH.Correspondence to:
A. Fujimori, MD
Department of Artificial Kidney, Konan Hospital
1-5-16 Kamokogahara, Higashinada-ku,
Kobe 658-0064, Japan
Email: louie@kcc.zaq.ne.jp
Originals
Acute effects of hemodialysis on salivary flow rate and composition
Abstract
C.P. Bots, H.S. Brand, E.C.I. Veerman, M. Valentijn-Benz, Y.M.C. Henskens, R.M. Valentijn, P.F. Vos, J.A. Bijlsma, P.M. Ter Wee, B.M. Van Amerongen and A.V. Nieuw Amerongen
1Department of Oral Biochemistry, Academic Centre for Dentistry Amsterdam, Vrije Universiteit and Universiteit van Amsterdam, Amsterdam, 2Laboratory for Hematology, University Hospital Maastricht, 3Department of Internal Medicine, Rode Kruis Hospital, The Hague, 4Stichting Dianet Dialysis Centres, Utrecht, 5Stichting Dianet Dialysis Centres, Amsterdam, 6Department of Nephrology, Institute for Cardiovascular Research, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
Aims: To evaluate acute effects of hemodialysis (HD) on the salivary flow rate, pH and biochemical composition before, during and after completion of a dialysis session. Material and methods: Unstimulated whole saliva (UWS) and chewing-stimulated whole saliva (CH-SWS) were collected in 94 HD patients. Salivary flow rate, pH, concentrations of total protein, albumin, cystatin C, secretory immunoglobulin A (S-IgA) and of sodium, potassium and urea were measured. Results: HD had an acute stimulating effect on the salivary flow rate (UWSbefore = 0.30 ± 0.22 ml/min, UWSduring = 0.39 ± 0.25 ml/min, p < 0.005). The mean pH of UWS showed a small but significant increase during HD mainly due to an increased watery secretion from the salivary glands. The salivary biochemical constituents changed markedly, but no significant difference in output was found. The electrolyte concentration did not change significantly during dialysis. The level of urea in CH-SWS declined to 40% (Ureabefore = 25.6 ± 6.4 mmol/l, Ureaduring = 15.3 ± 4.5 mmol/l). Conclusions: This study shows that HD has significant acute effects on both salivary secretion rate and protein concentrations in saliva. We conclude that the observed changes in salivary concentrations and proteins are mainly due to an increased watery secretion from the salivary glands. Correspondence to:
Dr. C.P. Bots (DDS, PhD)
ACTA Oral Biochemistry
Van der Boechorststraat 7
1081 BT, Amsterdam, The Netherlands
Email: c.bots@vumc.nl
Case Reports
Idiopathic nodular glomerulosclerosis: three Japanese cases and review of the literature
Abstract
T. Kusaba, T. Hatta, K. Sonomura, Y. Mori, T. Tokoro, T. Nagata, Y. Umeda, K. Nagata, T. Yasuda, T. Sato, K. Kimura and H. Matsubara
1Division of Hypertension and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, 2Division of Internal Medicine II, Kansai Medical University, Osaka, 3Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa and 4Division of Cardiology and Vascular Regenerative Medicine, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan
Idiopathic nodular glomerulosclerosis (ING) is characterized as diffuse nodular glomerulosclerotic lesions, closely resembling Kimmelstiel-Wilson lesions without diabetic mellitus. We report here three Japanese cases of ING and discuss the previous reports. The patients were 75-, 48- and 84-year-old males with a history of long-term hypertension. Laboratory examination revealed moderate proteinuria and mild renal dysfunction. Diabetes mellitus was excluded by repeated clinical and laboratory investigations. Renal histology revealed nodular glomerulosclerosis, and both afferent and efferent arteriolosclerosis in all patients. In electron microscopy, the glomerular basement membrane was markedly thick in all patients. A low-protein diet and potent anti-hypertensive treatment using angiotensin-converting enzyme inhibitors were initiated in all patients and urinary protein excretion significantly reduced without the progression of renal dysfunction. We reviewed 42 previously reported cases and our three cases. The analysis revealed that common clinical features of ING are being male (82.2%) of relatively advanced age (mean age 61.3 years), with hypertension (82.2%), mild renal dysfunction (mean serum creatinine 2.9 mg/dl) and moderate urinary protein excretion (mean 4.05 g/day). Common histopathological findings of ING are nodular glomerulosclerosis (100%), arterio-arteriolosclerosis (91.2 and 89.7%) and glomerular basement membrane thickening (85.7%). In conclusion, ING is one of the phenotypes of arteriosclerotic renal disease without diabetes mellitus. Severe arterio-arteriolosclerosis may contribute to the progression to glomerular nodular formation in ING. The combination of renin-angiotensin system inhibition and a low protein diet can be beneficial for the reduction of urinary protein excretion.Correspondence to:
T. Kusaba, MD Division of Nephrology and Hypertension Department of Internal Medicine Kyoto Prefectural University of Medicine 456 Kajii-cho, Kamigyo-ku, Kyoto-city, 602-8566 Japan
Email: fwnk5760@mb.infoweb.ne.jp
Case Reports
Rheumatoid arthritis-induced pseudotumoral AA amyloidosis of the bladder with vesico-peritoneal fistula
Abstract
K. Hajji, L. Martin, J.-M. Devevey, Y. Tanter, E. Justrabo, G. Rifle and C. Mousson
Departments of 1Nephrology, Intensive Care, Transplantation,
2Pathology and 3Urology, Centre Hospitalier Universitaire, Dijon, France
Rheumatoid arthritis-induced AA amyloidosis of the bladder is rare, with fewer than 25 cases reported so far. This localization may be life-threatening with a mortality rate of about 60%, most often due to massive hematuria or multiorgan failure as a result of systemic amyloidosis. We report the case of a 72-year-old woman with a long history of rheumatoid arthritis who developed gross hematuria that induced severe anemia. Ultrasonography and tomodensitometry revealed a large mass localized in the upper part of the bladder. Cystoscopy showed a congestive inflammatory area with a large vesico-peritoneal fistula. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as AA amyloidosis. The amyloid deposits were also found in the rectum, duodenum, uterus and kidneys. This case of rheumatoid arthritis-induced AA amyloidosis of the bladder is characterized by its pseudotumoral aspect and the existence of a vesico-peritoneal fistula: only 2 cases have been reported so far. Treatment was symptomatic, and the patient died from cachexia. The pseudotumoral forms of AA amyloidosis, including amyloidosis of the bladder, deserve an early correct diagnosis. Otherwise, an incorrect diagnosis, especially cancer, may prompt inappropriate treatments.Correspondence to:
C. Mousson, MD
Department of Nephrology, Intensive Care, Transplantation
Hôpital du Bocage,
2 Boulevard de Lattre de Tassigny
21034 Dijon, France
Email: christiane.mousson@chu-dijon.fr
Case Reports
Renal complications in two patients with dentatorubral-pallidoluysian atrophy
Abstract
T. Morita, H. Kotani, M. Ishihara, K. Naruse, M. Fujieda, H. Wakiguchi and H. Ogura
1Department of Pediatrics, Kochi Medical School, Kochi University,
2Department of Pediatrics, National Kochi Hospital,
3Department of Pathology, National Kochi Hospital, Kochi, Japan
Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder characterized by various combinations of myoclonus epilepsy, ataxia, choreoathetosis and dementia. No specific therapy has been established and renal complication is rare. We report two cases of DRPLA with renal complications. Hematuria and proteinuria had gradually progressed for 2 and 13 years in these patients. Renal biopsy findings revealed focal glomerulosclerosis in one case and end-stage kidney disease in the other case. Angiotensin-converting enzyme inhibitor and angiotensin receptor II antagonist were administered to both patients, resulting in improved proteinuria and preserved renal function in one patient, while renal function continued to deteriorate in the other patient. Although renal complication is rare in patients with DRPLA, the presence of renal disease has to be suspected in patients with persistent proteinuria.Correspondence to:
M. Fujieda, MD
Department of Pediatrics
Kochi Medical School
Kochi University
Kohasu, Oko-cho, Nankoku, Kochi 783-8505, Japan
Email: fujiedam@kochi-u.ac.jp
Case Reports
Smoking, polyuria and impaired vision
Abstract
A. Granata, G. Viola, C. Privitera, G. Romeo, S. Cacciaguerra, M. Gaeta, E. Sicurezza and M. Figuera
1Department of Nephrology and Dialysis 2Department of Radiology,
Vittorio Emanuele Hospital, 3Department of Pediatric Surgery,
Nuovo Garibaldi Hospital, Catania, 4Department of Radiology,
Policlinico G. Martino, University of Messina, Messina, Italy
The pituitary gland can be involved in a variety of medical conditions, including metastatic tumors. Metastases to the pituitary gland, although absolutely rare, more commonly affect the posterior pituitary lobe and so frequently present with diabetes insipidus. We report on a 48-year-old male heavy smoker patient suffering from sudden onset of polyuria and persistent thirst. Laboratory results revealed central diabetes insipidus. Computed tomography (CT) scan of the brain showed a mass located in the sella turcica and in the suprasellar region. CT scan of the chest showed a mass in the right superior lobe with mediastinal lymphadenopathy, with bronchoscopy and biopsy features of pulmonary adenocarcinoma. The patient received radiotherapy on the pituitary gland and adjuvant chemotherapy, and as intrasellar and suprasellar mass decreased in size, urinary output was accordingly reduced. Therefore, is that in patients with risk factors for cancer and sudden onset of diabetes insipidus pituitary metastasis should be taken into account in differential diagnosis.Correspondence to:
Dr. A. Granata
MDVia F. Paradiso n 78/a
Acireale (CT), Italy
Email: a.granata@mail.gte.it
Case Reports
Acute renal infarction in a patient with left atrial myxoma
Abstract
K. Fujisaki, Y. Shohno, T. Yoshida, A. Ono, T. Mizumasa, H. Kondoh, I. Katsuragi, K. Ikeda, H. Kumagai, H. Meno, T. Nishid, Y. Tomita and R. Tominaga
1Division of Nephrology, 2Division of Cardiology, Fukuoka Red Cross Hospital, Fukuoka, 3Division of Cardiovascular Surgery,
Research Institute of Angiocardiology, Kyushu University, Fukuoka, Japan
A 24-year-old male first attended our hospital with acute onset of right flank pain radiating to the right lower quadrant of the abdomen. A contrast-enhanced computer tomography (CT) scan showed renal infarction, and he was admitted immediately for treatment. On admission, the right lower abdominal pain diminished gradually. On the second day in hospital, a left atrial echogenic mass was detected which filled the left atrial cavity; it appeared to be a left atrial myxoma measuring 3.9 ± 4.9 cm. The patient was immediately transferred and underwent emergency surgery. Histologic examination confirmed the diagnosis of myxoma. Post-operatively, he recovered well and was discharged from hospital without any further specific treatment.Correspondence to:
K. Fujisaki, MD
Department of Medicine and Clinical Science
Graduate School of Medical Sciences
Kyushu University
Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan
Email: kiichiro.fujisaki@nifty.ne.jp
Case Reports
Heart and renal failure in renovascular hypertension caused by giant cell arteritis - case report
Abstract
D.V. Kalimanovska-Ostric,S.P. Simic-Ogrizovic, B.M. Bonaci-Nikolic, V.D. Bozic, V.Z. Ostric and L.B. Davidoviç
1Institute for Cardiovascular Diseases, 2Institute for Urology and Nephrology, 3Institute for Allergy and Clinical Immunology, Clinical Center of Serbia, Belgrade, Serbia
We report a case of a male teenager with severe heart and acute renal failure as the dominant clinical manifestations of renovascular hypertension (RVH) caused by atypical giant cell arteritis (GCA). Unrecognized RVH and treatment of the consequent heart failure by angiotensin-converting enzyme inhibitors (ACEI) probably contributed to progression of renovascular disease to bilateral renal artery occlusion. Recurrent “flash” pulmonary edemas could not be prevented until surgical revascularization of the only functioning right kidney was achieved by an aortorenal bypass. Prompt post-operative normalization of heart function and arterial hypertension occurred despite the histopathological finding of the resected renal artery compatible with GCA and 4-year duration of significant renovascular disease. At the last check-up, the patient was asymptomatic, with normal arterial pressure on the prescribed treatment: carvedilol, hydrochlorothiazide, prednisolone 20 mg daily and aspirin. Subsequent follow-up is necessary to observe the evolution of GCA as an exceptionally rare cause of RVH.Correspondence to:
S. Simic-Ogrizovic, MD Institute of Urology and Nephrology Clinical Center of Serbia Pasterova 2 Belgrade 11 000, Serbia and Montenegro
Email: dst@eunet.yu
Letters to the Editor
Lupus nephritis after hepatitis B vaccination: an uncommon complication
Abstract
D. Santoro, M. Stella, G. Montalto and S. Castellino
1Unit of Nephrology and Dialysis, San Vincenzo Hospital, Taormina ASL 5, 2Unit of Pathology Cervello Hospital- Palermo
Correspondence to:
Dr. D. Santoro
Unit of Nephrology and Dialysis
San Vincenzo Hospital
Taormina ASL 5, Italy
Email: santisi@hotmail.com
Letters to the Editor
Isoniazid induced acute bilateral cerebellar syndrome in chronic kidney disease
Abstract
D. Bhowmik, H.S. Mahapatra, S. Mahajan and S.C. Tiwari
1Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India
Correspondence to:
D. Bhowmik, MD
Department of Nephrology
All India Institute of Medical Sciences
New Delhi 110029, India
Email: dmbhowmik@rediffmail.com
