64 research outputs found
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
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The Late Quaternary glaciation of Africa: A regional synthesis
There are numerous mountain ranges in Africa which carry evidence of Quaternary glaciation, though few have glaciers now and these are rapidly disappearing. The best studied sites are in East Africa and Ethiopia. In East Africa, the three highest mountains, Kilimanjaro, Kenya and the Rwenzori, each have evidence of 3–5 major glaciations dating back to an estimated 400,000 BP, one of which is dated to the Last Glacial Maximum (LGM) by radiocarbon or cosmogenic radionucleide (CRN) methods; two other mountains have similar large moraine systems inferred to date to the LGM. In Ethiopia, three mountains have clear evidence of glaciation and on two of them radiocarbon dates give minimum ages which suggest that these are LGM features. In North Africa, the Atlas and other groups of mountains have evidence of glaciation but, in the absence of any dating, it is unclear whether these sites were glaciated at the LGM. Although geomorphic features resembling moraines and protalus ramparts have been described from the Drakensberg Mountains, South Africa, it is still unclear whether these features indicate glaciation and can be attributed to the LGM. Thus, although circumstances for fieldwork on African mountains are very difficult in many cases, investigations for more than a century provide an extensive picture of Quaternary glaciation. In some cases, there has been an increasingly detailed mapping of field evidence and the first CRN dates were carried out recently, thus improving the global picture considerably. Particularly in East Africa and Ethiopia there is sufficiently reliable field information to make useful estimates of equilibrium line altitude changes at the provisionally identified LGM, but this age needs confirmation by further CRN dating. By making some hypothetical assumptions, inferences can be drawn that indicate a temperature lowering of 5–6 °C during the LGM. Elsewhere in Africa, further field surveys are required.23 page(s
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
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.</p
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