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

Carbon sequestration and biodiversity following 18 years of active tropical forest restoration

Vast areas of degraded tropical forest, combined with increasing interest in mitigating climate change and conserving biodiversity, demonstrate the potential value of restoring tropical forest. However, there is a lack of long-term studies assessing active management for restoration. Here we investigate Above-Ground Biomass (AGB), forest structure, and biodiversity, before degradation (in old-growth forest), after degradation (in abandoned agricultural savanna grassland), and within a forest that is actively being restored in Kibale National Park, Uganda. In 1995 degraded land in Kibale was protected from fire and replanted with native seedlings (39 species) at a density of 400 seedlings ha-1. Sixty-five plots (50 m × 10 m) were established in restoration areas in 2005 and 50 of these were re-measured in 2013, allowing changes to be assessed over 18 years. Degraded plots have an Above Ground Biomass (AGB) of 5.1 Mg dry mass ha-1, of which 80% is grass. By 2005 AGB of trees ≥10 cm DBH was 9.5 Mg ha-1, increasing to 40.6 Mg ha-1 by 2013, accumulating at a rate of 3.9 Mg ha-1 year-1. A total of 153 planted individuals ha-1 (38%) remained by 2013, contributing 28.9 Mg ha-1 (70%) of total AGB. Eighteen years after restoration, AGB in the plots was 12% of old-growth (419 Mg ha-1). If current accumulation rates continue restoration forest would reach old-growth AGB in a further 96 years. Biodiversity of degraded plots prior to restoration was low with no tree species and 2 seedling species per sample plot (0.05 ha). By 2005 restoration areas had an average of 3 tree and 3 seedling species per sample plot, increasing to 5 tree and 9 seedling species per plot in 2013. However, biodiversity was still significantly lower than old-growth forest, at 8 tree and 16 seedling species in an equivalent area. The results suggest that forest restoration is beneficial for AGB accumulation with planted stems storing the majority of AGB. Changes in biodiversity appear slower; possibly due to low stem turnover. Overall this restoration treatment is an effective means of restoring degraded land in the area, as can be seen from the lack of regeneration in degraded plots, which remain low-AGB and diversity, largely due to the impacts of fire and competition with grasses

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