Skin Perfusion Pressure Outperforms Ankle-Brachial Index in Predicting Mortality and Cardiovascular Outcomes in Hemodialysis Patients.

AIMS: Skin perfusion pressure (SPP) and ankle-brachial index (ABI) are useful in screening for peripheral arterial disease in patients undergoing hemodialysis (HD). We compared the prognostic abilities of the SPP and ABI in predicting the composite outcomes of mortality and atherosclerotic vascular events. METHODS: This single-center prospective cohort study enrolled 258 patients undergoing HD. The patients with SPP and ABI measurements were divided into tertiles. Log-rank tests, Cox regression analyses, and discrimination parameters were used for comparisons. RESULTS: Over a median follow-up period of 3.7 (1.4-5.0) years, 119 composite events were recorded. The incidence rates of composite events were 27.5, 13.3, and 9.1 per 100 person years, respectively, across the SPP tertiles (log-rank: p＜0.001), and 23.2, 13.2, and 11.6 per 100 person years across the ABI tertiles (p=0.003). With the 3rd tertiles as references, the 1st tertiles of the SPP and ABI were significantly associated with the composite outcome (adjusted hazard ratio [aHR]: 2.58, 95% confidence interval [CI]: 1.57-4.23 and aHR: 1.70, 95% CI: 1.06-2.73, respectively). Adding the tertiles of the SPP to a predictive model with established risk factors significantly improved the model performance. This improvement was larger than that of the ABI in terms of net reclassification (0.330 vs. 0.275) and integrated discrimination (0.045 vs. 0.012). Furthermore, in patients with a normal ABI, the 1st SPP tertile (＜71 mmHg) was significantly associated with the outcome (aHR, 1.97; 95% CI, 1.13-3.41) when compared to the 3rd tertile. CONCLUSIONS: Even patients with a normal ABI have a poor prognosis if their SPP levels are low. SPP outperformed ABI in predicting mortality and cardiovascular outcomes in HD patients.

Nephrology referral slows the progression of chronic kidney disease, especially among patients with anaemia, diabetes mellitus, or hypoalbuminemia: A single-centre, retrospective cohort study.

BACKGROUND: The Kidney Disease Improving Global Outcomes guidelines recommend nephrology referral for patients with chronic kidney disease (CKD) stages 4 to 5, significant proteinuria and persistent microscopic haematuria. However, the recommendations are opinion-based and which patients with CKD benefit more from nephrology referral has not been elucidated. METHODS: In this retrospective cohort study, patients referred to our nephrology outpatient clinic from April 2017 to March 2019 were included. We excluded patients considered to have an acute decline in kidney function (annual decline in estimated glomerular filtration rate [eGFR] >10 mL/min/1.73 m2). The slopes of eGFR before and after nephrology referral were estimated and compared by linear mixed effects models. Interaction between time and referral status (before or after referral) was assessed and effect modifications by the presence of diabetes, proteinuria (defined by urine dipstick protein 2+ or more), urine occult blood, hypoalbuminemia (defined by albumin levels less than 3.5 g/dL) and anaemia (defined by haemoglobin levels less than 11.0 g/dL) were evaluated. RESULTS: The eGFR slope significantly improved from -2.05 (-2.39 to -1.72) to -0.96 (-1.36 to -0.56) mL/min/1.73 m2/year after nephrology referral (p < .001). The improvement in eGFR slope was more prominent among those with diabetes mellitus, anaemia, and hypoalbuminemia (all p-values for three-way interaction <.001 after adjustment for covariates). Further adjustments for time-dependent haemoglobin levels, the use of erythropoiesis-stimulating agents, iron supplementation, anti-hypertensives and anti-diabetic medications did not change the significance of the interactions. CONCLUSIONS: Nephrology referral slows CKD progression, especially among those with hypoalbuminemia, diabetes or anaemia. Patients with hypoalbuminemia, diabetes or anaemia might benefit more from specialized care and lifestyle modifications by nephrologists. The inclusion of anaemia and hypoalbuminemia in nephrology referral criteria should be considered.

Autopsy Findings of Heterozygous Fabry Disease with the Severe Phenotype: A Case Report.

Fabry disease (FD) is an inherited X-linked lysosomal storage disorder, with hemizygous males being more severely affected than heterozygous females. Herein, we report a rare case of FD in a heterozygous female with a severe phenotype. The patient had obesity and hyperlipidemia and had her first cerebral infarction at the age of 33 years. She underwent renal biopsy and was diagnosed with FD with morphological features of focal segmental glomerulosclerosis nephropathy at the age of 34 years. Her leukocyte alpha-galactosidase A activity was 2.3 Agal/U (normal: >20 Agal/U), and genetic analysis revealed the presence of the classical phenotype. Enzyme replacement therapy (ERT) was initiated at the age of 35 years; however, peritoneal dialysis owing to end-stage renal failure occurred at the age of 37 years. The patient died of a cerebral hemorrhage at the age of 44 years. Her Mainz Severity Score Index at the time of death was 48/76, suggestive of the severe phenotype. Autopsy findings revealed remarkable globotriaosylceramide accumulation on electron microscopy, particularly in the major organs and their vascular smooth muscle cells. Regarding the vertebral arteries which sourced the cerebral hemorrhage, the effects of FD-induced vascular thickening and long-term renal failure-induced atherosclerosis were confirmed. Furthermore, the patient's vascular sclerosis was modified with acquired factors such as lifestyle-related disease associated with obesity. We recommend intensified treatment for metabolic factors simultaneous with ERT to help in delaying the progression of FD.

Association between conscientiousness and peritoneal dialysis-related peritonitis.

AIM: Peritonitis is a critical complication in patients undergoing peritoneal dialysis. Patient characteristics, especially conscientiousness, potentially influence self-care activity. This study aimed to examine the relationship between the peritonitis risk and the patient conscientiousness. METHODS: This retrospective observational study was conducted at three Japanese medical centres in 2018. Adult patients undergoing peritoneal dialysis were evaluated for conscientiousness with the Japanese version of the Ten-Item Personality Inventory. We followed the patients from peritoneal dialysis induction retrospectively on chart review. The primary outcome was time to first episode of peritonitis after initiation of peritoneal dialysis in the entire observation period. Hazard ratios were estimated using a Cox model adjusted for potential confounders. RESULTS: Among 111 patients enrolled, 32 experienced peritonitis. The patients were divided into the more conscientious group (n = 64) and less conscientious group (n = 47) based on a mean conscientiousness score of 3.991. Additionally, less conscientious patients tended to be at higher risk for peritonitis during the entire study period (hazard ratio adjusted for age and sex, 1.99; 95% confidence interval, 0.98-4.03; p = .057). The increased risk in the less conscientious group was statistically significant in the first year (adjusted hazard ratio, 6.98; 95% confidence interval, 1.42-34.40; p = .017). CONCLUSION: Less conscientious patients significantly have shorter peritonitis-free survival in the early phase after peritoneal dialysis induction although not significantly during the entire study period. Personality trait assessment using the Ten-Item Personality Inventory could be helpful in educating and following up patients undergoing peritoneal dialysis.

Low plantar skin perfusion pressure predicts long-term atherosclerotic vascular events and mortality in maintenance haemodialysis patients.

BACKGROUND AND AIMS: End-stage renal disease is associated with an increased risk of atherosclerotic vascular disease (AVD). We investigated whether low plantar skin perfusion pressure (SPP), a useful indicator of peripheral arterial disease (PAD), predicts systemic AVD events and mortality in outpatients undergoing maintenance haemodialysis (HD). METHODS: A total of 206 HD patients were enrolled and followed for 5 years. They were divided into 3 groups according to measured SPP: group 1 (G1), SPP >70 mmHg (n = 123); G2, SPP 50-70 mmHg (n = 61); and G3, SPP <50 mmHg (n = 22). RESULTS: During the follow-up period (median, 4.2 years), 56 AVD events (27.2%) and 68 deaths (33.0%) occurred. In G1, G2, and G3, the event-free survival rates were 74%, 55% and 19%, respectively, for AVD events (p < 0.01) and 73%, 54% and 26%, respectively, for mortality (p < 0.01). A Cox multivariate analysis showed that lower SPP was an independent predictor for AVD events [hazard ratio (HR) 3.12, 95% confidence interval (CI) 1.45-6.77, p < 0.01 for G3 vs. G1] and mortality (HR 3.06, 95% CI 1.57-5.98, p < 0.01 for G3 vs. G1). Furthermore, the addition of the SPP value to a model with established risk factors improved the predictability of increasing the net reclassification improvement (NRI; 0.463, p < 0.01) and integrated discrimination improvement (IDI; 0.039, p < 0.01) for AVD events. Similar results were obtained for mortality. CONCLUSIONS: Low plantar SPP can stratify risk and improve the predictability of both systemic AVD events and mortality in the maintenance HD population.

Skin Perfusion Pressure and the Prevalence of Atherothrombosis in Hemodialysis Patients.

End-stage renal disease is associated with atherothrombosis (ATIS), which, in turn, can promote peripheral arterial occlusive disease (PAOD), coronary artery disease (CAD), and/or cerebrovascular disease (CVD). The aim of this study was to determine whether low plantar skin perfusion pressure (SPP) was related to ATIS among 122 patients receiving maintenance hemodialysis (HD) from March to November 2013 at our outpatient facility. We routinely measured SPP and used the value for analysis. In addition, we retrospectively evaluated the prevalence of ATIS with patients categorized to CAD, CVD, or PAOD groups. Of the 122 outpatients, ATIS was diagnosed in about half (N = 60, 49.2% vs. 62, 50.8%; average SPP, 56.6 vs. 72.9 mm Hg, respectively). These data show that SPP was significantly lower in patients with ATIS (difference, 16.3 mm Hg; P < 0.001) and there was a negative relationship between average SPPs and past history of ATIS complications. When the patients were stratified by the presence of diabetes mellitus, this trend was stronger. Particularly, receiver operating characteristic analysis of HD patients with diabetes revealed a cutoff point of 53.0 mm Hg and an area under the curve value of 0.84, with a sensitivity of 77.0% and specificity of 91.3%. Therefore, we concluded that SPP enables the evaluation of not only local PAOD, but also systemic ATIS. Moreover, we found that a cutoff point of 53.0 mm Hg was useful for detection of ATIS in HD patients.

Large duodenal somatostatinoma in the third portion associated with severe glucose intolerance.

A 51-year-old man was admitted with hyperglycemia and a duodenal tumor. Although his glycemic control was poor, basal C-peptide levels were not suppressed. Further examination revealed a mass measuring 7.8 cm in diameter in the third portion of the duodenum. Duodenectomy revealed a slow-growing sessile tumor located near Treitz's ligament. The immunohistochemical profile of sections of the specimen revealed the presence of somatostatin. The patient's serum somatostatin was elevated to 300 pg/ml preoperatively, but was reduced to 10 pg/ml postoperatively. Glycemic control also normalized after the operation.