Volume 77, No. 2/2012(February)
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 Clinical Nephrology
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Original
Serum fetuin-A levels increased following parathyroidectomy in uremic hyperparathyroidism
Chih-Chiang Wang, Yu-Juei Hsu, Chia-Chao Wu, Sung-Sen Yang, Giien-Shuen Chen, Shih-Hua Lin and Pauling Chu
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (89-96)
Serum fetuin-A levels increased following parathyroidectomy in uremic hyperparathyroidism
Chih-Chiang Wang1,2, Yu-Juei Hsu1, Chia-Chao Wu1, Sung-Sen Yang1, Giien-Shuen Chen1, Shih-Hua Lin1 and Pauling Chu1
1Division of Nephrology, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei and 2Division of Nephrology, Department of Medicine, Kaohsiung Armed Forces General hospital, Kaohsiung, Taiwan
Background: Both fetuin-A and hyperparathyroidism play crucial roles in vascular calcification (VC) and bone metabolism. However, the correlation between secondary hyperparathyroidism (SHPT), parathyroidectomy (PTX) and fetuin-A levels in dialysis patients has not yet been studied. Methods: For this study, we included 27 consecutive dialysis patients with severe SHPT who underwent total PTX with autotransplantation over a period of 2 years (from Oct 2006 to Sep 2008). Serum ionized calcium (iCa), phosphorus (Pi), bone-specific alkaline phosphatase (bAP), intact parathyroid hormone (iPTH), and fetuin-A were checked basally and 2, 7, 14, 30, and 60 days after PTX. Results: Two days after PTX, the iPTH, serum iCa, and Pi concentrations significantly decreased. Serum bAP levels gradually increased after PTX, peaked after 14 days (p < 0.05), and then gradually decreased. Serum fetuin-A levels significantly increased during the first 7 days after PTX, peaked 14 days after PTX (0.21 ± 0.05 vs. 0.35 ± 0.07 mg/ml, p < 0.05), and then remained at a stable level 60 days after PTX. There were significant correlations between percentage increase in serum fetuin-A levels and percentage decrease in serum iPTH levels 2 days and 7 days after PTX (r = 0.526, p < 0.01; r = 0.403, p < 0.05, respectively) and correlations between percentage increase in serum fetuin-A levels and percentage decrease in serum iCa levels 30 and 60 days after PTX (r = 0.449, p < 0.05; r = 0.474, p < 0.05, respectively). Conclusions: Serum fetuin-A significantly increased after PTX in uremic patients with SHPT. The percentage increase in serum fetuin-A after PTX was closely correlated with the percentage decrease in serum iPTH levels immediately after PTX, and with the percentage decrease in serum iCa levels in the later stage after PTX. Further investigations are necessary to further understand the regulation of fetuin-A in dialysis patients with sSHPT.Correspondence to:
Pauling Chu, MD, PhD
Division of Nephrology, Department of Medicine
Tri-Service General Hospital, 325, Section 2
Cheng-Kung Road, Neihu 114, Taipei, Taiwan
Email: pchu@seed.net.tw
Original
Is transiliac bone biopsy a painful procedure?
Maurice Audran, Emilie Maury, Béatrice Bouvard, Erick Legrand, Michel Felix Baslé and Daniel Chappard
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (97-104)
Is transiliac bone biopsy a painful procedure?
Maurice Audran1,2, Emilie Maury1, Béatrice Bouvard1,2, Erick Legrand1,2, Michel Felix Baslé2 and Daniel Chappard2
1Department of Bone Diseases, University Hospital, and 2INSERM, U922-LHEA “Remodelage Osseux et Biomatériaux” IRIS-IBS CHU d’Angers, Angers Cédex, France
Despite an increased availability of non-invasive procedures to assess bone mass, histological examination of undecalcified transiliac bone biopsies remains a very valuable tool in the diagnosis of metabolic or malignant bone disorders. Nonetheless, clinicians are sometimes reluctant to perform this “invasive” examination, arguing that it might be a painful procedure. The aim of our study was to evaluate pain and anxiety described by patients in the months following the biopsy and to characterize potential early or late side effects. A single interviewer conducted a phone survey (19 items questionnaire) in 117 patients in whom a bone biopsy had been performed by two experienced physicians, with the same material and similar anesthetic and technical procedure. The topics covered pain during or after the biopsy, anxiety, comparison of other potentially painful procedures, early or late side effects as well as global evaluation by the patients. Bone biopsy was judged as non-painful by almost 70% of patients; some discomfort was present in 25% in the following days. The procedure was described as similar as or less painful than bone marrow aspiration, venipuncture or tooth extraction. About 90% of the patients estimated that it was a quite bearable diagnostic procedure. Side effects were not serious. About 7% remembered a vasovagal episode, 47% of local bruising in the following days. There was no report of hematoma or infection. In experienced hands and adapted trephine, transiliac bone biopsy is a safe procedure that brings invaluable information in bone disorders.Correspondence to:
Prof. Maurice Audran
Department of Bone Diseases
CHU d’Angers
4 Rue Larrey
49993 Angers Cedex, France
Email: MaAudran@univ-angers.fr
Original
Impact of mean arterial pressure on progression of arterial stiffness in peritoneal dialysis patients under strict volume control strategy
Mettem Sezis Demirci, Ozkan Gungor, Fatih Kircelli, Juan Jesus Carrero, Erhan Tatar, Cenk Demirci, Meral Kayikcioglu, Gulay Asci, Huseyin Toz, Mehmet Ozkahya and Ercan Ok
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (105-113)
Impact of mean arterial pressure on progression of arterial stiffness in peritoneal dialysis patients under strict volume control strategy
Mettem Sezis Demirci1, Ozkan Gungor1, Fatih Kircelli1, Juan Jesus Carrero2, Erhan Tatar1, Cenk Demirci1, Meral Kayikcioglu3, Gulay Asci1, Huseyin Toz1, Mehmet Ozkahya1 and Ercan Ok1
1Ege University School of Medicine, Division of Nephrology, Izmir, Turkey, 2Divisions of Renal Medicine and Baxter Novum, Center for Gender Medicine and Center for Molecular Medicine, Karolinska Institute, Stockholm, Sweden, and 3Ege University School of Medicine, Division of Cardiology, Izmir, Turkey
Introduction: Arterial stiffness is an important contributor to the increased cardiovascular burden of uremia. The aim of the study was to identify determinants of arterial stiffness progression in peritoneal dialysis (PD) patients with strict volume control. Patients and methods: 89 prevalent PD patients were enrolled. Assessment of arterial stiffness was performed at baseline and after nine months on average (range 8 – 12 months) by carotid-femoral pulse wave velocity (cf-PWV). Results: Mean age was 51 ± 13 y; preceeding time on PD was 40 ± 34 months. 57% of the patients were men and 9% were diabetic. At baseline, mean cf- PWV was 8.7 ± 2.7 m/s and was significantly higher in patients with diabetes and on automated PD therapy. Cf-PWV was positively correlated with age, history of cardiovascular disease, mean arterial pressure (MAP), blood glucose, left atrium diameter and left ventricular mass index. Sixty patients underwent a second cf-PWV measurement. 36% had progression of arterial stiffness. Delta cf- PWV value was 2.08 ± 1.89 m/s for progressors and –1.25 ± 1.43 m/s; p < 0.01 for nonprogressors (p < 0.01). In logistic regression analysis, the change in MAP was the only predictor for progression of arterial stiffness. Conclusions: MAP is the main determinant of arterial stiffness progression. Our results suggest that efficient blood pressure control may contribute to preserved or reduced arterial stiffness in PD patients.Correspondence to:
Ozkan Gungor, MD
Ege University School of Medicine
Division of Nephrology
35100, Bornova, Izmir, Turkey
Email: ozkangungor@ yahoo.com.tr
Original
Duodenal biopsy for diagnosis of renal involvement in amyloidosis
Murvet Yilmaz, Abdulkadir Unsal, Mehmet Sokmen, Ozlem Harmankaya, Canan Alkim, Fevziye Kabukcuoglu and Aysim Ozagari
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (114-118)
Duodenal biopsy for diagnosis of renal involvement in amyloidosis
Murvet Yilmaz1, Abdulkadir Unsal2, Mehmet Sokmen3, Ozlem Harmankaya1, Canan Alkim4, Fevziye Kabukcuoglu5 and Aysim Ozagari5
1Nephrology Department, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, 2Nephrology Department, Sisli Etfal Training and Research Hospital, 3Gastroenterology Department, Umraniye Training and Research Hospital, 4Gastroenterology Department and 5Pathology Department, Sisli Etfal Training and Research Hospital, Istanbul, Turkey
Amyloidosis results from extracellular deposition of a fibrillary protein in various organs, and renal biopsy is the best, but a complicated tool for diagnosis. Therefore, alternative biopsy sites have been proposed with varying degrees of sensitivity. We aimed to find the most appropriate biopsy site in patients with chronic kidney disease (CKD) in whom renal biopsy is contraindicated or unavailable. 42 patients (29 male; mean age 46 ± 16 y) with CKD in whom amyloidosis was suspected as the underlying etiology on clinical grounds, but renal biopsy was not available (Group I), and 36 patients (25 male; mean age 40 ± 16 y) with CKD in whom renal biopsy revealed AA-amyloidosis (Group II) were investigated. Upper and lower gastrointestinal tract (GIT) endoscopies were performed and multiple biopsies from gingiva, esophagus, antrum, duodenum and rectum were obtained. In Group I, no amyloidosis was detected in gingival and GIT biopsies among 13 patients. In the remaining 29 patients AA-amyloidosis was detected in various sites with the following frequencies: duodenum 100%, rectum 83%, antrum 79%, esophagus 44% and gingiva 29%. In Group II, frequency of amyloid deposition was 97% in duodenum, 76% each in antrum and rectum, 59% in esophagus and 32% in gingival mucosa. In conclusion, duodenal biopsy is sensitive for diagnosing amyloidosis in CKD patients, and highly correlates with renal amyloidosis.Correspondence to:
Murvet Yilmaz, MD
Kartaltepe mah. Caglayan sok. No 9/5
Bakirkoy, Istanbul, Turkey
Email: murvetyilmaz@ hotmail.com
Original
Omega-3 fatty acids therapy for IgA nephropathy: a meta-analysis of randomized controlled trials
Lin-Lin Liu and Li-Ning Wang
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (119-125)
Omega-3 fatty acids therapy for IgA nephropathy: a meta-analysis of randomized controlled trials
Lin-Lin Liu and Li-Ning Wang
Department of Nephrology, The First Affiliated Hospital of China Medical University, Shenyang, People’s Republic of China
Background: The efficacy of omega-3 fatty acids (O3FA) in IgA nephropathy remains a controversial issue. The aim of the current updated meta-analysis is to assess the efficacy of O3FA treatment for adult IgA nephropathy. Methods: We searched PubMed/MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials for randomized controlled trials that compared O3FA treatments with placebo or no treatment in adult IgA nephropathy. Outcomes of interest were effects on urine protein excretion (UPE) and renal function. Results: Five RCTs (239 patients) were included for analysis. Compared with control groups, O3FA treatments did not show significant benefits for reducing UPE (standardized mean difference (SMD), –0.111; 95% confidence interval (CI), –0.369 – 0.147) or improving glomerular filtration rate (GFR) or estimated GFR (SMD, 0.177; 95% CI, –0.082 – 0.435), although the pooled results slightly favored O3FA. On the other hand, a lower risk of an increase of 50% or more in serum creatinine and ESRD were found in O3FA-treated IgA nephropathy patients (RR 0.189; 95% CI 0.068 – 0.524, p = 0.001; RR 0.236; 95% CI 0.094 – 0.594, p = 0.002), but the two outcomes were reported in only two trials. Conclusion: The current metaanalysis suggests that there are insufficient data to confirm the efficacy of O3FA treatments for proteinuria and renal function in IgA nephropathy. Further large scale trials are needed to shed more light on this issue.Correspondence to:
Li-Ning Wang
Department of Nephrology
The First Affiliated Hospital of China Medical University
155 Nanjing North Street, Heping District
Shenyang, 110001 China
Email: catherine_cmu@yahoo.com
Original
A multicenter, randomized trial of increased mycophenolic acid dose using enteric-coated mycophenolate sodium with reduced tacrolimus exposure in maintenance kidney transplant recipients
Nassim Kamar, Lionel Rostaing, Elisabeth Cassuto, Florence Villemain, Marie-Christine Moal, Marc Ladrière, Benoît Barrou, Didier Ducloux, Kamel Chaouche, Stephane Quéré, Fabienne Di Giambattista and François Berthoux
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (126-136)
A multicenter, randomized trial of increased mycophenolic acid dose using enteric-coated mycophenolate sodium with reduced tacrolimus exposure in maintenance kidney transplant recipients
Nassim Kamar1, Lionel Rostaing1, Elisabeth Cassuto2, Florence Villemain3, Marie-Christine Moal4, Marc Ladrière5, Benoît Barrou6, Didier Ducloux7, Kamel Chaouche8, Stephane Quéré8, Fabienne Di Giambattista8 and François Be
1Department of Nephrology, Dialysis and Organ Transplantation, Hôpital Rangueil, Toulouse, 2Department of Nephrology and Renal Transplantation, Hôpital Pasteur, Nice, 3Department of Nephrology, Dialysis and Renal Transplantation, CHU d’Angers, Angers, 4Department of Nephrology and Renal Transplantation, Hôpital La Cavale Blanche, Brest, 5Department of Nephrology and Renal Transplantation, Hôpital Brabois Adultes, Vandoeuvre les Nancy, 6Department of Urology, La Pitié- Salpêtrière University Hôpital, Paris, 7Department of Nephrology and Renal Transplantation, Hôpital Saint Jacques, Besançon, 8Novartis Pharma SAS, Rueil-Malmaison, and 9Department of Nephrology, Dialysis and Renal Transplantation, Hôpital Nord – CHU, Saint Étienne, France
Mycophenolic acid (MPA) dose is frequently reduced in tacrolimus-treated kidney transplant patients, but alternatively the recommended MPA dose can be maintained with reduced tacrolimus exposure. In a 6-month, multicenter, randomized, openlabel study, maintenance kidney transplant patients receiving MPA (mycophenolate mofetil 1g/d or enteric-coated mycophenolate sodium (EC-MPS) 720 mg/d) and tacrolimus were randomized to convert to EC-MPS 1,440 mg/d with reduced tacrolimus (n = 46), or receive EC-MPS 720 mg/d with unchanged tacrolimus (n = 48). Mean estimated GFR (eGFR, aMDRD) at Month 6 was 49.1 ± 11.1 and 44.7 ± 11.5 ml/min/1.73 m2 in the EC-MPS 1,440 mg and 720 mg groups, respectively (p = 0.07). The primary endpoint, change in eGFR from Day 0 to Month 6, was 2.48 ± 0.95 ml/min/1.73 m2 with EC-MPS 1,440 mg and –0.48 ± 0.93 ml/min/1.73 m2 with EC-MPS 720 mg (difference 2.96 ml/min/1.73 m2; 95% CI 0.32 – 5.60; p = 0.028). There were no deaths, graft losses or acute rejections. Adverse events were more frequent with EC-MPS 1,440 mg than 720 mg (66.7% vs. 44.7%, p = 0.034). Adverse events with suspected relation to EC-MPS occurred in 26.7% and 21.3% of patients, respectively (p = 0.59). Conversion of kidney transplant patients to increased MPA dosing using EC-MPS 1,440 mg/d, with reduced tacrolimus exposure, appears an effective immunosuppression strategy and may improve renal function. Adverse events overall, but not those with a suspected relation to EC-MPS, were higher with ECMPS 1,440 mg/d.Correspondence to:
Nassim Kamar, MD, PhD
Department of Nephrology, Dialysis and Organ
Transplantation
CHU-Rangueil, TSA 50032
31059 Toulouse CEDEX 9, France
Email: kamar.n@chu-toulouse.fr
Original
Impact of periodontal treatment in combination with tonsillectomy plus methylprednisolone pulse therapy and angiotensin blockade for pediatric IgA nephropathy
Chiyoko N. Inoue, Sachiko Matsutani, Masako Ishidoya, Rikako Homma, Yasushi Chiba and Takako Nagasaka
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (137-145)
Impact of periodontal treatment in combination with tonsillectomy plus methylprednisolone pulse therapy and angiotensin blockade for pediatric IgA nephropathy
Chiyoko N. Inoue1, Sachiko Matsutani2, Masako Ishidoya2, Rikako Homma2, Yasushi Chiba1 and Takako Nagasaka3
1Department of Pediatrics, 2Department of Otolaryngology, and 3Department of Dentistry, Red Cross Sendai Hospital, Sendai, Japan
Background: We previously reported the efficacy of extensive eradication of infectious foci in oral and ENT lesions, combined with tonsillectomy plus methylprednisolone (MP) pulse therapy, for curing pediatric Henoch-Schönlein purpura (HSP) and HSP nephritis. In the present study, we used this therapy in patients with pediatric IgA nephropathy (IgAN) to assess whether similar results could be obtained. Patients and methods: In 11 pediatric patients newly diagnosed with IgAN, exploration for infectious foci showed severe oral infection, including dental caries and apical periodontitis, in many. The overall decayed, missing and filled teeth score was elevated to 5.91. Two patients had rhinosinusitis. After extensive treatment of infectious foci, patients underwent tonsillectomy plus MP pulse therapy with angiotensin II receptor blockade. Results: Clinical remission was achieved in all patients with pediatric IgAN (various histologic grades). Remission was achieved by 7.2 ± 5.7 months after initiation of steroid therapy, and disappearance of proteinuria by 3.3 ± 3.0 months. The mean duration of oral steroid administration was 9.5 ± 3.6 months. No relapse has occurred during follow-up of 4.3 ± 2.4 y. Conclusions: Careful examination and thorough elimination of infectious foci in oral and ENT lesions can optimize the effect of tonsillectomy plus MP pulse therapy, promoting recovery from IgAN.Correspondence to:
Chiyoko N. Inoue, MD, PhD
Department of Pediatrics
Red Cross Sendai Hospital
2-43-3 Taihaku-ku, Yagiyamahoncho,
Sendai 982-8501, Japan
Email: chnagano@ sendai.jrc.or.jp
Nephrology Education
IgA nephropathy in a patient with ulcerative colitis, Graves’ disease and positive myeloperoxidase ANCA
Elaine Ku, Wanwarat Ananthapanyasut and Vito M. Campese
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (146-150)
IgA nephropathy in a patient with ulcerative colitis, Graves’ disease and positive myeloperoxidase ANCA
Elaine Ku, Wanwarat Ananthapanyasut and Vito M. Campese
Department of Medicine, Division of Nephrology, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
We report a case of a 38-yearold woman with a history of ulcerative colitis and Graves’ disease who presented with pyoderma gangrenosum, microscopic hematuria, proteinuria, and positive myeloperoxidase ANCA. A renal biopsy revealed a focal proliferative glomerulonephritis with IgA deposits. All these manifestations are likely secondary to ulcerative colitis or to a common pathogenetic mechanism.Correspondence to:
Vito M. Campese, MD
Professor of Medicine and Chief of Nephrology
University of Southern California
LAC + USC Medical Center, Division of Nephrology
2020 Zonal Avenue, Room 802
Los Angeles, CA 90033, USA
Email: campese@usc.edu
Nephrology Education
Peculiar renal endarteritis in a patient with acute endocapillary proliferative glomerulonephritis
Takehiko Kawaguchi, Asami Takeda, Yoshiaki Ogiyama, Yukako Yamauchi, Minako Murata, Taisei Suzuki, Yasuhiro Otsuka, Keiji Horike, Daijo Inaguma and Kunio Morozumi
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (151-155)
Peculiar renal endarteritis in a patient with acute endocapillary proliferative glomerulonephritis
Takehiko Kawaguchi, Asami Takeda, Yoshiaki Ogiyama, Yukako Yamauchi, Minako Murata, Taisei Suzuki, Yasuhiro Otsuka, Keiji Horike, Daijo Inaguma and Kunio Morozumi
Department of Nephrology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan
Acute glomerulonephritis (AGN) is one of the most common renal diseases. They are often associated with infections and can result in diffuse proliferative glomerulonephritis (GN). This case report reviews an interesting case in which renal endarteritis coexisted in AGN with diffuse endocapillary proliferation. The discussion highlights important pathological findings and clinical aspects in acute endocapillary proliferative GN with renal endarteritis. Coexisting endarteritis should be in the differential diagnosis of AGN in patients with persistent clinical courses.Correspondence to:
Takehiko Kawaguchi, MD, MPH, PhD
Department of Nephrology
Japanese Red Cross
Nagoya Daini Hospital
2-9 Myokencho, Showa-ku,
Nagoya, 466-8650 Japan
Email: kawatake45@gmail.com
Nephrology Education
Renal actinomycosis with concomitant renal vein thrombosis
Dong-Suk Chang, Won Ik Jang, Ji Yoon Jung, Sarah Chung, Dae Eun Choi, Ki-Ryang Na, Kang Wook Lee and Yong-Tai Shin
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (156-160)
Renal actinomycosis with concomitant renal vein thrombosis
Dong-Suk Chang, Won Ik Jang, Ji Yoon Jung, Sarah Chung, Dae Eun Choi, Ki-Ryang Na, Kang Wook Lee and Yong-Tai Shin
Internal Medicine, Chungnam National University Hospital, Daejeon, South Korea
Renal actinomycosis is a rare infection caused by fungi of the genus Actinomyces. A 74-year-old male was admitted to our hospital because of gross hematuria with urinary symptoms and intermittent chills. Computed tomography of the abdomen showed thrombosis in the left renal vein and diffuse, heterogeneous enlargement of the left kidney. After nephrectomy, sulfur granules with chronic suppurative inflammation were seen microscopically, and the histopathological diagnosis was renal actinomycosis. Our case is the first report of renal actinomycosis with renal vein thrombosis.Correspondence to:
Kang Wook Lee
Internal Medicine
Chungnam National University Hospital
(301-721) 640 Daesadong Chunggu Daejeon, South Korea
Email: kwlee@cnu.ac.kr
Nephrology Education
Hereditary renal hypouricemia: a cause of calcium oxalate urolithiasis in a young female
Naoto Nishizaki, Shuichiro Fujinaga, Daishi Hirano, Hiroaki Kanai, Hitoshi Kaya, Yoshiyuki Ohtomo, Toshiaki Shimizu, Kandai Nozu and Kazunari Kaneko
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (161-163)
Hereditary renal hypouricemia: a cause of calcium oxalate urolithiasis in a young female
Naoto Nishizaki1, 2, Shuichiro Fujinaga1, Daishi Hirano1, Hiroaki Kanai1, Hitoshi Kaya3, Yoshiyuki Ohtomo4, Toshiaki Shimizu5, Kandai Nozu6 and Kazunari Kaneko7
1Division of Nephrology, Saitama Children’s Medical Center, Saitama, 2Department of Neonatology, Juntendo University Shizuoka Hospital, Shizuoka, 3Division of Urology, Kawaguchi Municipal Medical Center, Saitama, 4Department of Pediatrics, Juntendo University Nerima Hospital, 5Department of Pediatrics, Juntendo University School of Medicine, Tokyo, 6Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, and 7Department of Pediatrics, Kansai Medical University, Osaka, Japan
Although renal hypouricemia is mostly asymptomatic, it is known to present a high risk of exercise-induced acute renal failure, especially in young males. However, there is little information regarding the clinical features of urolithiasis as a complication in childhood renal hypouricemia. Here we report a 4-yearold female with idiopathic renal hypouricemia who presented with macroscopic hematuria due to obstructive calcium oxalate urolithiasis. She was treated successfully with percutaneous nephrolithotripsy and thereafter hematuria disappeared. Sequence analysis of the patient and her family’s URAT1 gene confirmed a nonsense mutation in exon 4 (W258X). To the best of our knowledge, this is the youngest case of hereditary renal hypouricemia caused by URAT1 gene mutation, which was found by hematuria due to calcium oxalate urolithiasis.Correspondence to:
Shuichiro Fujinaga, MD
Division of Nephrology
Saitama Children’s Medical Center
2100 Magome, Iwatsuki-ward,
Saitama-city, Saitama 339 8551, Japan
Email: f_shuich@d2.dion.ne.jp
Nephrology Education
Thrombotic microangiopathic nephropathy, pulmonary hypertension and nephromegaly: case report of a patient treated with endothelin receptor antagonist
Attilio Losito, Loretta Pittavini and Carla Covarelli
Abstract
Clinical Nephrology, Vol. 77 – No. 2/2012 (164-170)
Thrombotic microangiopathic nephropathy, pulmonary hypertension and nephromegaly: case report of a patient treated with endothelin receptor antagonist
Attilio Losito1, Loretta Pittavini1 and Carla Covarelli2
1Struttura Complessa Nefrologia e Dialisi and 2Struttura Complessa Anatomia Patologica, Ospedale Santa Maria della Misericordia, Perugia, Italy
The coexistence of thrombotic microangiopathic nephropathy and pulmonary hypertension has only been described in association with malignancy and its treatment. Here we describe a 14-year-old boy with no prior medical history who presented with hypertension, proteinuria and nephromegaly, and then developed progressive pulmonary hypertension. Renal histology showed lesions consistent with glomerulopathy due to thrombotic microangiopathy (TMA). Pulmonary hypertension was controlled by the use of an oral endothelin receptor antagonist (bosentan). Although renal function deteriorated at the onset of pulmonary hypertension, an improvement was observed after the bosentan treatment. Nephromegaly persisted, but current creatinine clearance values are within the normal range. While this case exemplifies how thrombotic microangiopathic nephropathy may be associated with pulmonary hypertension, a therapeutic role of endothelin antagonists is suggested, not only for pulmonary hypertension but also for microangiopathic nephropathy.Correspondence to:
Dr. Attilio Losito
Via dei Mille 5, San Mariano
06070 Perugia, Italy
Email: alosito3@gmail.com
