End-stage Chronic Renal Failure Research Articles

Correlation of Parathormone and Bone Disorders in Black Adult Hemodialysis Patients in the Public Sector in Abidjan, Côte d’Ivoire

Introduction: The progression of chronic kidney disease (CKD) is characterized by several complications, including disorders of phosphocalcium metabolism characterized by secondary hyperparathyroidism. For this reason, major international recommendations call for at least annual parathyroid hormone (PTH) determinations in patients suffering from CKD, particularly in the terminal phase. This study aims to explore parathyroid hormone status and bone disorders in adult hemodialysis patients in the public sector of Abidjan, West Africa. Materials and Methods: This was a cross-sectional study of 100 end-stage chronic renal failure patients treated by hemodialysis. Parathyroid hormone (PTH) was determined by ELFA enzyme-linked immunosorbent assay on the VIDAS® platform. PTH values were interpreted in relation to the range of 2 to 9 times the upper normal limit in healthy subjects, in line with current KDIGO recommendations, and compared with clinical bone complications. Results: Median PTH level was 315.95 (123.37-725.22) pg/mL. 48% of patients had PTH levels above the recommended threshold. Of these, almost half had no bone complications. Conclusion: The present study did not show a direct relationship between bone complications and the PTH thresholds recommended by KDIGO. This result allows us to envisage a possible inadequacy of the cut-off values for these black African dialysis patients, through subsequent studies.

Pregnancies after kidney transplantation “the journey of hope”. Experience of the University Hospital of Batna, Algeria

Introduction. Renal transplantation is the treatment of choice for chronic end-stage renal failure. Transplantation restores the fertility of transplant recipients, which explains the increase in the number of successful pregnancies in this population. However, it remains a high maternal risk pregnancy fetal and/or neonatal which requires a multidisciplinary approach. Objective. The aim of this study is to provide the results of our experience on pregnancies in kidney transplant patients and to evaluate the impact of pregnancy on kidney graft function. Patients and methods. 79 pregnancies in kidney transplant recipients were analyzed and the long-term results of kidney transplantation were studied. We analyzed the results from clinical and biological data before, during and after pregnancy. Results. Average age of the patients was 35.3 ± 3 years and the time between transplantation and the start of pregnancy was 56.4 ± 31.5 months. There was no significant difference between biological data before and after pregnancy. We did not observe any acute rejection. The average maternal complications were preeclampsia in 30% of cases, low birth weight in 29% of cases, prematurity in 44% and cesarean sections in 57%. There was no impact of pregnancy on the kidney graft during follow-up. Conclusion. Despite the frequency of premature deliveries and comorbidities, monitoring and management of kidney transplant recipients revealed good functional results of the kidney graft.

Prevalence and Characteristics of Anemia in Patients with Chronic Kidney Disease at Herat Regional Hospital: A Descriptive Study (2018–2019)

Objectives: Anemia is common in patients with chronic kidney disease and reduces their quality of life. This anemia, caused by the kidneys' inability to produce erythropoietin, contributes to the progression of chronic kidney disease and increases comorbidities and mortality. Chronic kidney disease and end-stage renal failure reduce kidney function, which can lead to death if dialysis or kidney transplantation is not performed. Diabetes and hypertension are the main causes of End-Stage Renal Disease (ESRD). The objective of this study is to investigate the prevalence and causes of anemia in chronic kidney disease patients at Herat Regional Hospital. Methods: This descriptive study collected data from patient files, laboratory tests, echocardiography, patient registry, and the Health Management Information System (HMIS). Results: Out of a total of 278 patients, 65.5% were male. Most patients were over 50 years old, with approximately 75.5% coming from Herat districts and neighboring provinces. Among chronic kidney disease patients, 39.20% had anemia along with type 2 diabetes, and 35.25% had anemia with hypertension. About 64.4% of patients did not receive erythropoietin, and 60.8% were not on hemodialysis. Conclusion: This study shows that anemia is prevalent among chronic kidney disease patients, particularly among elderly men and rural residents. The findings highlight the need for improved access to treatments like erythropoietin and hemodialysis.

Expression of Autophagy Markers LC3B, LAMP2A, and GRP78 in the Human Kidney during Embryonic, Early Fetal, and Postnatal Development and Their Significance in Diabetic Kidney Disease.

Autophagy is the primary intracellular degradation system, and it plays an important role in many biological and pathological processes. Studies of autophagy involvement in developmental processes are important for understanding various processes. Among them are fibrosis, degenerative diseases, cancer development, and metastasis formation. Diabetic kidney disease is one of the main causes of chronic kidney disease and end-stage renal failure. The aim of this study was to investigate the immunohistochemical expression patterns of LC3B, LAMP2A, and GRP78 during different developmental stages of early-developing human kidneys and in samples from patients with type II diabetes mellitus. During the 7/8th DW, moderate expression of LC3B and LAMP2A and strong expression of GRP78 were found in the mesonephric glomeruli and tubules. In the 9/10th DW, the expression of LC3B and LAMP2A was even more pronounced in the mesonephric tubules. LC3B, LAMP2A, and GRP78 immunoreactivity was also found in the paramesonephric and mesonephric ducts and was stronger in the 9/10th DW compared with the 7/8th DW. In addition, the expression of LC3B, LAMP2A, and GRP78 also appeared in the mesenchyme surrounding the paramesonephric duct in the 9/10th DW. In the 15/16th DW, the expression of LC3B in the glomeruli was weak, that of LAMP2A was moderate, and that of GRP78 was strong. In the tubuli, the expression of LC3B was moderate, while the expression of LAMP2A and GRP78 was strong. The strongest expression of LC3B, LAMP2A, and GRP78 was observed in the renal medullary structures, including developing blood vessels. In postnatal human kidneys, the most extensive LC3B, LAMP2A, and GRP78 expression in the cortex was found in the epithelium of the proximal convoluted tubules, with weak to moderate expression in the glomeruli. The medullary expression of LC3B was weak, but the expression of LAMP2A and GRP78 was the strongest in the medullary tubular structures. Significantly lower expression of LC3B was found in the glomeruli of the diabetic patients in comparison with the nondiabetic patients, but there was no difference in the expression of LC3B in the tubule-interstitial compartment. The expression of LAMP2A was significantly higher in the tubule-interstitial compartments of the diabetic patients in comparison with the nondiabetic patients, while its expression did not differ in the glomeruli. Extensive expression of GRP78 was found in the glomeruli and the tubule-interstitial compartments, but there was no difference in the expression between the two groups of patients. These data give us new information about the expression of LC3B, LAMP2A, and GRP78 during embryonic, fetal, and early postnatal development. The spatiotemporal expression of LC3B, LAMP2A, and GRP78 indicates the important role of autophagy during the early stages of renal development. In addition, our data suggest a disturbance in autophagy processes in the glomeruli and tubuli of diabetic kidneys as an important factor in the pathogenesis of diabetic kidney disease.

The Functional Role of microRNAs and mRNAs in Diabetic Kidney Disease: A Review.

Diabetes is a group of diseases marked by poor control of blood glucose levels. Diabetes mellitus (DM) occurs when pancreatic cells fail to make insulin, which is required to keep blood glucose levels stable, disorders, and so on. High glucose levels in the blood induce diabetic effects, which can cause catastrophic damage to bodily organs such as the eyes and lower extremities. Diabetes is classified into many forms, one of which is controlled by hyperglycemia or Diabetic Kidney Disease (DKD), and another that is not controlled by hyperglycemia (nondiabetic kidney disease or NDKD) and is caused by other factors such as hypertension, hereditary. DKD is associated with diabetic nephropathy (DN), a leading cause of chronic kidney disease (CKD) and end-stage renal failure. The disease is characterized by glomerular basement membrane thickening, glomerular sclerosis, and mesangial expansion, resulting in a progressive decrease in glomerular filtration rate, glomerular hypertension, and renal failure or nephrotic syndrome. It is also represented by some microvascular complications such as nerve ischemia produced by intracellular metabolic changes, microvascular illness, and the direct impact of excessive blood glucose on neuronal activity. Therefore, DKD-induced nephrotic failure is worse than NDKD. MicroRNAs (miRNAs) are important in the development and progression of several diseases, including diabetic kidney disease (DKD). These dysregulated miRNAs can impact various cellular processes, including inflammation, fibrosis, oxidative stress, and apoptosis, all of which are implicated during DKD. MiRNAs can alter the course of DKD by targeting several essential mechanisms. Understanding the miRNAs implicated in DKD and their involvement in disease development might lead to identifying possible therapeutic targets for DKD prevention and therapy. Therefore, this review focuses specifically on DKD-associated DN, as well as how in-silico approaches may aid in improving the management of the disease.

ACUTE AND DISEASE WITH GLOMERULONEPHRITIS CYTOKINE BALANCE IN SICK PATIENTS

Glomerular kidney damage -glomerulonephritis (GN) is one of the serious medical and economic problems. Often, GN is the main cause of end-stage chronic renal failure, which is incompatible with life and requires renal replacement therapy (dialysis, donor kidney transplantation) [2]. According to the course of the disease, there is an acute form of GN (the durationof the disease is less than one year) and chronic (the duration of the disease is more than one year). In clinical practice, there are often difficulties in timely differentiation of chronic GN and acute GN exacerbation, in particular, in the urinary (hidden) form of the disease, due to the lack of clear criteria for their diagnosis.

Pituitary disorders in patients with end-stage chronic renal failure

Disorders in the kidneys lead to disturbance of homeostasis. As the glomerular filtration rate decreases, the metabolism of numerous biologically active substances, including pituitary hormones, decreases. The article presents an overview of pituitary dysfunction in patients with chronic kidney disease (CKD) and discusses the possible reasons of the pathogenetic mechanisms. Particular focus is being given to the assessment of changes in the concentration of pituitary hormones in patients with end-stage chronic kidney disease (CKD) and discusses the pathogenetic mechanisms of their formation. Particular attention is paid to the assessment of changes in the concentration of pituitary hormones in patients receiving renal replacement therapy (RRT). CKD leads to an increase in the level of prolactin, luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Concentrations of growth hormone (GH), isulin-like growth factor-1 (IGF-1), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH) and vasopressin may remain within normal values or increase in this group of patients. RRT does not reduce the levels of prolactin, LH, FSH, while the concentration of growth hormone, IGF-1, TSH tends to normalize. The content of ACTH and vasopressin may remain unchanged or decrease. Kidney transplantation in most cases corrects hormonal disorders. Correction of hormonal changes can improve the clinical outcome and quality of life of patients with end stage CKD.

State of Transplantation in the Republic Of Uzbekistan: Existing Problems of Donation.

Organ traTransplantation is one of the most successful achievements in modern medicine. For patients with end-stage chronic renal failure, transplantation undeniably improves their quality of life. The purpose of the study was to discuss the latest scientific data on the state of kidney transplantation in the world and to give an idea of the advantages, opportunities, and problems of kidney transplantation in the world and in the Republic of Uzbekistan. Transplantation in Uzbekistan developed in 5 stages. Stage I (1972-1991) began with the first kidney transplant in Uzbekistan on September 14, 1972, on the basis of the legislation on organ and tissue transplantation of 1970. Stage II (1991-1998) represented a break in the field of transplantation due to its prohibition by law. Stage III (1998-2017) comprised a series of 48 kidney transplants from living related donors on the basis of an order of the Ministry of Health of the Republic of Uzbekistan. Stage IV (2017-2022) comprised a series of 849 kidney transplants and 22 liver transplants from living related donors on the basis of Cabinet of Ministers Resolution No. 859 of October 17, 2017. Stage V began on May 11, 2022, with the adoption of the a new of the Republic of Uzbekistan, On Transplantation of Human Organs and Tissues. According to this new law, the objects of transplantation can be human organs and (or) tissues taken from either a living donor or a deceased donor. This law will expand the range of donors for patients in need. Transplantology is a sphere of life activity that can no longer be ignored; therefore, it is necessary to develop and implement humanistic principles on the basis of which it will be regulated.

Management of the Diabetic Microalbuminuria with Ayurvedic intervention- A Case Report

Background: One of the most prevalent side effects of diabetes, diabetic nephropathy causes Hypoalbuminemia, chronic kidney damage and end-stage renal failure worldwide. This study was performed for the effectiveness of the Ayurvedic treatment in the patient presented with the complications of Diabetic Microalbuminuria with special reference of Kaphaja Shotha. A 67-year-old, male patient consulted to the Ayurveda hospital with generalized body swelling, difficulty in breathing on the rest, Fatigue for 2 years, following secondary prevention for two years. Design: Single Case report. Materials and Methods: In this prospective case study, conducted on a subject experiencing edema as a complication of diabetic nephropathy for two years, the individual was admitted to the Indoor Patient Department of Medicine. Over a period of three months, the subject was assessed every 15 days. The intervention involved the administration of oral medications including mainly Gokshuradi guggulu, Punarnavadi guggulu, Hingwastak Churna, Triphala Churna, Syrup Neeri KFT, and Gomutra Haritaki. The edema reduced progressively, and there was an alleviation of associated symptoms such as difficulty in breathing and fatigue. Additionally, the subject showed improvement in renal function parameters and reported an enhanced sense of well-being. Results: Laboratory investigation showed protein in urine and hypoalbuminemia with normal blood urea and S. Creatinine., which was corrected after treatment, increased serum albumin from 1.9 (g/dl) to 3.9(g/dl) and serum globulin from 1.7(g/dl) to 2.7 (g/dl). After 18 days, a significant improvement was noted in all the clinical features, blood investigations, Chest X ray of the patient and no any unwanted effects were noted during the study period. The case was managed without encountering any adverse effects, following the treatment regimen for Shotha. Keywords: Diabetic Microalbuminuria, Hypoalbuminemia, Kaphaja Shotha

Epidemiological, Clinical, Therapeutic, and Evolutionary Aspects of Chronic End-Stage Renal Failure in the Nephrology Department of the University Hospital of Brazzaville in 2023

Epidemiological, Clinical, Therapeutic, and Evolutionary Aspects of Chronic End-Stage Renal Failure in the Nephrology Department of the University Hospital of Brazzaville in 2023

PATIENT TRANSFER FROM HEMODIALYSIS TO PERITONEAL DIALYSIS: A SINGLE CENTER EXPERIENCE

INTRODUCTION Peritoneal dialysis and hemodialysis are complementary ways of treating end-stage renal failure. Changing the dialysis modality from hemodialysis to peritoneal dialysis is a rare and poorly studied phenomenon. MATERIALS AND METHODS Retrospective cohort study conducted on the population of adult patients with end-stage chronic renal failure who were treated at the Nephrology Clinic of the Clinical Center of the University of Sarajevo in the period from 2006 to 2023. A total of 109 adult patients, whose medical documentation was complete and who were in the peritoneal dialysis program at the Nephrology Clinic of the Clinical Center of the University of Sarajevo during the observed period, were included in this study. One group started the treatment with peritoneal dialysis, and the other with hemodialysis. Demographic data were collected for each patient: age, gender, underlying kidney disease, comorbidities (heart disease and diabetes), duration of treatment modality, data on modality change, complications and treatment outcomes. Data from physical and electronic patient histories were used. RESULTS Total of 109 adult patients were included in this study. They are divided into two groups. Group 1 (n=99) in which peritoneal dialysis was the first treatment modality and Group 2 (n=10) in which haemodialysis was the first treatment modality, but in which patients, after a certain time, were transferred to peritoneal dialysis. The median age of patients in Group 1 was 60 (-/-14.07) years and 54 (-/+12.23) years for Group 2. Within Group 1 the most common cause of terminal renal failure was diabetic nephropathy (n=40, 40.4%) and nephroangiosclerosis (n=24, 24.24%). The mean age of onset of peritoneal dialysis was 60 (-/-14.07) years, while the mean age of cessation of peritoneal dialysis was 63 (±13.69) years. The average duration of peritoneal dialysis treatment was 38.36(±34.14) months. During the stay at peritoneal dialysis, death was recorded in 63 patients (62.38%). The number of patients who replaced peritoneal dialysis treatment with hemodialysis was 26. The most common reason for switching to haemodialysis was insufficiency of peritoneal dialysis (n=13, 14%). After switching to haemodialysis, the average length of staying on it was 10.22 months. The reason for discontinuation of haemodialysis was death (n=17, ) or transplantation (n=1, 3.7%). Kaplan-Meier test shown worse outcome in patients with haemodialyis first than peritoneal dialysis first. CONCLUSION Changing the dialysis modality carries with it a high risk of mortality, especially in the first month. Changing the way of active treatment with dialysis speaks in favor of severe comorbidities.

706. An Ad Hoc Subgroup Analysis of a Phase 3, Open-Label Study Indicates Efficacy and Safety of Fecal Microbiota, Live-jslm in Participants With Recurrent Clostridioides difficile Infection and Renal Impairment

Abstract Background Patients with renal comorbidity are at risk of severe Clostridioides difficile infection (CDI) and recurrence. Fecal microbiota, live-jslm (REBYOTA™, abbreviated here as RBL, previously known as RBX2660) is the first FDA-approved, microbiota-based live biotherapeutic for the prevention of recurrent CDI (rCDI) in adults following antibiotic treatment for rCDI. An ad hoc subgroup analysis of PUNCH CD3-OLS (NCT03931941), an ongoing, open-label, phase 3 trial evaluating the efficacy and safety of RBL, assessed outcomes in participants with renal comorbidity. Methods PUNCH CD3-OLS participants were ≥ 18 years old with medically documented rCDI, including first recurrence determined by the treating physician, and assessed with standard-of-care (SOC) diagnostic methods. After SOC antibiotics, participants received a single dose of rectally administered RBL. Treatment success was defined as remaining recurrence free for 8 weeks after treatment. Treatment-emergent adverse events (TEAEs) through 6 months of treatment were reported. Participants with renal comorbidity were identified from the medical history dictionary-derived terms in the modified intent-to-treat (mITT) population. Results Within the mITT population, 98 of 402 participants with adjudicated outcomes had renal comorbidity, including chronic kidney disease (n=29) and end-stage renal failure (n=5). Of participants with renal comorbidity, 50% had Charlson Comorbidity Index scores of ≥ 5, versus 18% of participants without renal comorbidity. Treatment success was achieved by 66% (65/98) and 77% (235/304) of participants with and without renal comorbidity, respectively. TEAEs occurred in 71% (n=70) of participants with renal comorbidity and 64% (n=194) of participants without renal comorbidity. In both groups, most TEAEs were moderate in severity and related to preexisting conditions. Serious TEAEs were reported by 16% (n=16) and 8% (n=24) of participants with and without renal comorbidity, respectively. The most commonly reported serious TEAE was CDI recurrence, occurring in 3.1% (n=3) and 1.6% (n=5) of participants with and without renal comorbidity, respectively. Conclusion RBL treatment success and TEAE incidence were numerically comparable for those with and without renal comorbidity. Disclosures Monika Fischer, MD, Ferring Pharmaceuticals Inc.: Advisor/Consultant|Rebiotix Inc., a Ferring Company: Board Member|Seres Pharmaceuticals: Advisor/Consultant Joan Thul, BA, Ferring Pharmaceuticals Inc.: Employee Beth Guthmueller, AS, Rebiotix Inc., a Ferring Company: Employee Christian Sandrock, MD, Allergan: Advisor/Consultant|National Institutes of Health: Grant/Research Support|Shionogi: Advisor/Consultant|The Health Resources & Services Administration: Grant/Research Support Nicholas Van Hise, PharmD, Ferring Pharmaceuticals Inc.: Advisor/Consultant|Ferring Pharmaceuticals Inc.: investigator Glenn S. Tillotson, PhD, Dynavax: Advisor/Consultant|Ferring Pharmaceuticals: Advisor/Consultant|Peggy Lillis Foundation: Honoraria|Spero Therapeutics: Advisor/Consultant

Simultaneous pancreas-kidney transplantation for end-stage renal failure in type 1 diabetes mellitus: Current perspectives.

Type 1 diabetes mellitus (T1DM) is one of the important causes of chronic kidney disease (CKD) and end-stage renal failure (ESRF). Even with the best available treatment options, management of T1DM poses significant challenges for cli nicians across the world, especially when associated with CKD and ESRF. Substantial increases in morbidity and mortality along with marked rise in treatment costs and marked reduction of quality of life are the usual consequences of onset of CKD and progression to ESRF in patients with T1DM. Simultaneous pancreas-kidney transplant (SPK) is an attractive and promising treatment option for patients with advanced CKD/ESRF and T1DM for potential cure of these diseases and possibly several complications. However, limited availability of the organs for transplantation, the need for long-term immunosuppression to prevent rejection, peri- and post-operative complications of SPK, lack of resources and the expertise for the procedure in many centers, and the cost implications related to the surgery and postoperative care of these patients are major issues faced by clinicians across the globe. This clinical update review compiles the latest evidence and current recommendations of SPK for patients with T1DM and advanced CKD/ESRF to enable clinicians to care for these diseases.

A case of uremic pruritus and papulo-pustular rash associated with anti-EGFR therapy in a patient with rectal cancer and chronic kidney disease

Uremic pruritus is a common symptom in chronic kidney disease and end-stage renal failure. In addition to physical discomfort uremic pruritus disrupts sleep, negatively affects the psycho-emotional state and quality of life. In this group of patients, the association of uremic pruritus with an increase in mortality due to any causes was demonstrated. At the same time, there are no standardized approaches to the treatment of uremic itching. There is also a special category of patients receiving antitumor therapy and developing dermatological adverse events, also potentially accompanied by itching.
 This article presents a case of uremic pruritus in a patient with papulo-pustular cutaneous reaction (grade II on a Scale Common Terminology Criteria for Adverse Events, CTCAE v 5.0) to the epidermal growth factor (EGFR) inhibitor cetuximab in combination with leucovorin and 5-fluorouracil for rectal cancer. Treatment of uremic pruritus with small doses of gebapentin (300 mg/day) led to complete regression of pruritus. Papulo-pustular rashes completely regressed after recommended systemic and topical therapy according to the severity of rush (doxycycline 100 mg 2 times a day for 5 days, cream with neomycin, natamycin and hydrocortisone 3 times a day for 7 days). Pruritus was absent during the next 6 months of follow-up. Antitumor therapy was not interrupted due to acneiform rush, and following supportive topical therapy allowed to control severity of exacerbations which did not exceed III grade according to CTCAE v 5.0 and did not require the addition of systemic therapy.
 Thus, therapy of uremic pruritus with gabapentin has shown was effective also in a patient with severe comorbid pathology. Supportive topical therapy consistent with the severity of papulo-pustular rash reduced the severity of exacerbations during following EGFR inhibitor therapy.

Artificial intelligence-assisted repurposing of lubiprostone alleviates tubulointerstitial fibrosis

Tubulointerstitial fibrosis (TIF) is the most prominent cause which leads to chronic kidney disease (CKD) and end-stage renal failure. Despite extensive research, there have been many clinical trial failures, and there is currently no effective treatment to cure renal fibrosis. This demonstrates the necessity of more effective therapies and better preclinical models to screen potential drugs for TIF. In this study, we investigated the antifibrotic effect of the machine learning-based repurposed drug, lubiprostone, validated through an advanced proximal tubule on a chip system and in vivo UUO mice model. Lubiprostone significantly downregulated TIF biomarkers including connective tissue growth factor (CTGF), extracellular matrix deposition (Fibronectin and collagen), transforming growth factor (TGF-β) downstream signaling markers especially, Smad-2/3, matrix metalloproteinase (MMP2/9), plasminogen activator inhibitor-1 (PAI-1), EMT and JAK/STAT-3 pathway expression in the proximal tubule on a chip model and UUO model compared to the conventional 2D culture. These findings suggest that the proximal tubule on a chip model is a more physiologically relevant model for studying and identifying potential biomarkers for fibrosis compared to conventional in vitro 2D culture and alternative of an animal model. In conclusion, the high throughput Proximal tubule-on-chip system shows improved in vivo-like function and indicates the potential utility for renal fibrosis drug screening. Additionally, repurposed Lubiprostone shows an effective potency to treat TIF via inhibiting 3 major profibrotic signaling pathways such as TGFβ/Smad, JAK/STAT, and epithelial-mesenchymal transition (EMT), and restores kidney function.

BONE MARROW OXALOSIS, A RARE CAUSE OF PANCYTOPENIA: A CASE REPORT

Primary hyperoxaluria is a rare congenital metabolic disease characterised by an overproduction of oxalate, secondary to a hepatic enzyme deficiency, alanine glyoxylate aminotransferase (AGT). Calcium oxalate crystals are deposited in many tissues. Involvement of the bone marrow is relatively rare. We report the case of a young 29-year-old patient with a history of recurrent renal lithiasis, progressing to end-stage chronic renal failure at the haemodialysis stage, admitted to the medical ward for investigation of mucocutaneous pallor and anaemia resistant to erythropoietin. Clinical and laboratory examination revealed an oedematous-ascitic syndrome and pancytopenia with normocytic normochromic anaemia. Imaging revealed moderate ascites and homogeneous splenomegaly. The myelogram was inconclusive. An osteomedullary biopsy was performed and showed deposits of oxalate crystals with bone marrow fibrosis associated with foreign body macrophagic granulomas. These crystals were grouped in a rosette and birefringent in polarised light. Primary hyperoxaluria is a rare disease. Involvement of the bone marrow indicates the advanced stage of the disease, hence the importance of anatomopathological diagnosis. Hepato-renal transplantation remains the only effective treatment for this disease.

Primary Hyperoxaluria Type 1: Clinical, Paraclinical, and Evolutionary Aspects in Adults from One Nephrology Center.

Primary hyperoxaluria type 1 (PH1) is a rare and inherited condition of urolithiasis. The aim of our study was to analyze clinical, paraclinical, and evolutionary aspects of PH1 in adult patients in our Nephrology department. We conducted a retrospective single-center study between 1990 and 2021. We collected patients followed for PH1 confirmed by genetic study and/or histopathological features of renal biopsy and morphoconstitutional analysis of the calculi. There were 25 patients with a gender ratio of 1.78. The median age at onset of symptoms was 18 years. A delay in diagnosis more than 10 years was noted in 13 cases. The genetic study found the I244T mutation in 17 cases and 33-34 InsC in 4 cases. A kidney biopsy was performed in 5 cases, on a native kidney in 4 cases and on a graft biopsy in one case. The analysis of calculi was done in 10 cases showing type Ic in 2 cases. After a median follow-up of 13 years (1 year-42 years), 14 patients progressed to end-stage chronic renal failure (ESRD). The univariate study demonstrated a remarkable association with progression to ESRD in our population (44% vs. 56%) RR = 13.32 (adjusted ORs (95% CI): 2.82-62.79) (p < 0.01). Progression to ESRD was frequent in our series. Early diagnosis and adequate management can delay such an evolution.

Genomic testing in patients with renal disease.

Inherited kidney disease accounts for a significant proportion of chronic kidney disease and end-stage renal failure. There is increasing evidence that genetic testing for inherited kidney disease should be integrated into clinical care pathways at the earliest opportunity so that patients and their families can maximally benefit from carefully tailored care. Despite increased availability of genetic testing, the proportion of patients with renal disease undergoing genetic investigations remains low. This article introduces key concepts of genetic and genomic testing to the renal physician and addresses some common barriers to the wider integration of genetic testing in routine clinical practice to fully capitalise on recent advances in genomic medicine and improve patient outcomes.

IgA nephropathy associated with Crohn's disease.

The relationship between IgA nephropathy (IgAN) and Crohn's disease was reported. IgAN is the most common primary glomerulonephritis and one of the leading causes of chronic kidney disease and end-stage renal failure, and up to 50% of cases progressed to end-stage renal disease within 25 years after IgAN diagnosis. However, specific and effective therapeutic strategies are still lacking. In this review, we discuss the possibility of the mechanism involved in IgAN associated with Crohn's disease based on the findings of basic and clinical studies. Although the etiology of IgAN associated with Crohn's disease is not permanent and various factors are thought to be involved, the stabilization of the disease condition of Crohn's disease is believed to help treat IgAN.

#3739 SHUNT PORTOSYSTEMIC COMPLICATED BY HYPERAMMONEMIA AND HEPATIC ENCEPHALOPATHY SUPPORTED BY DAILY DIALYSIS IN A CHRONIC HEMODIALYSIS PATIENT

Abstract Background and Aims Hepatic encephalopathy is a neuropsychiatric syndrome that may develop as a consequence of liver insufficiency. In acute liver failure, hepatic encephalopathy suggests the severity of the disease. In end-stage chronic liver diseases, episodes of hepatic encephalopathy are frequently fully and spontaneously reversible. Clinical manifestations vary from mild neuropsychiatric disorders to coma. The pathogenesis of hepatic encephalopathy is complex and not clearly understood. Ammonia plays a key role. In the healthy individual, ammonia is, during its first hepatic passage, directly degraded into urea, itself excreted by the kidney and the stools, and to a lesser degree in glutamine. Ammonia is also metabolized by the muscle striped. In case of liver failure and/or shunts portosystemic, there is a defect in hepatic clearance ammonia which is then found in excess in systemic circulation. Decreased muscle mass, linked to malnutrition, frequent in the patient cirrhotic and chronic hemodialysis, also helps to decrease metabolism ammonia. Due to the increase in permeability of the blood-brain barrier, the brain is exposed to excessive concentrations ammonia causing functional brain abnormalities and structural, which can partly explain the signs neurology of hepatic encephalopathy. In end stage chronic renal failure, clearance of ammonia via extracorporeal treatment has not been systematically evaluated. Several studies with small samples have demonstrated the effectiveness of hemodialysis, compared to peritoneal dialysis, in terms of ammonium purification in chronic renal failure, on dialysis or not on dialysis. The aim of this study was to evaluate the effectiveness of daily dialysis for the purification of ammoniumin a chronic hemodialysis patient with hepatic encephalopathy. Case Report A 40-year-old man undergoing chronic hemodialysis was referred to gastroenterology department of Mohammed VI University center of Marrakesh for inoperable shunt portosystemic complicated by hyperammonemia causing hepatic encephalopathy consisting of headaches and seizures. His medical history was significant for liver cirrhosis caused by infection with hepatitis B, end stage kidney disease due to membranoproliferative glomerulonephritis, for which the patient is on hemodialysis at the rate of 3 sessions per week. The patient went on to develop portal hypertension and ascites requiring frequent hospital admissions and treatment with diuretics. He was on a low-protein and low-sodium diet. While on oral furosemide 250 mg daily and spironolactone 100 mg daily, his diuresis was adequate. Before referral, this patient had already suffered 4 episodes of tonic clonic seizures. His 1st hemodialysis session in our training was on January 10, 2023, he was given daily hemodialysis sessions with daily blood tests, including immediate pre and post dialysis ammonia. Pre-dialytic ammonia levels varied between 103 and 161 µmol/l, as well as post-dialytic ammonia levels varied between 24 and 37 µmol/l, knowing that the normal value of laboratory ammonia varied between 10 and 51 µmol/l. The evolution was marked by a clear clinical improvement, with a disappearance of the seizures without a significant drop in ammonia after 14 daily hemodialysis sessions. Serum creatinine and urea declined, and sodium increased. Accumulation of ascites slowed and the patient could eat and sleep normally and was again independent with self care. The patient returned home on January 24. Conclusion This study shows the interest of daily hemodialysis sessions as a potential treatment for hyperammonemia in a patient on chronic intermittent hemodialysis, with a portosystemic shunt. The interest lies in the fact that the improvement is mainly clinical than biological (persistent hyperammonemia).