Safety and efficacy of preauricular fistulectomy with fascia-anchoring suture technique: A large case series.

OBJECTIVE: This study aimed to analyze the safety and efficacy of preauricular fistulectomy with fascia-anchoring suture technique through large case series. In addition, differences in surgical outcomes according to preoperative status and age were investigated. METHODS: In this retrospective study, 380 patients (450 ears) with preauricular fistula (PAF) who underwent preauricular fistulectomy with fascia-anchoring suture technique by a single surgeon (E.P) were enrolled. Patients were divided into fresh, previous incision and drainage (I&D), and the revision surgery groups according to the preoperative status. Additionally, they were divided into adult and pediatric groups according to age. Patient's demographics, postoperative infections, and recurrence rates were analyzed. RESULTS: The mean age of the patients was 28.3 years, and there were 119 males and 261 females. Out of 450 PAFs (n = 281 in the fresh groups, n = 119 in the previous I&D groups, and n = 50 in the revision groups), 21 (4.7 %) cases had postoperative infections and 12 (2.7 %) cases had recurrence. There was no difference in postoperative infections, regardless of the preoperative condition (I&D group, p = 0.701; revision group, p = 0.658). The recurrence rate was higher in the revision group than in the fresh and I&D groups (p = 0.004). There was no significant difference in postoperative infection (p = 0.221) or recurrence (p = 0.161) between adults and children. CONCLUSIONS: The study found that performing preauricular fistulectomy with a fascia-anchoring suture technique led to low rates of postoperative infections and recurrences. These positive outcomes were consistent across different patient groups categorized by preoperative status and age, indicating the technique's safety and effectiveness for all patients with preauricular fistulas.

Revisiting glomerular hyperfiltration and examining the concept of high dietary protein-related nephropathy in athletes and bodybuilders.

PURPOSE OF REVIEW: High-protein diets (HPDs) are popular but their consequences for kidney health, especially among athletes and bodybuilders who typically maintain a high protein intake for a long time, have not been investigated. This review focused on recent studies of the association of HPD with long-term kidney health and the concept of high dietary protein-related nephropathy. RECENT FINDINGS: Several long-term observational studies including large populations have reinforced the notion that HPDs are associated with a rapid decline of kidney function. An increase in renal blood flow and glomerular hyperfiltration caused by vasodilation, and increased levels of endocrine and paracrine factors (glucagon, IGF-1, prostanoids, and nitric oxide), facilitates the excretion of protein-derived nitrogenous waste. Inhibition of tubule-glomerular feedback and increased proximal tubular Na+ reabsorption after a HPD augment glomerular hyperfiltration and may trigger synthesis of proinflammatory cytokines and receptor for advanced glycation end-products (RAGE). Focal segmental glomerulosclerosis reported in association with anabolic steroid may indeed be a HPD nephropathy given that HPD results in progressive glomerulosclerosis, especially in remnant glomeruli or in diabetic kidney disease but can happen in any high-risk situation, such as solitary kidney and polycystic kidneys. SUMMARY: HPD among athletes and bodybuilders in an extreme way across a long-term period may pose a risk to renal health including high incidence of HPD nephropathy.

Prediction of Heart Function and Volume Status in End-Stage Kidney Disease Patients through N-Terminal Pro-Brain Natriuretic Peptide.

Background and Objectives: N-terminal pro-brain natriuretic peptide (NT-proBNP) is a biomarker used to predict heart failure and evaluate volume status in hemodialysis (HD) patients. However, it is difficult to determine the cutoff value for NT-proBNP in HD patients. In this study, we analyzed whether NT-proBNP helps predict heart function and volume status in HD patients. Materials and Methods: This prospective observational study enrolled 96 end-stage kidney disease patients with HD. All patients underwent echocardiography and bioimpedance spectroscopy (BIS) after an HD session. Overhydration (OH) was measured by BIS. Laboratory data were obtained preHD, while serum NT-proBNP was measured after HD. Interventions for blood pressure control and dry weight control were performed, and NT-proBNP was re-assessed after a month. Results: There was an inverse correlation between NT-proBNP and ejection fraction (EF) (ß = -0.34, p = 0.001). OH (ß = 0.331, p = 0.001) and diastolic dysfunction (ß = 0.226, p = 0.027) were associated with elevated NT-proBNP. In a subgroup analysis of diastolic dysfunction grade, NT-proBNP increased according to dysfunction grade (normal, 4177 pg/mL [2637-10,391]; grade 1, 9736 pg/mL [5471-21,110]; and grades 2-3, 26,237 pg/mL [16,975-49,465]). NT-proBNP showed a tendency toward a decrease in the 'reduced dry weight' group and toward an increase in the 'increased dry weight' group compared to the control group (ΔNT-proBNP, -210 pg/mL [-12,899 to 3142], p = 0.104; 1575 pg/mL [-113 to 6439], p = 0.118). Conclusions: We confirmed that NT-proBNP is associated with volume status as well as heart function in HD patients.

Validation of an international prediction model including the Oxford classification in Korean patients with IgA nephropathy.

BACKGROUND: Recently, a new international risk prediction model including the Oxford classification was published which was validated in a large multi-ethnic cohort. Therefore, we aimed to validate this risk prediction model in Korean patients with IgA nephropathy. METHODS: This retrospective cohort study was conducted with 545 patients who diagnosed IgA nephropathy with renal biopsy in three medical centers. The primary outcome was defined as a reduction in estimated glomerular filtration rate (eGFR) of >50% or incident end-stage renal disease (ESRD). Continuous net reclassification improvement (cNRI) and integrated discrimination improvement (IDI) were used to validate models. RESULTS: During the median 3.6 years of follow-up period, 53 (9.7%) renal events occurred. In multivariable Cox regression model, M1 (hazard ratio [HR], 2.22; 95% confidence interval [CI], 1.02-4.82; p = .043), T1 (HR, 2.98; 95% CI, 1.39-6.39; p = .005) and T2 (HR, 4.80; 95% CI, 2.06-11.18; p < .001) lesions were associated with increased risk of renal outcome. When applied the international prediction model, the area under curve (AUC) for 5-year risk of renal outcome was 0.69, which was lower than previous validation and internally derived models. Moreover, cNRI and IDI analyses showed that discrimination and reclassification performance of the international model was inferior to the internally derived models. CONCLUSION: The international risk prediction model for IgA nephropathy showed not as good performance in Korean patients as previous validation in other ethnic group. Further validation of risk prediction model is needed for Korean patients with IgA nephropathy.

What We Should Consider in Point of Care Blood Glucose Test; Current Quality Management Status of a Single Institution.

Background and Objectives: Point of care test (POCT) is generally performed by non-laboratory staff who often lack an understanding on the quality control and quality assurance programs. The purpose of this study was to understand the current status of quality management of point of care (POC) blood glucose testing in a single institution where non-laboratory staff perform the tests. Materials and Methods: From July to August 2020, management status of glucometer, test strips, quality control (QC) materials, quality assurance program, and operators' response to processing of displayed results was monitored in all Soonchunhyang University Bucheon hospital departments that performed POC blood glucose test. Results of the POC blood glucose test conducted from January 2019 to May 2020 were analyzed retrospectively. Results: A total 124 glucometers were monitored in 47 departments. Insufficient management of approximately 50% of blood sugar, test strips, and QC materials was observed. Although daily QC was conducted by 95.7% of the departments, the QC records were inaccurate. The method of recording test results varied with departments and operators. Various judgments and troubleshooting were performed on the unexpected or out of measurable range results, including some inappropriate processes. In POC blood glucose test results review, 4568 atypical results were identified from a total of 572,207 results. Conclusions: Sufficient training of the non-laboratory staff and ongoing assessment of competency through recertification is needed to maintain acceptable levels of POCT quality. In this study, various problems were identified in glucometer and reagent management, QC and post-analytic phase. We believe that these results provide meaningful basal information for planning effective operators' training and competency evaluation, and the development of an efficient POCT quality management system.

Comparing Patient Survival of Home Hemodialysis and Peritoneal Dialysis Patients.

BACKGROUND: It is not clear whether peritoneal dialysis (PD) and home hemodialysis (HHD) have similar outcomes, and little is known about how mortality associated with HHD versus PD differs according to the duration of dialysis. METHODS: We examined a national cohort of incident end-stage renal disease patients that was comprised of 1,993 and 16,514 patients transitioning to HHD and PD, respectively, from 2007 to 2011. The HHD patients were matched with PD patients using propensity score (PS). Demographics, comorbidities, duration of dialysis, and body mass index were adjusted for in logistic regression models using PS matching. We matched 1,915 HHD patients with 1,915 PD patients based on the PS. The patients were categorized by their vintage (duration of dialysis) at the time of the transition to HHD or PD (<3, 3 to <12, and ≥12 months). RESULTS: In the matched cohort, 237 and 359 deaths occurred in the HHD and PD patients, respectively (cumulative incidence 9.6 vs. 12.9/100 patient-years, p < 0.001). PD patients who transitioned within 12 months of starting dialysis had similar mortality risks, while PD patients who transitioned >12 months after starting dialysis had an 83% higher risk for mortality (hazard ratio 1.83; 95% CI 1.33-2.52). CONCLUSIONS: Whereas there was no meaningful survival difference in the first 12 months between HHD and PD, patients who transitioned to PD after 12 months of dialysis had worse survival than their HHD counterparts. Additional studies are warranted to investigate clinical implications of these differences.

Ultralow Anterior Resection and Coloanal Anastomosis for Low-Lying Rectal Cancer: An Appraisal Based on Bowel Function.

BACKGROUNDS/AIMS: On the basis of acceptable oncologic results, ultralow anterior resection (ULAR) and colo-anal anastomosis plus hand-sewn coloanal anastomosis have been performed for treating very low-lying rectal cancer. However, many patients experience bowel dysfunction after ULAR. Studies have provided inadequate data on bowel dysfunctions and only a few functional studies have focused on low rectal cancer. Therefore, we aimed to elucidate the severity of bowel dysfunction after ULAR in a single-surgeon cohort. METHODS: In this prospective observational study, we analyzed data of 203 patients who underwent sphincter-preserving surgery for low-lying rectal cancer (tumor located within 5 cm from the anus) between January 2011 and December 2014. During routine follow-up, examinations (3-6 months interval) after ileostomy closure, patients were asked about their bowel functions based on the Wexner incontinence and LAR syndrome (LARS) scores. Patients were divided into 2 groups: LAR group (LAR with double-stapled anastomosis) and ULAR group (ULAR with coloanal anastomosis), and functional scores were compared between 6 and 36 months. Seven risk factors for major LARS were analyzed. RESULTS: At 36 months after surgery, 94.2 and 70.6% of patients in the ULAR group still had moderate to severe incontinence and major LARS respectively. Fecal incontinence improved significantly over time (ULAR group, 14.4 vs. 7.2, p = 0.045; LAR group, 13.9 vs. 5.4, p < 0.05). However, improvement in LARS over time was observed in the LAR group only (26.5 vs. 19.7, p = 0.045). In the ULAR group, the difference did not reach a statistical significance (33.6 vs. 26.0, p = 0.10). Major LARS and moderate incontinence were significantly higher in the ULAR group than in the LAR group (70.6 vs. 47.6%, p = 0.001; 82.4 vs. 32.0%, p = 0.012 respectively). Among the 7 factors evaluated in multivariable analysis, old age (> 70), male sex, ULAR per se, and chemoradiation therapy were found to be meaningful risk factors for major LARS. CONCLUSION: In patients with low rectal cancers undergoing ULAR plus coloanal anastomosis, bowel dysfunctions were severe. Bowel dysfunctions improved over time, but most patients still experienced major bowel dysfunctions even 36 months after surgery. Risk factors for bowel dysfunctions were old age, male sex, adjuvant chemoradiation therapy, and ULAR. Therefore, ULAR should be performed in carefully selected patients with low-lying rectal cancer.

Differences in Perspectives of Medical Device Adverse Events: Observational Results in Training Program Using Virtual Cases.

BACKGROUND: Medical device adverse event reporting is an essential activity for mitigating device-related risks. Reporting of adverse events can be done by anyone like healthcare workers, patients, and others. However, for an individual to determine the reporting, he or she should recognize the current situation as an adverse event. The objective of this report is to share observed individual differences in the perception of a medical device adverse event, which may affect the judgment and the reporting of adverse events. METHODS: We trained twenty-three participants from twelve Asia-Pacific Economic Cooperation (APEC) member economies about international guidelines for medical device vigilance. We developed and used six virtual cases and six questions. We divided participants into six groups and compared their opinions. We also surveyed the country's opinion to investigate the beginning point of 'patient use'. The phases of 'patient use' are divided into: 1) inspecting, 2) preparing, and 3) applying medical device. RESULTS: As for the question on the beginning point of 'patient use,' 28.6%, 35.7%, and 35.7% of participants provided answers regarding the first, second, and third phases, respectively. In training for applying international guidelines to virtual cases, only one of the six questions reached a consensus between the two groups in all six virtual cases. For the other five questions, different judgments were given in at least two groups. CONCLUSION: From training courses using virtual cases, we found that there was no consensus on 'patient use' point of view of medical devices. There was a significant difference in applying definitions of adverse events written in guidelines regarding the medical device associated incidents. Our results point out that international harmonization effort is needed not only to harmonize differences in regulations between countries but also to overcome diversity in perspectives existing at the site of medical device use.

No Survival Benefit in Octogenarians and Nonagenarians with Extended Hemodialysis Treatment Time.

BACKGROUND: The population of elderly end-stage renal disease patients initiating dialysis is rapidly growing. Although longer treatment is supposed to benefit for hemodialysis (HD) patients through more solute clearance and slower fluid removal, it is not yet clear how treatment session length affects mortality risk in octogenarians and nonagenarians. METHODS: In a cohort of 112,026 incident HD patients between 2007 and 2011, we examined the association of treatment session length with all-cause mortality, adjusting for demographics and comorbid conditions. We also used restricted spline functions for age to evaluate continuous changes in the association of short (< 210 min) and extended (≥240 min) HD treatment (vs. 210 to < 240 min) with all-cause mortality over continuous age. RESULTS: During the first 91 days of dialysis, patients aged ≥80 years tended to have the lowest treatment session length (median [interquartile range] 211 [193-230] min, r > 0.5). Longer treatment was associated with better survival in patients < 65 and 65 to < 80 years but not in octogenarians/nonagenarians. The association of extended treatment (≥240 min) with better survival was attenuated across age and not significant among patients aged ≥80 years with a hazard ratio of 1.10 (95% CI 0.99-1.20). Shorter treatment sessions (< 210 min) was associated with higher mortality across all age groups. CONCLUSION: Extended HD was not associated with lower mortality among octogenarians and nonagenarians, while it was associated with better survival among younger patients. Further studies are needed to determine the optimal treatment session length in elderly incident HD patients.

Could mean platelet volume be a promising biomarker of progression of chronic kidney disease?

The mean platelet volume (MPV), a readily available indicator of platelet activation and function, is a useful predictive and prognostic biomarker of cardiovascular and cerebrovascular disease (CVD). It is associated with a variety of prothrombotic and proinflammatory diseases. Larger platelets are more likely to aggregate and release greater quantities of adhesive molecules. MPV has seldom been investigated in patients with chronic kidney disease (CKD). This study aimed to investigate the relationship between MPV levels and the glomerular filtration rate (GFR) in patients with CKD. We reviewed the medical records of patients with CKD who visited the nephrology outpatient clinics of Soonchunhyang University Bucheon Hospital between January 2010 and May 2013. A total of 553 patients were included in the present retrospective study. According to the estimated GFR (eGFR) calculated by the abbreviated the Modification of Diet in Renal Disease (MDRD) equation, the patients were allocated to Group 1 (GFR, 60-89 ml/minute/1.73 m(2); n = 64), Group 2 (GFR, 30-59 ml/minute/1.73 m(2); n = 268), Group 3 (GFR, 15-29 ml/minute/1.73 m(2); n = 147), or Group 4 (GFR, <15 ml/minute/1.73 m(2) and non-dialysis; n = 74). Data were analyzed by Student's t-test, the chi-squared test, Pearson's correlation coefficient (r), Tukey's honestly significant difference (HSD) test, and one-way analysis of covariance. The MPV values had a negative correlation with eGFR in patients with CKD (Pearson's correlation coefficient = -0.553, p < 0.001). The mean MPV values in Groups 1-4 were 9.81 ± 0.13 fl, 10.34 ± 0.08 fl, 10.86 ± 0.09 fl, and 11.19 ± 0.11 fl, respectively (p < 0.001). Multiple comparisons of MPV values in the four groups by Tukey's HSD test showed statistically significant intergroup differences, with all p values <0.001. Platelet counts and PDW decreased along with eGFR, and there were no significant differences with respect to plateletcrit. Patients with prevalent coronary artery disease (CAD) or CVD had higher MPVs than did those without CAD or CVD. MPV was significantly increased with progression of CKD. MPV may be a useful indicator of increased risks of CAD or CVD in patients with CKD.

Efficacy and cost-effectiveness of darbepoetin alfa once every 4 weeks versus continuous erythropoietin receptor activator once every 4 weeks for anemia correction in patients with chronic kidney disease not on dialysis.

BACKGROUND: For anemia management in patients with chronic kidney disease not on dialysis, darbepoetin alfa (DA), which has a shorter half-life but is more inexpensive than continuous erythropoietin receptor activator (CERA), is preferred in Korea. This study evaluated the efficacy, safety, and cost-effectiveness of once-in-4-weeks DA compared with once-in-4-weeks CERA in patients with chronic kidney disease not on dialysis. METHODS: In this randomized, prospective, non-inferiority study, 40 erythropoiesis-stimulating agent-naïve patients with chronic kidney disease not on dialysis were randomized 1:1 to the DA group and CERA group. They received the study drug once in 4 weeks during 10- or 12-week correction period and 24-week efficacy evaluation period. The primary outcomes were the mean difference in the changes in hemoglobin levels between baseline and efficacy evaluation period and hemoglobin response rates during the correction period. The secondary outcomes included differences in adverse events and costs. RESULTS: DA was non-inferior to CERA for anemia correction; the mean difference in the change in hemoglobin levels between the groups was -0.070 g/dL (95% confidence interval, -0.730 to 0.590 g/dL). Hemoglobin response rates were 100% with DA and 94.1% with CERA. Adverse events were comparable. The mean cost of DA was approximately one-third that of CERA (34,100 ± 7,600 Korean won/4 weeks vs. 115,500 ± 23,600 Korean won/4 weeks; p < 0.001). CONCLUSION: Once-in-4-weeks DA safely corrects anemia in erythropoiesis-stimulating agent-naïve patients with chronic kidney disease not on dialysis and is more cost-effective than once-in-4-weeks CERA.

KIM-1 expression predicts renal outcomes in IgA nephropathy.

BACKGROUND: Kidney injury molecule-1 (KIM-1) is a sensitive biomarker for proximal tubular injury. Recently, a few studies have shown that urinary KIM-1 has clinical implications in IgA nephropathy (IgAN). We performed this study to determine whether tissue KIM-1 has clinical implications for predicting long-term outcome and whether urinary KIM-1 is correlated with tissue KIM-1 and kidney injury in IgAN patients. METHODS: We assessed the prognostic prediction capability of tissue KIM-1 expression in 69 adult patients with IgAN retrospectively. Renal biopsies from all patients were scored by a pathologist who was blinded to the clinical data for the pathologic variables. The primary outcome was the composite of a 50 % reduction in eGFR or end-stage renal disease. Tissue KIM-1 expression was assessed semiquantitatively by counting the stained tubules per 100× power field; 0 tubule indicates grade 0; 1-5 tubules, grade 1; 6-10 tubules, grade 2; and more than 10 tubules, grade 3. Comparing urinary KIM-1 and tissue KIM-1 expression, 50 consecutive IgAN patients were prospectively enrolled to measure urinary KIM-1 levels and examine their biopsy specimens by KIM-1 immunohistochemistry. RESULTS: Univariate analysis showed that tissue KIM-1 expression was associated with the renal outcome in IgAN. Multivariate regression analysis, as the relationship of tissue KIM-1 with prognosis, was consistent. Proteinuria at biopsy and tissue KIM-1 grade 3 were shown to have a prognostic value. The concentration of urinary KIM-1/Cr in patients with IgAN was significantly higher than that in the normal controls. CONCLUSION: Tissue KIM-1 expression is an independent predictor of adverse renal outcomes in IgA nephropathy patients.

Urinary cystatin C levels as a diagnostic and prognostic biomarker in patients with acute kidney injury.

AIM: Acute kidney injury (AKI) is a frequent complication in critically ill patients and is associated with a high mortality. Clinicians have limited tools to predict the course of AKI at the time of serum creatinine increase. We evaluated the diagnostic and prognostic utility of urinary cystatin C (uCysC) in patients with AKI. METHODS: In this study, serum and uCysC and urinary creatinine (uCr) were measured in patients presenting with acute kidney injury. The patients were divided into two groups: those with prerenal AKI and those with an intrinsic AKI. Prerenal AKI was defined as a new-onset increase in serum creatinine (sCr) that resolved within 72 h and returned to the baseline kidney function level. Patients with intrinsic AKI were defined and classified according to the Acute Kidney Injury Network (AKIN) criteria. RESULTS: Of the total number of patients (n = 213), 40.4% (n = 86) were judged to have prerenal AKI and 59.6% (n = 127) intrinsic AKI. uCysC values and the uCysC/uCr ratio were significantly higher in intrinsic AKI versus prerenal AKI. In intrinsic AKI, the uCysC concentration increased with AKI severity. The uCysC/uCr ratio was significantly higher in the RRT group versus the non-RRT group (0.15 vs. 0.08, respectively; P = 0.037). In a multivariate analysis, the uCysC/uCr ratio was associated with in-hospital mortality (P = 0.019). CONCLUSIONS: uCysC level and the uCysC/uCr ratio were useful biomarkers of intrinsic AKI, and the uCysC/uCr ratio was predictive of in-hospital death in AKI patients.

Effect of treatment on urinary kidney injury molecule-1 in IgA nephropathy.

BACKGROUND: Kidney injury molecule-1 (KIM-1) is a biomarker useful for detecting early tubular damage and has been recently reported as a useful marker for evaluating kidney injury in IgA nephropathy (IgAN). We therefore investigated whether treatment decreases urinary KIM-1 excretion in IgAN. METHODS: We prospectively enrolled 37 patients with biopsy-proven IgAN. Urinary KIM-1 was assessed before and after treatment, which included low salt diet, blood pressure control, pharmacotherapy with angiotensin receptor blockers and/or angiotensin converting enzyme inhibitors, and immunosuppressive agents as necessary. The median treatment duration was 24 months. RESULTS: Urinary KIM-1/creatinine (Cr) was significantly decreased in patients with IgAN after treatment compared to baseline (P < 0.0001, 1.16 [0.51-1.83] vs 0.26 [0.12-0.65] ng/mg). There was a decrease in the amount of proteinuria after treatment, but it was not statistically significant (P = 0.052, 748.1 [405-1569.7] vs 569.2 [252.2-1114] g/d). Estimated glomerular filtration rate (eGFR) did not change with treatment (P = 0.599, 79.28 ± 30.56 vs 80.98 ± 32.37 ml/min/1.73 m2). Urinary KIM-1 was not correlated with proteinuria baseline or follow up (pre-: R = - 0.100, P = 0.577, post-: R = 0.001, P = 0.993). In patients with higher baseline urinary KIM-1, both urinary KIM-1 level and proteinuria were significantly decreased following treatment. CONCLUSIONS: Treatment decreases urinary KIM-1/Cr in patients with IgAN. It also reduces proteinuria in patients with higher baseline urinary KIM-1. These results suggest a potential role for urinary KIM-1 as a biomarker for predicting treatment response in IgAN, however, further study is needed to verify this.

Management of Hypertension in Fabry Disease.

Fabry disease (FD), a rare X-linked lysosomal storage disorder that depletes alpha-galactosidase A (α-GalA), is caused by mutations in the GLA gene. Diminished α-GalA enzyme activity results in the accumulation of Gb3 and lyso-Gb3. The pathophysiology of hypertension in FD is complex and unclear. The storage of Gb3 in arterial endothelial cells and smooth muscle cells is known to produce vascular injury by increasing oxidative stress and inflammatory cytokines as a primary pathophysiological mechanism. In addition, Fabry nephropathy developed, resulting in a decrease in kidney function and contributing to hypertension. The prevalence of hypertension in patients with FD was between 28.4% and 56%, whereas hypertension in patients with chronic kidney disease ranged between 33% and 79%. A study using 24-hour ambulatory blood pressure monitoring (ABPM) to measure blood pressure (BP) indicated a high prevalence of uncontrolled hypertension in FD. Thus, 24-hour ABPM ought to be considered for FD hypertension assessments. Appropriate treatment of hypertension is believed to reduce mortality in patients with FD caused by kidney disease, cardiovascular disease, and cerebrovascular disease because hypertension significantly impacts organ damage. Up to 70% of FD patients have been reported to have kidney involvement, and angiotensin-converting enzyme inhibitors and angiotensin receptor blockers prescribed for proteinuria are recommended as first-line therapy with antihypertensive drugs. In conclusion, hypertension should be controlled appropriately, given the different morbidity and mortality caused by significant organ involvement in FD patients.

Common Data Model-based Analysis of Selective Leukoreduction Protocol Compliance at Three Hospitals.

Background: The selective leukoreduction protocol (SLP) is limited in that patients who require it can be overlooked. We estimated SLP compliance (SLPC) using the Observational Medical Outcomes Partnership common data model (CDM). Methods: Patients were classified into eight groups: pre- and post-hematology disease (A and B), pre- and post-solid organ transplantation (C and D), solid cancer (E), immunodeficiency (F), anticancer therapy (G), and cardiovascular surgery (H). We examined the red blood cell (RBC) transfusion history from three hospital datasets comprising approximately three million patients over 20 years using CDM-based analysis. SLPC was calculated as the percentage of patients who received only leukoreduced RBCs in total patients transfused RBCs. Results: In total, 166,641 patients from three hospitals were enrolled in this study. From 2001 to 2021, SLPC in all groups, except H, tended to increase, although there were differences among the hospitals. Based on the most recent values (2017-2021), the SLPC in groups A, B, D, and G was maintained at ≥75% until 1,095 days before or after diagnosis or treatment. Groups E, F, and H had < 50% SLPC one day after diagnosis and treatment. Conclusions: CDM analysis supports the review of large datasets for SLPC evaluation. Although SLPC tended to improve in most patient groups, additional education and monitoring are needed for groups that continue to show low SLPC.

Dynamic Range and Neural Response Threshold in Cochlear Implant Mapping Can Be Useful in Predicting Prognosis Related to Postoperative Speech Perception.

BACKGROUND AND OBJECTIVES: To analyze mapping changes in dynamic range (DR) and neural response threshold (NRT) as prognostic factors for cochlear implant (CI). To analyze whether postoperative speech perception performance could be predicted using DR change and initial NRT. SUBJECTS AND METHODS: The speech comprehension data of 33 patients with CI were retrospectively analyzed after 1, 3, 6, and 12 months of device use. All subjects were adult, postlingually hearing-impaired, and Cochlear Nucleus CI users. Speech perception performance was evaluated using aided pure tone audiometry, consonant, vowel, one-word, two-word, and sentence tests. RESULTS: The averages of initial NRT and DR changes were 197.8±25.9 CU (104-236) and 22.2±18.4 CU (-15-79), respectively. The initial DR was 40.8±16.6 CU. The postoperative DR was 50.3±16.4 CU at 3 months, 58±12.3 CU at 6 months, and 62.9±10.4 CU at 12 months. A gradual increase of DR was observed during the first year of CI. Compared with the initial DR, significant increases in DR were observed at 3 (p<0.05), 6 (p<0.001), and 12 (p<0.001) months. Compared with initial speech performance outcomes, a significant gain in all performance outcomes was achieved at 12 months (p<0.001). CONCLUSIONS: Patients with low NRT after CI surgery could initially set DR to a wider range and had better final speech perception outcomes. Conversely, patients with high NRT after CI surgery had to set up a gradual increase in DR while adjusting the T-C level, and the final speech perception outcomes were worse. DR and NRT, the main CI mapping variables, can help predict prognosis related to speech perception outcomes after CI surgery. In conclusion, the post-CI speech perception is better with a lower initial NRT, wider final DR, or younger age.

A questionnaire survey on the diagnosis and treatment of Fabry nephropathy in clinical practice.

BACKGROUND: Fabry nephropathy is characterized by a deficiency of lysosomal alpha-galactosidase A, which results in proteinuria and kidney disease. The ineffectiveness of enzyme replacement therapy (ERT) for severe kidney failure highlights the need for early detection and meaningful markers. However, because the diagnosis and treatment of Fabry disease can vary according to the expertise of physicians, we evaluated the opinions of Korean specialists. METHODS: A questionnaire regarding the management of Fabry nephropathy was emailed to healthcare providers with the experience or ability to treat individuals with Fabry nephropathy. RESULTS: Of the 70 experts who responded to the survey, 43 were nephrologists, and 64.3% of the respondents reported having treated patients with Fabry disease. Pediatricians are treating primarily patients with classic types of the disease, while nephrologists and cardiologists are treating more patients with variant types. Only 40.7% of non-nephrologists agreed that a kidney biopsy was required at the time of diagnosis, compared with 81.4% of nephrologists. Thirty-eight of 70 respondents (54.3%) reported measuring globotriaosylsphingosine (lyso-Gb3) as a biomarker. The most common period to measure lyso-Gb3 was at the time of diagnosis, followed by after ERT, before ERT, and at screening. For the stage at which ERT should begin, microalbuminuria and proteinuria were chosen by 51.8% and 28.6% of respondents, respectively. CONCLUSION: Nephrologists are more likely to treat variant Fabry disease rather than classic cases, and they agree that ERT should be initiated early in Fabry nephropathy, using lyso-Gb3 as a biomarker.

Syk/Src pathway-targeted inhibition of skin inflammatory responses by carnosic acid.

Carnosic acid (CA) is a diterpene compound exhibiting antioxidative, anticancer, anti-angiogenic, anti-inflammatory, anti-metabolic disorder, and hepatoprotective and neuroprotective activities. In this study, the effect of CA on various skin inflammatory responses and its inhibitory mechanism were examined. CA strongly suppressed the production of IL-6, IL-8, and MCP-1 from keratinocyte HaCaT cells stimulated with sodium lauryl sulfate (SLS) and retinoic acid (RA). In addition, CA blocked the release of nitric oxide (NO), tumor necrosis factor (TNF)-α, and prostaglandin E2 (PGE2) from RAW264.7 cells activated by the toll-like receptor (TLR)-2 ligands, Gram-positive bacterium-derived peptidoglycan (PGN) and pam3CSK, and the TLR4 ligand, Gram-negative bacterium-derived lipopolysaccharide (LPS). CA arrested the growth of dermatitis-inducing Gram-positive and Gram-negative microorganisms such Propionibacterium acnes, Pseudomonas aeruginosa, and Staphylococcus aureus. CA also blocked the nuclear translocation of nuclear factor (NF)-κB and its upstream signaling including Syk/Src, phosphoinositide 3-kinase (PI3K), Akt, inhibitor of κBα (IκBα) kinase (IKK), and IκBα for NF-κB activation. Kinase assays revealed that Syk could be direct enzymatic target of CA in its anti-inflammatory action. Therefore, our data strongly suggest the potential of CA as an anti-inflammatory drug against skin inflammatory responses with Src/NF-κB inhibitory properties.