Volume 74 (2010), No. 5/2010(November)
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Lead Article
Erythropoietin production by distal nephron in normal and familial amyloidotic adult human kidneys
I. Beirão, L. Moreira, T. Barandela, L. Lobato, P. Silva, C.M. Gouveia, F. Carneiro, I. Fonseca, G. Porto and P. Pinho e Costa
Page No. 327
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (327-335)
Erythropoietin production by distal nephron in normal and familial amyloidotic adult human kidneys
I. Beirão1,2,3*, L. Moreira2*, T. Barandela2,3, L. Lobato1,2, P. Silva4, C.M. Gouveia5, F. Carneiro4, I. Fonseca1, G. Porto3,6 and P. Pinho e Costa2,3
1Nephrology, Santo António Hospital, CHP, 2Genetics Department, INSA Dr. Ricardo Jorge, 3UMIB, Abel Salazar Biomedical Sciences Institute, 4Anatomy Service, S. João Hospital, 5Anatomy Service, Santo António Hospital, and 6Hematology, Santo António Hospital, Porto, Portugal
Background/Aim: The kidney is the major site of erythropoietin production. Many efforts have been made to identify renal erythropoietin-producing cells. Previous studies showed conflicting results, but the predominant localization reported was the peritubular interstitial and tubular epithelial cells. This study was conducted to identify the erythropoietin-producing cells in renal biopsies from 10 cadaveric donors and 45 patients with familial amyloidosis ATTR V30M, thirteen of them with anemia. Familial amyloidosis Type I (FAP-I) is a genetic disorder caused by a transthyretin (TTR) protein variant presenting a single amino acid substitution of methionine for valine at position 30 of the polypeptide chain (TTR V30M). Anemia in FAP-I is associated with inappropriately low serum erythropoietin levels. Methods: Erythropoietin expression was detected by in situ hybridization (ISH) and confirmed by laser capture microdissection followed by PCR. Renal segments were identified by immunohistochemistry. Results: Erythropoietin was mainly expressed by epithelial distal tubular cells and collecting tubules and additionally, in a few biopsies, by glomerular cells. A similar expression pattern was observed in donors and FAP-I patients. No increased mRNA erythropoietin expression was found in anemic patients, all of them presenting only a slight expression in medulla and cortex. Conclusions: These results suggest the distal nephron as the major site of erythropoietin production, and support the notion that an inappropriate erythropoietin production is the cause of anemia in familial amyloidosis ATTR V30M.Correspondence to:
I. Beirão, MD
Serviço de Nefrologia
Hospital de Santo António
Rua D. Manuel II
4050-345, Porto, Portugal
Email: [email protected]
Original
Nutritional markers, not markers of bone turnover, are related predictors of bone mineral density in chronic peritoneal dialysis patients
J.U. Jeong, H.K. Lee, Y.J. Kim, J.S. Kim, S.S. Kang and S.B. Kim
Page No. 336
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (336-342)
Nutritional markers, not markers of bone turnover, are related predictors of bone mineral density in chronic peritoneal dialysis patients
J.U. Jeong1, H.K. Lee1, Y.J. Kim1, J.S. Kim2, S.S. Kang3 and S.B. Kim1
1Division of Nephrology, Department of Internal Medicine, 2Department of Nuclear Medicine, and 3Department of Dietetics and Nutrition Service Team, Asan Medical Center, University of UIsan College of Medicine, Seoul, South Korea
Background and aims: This study evaluated the factors associated with bone mineral density (BMD) in chronic peritoneal dialysis (CPD) patients. Methods: In this cross-sectional study in 91 stable CPD patients, BMD was measured using dual-energy X-ray absorptiometry. Markers of bone turnover (iPTH, osteocalcin, bone alkaline phosphatase, serum C-telopeptide), 25-hydroxy (OH) vitamin D3 and nutritional markers (prealbumin, nPNA, BMI) were measured by standard techniques. Results: Of the 91 patients, 48 were female and 22 (24%) had Type 2 diabetes. Mean age of the patients was 52.7, and patients had been on PD for about 44 months. For the lumbar spine (LS) and femoral neck (FN), the mean T-scores were –1.19 ± 1.53 and –1.24 ± 1.01, respectively, and the mean Z-scores were –0.78 ± 1.33 and –0.40 ± 0.92, respectively. Using the WHO-based criteria, osteopenia (–2.5 < T-score < –1.0) at the LS and FN was observed in 37 (41%) and 48 (53%) patients, respectively, and osteoporosis (T-score ≤ –2.5) at the LS and FN was observed in 15 (17%) and 6 (7%) patients, respectively. LS T-score was positively correlated with BMI (r = 0.40, p < 0.001) and albumin (r = 0.29, p = 0.005), and FN T-score was positively correlated with albumin (r = 0.40, p < 0.001), prealbumin (r = 0.38, p < 0.001), age (r = –0.32, p = 0.002) and BMI (r = 0.29, p < 0.006). Markers of bone turnover were not associated with BMD. In multiple linear regression models, independent predictors of FN T-score were age, BMI, albumin and prealbumin (r2 = 0.259, F = 6.23, p < 0.001), whereas BMI was the only independent predictor of LS T-score (r2 = 0.24, F = 6.63, p < 0.001). Conclusion: Nutritional markers, not markers of bone turnover, are correlated predictors of BMD in CPD patients.Correspondence to:
S.B. Kim, MD, Ph
388-1, Pungnap-dong, Songpa-gu, Seoul, Republic of Korea
Email: [email protected]
Original
Relationship between carnosinase gene CNDP1 leucine repeat polymorphism and the clinical outcome of Chinese PD patients
P.Y.-K. Poon, C.-C. Szeto, B.C.-H. Kwan, K.-M. Chow and P.K.-T. Li
Page No. 343
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (343-345)
Relationship between carnosinase gene CNDP1 leucine repeat polymorphism and the clinical outcome of Chinese PD patients
P.Y.-K. Poon, C.-C. Szeto, B.C.-H. Kwan, K.-M. Chow and P.K.-T. Li
Departments of Medicine & Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong, China
Background: Pre-clinical studies showed that carnosine may have a beneficial cardiovascular effect. We studied the effect of tri-nucleotide repeat (CTGs) polymorphisms in exon 2 of the CNDP1 gene, which codes for carnosinase and is responsible for the degradation of carnosine, on the clinical outcome of Chinese peritoneal dialysis (PD) patients. Methods: We studied 442 PD subjects. Genotyping was done by direct sequencing of genomic DNA. Patients were followed for 43.5 ± 16.2 months. Results: The prevalence of 6-6, 5-6, 5-5 and 4-6 CTGs genotypes was 80.3%, 18.6%, 0.9% and 0.2%, respectively. A total of 270 patients (61.1%) developed the primary composite end point during follow-up. The 5-year event-free survival of the 6-6 CTGs and non 6-6 group was 37.1% and 21.3%, respectively (log rank test, p = 0.3). Conclusion: The CTGs polymorphism of the CNDP1 gene does not affect survival of Chinese PD subjects. The role of carnosine and CNDP1 gene polymorphism in the pathogenesis of cardiovascular disease requires further study.Correspondence to:
Dr. C.C. Szeto
Department of Medicine & Therapeutics
Prince of Wales Hospital
Chinese University of Hong Kong
Shatin, N.T., Hong Kong, China
Email: [email protected]
Original
Hemodialysis patients’ experience with and attitudes toward the buttonhole technique for arteriovenous fistula cannulation
A. Hashmi, M.Q. Cheema and A.H. Moss
Page No. 346
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (346-350)
Hemodialysis patients’ experience with and attitudes toward the buttonhole technique for arteriovenous fistula cannulation
A. Hashmi, M.Q. Cheema and A.H. Moss
Section of Nephrology, Department of Medicine, West Virginia University School of Medicine (AH,MQC, AHM), and the Center for Health Ethics and Law (AHM), Robert C. Byrd Health Sciences Center, West Virginia University, Morgantown, WV, USA
Background: The buttonhole technique for arteriovenous fistula cannulation has been recommended as an alternative to the rope ladder technique. Aim: We compared patients’ experiences and hypothesized that patients would prefer the buttonhole technique. Methods: In an observational cross-sectional study using a 23-item questionnaire, we surveyed hemodialysis patients at 3 dialysis centers who had previously used the rope ladder technique and were presently using the buttonhole technique for their experiences with and attitudes toward the two techniques. Twelve months later, patients’ attitudes were reassessed. Results: 26 patients met inclusion criteria. On a scale of 0 (not at all satisfied) to 10 (very satisfied), patients reported a mean satisfaction with the buttonhole technique of 8.5 ± 1.9. 96% of patients said they would recommend it, and 83% reported that the buttonhole technique was easier for the nurses to use. Fewer patients reported fistula infiltration with the buttonhole technique (12% vs. 59%, p = 0.008). Twelve months later, 18 of 23 patients (78%) still dialyzing in the study units remained satisfied with the buttonhole technique. Conclusions: Patients reported a high level of satisfaction, fewer complications, and a positive attitude about suggesting the buttonhole technique to other patients. Our findings support the recommendation for its more widespread use.Correspondence to:
A.H. Moss, MD
Center for Health Ethics and Law
West Virginia University School of Medicine
PO Box 9022
Morgantown, WV 26506-9022, USA
Email: [email protected]
Original
A single-center experience of preemptive anticoagulation for patients with risk factors for allograft thrombosis in renal transplantation
M. Murashima, B.A. Konkle, R.D. Bloom, S.L. Sood, R.A. Grossman, S.M. Brunelli and S.H. Stein
Page No. 351
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (351-357)
A single-center experience of preemptive anticoagulation for patients with risk factors for allograft thrombosis in renal transplantation
M. Murashima1, B.A. Konkle2, R.D. Bloom1, S.L. Sood2, R.A. Grossman1, S.M. Brunelli3 and S.H. Stein2,4
1Renal Electrolyte and Hypertension Division, 2Hematology and Oncology Division, Hospital of the University of Pennsylvania, PA, 3Renal Division, Brigham and Women’s Hospital, Boston, MA, and 4Oncology, GlaxoSmithKline, Collegeville, PA, USA
Aims: To review our single-center experience of preemptive anticoagulation for the prevention of allograft thrombosis in patients with hypercoagulable states. Material and methods: This is a retrospective cohort study. Included subjects were first-time kidney allograft recipients transplanted between 2003 and 2007 at a single center, with hypercoagulable states: prior venous thromboembolism, multiple vascular access thromboses, or identifiable thrombophilia. The predictor variable was preemptive anticoagulation and outcome variable was allograft thrombosis. Other risk factors for allograft thrombosis, characteristics of transplantation, and hemorrhagic complications were also examined. Results: Among this high-risk cohort (n = 48), 16 received preemptive anticoagulation and 32 did not. The anticoagulated group included significantly more subjects with identifiable thrombophilia (50.0% vs. 0%; p < 0.001). One subject (6.3%) in the anticoagulated group and 6 (18.8%) without anticoagulation developed allograft thrombosis (p = 0.40). A perinephric hematoma was observed in 5 (31.3%) and 2 (6.3%) with and without anticoagulation, respectively (p = 0.03). Conclusions: Preemptive anticoagulation was associated with a non-significant trend towards decreased allograft thrombosis. It may be associated with increased risk of hemorrhage and should be considered cautiously in high-risk patients.Correspondence to:
M. Murashima, MD
Renal Electrolyte and Hypertension Division
Hospital of the University of Pennsylvania
3400 Spruce Street, Philadelphia, PA 19104, USA
Email: [email protected]
Original
Comparison of estimated glomerular filtration rate (eGFR) using the MDRD and CKD-EPI equations for CKD screening in a large population
D. Giavarina, D.N. Cruz, G. Soffiati and C. Ronco
Page No. 358
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (358-363)
Comparison of estimated glomerular filtration rate (eGFR) using the MDRD and CKD-EPI equations for CKD screening in a large population
D. Giavarina1, D.N. Cruz2, G. Soffiati1 and C. Ronco2
1Dipartimento di Patologia Clinica, and 2Dipartimento Interaziendale di Nefrologia, Dialisi e Trapianto Renale, Ospedale San Bortolo, Vicenza, Italy
Background/Aims: Recently, the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) proposed a new equation for estimating glomerular filtration rate (eGFR), which could potentially replace the Modified Diet for Renal Disease Study (MDRD) equation in routine clinical use. Our aim was to evaluate the correlation between them and to compare the prevalence of each CKD stage using these two equations. Methods: We measured serum creatinine in 38,188 consecutive patients and calculated eGFR using the CKD-EPI and MDRD equations. We also compared the distribution of CKD stages for both equations. Results: There was very good correlation between eGFR estimated by CKD-EPI and MDRD at values < 60 ml/min × 1.73 m2, but not at higher values. Estimated prevalence of CKD (eGFR < 60 ml/min × 1.73 m2) was 5.9% with CKD-EPI and 7.5% with MDRD. Furthermore, the prevalence of CKD Stage 2 was lower with CKD-EPI (33.8% vs. 49.1%. with MDRD). Conclusion: The use of the CKD-EPI equation results in a lower estimated prevalence of CKD, compared to the MDRD equation. This may have important implications for public health and clinical practice, as well as for future modification of guidelines for laboratories.Correspondence to:
Dr. D. Giavarina
Laboratorio di Chimica ed Ematologia
Viale Rodolfi 37
36100 Vicenza, Italy
Email: [email protected]
Original
Gastrointestinal sensitivity to soy and milk proteins in patients with IgA nephropathy
H. Kloster Smerud, B. Fellström, R. Hällgren, S. Osagie, P. Venge and G. Kristjánsson
Page No. 364
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (364-371)
Gastrointestinal sensitivity to soy and milk proteins in patients with IgA nephropathy
H. Kloster Smerud1,5, B. Fellström1, R. Hällgren2, S. Osagie1,6, P. Venge3 and G. Kristjánsson4,7
1Departments of Nephrology, 2Rheumatology, 3Clinical Chemistry, 4Gastroenterology, Department of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden, 5Smerud Medical Research International AS, Oslo, Norway, 6Current address: Department of Internal Medicine, Nyköping Hospital, Nyköping, Sweden and 7Current address: Department of Internal Medicine, University Hospital of Akureyri, Akureyri, Iceland
Background: Sensitivity to food antigens has been postulated as a contributing factor to the pathogenesis of IgA nephropathy (IgAN). Methods: In this study we used a recently developed mucosal patch technique to evaluate rectal mucosal sensitivity to soy and cow’s milk (CM) proteins in IgAN patients (n = 28) compared to healthy subjects (n = 18). The rectal mucosal production of nitric oxide (NO) and release of myeloperoxidase (MPO) and eosinophil cationic protein (ECP) were measured. Serum samples were analyzed for IgA and IgG antibodies to α-lactalbumin, β-lactoglobulin, casein and soy. Results: 14 of 28 (14/28) patients experienced a rectal mucosal reaction, measured by increased NO and/or MPO levels, upon rectal challenge with soy and/or cow’s milk proteins. The levels of IgG antibodies to α-lactalbumin, β-lactoglobulin and casein were significantly higher in CM sensitive as compared with non-sensitive IgAN patients, whereas the mean serum levels of IgA antibodies were similar. No differences were seen in serum levels of IgA or IgG antibodies to soy. Conclusion: It is concluded that approximately half of our IgAN patients have a rectal mucosal sensitivity to soy or CM, and that an immune reactivity against antigens may be involved in the pathogenesis of IgAN in this subgroup of patients.Correspondence to:
H. Kloster Smerud, MscPharm
Uppsala University Hospital
75185 Uppsala, Sweden
Email: [email protected]
Original
The changing pattern of primary glomerulonephritis in Singapore and other countries over the past 3 decades
K.-T. Woo, C.-M. Chan, C.Y. Mooi, H.-L- Choong, H.-K. Tan, M. Foo, G.S.L. Lee, V. Anantharaman, C.-H. Lim, C.-C. Tan, E.J.C. Lee, G.S.C. Chiang, P.H. Tan, T.H. Boon, S. Fook-Chong and K.-S. Wong
Page No. 372
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (372-383)
The changing pattern of primary glomerulonephritis in Singapore and other countries over the past 3 decades
K.-T. Woo1, C.-M. Chan1, C.Y. Mooi1, H.-L- Choong1, H.-K. Tan1, M. Foo1, G.S.L. Lee1, V. Anantharaman1, C.-H. Lim1, C.-C. Tan2, E.J.C. Lee2, G.S.C. Chiang3, P.H. Tan3, T.H. Boon1, S. Fook-Chong4 and K.-S. Wong1
1Department of Renal Medicine, Singapore General Hospital, 2Department of Nephrology, National University of Singapore, 3Department of Pathology, and 4Department of Clinical Research, Singapore General Hospital, Singapore
This review of 2,586 renal biopsies over the past 3 decades in Singapore documents the changing pattern of glomerulonephritis (GN) from that of a third world country to that of a developed nation. In the 1st decade, mesangial proliferative glomerulonephritis was the most common form of primary GN, just as it was in the surrounding Asian countries. In the 2nd decade, the prevalence of mesangial proliferative GN decreased with a rise in membranous, GN which is also seen in China and Thailand. In the 3rd decade, there was a dramatic increase in focal sclerosing glomerulosclerosis. This increase reflects aging and obesity in keeping with more developed countries like Australia, India, Thailand and the United States of America. IgA nephritis remains the most common GN. Apart from the geographical influence, other socioeconomic factors play a significant role in the evolution of the renal biopsy pattern. Mesangial proliferative GN remains prevalent in many Asian countries, but in Singapore the prevalence is decreasing just as it is in Japan, Korea and Malaysia. Worldwide, the prevalence of focal sclerosing glomerulosclerosis continues to increase in many countries.Correspondence to:
Prof. K.-T. Woo
Department of Renal Medicine
Singapore General Hospital
Outram Road, Singapore 169608
Email: [email protected]
Case Report
Resolution of mesangial light chain deposits 3 years after high-dose melphalan with autologous peripheral blood stem cell transplantation
K. Harada, Y. Akai, H. Sakan, Y. Yamaguchi, K. Nakatani, M. Iwano and Y. Saito
Page No. 384
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (384-388)
Resolution of mesangial light chain deposits 3 years after high-dose melphalan with autologous peripheral blood stem cell transplantation
K. Harada, Y. Akai, H. Sakan, Y. Yamaguchi, K. Nakatani, M. Iwano and Y. Saito
First Department of Internal Medicine, Nara Medical University, Nara, Japan
A 52-year-old woman was admitted to our hospital for treatment of nephrotic syndrome. Funduscopic findings showed fundal hemorrhage and soft exudates, and serologic analysis showed a monoclonal serum component that was identified as Bence Jones protein-k type. A bone marrow biopsy showed diffuse proliferation of atypical plasma cells, while a renal biopsy showed diffuse and nodular mesangial proliferation. Immunohistochemical staining confirmed the presence of k chains along the glomerular basement membrane and in mesangial areas. The patient was diagnosed as multiple myeloma (Bence Jones k type) with light chain deposition disease (LCDD). After high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT), the multiple myeloma and nephrotic syndrome were in complete remission; her renal function was improved, but a renal biopsy performed 6 months after PBSCT showed the persistence of diffuse and nodular lesions. By contrast, a renal biopsy performed 3 years later showed complete resolution of the diffuse and nodular mesangial proliferation.Correspondence to:
M. Iwano, MD
First Department of Internal Medicine
Nara Medical University
840 Shijo, Kashihara, Nara 634-8522 Japan
Email: [email protected]
Case Report
Acute neurological presentation due to copper deficiency in a hemodialysis patient following gastric bypass surgery
E. Rounis, C.M. Laing and A. Davenport
Page No. 389
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (389-392)
Acute neurological presentation due to copper deficiency in a hemodialysis patient following gastric bypass surgery
E. Rounis, C.M. Laing and A. Davenport
UCL Center for Nephrology, Royal Free Campus, University College London Medical School, London, UK
Acquired copper deficiency has been recently recognized as a cause of myelopathy, and has been reported to occur many years after gastric bypass surgery performed to aid weight reduction in morbidly obese patients. We report a case of a young woman treated by hemodialysis who presented with acute neurological symptoms 5 months after gastric bypass surgery for severe obesity. She had symptoms and signs of cerebellar, spinal cord and peripheral nerve disease, which improved following parenteral copper supplementation. Now that gastric bypass surgery is being offered to morbidly obese hemodialysis patients, this case highlights the importance of monitoring copper levels in hemodialysis patients following gastric bypass surgery.Correspondence to:
A. Davenport, MD
UCL Center for Nephrology
Royal Free Campus
University College London Medical School
Rowland Hill Street, London NW3 2PF, UK
Email: [email protected]
Case Report
Histopathological manifestations of membranoproliferative glomerulonephritis and glomerular expression of plasmalemmal vesicle-associated protein-1 in a patient with polycythemia vera
Y. Nishi, T. Namikoshi, T. Sasaki, T. Tokura, H. Nagasu, H. Nakanishi, Y. Kozuka and N. Kashihara
Page No. 393
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (393-398)
Histopathological manifestations of membranoproliferative glomerulonephritis and glomerular expression of plasmalemmal vesicle-associated protein-1 in a patient with polycythemia vera
Y. Nishi1, T. Namikoshi1, T. Sasaki1, T. Tokura1, H. Nagasu1, H. Nakanishi2, Y. Kozuka3 and N. Kashihara1
Divisions of 1Nephrology, 2Hematology, Department of Internal Medicine and Department of 3Pathology, Kawasaki Medical School, Kurashiki, Japan
Only a few cases of various glomerulonephropathies have been reported in patients with polycythemia vera. We report the case of a 72-year-old female with polycythemia vera in whom renal biopsy examination showed membranoproliferative glomerulonephritis (MPGN)-like lesion and glomerular expression of plasmalemmal vesicle-associated protein-1 (PV-1), a marker of glomerular capillary remodeling after injury. Prior to admission to our hospital for nephrotic syndrome, she had received hydroxyurea and phlebotomy. On admission, she was hypertensive with pretibial edema, hepatosplenomegaly, massive proteinuria (6.14 g/day), low serum albumin (2.9 g/dl), high fibrinogen, fibrin/fibrinogen degradation products and thrombomodulin levels, but with normal serum creatinine and complement levels. Microscopic examination of a renal biopsy demonstrated MPGN-like features with double contour and mesangial interposition. Electron microscopy showed subendothelial deposits, platelets attached to glomerular capillary walls and fibrin deposition. Immunofluorescence study identified IgM deposition along part of the capillary wall and mesangium. CD42b-positive platelets and megakaryocytes were detected in glomerular capillaries, accompanied with increased expression of platelet-derived growth factor receptor b and thrombomodulin in the mesangium and glomerular capillary, respectively. PV-1 was expressed along the glomerular capillary. Anti-platelet and anticoagulant combination therapy, together with the use of anti-hypertensive agents and hydroxyurea, resulted in improvement of the nephrotic syndrome. The findings suggested that activated platelets, enhanced coagulation state and endothelial damage may contribute to glomerulonephropathy associated with polycythemia vera.Correspondence to:
T. Namikoshi, MD
Division of Nephrology
Department of Internal Medicine
Kawasaki Medical School
577 Matsushima, Kurashiki 701-0192, Japan
Email: [email protected]
Case Report
An 8-month-old boy with congenital fibromuscular dysplasia presenting with shock caused by sudden renal hemorrhage
K. Takano, Y. Kawasaki, T. Imaizumi, S. Ohara, A. Takeyama, K. Suyama, K. Hashimoto and M. Hosoya
Page No. 399
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (399-402)
An 8-month-old boy with congenital fibromuscular dysplasia presenting with shock caused by sudden renal hemorrhage
K. Takano, Y. Kawasaki, T. Imaizumi, S. Ohara, A. Takeyama, K. Suyama, K. Hashimoto and M. Hosoya
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan
Fibromuscular dysplasia (FMD) is a non-atheromatous, non-inflammatory, multifocal segmental angiopathy. FMD is the most common cause of pediatric renovascular hypertension. Aneurysmal formation of the main renal artery and distal branches is a rare complication of FMD in infancy. We report an 8-month-old boy with FMD presenting with shock caused by sudden renal hemorrhage that necessitated removal of one kidney. A diagnosis of renovascular hypertension resulting from intimal type FMD with aneurysmal formation was made on the basis of the presence of hypertension, elevation of PRA and aldosterone activity, pathological findings and the results of renal angiography. Our findings suggest that it is therefore necessary to consider FMD with aneurysmal formation as a possible cause of hypertension and renal hemorrhage in infants.Correspondence to:
Y. Kawasaki, MD
Department of Pediatrics
Fukushima Medical University School of Medicine
1 Hikariga-oka, Fukushima City
Fukushima 960-1295, Japan
Email: [email protected]
Case Report
Pseudoaldosteronism with increased serum cortisol associated with pneumonia, hypouricemia, hypocalcemia, and hypophosphatemia
A. Onishi, S. Muto, S. Homma, T. Inaba, R. Shuto, E. Kusano and Y. Asano
Page No. 403
Abstract
Clinical Nephrology, Vol. 74 – No. 5/2010 (403-408)
Pseudoaldosteronism with increased serum cortisol associated with pneumonia, hypouricemia, hypocalcemia, and hypophosphatemia
A. Onishi1, S. Muto1, S. Homma2, T. Inaba2, R. Shuto2, E. Kusano1 and Y. Asano2
1Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Tochigi, and 2Department of Internal Medicine, Koga Red-Cross Hospital, Koga, Ibaraki, Japan
We describe here the interesting case of a 73-year-old hypertensive man with pseudoaldosteronism. He had been taking glycyrrhizin at a dose of 75 mg/day for 12 years because of mild liver damage, but had never experienced any previous symptoms associated with hypokalemia. He was referred to our hospital because of hypokalemic tetraparesis and rhabdomyolysis. At that time, we noted mineralocorticoid excess characterized by hypokalemia due to urinary K loss, exacerbation of hypertension due to increased tubular Na reabsorption, metabolic alkalosis, and suppression of both plasma renin activity and plasma aldosterone concentration. His urinary free cortisol excretion rate and the urinary ratio of free cortisol to free cortisone were markedly elevated. Thus we diagnosed pseudoaldosteronism that was related to the long-term use of glycyrrhizin. When he developed pseudoaldosteronism, he also contracted pneumonia, and exhibited elevated levels of serum cortisol and creatinine clearance (CCr) as well as hypouricemia, hypocalcemia, and hypophosphatemia. All normalized after the recovery from pneumonia and the administration of spironolactone. The extracellular volume expansion associated with increased tubular Na reabsorption by the aldosterone-sensitive distal nephron and the resulting increase in CCr caused an inhibition of proximal tubular reabsorption of uric acid, Ca, and inorganic phosphate, leading to their renal loss and therefore hypouricemia, hypocalcemia, and hypophosphatemia, respectively. In this patient, the increased circulating cortisol associated with the stress of inflammation caused by pneumonia triggered the development of pseudoaldosteronism.Correspondence to:
S. Muto, MD, PhD
Division of Nephrology
Jichi Medical University
Shimotsuke, Tochigi 329-0498, Japan
Email: [email protected]
Letter to the Editor
Prevalence of the antiheparin-platelet factor 4 antibody in hemodialysis (HD) patients and its effects upon dialysis access
K. Wallace, I. Dasgupta, J. Stratton, P. Johnston and R. Parry
Page No. 409
Abstract
Prevalence of the antiheparin-platelet factor 4 antibody in hemodialysis (HD) patients and its effects upon dialysis access
