Nephrology Unit Research Articles

Monoclonal gammopathy is present in one fourth of patients undergoing renal biopsy but is pathogenic only in half of them.

About 4-7% of renal biopsies show a monoclonal gammopathy-related nephropathy, such as AL amyloidosis, cast nephropathy, or light chain deposition disease. Both a high prevalence and a causal role of monoclonal gammopathy have been observed in patients with C3 glomerulopathy or thrombotic microangiopathy, although a definitive causative role cannot be established in most cases (potentially monoclonal gammopathy-related nephropathies). A coexisting monoclonal gammopathy has been identified in many cases of nephropathy without a defined causative role (monoclonal gammopathy-unrelated nephropathies). The aim of this study was to investigate the prevalence and distribution of monoclonal gammopathy in patients who underwent arenal biopsy and assess its possible causal role in nephropathies not ordinarily related to monoclonal gammopathy. In our single-center retrospective observational study, we considered patients who underwent native kidney biopsy from 2009 to 2023 at the Nephrology Unit, Careggi University Hospital, Florence (Italy)and for whom a complete monoclonal gammopathy workup (serum electrophoresis, serum and urinary immunofixation, serum free light chains) was available. Overall, 827 patients were included: 208 (25%) had a monoclonal gammopathy: in104 cases the monoclonal gammopathywasunrelated tothekidney disease; 87 subjects showed renal pathology related to monoclonal gammopathy (monoclonal gammopathy-related nephropathies). Patients with thrombotic microangiopathy and C3 glomerulopathy (potentially monoclonal gammopathy-related nephropathies) exhibited a prevalence of monoclonal gammopathy > 30%. In a subgroup of diagnoses (e.g. tubulointerstitial nephritis, membranoproliferative glomerulonephritis) a possible causal and/or prognostic role of a concomitant monoclonal gammopathy may be hypothesized. In our cohort, one fourth of patients undergoing arenal biopsy had a monoclonal gammopathy, although in half of them the monoclonal gammopathy did not have a causative rolein the kidney disease.Hence, it is impossible to conclude that a monoclonal gammopathy in the context of renal disease equates to a causal association without performing a renal biopsy because of the high frequency of monoclonal gammopathy in patients undergoing a kidney biopsy.

Study protocol: The effect of a Medication Coordinator on the quality of patients' medication treatment (MEDCOOR)-Randomized controlled trial.

Patients' safety can be compromised in the transition of care between healthcare sectors. Optimal information flow across healthcare sectors and individualized medication treatment tailored to each patient is vital to prevent adverse events like drug-related problems. When medication changes are made during hospitalization, it is essential to ensure that the relevant general practitioner (GP) is included in the communication chain. This randomized controlled trial examines the effect of a Medication Coordinator who facilitates medication reviews in close collaboration with patients using My Medication Plan. Patients in the intervention group receive the medication review in combination with including suggested medication amendments documented in their electronic discharge letter send, which is sent to their GP. The patients randomized to the control group receive standard care by the ward staff. Seventy patients from the Endocrinology and Nephrology Unit at the Hospital Sønderjylland will be included in the intervention and control groups, respectively. The primary outcome is the proportion of potentially inappropriate medications. Secondary outcomes include patient-reported outcomes, i.e., quality of life and medication burden. Additional outcomes include the patient's medication risk score, whether the patient is readmitted, and whether the patient has contacted the staff at the hospital unit after the hospital discharge. The framework for complex intervention is applied, because it allows flexibility and adaption in meeting patients' needs by implementing tailored, possibly complex interventions in different healthcare settings. This project will examine a particular piece in the puzzle of the complexity of conducting a medication review and communication of suggested medication amendments to the patients, healthcare at the hospital, and the GP. Hopefully, this can contribute to a reduction in the risk of potentially inappropriate post-hospital medication usage. Trial registration: The study has been registered at ClinicalTrial.gov with the registration number: NCT06383364. https://clinicaltrials.gov/study/NCT06383364.

C3 glomerulopathy in children: a European longitudinal study evaluating outcome.

C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce. Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6months, 12months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3mg/mg with normal eGFR. Lack of remission was defined as non-response. A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8years and of 78% at 7years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR. Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.

The European Rare Kidney Disease Reference Network

Rare kidney diseases encompass awide range of congenital, inherited and acquired conditions. Two million Europeans are affected by rare kidney diseases. The European Rare Kidney Disease Reference Network (ERKNet) aims to improve the clinical management of patients with these diseases. ERKNet encompasses 95highly specialized adult and pediatric nephrology units at 72sites in 24European Union (EU) member states, as well as agroup of patient advocates (European Patient Advocacy Group, ePAG). ERKNet centers care for more than 65,000 rare kidney disease patients and pursues avariety of activities. An online consultation service helps improve the management of complex cases. Expert working groups develop clinical practice guidelines for individual groups of rare kidney diseases. In a3-year postgraduate program, junior physicians at ERKNet centers are trained by experts in the diagnosis and treatment of rare kidney diseases through webinars and case-based eLearning modules. Information brochures and online texts on rare kidney diseases for patients, relatives and the general public are produced and disseminated. Clinical research is supported by aEuropean Registry for Rare Kidney Diseases (ERKReg), which provides important information on demographics and disease progression and facilitates the identification of patient cohorts for therapeutic studies. In addition, the registry provides clinical performance statistics of reference centers and allows benchmarking to promote the harmonization and standardization of care for rare kidney disease patients across Europe.

Percutaneous Ultrasound-Guided Renal Biopsy in Greek Children: 15 Years of Experience at a Single Center.

Percutaneous ultrasound-guided renal biopsy (PRB) is a key element for diagnosis and management of several renal pathologies. We aimed to lay out the experience of our pediatric nephrology unit performing PRBs. The rationale and findings of these biopsies, safety issues and considerations of the extracted data are going to be analyzed. A retrospective study was conducted from 2008 to 2023 based on the review of the medical records of pediatric patients who underwent PRBs. In total, 216 kidney biopsies in 206 patients were performed: 115 (53.2%) during the 2008-2015 period and 101 (46.8%) during the 2016-2023 period. The most frequent clinical indication for PRBs was nephritic syndrome followed by nephrotic syndrome, observed in 84 (40.8%) and 72 (34.9%) patients, respectively. The predominant diagnosis was minimal change disease (MCD) (23.3%), followed by focal segmental glomerulosclerosis (FSGS) (15%) equal to lupus nephritis (LN) (15%), and immunoglobulin A nepropathy (10.2%). Minor complications, such as subcapsular hematomas were observed in approximately 15% of patients while no therapeutic intervention was needed. This report is the first review of pathohistological data covering a pediatric population over a 15-year period in Greece and one of the largest in southeastern Europe, especially in the Balkan region. The main indication for a PRB was nephritic syndrome; however, MCD was the main histological diagnosis. This study emphasis the fact that PRBs constitute a safe and reliable method of diagnostic approach to kidney diseases in childhood and offers important information on therapeutic approaches as well as the prognosis of these patients.

Survey and Intervention Tools for Burnout in Dialysis Healthcare Staff

The psychological clinical activity (2019-present) addressed to patients and family members carried out in the Nephrology and Dialysis Unit of Chieti Hospital allowed to achieve various objectives: the support of the patient and her/his family in the process of adaptation to the disease and its therapy, and the support of the treatment team to guarantee and safeguard the relationship with patient. Here we report the training interventions to healthcare personnel, which aim to analyze the communication methods and defensive styles used in the relationship with the patient, to explore the emotional perceptions of the self that can influence the therapeutic relationship. Our results show that the psychological intervention to the healthcare personnel working in a nephrology/dialysis unit may represent an effective tool for preventing burnout and a resource for increasing motivation in performing the professional activity.

A Cross Sectional Study of the Prevalence of Retinopathy in Patients with Chronic Kidney Disease on Hemodialysis in a Tertiary Care Centre in Chengalpattu District, South India

Context: Many studies have reported varying proportions of retinopathy among cases with chronic kidney disease (CKD). Cases with severe grades of CKD were more likely to develop ocular complications, especially diseases which occurs due to small vessel pathology. Aim: To estimate the prevalence of various types of retinopathies in patients with CKD undergoing hemodialysis in a tertiary care centre in South India. Settings and Design: The outpatient Department of Ophthalmology and Hemodialysis unit of Nephrology, Karpaga Vinayaga Institute of Medical Sciences from January 2022 to June 2023 A cross-sectional hospital-based observational study in a low resource setting. Methods and Material: The study consists of 28 patients who were known cases of CKD on hemodialysis. Patients underwent complete ocular evaluation and findings were noted. The pre hemodialysis values of blood urea and serum creatine, hemoglobin, liver function tests and vital parameters were also noted. Data analysis was done utilising version 20 of Statistical Packages for the Social Sciences (SPSS). Results: 56 eyes of 28 patients on hemodialysis were studied. The mean age was 53 years + 10.9 with a male preponderance. 31.6% had retinopathy of which 26.3% had hypertensive retinopathy and 5.3% had diabetic retinopathy. This is contrary to the previous studies that have reported a higher incidence of diabetic retinopathy in patients with end stage CKD. Hypertension resulted in CKD earlier than diabetes. Conclusion: The results emphasize the importance of frequent eye examination in patients with CKD to identify retinopathy earlier, thus potentially averting permanent vision impairment.

Exposure to microplastics via hemodialysis: differences between women and men

Abstract Background Hemodialysis (HD) may contribute to microplastics (MPs) burden in dialysis patients. The aim of the study was to estimate any differences in exposure to MPs between cases (on dialysis patients) and controls (not on dialysis patients), investigating any sex differences. Methods We organized a case-control study. Both cases and controls were recruited at the Policlinic “G. Rodolico-San Marco” Hospital Nephrology Unit. Preparation and determination of MPs smaller than 10 µm in blood samples were carried out using a patented method (Italian Patent n. 102018000003337-07 March 2018, European Patent n.3788344 20 July 2022) and SEM-EDX electron microscopy analysis, respectively. Results A convenience sample was recruited including 15 dialysis (60.0% women) and 10 not on dialysis patients (60.0% women) with a median (IQR) age of 77.0 (59.0-83.0) in dialysis patients and 65.5 (49.3-79.0) in those not on dialysis. No significant differences were found between cases and controls on the consumption of bottled water (100% vs 80%, p = 0.07), bagged foods (93.3% vs 70%, p = 0.12) unlike the use of drugs (100% vs 70%, p = 0.02). Women in general declared a greater use of drugs than men (F 93.3%, M 80.0%, p = 0.32). Dialysis patients had higher MPs levels than non-dialysis patients [9132(4896-15847) vs 3554(1661-7340), p-value=0.06]. In particular, female dialysis patients had values comparable to males [9132 (4665-17205) p/d vs men: 10438 (3672-16699) p/d] while non-dialysis female patients had higher levels than males [3274 (1681- 7814) p/g vs 1661 (1661-3354) p/g]. No difference was detected about the diameter of the MPs (IQR) [dialysis patients 4.28 (3.72-4.66) µm vs non-dialysis patients [4.67 (4.15-5.08) µm]. Conclusions Future studies must focus on studying the relationship between exposure to MPs and their levels in dialysis patients as well as investigating the differences between men and women and the possible consequences of dialysis as a further source of exposure. Key messages • On dialysis patients, both men and women, had higher MPs exposition compared to not on dialyzed ones. • Not on dialysis women had higher MPs levels than not on dialysis men.

Oral health and chronic kidney disease: Findings from a descriptive survey in Lagos, Nigeria.

Background: Chronic kidney disease (CKD) is a global public health problem, characterized by a progressive and irreversible decline in renal functions, and affecting up to 22.3% of Nigerians. Oral diseases are increasingly recognized to have a bidirectional relationship with CKD, contributing to systemic inflammation, infections, protein-energy wasting, and atherosclerotic complications. Given this interconnectedness, we aimed to explore the relationship between CKD and oral conditions among LASUTH patients to better understand and address these co-occurring health issues. Methods: This descriptive observational study was conducted in the nephrology unit of LASUTH. Patients aged &gt;20 years with a diagnosis of CKD were enlisted and a validated questionnaire with the modified WHO oral health status proforma was utilized to record clinical data: Estimated Glomerular Filtration Rate (EGFR), End-Stage Renal Disease (ESRD), Creatinine and Urinalysis from medical records and oral examination findings, Oral Hygiene Index-Simplified (OHI-S), Gingival Index (GI), Community Periodontal Index of Treatment needs (CPITN), oral malodour, and Oral lesions. Multivariate logistic regression analysis was conducted with adjustments for covariates like age, alcohol use, and BMI to determine the association between oral diseases and CKD. The significance level (p) was set at 0.05, with a confidence interval (CI) of 95%. Results: A total of 200 patients at different stages of CKD were included. The age range was 22–83 years. The majority were aged 31-60 years (mean age 55.22 ± 14.35 years); 69% were males; 7% were current smokers while 12% currently drank alcohol; 27% had eGFR&lt; 15 mL/min/1.73m2 (ESRD); 27% had Creatinine values &gt;1.3mg/dl; prevalence of Gingivitis was 72%; periodontitis was 14% (CPI score ≥3: Pocket depth of ≥4 mm); and poor OHI was 22%. The commonest oral lesions were white lesions 2%, candidiasis 4.5%, hyperpigmentation 22%, oral ulcers 5%, hemorrhagic lesions 12.5%, and Oral malodor 74.5%. Multiple logistic regression analysis revealed significantly increased odds of ESRD among uneducated respondents (OR 1.194; CI: 0.832-1.713); those &gt;60years (OR: 1.129; CI:0.436-2.923); current alcohol drinkers (OR:1.125; CI: 0.281-4.409); and smokers (OR:2.328; CI: 0.27-20.289). The odds of low stimulated saliva flow rate (OR: 1.181; CI:0.768-4.512), oral malodor (OR:1.093; CI: 0.763-1.565), and periodontal disease (OR: 1.242; CI: 0.698-2.209) were also significantly higher among those with ESRD. Conclusion: Individuals with CKD and ESRD may have a higher prevalence of oral diseases and conditions. There were significantly increased odds of ESRD among uneducated respondents, those over 60, current alcohol consumers, and smokers. Additionally, the odds of low stimulated saliva flow rate, oral malodor, and periodontal disease was also significantly higher among those with ESRD. This relationship may also be bidirectional, highlighting the need for appropriate oral self-care and regular dental-care utilization in patients with renal disease. The findings underscore the need for integrated healthcare approaches that consider both oral and renal health, providing a foundation for further research and targeted interventions.

Pulmonary-Renal Syndrome in the Elderly: Immunosuppressive Treatment – Case Report

Pulmonary-renal syndrome is called alveolar hemorrhage accompanied by glomerulonephritis. The above condition can occur as a manifestation of ANCA-associated granulomatosis with vasculitis. The course of the disease varies from mild, limited inflammation to rapid progression with multi-organ changes that can be life-threatening. The mainstay of therapy is immunosuppression - both in the acute phase of the disease and after clinical improvement. Unfortunately, common causes of death in addition to complications of the disease include side effects of immunosuppressive treatment. A 72-year-old man is transferred to the Nephrology Unit for acute kidney injury of unclear etiology. On the basis of clinical examination, laboratory and imaging studies, a diagnosis of systemic small vessel inflammation with c-ANCA occupying the kidneys, lungs and intestines was made. Due to acute respiratory failure, the patient was transferred to the ICU, where renal replacement therapy, plasmapheresis, steroid pulses and cyclophosphamide were administered. After clinical improvement, it was decided to continue cyclophosphamide therapy and monitor blood IgG levels. Unfortunately, 4 months later, the patient died of multiple organ failure caused by septic shock of bacterial etiology. In the described case, age and comorbidities were the biggest risk factors for infectious complications of immunosuppression. The population is aging, so it is necessary to analyze the current standards of immunosuppressive treatment to improve the prognosis of the elderly.

Human Immunodeficiency Virus Associated Neuropathy Perinatally Infected HIV Children In Calabar, Nigeria. Clinical Features, Treatment and Outcome

Background Renal disease is increasing being recognized as a significant cause of morbidity and mortality in HIV positive patients. Human Immunodeficiency virus associated nephropathy (HIVAN) is the most common type of HIV related renal disease and rapidly progress to end stage renal disease(ESRD). There is paucity of report on true prevalence of HIVAN in African. This study was aimed at determining the prevalence of HIVAN, clinical features, treatment and outcome, in Calabar Nigeria. Methods/Subject This was a retrospective review of all renal patients managed by Pediatric nephrology unit of University of Calabar Teaching Hospital from January 2016 to December 2022 enrolled in the Renal register. During the period of study 215 patients had renal diseases out of which 15 had HIVAN. The following information were extracted from the HIVAN patients; Demographics and clinical data as well as mode of transmission, laboratory investigations, renal Ultrasound scan, treatment and Outcome were obtained and analyzed using SPSS version 20. Results There were 215 cases of renal diseases seen during the study period of which 15 had HIVAN giving a prevalence of 6.9% There were 5 males and 10 females giving a ratio of 1:2, age range 69-192months. with a mean age of 127±43.7months All received HAART and had acquired HIV infection through vertical transmission. 8 (53%) of patient were asymptomatic with 6 (40%) presenting with both legs and facial swelling. Nephrotic range proteinuria was a common presentation seen in 40% of the patients and 5(33.3%) had hypertension. Only 4 (33%) had eGFR&lt;60 ml/min/1.73m2 and 2(16%) had ESRD. Two were lost to follow-up and 4 (26%) died with two requiring dialysis. Conclusion HIVAN is common in patients with renal diseases and there is need to monitor patient at initiation of HAART and at risk patients with low CD4+ count and exposure to nephrotoxic HAART.

Project on Geriatric Assessment in Nephrology Unit: Evaluation of Frailty and Vulnerability, Physical Capacity, and Kidney Function

Project on Geriatric Assessment in Nephrology Unit: Evaluation of Frailty and Vulnerability, Physical Capacity, and Kidney Function

Clinical Characteristics and Outcomes of Young Patients with Thrombotic Microangiopathy at Ain Shams University Children’s Hospital

Abstract Introduction Thrombotic microangiopathies (TMA) is a life-threatening condition that requires urgent management. Survival of patients has improved greatly over the past two decades with improved supportive care and other therapeutic options. Objectives: to describe the clinical features and outcomes of patients with TMA at Ain Shams University Children’s Hospital. Methods In a retro-prospective study, we included patients diagnosed with TMA in the Pediatric Hematology and Pediatric Nephrology Units at Ain Shams University Children’s Hospital from 2009 through 2023. Results Thirty-three patients were enrolled, they were 15 females and 18 males with age ranges from 0.31 to 13.9 years and median (IQR) of 4.3 (1.4 – 9.0) years. Almost half of our patients had infection related hemolytic uremic syndrome (HUS) (51.5%), 30.3% had atypical HUS (aHUS), 9.1% had congenital thrombotic thrombocytopenic purpura (cTTP), while 9.1% were diagnosed with immune-mediated TTP (iTTP). Renal manifestations were the most common presenting symptoms (90.9%), hematological manifestations were present in (66.7%), while neurological manifestation were evident in (36.4%) of patients. Conservative management was the most commonly used approach (87.9%),while (75.8%) received plasma infusion and (69.7%) needed renal replacement therapy. On studying the outcome of our TMA cases, 33.3% had residual hematological manifestations, meanwhile 60.6% left with chronic kidney disease. Mortality was reported in 18.2% during the whole follow-up period. Conclusion infection-related HUS was the most common type of TMA in our cohort, and most patients were managed conservatively with disease resolution at 3 and 6 months follow up.

The impact of life events on child and adolescent psychiatry consultations

Purpose: Children are exposed to many adverse life events, and this may cause psychiatric disorders. In this study, we aimed to investigate the effects of two different life events, the COVID-19 pandemic and the earthquake, on the consultations requested. Materials and Methods: 1268 consultations requested from the child and adolescent psychiatry department between March 2019 and March 2023 were reviewed from hospital records. The specified period was divided into four distinct parts with one-year intervals: pre-pandemic, the first year of the pandemic, the second year of the pandemic, and post-pandemic. The age, gender, diagnoses of the cases, the department requesting consultation and the reason for consultation were recorded. Results: There were statistically significant differences between periods in terms of departments requested consultation, consultation reasons, and diagnoses. Compared to previous periods, a decrease was found in pediatric emergency and general pediatric clinic consultations in the post-pandemic period. In contrast, an increase was seen in nephrology and intensive care unit department consultations because of earthquakes. In the first year of the pandemic, more consultations were requested from the hematology &amp; oncology department compared to other years. While the rate of consultations requested for suicide attempts decreased in the post-pandemic period, consultations for psychiatric assessment increased. Conclusion: Consultation-liaison psychiatry, which is at the center of interdisciplinary interaction, maintains its importance in the prevention and early diagnosis of psychological symptoms that can develop due to both organic events and the effect of trauma itself after the life events.

Clinical evaluation of a machine learning-based early warning system for patient deterioration.

The implementation and clinical impact of machine learning-based early warning systems for patient deterioration in hospitals have not been well described. We sought to describe the implementation and evaluation of a multifaceted, real-time, machine learning-based early warning system for patient deterioration used in the general internal medicine (GIM) unit of an academic medical centre. In this nonrandomized, controlled study, we evaluated the association between the implementation of a machine learning-based early warning system and clinical outcomes. We used propensity score-based overlap weighting to compare patients in the GIM unit during the intervention period (Nov. 1, 2020, to June 1, 2022) to those admitted during the pre-intervention period (Nov. 1, 2016, to June 1, 2020). In a difference-indifferences analysis, we compared patients in the GIM unit with those in the cardiology, respirology, and nephrology units who did not receive the intervention. We retrospectively calculated system predictions for each patient in the control cohorts, although alerts were sent to clinicians only during the intervention period for patients in GIM. The primary outcome was non-palliative in-hospital death. The study included 13 649 patient admissions in GIM and 8470 patient admissions in subspecialty units. Non-palliative deaths were significantly lower in the intervention period than the pre-intervention period among patients in GIM (1.6% v. 2.1%; adjusted relative risk [RR] 0.74, 95% confidence interval [CI] 0.55-1.00) but not in the subspecialty cohorts (1.9% v. 2.1%; adjusted RR 0.89, 95% CI 0.63-1.28). Among high-risk patients in GIM for whom the system triggered at least 1 alert, the proportion of non-palliative deaths was 7.1% in the intervention period, compared with 10.3% in the pre-intervention period (adjusted RR 0.69, 95% CI 0.46-1.02), with no meaningful difference in subspecialty cohorts (10.4% v. 10.6%; adjusted RR 0.98, 95% CI 0.60-1.59). In the difference-indifferences analysis, the adjusted relative risk reduction for non-palliative death in GIM was 0.79 (95% CI 0.50-1.24). Implementing a machine learning-based early warning system in the GIM unit was associated with lower risk of non-palliative death than in the pre-intervention period. Machine learning-based early warning systems are promising technologies for improving clinical outcomes.

Effects of SLC34A3 or SLC34A1 variants on calcium and phosphorus homeostasis.

Variants in SLC34A1 and SLC34A2 genes, which encode co-transporters NaPi2a and NaPi2c, respectively, can lead to hypophosphatemia due to renal phosphate loss. This condition results in hypercalcitriolemia and hypercalciuria, leading to formation of kidney stones and nephrocalcinosis. Phenotype is highly variable. Management includes hyperhydration, dietary modifications, and/or phosphate supplementation. Thiazides and azoles may be used, but randomized studies are needed to confirm their clinical efficacy. We conducted a retrospective study in the pediatric nephrology unit at Grenoble University Hospital from January 2010 to December 2023. The study aimed to describe clinical and biological symptoms of patients with confirmed SLC34A1 and SLC34A3 gene variants and their outcomes. A total of 11 patients (9 females) from 6 different families had variants in the SLC34A1 (5 patients) and SLC34A3 (6 patients) genes. Median age at diagnosis was 72 [1-108] months. Average follow-up duration was 8.1 ± 4.5years. Presenting symptom was nephrocalcinosis (4 cases), followed by renal colic (3 cases). At diagnosis, 90% of patients had hypercalciuria and 45% had hypercalcitriolemia. Management included hyperhydration and dietary advice. All patients showed favorable outcomes with normal growth and school attendance. One patient with an SLC34A3 variant showed regression of nephrocalcinosis. Kidney function remained normal. Clinical and biological manifestations of SLC34 gene variants are highly variable, even among siblings; therefore, management must be personalized. Hygienic and dietary measures (such as hyperhydration, a low sodium diet, and age-appropriate calcium intake) result in favorable outcomes in most cases. Use of azoles (e.g., fluconazole) appears to be a promising therapeutic option.

Acute kidney injury: the experience of a tertiary center of Pediatric Nephrology.

Acute kidney injury (AKI) is an abrupt deterioration of kidney function. The incidence of pediatric AKI is increasing worldwide, both in critically and non-critically ill settings. We aimed to characterize the presentation, etiology, evolution, and outcome of AKI in pediatric patients admitted to a tertiary care center. We performed a retrospective observational single-center study of patients aged 29 days to 17 years and 365 days admitted to our Pediatric Nephrology Unit from January 2012 to December 2021, with the diagnosis of AKI. AKI severity was categorized according to Kidney Disease Improving Global Outcomes (KDIGO) criteria. The outcomes considered were death or sequelae (proteinuria, hypertension, or changes in renal function at 3 to 6 months follow-up assessments). Forty-six patients with a median age of 13.0 (3.5-15.5) years were included. About half of the patients (n = 24, 52.2%) had an identifiable risk factor for the development of AKI. Thirteen patients (28.3%) were anuric, and all of those were categorized as AKI KDIGO stage 3 (p < 0.001). Almost one quarter (n = 10, 21.7%) of patients required renal replacement therapy. Approximately 60% of patients (n = 26) had at least one sequelae, with proteinuria being the most common (n = 15, 38.5%; median (P25-75) urinary protein-to-creatinine ratio 0.30 (0.27-0.44) mg/mg), followed by reduced glomerular filtration rate (GFR) (n = 11, 27.5%; median (P25-75) GFR 75 (62-83) mL/min/1.73 m2). Pediatric AKI is associated with substantial morbidity, with potential for proteinuria development and renal function impairment and a relevant impact on long-term prognosis.

Prevalence, risk factors, and treatment of anemia in hospitalized older patients across geriatric and nephrological settings in Italy

Anemia is a common but often underdiagnosed and undertreated geriatric syndrome in hospitalized older patients. In this retrospective multicenter study, we aimed at characterizing the prevalence, risk factors, diagnostic and treatment approach to anemia in older patients admitted to acute care hospitals, focusing on differences between nephrology and geriatrics units. Prevalence and risk factors for anemia, diagnostic inertia (lack of iron, vitamin B12, and folate status assessment), replacement inertia (omitted treatment with iron, vitamin B12 or folic acid), and erythropoiesis-stimulating agents (ESA) inertia were explored. 1963 patients aged 82.7 (6.8) years were included in the study; 66.7% of the study population had anemia; among anemic patients, diagnostic inertia and replacement inertia were common with rates of 22–31% and 50–87%, respectively; omitted treatment with ESA affected 67.2% of patients and was more prevalent in geriatric units. In most cases, patients with ESA inertia were not routinely screened for iron tests. COPD, cancer, eGFR 45–60 ml/min were associated with increased tendency to ESA inertia. In conclusion, anemia had a high prevalence in older patients discharged from acute care units, but it is often underdiagnosed and undertreated.

Malformative uropathies in children: epidemiological, clinical, therapeutic and evolutive aspects in an ivoirian hospital setting

Congenital anomalies of the kidney and the urinary tract are a major cause of chronic kidney failure in children. Prevalence in paediatrics varies according to studies. The data being rare in the ivorian context, this study aims to describe these defects' epidemiological, clinical, therapeutic and evolutionary aspects in children in a reference hospital setting. We performed a retrospective and descriptive study held in the Yopougon's Teaching Hospital Pediatric Nephrology Unit from December 1st 2008 to December 31st 2020. It involved 152 children aged 0 to 15 years, admitted to the unit, with a congenital anomaly of the kidney and the urinary tract diagnosed with radiology. Socio-demographic characteristics: congenital anomalies of the kidney and urinary tract's prevalence was 11%. The median age at admission was 36 months. The median age at diagnosis of malformative uropathy was 17.5 months. The sex ratio (M/F) was 2.3. Clinical characteristics: posterior urethra valves were the most common malformative uropathy (38%). Malformative uropathy was associated with other defects in 4% of cases. The antenatal diagnosis involved 24% of patients. The average gestational age of discovery was 32 weeks of amenorrhea. In the postnatal period, abdominal pain was the main circumstance for discovery (39%). Therapeutic characteristics: surgery was indicated in 58% of patients and performed in 64% of cases. Evolutionary characteristics: evolution was better in patients who had received surgical treatment (asymptomatic in 83% of cases, occurrence of urinary tract infection in 35% of cases and chronic renal failure in 23% of cases). 72% of the study population was lost. In Côte d'Ivoire, malformatives uropathies are late-discovered and are dominated by posterior urethra valves. Knowledge and management of these renal and urinary tract defects deserve to be improved through the development of antenatal diagnosis and training of practitioners in early recognition of clinical signs. The high rate of lost patients must be reduced by the implementation of an active patients' follow-up system.

COVID-19 in Egyptian Children on Regular Hemodialysis: Experience of a Single Center

Abstract Background COVID-19 is clinically characterized by high rates of transmission, and pathological abnormalities. Pediatric patients with CKD are at high risk of COVID-19 infection &amp; morbidity. Aim of the Work We aimed at studying the rate of infection by COVID-19 in pediatric patients following up at our Pediatric dialysis &amp; Nephrology unit, Ain Shams University. Patients and Methods This retrospective study was conducted over 12 months from 1st of March 2020 to end of February 2021 on 70 pediatric patients with chronic kidney disease stage 5 on regular hemodialysis (CDK5d). Results The mean age &amp; dialysis duration of our studied patients was 11.89 (±3.01) years &amp; 2.17(±1.2)years respectively, where the incidence of COVID-19 infection confirmed by PCR on nasal swab sampleswas 7%. The younger patients had a higher rate of COVID-19 infection than older ones (p = 0.05). Using public transportation during past 14 days before getting COVID-19 infection was significant in COVID-19 positive patients (p = 0.01). The common associated symptoms in our patients were fever, joint ache, muscle ache, loss of appetite, headache, shortness of breath, fatigue, cough, sore throat, nausea, loss of smell or taste, diarrhea, vomiting. Conclusion COVID-19 infection among our CDK5d patients was not uncommon with incidence of 7%, where younger patients were infected more frequently than older patients The common associated symptoms in our patients were fever, joint ache, muscle ache, loss of appetite, headache, shortness of breath, fatigue, cough, sore throat, nausea, loss of smell or taste, diarrhea, vomiting.