Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort

\ua9 2024 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 licenseBackground: Individuals with rare kidney diseases account for 5–10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. Methods: People aged 0–96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan–Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1\ub773 m2 or more to first eGFR of less than 30 mL/min per 1\ub773 m2 (the therapeutic trial window). Findings: Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9\ub76 years (IQR 5\ub79–16\ub77). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2\ub781 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0\ub70001), but better survival rates (standardised mortality ratio 0\ub742 [95% CI 0\ub732–0\ub752]; p<0\ub70001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. Interpretation: Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3–5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. Funding: RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity

Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort

Background Individuals with rare kidney diseases account for 5–10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. Methods People aged 0–96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan–Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m2 or more to first eGFR of less than 30 mL/min per 1·73 m2 (the therapeutic trial window). Findings Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9–16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32–0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. Interpretation Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3–5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. Funding RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity

Synthesis and physico-chemical study of polyester polyol of epoxy resin of 1, 1’-bis (3-methyl-4-hydroxy phenyl) cyclohexane and ricinoleic acid and its polyurethanes with polyethylene glycol

377-384Cardo polyester polyol (acid value, 2.14; hydroxyl value, 911 mg KOH/g) has been synthesized by reacting epoxy resin of 1,1’-bis (3-methyl-4-hydroxy phenyl) cyclohexane (EE = 436) and ricinoleic acid (8.94 g) by using triethyl amine (0.5 g) as a catalyst and 1,4-dioxane as a solvent at reflux temperature for 6h. Cardo polyester polyol-TDI based copolyurethanes have been synthesized by reacting polyol (3.0 g) and TDI (0.9 g) in MEK at room temperature by using 20% and 30% PEG- 400 on the basis of polyol weight. PU-20 and PU-30 films were cast by controlled evaporation of MEK. The formation of polyol and copolyurethanes are supported by FTIR spectral data. Densities of PU-20 (1.2379 g/cm³) and PU-30 (1.2016 g/ cm³) are determined by floatation method at room temperature by using CCl₄₋ n- hexane system. Density decreased with PEG-400 concentration due to increase in intrinsic volume of the repeating unit. Tensile strength, electric strength and volume resistivity of both co polyurethanes decreased with increasing concentration of PEG-400 due to presence of soft and hard segments in polymer chain. PU-20 and PU-30 remained unaffected in pure water and 10% NaCl solution and showed moisture uptake tendency in acidic and alkaline atmosphere. Max. moisture uptake, observed within 24-48h, is 4.7-15% in 10% each of HCl, H₂SO₄ and HNO₃ and 8.7-14.2 % in 10% each of NaOH and KOH solutions. PU-30 has much low water absorption tendency as compared to PU-20 due to increasing PEG-400 concentration. PU-20 and PU-30 are thermally stable up to about 190-198°C and involved three-step degradation kinetics. No effect of PFG-400 was observed on thermal properties of co polyurethanes. For PU-20, T₀, T₁₀ and T₅₀ are 190, 225.1 and 362.6°C, while for PU-30 they are 198, 235.5 and 369.0°C, respectively. The decomposition ranges are 190-304, 342-440,472-540 and 198-307, 350-430, and 448-5900C, respectively for PU-20 and PU-30. The excellent hydrolytic stability and fairly good thermo-mechanical and electrical properties of PU-20 and PU-30 signify their industrial importance as coating and adhesive materials

Effects of cation disorder on terahertz emission from half-doped manganite thin films

We have observed terahertz (THz) emission from antiferromagnetic charge-ordered “undoped” Nd0.5Ca0.5MnO3 and “Ba-doped” Nd0.5Ca0.48Ba0.02MnO3 manganites by illuminating femtosecond laser pulses on the optical switch fabricated on highly oriented thin films. The THz emission and the magnetic characteristics of these manganite thin films are studied and compared. The THz emission shows strongly temperature-dependent characteristics. A pronounced broad peak is observed at the charge-ordering transition in the temperature-dependent THz emission of the films. On a comparison of the results of both the films, this peak is found largely suppressed for Ba-doped manganite. These results, corroborated by the magnetization of films, reveal a role of the A-site cation disorder in affecting the THz emission from ABO3-type charge-ordered manganites

Implications of phase-segregation on structure, terahertz emission and magnetization of Bi (Fe

Structural, magnetic and terahertz emission properties of Bi (Fe$_{1-x}$Mnx)O3  (0 $\leqslant$ x $\leqslant$ 0.5) thin films of various thicknesses were studied. A transition from coherently strained structure to relaxed structure at a film thickness (t) of ~ 80– 90 nm occurs only for x < 0.2. It is shown that terahertz-emission efficiency is not deteriorated with increasing Mn-doping (x). The magnetic moment of thin films (t $\leqslant$ 85 nm) exhibits only a weak enhancement with increasing x —a feature suggesting that Mn-doping is ineffective in inducing ferromagnetism in BiFeO3. The thicker films (t $\geqslant$ 150 nm), on the contrary, possess larger magnetic moment which evidently arises from the segregated magnetic MnFe2O4 phase