bluuebunny/crossref_metadata_2025_split huggingface dataset:
#6779 EXPERIENCE IN A GERIATRIC NEPHROLOGY CONSULTATION
Authors: Morales Orlando Siverio , Davila Maria Adela , Izquierdo Edduin Miguel Martín , Reguera María José , Garcia Patricia , Bethencourt Alejandro Alonso , Rodríguez Carla , Aragao Ana , Perez Rosa , Dominguez Virginia , Macia Manuel 
Published: June 2023

Abstract

Background and Aims
In the last two decades, the Spanish population over 65 years of age has increased by more than two million people. This has made the coexistence of comorbidities such as arterial hypertension (AHT) and diabetes (DM) more frequent, which act as risk factors in the onset of chronic kidney disease (CKD). This circumstance is conditioned by the presence of other factors associated with aging that condition greater frailty and a greater degree of dependency. Due to this, the application of the comprehensive geriatric assessment could be useful for its adequate stratification, obtaining a better evaluation of kidney disease and, with it, trying to improve the functionality of the patient.

Method
To analyze the parameters associated with CKD and their possible relationship with the degree of frailty and/or functionality in patients older than 75 years referred to a specific Nephrology consultation.
Material and methods: descriptive and observational epidemiological study corresponding to a series of cases, which includes patients from the southern area of Tenerife who were referred for evaluation by the HUNSC Nephrology Service over a period of 8 months.

Results
It was obtained that, of 1263 patients referred, 41.1% of patients were older than 75 years. Of these, 19.1% of patients required face-to-face assessment in the Nephrogeriatrics consultation. When analyzing the relationship between frailty and functionality, we observed that the groups with greater frailty had both older ages and lower hemoglobin and albumin values. Likewise, it was observed that patients classified as frail had a higher risk of mortality than those who were not frail. Regarding functionality, when comparing the means of the parameters associated with CKD in relation to the degree of functionality (Barthel), it was obtained that age, creatinine, hemoglobin and albumin presented statistical significance.

Conclusion
The geriatric population represents a significant number of all consultations referred to Nephrology. The high prevalence of associated diseases that affect renal function (HBP and DM) together with the physiological changes that occur in aging make CKD an important public health problem. Carrying out adequate frailty and functionality scales may constitute one of the most important parameters to assess in nephrogeriatric patients, since it would allow us to improve the efficiency of the care process in CKD.
#6786 ADTKD DUE TO MUC-1 GENE MUTATION: A MAJOR DIAGNOSTIC CHALLENGE, NOT IDENTIFIED BY EXOME SEQUENCING
Authors: Sossai Giulia , Moscato Marco , Gandolfini Ilaria , Delsante Marco , Mellone Simona , Giordano Mara , Cirami Calogero , Allinovi Marco 
Published: June 2023

Abstract

Background and Aims
Autosomal dominant tubulointerstitial renal disease (ADTKD) is a rare cause of end-stage renal disease (ESKD) in adulthood. In addition to its rarity, ADTKD is probably underdiagnosed due to the very non-specific and heterogeneous clinical-laboratory-histopathological-instrumental picture and because of the diagnostic need to use specific methods of genetic investigation in order to highlight the mutations within the VNTR sequences of the MUC1 gene, not recognized by the most common exome sequencing. Mutations in the MUC1 gene, together with those in the UMOD gene, are the main mutations implicated in the pathogenesis of ADTKD.

Method
We analyzed the clinical, laboratory and histopathological characteristics of four families followed by our center with suspected diagnosis of ADTKD and detection of mutation of the MUC1 gene.

Results
Our data (Table 1) revealed a marked phenotypic variability, even within members of the same family (Fig. 1), and in particular as regards the age of disease onset and the different rate of progression towards ESKD (range 16 – 88 years). From a laboratory point of view, in 10 out of 13 patients the sediment was negative, and only in 3 patients proteinuria &gt;1 g/24h was highlighted. In 6/13 the onset occurred at the age of less than 35 years and among these, 4 of them presented ESKD early. In the few cases in which renal biopsy was performed (4/17), representing 0.4% of all biopsies in our centre, the most frequent finding was the presence of tubular atrophy and interstitial fibrosis (3/4), with cystic ectasia of the tubular lumen at the cortical-medullary level in half of the cases. Among the 10 patients subjected to genetic analysis, only in one case the mutation was highlighted by exome sequencing.

Conclusion
The large phenotypic variability, in particular of age at achievement of ESKD, suggests that other factors (modifier or environmental genes) could also affect disease progression. Common gene sequencing methods (e.g. exome sequencing, Sanger) have proved unable, in most cases, to identify mutations within the VNTR sequences of the MUC1 gene.
#6795 DISSEMINATED STRONGYLOIDES INFECTION IN A PATIENT WITH IGA NEPHROPATHY ON IMMUNOSUPPRESSIVE MEDICATIONS
Authors: Parry Manzoor , Dogra Ajay , Yousuf Rayees 
Published: June 2023

Abstract

Background and Aims
Immunosuppressive medications such as high dose steroids and antimetabolites are frequently used in glomerulonephritis. However, use of these medications is usually associated with various adverse effects. Among the drawbacks of immunosuppressive therapy, the risk of reactivation of latent infections, including parasitic infestations, is to be considered.

Method
Case report.

Results
Our case is a 28 year old male patient who was diagnosed as a case of IgA nephropathy 7 months back with M1E1S1T1C0 lesions with a serum creatinine of 1.7 mg/dL and eGFR of 56 ml/min/1.73 m2. He was started on prednisolone (40mg daily) and mycophenolate mofetil (1000mg twice daily) for 1 month. He presented with us with diarrhea. He was treated with IV fluids and mycophenolate mofetil was stopped. After two weeks, he was readmitted with a 4-day history of fever, vomiting, and epigastric pain. On examination, he showed mild epigastric tenderness, normal vitals. The laboratory investigations on admission showed hemoglobin-15 g/dL, total leucocyte count-8600, eosinophilia with absolute eosinophil count 1900/ mm3, urea-92 mg/dL, creatinine-2 mg/dL. ECG showed sinus tachycardia, and HRCT chest revealed bilateral ground glass opacities and a calcified nodule in the left upper lobe. Liver function test was normal with normal amylase and calcium level. Blood culture was sterile. Ultrasound abdomen showed a bilateral raised Eco pattern in kidneys rest were normal. Upper GI endoscopy revealed diffuse edema, erythema, and superficial ulceration in duodenum and biopsy was taken. The duodenal biopsy showed features of strongyloidiasis stercoralis (Figure 1). Bronchoesophageal lavage was done which revealed strongyloidiasis stercoralis larvae. Meanwhile, the patient developed subacute small bowel obstruction with abdominal x ray showing multiple air fluid levels and CT scan showed features of small bowel obstruction with transition at mid-ileal level secondary to short segment thickening. Small bowel obstruction was relived with conservative mangement with ryles tube decompression and enemas. He was diagnosed with disseminated strongyloidiasis and treated with ivermectin for 14 days with full recovery

Conclusion
Our case signifies need of awareness among clinicians of the risk of disseminated strongyloidiasis as a complication of the treatment with immunosuppressive medications even in non-endemic areas.
#6797 EFFICACY OF DESENSITIZATION TREATMENT IN HYPERIMMUNIZED PATIENTS AWAITING A CADAVERIC KIDNEY TRANSPLANTATION
Authors: Garcia Maria Elena Gonzalez , Oliva Maria Ovidia Lopez , Mancebo Esther , Jimenez Carlos 
Published: June 2023

Abstract

Background and Aims
On all kidney waiting lists, patients who have antibodies against more than 80% of a panel reactive antibody (PRA) are difficult to transplant, even with national or regional programs. Desensitization treatment with high-dose intravenous immunoglobulin and rituximab could be offered to patients with a long waiting time for a cadaveric donor to improve their odds of finding a kidney.

Method
Retrospective, single-center study including all hyperimmunized patients on the waiting list for a cadaveric kidney donor who received a desensitization treatment between 2010-2020. To be considered to the desensitization protocol, all the patients must have a PRA above 75% and have been on the waiting list more than 3 years, without any offer. Our treatment was based on the modified Cedars-Sinai Medical Center protocol. Eight hyperimmunized patients on the waiting list for a cadaveric donor kidney transplant (50% male with a mean age of 41.5±16.4 years) received desensitization treatment with intravenous immunoglobulin and Rituximab. Six of the 8 patients (75%) were on renal replacement therapy with hemodialysis and two on peritoneal dialysis (mean time on dialysis 48±14 months). 75% had received a previous transplant. The median PRA calculated was 98%. The time between desensitization and kidney transplantation, the percentage of reactivity against panel cells (PRA) before and after therapy, graft and donor survival, as well as episodes of acute rejection and viral infections were analyzed. The mean follow-up time after transplantation was 67 months.

Results
No patient presented significant side effects to desensitization treatment. Seven of the 8 patients (87.5%) could be transplanted from a cadaveric donor (negative crossmatch test), in a median 8 months after desensitization. No significant changes were observed in the PRA levels. In the immediate post-transplant period, there were 2 graft losses (28.6%) due to non-immunological causes (1 venous thrombosis in a patient with a coagulation disorder and 1 primary graft failure). Creatinine at discharge was 1.6±0.4 mg/dl and 1.4±0.2 a year after the transplant. Five-year censored death graft survival was 100%. Three patients presented viral replication (1 due to cytomegalovirus (CMV), 1 due to polyomavirus (BK) and another CMV+BK), which became negative after switching to m-Tor therapy. There were no episodes of acute rejection. No patient developed cancer during the follow-up.

Conclusion
Desensitization treatment with immunoglobulin and rituximab on hyperimmunized patients on the cadaveric waiting list is a safe and effective treatment that increases the chances of achieving a kidney transplant in highly sensitized patients.
#6829 DIFFERENTIAL LIPIDIC PROFILE WITH THE USE OF CITRATE OR ACETATE DIALYSATES
Authors: Monzo Jose Jesus Broseta , Rodríguez Diana , Cuadrado Elena , Romero Luis Carlos López , Granell Sergio Bea , Jaras Julio Hernandez 
Published: June 2023

Abstract

Background and Aims
Unlike other high-cardiovascular-risk populations, the efficacy obtained from lipid-lowering therapy remains uncertain in dialysis-dependent chronic kidney disease (DD-CKD) patients. This is probably given to the exclusion in clinical trials of patients with very high LDL levels, their population's heterogeneity, accompanying cardiovascular morbidities, and the higher mortality risk explained partly by chronic inflammation. The latter has been associated with the accumulation of uremic toxins, membrane biocompatibility, and acetate-based dialysates, which promote inflammation. Different dialysate weak acidifying agents such as lactic, pyruvic, hydrochloric, or citric acids have surged as less-inflammatory alternatives to acetate. This study aims to evaluate the effects of the metabolism of acetate and citrate in the lipidic profile of DD-CKD patients.

Method
In a unicentric, cross-over, prospective study, we compared the lipidic profile by assessing the plasma levels of total cholesterol (TC), low-density lipoprotein (LDL), triglycerides (TG), high-density (HDL) and very low-density (VLDL) lipoprotein cholesterol, and lipoprotein (a) [Lp(a)] comparing the use of citrate (CD) vs. acetate (AD) as dialysate buffers, after twelve sessions with each one. Prealbumin was also measured to rule out malnutrition as a potential confounding factor. Prevalent (i.e., dialysis vintage ≥3 months) adult patients on maintenance hemodialysis were included. The dialysis prescription parameters and additional medical treatments remained unchanged during their sessions with both dialysates. Quantitative variables are reported with mean and standard deviation. Normal distribution was assessed with the Shapiro-Wilk test, and the comparisons were made with the Student's paired T-test or Wilcoxon's signed-rank test, accordingly. A two-sided p-value ≤0,05 was considered statistically significant.

Results
After twelve dialysis sessions with CD, compared to AD, there was a statistically significant decline in TG and HDL and an increase in LDL. There was also a notable but non-significant reduction in VLDL (Table 1).

Conclusion
There were noteworthy differences in the lipid profile that can only be attributed to the change of dialysis fluid, as we avoided inter-individual (with a cross-over design) and intra-individual (different inflammatory or nutritional profiles would not be expected with this short-term setting) variabilities. Further studies must elucidate if the short-term changes induced in the lipidic profile of DD-CKD patients by the dialysate's weak acid translate into clinical implications.
#6837 ASSOCIATION BETWEEN SELF-REPORTED FATIGUE AND THE NEED FOR RECOVERY TIME AFTER THE MAINTENANCE HEMODIALYSIS SESSION: PROHEMO
Authors: Peredo Gabriel Brayan Gutierrez , Martins Márcia Tereza Silva , Da Silva Fernanda Albuquerque , Lopes Marcelo , Lopes Gildete Barreto , Lopes Antonio Alberto 
Published: June 2023

Abstract

Background and Aims
Fatigue and the need for recovery time after a hemodialysis session are frequent complaints in patients with chronic kidney disease treated with maintenance hemodialysis (MHD). In the present study, we investigated whether there is an association between self-reported fatigue and the need for recovery time after HDM sessions.

Method
This is a cross-sectional study with data from 239 adult patients participating in the prospective cohort study “Prognostic Study of Patients on Chronic Hemodialysis” (PROHEMO), developed in Salvador, BA, Brazil. We used “The Chalder Fatigue” questionnaire (CFQ-11) to calculate fatigue scores. CFQ-11 scores can range from 0 to 33; Higher scores mean more intense fatigue. Fatigue was categorized as: absent or mild if the score was &lt;4 and moderate to severe if the score was ≥4. To assess the need for recovery time from hemodialysis, participants answered a question: After finishing dialysis, do you usually need some time to recover? with two answer options (yes or no). We used logistic regression to estimate the odds ratio (OR) of the association between fatigue and need for recovery after hemodialysis, with adjustments for age, sex, diabetes, and heart failure.

Results
The mean age was 52.5 ± 12.5 years, 57.7% were men, 94.5% were non-white. Self-reported fatigue as absent or mild was 28.4% (68/233) and moderate to severe fatigue was 71.5% (171/233). The prevalence of need for recovery time after hemodialysis was 2.5 times higher in patients with moderate to severe fatigue (prevalence: 51.5%) than in patients with no or mild fatigue (prevalence: 20.6%); prevalence ratio: 2.50 (95% confidence interval: 1.53; 4.08). In logistic regression, an association between moderate to severe fatigue and need for recovery was observed even after adjusting for the effects of age, sex, diabetes, and heart failure (OR = 4.36; 95% CI: 2.21; 8.59).

Conclusion
The results suggest that patients who report a more intense degree of fatigue, more often have a greater need for time to recover after the end of the hemodialysis session. It is interesting to investigate whether interventions that reduce fatigue in patients undergoing MHD treatment result in a reduction in the need for recovery time after the hemodialysis session.
#6861 FABRY DISEASE: A DESCRIPTIVE STUDY OF A COHORT OF PATIENTS
Authors: Pablos Nuria García , Garofano Remedios , Gomez Carlos , Gonzalez Francisco 
Published: June 2023

Abstract

Background and Aims
Fabry disease is a rare X-linked disease, caused by deficient activity of the lysosomal enzyme α-galactosidase A. Accumulation of globotriaosylceramide leads to organ failure and premature death. The aim of this study is to describe the population of patients with Fabry disease in our healthcare area.

Method
Descriptive observational study of patients with Fabry disease (n = 23). Demographic, clinical and treatment variables were analysed.

Results
The mean age of the patients was 47.5 years; 70% female. Symptom onset was at 18 years, being diagnosed 25 years later, similar to that described in the literature. Enzyme deficiency was observed in 61% of patients. Four types of pathogenic variants of the GLA gene were detected. The most prevalent was P205S,followed by W262X,V199G,G80D. The phenotype was classical (V199G, W262X) or late (P205S, G80D).48% of the patients had left ventricular hypertrophy (55% women). 48% had right bundle branch block, atrial fibrillation and sinus bradycardia.47% (64% women) had renal involvement, defined by proteinuria. Other manifestations: hypoacusis (17%), acroparaesthesia and neuropathic pain (13%), ophthalmological (13%) and dermatological (4%).22% were treated with ACE inhibitors/ARA II.1 patient received iSGLT2.Other complementary treatments: statins (22%), antiaggregants (13%), anticoagulants ACOD (1 patient). Regarding specific treatment, 43% were treated, the majority (39%) received enzyme replacement therapy with agalsidase beta (only 1 patient with agalsidase alpha), one patient received migalastat.

Conclusion
The characteristics of our patients are similar to those described in the literature. We observed a predominant general involvement in women, in contrast to other series. Cardiac involvement predominates with left ventricular hypertrophy, and renal involvement with proteinuria.The diagnostic delay is similar to that described, emphasizing the need for early diagnosis and treatment to avoid severe complications and death.
#6863 MANAGEMENT AND OUTCOMES OF SEPSIS ASSOCIATED ACUTE KIDNEY INJURY IN CRITICALLY ILL PATIENTS
Authors: Naouaoui Salma , Chettati Mariam , Fadili Wafaa , Laouad Inass 
Published: June 2023

Abstract

Background and Aims
Sepsis-associated acute kidney injury (AKI) remains a major cause of mortality in patients with critical illness and has an especially high health-related burden in developing countries. However, only a few clinical studies have focused on the clinical characteristics and prognosis of septic AKI, especially in low-income settings. Therefore, the aim of our study was to describe the clinical profile, management and outcome of critically ill patients with sepsis associated AKI.

Methods
A retrospective observational study of critically ill patients with septic AKI was performed from March 2020 to June 2021 at our university hospital. Sepsis was diagnosed clinically by the presence of acute infection and new organ dysfunction. The presence of AKI was evaluated using the Acute Kidney Injury Network (AKIN) criteria. Patients with preexisting ESRD and those who did not fulfill at least one predefined criterion for AKI were excluded.

Results
A total of 105 patients with a median age of 58.9 years (31-78) were enrolled, including 69 and 36 patients with persistent and transient AKI, respectively. The mean SAPS II score was 51.8 (38-77). The primary source of sepsis was mainly abdominal (42.8%). AKI was defined by oliguria in 18 patients (17.1%), by creatinine elevation in 65 patients (61.9%), and by both criteria in 22 patients (21%). Septic AKI was associated with great aberrations in hemodynamics, with 71 patients (67.6%) requiring vasoactive therapy and 65 patients (61.9%) requiring mechanical ventilation. AKI severity according to the AKIN classification scheme was determined to be stage 1 in 32 patients (30.5%), stage 2 in 22 patients (20.9%), and stage 3 in 51 patients (48.6%). Overall, 75.2% of the cohort required renal replacement therapy (RRT). Hospital survival was 63.8% (n = 23) in patients with transient AKI and 43.5% (n = 30) in patients with persistent AKI.

Conclusion
Sepsis is the main cause of AKI in intensive care units, accounting for up to 50% of cases. Our results suggest that septic AKI is associated with higher disease severity scores at admission, requirement of vasoactive drugs, need for mechanical ventilation and increased hospital mortality. Further studies are required, especially in developping countries, to implement more preventive measures and therapeutic interventions to decrease CKD progression and mortality in these patients.
#6866 FRENCH EXPERIENCE OF HOME HEMODIALYSIS PATIENTS TREATED WITH NXSTAGE HAEMODIALYSIS CYCLER WITH THE PURE FLOW SL SYSTEM
Authors: Hebibi Hadia , Berthy Fabien , Attaf David , Ridel Christophe , Stephane Edet 
Published: June 2023

Abstract

Background and Aims
NxStage haemodialysis cycler with the Pure Flow SL system was introduced for the first time in France in June 2022. This system is designed to produce an ultrapure dialysis fluid and mixing a concentrate to the source water to prepare 60 L batch of dialysate. It can be used for a maximum of 3 home treatments. No major plumbing modifications are required to install this system at home. Here, we report to our knowledge the first French clinical evaluation of the use of the Pure Flow system in haemodialysis patients using this device and trained in NephroCare Villejuif haemodialysis center and Rouen Hospital.

Method
This retrospective study was performed in a cohort of 8 hemodialysis patients From June 2022 to January 2023. We describe the clinical benefits of Pure Flow including data on demographic, dialysis vintage, and short-term biological parameters. Patient related outcomes based on a specific questionary, at 3 and 6 months are also analyzed

Results
8 patients were included with 5 women. The mean age was 48 years. The dialysis vintage was 24 months. The median follow-up was 4 months. 2 Patients were treated by pre-mixed 5L dialysate bags 6 months prior the implementation of Pure Flow, and 6 patients started the hemodialysis program directly with Pure Flow after a period of 10 days of training. We show that PF provide adequate dialysis dose. The mean stdkt/v was 2.3. The mean of weekly dialysate volume was 146L.We note optimal ultrafiltration rate, improvement of biological markers of adequacy. Moreover, good results technique survival was recorded. We also show Reduced handling, and setup

Conclusion
The NxStage with Pure Flow system is a major advantage against the premixed bags with a simple installation and a low water consumption. Its use is associated with great clinical outcomes in patients. The Pure Flow should reduce shipments and storage of dialysis treatments and facilitate the development of home hemodialysis in France. Prolonged follow-up on a cohort with a larger number of patients is necessary and will allow an appropriate evaluation of the clinical benefice of the use of the Pure Flow.