Long-term peritoneal dialysis followed by kidney transplantation for Takayasu arteritis: a case report.

BACKGROUND: Takayasu arteritis (TA) is a chronic vasculitis of unknown etiology that primarily affects large vessels. Although renal involvement is frequent in TA, patients with TA undergoing renal replacement therapy, especially long-term peritoneal dialysis (PD) and kidney transplantation (KTx), are rarely reported. We herein present the case of an elderly patient with TA treated by PD for more than 5 years and underwent KTx thereafter. CASE PRESENTATION: A 69-year-old female diagnosed with TA at the age of 19 was treated by PD for seven and a half years for end-stage renal disease due to TA. Dialysate-to-plasma ratio of creatinine, which was well maintained during this period, reflected the efficacy of long-term PD. However, her residual renal function declined; she developed malnutrition, inflammation, and atherosclerosis syndrome and underwent living-related KTx from her husband. Due to the total occlusion of the external iliac arteries with compensatory development of the internal iliac arteries, the right internal iliac artery was used as the anastomosis site. After KTx, the patient developed chronic active antibody-mediated rejection; however, the graft function was maintained throughout the follow-up period. Despite severe aortic calcification and intermittent claudication in the legs, her condition did not worsen, and the blood flow of the graft was preserved. CONCLUSIONS: The current case illustrating the success of long-term PD and living-related KTx in maintaining kidney function in an elderly patient with TA is the first to demonstrate the potential of PD and KTx as feasible options for renal replacement therapy in TA accompanied by severe cardiac involvement.

Continuous monitoring of blood pressure by analyzing the blood flow sound of arteriovenous fistula in hemodialysis patients.

BACKGROUND: Patients with end-stage renal disease undergoing hemodialysis (HD) have an elevated risk of cardiovascular disease-related morbidity and mortality. To prevent from such a life-threatening event, the continuous blood pressure (BP) monitoring system may contribute to detect BP decline in early stages and may help to do appropriate disposal. Our research team has introduced an electronic stethoscope (Asahi Kasei Co, Ltd., Tokyo, Japan), which translates sound intensity of Arteriovenous Fistula (AVF) to BP data using the technique of Fourier transformation that can predict continuous BP non-invasively. This study, we investigated whether electronic stethoscope-guided estimated BP (e-BP) would actually reflect systolic BP measured by sphygmomanometer (s-BP), and whether e-BP could predict fall of BP during HD. METHODS: Twenty-six patients who underwent HD treatment in our hospital were evaluated prospectively. We obtained sound intensity data from the electronic stethoscope which was equipped with the return line of HD. Then, the data were translated into e-BP data to be compared with s-BP. Correlation of total of 315 data sets obtained from each method was examined. An accuracy of diagnosis of intra-dialytic hypotension (IDH) was evaluated. RESULTS: Total of 315 data sets were obtained. A close correlation was observed between e-BP and s-BP (r = 0.887, p < 0.0001). Sensitivity and positive predictive value of predicted-BP for detection of IDH was 90 and 81.3%, respectively. CONCLUSIONS: Electronic stethoscope-guided BP measurement would be helpful for real-time diagnosis of BP fall in HD patients. Further investigations are needed.

Spot Urine-guided Salt Reduction in Chronic Kidney Disease Patients.

OBJECTIVE: Dietary salt restriction is important in patients with chronic kidney disease (CKD) to reduce hypertension, cardiovascular events, progression of CKD, and mortality. However, recommending salt reduction for patients is difficult without knowing their actual sodium intake. This study evaluated the effectiveness of spot urine-guided salt reduction in CKD outpatients. DESIGN: A prospective cohort study was used. SUBJECTS: This study included a total of 127 adult outpatients (aged 60 ± 18 years, 80 males) with CKD. Their baseline estimated glomerular filtration rate was 51.4 ± 25.1 (mL/minute/1.73 m2), and 64 (50%) of them were with CKD stage 3a or 3b (both 32 [25%]). INTERVENTION: We informed the patients of their individual spot urine-estimated salt intake every time they visited the outpatient clinic. Based on the data, the nephrologist encouraged the patients to achieve their salt restriction goal. MAIN OUTCOME MEASURE: The primary outcome was the estimated salt excretion, and the secondary outcome was the urinary protein-to-Cr ratio (UPCR). Multiple regression analyses were performed to clarify the contributing factors of changes in both outcomes. RESULTS: Over a follow-up of 12 months, the median number of patients' visits was 7 (5-8). The estimated salt intake was significantly reduced from 7.98 ± 2.49 g/day to 6.77 ± 1.77 g/day (P < .0001). The median UPCR was also reduced from 0.20 (0.10-0.80) to 0.10 (0.10-0.48) (P < .0001). On multiple regression analysis, a reduction in UPCR was positively associated with the baseline UPCR and a reduction in systolic blood pressure significantly (P < .0001 and P < .01, respectively) as well as positively correlated with a reduction in the estimated salt intake, with borderline significance (P = .08). CONCLUSIONS: Providing spot urine-estimated salt intake feedback effectively motivated CKD patients to reduce their salt intake. Spot urine-guided salt reduction may slow CKD progression through decreased urinary protein excretion.

Seasonal variation in predialysis systolic blood pressure and cardiovascular events in patients on maintenance hemodialysis.

Predialysis systolic blood pressure (SBP) in patients on hemodialysis (HD) consistently followed a seasonal pattern, reaching a peak in winter and nadir in summer, similar to blood pressure in the general population. However, the relationship between seasonal variations in predialysis SBP and clinical outcomes is still under-investigated in Japanese patients on HD. This retrospective cohort study included 307 Japanese patients undergoing HD for >1 year in three dialysis clinics and evaluated the association between the standard deviation (SD) of predialysis SBP and clinical outcomes, including major adverse cardiovascular events (MACEs; cardiovascular death, nonfatal myocardial infarction or unstable angina, stroke, heart failure, and other severe cardiovascular events requiring hospitalization) with 2.5 years follow-up. The SD of predialysis SBP was 8.2 (6.4-10.9) mmHg. In the model fully adjusted for the SD of predialysis SBP, predialysis SBP, age, sex, HD vintage, Charlson comorbidity index, ultrafiltration rate, renin-angiotensin system inhibitors, corrected calcium, phosphorus, human atrial natriuretic peptide, C-reactive protein, albumin, hemoglobin, body mass index, normalized protein catabolism rate, and intradialytic SBP decline, Cox regression analyses showed that a higher SD of predialysis SBP (per 10 mmHg) was significantly associated with increased MACE risk (hazard ratio [HR], 1.89; 95% confidence interval [95% CI], 1.07-3.36) and all-cause hospitalization (HR, 1.57; 95% CI, 1.07-2.30). Therefore, greater seasonal variations in predialysis SBP were associated with worse clinical outcomes, including MACEs and all-cause hospitalization. Whether interventions to reduce seasonal variations in predialysis SBP will improve the prognosis of Japanese patients on HD must be investigated further.

Mean annual intradialytic blood pressure decline and cardiovascular events in Japanese patients on maintenance hemodialysis.

An intradialytic systolic blood pressure (SBP) decline, which defines intradialytic hypotension, may be associated with higher all-cause mortality. However, in Japanese patients on hemodialysis (HD), the association between intradialytic SBP decline and patient outcomes is unclear. This retrospective cohort study included 307 Japanese patients undergoing HD over 1 year in three dialysis clinics and evaluated the association between the mean annual intradialytic SBP decline (predialysis SBP-nadir intradialytic SBP) and clinical outcomes, including major adverse cardiovascular events (MACEs; cardiovascular death, nonfatal myocardial infarction or unstable angina, stroke, heart failure, and other severe cardiovascular events requiring hospitalization) by following up for 2 years. The mean annual intradialytic SBP decline was 24.2 (25-75th percentile, 18.3-35.0) mmHg. In the model fully adjusted for intradialytic SBP decline tertile group (T1, <20.4 mmHg; T2, 20.4 to <29.9 mmHg; T3, ≥29.9 mmHg), predialysis SBP, age, sex, HD vintage, Charlson comorbidity index, ultrafiltration rate, use of renin-angiotensin system inhibitors, corrected calcium, phosphorus, human atrial natriuretic peptide, geriatric nutritional risk index, normalized protein catabolism rate, C-reactive protein, hemoglobin, and use of pressor agents, Cox regression analyses showed that the hazard ratio (HR) was significantly higher for T3 than for T1 for MACEs (HR, 2.38; 95% confidence interval 1.12-5.09) and all-cause hospitalization (HR, 1.68; 95% confidence interval 1.03-2.74). Therefore, in Japanese patients on HD, a greater intradialytic SBP decline was associated with worse clinical outcomes. Further studies are warranted to investigate whether interventions to attenuate the intradialytic SBP decline will improve the prognosis of Japanese patients on HD.

The effect of trichlormethiazide in autosomal dominant polycystic kidney disease patients receiving tolvaptan: a randomized crossover controlled trial.

The vasopressin V2 receptor antagonist tolvaptan delays the progression of autosomal dominant polycystic kidney disease (ADPKD). However, some patients discontinue tolvaptan because of severe adverse aquaretic events. This open-label, randomized, controlled, counterbalanced, crossover trial investigated the effects of trichlormethiazide, a thiazide diuretic, in patients with ADPKD receiving tolvaptan (n = 10) who randomly received antihypertensive therapy with or without trichlormethiazide for 12 weeks. The primary and secondary outcomes included amount and osmolarity of 24-h urine and health-related quality-of-life (HRQOL) parameters assessed by the Kidney Disease Quality of Life-Short Form questionnaire, renal function slope, and plasma/urinary biomarkers associated with disease progression. There was a significant reduction in urine volume (3348 ± 584 vs. 4255 ± 739 mL; P < 0.001) and a significant increase in urinary osmolarity (182.5 ± 38.1 vs. 141.5 ± 38.1 mOsm; P = 0.001) in patients treated with trichlormethiazide. Moreover, trichlormethiazide improved the following HRQOL subscales: effects of kidney disease, sleep, emotional role functioning, social functioning, and role/social component summary. No significant differences were noted in renal function slope or plasma/urinary biomarkers between patients treated with and without trichlormethiazide. In patients with ADPKD treated with tolvaptan, trichlormethiazide may improve tolvaptan tolerability and HRQOL parameters.

Sarcomatoid renal cell carcinoma with autosomal dominant polycystic kidney disease: a case report and literature review.

Diagnosis of renal cell carcinoma (RCC) in patients with autosomal dominant polycystic kidney disease (ADPKD) is challenging and often delayed due to accompanying multiple renal cysts. Sometimes, it is difficult to distinguish RCC from cyst infection or hemorrhage. We herein present the case of a patient with ADPKD undergoing long-term hemodialysis whose sarcomatoid RCC was difficult to diagnose and was confirmed via nephrectomy. A 53-year-old male, undergoing hemodialysis since 20 years for end-stage renal disease secondary to ADPKD, was admitted to our hospital with a 3-week history of fever at > 38 °C and right flank pain. Clinical manifestations were compatible with cyst infection. Magnetic resonance images of the lesion identified in the lower right kidney, revealing slightly high signal intensity on T1-weighted images, low signal intensity on T2 weighted images, and restricted diffusion on diffusion-weighted images, were also consistent with those of cyst infection. Therefore, antibiotic therapy with ciprofloxacin, doripenem, and vancomycin was initiated. However, the patient's symptoms did not improve. Consequently, right nephrectomy was performed for both diagnosis and treatment, which revealed a sarcomatoid RCC with metastasis to the regional lymph node. The patient gradually developed cachexia and died on day 106 post-admission. The present case illustrates the difficulty of diagnosing RCC in patients with ADPKD, particularly sarcomatoid RCC, which is a rare and aggressive variant of RCC, even with the use of various types of imaging modalities. An early decision of nephrectomy may be necessary in such cases.

Seasonal variation and predictors of intradialytic blood pressure decline: a retrospective cohort study.

The risk factors for intradialytic systolic blood pressure decline remain poorly understood. We aimed to identify clinical and laboratory predictors of the intradialytic systolic blood pressure decline, considering its seasonal variation. In a retrospective cohort of 47,219 hemodialysis sessions of 307 patients undergoing hemodialysis over one year in three dialysis clinics, the seasonal variation and the predictors of intradialytic systolic blood pressure decline (predialysis systolic blood pressure--nadir intradialytic systolic blood pressure) were assessed using cosinor analysis and linear mixed models adjusted for baseline or monthly hemodialysis-related variables, respectively. The intradialytic systolic blood pressure decline was greatest and least in the winter and summer, respectively, showing a clear seasonal pattern. In both models adjusted for baseline and monthly hemodialysis-related parameters, calcium channel blocker use was associated with a smaller decline (-4.58 [95% confidence interval (CI), -5.84 to -3.33], P < 0.001; -3.66 [95% CI, -5.69 to -1.64], P < 0.001) and α blocker use, with a greater decline (3.25 [95% CI, 1.53-4.97], P < 0.001; 3.57 [95% CI, 1.08-6.06], P = 0.005). Baseline and monthly serum phosphorus levels were positively correlated with the decline (1.55 [95% CI, 0.30-2.80], P = 0.02; 0.59 [95% CI, 0.16-1.00], P = 0.007), and baseline and monthly normalized protein catabolic rates were inversely correlated (respectively, -22.41 [95% CI, -33.53 to -11.28], P < 0.001; 9.65 [95% CI, 4.60-14.70], P < 0.001). In conclusion, calcium channel blocker use, α blocker avoidance, and serum phosphorus-lowering therapy may attenuate the intradialytic systolic blood pressure decline and should be investigated in prospective trials.

Uremic encephalopathy in patients undergoing assisted peritoneal dialysis: a case series and literature review.

We sometimes hesitate to switch renal replacement therapy from peritoneal dialysis (PD) particularly in elderly patients due to their physical tolerance levels and lifestyles. Here, we describe the cases of three patients treated with PD alone despite an anuric status who subsequently developed uremic encephalopathy, which was successfully treated with hemodialysis (HD). The first patient was a 75-year-old woman who developed uremic encephalopathy with an anuric status and inadequate PD after 7 months of treatment. HD immediately improved her condition; encephalopathy did not recur with combined therapy of PD and HD. The second patient was a 69-year-old woman who developed anuria and was treated with combined therapy. Her arteriovenous fistula was obstructed; therefore, she was treated with PD alone. Total weekly Kt/V was sufficiently high at 1.95; however, she developed uremic encephalopathy the following month, which was successfully treated with HD. The third patient was an 84-year-old woman who developed anuria, but was treated with PD alone with adequate total weekly Kt/V of 2.2. PD could not be performed for 2 days because of myocardial infarction intervention; subsequently, she developed uremic encephalopathy, which was successfully treated with HD. These cases are the first of their kinds, wherein patients undergoing PD, developed uremic encephalopathy without any obvious triggers, including drugs, and illustrate the necessity of initiating combined therapy for such patients considering the risk of developing severe uremia leading to uremic encephalopathy, in spite of it being less preferable for elderly patients due to their physical conditions and lifestyles.

Baseline and Time-Averaged Values Predicting Residual Renal Function Decline Rate in Japanese Peritoneal Dialysis Patients.

Residual renal function (RRF) is a strong prognostic factor of morbidity and mortality in patients undergoing peritoneal dialysis (PD). We determined predictors of the RRF rate of decline using both baseline values and time-averaged ones. We retrospectively analyzed 94 patients being treated with PD at the Japanese Red Cross Medical Center. The decline rate of RRF was calculated by a diminution in the weekly renal Kt/V between the first and last follow up divided by follow-up years. The mean follow-up period was 2.28 years, and the mean decline rate of weekly renal Kt/V was 0.25 per year. A multivariate analysis using baseline parameters identified dialysis-to-plasma ratios of creatinine at 4 h (P = 0.02), urinary protein (P = 0.02), and mean blood pressure (MBP) (P < 0.01) as being positively associated with the RRF rate of decline, while the use of angiotensin converting enzyme inhibitor (ACEI)/angiotensin receptor blocker (ARB) had a negative correlation (P = 0.03). When using time-averaged values as independent variables, a lower weekly total renal Kt/V (P < 0.0001), higher urinary protein (P < 0.0001), and higher MBP (P = 0.04) independently predicted a faster RRF rate of decline. We demonstrated that PD patients with a lower MBP and lower urinary protein both at baseline and throughout their PD duration had a slower RRF rate of decline. We recommend strict control of blood pressure and anti-proteinuric therapy for PD patients.