Ten-year Patient Survival Research Articles

Long-term Outcomes of Liver Transplantation for Inborn Errors of Metabolism in Children

BackgroundLiver transplantation for inborn errors of metabolism is increasingly common and has historically had positive outcomes. However, this therapeutic modality is not without risks, and patient post-transplant quality of life should be part of the consideration. MethodsThis retrospective, observational cohort study included all pediatric patients receiving liver transplant from 2010 through 2020 at a single center. Recipients were split into 2 groups based on metabolic or non-metabolic indications for liver transplant. Ten-year patient survival and graft survival were analyzed. The PedsQL Transplant Module and RAND 36-Item Health Survey 1.0 were administered prospectively to those recipients with metabolic indications. ResultsTen-year patient survival was statistically significantly higher in the metabolic group than in the non-metabolic (p < .05), and there was no difference in 10-year graft survival between groups. Of the 12 patients in the metabolic group who completed the PedsQL Transplant Module or RAND 36-Item Health Survey 1.0, the median score was 88, similar to the score seen in healthy children. ConclusionsLiver transplantation for inborn errors of metabolism provides excellent long-term outcomes in terms of patient and graft survival, while maintaining a high quality of life.

Десятилетние результаты комплексного лечения больных первичным местнораспространенным раком молочной железы

Purpose: To present ten-year results of complex treatment of patients with primary locally advanced breast cancer, depending on the type of ionizing radiation used and chemotherapy regimens. Material and methods: The study included 250 patients with stage T2‒4N0‒3M0 breast cancer, aged 34–69 years (mean age 48.1±5.6 years), who underwent complex treatment (courses of NChT and AChT, hormonal and targeted therapy (according to indications), radical mastectomy and radiation therapy with various types of ionizing radiation) at the Research Institute of Oncology from 2007 to 2020. The mean follow-up period was 10±2.7 years. Main group (n=110) ‒ neutron therapy, comparison group I (n=80) ‒ photon therapy, comparison group II (n=60) ‒ electron therapy. Results: Ten-year relapse-free survival of patients with locally advanced breast cancer (PL BC) after adjuvant neutron therapy on the area of the anterior chest wall was 92.5±3.5 %, after photon therapy ‒ 70.9±5.6 %, after electron therapy ‒ 73.6±7.1 %. There were statistically significant differences between the main group and comparison groups (p&lt;0.05). Overall survival over a ten-year follow-up period in the main group was 87.5±3.8%, in comparison group I ‒ 73.6±8.6 %, in comparison group II ‒ 38.8±10.0 %. Between neutron and electron therapy – p&lt;0.05. In the group with neutron therapy and chemotherapy according to doxorubicin + taxane regimens, ten-year metastatic-free and overall survival was 72.8±10.0 % and 96.7±3.3 %, respectively, compared with neutron therapy and chemotherapy according to FAC/CAF regimens ‒ 44.0±14.7 % and 83.7±6.7 %, respectively (p&lt;0.05). Neutron therapy was well tolerated by all breast cancer patients. Radiation reactions of the skin predominantly I‒II degree. Radiation pneumonitis after neutron therapy ‒ in 6 (5.4 %) out of 110 patients, after photon therapy ‒ in 17 (21.25 %) out of 80 patients with PL BC (p=0.023). Conclusion: Thus, the use of adjuvant neutron therapy in patients with breast cancer T2‒4N0‒3M0 is a safe method and has significantly better results in ten-year relapse-free survival, which makes it possible to increase the effectiveness of complex treatment. In combination with doxorubicin + taxane chemotherapy regimens, neutron therapy increases the 10-year metastatic-free and overall survival rates.

Effect of Comorbidities on Ten-Year Survival in Patients with Dementia.

There is a verified association between comorbidity and survival in patients with dementia. To describe the ten-year survival probability of patients with dementia and to identify the impact of comorbidity. The prognostic retrospective cohort study was conducted using data from adults with dementia who had visited the outpatient departments at Maharaj Nakorn Chiang Mai hospital between 2006 and 2012. Dementia was verified in accordance with standard practice guidelines. Secondary data detailing about patient age, gender, date of dementia diagnosis and death, types of dementia, and comorbidities at the time of dementia diagnosis was obtained from electronic medical records. The association between comorbidity, patients' underlying disease at dementia diagnosis, and overall survival were analyzed using a multivariable Cox proportional hazard model adjusted for age, gender, types of dementia, and other comorbidities. Of the 702 patients, 56.9% were female. Alzheimer's disease (39.6%) was the most prevalent type of dementia. Median overall survival was 6.0 years (95% CI 5.5- 6.7). The comorbidities associated with a high risk of mortality included liver disease (aHR 2.70, 95% CI 1.46- 5.00), atrial fibrillation (aHR 2.15, 95% CI 1.29- 3.58), myocardial infarction (aHR 1.55, 95% CI 1.07- 2.26), and type 2 diabetes mellitus (aHR 1.40, 95% CI 1.13- 1.74). Overall survival rate of patients with dementia in Thailand was comparable to previous studies. Several comorbidities were associated with a ten-year survival. The prognosis of patients with dementia may be improved by appropriate care of comorbidities.

Long term outcomes following kidney transplantation in children who weighed less than 15kg - report from the UK Transplant Registry.

Kidney transplantation is the treatment of choice in chronic kidney disease (CKD) stage 5. It is often delayed in younger children until a target weight is achieved due to technical feasibility and historic concerns about poorer outcomes. Data on all first paediatric (aged < 18years) kidney only transplants performed in the United Kingdom between 1 January 2006 and 31 December 2016 were extracted from the UK Transplant Registry (n = 1,340). Children were categorised by weight at the time of transplant into those < 15kg and those ≥ 15kg. Donor, recipient and transplant characteristics were compared between groups using chi-squared or Fisher's exact test for categorical variables and Kruskal-Wallis test for continuous variables. Thirty day, one-year, five-year and ten-year patient and kidney allograft survival were compared using the Kaplan-Meier method. There was no difference in patient survival following kidney transplantation when comparing children < 15kg with those ≥ 15kg. Ten-year kidney allograft survival was significantly better for children < 15kg than children ≥ 15kg (85.4% vs. 73.5% respectively, p = 0.002). For children < 15kg, a greater proportion of kidney transplants were from living donors compared with children ≥ 15kg (68.3% vs. 49.6% respectively, p < 0.001). There was no difference in immediate graft function between the groups (p = 0.54) and delayed graft function was seen in 4.8% and 6.8% of children < 15kg and ≥ 15kg respectively. Our study reports significantly better ten-year kidney allograft survival in children < 15kg and supports consideration of earlier transplantation for children with CKD stage 5. A higher resolution version of the Graphical abstract is available as Supplementary information.

Ten-Year Survival in Patients with Idiopathic Pulmonary Fibrosis After Lung Transplantation

Ten-Year Survival in Patients with Idiopathic Pulmonary Fibrosis After Lung Transplantation

The Anti-fibrosis drug Pirfenidone modifies the immunosuppressive tumor microenvironment and prevents the progression of renal cell carcinoma by inhibiting tumor autocrine TGF-β

ABSTRACT Transforming growth factor-β (TGF-β) plays a critical role in regulating cell growth and differentiation. Epithelial to mesenchymal transition (EMT) induced by TGF-β promotes cancer cell migration, invasion, and proliferation. Pirfenidone (5-methyl-1-phenyl-2(1 H)-pyridone, PFD), an approved drug for treating pulmonary and renal fibrosis, is a potent TGF-β inhibitor and found reduced incidence of lung cancer and alleviated renal function decline. However, whether PFD plays a role in controlling renal cancer progression is largely unknown. In the present study, we demonstrated that high TGF-β1 expression was negatively associated with ten-year overall survival of patients with renal cancer. Functionally, blockade of TGF-β signaling with PFD significantly suppressed the progression of renal cancer in a murine model. Mechanistically, we revealed that PFD significantly decreased the expression and secretion of TGF-β both in vitro and in vivo tumor mouse model, which further prevented TGF-β-induced EMT and thus cell proliferation, migration, and invasion. Importantly, the downregulation of TGF-β upon PFD treatment shaped the immunosuppressive tumor microenvironment by limiting the recruitment of tumor-infiltrating MDSCs. Therefore, our study demonstrated that PFD prevents renal cancer progression by inhibiting TGF-β production of cancer cells and downstream signaling pathway, which might be presented as a therapeutic adjuvant for renal cancer.

Patient-Prosthesis Mismatch in Contemporary Small-Size Mechanical Prostheses Does Not Impact Survival at 10 Years.

Background: The effect of PPM in mechanical prostheses on long-term survival is not well-established. Methods: Patients who received a 21 mm or smaller aortic valve between 2000 and 2011 were retrospectively analyzed (n = 416). Propensity matching was used in order to account for baseline differences in patient subgroups (PPM vs. no PPM; severe PPM vs. no severe PPM). Results: Five- and ten-year survival was 78 ± 3.52% and 64.51 ± 4.51% in patients with PPM, versus 83.3 ± 3.12% and 69.37 ± 4.36% in patients without (p = 0.28) when analyzed at 10.39 ± 5.25 years after the primary procedure. Independent risk factors for impaired survival, after matching, were age, serum creatinine, and severe pulmonary hypertension. Five- and ten-year survival in patients with severe PPM was 73.34 ± 6.01% and 61.76 ± 8.17%, respectively, versus 74.72 ± 5.68% and 67.50 ± 7.09% in those without (p = 0.49), at 8.82 ± 5.17 years after SAVR. Age was the only independent variable that influenced long-term survival when severe PPM was added to the model. Conclusions: PPM or severe PPM does not impact long-term survival up to 10 years in mechanical valve recipients when matching for preoperative variables.

Critical aortic stenosis in neonates: Balloon valvuloplasty versus surgical valvotomy

Congenital aortic valvular stenosis in neonates is a life-threatening condition requiring urgent management. Therapeutic strategy remains controversial between balloon valvuloplasty (BV) and surgical valvulotomy (SV). We reviewed our long-term single-center experience of neonatal aortic stenosis management and compared aortic valve outcomes regarding BV versus SV. Between 1990 and 2020, 137 patients underwent aortic stenosis treatment (SV [ n = 83] and BV [ n = 54]) with a median age of 5.5 days (range: 0 to 30 days) and a median weight of 3 kg (range: 1.8 to 4.8 kg). Procedure strategy was decided according to the surgeon availability especially at the beginning of the study. Additionally, there was a traditional local protocol favoring balloon in case of left ventricular dysfunction like in many centers. The median follow-up was 6.7 years (up to 28y, 88% complete). We did not report any operative death but the intra-hospital mortality was notably greater for patients after balloon procedure (20.3%) versus SV group (8.4%). The one-year and ten-year patient survival was respectively 90 and 89% in SV group versus 75 and 69% in BV group ( P < 0.005). In our cohort, 77 patients underwent reoperation for persistent aortic stenosis. Most of the patients after BV ( n = 38, 70%) required a reintervention. The median age for reintervention was 2.9 years in the SV group versus 0.3 years for patients in the BV group. Associated intracardiac lesions and the initial valvuloplasty procedure were significantly associated with the risk of reoperation independently of the maximal gradient and the aortic annulus size. Ten-year freedom from reintervention for SV and BV group were respectively 53% and 35% ( P < 0.005, Fig. 1 ). Long-term survival and freedom from reintervention are excellent after surgical valvotomy. However, poor results are obtained when patients had initial left ventricular dysfunction or multiple left ventricular outflow lesions.

Association of suboptimal lymph node yield with inferior survival in resected stage 1 colon cancer patients.

3601 Background: A minimum of 12 lymph nodes are required during colectomy to accurately stage colon cancer. Prior studies in stage II colon cancer patients demonstrate association of inadequate lymph node examination (LNE) with worse overall survival (OS). No large-scale analogous studies related to LNE have been completed in stage I colon cancer patients. We evaluated patients with stage I colon cancer to determine the association between lymph node yield and OS. Methods: We reviewed the National Cancer Database between 2004-2015 to identify patients with pathologic stage I colon cancer (pT1N0 or pT2N0) who underwent definitive surgical resection. Patients who received radiation therapy or had missing values were excluded. Clinical and demographic characteristics were analyzed. Based on LNE, patients were stratified into 4 cohorts (LNE, 0-5, 6-11, 12-19, 20+) and 2 cohorts (0-11, 12+). Univariable and multivariable analyses were performed to identify variables associated with OS. Kaplan-Meier survival curves were computed to compare the cohorts. Results: We included 81,909 patients for analyses. Median age at diagnosis was 69. A majority were female (51.1%), white (83.8%), received care in a community cancer program (59.5%), and had a Charlson-Deyo score of 0 (66.6%). Only 0.7% of patients had a margin positive resection with a 2.5cm median tumor size. Patients were similarly split between pT1 and pT2. Suboptimal LNE was noted in 27.8% of patients. Patients with LNE were distributed - 10.7% (0-5), 17.1% (6-11), 43.4% (12-19) and 28.9% (20+). Postoperative 30-day mortality was 1.9%. 521 (0.7%) received systemic therapy. Ten-year survival in patients with 0-5 LNE was 52.8% compared to 60.1% with 20+ LNE. On multivariable analyses, patients aged ≥ 69, male sex, increasing tumor size (quartile), pT2 staging and a higher Charlson-Deyo score independently predicted worse OS (p &lt; 0.001). LNE categories were significantly associated with OS (p &lt; 0.001) (Table). On regrouping into 0-11 and 12+ LNE groups, 0-11 LNE group predicted worse OS (HR 1.22, p &lt; 0.001). On multivariable analysis, the above variables continued to show similar association with OS (p &lt; 0.001). Conclusions: Our study demonstrates that lymph node yield is associated with overall survival in patients with stage 1 colon cancer undergoing surgical resection. Furthermore, patients with suboptimal lymph node yield are associated with an inferior overall survival compared to those with optimal lymph node yield. Moreover, this study finds that a large number of patients ( &gt; 25%) continue to have suboptimal lymph node yields. Future efforts should focus on improving the lymph node yield with optimal efforts by the surgeon and pathologist. Future studies should examine the role of systemic therapy in patients with inadequate lymph node yield.[Table: see text]

Long-Term Survival after Liver Resection for Colorectal Liver Metastases: A Retrospective Single-Centre Study with 10-Year Follow-Up

Introduction: Hepatic resection is the curative treatment for colorectal liver metastases (CRLM). The reporting of 10-year survival data is important for assessment of cure rates, frequency and outcomes of late recurrences. The aim of this study was to assess ten-year survival of patients undergoing liver resection at a single centre. Methods: Liver resections for CRLM performed between 1995 and 2008 with follow-up to 2018 were retrospectively reviewed. Demographic, clinicopathological and survival data were analysed. The Fong clinical risk score was dichotomised into low (0-2) and high (3-5) risk and ten-year survival was the primary outcome measure. Kaplan Meier curves and Cox regression were used to analyse factors associated with 10-year survival. Results: A total of 1120 patients underwent liver resection for CRLM. The median age was 68 (range 23-91) years. Synchronous disease was present in 50%, with 56% of patients having adjuvant chemotherapy after primary colorectal cancer resection. 29.4% had a high Fong score (3-5) with 15.4% of patients having repeat liver resections for recurrent metastases. The median post-operative survival was 2 years. Overall ten-year survival was 12.3%. Fong risk classification had a statistically significant association (p=0.006, 95% CI 0.706 - 0.943) with ten-year survival. 2.6% (n=29) of patients with Fong score 3-5 were alive at 10 years. Conclusion: In this single center analysis the ten-year survival rate for resection in CRLM was 12.3%. Patients with high Fong clinical risk score have poorer long-term outcomes. Therefore, further trials are required to assess the benefit of additional treatment such as chemotherapy.

The impact of donor and recipient diabetes on renal transplant outcomes.

The use of diabetic kidneys is increasing worldwide with better outcome than being on waitlist and possible reversal of diabetic changes in transplanted kidneys. But particular caution is warranted in diabetic donor-recipient combination. Total 1223 deceased donor kidney transplants were performed at our center between 2008 and 2018. 689 from non-diabetic donor (NDD) to non-diabetic recipient, 400 from non-diabetic donor to diabetic recipient, 97 from diabetic to non-diabetic recipient, and 32 from diabetic donor (DD) to diabetic recipient. The DD was older than NDDs (median age 48 vs 39years, P<0.0001). DD had higher BMI (35.6 vs 26.9, P<0.0001), higher KDPI (74% vs 37%, P<0.0001), and higher terminal creatinine (1.10mg/dl vs 0.95mg/dl, p 0.0046) than the NDD. Diabetes recipients were comparatively older (57 vs 54, P<0.001). DD recipients had higher serum creatinine at 6months (1.70 vs 1.50mg/dl, p 0.00304) and 2years post-transplant (1.70 vs 1.50mg/dl P<0.0002). DD recipients had more favorable end CPRA than NDD recipients (77.5% at 0% vs 67.4% at 0, P=0.0074). Ten-year patient and graft survival was best in NDD-recipient pair and worse in DD-recipient pair. Diabetic donor kidneys to diabetic recipients have lower 1-, 3-, and 5-year graft survival.

Ten-year survival of patients undergoing coronary angioplasty with first-generation sirolimus-eluting stents and bare-metal stents

Compared to bare-metal stents (BMS), drug-eluting stents reduce stent restenosis and improve subsequent revascularization rates. The impact on patients’ survival has been the subject of debate. To assess the long-term (10-year) survival of patients undergoing percutaneous coronary intervention (PCI) with first-generation sirolimus-eluting stents (SES) in comparison with BMS. In a single-center registry, 600 consecutive patients who underwent successful PCI with SES between April 2002 and February 2003 were compared to 594 patients who underwent PCI with BMS between January 2002 and April 2002, just before the introduction of SES. Clinical and procedural data were collected at the time of intervention and 10-year survival status was assessed via the national life status database. All baseline characteristics were similar between groups except for smaller stent diameter (2.84±0.38 vs. 3.19±0.49 mm; p<0.001), greater stent length (18.50±8.2 vs. 15.96±6.10 mm; p<0.001) and higher number of stents per patient (1.95 vs. 1.46, p<0.001) in the SES group. Overall five- and 10-year all-cause mortality was 9.6% (n=110) and 22.7% (n=272), respectively. The adjusted HR for 10-year mortality in patients undergoing PCI with SES was 0.74 (95% CI 0.58-0.94; p=0.013), corresponding to a relative risk reduction of 19.8%. Other than PCI with BMS, older age, chronic kidney disease, chronic obstructive pulmonary disease and lower ejection fraction were independent predictors of 10-year mortality. To date, this is the longest follow-up study ever showing a potential survival benefit of first-generation sirolimus-eluting stents versus bare-metal stents, supporting prior observations on their sustained efficacy and safety relative to contemporary BMS. Em comparação com os stents de metal nulo (BMS), os stents com efeito de droga reduzem a restenose do stent e melhoram as taxas de revascularização subsequente. O impacto na sobrevivência dos pacientes tem sido objecto de debate. Avaliar a sobrevivência a longo prazo (10 anos) de pacientes submetidos a intervenção coronária percutânea (ICP) com stents sirolimus-eluting de primeira geração (SES) em comparação com a BMS. Num registo de um único centro, 600 pacientes consecutivos submetidos a ICP com SES entre Abril de 2002 e Fevereiro de 2003 foram comparados com 594 pacientes submetidos a ICP com BMS entre Janeiro de 2002 e Abril de 2002, imediatamente antes da introdução da SES. Os dados clínicos e processuais foram recolhidos no momento da intervenção e o estado de sobrevivência de 10 anos foi avaliado através da base de dados nacional do estado de vida. Todas as características de linha de base foram semelhantes entre os grupos, excepto para stents de menor diâmetro (2,84±0,38 vs. 3,19±0,49 mm; p<0,001), maior comprimento do stent (18,50±8,2 vs. 15,96±6,10 mm; p<0,001) e maior número de stents por paciente (1,95 vs. 1,46, p<0,001) no grupo SES. A mortalidade global de cinco e 10 anos por todas as causas foi de 9,6% (n=110) e 22,7% (n=272), respectivamente. A FC ajustada para mortalidade de 10 anos em pacientes submetidos a ICP com SES foi de 0,74 (95% CI 0,58-0,94; p=0,013), correspondendo a uma redução relativa do risco de 19,8%. Para além da ICP com SES, a idade avançada, doença renal crónica, doença pulmonar obstrutiva crónica e a fracção de ejecção inferior foram preditores independentes da mortalidade de 10 anos. Até à data, este é o estudo de seguimento mais longo a demonstrar um potencial benefício de sobrevida dos stents farmacológicos de primeira geração versus stents metálicos, em linha com estudos prévios que demonstram a sua eficácia e segurança quando comparados com SC contemporâneos.

Ten-year survival of patients undergoing coronary angioplasty with first-generation sirolimus-eluting stents and bare-metal stents

IntroductionCompared to bare-metal stents (BMS), drug-eluting stents reduce stent restenosis and improve subsequent revascularization rates. The impact on patients’ survival has been the subject of debate. ObjectiveTo assess the long-term (10-year) survival of patients undergoing percutaneous coronary intervention (PCI) with first-generation sirolimus-eluting stents (SES) in comparison with BMS. MethodsIn a single-center registry, 600 consecutive patients who underwent successful PCI with SES between April 2002 and February 2003 were compared to 594 patients who underwent PCI with BMS between January 2002 and April 2002, just before the introduction of SES. Clinical and procedural data were collected at the time of intervention and 10-year survival status was assessed via the national life status database. ResultsAll baseline characteristics were similar between groups except for smaller stent diameter (2.84±0.38 vs. 3.19±0.49 mm; p<0.001), greater stent length (18.50±8.2 vs. 15.96±6.10 mm; p<0.001) and higher number of stents per patient (1.95 vs. 1.46, p<0.001) in the SES group. Overall five- and 10-year all-cause mortality was 9.6% (n=110) and 22.7% (n=272), respectively. The adjusted HR for 10-year mortality in patients undergoing PCI with SES was 0.74 (95% CI 0.58-0.94; p=0.013), corresponding to a relative risk reduction of 19.8%. Other than PCI with BMS, older age, chronic kidney disease, chronic obstructive pulmonary disease and lower ejection fraction were independent predictors of 10-year mortality. ConclusionTo date, this is the longest follow-up study ever showing a potential survival benefit of first-generation sirolimus-eluting stents versus bare-metal stents, supporting prior observations on their sustained efficacy and safety relative to contemporary BMS.

The association of living donor source with patient and graft survival among kidney transplant recipients in the ERA-EDTA Registry - a retrospective study.

SummaryIn this study we aimed to compare patient and graft survival of kidney transplant recipients who received a kidney from a living‐related donor (LRD) or living‐unrelated donor (LUD). Adult patients in the ERA‐EDTA Registry who received their first kidney transplant in 1998–2017 were included. Ten‐year patient and graft survival were compared between LRD and LUD transplants using Cox regression analysis. In total, 14 370 patients received a kidney from a living donor. Of those, 9212 (64.1%) grafts were from a LRD, 5063 (35.2%) from a LUD and for 95 (0.7%), the donor type was unknown. Unadjusted five‐year risks of death and graft failure (including death as event) were lower for LRD transplants than for LUD grafts: 4.2% (95% confidence interval [CI]: 3.7–4.6) and 10.8% (95% CI: 10.1–11.5) versus 6.5% (95% CI: 5.7–7.4) and 12.2% (95% CI: 11.2–13.3), respectively. However, after adjusting for potential confounders, associations disappeared with hazard ratios of 0.99 (95% CI: 0.87–1.13) for patient survival and 1.03 (95% CI: 0.94–1.14) for graft survival. Unadjusted risk of death‐censored graft failure was similar, but after adjustment, it was higher for LUD transplants (1.19; 95% CI: 1.04–1.35). In conclusion, patient and graft survival of LRD and LUD kidney transplant recipients was similar, whereas death‐censored graft failure was higher in LUD. These findings confirm the importance of both living kidney donor types.

Liver transplant for inherited metabolic disease among siblings.

Liver transplantation is a successful option for inherited metabolic disease yet little is published on the outcome among siblings. We report outcomes of siblings who have undergone liver transplantation for metabolic disease in a single program. Seventy-one siblings (35 males) from 33 individual families underwent liver transplantation since 1982. Outcomes were compared over three consecutive eras. Twenty-eight families had two siblings, four had three siblings, and one had four siblings. In half of families where dates of listing were known, siblings were listed simultaneously. Mean (SD) age at listing for the oldest and second sibling was 13.2 (7.1) and 9.8 (5.7) years, respectively (p<.01). In 18/33 families, the oldest sibling underwent transplantation first. Mean (SD) age at transplant fell from the oldest to second sibling from 12.9 (7.2) to 9.5 (6.3) years, respectively (p<.001). Ten-year patient survival was 83.5% which improved over the eras: era 1 (1982-1994) 65.0%, era 2 (1995-2007) 87.5%, and era 3 (2008-2019) 93.8%: p<.03. Sex, age at transplant, order of transplant, and presence of structural liver disease did not significantly impact survival. When siblings undergo liver transplant for inherited metabolic disease, later siblings are listed and transplanted at a significantly younger age.

Bicuspid Aortic Valve Repair: Causes of Valve Failure and Long-Term Outcomes

Repair of bicuspid aortic valves (BAVs) for aortic regurgitation (AR) has favorable outcomes, but the impact of natural disease progression on durability of repair is uncertain. We evaluated causes of reoperation and compared outcomes of BAV repair to those of patients undergoing aortic valve replacement (AVR). Between January 1993 and December 2016, 113 patients had BAV repair at our institution for significant AR. Operative notes and pathology reports were studied to identify late causes of repair failure. For comparison with AVR, we utilized propensity score weighting with the score derived from preoperative and operative characteristics using gradient boosting machine model. A total of 26 patients had late AVR after initial repair. Causes of late valve dysfunction included calcification or fibrosis of the cusps (68%), concomitant replacement addressing moderate degree of aortic valve disease to avoid future operation (20%), and cusp prolapse (12%). Pathological evaluation of these excised valves reported calcification and fibrosis in 88% of the valves. Ten-year survival of patients undergoing BAV repair was 91% compared with 90% for patients undergoing AVR with a mechanical valve and 79% for AVR with a bioprosthesis (P= .6). Incidence of reoperation after AVR with a bioprosthesis was similar to risk after repair whereas AVR with mechanical valve showed significant advantage. Disease progression with calcification or fibrosis is the most common cause of valve failure after initial repair of BAV. Clinical outcomes of BAV repair for severe AR appear superior to AVR with bioprosthesis.

Ten-year survival of patients treated with peritoneal dialysis: A prospective observational cohort study.

Few studies evaluated over 5-year outcomes of dialysis patients. This study examined 10-year all-cause mortality and death-censored technique failure in a cohort of incident peritoneal dialysis (PD) individuals. Five hundred and thirty-three incident PD individuals from 2006 to 2008 were prospectively followed up for more than 10 years until 2018. Clinical characteristics at PD initiation were collected. The primary outcome was all-cause mortality, and the secondary outcome was death-censored technique failure. Cox hazards models were fit using clinical characteristics at PD initiation. The mean age starting PD for these participants was 48 ± 16 years; 130 (24%) patients had diabetic nephropathy. During follow-up, 208 patients died, and 84 patients experienced technique failures. The 1, 3, 5, and 10 years' survival rates for incident PD patients were 93%, 81%, 64%, and 36%, respectively, and the technical survival rates were 98%, 93%, 85%, and 62%, respectively. Mortality risk was much higher after 3 years on PD. The peritonitis rate was 0.19 episodes per patient-year, and 7 (1.3%) patients had encapsulating peritoneal sclerosis (EPS) giving an incidence rate of 3.1 of 1000 patient-years. The main causes of death were cardiovascular events (97 of 208, 47%), and technique failure was mainly due to peritonitis (41 of 84, 49%). Older age, higher Charlson comorbidity index, and lower level of education were strongly associated with mortality, and diabetic nephropathy was an independent risk factor for technique failure. The 10-year's survival and technique survival rates of incident PD patients were 36% and 62%. Long-term PD can be continued successfully with improved outcomes and low risk for EPS.

Beyond 10years, with or without an intestinal graft: Present and future?

Long-term outcomes in children undergoing intestinal transplantation remain unclear. Seventy-one children underwent intestinal transplantation in our center from 1989 to 2007. We report on 10-year posttransplant outcomes with (group 1, n=26) and without (group 2, n=9) a functional graft. Ten-year patient and graft survival rates were 53% and 36%, respectively. Most patients were studying or working, one third having psychiatric disorders. All patients in group 1 were weaned off parenteral nutrition with mostly normal physical growth and subnormal energy absorption. Graft histology from 15 late biopsies showed minimal abnormality. However, micronutrient deficiencies and fat malabsorption were frequent; biliary complications occurred in 4 patients among the 17 who underwent liver transplantation; median renal clearance was 87mL/min/1.73m2 . Four patients in group 1 experienced late acute rejection. Among the 9 patients in group 2, 4 died after 10years and 2 developed significant liver fibrosis. Liver transplantation and the use of a 3-drug regimen including sirolimus or mycophenolate mofetil were associated with improved graft survival. Therefore, intestinal transplantation may enable a satisfactory digestive function in the long term. The prognosis of graft removal without retransplantation is better than expected. Regular monitoring of micronutrients, early psychological assessment, and use of sirolimus are recommended.

Patient Survival After Kidney Transplantation: Important Role of Graft-sustaining Factors as Determined by Predictive Modeling Using Random Survival Forest Analysis

Identification of the relevant factors for death can improve patient's individual risk assessment and decision making. A well-documented patient cohort (n = 892) in a renal transplant program with protocol biopsies was used to establish multivariable models for risk assessment at 3 and 12 months posttransplantation by random survival forest analysis. Patients transplanted between 2000 and 2007 were observed for up to 11 years. Loss to follow-up was negligible (n = 15). A total of 2251 protocol biopsies and 1214 biopsies for cause were performed. All rejections and clinical borderline rejections in protocol biopsies were treated. Ten-year patient survival was 78%, with inferior survival of patients with graft loss. Using all pre- and posttransplant variables until 3 and 12 months (n = 65), the obtained models showed good performance to predict death (concordance index: 0.77-0.78). Validation with a separate cohort of patients (n = 349) showed a concordance index of 0.76 and good discrimination of risks by the models, despite substantial differences in clinical variables. Random survival forest analysis produced robust models over a wide range of parameter settings. Besides well-established risk factors like age, cardiovascular disease, type 2 diabetes, and graft function, posttransplant urinary tract infection and rejection treatment were important factors. Urinary tract infection and rejection treatment were not specifically associated with death due to infection or malignancy but correlated strongly with inferior graft function and graft loss. The established models indicate the important areas that need special attention in the care of renal transplant patients, particularly modifiable factors like graft rejection and urinary tract infection.

Ten-year survival in patients with endometrial adenoacanthoma and endometrial adenocarcinoma with malignant squamous cell differentiation

Ten-year survival in patients with endometrial adenoacanthoma and endometrial adenocarcinoma with malignant squamous cell differentiation