A 20-Year Analysis of Ruptured Abdominal Aortic Aneurysm Outcomes and Associated Factors in Korea.

BACKGROUND: Unlike western countries, which have reported distinct decreases in incidence of ruptured abdominal aortic aneurysm (rAAA) over the last few decades, epidemiologic studies in Korea have not shown significant changes in incidence or mortality of rAAA. The purpose of this study was to analyze the changes in rAAA treatment outcomes and various associated risk factors over the past 2 decades. METHODS: A 20-year retrospective multicenter review for rAAA cases from the period of January 2000 to December 2020 was undertaken. Preoperative, intraoperative and postoperative clinical data were extracted for patients diagnosed with rAAA. For analysis, outcomes from the early era, defined as patients treated between January 1, 2000, and December 31, 2010, were compared with outcomes from the late era, defined as patients treated between January 1, 2011, and December 31, 2020. RESULTS: The total in-hospital mortality was 34.1% in the early era compared to 44.8% in the late era. Patients in the late era were older than those in the early era (75.2 ± 10.3 years vs. 70.3 ± 8.9 years; P = 0.009). Treatment with rAAA endovascular aneurysm repair increased from 2.3% in early to 13.8% in late era (P = 0.031). In the early era, more patients were operated by experienced surgeons than the late era (78.1% vs. 45.9%; P = 0.002). The emergency room to operating room time did not show improvement over the 20 years. CONCLUSIONS: The results indicate that mortality rate of rAAA in Korea has not changed over the last 2 decades. The study suggests the need for national preventive strategies, improved systemic coordination, and potential centralization of vascular services to enhance survival rates for rAAA.

Validation of I71.3 code for ruptured abdominal aortic aneurysm in Korea: misplaced diagnosis in claims data.

Purpose: Studies in western countries have shown a decline in the incidence of ruptured abdominal aortic aneurysm (rAAA) with advancements in endovascular repair and screening. However, according to health insurance data in Korea based on rAAA code (I71.3), overall rAAA has been increasing. This study aimed to validate the I71.3 code for rAAA and attempt to define the true incidence of rAAA in Korea. Methods: A 20-year multicenter retrospective review of rAAA was undertaken from the period of January 1, 2000 to December 31, 2020. All patients were diagnosed with the rAAA code I71.3 in each of the 4 hospitals. The CT images and surgical records of these patients were reviewed to differentiate true rAAA and misdiagnosis. Further data on true rAAA patient outcomes including mortality and treatment success were also collected. Results: A total of 305 rAAA (I71.3) codes were identified in the 4 centers. However, medical record review showed true rAAA in only 131 (43.0%). The remaining 174 cases (57.0%) were misdiagnosed. Impending ruptures were the most common misdiagnoses (37.9%). The total in-hospital mortality including deaths before treatment was 38.9% (n = 51), while mortality of treated patients was 24.4% (n = 15). Conclusion: The analysis of I71.3 code for rAAA showed that only 43.0% were true rAAA and the remaining 57.0% were misdiagnosed. This indicates that the I71.3 code is overestimated in National Health Insurance-based data and that the true incidence of rAAA could be much lower.

Comparison between radiocephalic and brachiocephalic arteriovenous fistula in octogenarians: A retrospective single center study.

PURPOSE: The number of older patients who need vascular access for end-stage renal disease is rapidly increasing. However, determining the optimal vascular access for older patients is difficult. We aimed to compare the outcomes of radiocephalic (RC) and brachiocephalic (BC) arteriovenous fistula (AVF) in patients aged >80 years. METHODS: This study included 94 patients undergoing hemodialysis who underwent the procedure for the first time between 2013 and 2019 in Korea University Guro Hospital. The primary outcomes were primary patency (PP) and cumulative patency (CP). The secondary outcome was maturation failure and death with functional vascular access. RESULTS: Of the 94 patients (mean age, 83.9 ± 2.97 years), 66 (70.2%) and 28 (29.8%) patients belonged to the RC and BC AVF groups, respectively. One-year PP was worse in the RC AVF group than in the BC AVF group (59.6% vs. 87.4%, p = 0.013). However, no significant difference was observed in 1-year CP between the groups (87.4% vs. 91.2%, p = 0.441). The unassisted maturation rate was higher in the BC AVF group than in the RC AVF group (74.2% vs. 96.4%, p = 0.011). During follow-up (649 ± 612 days), only 6 (6.4%) patients required secondary fistula placement. Eighteen patients (19.1%), all of whom had functional AVF, died. CONCLUSION: BC AVF showed better PP and a smaller number of interventions than RC AVF in octogenarians. Therefore, BC AVF could be a primary choice of vascular access in the octogenarian patient. However, further research is warranted to confirm these findings.

The appearance of C1q deposition in transplanted kidney allografts and its clinical and histopathologic features.

Background: C1q nephropathy is an uncommon type of glomerulonephritis characterized by extensive and dominant C1q mesangial deposition in the absence of systemic lupus erythematosus. However, there are limited studies about C1q deposition in renal allografts. This study aimed to report the prevalence of C1q deposition in transplanted kidney allograft biopsies and describe its clinical and histopathologic features. Methods: Between January 2005 and December 2018, a total of 1,742 kidney transplantations were performed at Seoul National University Hospital. All renal allograft biopsies (n=10,217) of these patients were retrospectively screened for C1q deposition. C1q deposition was detected in the renal allograft biopsies of 104 patients (6.0%). Only 28 cases (1.6%) had intense (≥2+) C1q dominance and were reviewed in this study. Results: Among the 28 cases, only four (14.3%) had accompanying proliferative glomerulonephritis. Most did not have any other glomerular changes on light microscopy. No patients had nephrotic-range proteinuria at the time of biopsy. A follow-up biopsy was undertaken in 15 of the cases (53.6%). In these follow-up biopsies, C1q deposition either completely disappeared (n=13, 86.7%) or showed diminished staining (n=2, 13.3%). Conclusions: The prevalence of dominant or codominant C1q deposition in transplanted renal allograft biopsies was 1.6%. Most cases did not have any other accompanying glomerular changes. The follow-up biopsies of these allografts showed spontaneous disappearance or diminished staining of C1q deposition. These findings suggest that C1q deposition found in renal allografts is most likely clinically benign, although this possibility should be confirmed in further large-scale studies.

Effects of tacrolimus intrapatient variability and CYP3A5 polymorphism on the outcomes of pediatric kidney transplantation.

BACKGROUND: The intrapatient variability (IPV) of tacrolimus (Tac) is associated with the long-term outcome of kidney transplantation. The CYP3A single-nucleotide polymorphism (SNP) may affect the IPV of Tac. We investigated the impact of IPV and genetic polymorphism in pediatric patients who received kidney transplantation. METHODS: A total of 202 pediatric renal transplant recipients from 2000 to 2016 were analyzed retrospectively. The IPV was calculated between 6 and 12 months after surgery. Among these patients, CYP3A5 polymorphism was analyzed in 67 patients. RESULTS: The group with high IPV had a significantly higher rate of de novo donor-specific human leukocyte antigen antibodies (dnDSA) development (35.7% vs. 16.7%, p = .003). The high IPV group also had a higher incidence of T-cell-mediated rejection (TCMR; p < .001). The high IPV had no significant influence on Epstein-Barr virus, cytomegalovirus, and BK virus viremia but was associated with the incidence of posttransplant lymphoproliferative disorders (p = .003). Overall, the graft survival rate was inferior in the high IPV group (p < .001). The CYP3A5 SNPs did not significantly affect the IPV of Tac. In the CYP3A5 expressor group, however, the IPV was significantly associated with the TCMR-free survival rate (p < .001). CONCLUSION: The IPV of Tac had a significant impact on dnDSA development, occurrence of acute TCMR, and graft failure in pediatric patients who received renal transplantation. CYP3A5 expressors with high IPV of Tac showed worse outcomes, while the CYP3A5 polymorphism had no impact on IPV of Tac.

Better Efficacy of Balloon Assisted Maturation in Radial-Cephalic Arteriovenous Fistula for Hemodialysis.

PURPOSE: Native arteriovenous fistula (AVF) is the first choice for hemodialysis access; however, the maturation failure rate remains high. Hence, balloon-assisted maturation (BAM) is increasingly being used to overcome maturation failure. This study evaluated the outcomes of BAM and compared the differences between radial-cephalic (RC) and brachial-cephalic (BC) AVF. MATERIALS AND METHODS: Between January 2013 and December 2017, 1,622 new AVFs were created. BAM was considered if the AVF did not satisfy the criteria for hemodynamic maturation (6-mm diameter and 500-mL/min flow rate within 8 weeks after the operation). RESULTS: Of the 1,622 AVFs, BAM was performed in 142 patients (8.75%). There were 92 RC and 50 BC AVFs. Multivariate analyses revealed that ipsilateral central vein catheter history was the sole risk factor for maturation failure after BAM. Oneyear functional primary patency (FPP) and functional secondary patency (FSP) in RC AVFs were higher than those in BC AVFs without statistical significance (FPP, RC vs. BC: 70.9% vs. 50.9%, P=0.099; FSP, 95.5% vs. 81.1%, P=0.146). Further, based on the multivariate analysis, the independent risk factors for FPP in the RC and BC AVFs were the number of BAMs (odds ratio [OR], 3.05; 95% confidence interval [CI], 1.11-8.37; P=0.03) and age (OR, 1.04; 95% CI, 1.00-1.07; P=0.04), respectively. CONCLUSION: BAM is a relatively good salvage method with tolerable patency. However, the risk factors for patency and the outcomes of BAM differ between RC and BC AVFs.

Intravascular Fasciitis in the Femoral Vein with Hypermetabolic Signals Mimicking a Sarcoma: The Role of Preoperative Imaging Studies with Review of Literature.

Intravascular fasciitis (IVF) is a very rare disease that is difficult to diagnose preoperatively. Frequently, it can be misdiagnosed as a malignancy or deep vein thrombosis. A 26-year-old man presented with a 6-month history of intermittent cramping pain in the right calf. Duplex ultrasonography, computed tomography, magnetic resonance imaging, and positron emission tomography were performed in various hospitals. The work-up revealed a hypermetabolic mass in the femoral vein, suggestive of a malignancy, such as leiomyosarcoma. The tumor was located inside the femoral vein with no invasion, and the mass was resected en bloc with the vein wall. Intraoperative frozen section biopsy revealed no malignancy, and the final pathological diagnosis was IVF. Herein, we report a case of IVF and discuss the role of imaging studies in its preoperative diagnosis, with an extensive literature review.

Association between medication adherence and intrapatient variability in tacrolimus concentration among stable kidney transplant recipients.

This study analyzed the association between medication adherence and the intrapatient variability (IPV) of tacrolimus concentrations among kidney transplant recipients through a post hoc analysis of the dataset from a recently conducted randomized controlled trial. Among 138 patients enrolled in the original trial, 92 patients with ≥ 5 months of medication event monitoring system (MEMS) use and ≥ 4 tacrolimus trough values were included in this post hoc analysis. The variability of tacrolimus trough levels was calculated using coefficient variation (CV) and mean absolute deviation. Adherence was assessed using MEMS and self-report via the Basal Assessment of Adherence to Immunosuppressive Medication Scale. There were no statistically significant differences in the CV [median 16.5% [interquartile range 11.6-25.5%] and 16.0% [11.5-23.5%], respectively, P = .602] between the nonadherent (n = 59) and adherent groups (n = 33). There was also no significant correlation between the CV and adherence detected by MEMS (taking adherence, ρ = - 0.067, P = .527; dosing adherence, ρ = - 0.098, P = .352; timing adherence, ρ = - 0.113, P = .284). Similarly, adherence measured by self-report did not significantly affect the IPV (P = .452). In this post hoc analysis, nonadherent behavior, measured through electronic monitoring or self-report, did not affect the IPV.

Unraveling the Genomic Architecture of the CYP3A Locus and ADME Genes for Personalized Tacrolimus Dosing.

BACKGROUND: Tacrolimus (TAC) is an immunosuppressant widely prescribed following an allogenic organ transplant. Due to wide interindividual pharmacokinetic (PK) variability, optimizing TAC dosing based on genetic factors is required to minimize nephrotoxicity and acute rejections. METHODS: We enrolled 1133 participants receiving TAC from 4 cohorts, consisting of 3 with kidney transplant recipients and 1 with healthy males from clinical trials. The effects of clinical factors were estimated to appropriately control confounding variables. A genome-wide association study, haplotype analysis, and a gene-based association test were conducted using the Korea Biobank Array or targeted sequencing for 114 pharmacogenes. RESULTS: Genome-wide association study verified that CYP3A5*3 is the only common variant associated with TAC PK variability in Koreans. We detected several CYP3A5 and CYP3A4 rare variants that could potentially affect TAC metabolism. The haplotype structure of CYP3A5 stratified by CYP3A5*3 was a significant factor for CYP3A5 rare variant interpretation. CYP3A4 rare variant carriers among CYP3A5 intermediate metabolizers displayed higher TAC trough levels. Gene-based association tests in the 61 absorption, distribution, metabolism, and excretion genes revealed that CYP1A1 are associated with additional TAC PK variability: CYP1A1 rare variant carriers among CYP3A5 poor metabolizers showed lower TAC trough levels than the noncarrier controls. CONCLUSIONS: Our study demonstrates that rare variant profiling of CYP3A5 and CYP3A4, combined with the haplotype structures of CYP3A locus, provide additive value for personalized TAC dosing. We also identified a novel association between CYP1A1 rare variants and TAC PK variability in the CYP3A5 nonexpressers that needs to be further investigated.

Single versus en bloc kidney transplant from donors less than or equal to 15 kg to pediatric recipients.

With small kidneys, EBKTs could provide sufficient renal mass but could lead to inefficient use of resources, while SKTs could result in insufficient function due to small renal mass. We aimed to compare the outcomes of EBKT and SKT from small donors weighing ≤15 kg to pediatric recipients. We retrospectively reviewed all pediatric patients who met the inclusion criteria between January 1, 1984, and April 30, 2019, at a single institution. Of a total of 23 patients, 12 received EBKT and 11 received SKT. The median age of donors, weight of donors, and weight of recipients were comparable between the two groups. The median age of recipients and median weight of allografts were greater in the EBKT group than in the SKT group. The median follow-up was 53.9 months. There was no significant difference in eGFR, protein creatinine ratios at 1-year follow-up, and overall graft survival. The size of the kidney increased by approximately 13%-43% in the EBKT group and 40%-60% in the SKT group. This study demonstrated that kidneys from small donors weighing 5-15 kg could be split in pediatric recipients without compromising the outcome.

Standard or Variant Meso-Rex Shunts for Children with Variceal Bleeding due to Extrahepatic Portal Vein Obstruction: A Report of Two Cases.

Extrahepatic portal vein obstruction (EHPVO) is the most common cause of pediatric portal hypertension and can cause life-threatening variceal bleeding. Meso- Rex shunt (MRS) is a surgical procedure that restores physiological portal venous blood flow to the liver by using a graft to connect the superior mesenteric vein and the left portal vein within the Rex recess, and can relieve variceal bleeding and other complications associated with EHPVO. Although the MRS is regarded as an optimal and potentially curative treatment with good long-term patency, graft thrombosis or failure due to unknown causes is not rare, prompting the need for further research on the risk factors of graft failure or poor patency. Herein, we report two cases of EHPVO in patients with recurrent or uncontrolled variceal bleeding, one treated with the classic Rex shunt and the other with the modified Rex shunt, which resulted in a failure and success, respectively.

Renal transplantation in patients with an augmentation cystoplasty.

Background: The effects of renal transplantation in patients with augmentation cystoplasty are still controversial. We retrospectively analyzed nine patients who underwent renal transplantation after augmentation cystoplasty. Methods: A total of nine patients who underwent augmentation cystoplasty prior to renal transplantation between January 1990 and May 2020 were reviewed. Basic information on augmentation cystoplasty, transplant procedures, and long-term outcomes of renal transplantation were analyzed. Results: The bowel segments utilized for augmentation cystoplasty were the stomach in two patients (one patient needed revision using the ileum), the ileum in four patients, the ileocolic pouch in one patient, the sigmoid in one patient, and the ureter in one patient. All the cystoplasties were performed prior to renal transplantation. The mean follow-up period after transplantation was 161 months (range, 2-341 months). Two patients had an episode of acute rejection each; however, their graft functions were well-maintained. Five patients had recurrent urinary tract infections, and three of these patients progressed to allograft failure. One patient died from bladder cancer with a functioning graft. Five of nine patients showed well-maintained graft function. Conclusions: Renal transplantation after bladder augmentation surgery is a major operation requiring a high level of surgical skill. Based on our long-term experiences, we recommend diligent postoperative monitoring for urinary tract infections, optimal catheter use, and use of appropriate antibiotic prophylaxis to avoid severe complications.

Thrombosis of a Long-Segment Aneurysm from the Iliac to Popliteal Artery Associated with Arteriovenous Malformation and Varicose Veins.

A 58-year-old male patient with severe claudication due to thrombosis of the left ilio-femoro-popliteal artery aneurysm. He also had a venous stasis ulcer with a history of multiple embolotherapy of arteriovenous malformation. Duplex sonography revealed reflux and varicose veins of the left great saphenous vein (GSV). A sequential bypass surgery was performed that consisted of excision of the left external iliac and common femoral artery aneurysm, external iliac to deep femoral interposition with an expanded polytetrafluoroethylene graft, and femoro-posterior tibial artery bypass with the reversed left GSV. Symptoms of claudication were alleviated and the chronic ulcer was healed in time. To our knowledge, this is the first report of successful bypass in a patient with arterial aneurysm, arteriovenous malformation, and venous insufficiency that can be diagnosed as an atypical case of Parkes Weber syndrome. Long-term follow-up is needed to define the fate of aneurysms and varicose vein graft.