An international, multi-center study evaluated rituximab therapy in childhood steroid-resistant nephrotic syndrome.

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was complete/partial remission (CR/PR) as defined by IPNA/KDIGO guidelines. Secondary outcomes included kidney failure and adverse events. Two-hundred-forty-six children (mean age, 6.9 years; 136 boys; 57% focal segmental glomerulosclerosis, FSGS) were followed a median of 32.4 months after rituximab. All patients were in non-remission before rituximab. (146 and 100 children received CNIs for 6 month or more or under 6 months before rituximab, respectively). In patients with CNI-resistant SRNS, the remission rates (CR/PR) at 3-, 6-, 12- and 24-months were 26% (95% confidence interval 19.3-34.1), 35.6% (28.0-44.0), 35.1% (27.2-43.8) and 39.1% (29.2-49.9), respectively. Twenty-five patients were in PR at 12-months, of which 22 had over 50% reduction in proteinuria from baseline. The remission rates among children treated with CNIs under 6 months before rituximab were 42% (32.3-52.3), 52% (41.8-62.0), 54% (44.3-64.5) and 60% (47.6-71.3) at 3-, 6-, 12-, and 24-months. Upon Kaplan-Meier analysis, non-remission and PR at 12-months after rituximab, compared to CR, were associated with significantly worse kidney survival. Adverse events occurred in 30.5% and most were mild. Thus, rituximab enhances remission in a subset of children with SRNS, is generally safe and CR following rituximab is associated with favorable kidney outcome.

Daily compared with alternate-day levamisole in pediatric nephrotic syndrome: an open-label randomized controlled study.

BACKGROUND: Levamisole is less expensive and has a better toxicity profile compared to other steroid sparing agents used in nephrotic syndrome. It has a plasma half-life of 2.0 to 5.6 hours, but is conventionally administered on alternate days. We aimed to assess whether daily levamisole is safe and more effective than standard alternate-day therapy in maintaining remission in children with frequently relapsing or steroid-dependent nephrotic syndrome (FR/SDNS). METHODS: An open-label randomized controlled trial was conducted in children with FR/SDNS. Group A received daily while Group B received alternate-day levamisole (2-3 mg/kg/dose) for 12 months. Prednisolone was tapered off by 3 months. Patients were monitored for relapses, further steroid requirement, and adverse effects. RESULTS: A total of 190 children with FR/SDNS (94 in Group A and 96 in Group B) were analyzed. Sustained remission for 12 months was observed in 36% of Group A and 27% of Group B patients (p = 0.18). Numbers completing 12 months in the study were 67% in Group A and 56% in Group B (p = 0.13). Time to first relapse, persistent FR/SDNS, and withdrawal due to poor compliance were statistically similar in both groups, while relapse rate and cumulative steroid dosage were significantly lower in Group A compared to Group B (p = 0.03 and p = 0.02, respectively). The incidence of adverse effects was comparable in both groups, with reversible leucopenia and hepatic transaminitis being the commonest. CONCLUSIONS: Daily levamisole therapy was not superior to alternate-day therapy in maintaining sustained remission over 12 months. Nevertheless, relapse rate and cumulative steroid dosage were significantly lower without increased adverse effects.

Antineutrophil cytoplasmic antibody in children with nephrotic syndrome treated with levamisole: a cross-sectional cohort study.

BACKGROUND: Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning. METHODS: Observational cross-sectional study wherein children aged 2 to 18 years with frequently relapsing/steroid dependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12 months were tested for ANCA. RESULTS: A total of 210 children (33% female), median age of 7.3 (IQR: 5.6-9.6) years, and a median duration of levamisole exposure of 21 (IQR: 15-30) months were tested. ANCA was positive in 18% (n = 37): 89% (n = 33) perinuclear ANCA (pANCA), 3% (n = 1) cytoplasmic ANCA (cANCA), and 8% (n = 3) both. Of ANCA-positive children, none had reduced eGFR or abnormal urinalysis. The majority of these children were asymptomatic (81%, n = 30). Rash was more common among ANCA-positive children [6/37 (16%) vs. 3/173 (2%), p = 0.0001]. On multivariate analysis, higher age (OR = 1.02, [95th CI: 1.01 to 1.03], p = 0.007) and longer duration of levamisole exposure (OR = 1.05, [95th CI: 1.02 to 1.08], p = 0.0007) were associated with ANCA positivity. Levamisole was stopped in ANCA-positive children with the resolution of any clinical manifestations if present. Repeat ANCA testing was performed in 54% (20/37), and all were ANCA negative by 18 months. CONCLUSIONS: Children with FRNS/SDNS on longer duration of levamisole were associated with increasing prevalence of ANCA positivity, but most of these children were clinically asymptomatic. Prospective studies are required to determine the chronology of ANCA positivity and its clinical implication.

Human exposure to uranium through drinking water and its detrimental impact on the human body organs.

Human exposure to high concentrations of uranium is a major concern due to the risk of developing numerous internal organ malignancies over time. In addition to the numerous attributes of uranium in the nuclear power industry, the radiological characteristics and chemical toxicity of uranium present a substantial risk to human health. This study aims to evaluate potential negative health impacts associated with the ingestion of uranium through drinking water in the Noida and Greater Noida region within the Gautam Buddha districts of Uttar Pradesh (India), due to extreme industrial revolution in this geological location. The mean concentration of uranium in drinking water of the examined area was estimated to range from 0.23 to 78.21 µg l-1. The hair compartment biokinetic model is used to estimate the retention and radiological doses of uranium in distinct organs and tissues. Studies on time-dependent factors revealed variations in uranium retention, with lower levels observed in the Gastrointestinal Tract (GIT) region and higher levels on cortical bone surfaces causes the skeletal deformities. The kidney, liver, and other soft tissues (OST) exhibited a non-saturation pattern in the retention of uranium via exposure of drinking water. The age-wise non-carcinogenic and carcinogenic doses were estimated for the health hazards studies. The outcome of this study will be useful for water resource management authorities to supply safe potable water to the local residents.

The clinical relevance of native vitamin D in pediatric kidney disease.

Hypovitaminosis D has been reported to be common in chronic kidney disease (CKD) as well as in proteinuric disorders. We reviewed available evidence to assess clinically relevant effects of low vitamin D status and native vitamin D (NVD) therapy, in pediatric renal diseases. Online medical databases were searched for articles related to vitamin D status, associations of hypovitaminosis D and effects of NVD therapy in kidney disease. Hypovitaminosis D was associated with worse skeletal, cardiovascular, inflammatory, and renal survival outcomes in CKD. Low serum 25 hydroxy-vitamin D (25[OH]D) levels correlated positively with glomerular filtration rate and negatively with serum parathyroid (PTH) levels. However, to date, evidence of benefit of NVD supplementation is restricted mainly to improvements in serum PTH, and biochemical 25[OH]D targets form the basis of clinical practice recommendations for NVD therapy. In nephrotic syndrome (NS) relapse, studies indicate loss of 25[OH]D along with vitamin D binding protein in urine, and serum total 25[OH]D levels are low. Preliminary evidence indicates that free 25[OH]D may be a better guide to the biologically active fraction. NVD therapy in NS does not show consistent results in improving skeletal outcomes and hypercalciuria has been reported when total 25[OH]D levels were considered as indication for therapy. NVD formulations should be regularised, and therapy monitored adequately to avoid adverse effects.

IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.

Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given.

Healthcare team communication training in the United States: A scoping review.

The purpose of this literature review was to identify interventions designed to improve healthcare team communication in the United States. We conducted a review of peer-reviewed, English-language articles describing interventions aimed at improving healthcare team communication. We analyzed articles that met pre-specified inclusion and exclusion criteria and characterized who is testing communication interventions, the rationale for testing, and ways of measuring effectiveness. We descriptively categorized the strength and types of study findings. Thirty articles were retained in our analysis. Most assessments were conducted by academic medical centers, the Veterans Health Administration, and teaching hospitals. Interventions sought to improve teamwork, patient safety, clinical outcomes, costs of care, and enhance provider job satisfaction and well-being. Intervention strategies included didactic lectures, simulation, Crew Resource Management, quality improvement, or a combination of these approaches. The vast majority employed a pre-post survey design and measured outcomes using participant feedback. Many assessments failed to utilize a social science theory or communication-specific measures. Interventions with the best training content were conducted at academic medical centers, used a pre-post design, and utilized statistical analysis to analyze results. While interventions for improving healthcare team communication are diverse and have uneven effectiveness, early markers of success merit continued development and assessment.

Paediatric nephrology in under-resourced areas.

BACKGROUND: Nearly 50% of the world population and 60% of children aged 0 to 14 years live in low- or lower-middle-income countries. Paediatric nephrology (PN) in these countries is not a priority for allocation of limited health resources. This article explores advancements made and persisting limitations in providing optimal PN services to children in such under-resourced areas (URA). METHODS: Medline, PubMed and Google Scholar online databases were searched for articles pertaining to PN disease epidemiology, outcome, availability of services and infrastructure in URA. The ISN and IPNA offices were contacted for data, and two online questionnaire surveys of IPNA membership performed. Regional IPNA members were contacted for further detailed information. RESULTS: There is a scarcity of published data from URA; where available, prevalence of PN diseases, managements and outcomes are often reported to be different from high income regions. Deficiencies in human resources, fluoroscopy, nuclear imaging, immunofluorescence, electron microscopy and genetic studies were identified. Several drugs and maintenance kidney replacement therapy are inaccessible to the majority of patients. Despite these issues, regional efforts with support from international bodies have led to significant advances in PN services and infrastructure in many URA. CONCLUSIONS: Equitable distribution and affordability of PN services remain major challenges in URA. The drive towards acquisition of regional data, advocacy to local government and non-government agencies and partnership with international support bodies needs to be continued. The aim is to optimise and achieve global parity in PN training, investigations and treatments, initially focusing on preventable and reversible conditions.

Whole-exome sequencing and variant spectrum in children with suspected inherited renal tubular disorder: the East India Tubulopathy Gene Study.

BACKGROUND: Inherited tubulopathies are a heterogeneous group of genetic disorders making whole-exome sequencing (WES) the preferred diagnostic methodology. METHODS: This was a multicenter descriptive study wherein children (< 18 years) with clinically suspected tubular disorders were recruited for molecular testing through WES. Multiplex ligation-dependent probe amplification (MLPA) and Sanger sequencing were done when required. Variants were classified as per American College of Medical Genetics 2015 guidelines and pathogenic (P)/likely pathogenic (LP) variants were considered causative. RESULTS: There were 77 index cases (male =73%). Median age at diagnosis was 48 months (IQR 18.5 to 108 months). At recruitment, the number of children in each clinical group was as follows: distal renal tubular acidosis (dRTA) = 25; Bartter syndrome = 18; isolated hypophosphatemic rickets (HP) = 6; proximal tubular dysfunction (pTD) = 12; nephrogenic diabetes insipidus (NDI) = 6; kidney stone/nephrocalcinosis (NC) = 6; others = 4. We detected 55 (24 novel) P/LP variants, providing genetic diagnoses in 54 children (70%). The diagnostic yield of WES was highest for NDI (100%), followed by HP (83%; all X-linked HP), Bartter syndrome (78%), pTD (75%), dRTA (64%), and NC (33%). Molecular testing had a definite impact on clinical management in 24 (31%) children. This included revising clinical diagnosis among 14 children (26% of those with a confirmed genetic diagnosis and 18% of the overall cohort), detection of previously unrecognized co-morbidities among 8 children (sensorineural deafness n = 5, hemolytic anemia n = 2, and dental changes n = 1) and facilitating specific medical treatment for 7 children (primary hyperoxaluria n = 1, cystinosis n = 4, tyrosinemia n = 2). CONCLUSION: WES is a powerful tool in the diagnosis and management of children with inherited tubulopathies in the Indian population. A higher resolution version of the Graphical abstract is available as Supplementary information.

Free vitamin D levels in steroid-sensitive nephrotic syndrome and healthy controls.

INTRODUCTION: Body stores of vitamin D are measured as "total" serum 25-hydroxy vitamin D (25(OH)D). Its largest component is protein bound and lost in urine in nephrotic syndrome (NS). Our study investigates whether "free" 25(OH)D levels are a better guide to bone health and need for vitamin D supplementation in patients with steroid-sensitive NS (SSNS). METHODS: A cross-sectional study was performed in children with SSNS and healthy controls. Blood was tested for albumin, creatinine, calcium, phosphate, ALP, total and free (by direct ELISA) 25(OH)D, iPTH, and urine for protein-creatinine ratio. RESULTS: Seventy-nine NS patients (48 in relapse, 31 in remission) and 60 healthy controls were included. The levels of total 25(OH)D were significantly different (lowest in NS relapse and highest in controls) (p < 0.001). Corrected calcium and phosphate levels were normal, and there were no differences in free 25(OH)D, ALP, or iPTH levels between groups. Only total and not free 25(OH)D correlated significantly and negatively with urinary protein creatinine ratios (rs = - 0.42 vs. 0.04). Free 25(OH)D values of 3.75 and 2.85 pg/ml corresponded to total 25(OH)D levels of 20 and 12 ng/ml, respectively, in healthy controls. CONCLUSION: These results confirm that total 25(OH)D levels are low in NS and related to degree of proteinuria. However levels of free 25(OH)D, ALP, and iPTH did not change in relapse or remission in comparison with healthy controls. Our results suggest that in proteinuric renal diseases, free 25(OH)D rather than total 25(OH)D levels should be used to diagnose vitamin D deficiency and guide therapy.

Synthesis and application of Zn/Ce bimetallic oxides for the decontamination of arsenite (As-III) ions from aqueous solutions.

Arsenic contamination has threatened water safety due to its high toxicity and carcinogenicity. Therefore, it is urgent and significant to develop simple and effective approach for dearsenification of drinking water. In present study, Zn/Ce bimetallic oxide particles of various atomic ratios were synthesized by sol-gel process and were applied for adsorption of arsenite from aqueous solutions. The Zn/Ce bimetallic oxide of atomic ratio Zn0.2:Ce0.05 shows better adsorption proficiency in comparison to their monometallic counterparts as well as synthesized bimetal oxides of other atomic ratios. Sorption behavior of arsenite on Zn/Ce bimetal oxide was investigated through batch experiments and optimum conditions were found to be pH = 7.5, adsorbent dose = 0.36 g/L, and contact time = 30 min. The arsenite adsorption data was explained by Langmuir isotherm model and maximum adsorption capacity found to reaching 88.49 mg/g at 318 K. Adsorption mechanism was interpreted using FTIR and XPS data, the former suggesting formation of bond between As(III)Zn/Ce oxide nanoparticles while, latter reveals presence of both As(III) and As(V) peak which further infer that some fraction of As(III) may be get oxidized to As(V) by O2 based on Ce3+ as electron mediation agent between As(III) and O2.

Process dynamic investigations and emission analyses of biodiesel produced using Sr-Ce mixed metal oxide heterogeneous catalyst.

The present study explores the feasibility of Sr-Ce based mixed metal oxides for its performance in transesterification reaction of waste cooking oil. The catalyst synthesis was carried out through gel combustion route and was characterized through several techniques including thermal analysis (TGA-DTA), X-ray diffraction (XRD), attenuated total reflectance based Fourier transform infrared spectroscopy (ATR-FTIR), high resolution scanning electron microscopy (HR-SEM) assisted with EDX, BET specific surface area and Hammett indicator basicity. The enhanced activity of the catalyst was investigated at pH 7.0 with Sr-Ce atomic ratio of 3:1 at 900 °C of calcination temperature. Influences of various process parameters on transesterification efficiency were carefully investigated. The experimental results demonstrated that maximum transesterification efficacy of 99.5% was achieved under optimized reaction conditions with catalyst dose of 2.0 wt %, oil-to-methanol ratio 1:14, reaction time 120 min, reaction temperature 65 °C and stirring speed of 700 rpm. For better interpretation of the process, the reaction rate was computed by employing pseudo-first and pseudo-second order kinetics model at varying reaction temperature (50 °C-75 °C). The transesterification data agreed well with pseudo-first order model with highest rate constant value of 2.5 × 10-3 min-1 was evaluated at 65 °C. Activation energy and frequency of the reaction was quantified from the Arrhenius expression as 17.04 kJ/mol and 9.92 min-1, respectively. Thermodynamic analysis of the reaction system suggests that the transesterification of the waste cooking oil followed endergonic reaction pathway. Synthesis of biodiesel was ascertained from the H1-NMR and FTIR analysis of the transesterified product, further, the physicochemical properties of the biodiesel were also compared with that of diesel fuel and the resultant values were found to be within ASTM limits. Reusability study was also conducted and it indicated that the catalyst can be easily regenerated and could be effectively reused up to four runs.

The effect of vitamin D and calcium supplementation in pediatric steroid-sensitive nephrotic syndrome.

BACKGROUND: Low serum levels of total 25-hydroxycholecalciferol (25(OH)D) occur in nephrotic syndrome (NS). We aimed to assess the effects of vitamin D3 and calcium supplementation on 25(OH)D levels, bone mineralization, and NS relapse rate in children with steroid-sensitive NS. METHODS: A randomized controlled trial (RCT) was performed in children with steroid-sensitive NS. The treatment group received vitamin D3 (60,000 IU orally, weekly for 4 weeks) and calcium supplements (500 to 1,000 mg/day for 3 months) after achieving NS remission. Blood samples for bone biochemistry were taken during relapse (T0), after 6 weeks (T1) and 6 months (T2) of randomization, whereas a lumbar DXA scan was performed at T0 and T2. Renal ultrasound was performed after study completion in the treatment group and in all patients with hypercalciuria. RESULTS: Of the 48 initial recruits, 43 patients completed the study. Post-intervention, 25(OH)D levels showed significant improvements in the treatment group compared with controls at T1 (p < 0.001) and T2 (p < 0.001). However, this was not associated with differences in bone mineral content (BMC) (p = 0.44) or bone mineral density (BMD) (p = 0.64) between the groups. Additionally, there was no reduction in relapse number in treated patients (p = 0.54). Documented hypercalciuria occurred in 52% of patients in the treatment group, but was not associated with nephrocalcinosis. CONCLUSIONS: Although supplementation with calcium and vitamin D improved 25(OH)D levels significantly, there was no effect on BMC, BMD or relapse rate over a 6-month follow-up. Occurrence of hypercalciuria mandates caution and appropriate monitoring if using such therapy. Appropriate dosage of vitamin D3 remains uncertain and studies examining biologically active vitamin D may provide answers.

Vaccinations in children on immunosuppressive medications for renal disease.

Renal diseases are often treated with immunosuppressive medications, placing patients at risk of infections, some of which are vaccine-preventable. However, in such patients vaccinations may be delayed or disregarded due to complications of the underlying disease process and challenges in its management. The decision to administer vaccines to immunosuppressed children is a risk-benefit balance as such children may have a qualitatively diminished immunological response or develop diseases caused by the vaccine pathogen. Vaccination may cause a flare-up of disease activity or provocation of graft rejection in renal transplant recipients. Moreover, it cannot be assumed that a given antibody level provides the same protection in immunosupressed children as in healthy ones. We have evaluated the safety and efficacy of licensed vaccines in children on immunosuppressive therapy and in renal transplant recipients. The limited evidence available suggests that vaccines are most effective if given early, ideally before the requirement for immunosuppressive therapy, which may require administration of accelerated vaccine courses. Once treatment with immunosuppressive drugs is started, inactivated vaccines are usually considered to be safe when the disease is quiescent, but supplemental doses may be required. In the majority of cases, live vaccines are to be avoided. All vaccines are generally contraindicated within 3-6 months of a renal transplant.

Vitamin D in nephrotic syndrome remission: a case-control study.

BACKGROUND: Vitamin D deficiency may contribute to osteoporosis in nephrotic syndrome (NS). METHODS: A cross-sectional case-control study was performed to investigate 25 hydroxycholecalciferol [25(OH)D] status in 40 patients with NS in remission and 40 healthy controls. Serum levels of 25(OH)D, calcium, phosphate, alkaline phosphatase (ALP), and intact parathyroid hormone (PTH) were assayed. NS patients were segregated by age at onset, current age, type and duration of NS, months since relapse and current drug therapy. RESULTS: Levels of 25(OH)D showed a positive correlation with months elapsed since last NS relapse (r s = +0.4, p = 0.012) and were lower in NS patients within 3 months of relapse but similar to that of controls in patients in remission for >3 months [median 14.23 (interquartile range 12.19-17.63) vs. 19.75 (14.04-28.38) ng/ml, respectively; p = 0.039]. There was no correlation of 25(OH)D levels with other disease characteristics or drug therapy. ALP levels were also lowest after relapse (r s = +0.34, p = 0.036). Overall, 25(OH)D levels of <20 ng/ml occurred in 62.5 % of NS patients + controls, and correlated negatively with age (r s = -0.24, p = 0.037) but showed no significant correlation with calcium, phosphate, or PTH levels. CONCLUSIONS: In our patients with NS, vitamin D stores remained low for 3 months after NS relapse but showed an increase with longer remission time to control levels. Vitamin D stores were not influenced by disease characteristics or therapy. Longitudinal studies are required to confirm these findings and evaluate the effect of vitamin D on bones, particularly in frequent relapsers.

Outcome of severe steroid-dependent nephrotic syndrome treated with mycophenolate mofetil.

BACKGROUND: Mycophenolate mofetil (MMF) is used as a steroid-sparing agent in pediatric nephrotic syndrome (NS). However, data about its long-term efficacy and safety is limited. METHODS: We report the long-term outcome of MMF therapy in 46 NS patients who remained steroid dependent (SD) despite previous treatment with levamisole and cyclophosphamide. RESULTS: After 1 year of MMF initiation, 32 (70 %) patients had reduced steroid requirement: 12 with decreased threshold dose and 20 were able to stop steroids. At follow-up of mean 3.56 (standard deviation + 1.76) years, 25 (54 %) children required no further alternative immunosuppression (IS), having infrequent or no relapses, of which 14 stopped MMF after a mean 2.4 (standard deviation + 0.9) years; 11 are continuing on MMF for a median of 2.25 years (range 1.33-7.75 years). One patient had a psoriasis flare, and MMF was stopped. No other patient required permanent drug withdrawal due to side effects. The outcome of patients who did not require further alternate IS was significantly better than those who did, with 56 % vs. 10.5 %, respectively, being off regular medications at last follow-up. CONCLUSIONS: We conclude that MMF therapy is safe in the long term and allows >50 % of severe SDNS patients to avoid further toxic IS.

A novel combination of OHVIRA syndrome and likely causal variant in UMOD gene.

OHVIRA syndrome (or Herlyn-Werner-Wunderlich syndrome) is a rare condition, consisting classically of obstructed hemi-vagina with ipsilateral renal agenesis. It may be associated with complex uterine malformations and more rarely with lower urinary tract anomalies. The contralateral kidney usually has normal function. A genetic etiology of this syndrome has not yet been confirmed. We report a patient who was diagnosed to have unilateral renal agenesis in early childhood, and then presented after menarche with features of OHVIRA syndrome. The contralateral kidney was relatively small and echogenic, and serum creatinine and uric acid were raised. A likely causal variant of the UMOD gene was detected on whole exome sequencing. Genetic studies in more patients with OHVIRA syndrome may elucidate further, whether the association with UMOD gene is causal in nature.

Investigation on water defluoridation via batch and continuous mode using Ce-Al bimetallic oxide: Adsorption dynamics, electrochemical and LCA analysis.

With variable atomic ratios, Ce-Al bimetallic oxides were fabricated using the sol-gel combustion method and utilized for efficient fluoride removal. The synthesized bimetallic oxides were extensively studied using advanced characterization techniques, including TGA, XRD, FTIR, BET surface area analysis, EDX-assisted FESEM, XPS and impedance analysis. These techniques facilitate the interpretation of the chemical and physical properties of the synthesized material. The Ce-Al (1:1) bimetallic oxide was selected as an adsorbent for the defluoridation. The Ce-Al (1:1) oxide demonstrates a moderately high surface area of 108.67 m2/g. The sorption behaviour of fluoride on Ce-Al (1:1) was thoroughly investigated using batch and column modes. The maximum fluoride removal efficiency (99.4%) was achieved at a temperature of 45 °C and pH of 7.0 using an adsorbent dose of 0.18 g/L for 35 min. Pseudo-second-order kinetic model appropriately describes the sorption process. Freundlich's adsorption isotherm was more pertinent in representing fluoride adsorption behaviour. The maximum fluoride adsorption capacity is 146.73 mg/g at 45 °C. Thermodynamics study indicates fluoride adsorption on Ce-Al (1:1) bimetallic oxide is spontaneous and feasible. The adsorption mechanism was interpreted through XPS spectra, indicating that the physisorption process is mainly responsible for fluoride adsorption. An in-depth investigation of the adsorption dynamics was carried out using mass transfer models and found that the external diffusion process limits the overall adsorption rate. An electrochemical investigation was performed to understand the effect of fluoride adsorption on the electrochemical behaviour of bimetallic oxide. The fixed-bed column adsorption study suggested that the lower flow rate and increased bed height favourably impacted the overall defluoridation process, and column adsorption results were suitably interpreted through both the Adam-Bohart model and Yoon-Nelson dynamics model. The sustainable aspect of the defluoridation process was elucidated in terms of carbon footprint measurement using life cycle assessment analysis. The carbon footprint of the entire treatment process was calculated as 0.094 tons/year.