Long-term Outcome Of Adult Patients Research Articles

P0311INFECTION-RELATED GLOMERULONEPHRITIS IN ADULTS: TIME FOR A DEFINITIVE PARADIGM SHIFT?

Abstract Background and Aims The recent decades have witnessed significant changes in the epidemiology and clinical course of infection-related glomerulonephritis (IRGN). We analyzed the clinicopathological features and long-term outcome of adult patients with biopsy-proven IRGN followed in a large Italian referral centre. Method We included patients with biopsy-proven IRGN diagnosed from 2000 to 2018. Clinical and laboratory findings, histological features, possible risk factors and therapy were assessed for both renal and patient outcome. Results Forty-one patients met the inclusion criteria (male:female ratio 3:1, mean age 61±16 years). Smoke habit (47.2%), alcoholism (30.6%), and diabetes (27.5%) were the most common risk factors. The most frequently identified sites of infection were skin, lung and heart (Table 1). Staphylococci spp. accounted for 76.5% of positive cultures (Figure 1). Hypocomplementaemia emerged in 48.5% of cases. The most frequent histologic patterns were diffuse proliferative (56.0%) and membranoproliferative (29.3%) glomerulonephritis. Haemodialysis was required by 22.5% of patients at inception. Two thirds of patients developed chronic kidney disease; half of them reached end-stage renal disease (ESRD). By multivariate analysis ESRD was associated with diabetes (HR 13.7; 95% CI, 1.6-121.0; p=0.018), crescents (HR 25.2; 95% CI, 2.7-235.7; p=0.005), and interstitial fibrosis (HR 31.0; 95% CI, 3.3-287.3; p=0.003). Male gender (HR 12.7; 95% CI, 10.8-14.6; p=0.008) hypertension (HR 40.8; 95% CI, 38.6-43.1; p=0.001), gross haematuria (HR 11.8; 95% CI, 9.4-14.2; p=0.047), need for haemodialysis at onset (HR 16.3; 95% CI, 14.7-17.8; p<0.0001), and interstitial infiltrate (HR 13.3; 95% CI, 11.6-15.0; p=0.003) significantly affected survival. Corticosteroids did not affect prognosis. Conclusion While traditionally considered a “benign” disease with a favorable course in children, IRGN is a potentially severe disease in adults, particularly when a background of major comorbidities and older age are present. A significant proportion of patients does not recover renal function, with a remarkable risk of ESRD.

EPS1.01 Long-term outcomes of adult patients with cystic fibrosis requiring non-invasive ventilation

ePS1.01 Long-term outcomes of adult patients with cystic fibrosis requiring non-invasive ventilation

Long-term outcomes of percutaneous closure of coronary artery fistulae in the adult: A single-center experience.

We describe our 20-year experience with percutaneous closure of CAF in adult patients, focusing on long-term outcomes. From 1995 to 2015, 25 adult patients underwent 33 transcatheter interventions to close 26 CAF at our institution. Mean age was 51.4 ± 16.8 years, and 14 (56%) were female. All the patients were symptomatic with dyspnea (21/25, 84%) being the most common complaint followed by chest pain (17/25, 68%), palpitations (9/25, 36%), and heart failure (2/25, 8%). Two patients presented with acute coronary syndrome, whereas 10/25 (40%) also had inducible ischemia on stress test. These CAFs were classified as medium (12, 46.2%), large (5, 19.2%), and giant (9, 34.6%). Immediately postprocedure complete occlusion was achieved in 21 (21/26, 81%), and residual trace leak was observed in three fistulae, whereas we could not deliver coils/device in two cases. Periprocedural complications occurred in the form of coronary dissection (n = 1), myocardial infarction (n = 2), coil embolization (n = 1), transient ST elevation (n = 1); the majority of complications were observed in the early years of our experience. Patients reported marked improvement in symptoms after the intervention. During a mean follow-up of 76 ± 69 (5-214 months) (n = 22), 15 patients were investigated by either conventional (n = 6) or CT angiography (n = 8) or both (n = 1) that demonstrated thrombotic occlusion of a proximal coronary artery where a distal fistula was treated. Another patient was noted to have thrombus proximal to a vascular plug in a distal giant fistula. Three patients were lost to follow-up. None of the investigated patients had recanalization of their fistula. We did not observe remodeling of the native coronary artery that was feeding the fistula, even after successful complete closure. Percutaneous device closure of CAF is a feasible and effective therapeutic intervention in adult patients; however, patients with distal fistulas represent a significant challenge and the outcomes of these procedures are in question. All patients should have long-term angiographic follow-up.

Characteristics and outcomes of patients with solid tumors receiving chemotherapy in the intensive care unit.

The objective of this study was to evaluate the short- and long-term outcomes of adult patients with solid tumors receiving chemotherapy in the intensive care unit (ICU). This was a retrospective single-center study comparing the outcomes of patients with solid tumors who received chemotherapy in the ICU with a matched cohort of ICU patients (by age, sex, and tumor type) who did not receive chemotherapy. Conditional logistic regression and shared frailty Cox regression were used to assess short-term (ICU and hospital) mortality and death by 12-month post-hospital discharge, respectively. Seventy-three patients with solid tumors who received chemotherapy in the ICU were successfully matched. The most common solid tumors included thoracic (30%), genitourinary (26%), and breast (16%). The ICU, hospital, and 12-month (post discharge) mortality rates of patients who recieved chomtherapy in the ICUwere 23%, 36%, and 43%, respectively. When compared to the matched cohort of patients who did not receive chemotherapy, patients who received chemotherapy had a significantly longer length of stay in the ICU (median 7 vs. 4days, p < 0.001) and hospital (median 15 vs. 11days, p = 0.011) but similar short-term ICU and hospital mortality rates (23% vs. 18% and 36% vs. 38%, respectively). Patients who received chemotherapy in the ICU were at a lower risk of death by 12months (HR 0.31, p < 0.001) compared to the matched cohort onmultivariable analysis. Patients with solid tumors who received chemotherapy had increased ICU and hospital length of stay compared to patients who did not. Although short-term mortality did not differ, patients who received chemotherapy in the ICU had improved long-term survival. Our data can inform critical care triage decisions to include patients who are to receive chemotherapy in the ICU.

Long-Term Outcomes After Transcatheter Atrial Septal Defect Closure Among Adults in Comparison to Surgery and General Population

Background: Owing to the limited data on long-term outcomes following the implantation of atrial septal occluders, their permanent nature and potential for adverse outcomes remain concerning. Methods: In this retrospective cohort study, the long-term outcomes of adult patients who underwent transcatheter and surgical closure at Peter Munk Cardiac Centre, Toronto, Canada between 1991-2015 were determined using administrative databases linkages. The transcatheter group was compared to an age-sex matched population (each included 1278 participants) in 1:1 fashion and to a surgical group (240 patients) using propensity weighting. The primary endpoint was all-cause mortality. Results: Median age was 48 years and 39 years for the transcatheter and surgical groups respectively. Compared to age-sex matched group, mortality following transcatheter closure was not significantly different after a median follow-up of 9 years [7.9 vs 6.4 per 1000-patient-years, (difference-95%CI, -0.68,4.49), P=0.20], with non-significantly different rates of stroke [1.9 vs 1.1 per 1000-patient-years, (difference-95% CI, -0.2,2.69), P=0.16] but significantly higher rates of atrial fibrillation [22 vs 4.4 per 1000-patient-years, (difference-95% CI, 11.6,25.9), P<0.001] and heart failure [2.3 vs 0.5 per 1000-patient-years, (difference-95% CI,0.43,5.64), P<0.001]. Compared to surgery, rates of stroke [1.4 vs 11.4 per 1000-patient-years, (difference-95% CI, -11.3, -2.22), P=0.03] and atrial fibrillation [79.1 vs 142 per 1000-patient-years, (difference-95% CI, -89.5, -22.4), P<0.001] were significantly lower over 1 year follow-up. Mortality rates were not significantly different over 1 year [7.4 vs 6.3 per 1000-patient-years, (difference-95% CI, -5,35.4), P=0.85] and in long-term follow-up [8.2 vs 9.7 per 1000-patient-years, (difference-95% CI, -3.98,2.22), P=0.39] for the transcatheter and surgery groups, respectively. The need for any open-heart surgery during follow-up was not significantly different between the two approaches [3.6 vs 3.5 per 1000-patient-years, (difference-95% CI, -1.54, 2.94) P=0.95]. Conclusions and Interpretation: Among patients undergoing transcatheter or surgical closure between 1991 and 2015, there was no significant difference in mortality after a median 9 years of follow-up. Transcatheter closure was also not associated with greater mortality than age- and sex-matched individuals from the general population. Funding Statement: ICES is supported in part by a grant from the Ontario Ministry of Health and Long Term Care (MOHLTC). Parts of this material are based on data and information compiled and provided by the Canadian Institute for Health Information (CIHI). However, the analyses, conclusions, opinions and statements expressed in the material are those of the authors and not necessarily of CIHI and no endorsement by MOHLTC or ICES is intended or should be inferred. The statistical analysis was conducted independently by ICES. The study was supported by funding from the Peter Munk Chair in Structural intervention and a grant from Abbott Structural Heart®. Declaration of Interests: Dr. Lee is supported by a Mid-Career investigator award from the Heart and Stroke Foundation and the Ted Rogers Chair in Heart Function Outcomes, a joint Hospital468 University Chair of the University Health Network and the University of Toronto. Dr. Austin is a Mid-Career investigator of the Heart and Stroke Foundation. Dr. Eric Horlick and Dr. Mark Osten were consultants for Abbott Structural Heart®. Dr. Meier is supported by an educational grant from the Gottfried und Julia Bangerter-Rhyner-Stiftung Foundation. All other authors have no disclosures. Ethics Approval Statement: This study was approved by the University Health Network Ethics Review Board and had a waiver of participant consent granted by the institutional ethics board.

SUN-LB074 Racial Distribution, Presentation and Outcome in Acromegaly and Cushing's Disease: A Tertiary Referral Center Study in 220 Patients

Background: Transsphenoidal surgery (TSS) is the primary therapy for acromegaly (ACM) and Cushing’s disease (CD) with superior outcomes at high-volume centers. According to the U.S. Central Brain Tumor Registry, African Americans (AA) have higher incidence of pituitary adenomas than non-Hispanic white (W) patients, but race-specific data in ACM and CD are not available. Our aims are to evaluate racial distribution, presentation, and long-term outcomes of adult patients who underwent TSS for these conditions at our pituitary center by a single dedicated neurosurgeon. Methods: We reviewed all cases of TSS operated between 1994-2016 and compared presentation and postoperative events (biochemical remission, recurrence, reoperation, radiation, and death) across racial demographic groups. For patients living in Metropolitan Atlanta, we used U.S. Census data to compare patient racial distribution to the demographics. Results The ACM group consisted of 112 patients (54 men and 58 women), of whom 87 were W and 13 AA. Age and gender were similar in AA and W. Main reasons for referral were physical changes (23% AA vs 44% W), headaches (38% AA vs 17% W), and pituitary incidentaloma (31% AA vs 14% W). AA had higher rates of diabetes (54% vs 16%, p 0.005) and hypertension (69% vs 42% p 0.08), lower mean IGF-1 levels (662±324 ng/ml vs 823±321 ng/ml, p 0.06), and lower median GH levels (8.6. vs 45 ng/ml p 0.002). Mean tumor diameter and cavernous sinus invasion were similar. Biochemical remission at 3 months (46% AA and 55% W) and event-free survival during follow-up (median 3.8 years) were similar in AA and W. Normal IGF-1 at last follow-up was achieved by 92% AA and 80%W after multimodality therapy. The CD group consisted of 108 patients (15 men and 93 women) of whom 70 were W and 24 AA. Age, gender, and prevalence of diabetes and hypertension were similar in AA and W. Mean urinary cortisol and ACTH levels were similar. Mean tumor diameter was similar but AA had more macroadenomas (33% AA vs 14% W). During follow-up (median 4.3 years), event-free survival was similar although AA had fewer recurrences (4.35% vs 22.0% W, p 0.06). Of the total 220 operated patients, 88% lived in Georgia and 59% in Metropolitan Atlanta. In the latter group, W represented 75% of ACM and 62% of CD patients; AA 16% ACM and 28% CD; Hispanics (H) 0% ACM and 6.5% CD; Asian 8.2% ACM and 3% CD. Census racial distribution during study period was 58-67% W, 33-34% AA, 7-10% H, and 3-5% Asians. Conclusions To our knowledge, this is the first report of race-specific patient characteristics in CD and ACM. Proportion of AA and H with ACM operated at our tertiary referral center was lower than expected, while such disparity was not seen in CD. In addition AA with ACM exhibited different clinical presentations and milder biochemical activity. Notably, the long-term outcomes of patients with ACM and CD treated at our center were similar in AA and W. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

Long-term outcomes after extracorporeal membrane oxygenation in patients with dialysis-requiring acute kidney injury: A cohort study.

BackgroundAcute kidney injury (AKI) is a common complication of extracorporeal membrane oxygenation (ECMO) treatment. The aim of this study was to elucidate the long-term outcomes of adult patients with AKI who receive ECMO.Materials and methodsThe study analyzed encrypted datasets from Taiwan’s National Health Insurance Research Database. The data of 3251 patients who received first-time ECMO treatment between January 1, 2003, and December 31, 2013, were analyzed. Characteristics and outcomes were compared between patients who required dialysis for AKI (D-AKI) and those who did not in order to evaluate the impact of D-AKI on long-term mortality and major adverse kidney events.ResultsOf the 3251 patients, 54.1% had D-AKI. Compared with the patients without D-AKI, those with D-AKI had higher rates of all-cause mortality (52.3% vs. 33.3%; adjusted hazard ratio [aHR] 1.82, 95% confidence interval [CI] 1.53–2.17), chronic kidney disease (13.7% vs. 8.1%; adjusted subdistribution HR [aSHR] 1.66, 95% CI 1.16–2.38), and end-stage renal disease (5.2% vs. 0.5%; aSHR 14.28, 95% CI 4.67–43.62). The long-term mortality of patients who survived more than 90 days after discharge was 22.0% (153/695), 32.3% (91/282), and 50.0% (10/20) in the patients without D-AKI, with recovery D-AKI, and with nonrecovery D-AKI who required long-term dialysis, respectively, demonstrating a significant trend (Pfor trend <0.001).ConclusionAKI is associated with an increased risk of long-term mortality and major adverse kidney events in adult patients who receive ECMO.

The Impact of Low-Dose Cranial Boost on the Long-Term Outcomes of Adult Patients with High-Risk Acute Lymphoblastic Leukemia Undergoing Total Body Irradiation and Allogeneic Hematopoietic Stem Cell Transplantation

PurposeTotal body irradiation (TBI) is an integral part of the conditioning regimen for patients with acute lymphoblastic leukemia (ALL) undergoing allogeneic, hematopoietic, cell transplantation (allo-HCT). There are conflicting data in the literature regarding the utility of a cranial irradiation boost in high-risk adult ALL without evidence of preexisting central nervous system (CNS) involvement. This study investigates the posttransplant clinical outcomes of patients with high-risk adult ALL undergoing TBI conditioning for allo-HCT with or without a whole-brain boost, without overt CNS involvement at the time of diagnosis. Methods and materialsA retrospective cohort study was conducted using a medical record analysis. We identified 58 patients who were treated between January 1998 and December 2016, and met our preset inclusion criteria of adults (age >18 years old) who carried a pathologically confirmed diagnosis of CNS-negative, high-risk ALL, who underwent hematopoietic stem cell transplantation with TBI conditioning. A multivariate analysis of correlation between patient outcomes and collected categorical variables was assessed with stepwise Cox logistic regression. Survival analyses were assessed using the Kaplan-Meier technique with a log-rank test. ResultsWith a median follow-up time of 5.3 years, there was a statistically significant improvement in actuarial 7-year CNS relapse-free survival (100% vs 76.4%; P = .043) in favor of patients undergoing a cranial boost. There was no statistically significant improvement in 7-year progression-free survival (78.3% vs 62.5%; P = .076) or overall survival (49.4% vs 43.5%; P = .921) with versus without a cranial boost. On multivariate analysis, the presence of a cranial boost was the only identified variable with an independent relationship to CNS relapse-free survival. ConclusionsAdult patients with high-risk, CNS-negative ALL were found to have a statistically significant improvement in CNS relapse-free survival and a trend toward improved progression-free survival with the inclusion of a cranial boost with TBI pretransplant conditioning. Our data indicate that further investigation into the use of cranial boost in this patient population is warranted.

Long-Term Outcomes of Adult Patients with High-Risk Acute Lymphoblastic Leukemia Undergoing Total Body Irradiation with or without Whole Brain Boost

Long-Term Outcomes of Adult Patients with High-Risk Acute Lymphoblastic Leukemia Undergoing Total Body Irradiation with or without Whole Brain Boost

Healthcare costs and outcomes in adult patients with juvenile idiopathic arthritis: a population-based study

Objectives: Evidence of the economic burden and long-term outcomes of juvenile idiopathic arthritis (JIA) remains scarce. Our aim was to explore healthcare costs and long-term outcomes in adult patients with JIA.Method: We identified all adult patients (≥ 18 years) with JIA who visited Jyväskylä Central Hospital rheumatology unit between May 2007 and March 2016. We considered individual medians of time-dependent clinical variables. These data were linked to administrative data from the area from the fiscal year 2014, which include information on all public healthcare contacts. Healthcare utilization is presented as direct costs in euros (EUR). Factors affecting direct costs were assessed with a generalized linear model.Results: In 218 patients, median 28-joint Disease Activity Score with three variables (DAS28-3) was < 2.6 in 88.6% in those aged < 30 and in 72.9% in those aged ≥ 30 years, and median Health Assessment Questionnaire (HAQ) score was < 0.5 in 85.7% and 45.4%, respectively. In the utilization data (four municipalities, 137 patients), the total annual health services-related direct costs were 432 257 EUR (mean = 3155 EUR/patient/year). Thirty-six patients (26.3%) used biological disease-modifying anti-rheumatic drugs (bDMARDs) in 2014 for a total of 355 months, and the annual cost of bDMARDs was estimated at 355 000 EUR. Those with active disease had mean costs 2.4-fold higher than those with low or no disease activity. A one-point increase in median raw HAQ incurred an average 228 EUR increase in annual costs (p = 0.03).Conclusion: Most adult patients with JIA seem to manage well with their arthritis, bearing in mind that there still is room for improvement in long-term outcomes.

Fatal heart failure caused by severe pulmonary regurgitation, tricuspid regurgitation and late-onset mitral stenosis in an adult patient with Noonan syndrome: a case report

BackgroundIn patients with Noonan syndrome (NS), cardiac disorders such as pulmonary valve stenosis (PS) or hypertrophic cardiomyopathy (HCM) are common. While some patients can develop heart failure associated with HCM, the long-term outcome of adult patients with NS is reported to be good. Fatal outcomes of heart failure in patients with NS but without HCM are rare.Case presentationWe report a 25-year-old Japanese woman diagnosed with NS in adulthood. She exhibited short stature and minor facial dysmorphism and was diagnosed with PS at 1 year of age. After surgical valvuloplasty for PS at 6 years of age, her general condition became stable without specific medical treatment. She discontinued regular medical follow-up for PS. At 21 years of age, she developed acute decompensated heart failure, which was mainly right-sided heart failure due to severe pulmonary regurgitation (PR) and tricuspid regurgitation (TR). There was no evidence of HCM or PS recurrence. On the basis of the history of PS and characteristic physical features including short stature, webbed neck, and hypertelorism, she was clinically diagnosed with NS. At 25 years of age, she developed heart failure of both sides due to PR, TR and late-onset severe mitral stenosis (MS). The etiology of MS was uncertain. Owing to the patient’s condition, surgical options were considered to be extremely high risk. She was treated with optimal medical treatment as well as the occasional abdominal cavity drainage for recurrent ascites; however, she died of decompensated heart failure at 27 years of age.ConclusionsWe describe an adult patient with NS without HCM who died of heart failure caused by severe PR, TR and MS. Clinicians should recognize that ongoing or late-onset cardiac disorders can develop in patients with NS, and lead to fatal heart failure. Optimal medical follow-up to monitor cardiac function and early identification of heart failure are important.

Characteristics and outcomes of patients with hematologic malignancies receiving chemotherapy in the intensive care unit.

The objective of this study was to evaluate the short-term and long-term outcomes of adult patients with hematologic malignancies who received chemotherapy in the intensive care unit (ICU). This was a retrospective, single-center study comparing the outcomes of patients with hematologic malignancies who received chemotherapy in the ICU with a matched cohort of ICU patients who did not receive chemotherapy. Conditional logistic regression and shared-frailty Cox regression were used to assess short-term (ICU and hospital) mortality and death by 12 months after hospital discharge, respectively. One hundred eighty-one patients with hematologic malignancies received chemotherapy in the ICU. The ICU and hospital mortality rates were 25% and 42% for chemotherapy patients and 22% and 33% for non-chemotherapy patients, respectively. Higher severity of illness scores on ICU admission were significantly associated with higher ICU mortality (odds ratio, 1.07; P < .001) and hospital mortality (odds ratio, 1.05; P ≤ .001). Six-month and 12-month survival estimates posthospital discharge were 58% and 50%, respectively. Compared with the matched cohort of patients who did not receive chemotherapy, those who did receive chemotherapy had a significantly longer length of stay in the ICU (median, 6 vs 3 days; P < .001) and in the hospital (median, 22 vs 14 days; P = .024). In multivariable analysis, the patients who received chemotherapy in the ICU had a trend toward a higher risk of dying by 12 months (hazard ratio, 1.45; P = .08). Short-term mortality was similar among patients with hematologic malignancies who did and did not receive chemotherapy in the ICU, although patients who received chemotherapy had increased resource utilization. These results may inform ICU triage and goals-of-care discussions with patients and their families regarding outcomes after receiving chemotherapy in the ICU. Cancer 2018;124:3025-36. © 2018 American Cancer Society.

Long-Term Outcomes of Adult Patients with Ependymoma (P6.150)

Long-Term Outcomes of Adult Patients with Ependymoma (P6.150)

A systematic review of segmental vs subtotal colectomy and subtotal colectomy vs total proctocolectomy for colonic Crohn's disease.

Surgical management of colonic Crohn's disease (CD) is still unclear because different procedures can be adopted. The choice of operation is dependent on the involvement of colonic disease but the advantages and disadvantages of the extent of resection are still debated. The aim of the present study was to evaluate the differences in short-term and long-term outcomes of adult patients with colonic CD who underwent either subtotal colectomy and ileorectal anastomosis (STC) or segmental colectomy (SC) or total proctocolectomy and end ileostomy (TPC). Studies published between 1984 and 2012 including comparisons of STC vs SC and of STC vs TPC were selected. The study end-points were overall and surgical recurrence, postoperative morbidity and incidence of permanent stoma. Fixed effect models were used to evaluate the study outcomes. Eleven studies, consisting of a total of 1436 patients (510 STC, 500 SC and 426 TPC), were included. Analysis of the data showed no significant difference between STC and SC in terms of overall and surgical recurrence of CD. In contrast, STC showed a higher risk of overall and surgical recurrence of CD than TPC (OR 3.53, 95% CI 2.45-5.10, P<0.0001; OR 3.52, 95% CI 2.27-5.44, P<0.0001, respectively). SC had a higher risk of postoperative complications compared to STC, and STC had a lower risk of complications than TPC (OR 2.84, 95% CI 1.16-6.96, P<0.02; OR 0.19, 95% CI 0.09-0.38, P<0.0001, respectively). SC resulted in a lower risk of permanent stoma than STC (OR 0.52, 95% CI 0.35-0.77). All three procedures were equally effective as treatment options for colonic CD and the choice of operation remains intrinsically dependent on the extent of colonic disease. However, patients in the TPC group showed a lower recurrence risk than those in the STC group. Moreover, SC had a higher risk of postoperative complications but a lower risk of permanent stoma. These data should be taken into account when deciding surgical strategies and when informing patients about postoperative risks.

Single‐center experience of living donor liver transplantation in patients with portal vein thrombosis

Portal vein thrombosis (PVT) is a relative contraindication in living donor liver transplantation (LDLT). We investigated the long-term outcome of adult patients with PVT in LDLT. Between 2004 and 2009, 471 cases of adult LDLT were performed and 56 patients had PVT (11.8%). Thrombectomy was attempted using a modified eversion technique. We evaluated the outcome of patients with PVT according to grade and compared with no-PVT patients. There was no difference in terms of age, gender, Child-Pugh score, MELD score, proportion of malignance, operation time, and total amount of transfused blood. Complete thrombectomy was successful in 73.2% (41/56), partial thrombectomy in 26.8% (15/56), and one case needed jump graft for portal vein reconstruction. Among patients with partial thrombectomy, when the PV velocity was above 20cm/s, the remnant thrombus disappeared in 46%. The rate of PV complication was statistically not different (8.9% vs 3.4%, P=.062). Five-year survival of mild PVT was 69.3%, 60.6% for severe PVT, and 80.4% for no-PVT (P=.059). Eversion thrombectomy by modified technique is feasible in most cases of PVT. Good long-term outcome may be expected in LDLT with PVT.

Incidence and long-term outcomes of adult patients with diabetic ketoacidosis admitted to intensive care: A retrospective cohort study.

Diabetic ketoacidosis is a life-threatening but avoidable complication of diabetes mellitus often managed in intensive care units. The risk of emergency hospital readmission in patients surviving an intensive care unit episode of diabetic ketoacidosis is unknown. We aimed to report the cumulative incidence of emergency hospital readmission and costs in all patients surviving an intensive care unit episode of diabetic ketoacidosis in Scotland. We used a national six-year cohort of survivors of first diabetic ketoacidosis admissions to Scottish intensive care units (1 January 2005-31 December 2010) identified in the Scottish Intensive Care Society Audit Group registry linked to acute hospital and death records (follow-up censored 31 December 2010). Diabetic ketoacidosis-related emergency readmissions were identified using International Classification of Disease-10 codes. During the study period, 386 patients were admitted to intensive care units in Scotland with diabetic ketoacidosis (admission rate 1.5/100,000 Scottish population). Median age was 44 (IQR 29-56); 51% male; 55% required no organ support on admission. Mortality after intensive care unit admission was 8% at 30 days, 18% at one year, and 35% at five years. A total of 349 patients survived their first intensive care unit diabetic ketoacidosis admission [mean (SD) age 42.5 (18.1) years; 50.4% women; 46.1% required ≥1 organ support]. Following hospital discharge, cumulative incidence of 90-day, one-year, and five-year diabetic ketoacidosis readmission (all-cause readmission) was 13.8% (31.8%), 29.7% (58.9%) and 46.4% (82.6%). Diabetic ketoacidosis in patients requiring intensive care unit admission is associated with high risk of long-term mortality and high hospital costs. An understanding of the precipitating causes of diabetic ketoacidosis in patients admitted to intensive care units may allow patients who are at high risk to be targeted, potentially reducing future morbidity and the substantial burden that diabetic ketoacidosis currently places on the healthcare system.

Long-term outcomes of adult patients admitted with sepsis to brazilian public hospitals: a national retrospective matched cohort study.

Although sepsis outcomes are relatively well known for developed countries, very few studies evaluated the long-term prognosis of septic patients at a national level in developing countries.

Long-term outcomes in adult patients with ischemic-type moyamoya disease involving posterior circulation.

The object of this study was to compare the long-term outcomes in adult patients with moyamoya disease (MMD) with posterior circulation involvement (PCi) treated through surgical revascularization with those in adult patients without PCi. The records of 32 consecutive adult patients with ischemic-type MMD who were treated with revascularization were reviewed. Twelve of these patients (38 %) had PCi at initial onset. Clinical characteristics of the patients with PCi were compared to those without PCi. Neurological outcomes were assessed using the modified Rankin Scale (mRS) in the preoperative and postoperative follow-up periods. A five-year Kaplan-Meier stroke risk was calculated. The frequency of presenting with infarction was significantly higher among patients with PCi than among those without PCi (p = 0.006). mRS scores in the preoperative period were significantly higher in patients with PCi than in patients without PCi (p = 0.0004). There were no significant differences in mRS scores between the preoperative and postoperative follow-up period in patients with PCi (p = 0.3), nor were there any between the preoperative and postoperative follow-up periods in patients without PCi (p = 0.2). The five-year Kaplan-Meier risk of surgical morbidity and ipsilateral stroke was 14.3 % in surgically treated hemispheres with PCi versus 14.9 % in surgically treated hemispheres without PCi (p = 0.96). PCi at initial onset was significantly correlated with poor outcome. Revascularization for the middle cerebral artery territory in patients with PCi was effective at preventing recurrent ischemic stroke.

Long-term follow-up of children who underwent severe hypospadias repair using an online survey with validated questionnaires

ObjectiveFew studies of hypospadias repair in childhood have used validated questionnaires to investigate outcomes of cosmesis, urinary function, and sexual function in adulthood. We sought to investigate long-term outcomes in adult patients who had undergone severe hypospadias repair as children using an existing web-based application available to multiple institutions in order to develop an online patient survey of previously validated questionnaires. Materials and methodsPatients aged 18 years or older who underwent severe hypospadias repair between 1992 and 1997 at our institution were contacted to complete an online survey. Through medical chart reviews, we analyzed the location of meatus, type of repair, and complications. The online survey included questions about penile appearance, and validated questionnaires to assess urinary and sexual function. ResultsOf 58 patients who met the inclusion criteria, we contacted 19, and 13 completed the survey. Fifty-nine percent had complications, with an average of 2.2 procedures per patient. Most (85.0%) were satisfied with penile appearance, although 38.0% had residual penile curvature. Hypospadias patients had mean lower orgasmic function than normal controls. Mean scores for urinary function and other domains of sexual function were similar to normal controls. ConclusionsAlthough the majority of adult patients were satisfied with the outcomes of penile appearance, urinary function, and sexual function, our online survey suggests decreased lower orgasmic function as measured by validated questionnaire. An online survey accessible to multiple institutions with validated questionnaires may facilitate assessment of long-term hypospadias results.

The Long-Term Outcome Of Adult Patients With Acute Myeloid Leukemia NPM+ and FLT3/ITD- Is Not Significantly Improved By The Application Of An Allogeneic Stem Cell Transplantation

▪ IntroductionThe discovery of the NPM mutation in acute myeloid leukemia (AML) allowed to identify a distinct entity with intermediate-good prognosis particularly when the FLT3/ITD mutation is absent. The most appropriate consolidation treatment of these patients upon the achievement of the first complete remission has not been established yet and the role of allogeneic stem cell transplantation (SCT) is still debated. Aimto assess long-term outcome of adult patients with NPM positive acute myeloid leukemia according to type and intensity of consolidation therapy. Patients and methodsBetween May 2000 and February 2012, 1155 patients were enrolled into two consecutive, prospective Northern Italy Leukemia Group (NILG) trials (00/01 and 02/06). Six-hundred sixty nine were studied for NPM mutation and 218 (33%) proved positive (by immunohistochemistry or by molecular analysis) (Falini et al, Haematologica. 2007 Apr;92(4):519-32). Median age of NPM+ patients was 50 years (range 16-72) and 134 (61%) were female. Median WBC was 33.3 x 10^9/L (range 0.9-313.9), 71 (33%) had myelomonocytic leukemia (FAB M4), and 211 (96%) had de novo AML. According to the European Leukemia Net (ELN) classification, cytogenetic risk groups were: normal 178 (82%), intermediate 26 (12%), unfavorable 2 (1%) and unknown 12 (5%). Eighty-two (38%) patients had a concurrent FLT3/ITD mutation and 31 (14%) a FLT3/TKD mutation. According to cytogenetics and additional risk factors (late response, WBC count >50x10^9/L, FAB class M0/6/7, hepato/splenomegaly, MDS-related/secondary AML, FLT3/ITD mutation), patients were stratified in standard (SR) and high (HR) risk groups.In both studies the remission induction was based on combination of cytarabine with idarubicin. Post-remission therapy was allogeneic SCT in HR while high-dose cytarabine or busulfan/cyclophosphamide with autologous SCT was given to SR patients. The molecular evaluation of minimal residual disease (MRD) was planned after induction, before the post-remission consolidation and the follow-up. ResultsComplete remission (CR) was achieved in 196/218 (90%) NPM+ patients. One hundred sixty-eight out of 196 remitters (86%) received post-remission consolidation therapy: allogeneic SCT 72 (37%), high dose Ara-C 74 (38%), autologous SCT 14 (7%), other therapy 8 (4%); 28 patients did not receive consolidation due to early relapse (n=24), CR death (n=3), and loss to follow-up (n=1). With a median follow-up of 1.8 years (range 0.008-12.66), 99 CR patients (50.5%) were alive in 1st CR, 13 (6.5%) died of complications, and 84 (43%) had recurrent AML. In a cumulative analysis, 5-year overall and disease-free survival were 46% (OS) and 43% (DFS), respectively. In univariate analysis FLT3/ITD mutation (n=82) affected negatively 5-year OS (29% vs. 49%, P < .0001) and DFS (27% vs. 46%, P < .0001), whereas FLT3/TDK mutation did not. In patients with FLT3/ITD mutation able to receive consolidation therapy (n=49), the application of allogeneic SCT improved DFS significantly (55% vs. 18%, P = .03) and reduced the cumulative incidence of relapse (CIR) (39% vs. 81%, P = .026). In patients with NPM+ and FLT3/ITD- AML, the risk of relapse after high dose cytarabine or autologous SCT (n=68) was more than doubled compared to that observed after allogeneic SCT (n=42) (50% vs. 23%, P= .08) (Figure). However, DFS (48% vs. 69.5%, p= .17), and OS (60% vs. 72%, p= .80) were not significantly different since allogeneic SCT was associated with higher treatment related mortality, albeit effective in the salvage of some relapsed patients. In multivariate analysis, FLT3/ITD mutation was the most powerful factor that unfavorably affected OS, DFS and CIR, while age > 55 years negatively affected OS and DFS. No other clinical factor was predictive for relapse in FLT3- patients. The relationship between MRD and clinical outcome is currently being studied and will be presented. ConclusionsOur data indicate that allogeneic SCT is the most active post-remission treatment for NPM+ AML, but its benefit over chemotherapy may be limited in patients without FLT3/ITD. For this reason the evaluation of MRD could help identify the patients for whom an allogeneic SCT should be preferable. [Display omitted] Disclosures:No relevant conflicts of interest to declare.