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

Geotourism, iconic landforms and island-style speciation patterns in National Parks of East Africa:

Many of the national parks in East Africa are equally as famous for their iconic landforms as they are for their diversity and concentrations of fauna and flora. The newly formed Ngorongoro-Lengai Geopark in northern Tanzania is the first geopark to be established in the region, but there is remarkable potential for geotourism in the majority of the national parks. The most spectacular landforms have been shaped by the East African Rift System. Formation of the two major rifts in the region, the Albertine Rift (or western branch) and the Gregory Rift (or eastern branch), was accompanied, or in some cases preceded, by extensive alkaline volcanism. The rifting and volcanism are primarily Late Cenozoic phenomenon that dissected and overprinted the older regional plateaus. Rifting impacted the regional drainage and captured major rivers, including the Victoria Nile

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

Reconstruction of equilibrium-line altitudes for tropical and sub-tropical glaciers

Past fluctuations of tropical and sub-tropical glaciers provide important palaeoclimate proxies for regions where other forms of evidence are rare. However, published equilibrium-line altitude (ELA) estimates for tropical and sub-tropical glaciers at the LGM vary widely, reflecting the diversity of methods and approaches employed by different research groups. This complicates regional and global comparisons of ELA estimates, and emphasises the need for standardised methods. The distinctive character of tropical and sub-tropical glaciers, however, means that standard methods for reconstructing former glacier limits, ELAs. and palaeoclimate need to be adapted for local conditions. Many methods of ELA reconstruction explicitly or implicitly make assumptions about glacier mass balance gradients, and care needs to be taken that the choice of accumulation area ratios (AARs), balance ratios (BRs) and terminus-to-head ratios (THARs) is appropriate, as such indices are influenced by climatic regime. debris cover and other factors. ELA reconstructions should employ multiple methods, and should be cross-checked and fully reported, to allow assessment of the accuracy of ELA estimates. Reliable glacier chronologies are equally important. Dating should be based on multiple radiometric techniques wherever possible, and method of dating, the type of material dated, and the context of the date must all be reported. (c) 2005 Elsevier Ltd and INQUA. All rights reserved. [References: 80

The 10Be deglaciation chronology of the Göschenertal, central Swiss Alps, and new insights into the Göschenen Cold Phases

The Göschenertal (Göschenen valley) is the type locality of the so‐called Göschenen Cold Phases I (~3–2.3 ka) and II (~1.8–1.1 ka). According to earlier studies, these Late Holocene climatic cooling periods were characterized by changes in vegetation and pronounced glacier advances. As a peculiarity, the Göschenen Cold Phase I was thought to be connected to a local surge‐type advance of the Chelengletscher (Chelen glacier) – an exceptional event of unparalleled dimension in the European Alps. Based on cosmogenic 10Be exposure ages from moraine boulders, we investigated the local glacier chronology. In contrast to former research, moraines at different positions within the Göschenen valley (central Swiss Alps) have been dated to the Younger Dryas and the Early Holocene. This questions the applicability of palaeo‐Equilibrium Line Altitude (ELA) calculations for stadial attributions without additional numerical age constraints. Furthermore, we have found compelling evidence that the proposed non‐climatic glacier advance attributed to the Göschenen Cold Phase I did not occur. The present results, along with a reappraisal of the original study, question the scientific reliability and the glaciological definition of the Göschenen Cold Phases as glacier advances that clearly exceeded the Little Ice Age positions. While our data do not exclude potential changes in climate and vegetation, we nonetheless show that the Göschenen Cold Phases are not suitable as reference stadials in the system of Alpine Holocene glacier fluctuations