Exercise in Patients on Dialysis: A Multicenter, Randomized Clinical Trial.

Previous studies have suggested the benefits of physical exercise for patients on dialysis. We conducted the Exercise Introduction to Enhance Performance in Dialysis trial, a 6-month randomized, multicenter trial to test whether a simple, personalized walking exercise program at home, managed by dialysis staff, improves functional status in adult patients on dialysis. The main study outcomes included change in physical performance at 6 months, assessed by the 6-minute walking test and the five times sit-to-stand test, and in quality of life, assessed by the Kidney Disease Quality of Life Short Form (KDQOL-SF) questionnaire. We randomized 296 patients to normal physical activity (control; n=145) or walking exercise (n=151); 227 patients (exercise n=104; control n=123) repeated the 6-month evaluations. The distance covered during the 6-minute walking test improved in the exercise group (mean distance±SD: baseline, 328±96 m; 6 months, 367±113 m) but not in the control group (baseline, 321±107 m; 6 months, 324±116 m; P<0.001 between groups). Similarly, the five times sit-to-stand test time improved in the exercise group (mean time±SD: baseline, 20.5±6.0 seconds; 6 months, 18.2±5.7 seconds) but not in the control group (baseline, 20.9±5.8 seconds; 6 months, 20.2±6.4 seconds; P=0.001 between groups). The cognitive function score (P=0.04) and quality of social interaction score (P=0.01) in the kidney disease component of the KDQOL-SF improved significantly in the exercise arm compared with the control arm. Hence, a simple, personalized, home-based, low-intensity exercise program managed by dialysis staff may improve physical performance and quality of life in patients on dialysis.

The role of deconditioning in the end-stage renal disease myopathy: physical exercise improves altered resting muscle oxygen consumption.

BACKGROUND: Skeletal muscle dysfunction and poor exercise tolerance are hallmarks of end-stage renal disease (ESRD). Noninvasively measured (near-infrared spectroscopy, NIRS) resting muscle oxygen consumption (rmVO2) is a biomarker of muscle dysfunction, which can be applied to study the severity and the reversibility of ESRD myopathy. We tested the hypothesis that deconditioning is a relevant factor in ESRD myopathy. METHODS: The whole dialysis population (n = 59) of two of the eight centers participating into the EXCITE study (ClinicalTrials.gov NCT01255969), a randomized trial evaluating the effect of a home-based exercise program on the functional capacity of these patients was studied. Thirty-one patients were in the active arm (exercise group) and 28 in the control arm (no intervention). Normative data for rmVO2 were obtained from a group of 19 healthy subjects. RESULTS: rmVO2 was twice higher (p < 0.001) in ESRDs patients (0.083 ± 0.034 ml/100 g/min) than in healthy subjects (0.041 ± 0.020 ml/100 g/min) indicating substantial skeletal muscle dysfunction in ESRD. rmVO2 correlated with resting heart rate (r = 0.34, p = 0.009) but was independent of age, dialysis vintage, biochemical, vascular and nutrition parameters. After the 6-month exercise program, rmVO2 reduced to 0.064 ± 0.024 ml/100 g/min (-23%, p < 0.001) in the exercise group indicating that skeletal muscle dysfunction is largely reversible but remained identical in the control group (0.082 ± 0.032 to 0.082 ± 0.031 ml/100 g/min). CONCLUSION: Deconditioning has a major role in ESRD myopathy. rmVO2 is a marker of physical deconditioning and has the potential for monitoring re-conditioning programs based on physical exercise in the ESRD population.

The role of physical activity in the CKD setting.

A sedentary lifestyle contributes to the development of cardiovascular disease, hypertension, diabetes and probably cancer in the general population; this cluster of disease may be defined the diseasome of physical inactivity. Also in CKD/ESRD patients physical activity is strikingly low. As a result of growing evidence suggestive of cardiovascular benefit among the CKD population with exercise, the National Kidney Foundation recommended counseling by nephrologists to increase patients' levels of physical activity in their guideline about management of cardiovascular disease. Therefore, to maintain the well-being and functional capacity of renal patients attention should be directed toward maintaining strength and aerobic fitness as well as focusing on renal function and anemia or other comorbidities. All CKD/ESRD patients should be counseled and regularly encouraged by nephrology and dialysis staff to increase their level of physical activity.

Fitness for entering a simple exercise program and mortality: a study corollary to the exercise introduction to enhance performance in dialysis (EXCITE) trial.

BACKGROUND/AIMS: In this corollary analysis of the EXCITE study, we looked at possible differences in baseline risk factors and mortality between subjects excluded from the trial because non-eligible (n=216) or because eligible but refusing to participate (n=116). METHODS: Baseline characteristics and mortality data were recorded. Survival and independent predictors of mortality were assessed by Kaplan-Meier and Cox regression analyses. RESULTS: The incidence rate of mortality was higher in non-eligible vs. eligible non-randomized patients (21.0 vs. 10.9 deaths/100 persons-year; P<0.001). The crude excess risk of death in non-eligible patients (HR 1.96; 95% CI 1.36 to 2.77; P<0.001) was reduced after adjustment for risk factors which differed in the two cohorts including age, blood pressure, phosphate, CRP, smoking, diabetes, triglycerides, cardiovascular comorbidities and history of neoplasia (HR 1.60; 95% CI 1.10 to 2.35; P=0.017) and almost nullified after including in the same model also information on deambulation impairment (HR 1.16; 95% CI 0.75 to 1.80; P=0.513). CONCLUSIONS: Deambulation ability mostly explains the difference in survival rate in non-eligible and eligible non-randomized patients in the EXCITE trial. Extending data analyses and outcome reporting also to subjects not taking part in a trial may be helpful to assess the representability of the study population.

Physical performance and clinical outcomes in dialysis patients: a secondary analysis of the EXCITE trial.

BACKGROUND/AIMS: Scarce physical activity predicts shorter survival in dialysis patients. However, the relationship between physical (motor) fitness and clinical outcomes has never been tested in these patients. METHODS: We tested the predictive power of an established metric of motor fitness, the Six-Minute Walking Test (6MWT), for death, cardiovascular events and hospitalization in 296 dialysis patients who took part in the trial EXCITE (ClinicalTrials.gov Identifier: NCT01255969). RESULTS: During follow up 69 patients died, 90 had fatal and non-fatal cardiovascular events, 159 were hospitalized and 182 patients had the composite outcome. In multivariate Cox models - including the study allocation arm and classical and non-classical risk factors - an increase of 20 walked metres during the 6MWT was associated to a 6% reduction of the risk for the composite end-point (P=0.001) and a similar relationship existed between the 6MWT, mortality (P<0.001) and hospitalizations (P=0.03). A similar trend was observed for cardiovascular events but this relationship did not reach statistical significance (P=0.09). CONCLUSIONS: Poor physical performance predicts a high risk of mortality, cardiovascular events and hospitalizations in dialysis patients. Future studies, including phase-2 EXCITE, will assess whether improving motor fitness may translate into better clinical outcomes in this high risk population.

Multidrug therapy for polycystic kidney disease: a review and perspective.

Autosomal dominant polycystic kidney disease (ADPKD) is a renal disorder characterized by the development of cysts in both kidneys leading to end-stage renal disease (ESRD) by the fifth decade of life. Cysts also occur in other organs, and phenotypic alterations also involve the cardiovascular system. Mutations in the PKD1 and PKD2 genes codifying for polycystin-1 (PC1) and polycystin-2 (PC2) are responsible for the 85 and 15% of ADPKD cases, respectively. PC1 and PC2 defects cause similar symptoms; however, lesions of PKD1 gene are associated with earlier disease onset and faster ESRD progression. The development of kidney cysts requires a somatic 'second hit' to promote focal cyst formation, but also acute renal injury may affect cyst expansion, constituting a 'third hit'. PC1 and PC2 interact forming a complex that regulates calcium homeostasis. Mutations of polycystins induce alteration of Ca(2+) levels likely through the elevation of cAMP. Furthermore, PC1 loss of function also induces activation of mTOR and EGFR signaling. Impaired cAMP, mTOR and EGFR signals lead to activation of a number of processes stimulating both cell proliferation and fluid secretion, contributing to cyst formation and enlargement. Consistently, the inhibition of mTOR, EGFR activity and cAMP accumulation ameliorates renal function in ADPKD animal models, but in ADPKD patients mild results have been shown. Here we briefly review major ADPKD-related pathways, their inhibition and effects on disease progression. Finally, we suggest to reduce abnormal cell proliferation with possible clinical amelioration of ADPKD patients by combined inhibition of cAMP-, EGFR- and mTOR-related pathways.

Deficiency of polycystic kidney disease-1 gene (PKD1) expression increases A(3) adenosine receptors in human renal cells: implications for cAMP-dependent signalling and proliferation of PKD1-mutated cystic cells.

Cyst growth and expansion in autosomal dominant polycystic kidney disease (ADPKD) has been attributed to numerous factors, including ATP, cAMP and adenosine signalling. Although the role of ATP and cAMP has been widely investigated in PKD1-deficient cells, no information is currently available on adenosine-mediated signalling. Here we investigate for the first time the impact of abnormalities of polycystin-1 (PC1) on the expression and functional activity of adenosine receptors, members of the G-protein-coupled receptor superfamily. Pharmacological, molecular and biochemical findings show that a siRNA-dependent PC1-depletion in HEK293 cells and a PKD1-nonsense mutation in cyst-derived cell lines result in increased expression of the A(3) adenosine receptor via an NFkB-dependent mechanism. Interestingly, A(3) adenosine receptor levels result higher in ADPKD than in normal renal tissues. Furthermore, the stimulation of this receptor subtype with the selective agonist Cl-IB-MECA causes a reduction in both cytosolic cAMP and cell proliferation in both PC1-deficient HEK293 cells and cystic cells. This reduction is associated with increased expression of p21(waf) and reduced activation not only of ERK1/2, but also of S6 kinase, the main target of mTOR signalling. In the light of these findings, the ability of Cl-IB-MECA to reduce disease progression in ADPKD should be further investigated. Moreover, our results suggest that NFkB, which is markedly activated in PC1-deficient and cystic cells, plays an important role in modulating A(3)AR expression in cystic cells.

Renal replacement therapy in elderly patients: peritoneal dialysis.

Management of chronic uremia in elderly patients presents several clinic and organizational difficulties. Hemodialysis (HD) and chronic peritoneal dialysis (CPD) are both available for the elderly, and the choice depends on the individual, clinical and familial conditions. Several reports have compared the outcomes for older patients treated by HD or peritoneal dialysis, with those for younger or older patients undergoing peritoneal dialysis. CPD is a successful dialysis option for elderly patients, in both patient and technique survival terms. All nutritional parameters are of pivotal importance. Several barriers, such as medical and social factors, physician bias, late referral and education irrespective of the needs of older patients, influence the choice of CPD. The development of assisted peritoneal dialysis, using community-based nurses or health care assistants, can overcome some of the barriers and enable frail older patients to have home-based dialysis treatment. Increasing age is associated with higher peritonitis rates among patients who started CPD in the 1990s, while age is not associated with peritonitis in more recent CPD cohorts, and no greater frequency of adverse outcomes of peritonitis has been seen among those who began CPD after the year 2000. In elderly dialysis patients, the management of quality of life (QOL) is important as well as adequacy of dialysis, nutritional status and survival rate. To obtain a good standard of QOL, it is essential to select carers who are properly educated and who can access an adequate support system, both physical and psychological, to help them cope with their burden.

Maintaining over time clinical performance targets on anaemia correction in unselected population on chronic dialysis at 20 Italian centres. Data from a retrospective study for a clinical audit.

BACKGROUND: The Italian and European Best Practice Guidelines (EBPG) recommend a target haemoglobin value greater than 11 g/dl in most patients with Chronic Kidney Diseases. However, it is still difficult to maintain these values at a steady rate. Thus, the main aim of the study was to evaluate, throughout 2005, how many patients steadily maintained the performance targets related to anaemia treatment. METHODS: The survey was conducted on 3283 patients on haemodialysis (HD) and peritoneal dialysis (PD) at 20 Italian dialysis centres. 540 patients were randomly selected; each centre provided a statistically significant sample proportional to its total number of patients. Maintenance of the following target levels was assessed over time: Haemoglobin (HB) 11-12 gr/dl; Iron: 60-160 mcg/dl; Ferritin: 30-400 mcg/l; Transferrin: 200-360 mg/dl; Transferrin saturation percentage (TSAT %):> 25 <50; Dialysis doses (KT/V): >1.2 <2.0 for non-diabetic HD patients; >1.5 <2.2 for diabetic HD patients; DP: >1.8 <2.5.Outcome included:1- Percentage of target maintenance for each parameter.2- Erythropoietin dose in relation to dialysis techniques, presence of cancer or myeloma, diabetic status, Vitamin B therapy.3- Erythropoietin dose (International Units/kg/week) (IU/kg/wk) depending on: haemoglobin values, hospitalization of more than 3 days. RESULTS: Mean age was 65.1; mean haemoglobin concentration over the whole population was 11.3 gr/dl (Standard Deviation (SD): 0.91). The clinical performance targets were maintained over time as follows: HB: 4.3% (Mean 11.43 gr/dl) (SD: 0.42); Ferritin: 71.1% (Mean: 250.23 mcg/L (SD:104.07); Iron: 95.0% (Mean 59.79 mcg/dl)(SD:16.76); Transferrin: 44.8% (Mean 216.83 mg/dl) (SD: 19,50); TSAT %: in 8.4% (Mean: 34.33% (SD: 6.56); HD KT/V: 61.0% (Mean:1.46) (SD: 0.7); PD KT/V:31.4% (Mean: 2.10) (SD: 0.02). The average weekly dose of Erythropoietin (IU/Kg/Wk) was significantly lower for the peritoneal dialysis technique; the higher haemoglobin values, the lower the Erythropoietin dose (IU/Kg/Wk). CONCLUSION: A very low percentage of patients maintained haemoglobin target values over time. We need to identify precise criteria to evaluate the stability over time of clinical performance targets proposed by the guidelines.

Acute and long-term effects of an exercise program for dialysis patients prescribed in hospital and performed at home.

BACKGROUND: Exercise has positive psychophysical effects on dialysis patients, thus effective programs should be identified. We evaluated the effects of an original 6-month walking program on physical capacity, health-related quality of life (HRQL) and postdialysis fatigue (PDF). METHODS: Thirty-one dialysis patients (19 male, mean age 65 -/+ 11 years) were divided into exercise (group E; n=17) and control (group C; n=14) groups, and evaluated upon entry, after the 6-month program and 19 -/+ 3 months later. Outcome measures were 6-minute walking distance (6MWD), SF-36 scale scores, self-reported PDF and recovery time. E group was assigned 2 daily 10-minute home walking sessions on the nondialysis day at a speed 50% below maximal treadmill speed as determined and updated monthly at the hospital. C group: no exercise. RESULTS: Twenty patients (13 from E, 7 from C) completed the study. The E group, unlike the C group, increased 6MWD (308 -/+ 105 m, to 351 -/+ 118 m, p=0.0007), and HRQL, significantly for bodily pain, physical role and mental health (p<0.05), decreased PDF and recovery time (p<0.05). At the follow-up, 15 patients were reevaluated (9 from E, 6 from C). The E group was still active and showed 6MWD similar to baseline, with a decline of 0.13 -/+ 1.72 m/mo. The C group decreased 6MWD (p=0.026) with a decline of 3.43 -/+ 3.2 m/mo. For both groups, HRQL, PDF and recovery time showed slight variations from baseline. CONCLUSIONS: In dialysis patients, a 6-month exercise program prescribed at the hospital and performed at home improved physical capacity, HRQL and PDF symptoms. Patients maintained an active lifestyle after discharge and showed a slow functional decline over a 2-year period.

Clinical features of sarcomatoid renal cell carcinoma causing bilateral urinary tract obstruction.

We report on a 52-year-old female patient hospitalized because of uremia due to bilateral urinary tract obstruction caused by bilateral sarcomatoid renal cell carcinoma (SRCC). Abdominal computed tomography with contrast showed a large mass on the left side, infiltrating the left kidney, while the right kidney was described as enlarged. The latter was investigated with sonographic angiography using contrast and selective arteriography of the renal arteries, demonstrating a pseudonodular area at the inferior pole of the right kidney. The patient underwent bilateral nephrectomy and chronic hemodialysis treatment; unfortunately, after one month she died from cachexia. To the best of our knowledge this is the first case to be reported on bilateral SRCC causing bilateral urinary tract obstruction.

Exercise training in peripheral vascular arterial disease in hemodialysis patients: a case report and a review.

BACKGROUND: Peripheral arterial disease (PAD) is frequently diagnosed in subjects with chronic kidney disease. Hemodialysis (HD) patients with PAD show increased morbidity and mortality and health care costs increase. Management of this complication requires time and skill by nephrologists, although negative results are frequent. CASE REPORT: A 59-year-old Caucasian man on HD with advanced lower extremities peripheral disease and massive calcification of a plaque in the abdominal aorta has been enrolled in a home-based exercise training program. His compliance was high and claudication improved. Pain threshold speed (PTS) and maximal walking speed rose from 2.8 and 3.3 to 3.6 and 4.6 Km/h respectively. The increasing functional capability improved his quality of life and changed positively his life-style. CONCLUSIONS: Physical exercise confirms its effectiveness in reducing symptoms due to PAD. A rehabilitation program performed at home at a specific velocity, just below the PTS, and maintained by a metronome appears to be well suited for HD patients because it induces functional improvements and vascular adaptations with low costs.

Respiratory muscle impairment in dialysis patients: can minimal dose of exercise limit the damage? A Preliminary study in a sample of patients enrolled in the EXCITE trial.

AIM: Skeletal muscle atrophy and dysfunction with associated weakness may involve the respiratory muscles of dialysis patients. We evaluated the effect of moderate-intensity exercise on lung function and respiratory muscle strength. METHODS: Fifty-nine patients (25 F, aged 65 ± 13 years) from two centers participating in the multicenter randomized clinical trial EXerCise Introduction To Enhance Performance in Dialysis (EXCITE) were studied. Subjects were randomized into a prescribed exercise group (E), wherein subjects performed two 10-min walking sessions every second day at an intensity below the self-selected speed, or a control group (C) with usual care. Physical performance was assessed by the 6-min walk test (6MWT). Patient lung function and respiratory muscle strength were evaluated by spirometry and maximal inspiratory pressure (MIP), respectively. RESULTS: Forty-two patients (14 F) completed the study. At baseline, the groups did not differ in any parameters. In total, 7 patients (4 in E; 3 in C) showed an obstructive pattern. The pulmonary function parameters were significantly correlated with 6MWT but not with any biochemical measurements. Group E safely performed the exercise program. At follow-up, the spirometry parameters did not change in either group. A deterioration of MIP (-7 %; p = 0.008) was observed in group C, but not in group E (+3.3 %, p = ns). In E, an increase of 6MWT was also found (+12 vs. 0 % in C; p = 0.038). CONCLUSION: In dialysis patients, a minimal dose of structured exercise improved physical capacity and maintained a stable respiratory muscle function, in contrast to the control group where it worsened.

Evaluation of aortic arch calcification in hemodialysis patients.

BACKGROUND: In the general population, aortic arch calcification (AAC) is related to cardiovascular (CV) disease. Vascular calcifications are common findings in dialysis patients; therefore, we carried out a retrospective study evaluating which risk factors are associated to AAC in stable hemodialysis (HD) patients. METHODS: Standard posterior-anterior chest radiographs, performed the day after the midweek HD session in 132 patients (mean age 65 +/- 12 yrs) who had been on renal replacement therapy (RRT) for 33 months (range 1-471), were analyzed. Cardiothoracic ratio (CTR) was also calculated. RESULTS: AAC was detected in 51% of patients. They were older (68 +/- 8 vs. 62 +/- 14 yrs; p = 0.003), were on RRT for longer (51 (range 2-471) vs. 22 (range 1-195) months; p = 0.0001), had greater CTR (54 (32-71) vs. 50% (40-65); p = 0.034) and higher prevalence of peripheral vascular disease (PVD) (40 vs. 17%; p = 0.049), whilst body weight was lower (62 +/- 14 vs. 68 +/- 14 kg; p = 0.04) than those without AAC. On the contrary, sex, diabetes frequency, smoking habit, history of hypertension and hyperphosphatemia, cerebrovascular and ischemic heart disease (IHD), blood pressure (BP) and antihypertensive therapy, lipids, albumin, degree of anemia, calcium, phosphate and their product were no different between the two groups. Logistic regression analysis showed that age (odds ratio (OR) 1.069 95% confidence interval (95% CI) 1.02-1.11; p = 0.003), length of time on RRT (OR 1.02 95% CI 1.01-1.03; p = 0.0002), calcium-phosphate product (OR 1.03 95% CI 1.007-1.07; p = 0.016), systolic BP (OR 1.03 95% CI 1.005-1.06; p = 0.02) and PVD (OR 3.08 95% CI 1.17-8.06; p = 0.02) were independently associated to AAC. CONCLUSIONS: We conclude that AAC is related to atherosclerosis and to renal failure-related CV risk factors. A careful evaluation of a frequently performed investigation is useful in CV disease risk stratification in HD patients.

Polycystin-1 regulates amphiregulin expression through CREB and AP1 signalling: implications in ADPKD cell proliferation.

In autosomal dominant polycystic kidney disease (ADPKD), renal cyst development and enlargement, as well as cell growth, are associated with alterations in several pathways, including cAMP and activator protein 1 (AP1) signalling. However, the precise mechanism by which these molecules stimulate cell proliferation is not yet fully understood. We now show by microarray analysis, luciferase assay, mutagenesis, and chromatin immunoprecipitation that CREB and AP1 contribute to increased expression of the amphiregulin gene, which codifies for an epidermal growth factor-like peptide, in ADPKD cystic cells, thereby promoting their cell growth. Increased amphiregulin (AR) expression was associated with abnormal cell proliferation in both PKD1-depleted and -mutated epithelial cells, as well as primary cystic cell lines isolated from ADPKD kidney tissues. Consistently, normal AR expression and proliferation were re-established in cystic cells by the expression of a mouse full-length PC1. Finally, we show that anti-AR antibodies and inhibitors of AP1 are able to reduce cell proliferation in cystic cells by reducing AR expression and EGFR activity. AR can therefore be considered as one of the key activators of the growth of human ADPKD cystic cells and thus a new potential therapeutic target.

Henoch-Schönlein purpura due to methicillin-sensitive Staphylococcus aureus bacteremia from central venous catheterization.

A 69-year-old Caucasian man was admitted to our hospital because of myocardial infarction. A central venous catheter (CVC) for infusive therapy was inserted. After two weeks he developed fever, purpura, and knee arthralgia. Hemoculture yielded methicillin-sensitive Staphylococcus aureus. Subsequently, oliguric renal failure, hematuria, and nephrotic range proteinuria were recorded. Renal biopsy showed mesangial proliferation and crescent formation. In an immunofluorescence study, IgA, IgG, and C3 deposition in the mesangium and along arteriolar walls were observed. A diagnosis of Henoch-Schönlein purpura associated with infection caused by CVC was made. After administration of antibiotic and steroid therapy, proteinuria was markedly reduced, renal function improved, and purpura disappeared. The association of HSP with methicillin-resistant Staphylococcus aureus has frequently been reported in the literature. We present here a case of HSP in association with MSSA bacteremia from central venous catheterization, a finding not reported previously.

Ultrastructural pathology of nephropathies with organized deposits: a case series.

Renal organized or structured deposits are much less frequent than those with usual type immunocomplex deposits and are encountered in a wide variety of primary and systemic disorders. It has been suggested that immunoglobulins (Igs) are responsible for organized deposits. We report 5 cases who have been diagnosed and treated in our hospital. Patients were aged 52 to 72 years, three of them were males and had variable degree of renal function, from normal serum creatinine to uraemia. Proteinuria was detected in all patients while monoclonal component was present only in the serum of one subject. Ultrastructural analysis of renal specimens revealed organized deposits. Diagnoses that were made are the following: membranoproliferative glomerulonephritis with finger print, immunotactoid glomerulopathy, membranoproliferative glomerulonephritis with arched deposits, primary amyloidosis and light chain deposition disease. In systemic disorders ultrastructural pathology could be particularly valuable for correct deposits classification, precise localization and pattern of deposition of Igs.

Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report.

INTRODUCTION: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation. CASE PRESENTATION: A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications. DISCUSSION: Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.