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Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (181-187)

Cinacalcet may reduce arterial stiffness in patients with chronic renal disease and secondary hyperparathyroidism – results of a small-scale, prospective, observational study

J. Bonet¹, B. Bayés¹,⁵, P. Fernández-Crespo², M. Casals³, J. López-Ayerbe⁴ and R. Romero¹,⁵

¹Service of Nephrology, Hospital Universitari Germans Trias i Pujol, ²Institut Mèdic de Badalona, ³Diaverum Mataró, ⁴Service of Cardiology, Hospital Universitari Germans Trias i Pujol de Badalona, and ⁵Members of REDinREN (RETICS ⁰⁶/⁰⁰¹⁶), Instituto Carlos III, Barcelona, Spain
Aims: This study evaluated the impact of cinacalcet on arterial stiffness, determined by pulse wave velocity (PWV), in patients with chronic renal disease and secondary hyperparathyroidism (SHPT). Patients and methods: This prospective, observational study included, SHPT patients with chronic renal disease on dialysis undergoing cinacalcet treatment with a follow-up of 12 months. Results: 21 patients, 62% males, with a mean age of 51.3 years (± 18.0) were included. Cinacalcet was given for at least a year with a mean daily dose of 35 mg (range 30 – 60 mg). Aortic PWV significantly decreased after 12 months of cinacalcet treatment (9.35 ± 1.83 m/sg vs. 8.66 ± 1.86 m/sg; p = 0.030). Additionally, there was a notable reduction trend in the left ventricular mass index (166.6 ± 39.4 g/m² vs. 156.1 ± 31.8 g/m²), although it did not achieve statistical significance (p = 0.063). Alkaline phosphatase and PTH were significantly decreased during the study. However, serum calcium, phosphorus and blood pressure remained stable. Conclusion: The results of this study support the possibility that cinacalcet reduces arterial stiffness of SHPT patients with chronic renal disease after 12 months of treatment. Prospective, randomized clinical trials are needed to confirm these preliminary findings.Correspondence to:
J. Bonet, MD, PhD
Nephrology Department
Hospital Universitari Germans Trias i Pujol
Universitat Autònoma de Barcelona
Ctra. De Can Ruti, s/n.
08916 Badalona, Barcelona, Spain
Email: Jbonet.germanstrias@gencat.cat

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (188-194)

Effects of peritoneal dialysis and hemodialysis on arterial stiffness compared with predialysis patients

L. Yang*, Y. Lin*, C. Ye, Z. Mao, S. Rong, X. Zhao and C. Mei

Department of Nephrology, Changzheng Hospital, Second Military Medical University, Shanghai, China

*These two authors contributed equally to this work.
Objective: Arterial stiffness is one of the major complications in chronic kidney disease. In this study, we investigated the association between glomerular filtration rate (GFR) and arterial stiffness in chronic kidney disease (CKD), and studied the effect of dialysis on arterial stiffness. Methods: A total of 62 stable continuous ambulatory peritoneal dialysis (CAPD) patients, 56 patients on maintenance hemodialysis, 128 predialysis CKD patients and 40 healthy controls were included into this study. These four groups were all nondiabetic and comparable in age and gender. The pulse wave velocity (PWV) and augmentation index (AIx) were evaluated by an applanation tonometry (SphygmoCor). Clinical data and lab tests were collected from inpatient case history and dialysis data base. Results: Female patients had higher AIx than male patients in both predialysis and dialysis patients. Both PWV and AIx were positively correlated with age, and were significantly higher in patients requiring antihypertensive drugs (p < 0.05). PWV and AIx were negatively correlated with GFR in predialysis CKD patients (p < 0.05). Patients on peritoneal dialysis or hemodialysis had better PWV and AIx than predialysis CKD 5 patients (19.8 ± 10.9%, 19.7 ± 9.4% vs. 25.3 ± 10.1%, p < 0.05), indicating that dialysis may improve arterial stiffness. No difference was found between peritoneal dialysis and hemodialysis patients. Conclusion: This study demonstrates that arterial stiffness progresses with deterioration of renal function in CKD patients, and both peritoneal dialysis and hemodialysis can improve arterial stiffness in patients with uremia.Correspondence to:
Prof. C. Ye
Division of Nephrology
Changzheng Hospital
Second Military Medical University
415 Fengyang Road
Shanghai, 200003, China
Email: yechaoyang63@126.com

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (195-203)

Association of adiponectin and leptin with serum lipids and erythrocyte omega-3 and omega-6 fatty acids in dialysis patients

W.S. An¹, Y.K. Son¹, S.E. Kim¹, K.H. Kim¹, H.R. Bae², S. Lee³, Y. Park⁴, H.J. Kim⁵ and N.D. Vaziri⁵

¹Department of Internal Medicine, ²Department of Physiology, Dong-A University, ³Department of Family Medicine and Medical Research Institute, Pusan National University, Busan, ⁴Department of Food and Nutrition, Hanyang University, Seoul, Republic of Korea, and ⁵Division of Nephrology and Hypertension, University of California, Irvine, CA, USA
Aims: Besides regulating energy metabolism, leptin promotes and adiponectin suppresses inflammation which is a common feature of end-stage renal disease (ESRD). Omega-3 fatty acids (n-3FA) exert anti-inflammatory actions by inhibiting pro-inflammatory signal transduction pathways whereas arachidonic acid (an n-6FA) facilitates inflammation by mediating inflammatory signals and serving as precursor of pro-inflammatory eicosanoids. Given the functional overlap between adipokines and n-3FA and n-6FA, we sought to explore their interrelationship in patients with ESRD. Methods: 44 ESRD patients maintained on hemodialysis (HD), 29 patients receiving peritoneal dialysis (PD), and 10 healthy subjects were enrolled. Body mass index (BMI), plasma leptin, adiponectin, lipids and CRP and erythrocyte fatty acids were measured. Results: Compared to controls adiponectin was elevated and leptin level was reduced in the ESRD group. Adiponectin levels were comparable among PD and HD patients, but leptin and BMI were higher in PD than in HD patients. Despite comparable BMIs, female patients had higher leptin than male patients. Leptin levels were positively associations with BMI, total and LDL cholesterol whereas adiponectin was inversely related with BMI, triglycerides and CRP and directly associated with HDL cholesterol in ESRD patients. Plasma adiponectin was directly associated with erythrocyte n-3 FA (r = 0.581, p = 0.023) and inversely associated with n-6FA (r = –0.640, p = 0.010) in the HD patients. Conclusion: A direct association was found between plasma levels of adiponectin and HDL and erythrocyte n-3FA in ESRD patients. Prospective trials are needed to explore the effect of n-3FA supplementation on plasma adipokines and markers of oxidative stress and inflammation in this population.Correspondence to:
W.S. An, MD
Department of Internal Medicine
Dong-A University, 3Ga-1
Dongdaesin-Dong, Seo-Gu
Busan 602-715, Republic of Korea
Email: anws@dau.ac.kr

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (204-211)

Warfarin use in hemodialysis patients: what is the risk?

P.J. Phelan¹, P. O’Kelly¹, J. Holian¹, J.J. Walshe¹, C. Delany¹, J. Slaby², S. Winders³, D. O’Toole⁴, C. Magee¹ and P.J. Conlon¹

¹Department of Nephrology, ²Department of Haematology, ³Department of Computer Science, and ⁴HIPE Department, Beaumont Hospital, Dublin, Ireland
Background: There is a paucity of data concerning the risks associated with warfarin in hemodialysis (HD) patients. We compared major bleeding episodes in this group with HD patients not receiving warfarin and with a cohort of non-HD patients receiving warfarin. Methods: A retrospective review of 141 HD patients on warfarin (HDW), 704 HD patients not on warfarin (HDNW) and 3,266 non-dialysis warfarin patients (NDW) was performed. Hospital admissions for hemorrhagic events and ischemic strokes were examined as was hospital length of stay and blood product use. INR variability was also assessed. Results: The incidence rates for major hemorrhage per 100 patient years was 10.8 in the HDW group as compared to 8.0 in the HDNW (p = 0.593) and 2.1 in the NDW (p < 0.001) groups. Mean units of red blood cell transfusions required was higher in patients on dialysis with no significant difference between HDW and HDNW groups. The risk of ischemic stroke per 100 patient years was 1.7 in the HDW group as compared to 0.7 in the HDNW groups (p = 0.636) and 0.4 in the NDW (p = 0.003). The HDW group had higher inter-measurement INR variability compared to the NDW group (p = 0.034). In patients with atrial fibrillation, HDW group had a higher incidence of ischemic stroke than the NDW group (2.2 versus 0.4 events per 100 patient years; p = 0.024). Conclusions: This study confirms the higher bleeding risk associated with HD/ESRD but suggests that warfarin use in these patients may not add significantly to this risk. We also demonstrated high rates of ischemic stroke in HD patients despite warfarin use. Summary: Our study compares the frequency of major hemorrhage and secondarily, ischemic stroke in HD patients receiving or not receiving warfarin, with non-HD patients receiving warfarin. The major finding was that frequency of hemorrhage was higher in HD patients receiving warfarin than in non-HD patients receiving warfarin, but not different in HD patients with or without warfarin. A secondary finding was that INR variability was significantly higher in HD patients than non-HD patients on warfarin.Correspondence to:
Dr. P.J. Phelan, MB BCh BAO, MRCPI
Department of Nephrology
Beaumont Hospital, Dublin 9, Ireland
Email: paulphel@gmail.com

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (212-217)

The effect of heparinized catheter lock solutions on systemic anticoagulation in hemodialysis patients

P.C. Thomson¹, S.T. Morris² and R.A. Mactier²

¹Specialist Registrar in Renal Medicine, and ²Consultant Nephrologist, Renal Unit, Glasgow Royal Infirmary, UK
Background: Hemodialysis catheter thrombosis is associated with loss of catheter patency, catheter-related bacteremia and sepsis. To limit these risks, many renal units use heparin as a catheter-locking solution. In this study we investigate the effect of different concentrations of heparin catheter lock solution on systemic anticoagulation in an investigator-blinded randomized study of patients with non-tunneled (temporary) central venous catheters. Methods: 28 consecutive patients requiring insertion of a temporary non-tunneled dual lumen central venous hemodialysis catheter were randomly allocated to receive either heparin 5,000 IU/ml or heparin 1,000 IU/ml as catheter lock solutions. The primary outcome measure was the difference in activated partial thromboplastin time (APTT) at 10 minutes following catheter locking with heparin 5,000 IU/ml and heparin 1,000 IU/ml. Secondary outcomes included intradialytic blood flow rates, catheter removal due to insufficient hemodialysis blood flow to maintain hemodialysis and catheter-related bacteremia. Results: 13 patients were randomized to the heparin 1,000 IU/ml group with 15 patients randomized to the heparin 5,000 IU/ml group. There was a statistically significant increase in APTT at 10 minutes between groups with median +22.2% (range 0 – 210) rise in APTT in the heparin 1,000 IU/ml group compared with +373.7% (range 133 – 800) in the heparin 5,000 IU/ml group (p < 0.001). There was no statistically significant difference between groups with the secondary outcomes of intradialytic blood flow, catheter failure rates and catheter-related bacteremia rates. Conclusions: Heparin 1,000 IU/ml catheter lock solution confers a significantly lower risk of systemic heparinization than heparin 5,000 IU/ml without any overtly detrimental effect on intradialytic blood flow, catheter failure rates and catheter-related bacteremia rates.Correspondence to:
Dr. P.C. Thomson
Specialist Registrar, Renal Unit
Glasgow Royal Infirmary
Castle Street, Glasgow, G4 0SF, UK
Email: peter.thomson@northglasgow.scot.nhs.uk

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (218-225)

Assessment of habitual physical activity and energy expenditure in dialysis patients and relationships to nutritional parameters

A. Cupisti¹, A. Capitanini², G. Betti³, C. D’Alessandro¹ and G. Barsotti¹

¹Division of Nephrology, Department of Internal Medicine, University of Pisa, Pisa, ²Nephrology and Dialysis, Pescia Hospital, Pescia, and ³Nephrology and Dialysis, Massa-Carrara Hospital, Massa-Carrara, Italy
Background and aim: Assessment of physical activity level and of energy expenditure is important in the clinical and nutritional care of dialysis patients, but it is not so easy to accomplish. The SenseWear™ Armband (SWA) is a novel multisensory device that is worn on the upper arm and collects a variety of physiologic data related to physical activity. Thus, duration and intensity of physical activity is recorded and expressed as METs (Metabolic Equivalent Task), and energy expenditure is estimated. The aim of our study was to assess interdialytic spontaneous physical activity in stable chronic hemodialysis (HD) patients and the relation to nutritional status and dietary nutrient intake. Patients and methods: In 50 stable patients on maintenance hemodialysis treatment and 33 normal subjects (control group), level of spontaneous physical activity and estimated daily energy expenditure was assessed by SWA and related to biochemistry and anthropometry data, bioelectric impedance vector analysis, and energy and nutrient intake information coming from a 3-day food recall. Results: In respect to controls, HD patients showed lower mean daily METs value (1.3 ± 0.3 vs. 1.5 ± 0.2, p < 0.01), a lower time spent on activities > 3 METs (89 ± 85 vs. 143 ± 104 min/day, p < 0.05), lower number of steps per day (5,584 ± 3,734 vs. 11,735 ± 5,130, p < 0.001), resulting in a lower estimated energy expenditure (2,190 ± 629 vs. 2,462 ± 443 Kcal/day, p < 0.05). 31 out of the 50 HD patients (62%) had a mean daily value < 1.4 METs and hence were defined as sedentary. They differed from the active patients for higher age (63 ± 12 vs. 54 ± 12 y, p < 0.01), lower energy intake (26.1 ± 6.4 vs. 32.4 ± 11.3 Kcal/day, p < 0.05) and lower phase angle (5.5 ± 1.0 vs. 6.3 ± 0.9, p < 0.05). SWA-based estimation of daily energy expenditure was negatively related to age (r = –0.31, p < 0.05), whereas positive relations were observed with BMI (r = 0.51, p < 0.001), phase angle (r = 0.40, p < 0.01), serum phosphate (r = 0.49, p < 0.001) and albumin (r = 0.41, p < 0.01). The mean daily METs values were strongly related to normalized energy intake (r = 0.47, p < 0.001) and also to protein intake (r = 0.33, p < 0.05) and to phase angle (r = 0.38, p < 0.01). Multiple regression analysis showed that energy intake and dietary protein intake were independently related to the intensity of physical activity. Conclusion: Our findings indicate that poor physical activity is highly prevalent in stable dialysis patients even when free from physical or neurological disabilities or severe comorbid conditions. The level and intensity of physical activity is positively related to body composition and to dietary nutrient intake. This confirms the strong interrelationship between exercise and nutrition, which in turn are associated with survival, rehabilitation and quality of life in dialysis patients.Correspondence to:
A. Cupisti, MD, PhD
Division of Nephrology
Department of Internal Medicine
University of Pisa
Via Roma 67, 56126 Pisa, Italy
Email: acupisti@med.unipi.it

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (226-232)

Serum relaxin levels and kidney function in late pregnancy with or without preeclampsia

R.A. Lafayette, M.A. Hladunewich, G. Derby, K. Blouch, M.L. Druzin and B.D. Myers

Stanford University Medical Center, Stanford, CA, USA
Objective: Relaxin, a potent pregnancy-related hormone, has been proposed to be a major mediator of renal physiology in normal pregnancy. We wished to test relaxin levels in pregnancy and preeclampsia. Methods: We performed precise physiologic measurements of kidney function in 38 normal peripartum women and 58 women with preeclampsia. We measured serum relaxin levels prior to delivery and over the first 4 postpartum weeks utilizing a modern, validated ELISA. Results were compared to those of 18 normal women of childbearing age. Results: Relaxin levels were substantially elevated in women prior to delivery (364 ± 268 vs. 15 ± 16 pg/ml) and fell rapidly over the first postpartum week reaching normal non pregnant levels by Week 2 (32 ± 64 vs. 15 ± 16 pg/ml). No differences were seen between relaxin levels in normal pregnancy as compared to preeclampsia (364 ± 268 vs. 376 ± 241 pg/ml) despite substantial and persistent abnormalities in GFR (149 ± 33 vs. 89 ± 25 ml/min), albuminuria (14 vs. 687 mg/g) and mean arterial pressure (80 ± 8 vs. 111 ± 18). Furthermore no correlation could be established between physiologic measures (GFR, MAP, RBF, RVR) and relaxin levels (p > 0.3), either in the overall population or any of the subgroups. Conclusion: Relaxin is indeed significantly elevated in the serum of women during late pregnancy and the early puerperium. However, serum relaxin does not appear to influence BP, renal vascular resistance, renal blood flow or GFR in late pregnancy or in women with preeclampsia.Correspondence to:
R.A. Lafayette, MD, FACP
Stanford University Medical Center, S161
300 Pasteur Drive
Stanford, CA 94305, USA
Email: czar@stanford.edu

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (233-241)

Geographical variation in the response of lupus nephritis to mycophenolate mofetil induction therapy

S. Mohan and J. Radhakrishnan

Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA
Objective: The induction therapy used in the management of Class III and IV lupus nephritis has been narrowed down to a choice between cyclophosphamide (CYC) and mycophenolate mofetil (MMF). However, there has been variability in the response to these agents, which may relate to demographic factors. In this study, we analyzed geographic factors affecting the response to CYC or MMF with a stratified meta-analysis. Methods: We found and included 11 studies for our analyses – 7 randomized controlled trials (RCTs) and 4 non-RCTs – that were stratified as being in Asia or elsewhere. Results: Meta-analysis of Asian studies showed no difference between regimens (RR = 0.98, 95% CI 0.74 – 1.28, p = ns) and no heterogeneity. The 2 RCT’s from outside Asia showed significant advantage of MMF as induction therapy (RR = 3.69, 95%CI 1.45 – 9.3, p < 0.01) without heterogeneity. Together there was significant heterogeneity (Q = 11.69, I² = 57.2%, p < 0.05) suggesting that the overall heterogeneity results from a difference between these groups. We repeated this analysis with the multicenter ALMS trial with their reported ethnicity-specific data. Again, no significant heterogeneity was seen in either subgroup (Asian: Q = 2.08, I² = 0%, p = ns; Others: Q = 4.49, I² = 55.5%, p = ns). Studies from Asia did not show a significant improvement with the use of MMF (RR = 1.06, 95%CI 0.79 – 1.41, p = ns) while data from RCTs conducted outside Asia suggested a greater likelihood of complete remission with the use of MMF (RR = 1.85, 95%CI 1.03 – 3.29, p < 0.05). Conclusion: Our analysis demonstrates a higher likelihood of complete remission with MMF in patients outside Asia.Correspondence to:
S. Mohan, MD, MPH
Division of Nephrology
Columbia University Medical Center
622 W168th Street, PH4-124
New York, NY 10032, USA
Email: sm2206@columbia.edu

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (242-250)

An observational study of the effectiveness of darbepoetin alpha administered in dialysis patients once every 2 weeks for 12 months

J.B. Rottembourg¹, I. Bridges², W. Pronai³, M. Feriani⁴, L.P. McMahon⁵, J.M.J. De Meester⁶, M. Farouk⁷ and B. Molemans⁷

¹Centre Suzanne Levy, Diaverum Group, Clinique du Mont Louis, Paris, France, ²Amgen Ltd., Cambridge, UK, ³Barmherzige Brüder Eisenstadt, Eisenstadt, Austria, ⁴Ospedale dell’Angelo, Via Pacagnella, Mestre, Italy, ⁵Eastern Health, Melbourne, Australia, ⁶AZ Nikolaas, Sint-Niklaas, Belgium, and ⁷Amgen (Europe) GmbH, Zug, Switzerland
Aims: Erythropoiesis-stimulating agents (ESAs) are recommended for managing renal anemia. ALTERNATE is an observational study in European and Australian dialysis patients evaluating darbepoetin a (DA) once every 2 weeks (Q2W) in clinical practice. Methods: Adult dialysis patients initiating treatment with DA Q2W were eligible regardless of previous/current ESA use. Data were collected 6 months before and 12 months after Q2W initiation. The primary endpoint was hemoglobin (Hb) concentration 12 months after initiation. Results: A total of 6,112 patients were enrolled; 6,104 were eligible (87% hemodialysis, 12% peritoneal dialysis). Before initiation, 77.3%, 8.8%, and 7.8% of patients were receiving DA, epoetin beta, and epoetin alpha, respectively; 6% were ESA naïve. Mean (95% CI) Hb (g/dl) was 11.68 (11.63 – 11.72) 6 months before initiation, 12.00 (11.97 – 12.04) at initiation, and 11.62 (11.58 – 11.66) 12 months after initiation. Geometric mean (95% CI) weekly ESA dose (µg/wk) was 27.27 (26.62 – 27.93) immediately before initiation, 23.69 (23.28 – 24.10) at initiation, and 26.80 (26.12 – 27.49) 12 months after initiation. At month 12, 77.3% of patients were receiving DA Q2W. Conclusions: This large observational study demonstrates that Hb concentrations can be effectively maintained over 12 months in a general dialysis population with DA Q2W without an increase in ESA dose.Correspondence to:
J.B. Rottembourg, MD
Centre Suzanne LEVY, Diaverum Group
Clinique du Mont Louis
8 rue de la Folie Regnault, 75011 Paris, France
Email: jacques.rottembourg@wanadoo.fr

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2010 (251-254)

Rapid deterioration of renal insufficiency after magnetic resonance imaging with gadolinium-based contrast agent

K. Fujisaki¹, A. Ono-Fujisaki¹, N. Kura-Nakamura¹, N. Komune³, N. Hirakawa³, K. Tsuruya¹, ², S. Komune³ and M. Iida¹

¹Department of Medicine and Clinical Science, ²Department of Integrated Therapy for Chronic Kidney Disease and ³Department of Otorhinolaryngology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Gadolinium (Gd)-based contrast media were introduced as alternatives to iodinated media for magnetic resonance imaging (MRI). Although originally thought to be non-nephrotoxic, Gd-based contrast media have recently been reported to be associated with acute kidney injury. The underlying mechanism of Gd-induced renal injury is not completely understood. We report an 80-year-old patient with buccal mucosa cancer for whom MRI with Gd-based contrast agent was conducted 3 times within 3 weeks. The patient developed rapid deterioration of preexisting renal insufficiency, and developed uremic symptoms and pulmonary edema. The patient was hemodialyzed 3 times. This resulted in improvement of renal function and clinical symptoms. This case emphasizes the potential nephrotoxicity of Gd-based contrast media and suggests that renal insufficiency, diabetes mellitus, old age and high dose of Gd-based contrast medium are risk factors for acute kidney injury.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

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (255-262)

A notable case report of May-Hegglin anomaly with immune complex-related nephropathy: a genetic and histological analysis

Y. Ohtsuka¹, T. Kanaji², M. Nishi¹, N. Sakai¹, T. Sato¹, S. Aoki³, K. Wakayama⁴, S. Nakazato⁴, S. Hisano⁵, Y. Sado⁶, H. Kawachi⁷, K. Izuhara⁸ and Y. Hamasaki¹

¹Department of Pediatrics, ²Department of Biomolecular Sciences, ³Department of Pathology and Biodefense, Faculty of Medicine, Saga University, ⁴Department of Laboratory Medicine, Saga University Hospital, Saga, ⁵Department of Pathology, School of Medicine, Fukuoka University, Fukuoka, ⁶Department of Pathobiology Shigei Medical Research Institute, Okayama, ⁷Department of Cell Biology, Institute of Nephrology, Niigata University Graduate School of Medical and Dental Sciences, and ⁸Division of Medical Biochemistry, Department of Biomolecular Sciences, Saga University, Saga, Japan
May-Hegglin anomaly (MHA) is a rare autosomal dominant disease characterized by macrothrombocytopenia and leukocyte inclusions with microfilaments in the ribosomes. Mutations in the MYH9 gene, encoding non-muscle myosin heavy chain IIA (NMMHC-IIA) have been identified in patients with MHA and other MYH9-related diseases. Two young males (an older and younger brother) presented with macrothrombocytopenia and leukocyte inclusion bodies. Electron microscopy (EM) revealed parallel filaments in leukocyte inclusion bodies characteristic of MHA. Immunofluorescence microscopy (IF) showed NMMHC-IIA antibodies in 1 – 2 leukocyte inclusion bodies. These findings were consistent with MHA and they were identified to express the MYH9 mutation, D1424H. The older brother underwent a renal biopsy because of persistent proteinuria. Histology revealed mesangial proliferative glomerulonephritis with granular deposits of IgG and C1q. EM showed that the dense deposits were located in subendothelial cells, mesangial cells and Bowman’s capsule. Immunocytochemistry revealed that NMMHC-IIA antibodies were localized in podocyte and endothelial cells in the glomerulus. Moreover, the expression of nephrin and podocin, slit diagram protein, was normal. An inflammatory mechanism may occur separately from MYH9-related disease. This report presents a case of MHA with immune complex-related nephropathy.Correspondence to:
Y. Ohtsuka, MD
Department of Pediatrics
Faculty of Medicine
Saga University, 5-1-1 Nabeshima
Saga City 849-8501, Japan
Email: ootsuka2@cc.saga-u.ac.jp

Abstract

Clinical Nephrology, Vol. 75 – No. 3/2011 (263-268)

Cinacalcet in the treatment of persistent hyperparathyroidism after kidney transplantation

L.R.S. Pinho, M.J.C. Ribeiro Santos and M. Pestana Vasconcelos

Nephrology Department, Nephrology Research and Development Unit, Hospital S. Joao, Porto Medical School, Porto, Portugal
Background: Secondary hyperparathyroidism persists in 30 – 50% of patients after a successful kidney transplant and it is the most frequent cause of hypercalcemia after transplant, contributing to graft loss and mortality. Several medical therapies have been studied for the treatment of this condition without clear benefit. Recently, interest has grown in the use of cinacalcet in kidney transplant recipients. Methods: We describe the efficacy of cinacalcet in 18 kidney transplant patients with persistent hyperparathyroidism and progressive hypercalcemia treated for a period of 12-months. We analyzed serum calcium, phosphorus and parathyroid hormone levels every 6 months, and cinacalcet was titrated if necessary. Statistical analysis was performed using ANOVA. Results: With therapy, all patients exhibited significant reduction in parathyroid hormone levels, from a mean value of 242.04 ± 105.82 pg/ml to a mean value of 145.62 ± 54.99 pg/ml (p < 0.001) 12 months later. In addition, serum calcium levels normalized during the study period, from 11.16 mg/d to 9.95 mg/dl (mean values) (p < 0.001). No significant change in serum creatinine was found in this group of patients. Cinacalcet was well tolerated, with no side effects documented. Conclusion: This preliminary experience suggests that cinacalcet may be useful in the treatment of persistent hyperparathyroidism after kidney transplant. In addition, cinacalcet controlled hypercalcemia, which has well known adverse effects after transplant. This was accomplished with no evidence of declining kidney function or limiting side effects.Correspondence to:
L. Pinho, MD
Nephrology Department
Hospital S. Joao
Alameda Professor Hernani Monteiro
4200 Porto, Portugal
Email: lilianarspinho@gmail.com

Abstract

Insertion of the dialysis catheter into right atrium or superior vena cava?

P.-T. Liu, S. Samson, J.-D. Maurellet and C. Manthous

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Volume 75, No. 3/2011(March)