Obstructive Uropathy Research Articles

Obstructive uropathy in STAT 3 hyper immunoglobulin E syndrome: A 5year old Middle Eastern boy.

Autosomal dominant hyper immunoglobulin E (IgE) syndrome is a rare inborn error of immunity that affects approximately one in a million individuals worldwide. It presents with various symptoms owing to multisystem involvement (immunological and non-immunological). Recurrent infections (mainly in the skin and lungs) are common presentations. A 5-year-old Middle Eastern boy presented with symptoms suggestive of obstructive uropathy secondary to multiple large pelviabdominal abscesses and acute kidney injury with hyperkalemia that necessitated admission to the intensive care unit. Upon further investigation, the patient's genetic test (whole exome sequencing) demonstrated a heterozygous missense variant in the STAT3 gene. The patient completely recovered and did not require further admission after initiating prophylactic antibiotics. Although deep-seated infections are uncommon in STAT3 hyper IgE syndrome, skin and lung infections are most commonly observed. Multiple deep collections can occur and require prompt intervention and aggressive treatment.

Clinical, Microbiological Profile and Antibiotics Use in Admitted Patients of Urinary Tract Infection

Urinary tract infections (UTI) can vary from simple cystitis to pyelonephritis with severe sepsis. The objective of this study is to provide information about the clinical and microbiological profile of admitted patients of urinary tract infection, patterns of organisms isolated, antibiotic sensitivity pattern and antibiotics use. It was a prospective observational study conducted on 40 patients age >14 years admitted with diagnosis of UTI based on clinical and microbiological criteria over 8 months at a tertiary care hospital in North India. Data was collected for the clinical, microbiological profile, empirical and definite antibiotics use with duration of stay and outcome of patients. Among 40 cases of UTI; male to female ratio was 1:1 with mean age of 51.3± 16.32 years. Fever was present in almost all (97.5%) of the patients and three-fourth (75%) of them had dysuria. Type-2 Diabetes Mellitus was most common (55%) underlying condition and mean HbA1c was 9.37±2.27 followed by obstructive uropathy (17.50%). Most of cases (82.5%) were of complicated UTI; where Pyelonephritis was 42%, Emphysematous Pyelonephritis 12.5% and Renal Abscess 7.5%. Most common (37.5%) organism isolated from urine/ pus culture was Escherichia coli. More than half of the patients (55%) were given empirical antibiotics injection piperacillin tazobactam and carbapenems was used in more than one third (35%) of patients. The mean duration of antibiotics use was 14.55±4.94 days. Two (5.0%) patients expired out during the study period. Uncontrolled Diabetes Mellitus remains the major underlying condition in cases of complicated UTI. E coli is the most common organism isolated from urine/ pus culture. Most of the patients had favourable outcome with guided antibiotics and interventions. Bangladesh Med J. 2023 May; 52(2): 15-19

Gynaecological cancer-related deaths in a tertiary hospital: A four-year retrospective review.

The aim of this study is to describe the profile, causes of death, and associated complications among women who died with a diagnosis of gynecological cancer during a four-year period in a gynae oncology unit in a tertiary hospital. The study is based on a retrospective review of clinical records of patients. There were 368 gynecological cancer admissions during the study period and 51 gynecological cancer-related deaths (13.8%); however, only 48 (13%) of the 51 files were available for analysis. The mean age of the women who died was 52.7 years (SD ±16.92). Most of the women who died were South African citizens (41, 85%), black (44, 91.7%) and unemployed (37, 77.1%). The most common comorbidities were hypertension and HIV which occurred at similar frequencies (20, 41.7%), followed by diabetes mellitus (7, 14,6%). The three most common cancers were cervical (18, 37.5%), ovarian (13, 27.1%), and endometrial (12, 25,0%). All women who died (48, 100%) had some form of cancer-related complications on admission to the hospital. The most common complication at presentation was obstructive uropathy (16, 31.3%) followed by ascites (11, 21.6%) and pleural effusion (8, 15.8%). Just less than half of the patients (22, 45.8%) received palliative treatment due to advanced-stage disease, and the remainder, (20, 41.6%) and (5, 10.4%) surgical and radiation therapy, respectively. The surgical procedure performed was staging laparotomy for ovarian and endometrial cancer (19, 95%) and radical hysterectomy and lymph node dissection for operatable cervical cancer (01, 5%). Forty-nine complications were recorded among the 20 women who underwent surgical treatment. The most common complications were sepsis and hemorrhage followed by organ injury.

Desmoid Type of Fibromatosis of Ureter: A Rare Case

Abstract This study presents a rare case of desmoid-type fibromatosis (DTF) in the ureter of a young adult, highlighting its unusual occurrence within the urinary tract. The patient experienced right flank pain, leading to the discovery of gross hydroureteronephrosis with a suspicious distal ureteric mass. Surgical intervention revealed a well-defined tumor displaying characteristic histological features of DTF. Immunohistochemistry confirmed the diagnosis. Despite the absence of nuclear β-catenin expression, the patient underwent wide local excision. This case highlights the need for individualized treatment plans for DTF and emphasizes its consideration in cases of obstructive uropathy with atypical mass presentations.

Profile of End Stage Chronic Kidney Disease due to Obstructive Uropathy Patients Undergoing Hemodialysis

Introduction. Chronic Kidney Disease (CKD) is a condition of gradual decline in kidney function and ability caused by various kidney diseases. CKD can be caused by several conditions, including obstructive uropathy. Hemodialysis is one of the therapy options for CKD patients. The purpose of this study was to analyze the profile of CKD due to obstructive uropathy patients at Saiful Anwar General Hospital Malang, who underwent hemodialysis. Methods. This study is a descriptive observational research with a cross sectional design, which uses secondary data taken from medical records of patients with end-stage CKD due to obstructive uropathy who undergo hemodialysis in the hemodialysis unit of Dr. Saiful Anwar General Hospital Malang, for the period of 2021-2023. Result. Based on the gender, there were more male patients than female patients (53.13%:46.87%) were females. Hypertension comorbidity was the highest percentage, 71.88%. Based on various conditions, urinary tract stones were the highest cause of obstructive uropathy compared to other causes with a percentage of 62.5%. Conclusion. The result showed that patients were predominantly male with comorbid hypertension. The most common cause of obstructive uropathy in this study was urinary tract stones. Keywords: chronic kidney disease, hemodialysis, obstructive uropathy

Corticomedullary Differentiation in Fetal Kidneys: A Necessary Evil?

AbstractRenal corticomedullary differentiation (CMD) is a crucial indicator of fetal renal health and is detectable as early as 15 to 16 weeks of gestation. Abnormalities in CMD, such as accentuation or loss, may signal underlying renal diseases. CMD assessment via prenatal ultrasound evolves dynamically throughout gestation, reflecting changes in cortical echogenicity and cystic structures. While CMD alterations can indicate conditions like glomerulonephritis or obstructive uropathies, they also offer prognostic insights into future renal function. This case report highlights the importance of early detection and comprehensive evaluation of CMD for optimising prenatal renal care.

Risk stratification framework to improve the utility of renal ultrasound in acute kidney injury.

Acute kidney injury (AKI) is common among hospitalised patients and can lead to significant morbidity or mortality if not properly managed. Renal ultrasound (RUS) is often requested in the initial workup of AKI to rule out obstructive uropathy despite pre-renal aetiologies being implicated in most cases, especially in patients without risk factors for obstruction. Determine the utility of RUS in detecting bilateral hydronephrosis in the context of AKI, and identify risk factors that can be used to stratify patients to better guide patient management. Adults who underwent RUS for AKI between January 2019 and December 2021 were reviewed. Renal ultrasound studies that identified bilateral hydronephrosis and the patient characteristics associated with these studies were recorded. Seven hundred and fifty-eight RUS reports were included. Bilateral hydronephrosis was diagnosed in 43 patients (5.7%). Of these 43 patients, 39 (90.7%) had at least one risk factor for urinary tract obstruction. Bilateral hydronephrosis was only diagnosed in 4 (9.3%) patients without any risk factor for obstruction. The risk factors with the highest odds for being diagnosed with bilateral hydronephrosis included a history of previous ureteric stenting or nephrostomy tube insertion (OR 10.37), previous bilateral hydronephrosis (OR 14.56), or multiple risk factors (OR 23.06). Renal ultrasound has limited utility in the evaluation of AKI in low-risk patients. These risk factors can be used to assign patients to high- or low-risk categories to better guide management and reduce the number of unnecessary studies performed while still identifying clinically significant disease.

CRITICAL REVIEW OF PASHANBHINNA RASA ON ASHMARI WITH SPECIAL REFERENCE TO UROLITHIASIS

Ayurveda, an ancient system of medicine, addresses numerous disorders related to the Mutravaha Srotas (uri-nary system), such as Mutrakricchra (dysuria), Mutraghata (obstructive uropathy), and Mutrashmari (urinary calculi). Among these, Mutrashmari, corresponding to urolithiasis in modern medicine, is one of the most com-mon and distressing conditions, characterised by the formation of stones within the urinary tract. Sushruta iden-tifies two primary mechanisms of stone formation: urine stagnation and supersaturation and crystallisation of urinary crystalloids. Urolithiasis affects approximately 12% of the global population, with a high recurrence rate, especially in males. This review aims to provide a comprehensive analysis of Pashanbhinna Rasa, a herbo-mineral formulation from the Bhaishajya Ratnavali, Ashmari Prakaran, assessing its efficacy in treating Ash-mari (urolithiasis)—the study endeavours to bridge the knowledge gap in contemporary literature regarding this formulation. A thorough literature review focused on references to Pashanbhinna Rasa in classical Ayurvedic texts and modern scientific studies. The review highlights Pashanbhinna Rasa's multifaceted approach to man-aging urolithiasis. The herbo-mineral components of Pashanbhinna Rasa are believed to exert a synergistic ef-fect, potentially dissolving stones, preventing their formation, and providing symptomatic relief. This critical review elucidates the potential of Pashanbhinna Rasa as an effective treatment for urolithiasis. While traditional Ayurvedic formulations offer promising therapeutic benefits, further clinical studies are necessary to validate their efficacy scientifically. Establishing Pashanbhinna Rasa as a standardised treatment could integrate Ayur-vedic wisdom with modern medical practice, offering a holistic approach to managing urolithiasis.

A Study of Clinical Profile of Congenital Anomalies of Kidney and Urinary Tract (CAKUT) in a Tertiary Care Center.

Background Congenital anomalies of the kidney and urinary tract (CAKUT) encompass a diverse array of disorders arising from developmental irregularities in the renal parenchymal development, disrupted embryonic migration of the kidneys, and the urinary collecting system. This study aimed to investigate the clinical presentations, patterns of obstructive and non-obstructive CAKUT, and associated extrarenal manifestations in affected children. Methods This observational study was conducted in the Department of Pediatrics, Acharya Vinoba Bhave Rural Hospital, Wardha. Ethical clearance was obtained, and the study included 105 diagnosed CAKUT patients aged from birth to 18 years. Data collection spanned from June 2022 to May 2024. Clinical features, antenatal findings, associated anomalies, estimated glomerular filtration rate (eGFR), and serum creatinine levels were recorded. Descriptive and inferential statistical analyses were performed using Stata software. Results Among the 105 participants, 81 (77.14%) were males, with a male-to-female ratio of 3.37:1. The mean age was 42.49 months. Forty-two individuals (40%) were asymptomatic, while the most common symptomatic presentation was the ventral opening of the urethra (24.76%). Extrarenal malformations were present in 35 subjects (33.33%), with undescended testis (25.71%) and congenital heart disease (CHD) (20%) being the most common. The antenatal diagnosis was made in 63.8% of cases. Obstructive uropathy was present in 42.86% of subjects, with a significant association between antenatal diagnosis and bilateral hydronephrosis. Medical management was provided to 41.9% of subjects, while 58.1% underwent surgical interventions. Conclusion The study highlights the clinical variability and diverse presentations of CAKUT in children, with a substantial proportion being asymptomatic. Early detection through antenatal screening and prompt intervention can potentially prevent or delay the progression to ESRD. The findings underscore the importance of comprehensive evaluation and targeted management strategies to address both renal and extrarenal manifestations of CAKUT.

Right perirenal urinoma and urinothorax in an infant after neonatal ablation of posterior urethral valve: A rare complication diagnosed by pleural aspiration and treated with perinephric drainage: a case report

ObjectiveUrinothorax and urinoma are rare complications of obstructive uropathy. They might occur due to persistent high back pressure on the renal parenchyma. Urinothorax usually arises while the obstruction exists; in contrast to our case, the child presented after being operated on. He had falsely high creatinine before the operation, which was later explained by creatinine recirculation.Clinical presentation and interventionWe are reporting an uncommon case of late presentation of ruptured urinoma in a 2-month-old Kuwaiti male. It led to urinothorax/uroperitoneum that caused respiratory distress and was associated with creatinine recirculation, requiring retroperitoneal perinephric catheter insertion. The child had recovered and was discharged home.ConclusionA high index of suspicion is required to diagnose urinothorax, especially in patients with a history of obstructive uropathy. Aspiration of the pleural effusion will guide you to reach the diagnosis. Creatinine recirculation is rarely described in the literature. Having a patient with urinothorax/uroperitoneum should raise the suspicion of falsely elevated creatinine levels.

Troubled but in good hands: A qualitative study on patient experiences in diuresis renography examinations

IntroductionDiuresis renography is a nuclear medicine examination that can distinguish between obstructive and non-obstructive uropathy. Renography has been investigated before, but not from a patient perspective. The aim of this study was to gain knowledge and understanding of patient experiences with the diuresis renography process. MethodsThe study had a qualitative descriptive design; data was collected through semi-structured interviews with 17 adult patients that had recently undergone diuresis renography. The transcripts were analysed using inductive qualitative content analysis to identify their manifest and latent content. ResultsOne main theme was identified: ‘Not smooth all the way through, but still in good hands’. The participants understood the necessity of the performed renography and endured the examination despite its worrying elements. They were bothered by diuretic effects, worried about radioactivity and felt isolated during the imaging. The technologists' caring and informative approach eased their experience. The main theme was identified from three subthemes: ‘I experienced inconvenience’, ‘I was worried’ and ‘I felt safe’. ConclusionThe participants experienced worry throughout the renography process but were still satisfied with the examination, mostly because of the technologists being involved. Communication and interaction between patient and technologist are important for creating a good experience. Improved knowledge of patient experiences during renographies could be used to develop patient information and increase technologists’ awareness of factors patients may experience as troubling. Implications for practiceThis study describes issues that contribute to both negative and positive experiences. Improved professional awareness and knowledge about these issues can contribute to the development of patient information and professional encounters, and may improve patients’ compliance and overall experience.

Risk and Prognosis of Cancer in Patients with Obstructive Uropathy Compared with the General Danish Population.

To examine whether obstructive uropathy is associated with increased risk of cancer and whether mortality differs between patients with cancer with and without obstructive uropathy. In a nationwide population-based Danish cohort study including 37,275 adult patients with a first-time hospital-related diagnosis of obstructive uropathy in 1996 to 2022, we assessed cumulative cancer incidence (risk) and standardized incidence ratios (SIR). Furthermore, we compared the mortality of 7,485 patients diagnosed with cancer after obstructive uropathy diagnosis with that of 69,785 patients with cancer without obstructive uropathy matched by age, sex, cancer site, stage, and calendar year of cancer diagnosis. The 3-month risk of cancer after an obstructive uropathy diagnosis was 9.6%. The 3-month SIR was 34.2 [95% confidence interval (CI), 33.1-35.4] while the 1 to <5 year SIR was 1.2 (95% CI, 1.1-1.3). The 3-month SIRs were 82.7 (95% CI, 79.3-86.2) for urological cancer, 88.8 (95% CI, 79.8-98.5) for gynecological cancer, and 13.9 (95% CI, 12.0-15.9) for colorectal cancer. After 1 year of follow-up, the excess number of urological cancers decreased to 0.1 per 100 person-years, whereas we observed no excess risk of gynecological and colorectal cancers. The 5-year all-cause mortality following cancer was 64.1% (95% CI, 62.9-65.2) in patients with an obstructive uropathy diagnosis before cancer diagnosis and 53.2% (95% CI, 52.9-53.6) in those without. A first-time diagnosis of obstructive uropathy can be a clinical marker of underlying undiagnosed cancer and elevated mortality in relation to any cancer diagnosed after obstructive uropathy. These findings can inform the follow-up recommendations for obstructive uropathy.

Obstructive uropathy: Overview of the pathogenesis, etiology and management of a prevalent cause of acute kidney injury.

Obstructive uropathy is defined as the structural or functional interruption of urinary outflow at any level in the urinary tract. It is regarded as one of the most prevalent causes of acute kidney injury (AKI), accounting for 5%-10% of cases. Acute severe obstruction of the urinary tract is a potentially threatening situation for the kidneys and therefore requires prompt identification and management to relieve obstruction. The aim of the present article is to review and synthesize available evidence on obstructive uropathy, providing a clinical guideline for clinicians. A literature review on obstructive uropathy in the context of AKI was performed, focusing on the least clarified aspects regarding diagnosis and management. Recent literature searching was conducted in English and top-level evidence articles including systematic reviews, metanalyses and large series were prioritized. Acute obstruction of the urinary tract is a diagnostic and therapeutical challenge that may lead to important clinical complications together with direct structural and hemodynamic damage to the kidney. Early recognition of the leading cause and its exact location is essential to ensure prompt urinary drainage together with the most suitable drainage technique selection. A multidisciplinary approach, including urologists, nephrologists, and other medical specialties, is best suited to correctly manage concomitant hemodynamic changes, fluid and electrolyte imbalances, and other related issues. Obstructive uropathy is one of the leading causes of AKI. Recognition of patients suitable for early diversion and feasibility or adequate selection of the indicated technique is sometimes challenging. A thorough understanding of the physiopathology behind the development of urinary obstruction is vital for correct diagnosis and management.

Современные поколения урологических дренажных систем. Достижения и перспективы.

Introduction. Currently, stents are used in the treatment of various benign and malignant diseases of the upper urinary tract and urethra. Modern stents made of a superelastic alloy, coated with a polymer, regardless of location, are characterized by higher and longer-lasting patency in terms of decompression and relief of obstruction, fewer irritative symptoms of the genitourinary system and no need for frequent replacement, which leads to improved quality of life in addition to cost savings. Despite promising results, there are few studies in the literature regarding their use, and these tend to be peer-to-peer, retrospective, involving small numbers of patients, and based on the surgeon's personal experience. Materials and methods. In literature review used data about using minimally invasive methods for treatment of ureteral strictures, published in PubMed’s base (https://www.ncbi.nlm.nih.gov/pubmed/), scientific electronic library of RF – Elibrary.ru (https://elibrary.ru/) and also professional endourological association websites. The databases were searched using the following keywords: «ureteral stent», «urethral stent», «allium», «uventa» and «memokath». At the first stage, 93 sources no older than 5 years were found that were relevant to the topic of the review. Conference abstracts, short communications, and duplicate publications were excluded. Then, based on the relevance of the data, the reliability of the sources, the impact factors of the journals and the sequence of presentation of the material in the manuscript, 43 articles in scientific international peer-reviewed journals, practical guidelines and clinical recommendations were selected directly for citation in the review. Results. The stent is one of the most common urological prostheses used in the treatment of obstructive uropathy. The main causes of obstruction of the ureter and urethra include benign and malignant factors. Active introduction of minimally invasive technologies into everyday urological practice using ureteral or urethral stents are effective and safe methods of urine diversion (especially in comorbid patients), however, unfortunately, side effects from the use of these prosthetic systems continue to reduce the quality of life of patients. The use of polymer materials in urology has made it possible to introduce a huge number of non-metallic stents used by urologists, however, due to the occurrence of various kinds of complications, an attempt has been made to introduce a new generation of stents – made of a superelastic alloy, coated with polymer. Stents made of superelastic alloy coated with polymer have been used in the treatment of various pathologies of the ureter and urethra for more than two decades. The need for reliable long-term urinary tract drainage has led to the development of improved urinary tract stent designs such as Memokath, Allium, and Uventa. The main advantages of the latter are a convenient delivery system, a self-expanding mechanism, low adhesive properties, no tissue ingrowth, ease of removal, and no need for frequent replacement. Despite the evolution of ureteral and urethral stent designs, complications continue to arise that limit their use and the search for «ideal» drainage systems continues. Conclusion. Today it is difficult to overestimate the role of drainage systems in the treatment of obstructive urological and oncological diseases. However, despite significant success in their use, prostheses are constantly subject to constant modifications and developments aimed at increasing their suitability, durability, ease of implantation and functionality, while reducing the complications associated with them. Future developments in the field of urogenital prosthetic surgery and urology in general continue to attract interest, and ongoing research into new biomaterials, stem cells and tissue engineering is expanding the boundaries of possible interventions in the human genitourinary tract.

Nephrostomy tube versus ureteral stent for obstructing septic calculi: A nationwide propensity score–matched analysis

Background: Sepsis secondary to obstructive uropathy is a urological emergency that requires urgent decompression using placement of a percutaneous nephrostomy tube (PCN) or retrograde ureteric stent (RUS). Whether selection of PCN or RUS impacts mortality remains uncertain. Methods: We conducted a retrospective cohort analysis using the 2006–2014 Nationwide Inpatient Sample (NIS) of 34,009 patients with sepsis and obstructive uropathy who were treated with RUS or PCN. The primary outcome was in-hospital mortality. Secondary outcomes included hospital length of stay, need for mechanical ventilation, and need for dialysis. Multivariate logistic regression and propensity matched analyses were used to evaluate the effect of PCN or RUS on in-hospital mortality. Results: A total of 9,828 (28.9%) patients were treated with PCN and 24,181 (71.1%) with RUS. The unadjusted mortality for PCN and RUN patients was 5.3% and 2.8%, respectively. Those treated with PCN had a higher likelihood of requiring mechanical ventilation or hemodialysis. In the multivariate logistic regression analysis, RUS had lower odds of mortality compared to PCN (OR 0.72; 95% CI 0.63 to 0.83, p &lt; 0.01). After propensity score matching, the mortality for the RUS group was 3.4% and 4.0% for the PCN group ( p = 0.19). Conclusion: There were no significant differences in mortality for patients treated with PCN versus RUS after propensity matching. Method of decompression should be guided by local practice. Further prospective randomized trials are needed.

#1761 Whole-genome sequencing reveals contribution of rare and common variation to structural kidney and urinary tract malformations

Abstract Background and Aims Structural kidney and urinary tract malformations are the commonest cause of kidney failure in children and young adults. Targeted and whole-exome sequencing has identified over 50 monogenic causes for these phenotypically heterogeneous conditions but have largely focused on cohorts enriched for familial, syndromic, or consanguineous disease. We sought to better characterise the genetic architecture of these conditions using whole genome sequencing (WGS) data from the UK's 100 000 Genomes Project. Method 992 unrelated individuals with structural kidney and urinary tract malformations were assessed using the Genomics England clinical interpretation pipeline to determine a genetic diagnosis. Statistically robust case-control association testing was performed using WGS data from a subset of 813 patients and 25 205 ancestry-matched unaffected individuals seeking enrichment of common and rare single-nucleotide and structural variants (SNV/SVs) on a genome-wide, per-gene, and cis-regulatory element basis. Heritability analysis was carried out in 623 cases and 20 060 controls of European ancestry with polygenic risk scores (PRS) derived for each of the six phenotypes. The PUV-PRS was validated in an independent cohort of 77 patients from the AGORA data- and biobank and an additional 2 746 European controls. Results The overall diagnostic yield was 4.3% (43/992; Fig. 1) with age &amp;lt; 18 years (P = 2.0 × 10−4), family history (P = 7.5 × 10−3) and extra-renal features (P = 1.9 × 10−4) all independent predictors of a genetic diagnosis. The diagnostic yield was higher in cases with cystic kidney dysplasia (10.7%) or kidney anomalies (5.9%). HNF1B was the most frequently identified cause. Exome-wide rare variant analysis identified enrichment of SNV/indels affecting AUTS2 (P = 3.7 × 10−6), ARHGAP5 (P=6.0 × 10−6), known kidney malformation gene HNF1B (P = 6.1 × 10−6), and ZNF879 (P=7.0 × 10−6), all of which have been previously implicated in developmental and/or cancer pathways. An increased burden of rare SVs affecting HNF1B (P = 3.1 × 10−4) was observed. There was no evidence of enrichment for cis-regulatory element disrupting SVs. Genome-wide testing of 19 million common and low-frequency variants (minor allele frequency &amp;gt; 0.1%) did not detect any individual variants that were significantly associated in the total cohort, but together these variants were estimated to contribute to 23% (standard error 11%) of the heritability of kidney and urinary tract malformations in cases with European ancestry. Comparison of phenotype-specific PRS showed considerable overlap between kidney anomalies, cystic kidney dysplasia, obstructive uropathy and vesico-ureteric reflux but the polygenic contribution to PUV and bladder exstrophy was distinct (Fig. 2). A PUV-PRS consisting of 36, 106 variants was validated in an independent European cohort of 77 cases and 2 746 controls (empirical P = 1 × 10−4). Conclusion We show that non-Mendelian genomic factors are important for the pathogenesis of structural kidney and urinary tract malformations, supported by the observation that a minority of patients in this large unselected cohort have a monogenic diagnosis. In addition, a significant proportion of heritability can be attributed to common and low-frequency variation and there is overlap in the polygenic contributors to upper urinary tract malformations with distinct polygenic signatures relevant to PUV and bladder exstrophy. Larger cohorts of patients are needed to increase power to detect variants contributing to this heritability and identify biological mechanisms and pathways important for kidney and urinary tract development.

#2289 Pregnancy outcomes in patients with congenital abnormality of the urinary tract: experience of a tertiary Portuguese center

Abstract Background and Aims Congenital abnormalities of the urinary tract (CAKUT) represent a broad range of disorders that occur due to abnormal kidney development. Maternal, obstetric, and perinatal outcome in pregnant woman with CAKUT have not been extensively evaluated, although small series report an increased incidence of urinary tract infections (UTI) and obstructive uropathy. Method Retrospective analysis of maternal, obstetric, and perinatal outcomes in pregnant patients with CAKUT (including genetic cystic kidney disease) that were surveilled in our tertiary center by a nephro-obstetric team between 2011 and June 2023. Information was obtained from medical records. Results We evaluated 47 gestations in 41 patients, with mean age of 31 ± 5 years [17-40], 89% were Caucasian, 11% black; 40% were nulliparous, 36% had hypertension (HT) and 42% a previous history of UTI. Regarding CAKUT specificity, 17/41 patients had genetic cystic diseases, 10/41 ureteropelvic junction obstruction (UPJ), 4/41 duplicate collecting system, 5/41 vesicourethral reflux, 2/41 ectopic kidney, 3/41 renal hypoplasia. Genetic testing was done before, during or after pregnancy in 2/4/4 patients, respectively. Teratogenic therapy exposure occurred in 25% of gestations. Most patients were CKD stage 1 (33/41), with 2/4/1/1 with patients in CKD stage 2/3/4/5, respectively. Mean baseline SCr was 0.99 ± 0.8 mg/dL [0.4-4.6] and mean proteinuria was 194 ± 357 mg/g [22-1500]. Renal function (RF) deterioration occurred in 9/47 (19%) women due to pre-eclampsia (PE), pregnancy hyperfiltration (PH) and hydronephrosis in 3/3/3 patients, respectively. Urologic intervention was needed in 2 patients due to hydronephrosis, and one of the patients required transient dialysis. All patients fully recovered RF except for the 1 CKD stage 3 patients that had partial RF recovery. De novo and worsening proteinuria occurred in 7/47 gestations due to PE (3/7) and PH (4/7). De novo or aggravation of HTN occurred in 25% gestations, UTI in 19% and PE in 6% gestations. Regarding fetal outcomes, mean duration of gestation was 38 ± 2 weeks [32-41] and mean birth weight 2910 ± 634 g [1360-4600]. There was 1 miscarriage and 1 medical termination of pregnancy at 17 weeks (neural tube defect). Cesarean was performed in (8/47, 17%) gestations and 10/47 (17%) newborns were admitted to the neonatal care unit (NICU) mainly due to prematurity, sepsis and feeding intolerance. Conclusion Our cohort of CAKUT patients was very heterogenous and only 19.5% had CKD stage 2-5. Still, we found a significant incidence of maternal, obstetric, and perinatal complications, namely HT UTI, PE, and the need for NICU. UPJ obstruction can evolve with RF deterioration and the need for intervention during pregnancy. This study underlines the importance of the management of these patients by a multidisciplinary team.

Epidemiological Characteristics of Chronic Renal Failure Patients of Hodeidah, Yemen in 2023

Chronic renal failure (CRF) is an emergent worldwide public health problem. The increasing incidence of CRF warrants a need for an epidemiological approach to better understand the disease and its prevention. Therefore, the study aims to describe the epidemiological characteristics of chronic renal failure patients in Hodeidah, Yemen. The study included 560 patients with CRF in retrospective study (a case series based on medical files) in Center of Dialysis and Renal Diseases of Hodeidah, Yemen from 1st January 2023 to 31st December 2023. The patients were diagnosed based on evidence – based clinical practice guideline of all stages of CRF that was extracted from the National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKFKDOQI). Epidemiological data were reported with ethical approval consideration, data obtained were analyzed. 560 cases were diagnosed with renal failure, 555 patients (99.107 %) of them were diagnosed with CRF and 5 patients (0.89 %) were diagnosed with acute renal failure (ARF). CRF patients was founded 61.81 % in the males while 38.11 % in the females. However, this difference was statistically significant (X2= 30.921; p &lt; 0.00001). The age range of patients was from 5 to 95 years, where 54.64% of cases occurred more than 50 years then 39.28 % between 18 to 49 years and the lower frequency was in children less than 18 years (5.22%) with different significant ( X2= 226.314 ; p &lt; 0.00001). In addition, 81.98 % of patients in rural and 18.02 % in urban. The maximum cases were reported in summer session namely 13.21 % in July and 11.25 % in August. On the other hand , the major causes were chronic diseases 406 case (73.14 %) that diagnosed and recorded , 161 case (29 %) of hypertension ,132 case (23.78 %) of diabetes , 113 (20.36 %) of inflammation nephrolithiasis obstructive uropathy , 8 cases (1.44 %) of genetic polycystic renal disease , and 4 cases ( 0.72 % ) of sedative narcotic addiction and 137 case (24.68%) of unknown causes with different significant ( X2= 119.00 ; p &lt; 0.00001). The chronic diseases namely diabetes and hypertension were the major causes reported to develop the CRF, therefore, to prevent the development of CRF based on center for control diseases and control (CDC), managing risk factors.

#924 Characteristics and outcomes of emergency department patients with acute kidney injury (AKI) stage 2 and 3 electronic alerts at a single UK centre

Abstract Background and Aims The epidemiology of Acute Kidney Injury (AKI) in hospitalized and critically ill populations has been well described but less is known about AKI in the emergency department (ED) patients. Community-acquired AKI represents about a quarter of AKI events observed in the hospital, and its incidence is increasing. Our aim was to look at the characteristics, early management, triage and outcomes of patients with AKI stage 2 and 3 alerts from busy emergency department. Method A retrospective review of patients with AKI 2 and 3 alerts from ED at Doncaster royal Infirmary (DRI) and Bassetlaw sister units in England over a 2 month period from August - September 2022 was conducted. All alerts from Ambulatory/SDEC (Same day emergency care) units have been excluded from this study. Data was gathered from trust electronic health system and patient discharge summaries. Results 158 AKI stage 2 and 3 alerts were received from ED at DRI and Bassetlaw of which 91 (57.5%) were AKI 2 alerts and 67 (42.4%) were AKI 3 alerts. Average age of patients was 73 yrs. Median length of stay in ED was 9hrs (Range 3 hrs–41 hrs). 44 (28%) patients died during this hospital episode of which 28 (64%) died in medical wards, 10 (23%) in critical care unit, 5 died in ED and 1 patient died in surgical ward. Average age of patients who died was 70 yrs and Median length of stay in ED was no different at 9hrs. 23 (52%) had sepsis as primary cause of death. 8 (18%) who died had active malignancy. 9 (20%) had either end stage heart failure/Liver failure or primary lung condition. 8 (18%) had either Frailty, Old age and/or Dementia listed as contributing to death. 8 (18%) patients were discharged home from ED. Discharge diagnosis was Dehydration and/or Falls. Only 4 of the 8 patients had AKI communicated to Primary care on ED discharge letter. 3 of the discharged patients had hospital readmission within 30 days. Looking at the initial intervention in ED, all patients had early warning score and initial resuscitation documented however none of the patients had Fluid balance charted. Bladder scan was performed on 15 (34%) patients where there was high suspicion of obstructive uropathy. Medication as a contributing factor to AKI was commented in 7 (4%) cases. Out of 106 patients who were discharged from hospital wards, sepsis, community acquired pneumonia, urinary tract infection and gastroenteritis were the commonest discharge diagnosis. Median length of Hospital stay was 11 days. Only 2 patients were admitted to specialist Renal ward directly from ED. AKI was communicated to primary care in 70 (62%) patients at discharge. 13 (12%) patients were readmitted to hospital within 30 days of discharge. Conclusion AKI is a marker of severity of illness and we note significant mortality in patients who present to ED with stage 2 and 3 AKI. Sepsis was commonest cause of death but we note a significant proportion of patients had either active malignancy or severe underlying chronic health condition. With increasing waiting times in ED, early recognition and AKI focused management is a priority. AKI education for nursing and medical staff in ED and Pharmacy input for patients who spend long hours in ED might help reduce length of overall hospital stay.

Collagen 17A1 in the Urothelium Regulates Epithelial Cell Integrity and Local Immunologic Responses in Obstructive Uropathy

Collagen 17A1 (COL17A1), an epidermal hemidesmosome component, is ectopically induced in the urothelium of mouse and human renal pelvis (RP) in parallel with urinary tract-associated lymphoid structure development. Here, COL17A1 was induced in obstructive uropathy-prone ureter of humans and cats. To ascertain its function, murine urinary organs with unilateral ureteral obstruction (UUO) were analyzed during 1 week after surgery. One day after UUO, COL17A1 expression increased in urothelial cells of RP and ureter, and was positively correlated with renal tubulointerstitial lesions. A portion of RP where the smooth muscle layer from the ureter was interrupted was sensitive to urothelium deciduation and COL17A1 induction, showing urine leaked from the RP lumen into the parenchyma. After urine stimulation, cultured immune cells expressed Cxcl2, also up-regulated in CD11b+ cells following COL17A1 stimulation. One day after UUO, CXCL2+ CD11b+ cells infiltrated the urothelium-disrupted area. However, these numbers were significantly lower in Col17a1-deficient mice. COL17A1+ urothelial cells partially co-expressed cytokeratin-14, a progenitor cell marker for urothelium, whereas Col17a1-deficient mice had lower numbers of cytokeratin-14+ cells. Gene Ontology analysis revealed that expression of epithelial- and immune-associated genes was up-regulated and down-regulated, respectively, in the ureter of Col17a1-deficient mice 4 days after UUO. Thus, COL17A1 maintains urothelium integrity by regulating urothelial cell adhesion, proliferation, and differentiation, and activates local immune responses during obstructive uropathy in mammals.