End-stage Disease Research Articles

Off-label Use of Mycophenolate Mofetil in Immunoglobulin A Nephropathy: A Systematic Review and Meta-analysis.

Mycophenolate mofetil (MMF) is widely used off-label in patients with immunoglobulin A nephropathy (IgAN), although the literature does not consistently agree on its efficacy and safety. We systematically searched PubMed, Embase, CENTRAL, CNKI, VIP, Wanfang Data, and SinoMed from their inception to August 2023. We included randomized controlled trials that enrolled patients of IgAN who received MMF treatment and compared effects with placebo or as an add-on therapy to usual care. Literature screening, risk of bias assessment, and data extraction were independently conducted in duplicate. Fixed-effects or random-effects meta-analyses were performed for pooling data where eligible. The primary outcomes were the composite kidney outcomes of major adverse kidney events (MAKDE) defined as doubling of serum creatinine, end-stage renal disease (ESRD), or death from a kidney disease-related or cardiovascular cause. Of 13 studies identified, 918 participants (463 [50.4%] treated with MMF) with IgAN were included in the analysis. MMF treatment in IgAN was associated with decreasing the occurrence of MAKDE (RR, 0.32; 95%CI, 0.13 to 0.77), reducing proteinuria (RR, 1.41; 95%CI, 1.22 to 1.64), and lessening the probability of doubling blood creatinine (RR, 0.32, 95% CI, 0.14 to 0.72). No significant differences were detected in the incidence of ESRD (RR: 0.87, 95% CI: 0.38 to 2.03), or progression of chronic kidney disease (RR, 1.01; 95%CI, 0.22 to 4.57). Patients receiving MMF had a higher risk of infection (RR, 2.20; 95%CI, 1.21 to 4.00). MMF administration in IgAN indicates promising in decreasing the occurrence of MAKDE, reducing proteinuria level, and lessening the probability of doubling blood creatinine, but also comes with the risk of infection. These findings tend to be introduced to non-Caucasian population. The long-term favorable effects that MMF improved kidney outcomes still needs need further cross-regional and cross-ethnical verification.

Podocyte-Specific Expression of the Stress Response Protein REDD1 is Necessary for Diabetes-induced Podocytopenia.

Diabetic nephropathy (DN) is the leading cause of end-stage renal disease and effective treatment modalities that fully address its molecular etiology are lacking. Prior studies support that the stress response protein REDD1 (Regulated in Development and DNA Damage 1) contributes to the development of diabetic complications. This study investigated a potential role for REDD1 expression in podocytes in diabetes-induced podocyte loss and compromised glomerular filtration. Podocyte-specific REDD1 deletion protected against renal injury, as evidenced by reduced albuminuria, glomerular hypertrophy, and mesangial matrix deposition in streptozotocin (STZ)-induced diabetic mice. Podocyte-specific REDD1 expression was required for diabetes-induced reduction in slit diaphragm (SD) proteins podocin and nephrin. Notably, podocyte-specific REDD1 deletion protected against podocytopenia and preserved glomerular basement membrane and foot process architecture in diabetic mice. In the kidneys of diabetic mice and in human podocyte cultures exposed to hyperglycemic conditions, REDD1 was necessary for increased expression of the transient receptor potential canonical 6 (TRPC6) channel. More specifically, REDD1 promoted NF-κB-dependent transcription of TRPC6, intracellular calcium entry, and cytoskeletal remodeling under hyperglycemic conditions. Overall, the findings provide new insight into the role of podocyte-specific REDD1 expression in renal pathology and support the possibility that therapeutics targeting REDD1 in podocytes could be beneficial for DN.

Reference gene panel for urinary exosome-based molecular diagnostics in patients with kidney disease.

Kidney disease is a severe complication of diabetes that often leads to end-stage renal disease. Early diagnosis is crucial for prevention or delay. However, the current diagnostic methods, with their limitations in detecting the disease in its early stages, underscore the urgency and importance of finding new solutions. miRNAs encapsulated inside urinary exosomes (UEs) have potential as early biomarkers for kidney diseases. The need for reference miRNAs for accurate interpretation currently limits their translational potential. To identify consistently expressing reference miRNAs from UEs of controls and patients with type 2 diabetesmellitus (T2DM) and biopsy-confirmed kidney diseases. miRNA profiling was performed on UEs from 31 human urine samples using a rigorous and unbiased method. The UEs were isolated from urine samples collected from healthy individuals (n = 6), patients with T2DM (n = 13), and T2DM patients who also had kidney diseases (including diabetic nephropathy, n = 5; membranous nephropathy, n = 5; and IgA nephropathy, n = 2) through differential ultracentrifugation. After characterizing the UEs, miRNA expression profiling using microarray technology was conducted. Microarray data analysis identified 14 miRNAs that were consistently expressed in UEs from 31 human samples, representing various kidney conditions: diabetic controls, diabetic nephropathy, membrane nephropathy, IgA nephropathy, and healthy controls. Through in silico analysis, we determined that 10 of these miRNAs had significant potential to serve as reference genes in UEs. We identified uniformly expressing UE miRNAs that could serve as reference genes kidney disease biomarkers.

Polysaccharides from Sacha Inchi shell reduces renal fibrosis in mice by modulating the TGF-β1/Smad pathway and intestinal microbiota

Renal fibrosis is a common pathway involved in the progression of various chronic kidney to end-stage diseases, posing a substantial global public health challenge in the search for effective and safe treatments. This study investigated the effects and mechanisms of sacha inchi shell polysaccharide (SISP) on renal fibrosis induced by a high-salt diet (HSD) in mice. By analysing kidney-related protein pathways and the structure of gut microbiota, we found that SISP significantly reduced urinary protein levels induced by a HSD from 41.08 to 22.95 μg/mL and increased urinary creatinine from 787.43 to 1294.50 μmol/L. It reduced renal interstitial collagen fibres by 11.30 %, thereby improving the kidney function. SISP lowered the mRNA expression of TGF-B1, fibronectin, α-SMA, Smad2/3, and TGFBRII, leading to decreased protein levels of TGF-β1, p-Smad2/3, p-TGFβRII, fibronectin, α-SMA, p-Smad2/3/Smad2/3, and p-TGFβRII/TGFβRII. These changes blocked downstream transcription in the TGF-β1/Smad signalling pathway, thereby attenuating renal fibrosis in HSD mice. In addition, SISP altered the intestinal flora imbalance in HSD mice by reducing the relative abundance of the genera, Akkermansia, Faecalibaculum, and unidentified_Ruminococcaceae, and reversing the decline in the levels of the genera, Lactobacillus and Bacteroides. In conclusion, SISP is a promising nutraceutical for renal fibrosis management.

Comparison of the quality of life in peritoneal dialysis and hemodialysis patients

Chronic kidney disease (CKD) has a profound impact on patients' health-related quality of life (QoL). Particular problems are caused by the end-stage disease, which leads to the need for renal replacement therapy (RRT). Physicians and patients face the challenge of choosing a type of RRT. In this case, it is necessary to take into account not only survival rates but also the quality of life of patients who undergo the therapy, because the ability to function and maintain well-being is one of the treatment goals recommended by the World Health Organization. Therefore, assessing QoL and identifying factors influencing it becomes increasingly important in terms of therapy effectiveness and measures aimed at improving outcomes. The purpose of this work was to review recent scientific studies assessing the quality of life among peritoneal dialysis (PD) and hemodialysis (HD) patients, as well as identifying the better RRT type and risk factors that affect the quality of life. In order to identify the most relevant RRT type, the general questionnaires SF-36 and EQ-5D were used. A search for scientific studies was carried out in the international PubMed/MEDLINE database in April 2024 using the keywords: chronic kidney disease, quality of life, SF-36, EQ-5D, hemodialysis, and peritoneal dialysis. We selected papers published from 2018 to 2024 where the quality of life of patients with CKD receiving such RRT types as hemodialysis and peritoneal dialysis was studied. The results showed that PD is more preferable than HD. To achieve a high quality of life, it is also necessary to carefully treat disorders of calcium and phosphate metabolism and anemia, adequately manage concomitant diseases, assist patients with medical care provided at home, organize educational programs to increase patients’ self-efficacy and self-management, and provide psychological assistance if necessary.

Characteristics of Individuals Admitted to Vascular Surgery Service for a Major Lower Limb Amputation From 2017 to 2021 With Present on Admission Pressure Injuries.

The purpose of this study was to describe characteristics of persons admitted from 2017 to 2021 with peripheral arterial disease (PAD) who required a below knee amputation (BKA) or above knee amputation (AKA), including present on admission pressure injuries (POA-PIs). Descriptive, cross-sectional retrospective research design. The sample comprised 196 patients who underwent a total of 258 major lower limb amputations. A majority (65.9 %n=170) underwent BKAs and 88 (34.1 %) underwent AKAs. Coronary artery disease was present in 107 (54.6 %) and 143 (73.0%) had diabetes mellitus. The study site was a 670-bed level 1 trauma center in the Southeastern United States. A retrospective chart review of patients undergoing major lower limb amputations using CPT codes 27880 BKA, and 27590 AKA was completed. Variables extracted included age, sex, race, and comorbid conditions such as smoking, cardiovascular disease, diabetes mellitus, end stage renal disease requiring dialysis, and POA-PI. Descriptive statistics (frequencies, percentages, and means) were used to describe the sample. There were 25 (12.8%) individuals with POA-PI compared with 171 (87.2%) who did not have POA-PI. None of the reviewed significantly differed when patients with or without POA-PI were compared. Age was closest to statistical significance (P=.052). In addition, analysis revealed that 73% (n=143) of individuals had diabetes mellitus in addition to having PAD. Approximately 13% of a group of patients with PAD had POA-PI when admitted to hospital for major limb amputation. Analysis of multiple factors found that none significantly differed between patients with or without POA-PI. Age came nearest to statistical significance and we hypothesize that older age may increase the risk for POA-PI; additional research in samples with greater power to detect this effect are needed. Findings also indicate that diabetes mellitus is a common risk factor for major lower limb amputation. It is important to educate staff/patients/caregivers on the importance of PI prevention especially in the elderly vascular population who are at risk for undergoing major lower limb amputations.

Corticosteroids and invasive fungal infections in hospitalized COVID-19 patients – A single-center cross-sectional study

Background: During the coronavirus disease 2019 (COVID-19) epidemic, an increase in the incidence of fungal infections was observed. However, the real magnitude of these fungal infections and their risk factors among COVID-19 patients in the Indian population is unknown. Aim: To study the frequency, and spectrum of invasive fungal infections (IFI) among hospitalized COVID-19 patients, and the risk factors associated with invasive fungal infections. Methods: We performed a retrospective, cross-sectional study; including all adult patients, admitted to Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), a tertiary care hospital in Southern India between April 2020 and August 2021, with COVID-19. Based on clinical-radiological features, patients with fungal infections were grouped into three diagnostic categories. Demographics, clinical, and laboratory features of patients with fungal infections were analyzed to identify the risk factors. Results: About 10% (449 out of 4650) of the admitted patients with recent COVID-19, had some form of IFI. Among the patients with IFI, 80% (366 out of 449) were hospitalized for active COVID-19, whereas almost all the patients admitted with post-COVID complications had IFI. Of the 449 patients with IFI, 377 had mold infections and 88 had invasive candidiasis. Mucormycosis was the most common mold infection. Diabetes and diabetic ketoacidosis were strong independent predictors of IFI. We also found an association between end-stage renal disease, central venous catheterization, antibiotic usage, prior stroke, and corticosteroid therapy with IFI. Conclusions: The frequency of fungal infections among hospitalized COVID-19 patients was high. Special precautions in COVID-19 patients with diabetes mellitus, corticosteroid therapy, and prior antibiotic usage may help to reduce invasive fungal infections.

Novel Insights into Diabetic Kidney Disease.

Diabetic kidney disease (DKD) is a major complication of diabetes mellitus (DM), affecting over one-third of type 1 and nearly half of type 2 diabetes patients. As the leading cause of end-stage renal disease (ESRD) globally, DKD develops through a complex interplay of chronic hyperglycemia, oxidative stress, and inflammation. Early detection is crucial, with diagnosis based on persistent albuminuria and reduced estimated glomerular filtration rate (eGFR). Treatment strategies emphasize comprehensive management, including glycemic control, blood pressure regulation, and the use of nephroprotective agents such as angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs), sodium-glucose cotransporter-2 (SGLT2) inhibitors, and glucagon-like peptide-1 (GLP-1) receptor agonists. Ongoing research explores novel therapies targeting molecular pathways and non-coding RNAs. Preventive measures focus on rigorous control of hyperglycemia and hypertension, aiming to mitigate disease progression. Despite therapeutic advances, DKD remains a leading cause of ESRD, highlighting the need for continued research to identify new biomarkers and innovative treatments.

Predictors for COVID-19-Specific and Non-COVID-19-Specific Deaths: A Cohort Study in Taiwan

Predictors for coronavirus disease 2019 (COVID-19)-specific and non-COVID-19-specific deaths have not been extensively studied. This cohort study in Taiwan investigated predictors for COVID-19-specific and non-COVID-19-specific deaths among hospitalized patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. From January to July 2022, 2196 COVID-19 patients at Taipei City Hospital were consecutively recruited in this cohort study. Among the 175 deceased COVID-19 patients, 147 (84.0%) and 28 (16.0%) had COVID-19-specific and non-COVID-19-specific deaths, respectively. After controlling for other covariates, multinomial logistic regressions showed that age ≥ 65 was significantly associated with higher risks for both COVID-19-specific, adjusted odds ratio (AOR) = 6.21; 95% confidence interval (CI) [3.12, 12.35]; and non-COVID-19-specific deaths (AOR = 6.06; 95% CI [1.34, 27.34]). Fully vaccinated individuals (AOR = 0.50; 95% CI [0.33, 0.74]) and Paxlovid recipients (AOR = 0.45; 95% CI [0.20, 0.98]) had lower COVID-19-specific death risks, while comorbid cancer or end-stage renal disease patients faced higher risks of non-COVID-19-specific deaths. Our study findings suggest that vaccination and Paxlovid treatment are crucial for reducing SARS-CoV-2-specific mortalities, while comorbid patients need careful monitoring to reduce non-COVID-19-specific deaths.

Unveiling the silent struggle: navigating the labyrinth of mental health for parents of children with life-threatening illnesses in Pakistan.

Dear Editor, We write this letter to highlight the scarcely recognised complexity associated with the unrecognised mental health needs of parents of children diagnosed with life-threatening or life-limiting illnesses. It has been widely established that parents of children with disabilities are at an increased risk of developing variants of mental diseases when compared to parents of children without such disabilities. Parents tend to alter their lives socially to acclimatize themselves as caregivers for their children, therefore adopting a role that becomes far more labyrinthine as both parent and caregiver. (1) Mental health in general, is a subject that is insufficiently addressed in societal and scholarly contexts, specifically in Pakistan, particularly among parents. Within the scope of parents acting as sole caregivers, stress is a prominent determinant contributing to their mental health. Stress can attenuate parents’ and children’s abilities to navigate these circumstances and diminish their capacity for problem-solving and coping. (2) There are a myriad of factors that contribute to the reported prevalence of mental health-related disorders among parents of children with disabilities. Mental health remains a stigmatised entity. Such disregard impedes the delivery of healthcare towards patients suffering from mental health illnesses. There is a noteworthy hindrance in individuals facing such factors when amalgamatinge into society. When considering parental figures of diseased children, this facet serves to isolate further and reinforce such mental health illnesses. The lack of quantifiable data on anti-stigma interventions in Low and Middle-Income Countries, including Pakistan, underscores the urgent need for research. (3) Parents of children admitted to the ICU have a near-pervasive struggle with mental health and an innate inability to disclose their requirements for support. Factors barricading such disclosure should be identified and broken down through interventions, facilitating access to vital mental health support. (4) Caregivers must establish coping strategies, and remain a steady pillar of support and comfort to their child. The Keeping Hope Alive Toolkit intervention was established, which worked on fostering emotional experiences, living in the moment, remembering self and supporting social preferences, this intervention provided a template for emotional support. (5) We hope this letter can underscore how important supporting parental disclosure of unmet mental health needs is – a rampant and insufficiently reported plight in Pakistan. The ability to recognise these emotions and rectify them is not highlighted in this region but is imperative to implement, to improve the provision of healthcare to diseased children and their parents alike.

An integrated machine learning model enhances delayed graft function prediction in pediatric renal transplantation from deceased donors

BackgroundKidney transplantation is the optimal renal replacement therapy for children with end-stage renal disease; however, delayed graft function (DGF), a common post-operative complication, may negatively impact the long-term outcomes of both the graft and the pediatric recipient. However, there is limited research on DGF in pediatric kidney transplant recipients. This study aims to develop a predictive model for the risk of DGF occurrence after pediatric kidney transplantation by integrating donor and recipient characteristics and utilizing machine learning algorithms, ultimately providing guidance for clinical decision-making.MethodsThis single-center retrospective cohort study includes all recipients under 18 years of age who underwent single-donor kidney transplantation at our hospital between 2016 and 2023, along with their corresponding donors. Demographic, clinical, and laboratory examination data were collected from both donors and recipients. Univariate logistic regression models and differential analysis were employed to identify features associated with DGF. Subsequently, a risk score for predicting DGF occurrence (DGF-RS) was constructed based on machine learning combinations. Model performance was evaluated using the receiver operating characteristic curves, decision curve analysis (DCA), and other methods.ResultsThe study included a total of 140 pediatric kidney transplant recipients, among whom 37 (26.4%) developed DGF. Univariate analysis revealed that high-density lipoprotein cholesterol (HDLC), donor after circulatory death (DCD), warm ischemia time (WIT), cold ischemia time (CIT), gender match, and donor creatinine were significantly associated with DGF (P < 0.05). Based on these six features, the random forest model (mtry = 5, 75%p) exhibited the best predictive performance among 97 machine learning models, with the area under the curve values reaching 0.983, 1, and 0.905 for the entire cohort, training set, and validation set, respectively. This model significantly outperformed single indicators. The DCA curve confirmed the clinical utility of this model.ConclusionsIn this study, we developed a machine learning-based predictive model for DGF following pediatric kidney transplantation, termed DGF-RS, which integrates both donor and recipient characteristics. The model demonstrated excellent predictive accuracy and provides essential guidance for clinical decision-making. These findings contribute to our understanding of the pathogenesis of DGF.

Distinguishing Features of Childhood Renal Dysplasia

Abstract Background Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. Methods All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK). Results A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5–240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2–173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5–208) months, including 12 renal transplant patients. Conclusions As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

EFIKASI DIRI MENINGKATKAN KUALITAS HIDUP PASIEN GAGAL GINJAL KRONIK YANG MENJALANI TERAPI HEMODIALISIS

Hemodialysis therapy is routinely conducted on end-stage chronic kidney disease patients approximately 2-3 times a week for the patient's survival. However, numerous physical, psychological, and socio-economic problems arise in patients due to routine hemodialysis, thereby impacting the patient's quality of life. This study aims to determine the relationship between self-efficacy and the quality of life of end-stage renal disease (ESRD) patients undergoing hemodialysis (HD). The research employs an analytical observational design with a cross-sectional study approach. The study population includes all end-stage renal disease patients, and the sampling technique is accidental sampling, with a sample size of 80 patients. Data analysis involves univariate and bivariate analyses to examine the relationships among variables using statistical tests, specifically the Spearman correlation test. There is a positive correlation between self-efficacy and quality of life in the physical domain with a p-value 0.001, in the psychological domain with a p-value 0.001, in the social domain with a p-value 0.001, and in the environmental domain with a p-value 0.001.Self-efficacy has a significantly positive correlation with the improvement of the quality of life in the physical, psychological, social, and environmental domains among hemodialysis patients, with a p-value &lt; 0.005

Safety and efficacy of apixaban versus warfarin in peritoneal dialysis patients with non-valvular atrial fibrillation: protocol for a prospective, randomised, open-label, blinded endpoint trial (APIDP2)

IntroductionSeveral randomised controlled trials have demonstrated that novel oral anticoagulants are safer compared with vitamin K antagonists for the management of non-valvular atrial fibrillation (NVAF) to prevent thromboembolic events in...

Intraperitoneally Administered Vancomycin Results in Suboptimal Serum and Peritoneal Effluent Drug Levels in Patients with PD-related Peritonitis

Background Intraperitoneal vancomycin is commonly used to treat peritoneal dialysis (PD)-related peritonitis. Therapeutic drug level monitoring helps optimize the use of vancomycin in CKD patients. We studied whether sufficient serum levels were achieved in patients with PD-related peritonitis treated with the commonly used dose of vancomycin in patients with end stage renal disease. Materials and Methods All consecutive patients with PD-related peritonitis during 19 months were studied. Patients received IP Vancomycin 15 mg/kg Q96H and amikacin 2 mg/kg while awaiting culture reports. Vancomycin concentration in serum and dialysate was determined by a validated liquid chromatography-tandem mass spectrometry assay at 1, 12, 24, and 96 h. The primary outcome was the drug levels in serum and peritoneal fluid, and the secondary outcome was peritonitis treatment failure. Results A total of 45 episodes of PD-related peritonitis were treated, of which 41 fulfilled the inclusion criteria and were included in the PK analysis. Recommended serum vancomycin trough level of &gt;15 mg/L was achieved in only two cases. Twenty-one episodes required catheter removal. Conclusion In patients with PD-related peritonitis, intermittent intraperitoneal administration of 15 mg/kg of vancomycin every 96 h does not achieve the recommended serum and dialysate levels of vancomycin. There is an urgent need for pharmacokinetic studies on commonly used IP antibiotics in the PD population to facilitate correct dose recommendations.

Use of a hybrid closed loop system in a child with end stage renal disease, GAD positive diabetes and recurrent pancreatitis - a game changer!

Use of a hybrid closed loop system in a child with end stage renal disease, GAD positive diabetes and recurrent pancreatitis - a game changer!

Molecular Therapeutics for Diabetic Kidney Disease: An Update.

Diabetic kidney disease (DKD) is a common microvascular complication of diabetes mellitus (DM). With the increasing prevalence of DM worldwide, the incidence of DKD remains high. If DKD is not well controlled, it can develop into chronic kidney disease or end-stage renal disease (ESRD), which places considerable economic pressure on society. Traditional therapies, including glycemic control, blood pressure control, blood lipid control, the use of renin-angiotensin system blockers and novel drugs, such as sodium-glucose cotransporter 2 inhibitors, mineralocorticoid receptor inhibitors and glucagon-like peptide-1 receptor agonists, have been used in DKD patients. Although the above treatment strategies can delay the progression of DKD, most DKD patients still ultimately progress to ESRD. Therefore, new and multimodal treatment methods need to be explored. In recent years, researchers have continuously developed new treatment methods and targets to delay the progression of DKD, including miRNA therapy, stem cell therapy, gene therapy, gut microbiota-targeted therapy and lifestyle intervention. These new molecular therapy methods constitute opportunities to better understand and treat DKD. In this review, we summarize the progress of molecular therapeutics for DKD, leading to new treatment strategies.

Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis

Abstract Objectives Lung transplantation (LuTx) is a life-saving intervention for SSc patients with end-stage lung disease. The aim of this study was to evaluate patients’ survival and LuTx outcomes on systemic disease manifestations. Methods A retrospective evaluation was conducted on SSc patients who underwent LuTx between 2010 and 2021. Outcomes assessed at baseline, 6, 12 and 24 months post-LuTx included skin involvement by modified Rodnan skin score (mRSS), and global disease activity using a modified EUSTAR index (0–9 scale). Lung function rescue was evaluated by forced vital capacity (FVC). Patient survival was assessed by Kaplan–Meier analysis. Results Thirteen SSc patients were included, with a male/female ratio 9/4 and a median age of 48.7 years. Nine patients were affected by diffuse cutaneous scleroderma (dcSSc) and four by limited cutaneous scleroderma (lcSSc). FVC significantly increased from 56% of the predicted value at baseline to 78% at 2 years (P = 0.003). mRSS decreased from 7.4 ± 3.8 to 3.3 ± 2.5 in patients with dcSSc (P = 0.02). The modified EUSTAR index score decreased from 2.54 ± 1.8 at baseline to 0.49 ± 0.5 at 2 years (P = 0.02). Survival rate was 92.3% at 2 years, and 76.9% at 5 years. No unexpected adverse events were observed. Conclusions In SSc patients undergoing LuTx, an excellent 2-year survival was observed, without any disease-related adverse events. Our study supports LuTx as a viable option in SSc patients with end-stage lung disease. Apart from expected recovery of lung function, LuTx was associated with improvement of mRSS and global systemic disease activity.

I’m Telling You for the Last Time: Digital Mediations of Ambiguous Grief, Coping and Resilience on ‘Glioblastology’

This paper examines the way ambiguous grief related to terminal illness is mediated through a blog titled Glioblastology and via social media engagement with its content and author. Building from the psychology framework of ambiguous loss, this paper positions ambiguous grief as a salient lens through which to discover how users in need of coping spaces are engaging digital space as a medium for meaning making and coping during the diagnosis of a terminal illness.

Associations between end stage renal disease and HLA polymorphisms in the Guangxi Zhuang population

To investigate the genetic relationship between end stage renal disease (ESRD) and human leukocyte antigen (HLA) alleles in the Guangxi Zhuang population. We performed polymerase chain reaction reversed sequence-specific oligonucleotide (PCR-rSSO) in 325 patients with ESRD and genotyped the HLA-A, HLA-B, HLA-C, HLA-DRB1, and HLA-DQB1 loci. The direct counting method was used to determine the frequencies of HLA alleles, and Arlequin software (version 3.5.2.2) was used for haplotypic frequency analyses to compare the included ESRD patients with 350 healthy donors from the Guangxi Zhuang population. In our study, 120 HLA alleles, 284 HLA-A-B-DRB1 haplotypes, and 332 HLA-A-C-B-DRB1-DQB1 haplotypes were detected. We found that only A*11:01-B*15:02-DRB1*12:02 had a positive association with ESRD (P = 0.001, Pc = 0.020, OR = 3.106, 95% CI = 1.497–6.446) after Bonferroni correction; thus, individuals with this haplotype may be susceptible to ESRD. A*11:01-B*15:02-DRB1*12:02 is a potentially valuable haplotype for evaluating the risk of ESRD in the Guangxi Zhuang population.