Successful Sequential Liver and Isolated Intestine Transplantation for Mitochondrial Neurogastrointestinal Encephalopathy Syndrome: A Case Report.

BACKGROUND Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) is an autosomal recessive disease caused by thymidine phosphorylase deficiency leading to progressive gastrointestinal dysmotility, cachexia, ptosis, ophthalmoparesis, peripheral neuropathy and leukoencephalopathy. Although liver transplantation corrects thymidine phosphorylase deficiency, intestinal deficiency of the enzyme persists. Retrospective chart review was carried out to obtain clinical, biochemical, and pathological details. CASE REPORT We present a case of liver and subsequent intestine transplant in a 28-year-old man with MNGIE syndrome with gastrointestinal dysmotility, inability to walk, leukoencephalopathy, ptosis, cachexia, and elevated serum thymidine. To halt progression of neurologic deficit, he first received a left-lobe partial liver transplantation. Although his motor deficit improved, gastrointestinal dysmotility persisted, requiring total parenteral nutrition. After exhaustive intestinal rehabilitation, he was listed for intestine transplantation. Two-and-half years after liver transplantation, he received an intestine transplant. At 4 years after LT and 20 months after the intestine transplant, he remains off parenteral nutrition and is slowly gaining weight. CONCLUSIONS This is the first reported case of mitochondrial neurogastrointestinal encephalomyopathy to undergo successful sequential liver and intestine transplantation.

Outcome of reverse shoulder arthroplasty secondary to rotator cuff arthropathy in a low-income population.

BACKGROUND: Reverse shoulder arthroplasty (RSA) is a valuable treatment for rotator cuff arthropathy (RCA) in developed regions. Socioeconomic issues impact access to specialized care and there is a lack of data on RSA outcomes in developing regions. We present our 24-month follow-up on RSA surgeries to treat RCA in our low-income population. METHODS: Prospective evaluation of 26 patients subjected to RSA at Hospital Geral de Fortaleza-CE, Brazil, between January 2018 and December 2020. Literacy [>/≤ 8 school years(SY)] and income were documented. Outcomes considered pain (visual analogue scale; VAS) as well as SSV, SPADI, ASES, and UCLA scoring, and range of motion [forward flexion (FF); external rotation (ER)]. RESULTS: Patients were 68.5 ± 7.6 years-old with 16(61.5%) females; 65% had hypertension and 7 (26.9%) had diabetes. Over 90% declared < 900.00 US$ monthly family earnings and 10 (38.4%) patients declared ≤8 SY with > 80% exerting blue-collar jobs. Pain showed a significant reduction from baseline (8 ± 2) to 24 months (2.1 ± 2.3; p < 0.001). UCLA (10.3 ± 5.6 and 28.6 ± 7.2), ASES (16.7 ± 10.8 and 63.1 ± 28.4), SSV (326 ± 311 and 760 ± 234), and SPADI (98.3 ± 26.5) scores significantly improved from baseline to 24 months, achieving minimal clinically important difference. FF (89.2° ± 51.2° to 140.6 ± 38.3°) and ER (19.2° ± 22.5 to 33.4° ± 20.6°) significantly improved from baseline to 24 months (p = 0.004 and 0.027, respectively). There were 5 non-serious adverse events with one surgical revision. All patients returned to daily life activities. CONCLUSION: This is the first outcome report 2 years following RSA in a low-income population. Data indicate this procedure is justifiable regardless of socioeconomic issues.

Thirty-Day Readmissions Are Largely Not Preventable in Patients With Cirrhosis.

INTRODUCTION: Hospital readmissions are common in patients with cirrhosis, but there are few studies describing readmission preventability. We aimed to describe the incidence, causes, and risk factors for preventable readmission in this population. METHODS: We performed a prospective cohort study of patients with cirrhosis hospitalized at a single center between June 2014 and March 2020 and followed up for 30 days postdischarge. Demographic, clinical, and socioeconomic data, functional status, and quality of life were collected. Readmission preventability was independently and systematically adjudicated by 3 reviewers. Multinomial logistic regression was used to compare those with (i) preventable readmission, (ii) nonpreventable readmission/death, and (iii) no readmission. RESULTS: Of 654 patients, 246 (38%) were readmitted, and 29 (12%) were preventable readmissions. Reviewers agreed on preventability for 70% of readmissions. Twenty-two (including 2 with preventable readmission) died. The most common reasons for readmission were hepatic encephalopathy (22%), gastrointestinal bleeding (13%), acute kidney injury (13%), and ascites (6%), and these reasons were similar between preventable and nonpreventable readmissions. Preventable readmission was often related to paracentesis timeliness, diuretic adjustment monitoring, and hepatic encephalopathy treatment. Compared with nonreadmitted patients, preventable readmission was independently associated with racial and ethnic minoritized individuals (odds ratio [OR] 5.80; 95% CI, 1.96-17.13), nonmarried marital status (OR 2.88; 95% CI, 1.18-7.05), and admission in the prior 30 days (OR 3.45; 95% CI, 1.48-8.04). DISCUSSION: For patients with cirrhosis, readmission is common, but most are not preventable. Preventable readmissions are often related to ascites and hepatic encephalopathy and are associated with racial and ethnic minorities, nonmarried status, and prior admissions.

Chameleon: a cloud computing Industry 4.0 neutron spectrum unfolding code.

This work presents Chameleon, a cloud computing (CC) Industry 4.0 (I4) neutron spectrum unfolding code. The code was designed under the Python programming language, using Streamlit framework®, and it is executed on the cloud, as I4 CC technology through internet, by using mobile devices with internet connectivity and a web navigator. In its first version, as a proof of concept, the SPUNIT algorithm was implemented. The main functionalities and the preliminary tests performed to validate the code are presented. Chameleon solves the neutron spectrum unfolding problem and it is easy, friendly and intuitive. It can be applied with success in various workplaces. More validation tests are in progress. Future implementations will include improving the graphical user interface, inserting other algorithms, such as GRAVEL, MAXED and neural networks, and implementing an algorithm to estimate uncertainties in the calculated integral quantities.

Responses of a 6LiI Bonner Sphere Spectrometer using the Monte Carlo codes PHITS and MCNPX.

The response functions (RFs) of a Bonner Sphere Spectrometer (BSS) with a 6LiI thermal neutron detector were calculated using the Monte Carlo codes PHITS (version 3.26) and MCNPX (version 2.7.0), with their own default nuclear data libraries, and physics models. RFs were compared with other published data, obtained for the same spectrometer using the MCNP6.1 code with its own physics models. A discussion on the influence of using different nuclear data libraries and physics models using these codes/versions is analyzed.

SpecUnPy: A python GUI/App for neutron spectrum unfolding.

Bonner Sphere Spectrometers (BSS) are widely used for neutron spectrometry. Spectra are obtained by unfolding detector readings. In this work, a Python Graphical User Interface Application (GUI/App) for spectrum unfolding is presented; SpecUnPy. In this App, the user can choose three unfolding algorithms: SPUNIT/MLEM/GRAVEL. There is no limit for energy bins or detectors and after unfolding, a ".xlsx" file and a graphical comparison can be downloaded. This paper presents SpecunPy and some tests performed to validate it.

MONTE CARLO SIMULATION OF THE RADIATION SOURCE TERM FROM [18O]H2O CYCLOTRON TARGET BOMBARDMENT WITH PROTONS OF 16.5 MEV.

PET cyclotrons are widely used for producing medical diagnostic radionuclides. The main radionuclide produced in these facilities is the 18F, which is obtained from the [18O (p,n)18F] reaction when 18O-enriched water is bombarded with the proton beams. This work aimed to estimate the radiation source term from the bombardment of an 18O-enriched water target with protons of 16.5 MeV to determine the radiation neutron field around the accelerator.

Liver Transplantation in Recipients With Class III Obesity: Posttransplant Outcomes and Weight Gain.

BACKGROUND: There has been a dramatic increase in obesity in the United States. Several studies have reported conflicting results for the impact of obesity on outcomes of liver transplantation (LT). This study aims to assess the impact of obesity on LT and changes in body mass index (BMI) after transplantation. METHODS: All adult LTs performed at Indiana University between 2001 and 2018 were reviewed. BMIs of recipients were subdivided into 6 categories. Survival outcomes were compared across the subgroup. BMI was followed up in a cohort of patients from 2008 to 2018. RESULTS: Among 2024 patients, 25% were in class I obesity, 9.3% were in class II obesity, and 1.1% were in class III obesity. There was no significant difference in patient and graft survival at 10-y follow-up with respect to BMI. Among 1004 patients in the subgroup, BMI of all groups except the underweight group declined in the first 3 mo postoperatively; however, the BMI of all groups except the class III obesity group returned to the pre-LT level by 2 y and reached a plateau by 5 y. In the class III obesity group, there was a significant increase in body weight at 5 y. CONCLUSIONS: Class III obesity was not associated with higher mortality in our cohort. Because our cohort is small, it may be underpowered to detect a smaller difference in outcome. From our observation, obesity should not be considered a contraindication for LT. Post-LT interventions are required to prevent significant weight gain for the class III obesity group.

Surgical outcome following rotator cuff tear repair in a low-income population. Impact of obesity and smoking.

BACKGROUND: Comorbidities and socioeconomic issues impact outcome of rotator cuff tear (RCT) repair. There are no data on RCT repair outcome from developing regions. We determined the impact of obesity and smoking following RCT repair in a low-income population. METHODS: This is a retrospective case series. Forty-seven shoulders of 42 patients subjected to open or arthroscopic repair of a RCT with a minimum of 2 years follow-up were cross-sectionally evaluated. Patients were seen in the Orthopaedic Service of the Hospital Geral de Fortaleza-CE, Brazil between March and September 2018. RCT were classified as partial or full-thickness lesions. Fatty infiltration (Goutallier) and tendon retraction (Patte) were recorded as well as obesity (BMI > 30), literacy [>/≤ 8 school years (SY)] and smoking status 6 months prior to surgery (present/absent). Outcomes included pain (visual analogue scale; VAS, 0-10 cm), range of motion [active forward flexion and external rotation (ER)], UCLA and ASES scoring. RESULTS: Patients were 59.9 ± 7.4 years-old, 35(74.4%) female with 19 (17.1-30.2 IQR) median of months from diagnosis to surgery and 25 median months of follow-up (26.9-34.0 IQR); over 90% declared < 900.00 US$ monthly family income and two-thirds had ≤8 SY. Forty patients (85.1%) had full-thickness tears, 7 (14.9%) had Goutallier ≥3 and over 80% had < Patte III stage. Outcomes were similar regardless of fatty infiltration or tendon retraction staging. There were 17 (36.1%) smokers and 13 (27.6%) obese patients. Outcome was similar when comparing obese vs non-obese patients. Smokers had more pain (P = 0.043) and less ER (P = 0.029) with a trend towards worse UCLA and ASES scores as compared to non-smokers though differences did not achieve minimal clinically important difference (MCID) proposed for surgical RCT treatment. After adjusting for obesity, VAS and ER values in smokers were no longer significant (P = 0.2474 and 0.4872, respectively). CONCLUSIONS: Our data document outcomes following RCT repair in a low-income population. Smoking status but not obesity impacted RCT repair outcome though not reaching MCID for surgical treatment.

CAD-LT score effectively predicts risk of significant coronary artery disease in liver transplant candidates.

BACKGROUND & AIMS: Patients with cirrhosis and significant coronary artery disease (CAD) are at risk of peri-liver transplantation (LT) cardiac events. The coronary artery disease in liver transplantation (CAD-LT) score and algorithm aim to predict the risk of significant CAD in LT candidates and guide pre-LT cardiac evaluation. METHODS: Patients who underwent pre-LT evaluation at Indiana University (2010-2019) were studied retrospectively. Stress echocardiography (SE) and cardiac catheterization (CATH) reports were reviewed. CATH was performed for predefined CAD risk factors, irrespective of normal SE. Significant CAD was defined as CAD requiring percutaneous or surgical intervention. A multivariate regression model was constructed to assess risk factors. Receiver-operating curve analysis was used to compute a point-based risk score and a stratified testing algorithm. RESULTS: A total of 1,771 pre-LT patients underwent cardiac evaluation, including results from 1,634 SE and 1,266 CATH assessments. Risk-adjusted predictors of significant CAD at CATH were older age (adjusted odds ratio 1.05; 95% CI 1.03-1.08), male sex (1.69; 1.16-2.50), diabetes (1.57; 1.12-2.22), hypertension (1.61; 1.14-2.28), tobacco use (pack years) (1.01; 1.00-1.02), family history of CAD (1.63; 1.16-2.28), and personal history of CAD (6.55; 4.33-9.90). The CAD-LT score stratified significant CAD risk as low (≤2%), intermediate (3% to 9%), and high (≥10%). Among patients who underwent CATH, a risk-based testing algorithm (low: no testing; intermediate: non-invasive testing vs. CATH; high: CATH) would have identified 97% of all significant CAD and potentially avoided unnecessary testing (669 SE [57%] and 561 CATH [44%]). CONCLUSIONS: The CAD-LT score and algorithm (available at www.cad-lt.com) effectively stratify pre-LT risk for significant CAD. This may guide more targeted testing of candidates with fewer tests and faster time to waitlist. LAY SUMMARY: The coronary artery disease in liver transplantation (CAD-LT) score and algorithm effectively stratify patients based on their risk of significant coronary artery disease. The CAD-LT algorithm can be used to guide a more targeted cardiac evaluation prior to liver transplantation.

Impact of Recipient Age in Combined Liver-Kidney Transplantation: Caution Is Needed for Patients ≥70 Years.

Elderly recipients (≥70 y) account for 2.6% of all liver transplants (LTs) in the United States and have similar outcomes as younger recipients. Although the rate of elderly recipients in combined liver-kidney transplant (CLKT) is similar, limited data are available on how elderly recipients perform after CLKT. METHODS: We have previously shown excellent outcomes in CLKT using delayed kidney transplant (Indiana) Approach (mean kidney cold ischemia time = 53 ± 14 h). Between 2007 and 2018, 98 CLKTs were performed using the Indiana Approach at Indiana University (IU) and the data were retrospectively analyzed. Recipients were subgrouped based on their age: 18-45 (n = 16), 46-59 (n = 34), 60-69 (n = 40), and ≥70 years (n = 8). RESULTS: Overall, more elderly patients received LT at IU (5.2%) when compared nationally (2.6%). The rate of elderly recipients in CLKT at IU was 8.2% (versus 2% Scientific Registry of Transplant Recipient). Recipient and donor characteristics were comparable between all age groups except recipient age and duration of dialysis. Patient survival at 1 and 3 years was similar among younger age groups, whereas patient survival was significantly lower in elderly recipients at 1 (60%) and 3 years (40%) (P = 0.0077). Control analyses (replicating Scientific Registry of Transplant Recipient's survival stratification: 18-45, 46-64, ≥65 y) showed similar patient survival in all age groups. CONCLUSIONS: Although LT can be safely performed in elderly recipients, extreme caution is needed in CLKT due to the magnitude of operation.

Pre-Liver Transplant Cardiac Catheterization Is Associated With Low Rate of Myocardial Infarction and Cardiac Mortality.

BACKGROUND AND AIMS: A study at Indiana University demonstrated a reduction in myocardial infarction (MI) incidence with increased frequency of cardiac catheterization (CATH) in liver transplant (LT) candidates. A strict protocol for performing CATH based upon predefined risk factors, rather than noninvasive testing alone, was applied to a subgroup (2009-2010) from that study. CATH was followed by percutaneous coronary intervention (PCI) in cases of significant coronary artery disease (CAD; ≥50% stenosis). The current study applies this screening protocol to a larger cohort (2010-2016) to assess post-LT clinical outcomes. APPROACH AND RESULTS: Among 811 LT patients, 766 underwent stress testing (94%) and 559 underwent CATH (69%), of whom 10% had CAD requiring PCI. The sensitivity of stress echocardiography in detecting significant CAD was 37%. Predictors of PCI included increasing age, male gender, and personal history of CAD (P < 0.05 for all). Compared to patients who had no CATH, patients who underwent CATH had higher mortality (P = 0.07), and the hazard rates (HR) for mortality increased with CAD severity (normal CATH, HR, 1.35; 95% confidence interval [CI], 0.79-2.33; P = 0.298; nonobstructive CAD, HR, 1.53; 95% CI, 0.84-2.77; P = 0.161; and significant CAD, HR, 1.96; 95% CI, 0.93-4.15; P = 0.080). Post-LT outcomes were compared to the 2009-2010 subgroup from the previous study and showed similar 1-year overall mortality (8% and 6%, P = 0.48), 1-year MI incidence (<1% and <1%, P = 0.8), and MI deaths as a portion of all deaths (3% and 9%, P = 0.35). CONCLUSIONS: Stress echocardiography alone is not reliable in screening LT patients for CAD. Aggressive CAD screening with CATH is associated with low rate of MI and cardiac mortality and validates the previously published protocol when extrapolated over a larger sample and longer follow-up period.

Postoperative Atrial Fibrillation and Flutter in Liver Transplantation: An Important Predictor of Early and Late Morbidity and Mortality.

Postoperative atrial fibrillation/flutter (POAF) is the most common perioperative arrhythmia and may be particularly problematic after liver transplantation (LT). This study is a single-center retrospective analysis of POAF to determine its incidence following LT, to identify risk factors, to assess its impact on clinical outcomes, and to summarize management strategies. The records of all patients who underwent LT between 2010 and 2018 were reviewed. Extracted data included pre-LT demographics and cardiac evaluation, in-hospital post-LT cardiac events, early and late complications, and survival. Among 1011 patients, the incidence of post-LT POAF was 10%. Using binary logistic regression, pre-LT history of atrial fibrillation was the strongest predictor of POAF (odds ratio [OR], 6.72; 95% confidence interval [CI], 2.00-22.57; P < 0.001), followed by history of coronary artery disease (CAD; OR, 2.52; 95% CI, 1.10-5.81; P = 0.03). Cardiac stress testing abnormality and CAD on cardiac catheterization were also associated with higher risk. Median time to POAF onset after LT was 3 days with 72% of cases resolving within 48 hours. POAF patients had greater hospital length of stay, death during the LT admission, and 90-day and 1-year mortality. POAF was an independent risk factor for post-LT mortality (OR, 2.0; 95% CI, 1.3-3.0; P < 0.01). Amiodarone was administered to 73% of POAF patients with no evidence of increased serum alanine aminotransferase levels. POAF occurred in 10% of post-LT patients with early onset and rapid resolution in most affected patients. POAF patients, however, had significant morbidity and mortality, suggesting that POAF is an important marker for worse early and late post-LT outcomes.

ESTIMATION OF THE ANISOTROPY EMISSION OF LPN/CIEMAT NEUTRON SOURCES: EFFECT OF HEAVY CAPSULE HOLDERS.

In order to estimate the anisotropy emission of 241Am-Be and 252Cf neutron sources from the Spanish Neutron Standards Laboratory (LPN/CIEMAT) detailed models of sources capsules and capsule holders were designed with the MCNPX code. Simulations of the sources inside the capsules without the capsule holders were done to validate the MC model by comparison with experimental results provided by other authors. After that, the capsule holders were incorporated to the simulation. In general, a good agreement has been found between measurements and our calculations. Results show the additional encapsulations have significant influence on anisotropy factors, energy spectra and dose rates.

Expanding the Donor Pool With the Use of Extended Criteria Donation After Circulatory Death Livers.

Use of donation after circulatory death (DCD) donor livers for transplantation has remained cautious in the United States. The aim of this study was to demonstrate the expansion of a DCD liver transplantation (LT) program with the use of extended criteria donor (ECD) DCD livers. After institutional review board approval, 135 consecutive DCD LTs were retrospectively studied. ECD DCD livers were defined as those with 1 of the following factors: donor age >50 years, donor body mass index >35 kg/m2 , donor functional warm ischemia time >30 minutes, and donor liver macrosteatosis >30%. An optimization protocol was introduced in July 2011 to improve outcomes of DCD LT, which included thrombolytic donor flush and efforts to minimize ischemia times. The impact of this protocol on outcomes was evaluated in terms of graft loss, ischemic cholangiopathy (IC), and change in DCD LT volume. Of 135 consecutive DCD LTs, 62 were ECD DCDs. In total, 24 ECD DCD LTs were performed before (era 1) and 38 after the institution of optimization protocol (era 2), accounting for an increase in the use of ECD DCD livers from 39% to 52%. Overall outcomes of ECD DCD LT improved in era 2, with a significantly lower incidence of IC (5% versus 17% in era 1; P = 0.03) and better 1-year graft survival (93% versus 75% in era 1; P = 0.07). Survival outcomes for ECD DCD LT in era 2 were comparable to matched deceased donor LT. With the expansion of the DCD donor pool, the number of DCD LTs performed at our center gradually increased in era 2 to account for >20% of the center's LT volume. In conclusion, with the optimization of perioperative conditions, ECD DCD livers can be successfully transplanted to expand the donor pool for LT.

Ischemic Cholangiopathy 11 Years after Liver Transplantation from Asymptomatic Chronic Hepatic Artery Thrombosis.

Hepatic artery thrombosis is a concerning complication of orthotopic liver transplantation, and it most often occurs early in the posttransplant period. However, on rare occasions it can occur at a time remote from transplant. We present a case of ischemic cholangiopathy complicated by stricture and anastomotic bile leak from chronic hepatic artery thrombosis that occurred 11 years after the transplant. The initial biliary stenting helped with the resolution of the leak but she was found to have stones, sludge and copious pus at the time of stent exchange. Hepatic arteriography demonstrated complete occlusion of the transplant hepatic artery with periportal collaterals reconstituting intrahepatic hepatic arterial branches. The patient was subsequently referred for repeat liver transplantation.

Reliability of eye lens dosimetry in workers of a positron emission tomography radiopharmaceutical production facility.

A new regulatory statement was issued concerning the eye lens radiation protection of persons in planned exposures. A debate was raised on the adequacy of the dosimetric quantity and on its method of measurement. The aim of this work was to establish the individual monitoring procedure with the EYE-D™ holder and a MCP-N LiF:Mg,Cu,P thermoluminescent chip detector for measuring the personal dose equivalent Hp(3) in workers of a Positron Emission Tomography Radiopharmaceutical Production Facility.