5-year Patient Research Articles

Clinical outcomes of pediatric kidney replacement therapy after childhood cancer – ESPN/ERA Registry study

Cancer and its treatment may lead to kidney injury and need for kidney replacement therapy (KRT). We identified 287 pediatric KRT patients with a malignancy history from the ESPN/ERA Registry. Of these, 197 had cancer as a primary cause of KRT (group 1) and 90 had a malignancy diagnosis before KRT (group 2). Two matched controls without malignancy were randomly selected for each patient. Data were complemented with a questionnaire. Median time to kidney transplantation (KT) from KRT initiation was 2.4 (IQR: 1.5–4.7), 1.5 (IQR: 0.4–3.3), 3.6 (IQR: 1.3–Q3 not reached), and 1.1 (IQR: 0.3–3.6) years for group 1, their controls, group 2 and their controls, respectively. Overall 10-year mortality on KRT was higher among cancer patients vs. controls in group 1: 16% vs. 9% (aHR 2.02, 95% CI: 1.21–3.37) and in group 2: 23% vs. 14% (aHR 2.32, 95% CI: 1.11–4.85). In contrast, 10-year patient survival after first KT was comparable to controls (93% vs. 96%; 100% vs. 94%, in groups 1 and 2, respectively). In summary, childhood cancer survivors’ KT was delayed, their overall mortality on KRT was increased, but once transplanted, their long-term outcome was similar to other KT recipients.

The Disaggregated Oswestry Disability Index: What is the Most Predictive Subsection for Patient Satisfaction After Lumbar Surgery?

Retrospective review of cohort studies. To analyze how different ODI subsections and their improvement affect patient satisfaction 2 years after elective lumbar surgery for degenerative lumbar spondylolisthesis (DLS). The Oswestry Disability Index (ODI) is crucial in evaluating outcomes of spinal disorders and provides valuable insights into a patient's preoperative status. There is limited information available on the individual characteristics of its subsections and their relation to postoperative patient satisfaction. Pre- and 2-year postoperative ODI and patient satisfaction were assessed. The analysis included preoperative scores and improvements in each ODI subsection 2 years postoperatively. Satisfaction was rated on a scale of 1 to 5, with scores ≥4 deemed satisfactory. Univariate linear regression and ROC analysis established cutoffs for subsection improvement and postoperative target values to achieve postoperative satisfaction. 265 patients (60% female, 67±8 years) were included. ODI improvement was achieved in 91%, and postoperative patient satisfaction in 73%. Patients with lower postoperative subsection scores and greater differences between pre- and postoperative scores were more likely to be satisfied (all P<0.001). A postoperative subsection target score of ≤1 was associated with patient satisfaction. Change in degree of pain was the most predictive subsection for satisfaction, with an AUC of 0.84 (sensitivity 79%, specificity 86%). Walking (AUC 0.83, sensitivity 87%, specificity 65%), pain intensity (AUC 0.82, sensitivity 79%, specificity 79%), personal care (AUC 0.82, sensitivity 83%, specificity 68%), and standing (AUC 0.82, sensitivity 83%, specificity 83%) all had an AUC greater than 0.80. The postoperative subsection with the lowest predictability was sleeping (AUC 0.69). Pain domains, walking, standing, and personal care were the subsections with the highest predictability for patient satisfaction. These findings on the correlation between different ODI subscales and patient satisfaction are valuable for improving preoperative education, addressing disability, and ensuring postoperative satisfaction.

Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: Multicenter retrospective study in Japan.

To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children. We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020. A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.3%) had isolated congenital hepatic fibrosis and 16 (45.6%) had Caroli syndrome. Refractory gastrointestinal bleeding was the most frequent symptom related to the indication for LDLT, being found in 48.6% of our patients, followed by uncontrollable cholangitis and ascites. The median age at the time of LDLT was 8.1years old. Of the 27 patients presenting with renal involvement, 13 patients required kidney transplantation (KT). Overall, the renal function after LDLT decreased regardless of renal involvement; however, patients with renal involvement had a significantly lower estimated glomerular filtration rate than those without renal involvement throughout the course of this study (p<0.01). The 5-year overall patient survival rate was 97.1%. Two patients died with a median follow-up of 8.9years after LDLT; one died due to sepsis 2weeks after simultaneous liver-kidney transplantation and the other committed suicide 10years after LDLT. The prognosis of the pediatric patients who underwent LDLT for FLD was excellent. However, an individualized treatment approach based on the status of the renal function and liver disease is important, as a certain proportion of patients require KT.

Pediatric Kidney Transplantation in an Under-resourced Country: A Single-Center Experience.

Kidney transplant is the optimal method for managing children with end-stage kidney disease. This study aimed to present the experience and results of the pediatric kidney transplant program at our center. A single-center observational study was conducted at Children's University Hospital in Damascus, Syria. We reviewed the medical records of all kidney transplants performed between April 2018 and December 2022. All patients were under 14 years old when they underwent kidney transplant. All donors were living, as an effective deceased donor program is currently not an option in Syria. From April 2018 to December 2022, 33 pediatric kidney transplants were performed at our center. Most recipients were boys (n = 24; 72.7%), and 9 were girls (27.3%). Median age was 10 years (range, 4.5-14 y). The most frequent underlying diseases for kidney failure were renal dysplasia-hypoplasia (42.5%), reflux nephropathy (18.2%), and neurogenic bladder with posterior urethral valves (12%). In 10 patients, kidney transplant was performed preemptively (30.3%). Initial graft function was observed in all grafts (100%). During observation time (5 years), 28 patients (85%) retained adequate graft function. The 1-year graft and patient survival rates were 91%. Syria is still suffering from the consequences of the war, affecting organ transplantation in many ways. The good 1-year graft and patient survival in our center was encouraging. However, we admit that the number of patients was small and the follow-up duration was also not long enough; future research is needed to improve long-term outcomes.

Reexamining Transplant Outcomes in Acute Kidney Injury Kidneys Through Machine Learning.

Despite many people awaiting kidney transplant, kidney allografts from acute kidney injury (AKI) donors continue to be underutilized. We aimed to cluster kidney transplant recipients of AKI kidney allografts using an unsupervised machine learning (ML) approach. Using Organ Procurement and Transplantation Network-United Network for Organ Sharing (OPTN/UNOS) data, a consensus clustering cohort analysis was performed in 12356 deceased donor kidney transplant recipients between 2015 and 2019 in whom donors had a terminal serum creatinine ≥1.5mg/dL. Significant cluster characteristics were determined, and outcomes were compared. The median donor terminal creatinine was 2.2 (interquartile range [IQR] 1.7-3.3) mg/dL. Cluster analysis was performed on 12356 AKI kidney recipients, and three clinically distinct clusters were identified. Young, sensitized kidney re-transplant patients characterized Cluster 1. Cluster 2 was characterized by first-time kidney transplant patients with hypertensive and diabetic kidney diseases. Older diabetic recipients characterized Cluster 3. Clusters 1 and 2 donors were young and met standard kidney donor profile index (KDPI) criteria; Cluster 3 donors were older, more likely to have hypertension or diabetes, and meet high KDPI criteria. Cluster 1 had a higher risk of acute rejection, 3-year patient death, and graft failure. Cluster 3 had a higher risk of death-censored graft failure, patient death, and graft failure at 1 and 3 years. Cluster 2 had the best patient-, graft-, and death-censored graft survival at 1 and 3 years. Compared to non-AKI kidney recipients, the AKI clusters showed a higher incidence of delayed graft function (DGF, AKI: 43.2%, 41.7%, 45.3% vs. non-AKI: 25.5%); however, there were comparable long-term outcomes specific to death-censored graft survival (AKI: 93.6%, 93.4%, 90.4% vs. non-AKI: 92.3%), patient survival (AKI: 89.1%, 93.2%, 84.2% vs. non-AKI: 91.2%), and overall graft survival (AKI: 84.7%, 88.2%, 79.0% vs. non-AKI: 86.0%). In this unsupervised ML approach study, AKI recipient clusters demonstrated differing, but good clinical outcomes, suggesting opportunities for transplant centers to incrementally increase kidney utilization from AKI donors.

Acceptable outcomes of liver transplantation in uninsured patients under the coverage of a state assistance program.

The lack of health insurance is a major barrier in the access to healthcare even in case of life-saving procedures such as liver transplantation (LT). Concerns about worse outcomes in uninsured patients have also discouraged the evaluation and transplantation of patients without adequate health insurance coverage. The aim of this study is to evaluate outcomes from the largest cohort of uninsured patients who underwent LT with the support of a state payment assistance program (also called charity care). This study included all consecutive patients who underwent LT at a single center from 2002 to 2020. Demographic, clinical, social variables and outcome metrics were collected and compared between insured and uninsured patients. Among a total of 978 LT recipients, 594 had private insurance, 324 government insurance (Medicare/Medicaid), 60 were uninsured and covered under a state charity care program. In the charity care group, there was a higher proportion of Hispanic subjects, single marital status, younger age, and high-MELD score patients. The 1- and 3-year patient survival rates were 89.0% and 81.8% in private insurance patients, 88.8% and 80.1% in government insurance recipients, and 93.3% and 79.6% in those with charity care (p=0.49). There was no difference in graft survival between insured and uninsured patients (p=0.62). The three insurance groups presented similar hospital length-of-stay and 30-day readmission rates. In both univariate and multivariate analysis, uninsured status (charity care) was not associated with worse patient survival (HR:1.23, 95%CI: 0.84-1.80, p=0.29) or graft survival (HR:1.22, 95%CI:0.84-1.78, p=0.29). In conclusion, there was no difference in outcomes after LT between insured and uninsured patients. A charity care program may be an effective tool to mitigate socio-economic disparities in both outcomes and access to LT.

Failure to Rescue Pediatric Recipients of Living Donor Liver Transplantation: A Single-Center Study of Technical Complications in 500 Primary Grafts.

The concept of failure to rescue (FTR) has been used to evaluate the quality of care in several surgical specialties but has not been well-studied after living donor liver transplantation (LDLT) in children. This study retrospectively reviewed 500 pediatric LDLT performed at a single center between 1993 and 2022. The recipient outcomes were assessed by means of patient and graft survival rates, retransplantation rates, and arterial/portal/biliary complication rates. Graft and patient losses secondary to these complications were calculated regarding FTR for patients (FTRp) and grafts (FTRg). Overall 1- and 5-year patient survival rates were 94.5% and 92.1%, respectively, the corresponding figures for graft survival being 92.7% and 89.8%. One-year hepatic artery complication rate was 3.6% (n = 18 cases), the respective rates for portal vein complications and biliary complications being 5.7% (n = 57) and 15.6% (n = 101). One-year FTRp rates for hepatic artery thrombosis, portal vein thrombosis, anastomotic biliary stricture, and intrahepatic biliary stricture were 28.6%, 9.4%, 3.6%, and 0%, respectively. The corresponding FTRg rates being 21.4%, 6.3%, 0%, and 36.4%. Such novel analytical method may offer valuable insights for optimizing quality of care in pediatric LDLT.

Kidney Transplant Outcome Following Donation After Euthanasia.

In the Netherlands, organ donation after euthanasia (donation after circulatory death type V [DCD-V]) has been increasingly performed since 2012. However, the outcomes of DCD-V kidney grafts have not been thoroughly investigated. It is critical to assess the outcomes of these kidney grafts to ascertain whether DCD-V is a safe and valuable way to increase the kidney donor pool. To investigate the outcomes of DCD-V kidney transplantation and compare them with outcomes of kidney transplantation after circulatory death after withdrawal of life-sustaining therapies (DCD type III [DCD-III]) and donation after brain death (DBD). A retrospective cohort study was conducted using the database from the Dutch Transplant Foundation. All kidney transplants in the Netherlands between January 2012 (start of the euthanasia program) and July 2023 were included. Follow-up was obtained through 5 years after transplantation. Data analysis was performed from November 2023 until February 2024. Kidney transplantation with a DCD-V graft compared with DCD-III and DBD grafts. The primary outcome was death-censored graft survival until 5 years after transplantation. Secondary outcomes were the incidence of delayed graft function (DGF), permanent nonfunction (PNF), serum creatinine concentration, and patient survival until 5 years after kidney transplantation. A total of 145 DCD-V kidney transplants were compared with 1936 DCD-III and 1255 DBD kidney transplants. Median (IQR) recipient age was 59 (46-66) years in the DCD-V cohort, compared with 61 (50-68) years in the DCD-III cohort and 61 (50-68) years in the DBD cohort. The incidence of DGF with DCD-V kidney transplants (26%) was significantly less than that with DCD-III kidney transplants (49%; P < .001) and similar to that with DBD kidney transplants (22%; P = .46). PNF occurrence with DCD-V kidneys (6%) was similar to that with DCD-III kidneys (6%; P = .79) and higher than in DBD kidneys (4%; P < .001). There was no difference in 5-year death-censored graft survival between DCD-V grafts (82%) and DCD-III (86%; P = .99) or DBD (84%; P = .99) grafts. There was no difference in 5-year patient survival between DCD-V kidney transplants (69%) and DCD-III (76%; P = .45) or DBD (73%; P = .74) kidney transplants. A propensity score analysis was performed to match the DCD-V and DCD-III cohort, showing results similar to those of the unmatched cohort. This study found that DCD-V kidney transplantation yielded a lower incidence of DGF compared with DCD-III kidney transplantation and yielded long-term results similar to those of DCD-III and DBD kidney transplantation. The findings suggest that DCD-V is a safe and valuable way to increase the kidney donor pool.

Comprehensive Codified Algorithms to Identify the Underestimated Burden of Hidradenitis Suppurativa in the United States.

Hidradenitis suppurativa (HS) is a painful, inflammatory skin disease associated with a high disease burden and long diagnostic delay. Prevalence estimates of HS vary widely in the literature owing to differing estimation methodologies. This study aimed to apply stepwise algorithms to estimate the prevalence of possible/diagnosed cases of HS in the US. This was a retrospective cohort study in adult and pediatric patients with HS which utilized data from four US databases (MarketScan [Medicare and Medicaid] andOptum [electronichealth record (EHR) andClinformatics Data Mart (CDM)]). Patients withpossible/diagnosed HS were identified using two algorithms (termed Algorithm 1 and Algorithm 2), which assessed symptoms such as multiple skin boils in site-specific areas based on international classification of disease (ICD) codes. Patients with diagnosed HS were defined as having ≥ 2 outpatient or ≥ 1 inpatient diagnosis codes of HS. In each database, patients with continuous medical and pharmacy benefits in the 365days pre-index and 0-365days post-index periods were eligible for inclusion. Across all databases, Algorithm 2 (MarketScan Medicare [N = 309,916]; MarketScan Medicaid [N = 188,783]; Optum EHR [N = 366,158]; Optum CDM [N = 173,812]) identified more patients with possible/diagnosed HS than Algorithm 1 (MarketScan Medicare [N = 194,353]; MarketScan Medicaid [N = 99,276]; Optum EHR [N = 177,957]; Optum CDM [N = 112,244]). Based on ICD-9/10 codes, the 5-year period prevalence of HS ranged from 0.06% to 0.12% across all databases, while for Algorithm 1 and Algorithm 2, this ranged from 0.27% to 0.41% and 0.49% to 0.78%, respectively. Adults and females generally had a higher 5-year period prevalence versus pediatric patients and males, respectively. This real-world study highlights that HS diagnosis codes alone may be insufficient to estimate the prevalence of HS, demonstrating the value of employing algorithms in practice which assess for parameters such as multiple skin boils in site-specific areas. Integrating robust methods to identify the prevalence of HS may improve the diagnostic delay observed in HS and improve treatment outcomes.

Impact of Medicaid expansion on kidney transplantation in the State Oklahoma.

There is no data evaluating the impact of Medicaid expansion on kidney transplants (KT) in Oklahoma. To investigate the impact of Medicaid expansion on KT patients in Oklahoma. The UNOS database was utilized to evaluate data pertaining to adult KT recipients in Oklahoma in the pre-and post-Medicaid eras. Bivariate analysis, Kaplan Meier analysis was used to estimate, and cox proportional models were utilized. There were 2758 pre- and 141 recipients in the post-Medicaid expansion era. Post-expansion patients were more often non-United States citizens (2.3% vs 5.7%), American Indian, Alaskan, or Pacific Islander (7.8% vs 9.2%), Hispanic (7.4% vs 12.8%), or Asian (2.5% vs 8.5%) (P < 0.0001). Waitlist time was shorter in the post-expansion era (410 vs 253 d) (P = 0.0011). Living donor rates, pre-emptive transplants, re-do transplants, delayed graft function rates, kidney donor profile index values, panel reactive antibodies levels, and insurance types were similar. Patients with public insurance were more frail. Despite increased early (< 6 months) rejection rates, 1-year patient and graft survival were similar. In Cox proportional hazards model, male sex, American Indian, Alaskan or Pacific Islander race, public insurance, and frailty category were independent risk factors for death at 1 year. Medicaid expansion was not associated with graft failure or patient survival (adjusted hazard ratio: 1.07; 95%CI: 0.26-4.41). Medicaid expansion in Oklahoma is associated with increased KT access for non-White/non-Black and non-United States citizen patients with shorter wait times. 1-year graft and patient survival rates were similar before and after expansion. Medicaid expansion itself was not independently associated with graft or patient survival outcomes. Ongoing research is necessary to determine the long-term effects of Medicaid expansion.

Patient Factors Associated with 10-Year Survival After Arthroplasty for Hip Fracture: A Population-Based Study in Ontario, Canada.

The aim of this study was to describe long-term (10-year) patient survival after arthroplasty for hip fracture and to determine what patient factors are associated with that outcome. We performed a retrospective cohort analysis of patients ≥60 years old who underwent either hemiarthroplasty or total hip arthroplasty for femoral neck fracture between 2002 and 2009. We used routinely collected, validated health-care databases linked through ICES (formerly known as the Institute for Clinical Evaluative Sciences). We estimated the association between baseline variables and survival 10 years post-fracture using Poisson regression. Restricted cubic spline functions modeled the probability of 10-year survival by age and tested whether there was an inflection point after which the probability of 10-year survival decreased more rapidly. We estimated 10-year survival probabilities for different patient groups. There were 19,659 patients in the final cohort. Eighteen percent (3,564) of the patients were alive at 10 years postoperatively. Factors associated with a higher likelihood of 10-year survival included younger age, female sex (risk ratio [RR] = 1.56, 95% confidence interval [CI] = 1.46 to 1.68), lower American Society of Anesthesiologists (ASA) class (ASA I or II versus IV or V: RR = 1.96, 95% CI = 1.76 to 2.19), independent living status (RR = 2.68, 95% CI = 2.23 to 3.22), and fewer specific comorbidities. A threshold age of 73 years was the inflection point after which the probability of 10-year survival decreased more rapidly in females. Estimated 10-year survival probabilities ranged from 79.0% (95% CI = 75.5% to 82.5%) to 0.8% (95% CI = 0.6% to 1.0%). Approximately 1 in 6 patients live at least 10 years following a hip fracture. This study identifies baseline characteristics that predict survival greater than 10 years, including an age of <75 years, an ASA class of I or II, and independent living status prior to the hip fracture. Results can inform discussions around treatment choices, anticipated outcomes, and the natural history of hip fractures. Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

TAM RECEPTORS IN THE PATHOLOGY OF BLADDER CANCER: APPROACHES TO PERSONALISED MEDICINE

Bladder cancer (BC) is a cancer of the lining epithelium of the bladder. In 75% of cases, it is presented as a non-muscle invasive disease (NMIBC), with a 5-year patient survival of more than 90%. However, 25% of BC patients develop an aggressive and chemoresistant muscle-invasive form of cancer (MIBC) with less than 50% 5-year survival, making BC the 13th deadliest cancer. Moreover, the treatment for MIBC, including cystectomy and bladder reconstruction, is costly and available only in developed countries, leaving patients in other countries without efficient treatment. So, there is an urgent need to develop and implement new personalised treatment schemes in Western and low – to middle-income countries with progressively ageing populations. In our work, we study the regulatory role of the TAM Receptor Tyrosine kinases (AXL, MERTK, and TYRO3) in signalling in BC. It has been shown before that TAM receptors are activated in different cancer types, where they regulate drug resistance, immune evasion and metastasis. We analyse how TAM signalling contributes to the development of chemoresistance in BC and propose that interfering with these pathways may be a part of new combination therapy approaches. We analyse TAM receptor expression and activation in primary patient materials (solid and liquid biopsies) and develop organoid cultures for testing anti-TAM inhibitors and chemotherapeutic agents. According to our recent data, soluble extracellular domains of TAMs are present in urine samples of BC patients and are suitable as urine predictive biomarkers to use in BC.

Hydroxychloroquine as an add-on therapy for the induction therapy of MPO-AAV: a retrospective observational cohort study.

The remission rate of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients who received standard induction therapy is far from satisfactory. Improving the remission rate of MPO-AAV patients is essential. Hydroxychloroquine (HCQ), one of the classic antimalarial drugs, has been widely used in various autoimmune rheumatic diseases. This retrospective observational cohort study is aimed to evaluate the efficacy and safety of HCQ during induction treatment for MPO-AAV. The medical records of patients diagnosed with MPO-AAV at Xiangya Hospital, Central South University from January 2021 to September 2023 were collected. They were assigned to the HCQ group or control group according to whether they used HCQ. The patients included were screened by propensity score matching. To evaluate whether MPO-AAV patients benefited from HCQ, we compared the prognosis of the two groups. The adverse effects of HCQ during follow-up were recorded. The composition ratio of complete remission, response and treatment resistance between HCQ group and control group were different statistically (P=.021). There was no significant difference between the two groups in 1-year renal survival (P=.789). The HCQ group had better 1-year patient survival than the control group (P=.049). No serious adverse effects were documented in the HCQ group. HCQ together with standard induction treatment may improve the remission rate of MPO-AAV patients, and HCQ had good safety in our study.

Transition in Quality Of Life Among Older Patients With Bladder Cancer and Its Association With Treatment: A Clustering Approach

Purpose: To classify older patients with bladder cancer (BC) based on quality-of-life (QoL) measures and explore the clusters transitions over a 2-year period and patient characteristics, BC stages, and treatment. Patients with BC report a significant decline in functional status and QoL. The association of this transition and patient characteristics and treatment over time is not known. We aim to explore this association. Methods: Data were obtained from patients with BC in the SEER-MHOS database (1998-2021). Unsupervised cluster analysis was employed to identify homogeneous clusters of patients with similar functionality based on age, physical and mental component score, and ability to perform activities of daily living. Univariate and multivariable analyses were performed to assess the association of patient characteristics and treatment on cluster transitions over a 2-year interval. Results: The final cohort consisted of 2934 (age 65 or older) older patients with BC. We identified 3 distinct clusters: 67.9% in Cluster 1; 25.4% in Cluster 2; and 6.7% in Cluster 3. After 2 years, 11.7% patients showed improvement and 14% experienced a decline. Cancer/treatment characteristics were not associated with cluster transition. Younger age and being married were identified as factors associated with improvement. Older age, non–homeownership, and last-year fall were associated with transition to less healthy cluster. Conclusions: Over a 2-year interval after BC diagnosis, a quarter of patients will have substantial changes in QoL. No significant association was observed between cluster transition and BC characteristics. Age as well as marital, fall, and homeowner status do impact this transition.

Adult outcomes of childhood kidney replacement therapy in Europe from 2008 to 2019: an ERA Registry study.

Young adults starting kidney replacement therapy (KRT) during childhood and reaching their 18th birthday (i.e. adult survivors of childhood KRT) form a challenging population of interest to nephrologists treating adults, as during this period there will be a transition to adult renal centres. Nonetheless, few studies have focused on the epidemiology of KRT in this group. We aimed to provide an update on these patients' characteristics, treatment history, graft and patient survival, to report their 5-year prognosis, and expected remaining lifetime. Data on KRT patients reaching their 18th birthday in 2008-2019 were collected from 21 European countries/regions providing individual patient data to the European Renal Association (ERA) Registry. Patient characteristics and treatment trajectories were examined before and after turning 18 years. Kaplan-Meier and Cox proportional hazards regression were used for patient and graft survival analyses. In total, 2944 patients were included. The proportion of adult survivors initiating KRT at a very young age (0-4 years), and undergoing pre-emptive kidney transplantation increased. Unadjusted 5-year patient survival was 96.9% (95% CI: 96.2-97.5). Dialysis patients had a higher risk of death than kidney transplant recipients (adjusted hazard ratio 5.44 (95% CI: 3.34-8.86)). Between ages 18 and 23 years, about 21% of the adult survivors lost their kidney transplant and 34% of the dialysis patients continued this treatment. Compared with the general population, life expectancy for eighteen-year-old kidney transplant and dialysis patients was 17 and 40 years shorter, respectively. Life expectancy of 18-year-old kidney transplant recipients was lower compared with the general population. Yet, having a functioning kidney graft at age 18 years resulted in better outcomes than being on dialysis. Nevertheless, between ages 18 and 23 years, about one-fifth of the kidney grafts failed and one-third of the patients remained on dialysis.

Factors Affecting the Effectiveness of DIBH (Deep Inspiratory Breath Hold) in Patients with Left Breast Cancer: A Narrative Review

Deep Inspiratory Breath Hold (DIBH) has become a valuable technique in left-breast cancer radiotherapy, offering the possibility to reduce radiation exposure to organs at risks (OARs) and minimize the risk of cardiac complications. This treatment method involves stopping the breathing of patients during irradiation in order to temporarily distance the heart from the radiation field, which reduces potential cardiac risks and other complications. To identify factors that may affect the effectiveness of DIBH treatment, we analyzed the most important 5-year studies published in the PubMed database. Research shows that DIBH reduces the radiation dose to the heart and lungs. However, the effectiveness of DIBH is determined by a variety of factors, including the patient’s training, cooperation, anatomical features, age, and choice of radiotherapy technique. Additionally, cardiovascular risk factors, such as diabetes, smoking, and hypertension, can be impactful to the effectiveness and potential complications of DIBH. Moreover, if a patient has a substantial level of depression or anxiety, then they may be potentially disqualified from the DIBH treatment method. In addition to this, a lack of consent and/or fear may also disqualify a patient from DIBH treatment. Careful patient selection, comprehensive training, and optimization of treatment parameters are essential to maximize the benefits of DIBH whilst minimizing any potential side effects. DIBH enhancement techniques, such as IMRT and VMAT, also have an important role to play. The purpose of this narrative review article is to summarize the factors affecting the efficacy and side effects of DIBH in radiation therapy for left-breast cancer, with the aim of optimizing its clinical application while minimizing side effects. Patients who are likely to benefit most from DIBH are young women in good medical condition, able to cooperate with the procedure, and with smaller breasts. The increase in the estimated 10-year patient survival is significantly influenced by cardiovascular problems, so patients without diabetes and metabolic syndrome, and non-smokers, will benefit the most. An estimated 50–70% of breast cancer patients are likely to benefit from DIBH, and in the best case, it can result in a 50% reduction in the risk of cardiac problems after photodynamic therapy (PDT).

Insights from a 10-year Australasian idiopathic hypersomnia patient data registry study.

Idiopathic hypersomnia (IH) is a disorder of central hypersomnolence that results in excessive daytime sleepiness in the absence of another identifying cause. Case studies from sleep clinic patients may not be a fair representation of the wider IH population. This study aims to better characterize patients diagnosed with IH in Australia and New Zealand using online patient-driven survey data. A retrospective analysis of 686 participants from the Hypersomnolence Australia Patient Data Registry diagnosed with either IH (n = 554), narcolepsy type 1 (NT1, n = 54) or narcolepsy type 2 (NT2, n = 78) between January 2013 and October 2022 was performed. Participants with IH reported additional sleep disorders such as OSA (16.4%) and restless legs syndrome (7.9%) and notable comorbidities included depression (46.2%) and anxiety (50%). There was a mean delay in diagnosis of 10 years in participants with IH, when compared to symptom onset. IH presents with unique but also overlapping symptomatology with NT2, with similar reporting of long daytime naps, unrefreshed sleep and automatic behavior. Modafinil was the most common medication (45.5%) used by participants with IH followed by dexamphetamine (44.2%). Most participants with IH reported receiving physician advice regarding positive lifestyle changes but recommend that newly diagnosed patients be given more advice about medication use. This study demonstrates a delay in IH diagnosis when compared to symptom onset and overlapping features of IH and NT2. It also highlights the heterogeneous presentation of IH and the value of large patient registries in future research.

A retrospective study investigating the risk of graft loss in living donor liver transplant cases where size mismatching is predicted from graft-to-recipient weight ratio.

Living donor liver transplantation (LDLT) is vital for pediatric end-stage liver disease due to organ shortages. The graft-to-recipient weight ratio (GRWR) preoperatively measured predicts the outcomes of LDLT. We typically target between 0.8 and 3.0-4.0%, but the ideal GRWR remains controversial. We compared the outcomes of LDLT according to the GRWR to examine whether the criteria could be expanded while ensuring safety. We retrospectively reviewed 99 patients who underwent LDLT in our department by dividing them into three groups according to their GRWR: Group S, with GRWR values lower than the normal range (GRWR < 0.8%); Group M, with GRWR values in the normal range (GRWR ≥ 0.8 to < 3.5%); and Group L, with GRWR values above the normal range (GRWR ≥ 3.5%). In Groups S and L, 46.2 and 44.4% of patients underwent splenectomy and delayed abdominal wall closure, respectively. After these intraoperative adjustments, there were no significant differences between the groups in 5-year patient survival, 5-year graft survival, or the occurrence of post-transplantation thrombosis. When the GRWR is beyond the normal threshold, the risk of complications associated with graft size might be reduced by adjustments to provide appropriate portal blood flow and by delayed abdominal wall closure.

Outcomes of Kidney Transplantation in Highly HLA-Sensitized Patients Treated with Intravenous Immuno-Globulin, Plasmapheresis and Rituximab: A Meta-Analysis.

(1) Background: We aimed to investigate the outcomes of human leukocyte antigen (HLA)-incompatible transplantation for patients who received desensitization with intravenous immunoglobulins (IVIg), plasmapheresis, and rituximab. (2) Methods: A comprehensive search of multiple electronic databases to identify studies that utilized desensitization was conducted. The random-effects model was used to calculate the pooled rates and the 95% confidence interval (CI). (3) Results: A total of 1517 studies were initially identified. From these, 16 studies met the inclusion criteria, encompassing 459 patients, with a mean age of 45 years, of whom 40.8% were male. CDC crossmatch was positive in 68.3% (95% CI: 43.5-85.8; I2 87%), and 89.4% (95% CI: 53.4-98.4%; I2 89.8%) underwent living-donor transplantation. The 1-year graft survival pooled rate was 88.9% (95% CI: 84.8-92; I2 0%) and the 5-year graft survival rate was 86.1% (95% CI: 81.2-89.9; I2 0%). The 1-year patient survival rate was 94.2% (95% CI: 91-96.3; I2 0%), and the 5-year patient survival rate was 88.9% (95% CI: 83.5-92.7%; I2 7.7%). The rate of antibody-mediated rejection was 37.7% (95% CI: 25-52.3; I2 80.3%), and the rate of acute cell-mediated rejection was 15.1% (95% CI: 9.1-24; I2 55%). (4) Conclusions: Graft and patient survival are favorable in highly sensitized patients who undergo desensitization using IVIg, plasmapheresis, and rituximab for HLA-incompatible transplantation.

Long-Term Outcomes of Living Donor Liver Transplantation for Methylmalonic Acidemia.

Despite early diagnosis and medical interventions, patients with methylmalonic acidemia (MMA) suffer from multi-organ damage and recurrent metabolic decompensations. We conducted the largest retrospective multi-center cohort study so far, involving five transplant centers (NCCHD, KUH, KUHP, ATAK, and EMC), and identified all MMA patients (n = 38) undergoing LDLT in the past two decades. Our primary outcome was patient survival, and secondary outcomes included death-censored graft survival and posttransplant complications. The overall 10-year patient survival and death-censored graft survival rates were 92% and 97%, respectively. Patients who underwent LDLT within 2 years of MMA onset showed significantly higher 10-year patient survival compared to those with an interval more than 2 years (100% vs. 81%, p = 0.038), although the death-censored graft survival were not statistically different (100% vs. 93%, p = 0.22). Over the long-term follow-up, 14 patients (37%) experienced intellectual disability, while two patients developed neurological complications, three patients experienced renal dysfunction, and one patient had biliary anastomotic stricture. The MMA level significantly decreased from 2218.5 mmol/L preoperative to 307.5 mmol/L postoperative (p = 0.038). LDLT achieves favorable long-term patient and graft survival outcomes for MMA patients. While not resulting in complete cure, our findings support the consideration of early LDLT within 2 years of disease onset. This approach holds the potential to mitigate recurrent metabolic decompensations, and preserve the long-term renal function.