Multisystem Amyloidosis in a Coal Miner with Silicosis: Is Exposure to Silica Dust a Cause of Amyloid Deposition?

The over-secretion of monoclonal immunoglobulin light chains by clonal B cells followed by the aggregation and extracellular deposition of fibrillar deposits are responsible forthe clinical course AL amyloidosis. It is well documented that silica significantly increases the number of immunoglobulin-secreting cells. In the present paper, we report on a coal miner with silicosis and fast progressing primary amyloidosis with predominantly heart, kidney, and lung manifestations. Severeheart failure due to myocardial hypertrophy resulted in the patient's death. We conclude that long-term environmental silica exposure and silica deposition may contribute to the development of monoclonal gammopathy and amyloidosis due to chronic stimulus and the dysregulation of the immune system.

The efficacy of single suture for exit site wound closure and stabilization of hemodialysis central tunneled catheter.

INTRODUCTION: The majority of the end-stage renal disease patients begin hemodialysis with the central tunneled catheter as a permanent or bridge vascular access. The procedure of central tunneled catheter insertion can be complicated by exit site bleeding, prolonged tunnel healing, and infection. The study aimed at evaluating whether the catheter exit site wound closing with a single-suture method is equally effective as the double suture method, which is most frequently applied. The assumption was that the single-suture method, which is less traumatizing for the skin, could offer an advantage for the patients with "paper" skin, that is, elderly, after long-term immunosuppressive treatment. METHODS: Insertion of central tunneled catheter with antegrade tunnel formation was performed in 140 patients divided randomly into two groups of 70 patients using either single-suture method or double suture method. RESULTS: Follow-up observations revealed a comparable number of early complications, that is, bleeding or exit site infection, in about 26% of the patients from both groups. No difference appeared in the catheter displacement, either. The percentage of patients with a prolonged central tunneled catheter exit site wound healing was not significantly lower in the single-suture method group: 5.8% versus 11.3%, p = 0.367. There was no impact of single-suture method versus double suture method on the central tunneled catheter long-term function and survival. CONCLUSION: The single-suture method of the central tunneled catheter exit closure is equal in efficacy to the double suture method. Its potential benefit for the patients with an increased risk of poor wound healing should be tested in further study.

The Impact of the Introduction of Innovative REDS Scale for the Evaluation of Central Tunnelled Catheter (CTC) Exit Site on Infection Prevention in Long-Term Haemodialyzed Patients.

Central tunneled catheter (CTC)-related infections are a leading cause of a catheter loss, thus being the source of significant morbidity and mortality. The study aims at evaluating the impact of the implementation of the innovative redness, edema, discharge and tenderness, symptoms (REDS) scale (devised by the authors) for the description of the tunnel condition on the frequency of infection in long-term catheter users. The same cohort of the 40 patients was observed for 4 years altogether: 2 years before and 2 years after REDS application. The results, as well as follow-up evaluation of participants, were compared. The 2-year cumulative incidence of the CTC exit site infection (ESI) dropped significantly (log-rank p < 0.001) from 0.89 episode/1,000 catheter days (53.5%, 95% CI [35.9%; 66.2%]) in the period before REDS was used-to 0.26 episode/1,000 catheter days (18.6%, 95% CI [6.1%; 29.4%]) in the time of REDS application. There were also significantly fewer episodes of ESI complicated with catheter-related blood stream infection (CRBSI) requiring the CTC removal (0.6 episode/1,000 catheter days; 18.6%, 95% CI [6.1%; 29.4%] vs. 0.3 episode/1,000 catheter days; 4.7%, 95% CI [0.0; 10.7%]; log-rank p = 0.04, in pre-REDS and REDS time, respectively). The REDS scale appears to be a simple, cost-effective tool reducing the frequency of the tunneled CTC exit site infection and associated bloodstream infections.

Non-invasive tunnelled catheter reposition (NTCR): A simple and safe method to restore central tunnelled catheter function for haemodialysis.

Despite all efforts, still many end-stage kidney disease (ESKD) patients are dialysed using a central tunnelled catheter (CTC) as vascular access. When the CTC blood flow becomes ineffective, a number of protocols are advised. However, all of them are time- and cost-consuming. The manoeuvre of a non-invasive tunnelled catheter reposition (NTCR) was introduced to restore the CTC function. NTCR was based on gentle movements of the CTC, with or without a simultaneous flushing of the CTC lines, which resulted in a quick reposition of the CTC tip. This study comprises the analysis of a total of 297 NTCRs, which were performed in 114 patients, thus enabling an effective blood flow after 133 procedures (44.7%).Partially effective blood flow followed 123 procedures (41.4%), and it failed altogether in 41 cases (13.9%). Overall, 86% of conducted NTCRs improved the CTC patency to perform a haemodialysis session. The procedure could be successfully repeated, with a similar result after the first and the second attempt. Complications were observed only after 3.4% of all interventions. The novel NTCR manoeuvre was safe and effective in the majority of the CTC dysfunction episodes. It seemed to reduce fibrinolytic usage, allowed an immediate haemodialysis session commencement, therefore, it might save both the costs and the nursing staff time.

Tunnelled Haemodialysis Catheter Removal: An Underappreciated Problem, Not Always Simple and Safe.

BACKGROUND: Optimal care of patients treated with a central tunneled catheter (CTC) as vascular access for hemodialysis requires a number of procedures. One of them is CTC removal, usually carried out using mostly the cut-down method (CDM) and the traction method (TM). The procedure seems to be simple and safe; however, occasionally, serious complications may occur. To eliminate the risk of such events, we have introduced a modified cut-down method (MCDM). METHODS: The study included the analysis of retrospective results of 143 CTC removal procedures, 76 of which were performed using the standard cut-down method (CDM), and in 67 cases, the modified cut-down method (MCDM) was applied. RESULTS: As minor side effects occurred in patients treated with both methods with comparable frequency, serious complications were observed only in the CDM patients group. CONCLUSIONS: In our opinion, the new MCDM procedure is the simplest and safest method of CTC removal.

Difficulties with tunneling of the cuffed catheter: a single-centre experience.

Tunneling of the cuffed catheter for hemodialysis is an important part of insertion procedure with faulty techniques being the cause of catheter dysfunctions. We retrospectively analyzed 737 double-lumen cuffed catheter procedures between 2008 and 2015 in patients aged 60 ± 15years, requiring renal replacement therapy. Complications of tunneling included kinking, bleeding and other problems. In 20 of 737 (2.7%) procedures, the catheter kinked, which was observed in 7.7% of silicone and 0.6% of polyurethane catheters. Repositioning was attempted in 4, but was successful in only 2 cases. Catheter exchange was necessary in 16 cases, but the function was adequate in 2 cases, despite radiological signs of kinking. In 6 cases (1 patient with diabetes, 2 with chest anatomy changes and medical devices, 2 with systemic sclerosis and 1 with greatly enlarged superficial jugular veins) we faced particular difficulties requiring an individual solution by tunneling; these are described in detail. The cumulative catheter patency rate were 69%, 52% and 37% at 3, 6 and 12 months, respectively. In conclusion, the most frequent complication of tunneling was kinking, usually necessitating catheter exchange. The silicon catheter kinked more often than the polyurethane one. An individual approach is sometimes needed by patients with diabetes and anatomical changes of the chest.

[Vascular access in diabetic patients. Are these patients "difficult"?]. / Dostep naczyniowy u chorych na cukrzyce. Czy sa to chorzy problematyczni?

Diabetics with stage V chronic kidney disease (CKD) on hemodialysis (HD) are considered as "difficult patients", because of problems with creation of the vascular access. There is controversy regarding the results and recommendations for preparation of the vascular access in these patients. The aim of this retrospective study was to evaluate the results of creating different types of arteriovenous fistula (AVFs) in consecutive series of patients starting dialysis treatment. The analysis was performed in 741 patients (385 females and 356 males), average age 61.4±7 years, who started dialysis treatment in our department between January 2005 and December 2012. Native AVFs were created in all patients. No patients received an AVF requiring synthetic graft material. The number of patients with diabetic nephropathy was 166 (22.4%). Among them, 30 (18%) had type 1 diabetes and 136 (82%) type 2. In this group the occurrence of calcification in the forearm artery was estimated on the basis of physical examination, Allan's test, Doppler ultrasound and forearm X-ray. In a subgroup of patients with atherosclerotic changes in the arterial system the frequency of failed AVFs was analyzed. These results were compared with the group without diabetes. The number of procedures necessary for successfu AVF creation and type of access was counted in both groups. The assessment of the procedure frequency and AVF location in diabetic and in non-diabetic patients was made by χ² test with Yates correction. In the group of 166 patients with diabetes, in 100 cases (60%) atherosclerotic changes in forearm arteries were observed. In a subgroup of 30 patients with type 1 diabetes atherosclerosis was observed in 17 adults (57%). In this subgroup creation of a suitable forearm AVF in the first procedure in 9 patients was possible and in the other 8 cases the atherosclerotic changes necessitated repeated procedures and were an important obstacle to create the AVF. In the subgroup of 136 patients with type 2 diabetes, atherosclerosis in the forearm artery was observed in 83 cases (61%). In this subgroup the creation of a native AVF was possible in 40 patients during the first procedure and in 43 cases additional intervention was needed, but only in 8 cases was atherosclerosis the cause of fistula failure. Generally, among 166 patients only in 16 cases (10%) did atherosclerosis present an important obstacle for AVF creation, but the number of necessary procedures to create one functioning AVF was significantly greater in this population (2 v 2.7, p=0.0001). A functioning AVF in patients with diabetes was found significantly frequently in the arm localization in comparison to non-diabetic patients (4.3 v 2%, p=0.0478). Atherosclerotic changes in forearm arteries in diabetic patients appear in 60% of hemodialysis patients and make it difficult to create an AVF only in 10% of diabetic patients, but it is possible to create a native AVF in 90% of diabetic patients, although this requires more procedures. In patients with diabetes, AVF in the wrist region should be preferred.

Increased aortic wall stiffness associated with low circulating fetuin A and high C-reactive protein in predialysis patients.

BACKGROUND/AIMS: Vascular calcification and arterial stiffening are cardiovascular risk factors among chronic kidney disease (CKD) patients. The aim of the study was to analyze relationships between inflammatory markers, fetuin A and arterial wall stiffness in CKD patients in the predialysis period and on maintenance dialysis. METHODS: Serum C-reactive protein (hs-CRP), fetuin A, interleukin 6 (IL-6) and other classical markers of atherosclerosis were measured in a group of 155 CKD patients (77 on hemodialysis, HD, 29 on peritoneal dialysis, 49 in CKD stage 5 in the predialysis period) and in 30 healthy volunteers. The aortic pulse wave velocity (aoPWV) was recorded using a tonometric method. RESULTS: The aoPWV, serum hs-CRP and IL-6 were higher and fetuin A levels were lower in all CKD groups than in controls. In multiple regression analysis, the age appeared as the strongest, independent factor increasing arterial wall stiffness in all investigated groups, including controls, whereas the association of aoPWV with IL-6 and fetuin A remained significant only in HD patients. CONCLUSIONS: Aortic wall stiffness is higher in CKD patients than in controls, and it already develops in the predialysis period. Age is the principal determinant of arterial wall stiffness also in CKD patients. The acceleration of arterial wall stiffness in CKD is associated with additional factors, i.e. fetuin A deficiency and higher CRP and IL-6.

IL-18 is involved in vascular injury in end-stage renal disease patients.

BACKGROUND: The role of interleukin (IL)-6 and IL-18 in induction of the inflammatory reaction underlying arteriosclerosis, and protective effect of an anti-inflammatory cytokine IL-10 in this process, have been confirmed by experimental and clinical observations. A systemic inflammatory reaction marker, C-reactive protein (CRP), is known to be associated with the induction of IL-6 and IL-18 release. The chronic inflammatory state associated with renal insufficiency contributes to acceleration of arteriosclerosis, reflected by decreased elasticity which can be measured with aortal pulse wave velocity (PWV). It is well known that chronic kidney disease (CKD) is associated with the chronic inflammatory process, as evidenced by increase in CRP and IL-6 level. It also results in a drop of fetuin-A concentration which is the calcification inhibitor negatively regulated by inflammation. Part of the derangements associated with the progressive renal failure is also the rise of activated monocyte pool, which among others produces IL-18. The aim of the present study was to evaluate, through measurements of CRP, fetuin-A and aortal pulse wave velocity (aoPWV), whether IL-6 and IL-18 affect the arterial wall of CKD patients as a part of general inflammatory process or locally, through their effect on the arterial lesion development. Materials and methods. The study was performed in a group of 102 patients with stage V CKD (73 treated with haemodialysis and 29 treated with continuous ambulatory peritoneal dialysis) (CKD5 group) and in 30 healthy controls. We measured serum high-sensitivity C-reactive protein (hs-CRP), fetuin-A, IL-6, IL-18, IL-10 (ELISA) and others (haemoglobin level, white blood cell count, serum calcium, phosphate, calcium-phosphate product, albumin, fibrinogen, cholesterol, high-density lipoprotein (HDL), triglycerides and parathormone). ECG-gated carotid and femoral artery waveforms were recorded and analysed. RESULTS: Serum levels of hs-CRP, IL-6, IL-10 and IL-18 were higher and fetuin-A levels were lower in the CKD5 group than in controls [6.4 (0.6-22.3) mg/dl versus 2.5 (0.5-5.2) mg/dl; 8.29 pg/ml (0.96-74.48)] versus 2.78 (7.91-0.77) pg/ml; 6.5 (3.7-29.7) pg/ml versus 4.1 (3.8-7.2) pg/ml; 254.4 (468.8-47.5) pg/ml versus 89.3 (91.3-27.5) pg/ml]. The aoPWV was higher in the CKD5 group patients than in the control group (9.4 +/- 1.75 m/s versus 7.76 +/- 1.67 m/s; P < 0.05, respectively). Serum fetuin-A level was negatively associated with hs-CRP and IL-6 but not with IL-18 or IL-10. The aoPWV positively correlated with hs-CRP (r = 0.246; P < 0.05), IL-6 and IL-18 (r = 0.220; P < 0.05) and negatively correlated with fetuin-A (r = -0.204; P < 0.05). No relationship between IL-10 and aoPWV was found. In a multiple regression analysis model respecting inflammatory markers the influence of hs-CRP, IL-18 and fetuin-A on aoPWV remained significant. CONCLUSIONS: The novel observations in the present study are the data indicating that the distinctive contribution of IL-18, but not IL-6, to the arteriosclerosis occurrence in CKD patients, is independent from CRP, fetuin A or other factors involved in the general inflammatory process.

Pulse volume changes recorded by air plethysmography during single hemodialysis sessions.

AIMS: This study investigated whether fluid removal during a hemodialysis (HD) session affects the results of non-invasive pulse volume recording (PVR) on the lower extremities. METHODS: The results of PVR by air plethysmography (Portable Vascular Laboratory; BioMedix, USA) were compared with bioelectrical impedance analysis (Nutriguard-M device; Data Input, Germany) of the human body before and after a single HD in 28 anuric patients (10 women; 18 men) aged 33-76 years. RESULTS: Changes in the wave amplitude of the PVR correlated with the changes in the patient's weight during HD. Lower stroke volume at the end of HD due to blood volume withdrawal resulted in PVR reduction at the ankle level. Significant Spearman coefficients were found between extracellular water and PVR amplitude changes (r = 0.7, p < 0.01) as well as between intracellular water and PVR (r = 0.6, p < 0.04). CONCLUSIONS: To avoid falsification connected with hypervolemia and stroke volume change, the most appropriate time for PVR in vascular diagnosing seems to be the period of a few hours after dialysis session.

Native forearm fistulas utilizing the basilic vein: an underused type of vascular access.

BACKGROUND: Arteriovenous fistulas (AVFs) are the solution of choice among diverse types of vascular access. The forearm basilic vein is rarely used for creating autogenous vascular access. Its use presents a valuable option when autogenous wrist radial-cephalic direct access cannot be created due to the destruction of forearm veins. Results obtained with autogenous wrist ulnar-basilic direct access and autogenous wrist radial-basilic transposition are presented below. METHODS: In the decade 1993-2003, native fistulas utilizing the forearm basilic vein were performed in 27 patients (14 women, 13 men). The basilic vein was anastomosed to the ulnar artery or was transposed and anastomosed to the radial artery. RESULTS: AVF creation was successful in 22 patients (81.5%). The primary patency rate was 70.4% after 1 year, 61.6% after 2 years and 48.4% after 3 years. CONCLUSIONS: AVFs utilizing the forearm basilic vein can be considered for primary or secondary vascular access because of the acceptable survival rate and low incidence of hand ischemia. Transposition of the basilic vein is a valuable option in the reconstruction of a thrombosed or stenosed radial-cephalic fistula.

Arteriovenous fistula reconstruction in patients with kidney allograft failure.

BACKGROUND: Kidney recipients with failing allograft function face the vascular access problem again before returning to hemodialysis. An autologous arteriovenous fistula (AVF), according to the recent Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines, is the optimal vascular access and the use of prosthetic grafts and catheters should be limited. The objective of this study was to assess the feasibility of AVF reconstruction in patients reentering hemodialysis after kidney allograft failure. MATERIALS AND METHODS: Two hundred and forty-one transplant recipients reentered hemodialysis between 1990 and 2005. Before kidney transplantation, 221 patients had a functioning AVF on the forearm. Fistula reconstruction was attempted in 112 (51%) patients because of AVF thrombosis. Three strategies were applied according to forearm vein patency: a new radial-cephalic fistula, a radial-perforating vein fistula, or a radial-basilic forearm transposition was created. RESULTS: Forearm AVFs were successfully reconstructed in 85 of the 112 patients (73%). The primary patency of the reconstructed AVFs was 57.6% and 44% at 12 and 24 months. Secondary patency was 64.9% and 54.9% at 12 and 24 months, respectively. CONCLUSIONS: The reconstruction of an old, thrombosed AVF is possible in kidney recipients returning to dialysis, even if the time from thrombosis to fistula repair is a few years.

Myoglobinuria caused by exertional rhabdomyolysis misdiagnosed as psychiatric illness.

BACKGROUND: Rhabdomyolysis is severe and acute skeletal muscle damage resulting in sarcolemma disruption. During injury, intracellular muscle contents are released into the plasma. The consequences may cause hypovolemia, electrolyte abnormalities, compartment syndrome, or even acute renal failure and dialysis. CASE REPORT: We present the history of a patient in whom exertional rhabdomyolysis was misdiagnosed. A 20-year-old male police recruit was admitted to a psychiatric hospital because of complaints about black urine and severe thigh pain. Serum creatinine kinase (CK) was significantly elevated at 87,335 U/l. Urinalysis showed brown color and cloudiness. Serum myoglobin was also significantly increased. Aspartate aminotransferase was elevated as was alanine aminotransferase. Immediate intravascular fluid hydration and hospital rest under renal, metabolic, and hematological monitoring was performed. CONCLUSIONS: Gymnastic teachers and people at environmental risk of rhabdomyolysis, such as members of the armed forces, police, and supervisors of physical laborers, need to remember the risks of intensive and repetitive exercise. Symptoms such as dark urine, myalgia, and muscle weakness should immediately arouse suspicion of rhabdomyolysis. Especially dark-colored urine should always be investigated for the occurrence of rhabdomyolysis.

Relationship between fetuin-A concentration, elevated levels of inflammatory markers, and arterial wall stiffness in end-stage kidney disease.

The risk of cardiovascular mortality is significantly heightened in chronic dialysis patients. Aortic wall stiffness, as reflected by aortic pulse-wave velocity (PWV), is a strong predictor of cardiovascular events. Loss of the aortic wall's elasticity is accelerated in dialysis patients because of calcifying medial arteriosclerosis, an active cellular process, controlled by calcification inducers and inhibitors. A pivotal role in the inhibition of calcium x phosphorus (Ca x P) precipitation is played by fetuin-A, a circulating plasma glycoprotein. In hemodialysis patients, lower fetuin-A concentrations were associated with increases in both cardiovascular and overall mortality. In our own study, a significant negative correlation was established between fetuin-A level and aortic PWV in chronic hemodialysis patients. The arterial-stiffening process was unaffected by the Ca x P product, but occurred independent of elevated interleukin-6 levels.

Obesity is not an obstacle for successful autogenous arteriovenous fistula creation in haemodialysis.

BACKGROUND: Obesity, which is often associated with diabetes, is increasingly encountered in the haemodialysed population, and this may produce difficulty in autogenous arteriovenous fistula creation. Prosthetic angioaccess or catheters, when used in place of autogenous fistulas, increase thrombotic and infectious complications in these already challenged patients. METHODS: This prospective study was undertaken to assess the feasibility of autogenous arteriovenous fistula creation in 71 obese patients (BMI 34.6 +/- 7.8). We performed a two-stage procedure, in which radio-cephalic fistula formation was followed by subcutaneous transposition of the venous component for safe and easy puncture. RESULTS: Fistulas suitable for puncture, having blood flows of 799 +/- 285 ml/min, and sufficient to perform adequate haemodialysis (Kt/V 1.24) were achieved in 85% of the patients. Primary patency rates were 65% and 59% at 6 and 12 months, respectively, and secondary patency rates were 83% both at 6 and 12 months. CONCLUSIONS: Obesity does not prevent successful autogenous arteriovenous fistula formation, and may protect forearm venous vessels from the iatrogenic damage that occurs before the onset of haemodialysis therapy.

Radial artery-perforating vein fistula for hemodialysis.

BACKGROUND: The proximal forearm antecubital fistula described by Gracz is a valuable option for autogenous vascular access for hemodialysis in patients with destroyed forearm veins or advanced arteriosclerotic and calcified radial arteries. Results obtained with a variant of the Gracz fistula are presented. STUDY DESIGN: Patients with forearm vein destruction or failed distal radiocephalic fistulas were selected to have a variant of the Gracz fistula created and were followed up for 36 months. In each patient, the radial artery was anastomosed side to end or end to end to the perforating vein. Additionally, in some patients, the median cephalic or basilic vein was relocated subcutaneously to increase the accessibility of veins for puncture. SETTING & PARTICIPANTS: Native arteriovenous fistulas (AVFs) in the cubital region using a perforating vein were created in 77 patients (34 women, 43 men) referred to the Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Poland, from 1992 to 2006. OUTCOMES & MEASUREMENTS: Percentages of successful fistula creation and primary patency rates, defined from fistula placement to any maintaining intervention, and cumulative patency, defined from placement to fistula abandonment, were assessed. RESULTS: AVF creation was successful in 56 patients (73%). Primary patency rates during the follow-up period were 47% after 1 year, 43% after 2 years, and 39% after 3 years. Cumulative patency rates were 67% after 1 year, 56% after 2 years, and 53% after 3 years. LIMITATIONS: These results reflect performance of a single center and thus may not be generalizable to surgeons less experienced in this technique. CONCLUSIONS: Radial artery-perforating vein fistulas have an acceptable survival rate and do not produce circulatory complications. This method may be applicable for AVF creation in patients with forearm vein destruction/abnormalities and as a rescue procedure for an old clotted fistula after kidney transplant failure.

[Hypertension in haemodialysis and peritoneal dialysis patients]. / Nadcisnienie tetnicze u chorych w programach hemodializy i dializy otrzewnowej.

Hypertension occurs in many reports in 80-100% ESRD patients and is the most important risk factor of cardiovascular morbidity and mortality. Successful therapy of hypertension depends on many factors including specific, pathophysiological mechanisms, ability of optimal renal replacement therapy and pharmacotherapy in the circumstances of different drugs pharmacokinetics. The review is aimed on recent reports concerning the role of optimal dialysis especially dose, duration of the procedure and some pharmacological aspects of blood pressure control in ESRD patients.