This Type Of Kidney Disease Has A High Rate Of Failure And A High Survival Rate!

Rare kidney diseases are a group of extremely rare diseases that seriously endanger patients' lives and health. Although patients with rare kidney diseases account for only 5%-10% of the chronic kidney disease population, they account for more than 25% of patients undergoing renal replacement therapy such as dialysis or kidney transplantation.

Click to Cistanche for kidney disease

A recent study published in The Lancet conducted a longitudinal analysis of patient data from the British National Rare Kidney Disease Registry (RaDaR) and found that patients with rare kidney diseases have a much higher risk of kidney failure than common chronic kidney disease patients, but their survival rates are unexpected. The ground is high. This discovery challenges people's traditional understanding of chronic kidney disease and provides new ideas and directions for improving the prognosis of patients with rare kidney diseases.

Rare kidney disease: high-risk groups hidden in common chronic kidney disease

Chronic kidney disease is a general term covering impairment of renal function caused by various reasons. It can be divided into stages 1-5 according to the level of glomerular filtration rate (eGFR). Stages 3-5 represent moderate to severe renal insufficiency, affecting 6.1% of people over the age of 16 and 32.7% of people over the age of 75 in the UK. Diabetes and hypertension are the most common causes of stage 3 chronic kidney disease in high- and middle-income countries.

Rare diseases are defined as affecting less than 200,000 people (U.S. standards) or with an incidence rate of less than 1/2000 (European standards). About 80% of rare diseases are genetic diseases.

According to the definition of Kidney Disease Improving Global Outcomes (KDIGO), rare kidney diseases include more than 150 diseases and the prevalence in European and American populations is estimated to be 60-80 cases per 100,000 people. More than half of children and adolescents under the age of 20 who receive renal replacement therapy develop a rare kidney disease.

Unlike early chronic kidney disease, various rare forms of glomerulonephritis (which together account for a high proportion) are also more common among adults receiving renal replacement therapy than common chronic kidney diseases such as diabetes. The reason for this difference is unclear but may be related to a slower decline in kidney function and a higher risk of death in unselected people with chronic kidney disease.

Furthermore, although population-based data show differences in renal function decline between men and women, sex differences in patients with rare kidney diseases and the validation of kidney failure risk prediction tools (such as the kidney failure risk equation) in unselected chronic kidney disease populations Little is known about how this plays out in patients with rare kidney diseases.

Therefore, there is an urgent need to better understand the progression of rare kidney diseases and the renal and survival prognosis of patients. This not only helps to make more accurate prognostic assessments but also provides a reference for medical resource planning and determines the best time for new therapeutic interventions to delay the progression of renal failure, thereby reducing the overall burden of renal failure on population health.

Innovative treatments for rare kidney diseases, such as the new drug budesonide extended-release tablets for IgA nephropathy, are rapidly emerging. Therefore, real-world historical and current rare kidney disease prognostic data are urgently needed to evaluate safety and efficacy data from clinical trials and provide the basis for economic modeling for cost-effectiveness analyses.

However, in China, IgA nephropathy is the most common chronic primary glomerulonephritis and cannot be regarded as a rare kidney disease.

UK National Rare Kidney Disease Registry (RaDaR): the world's largest cohort study of rare kidney diseases

In 2010, the British Society of Nephrology launched a strategic plan for rare kidney diseases, which included the establishment of a national rare kidney disease registry (RaDaR).

To date, RaDaR has included more than 30,000 patients in 29 disease categories from 108 kidney disease centers in the UK. It has established data links with hospital laboratories and the British Kidney Registry, and can obtain information on the initiation of renal replacement therapy and death. The verified key clinical endpoint data is currently the largest rare kidney disease registration study in the world.

This Lancet study uses RaDaR data to describe the clinical demographics, disease characteristics, and renal and survival outcomes of patients with rare kidney diseases in the UK.

In China, the National Health Commission issued the "Notice of the General Office of the National Health Commission on Carrying out the Registration of Diagnosis and Treatment Information of Rare Disease Cases" in 2019, requiring all localities to pay attention to the registration of diagnosis and treatment information of rare disease cases. For newly diagnosed cases, one should be found, register an example. And do a good job in organizational management, information registration, and daily management.

The theme of this year's World Kidney Day is "Kidney Health for All: Promoting Equitable Medical Services and Optimized Drug Treatment Practices." It is believed that with the strong support of the country, the personalized treatment of rare kidney disease patients in my country will also become more and more effective. optimization.

The natural history of 27,285 patients with rare kidney disease emerges

The study included 27,285 rare kidney disease patients recruited between January 18, 2010 and July 25, 2022, involving 28 disease types. The median follow-up time was 9.6 years after diagnosis. RaDaR participants had a significantly higher 5-year cumulative incidence of kidney failure than the 2.81 million unscreened UK patients with chronic kidney disease (28% vs 1%, p<0.0001), but better survival (standardized mortality rate 0.42, p<0.0001 ).

Age at renal failure, age at death, time from initiation of dialysis to death, time from diagnosis to reaching a specific eGFR threshold, and the time window suitable for participation in therapeutic clinical trials (defined as the last eGFR ≥75 mL/min) among different rare kidney diseases /1.73m2 to the first eGFR <30mL/min/1.73m2) and other aspects showed significant differences. The study reports in detail on renal and survival outcomes for each of the rare kidney diseases.

In terms of cystic kidney disease, autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Patients are usually diagnosed in their 40s, with a median survival of over 70 years and a 10-year kidney survival rate of 76%.

In contrast, autosomal recessive polycystic kidney disease (ARPKD), which occurs in infancy and early childhood, progresses more rapidly. Children are diagnosed at a median age of 8.7 years and require renal replacement therapy at 19.3 years old. The 10-year renal survival rate is only 66 %.

In terms of glomerular diseases, the median age of patients with IgA nephropathy at diagnosis was 40.4 years old, and their eGFR had dropped to 40mL/min/1.73m2. Nearly half (48%) developed uremia within 10 years, and the clinical trial time window was only 4 years.

Membranous nephropathy tends to occur in middle-aged and elderly people. The median age at diagnosis is 56.8 years old. The eGFR is still 67.4mL/min/1.73m2. The 10-year kidney survival rate is 77%. The clinical trial time window is 11.2 years. Mesangial proliferative nephritis/C3 glomerulopathy (MPGN/C3G) also has a poor overall prognosis, with a 10-year renal survival rate of 67%.

Other diseases, such as cystic fibrosis, hyperoxaluria and other rare kidney diseases, also have different progressions. Overall, the risk of death in RaDaR patients with rare kidney disease within 5 years is more than twice that of the general population, and the risk of death in dialysis patients is four times that of the general population. But compared with unscreened patients with stage 3-5 chronic kidney disease, patients with rare kidney disease had a 58% lower risk of death.

Rare kidney diseases require different diagnosis and treatment strategies from common chronic kidney diseases

Based on RaDaR's large sample data, this study provides reliable estimates of the rate of kidney function decline, risk of kidney failure, and risk of death in 28 rare kidney diseases.

The results show that, overall, patients with rare kidney disease are very different from the general chronic kidney disease population: they have a higher risk of progression to uremia within 5 years, but they live longer and survive on dialysis treatment at a higher rate, so A high proportion of the total population requiring renal replacement therapy.

Notably, the study found that patients with rare kidney disease progressed faster than unscreened patients with chronic kidney disease, especially in people under 65 years old, and existing kidney failure risk models could not accurately predict their prognosis.

The heterogeneity in prognosis between different rare kidney diseases highlights the clinical value of a clear diagnosis: not only does it inform treatment decisions, but it also allows assessment of prognosis and identification of diseases and subgroups most in need of new therapies. The research results are helpful for patients to make informed decisions, provide a reference for managers and medical service providers to make decisions, and can also provide a basis for researchers to design clinical studies.

The data may also provide insight into the effectiveness of existing treatments and the optimal timing of intervention during the course of the disease. For example, patients with cystinosis are often diagnosed at a young age (median 1.9 years) and have relatively good kidney function. After treatment with cystine drugs, the median age at kidney failure increased to 15.4 years, compared with about 9 years before treatment. age. However research highlights the need to find new treatments that are more effective in protecting kidney function.

In contrast, patients with ANCA-associated vasculitis had already low eGFR at presentation, but renal function declined slowly in this cohort, possibly reflecting the effectiveness of existing treatments. The time window for clinical trials of IgA nephropathy is very short, which suggests that if the study includes patients who have been diagnosed for many years and whose renal function is well preserved, rare patients with mild disease may be inadvertently selected, resulting in a decrease in the sensitivity of drug effect detection.

Therefore, the observed course characteristics of each disease may require new interventions that are best targeted at early diagnosis, rescuing or protecting renal function during disease progression, or reducing adverse effects of long-term treatment.

The study also observed that in ADPKD, IgA nephropathy, MPGN/C3G and FSGS, female patients were younger at diagnosis and had higher eGFR. The median age for renal failure was similar in both sexes, although women took longer to progress to uremia.

This may reflect early detection in early adult women due to greater contact with the health care system. Therefore, if early screening strategies are introduced for these rare kidney diseases in the future, they may need to be more targeted at the male population.

In summary, this Lancet study uses large-scale data to provide reliable estimates of the rate of decline in kidney function, risk of kidney failure, and risk of death in 28 rare kidney diseases.

In the unselected chronic kidney disease population, death before renal failure is a common adverse outcome, whereas in patients with rare kidney disease, survival is better and the incidence of renal failure is much higher.

This means that, although strategies to reduce cardiovascular risk and other causes of death are important in the large population with chronic kidney disease, a large proportion of kidney failure is attributable to various rare kidney diseases that cannot be prevented by cardiovascular risk reduction progress.

Therefore, patients with rare kidney disease should be distinguished from chronic kidney disease due to more common causes, emphasizing the importance of early referral, clear diagnosis, and the need for specific treatments for rare kidney disease to slow the progression of renal failure. Successfully meeting the need for treatment of rare diseases could have a large and beneficial impact on reducing the need for renal replacement therapy, thereby providing economic benefits to the healthcare system.

In summary, this study used data from the UK National Rare Kidney Disease Registry to initially reveal the natural course characteristics of patients with various rare kidney diseases and found that their risk of kidney failure was much higher than that of common chronic kidney disease patients, but their survival rates were also unexpectedly high.

This discovery provides important clues and directions for further targeted research and the development of individualized diagnosis and treatment strategies in the future and is of great significance for improving the prognosis of patients with rare kidney diseases.

We expect that registry studies such as RaDaR will continue to expand the sample size, include more types of rare kidney diseases, conduct more in-depth stratified analysis, and accumulate more real-world evidence for accurate risk prediction and personalized diagnosis and treatment. At the same time, it also calls on the whole society to pay attention to and support the prevention and treatment of rare kidney diseases, and increase investment in basic research, drug development, screening diagnosis, standardized treatment, etc., so that more patients can benefit from medical progress and get rid of the disease as soon as possible. , the right to enjoy a better life.

How Does Cistanche Treat Kidney Disease?

Cistanche is a traditional Chinese herbal medicine used for centuries to treat various health conditions, including kidney disease. It is derived from the dried stems of Cistanche deserticola, a plant native to the deserts of China and Mongolia. The main active components of cistanche are phenylethanoid glycosides, echinacoside, and acteoside, which have been found to have beneficial effects on kidney health.

Kidney disease, also known as renal disease, refers to a condition in which the kidneys are not functioning properly. This can result in a buildup of waste products and toxins in the body, leading to various symptoms and complications. Cistanche may help treat kidney disease ase through several mechanisms.

Firstly, cistanche has been found to have diuretic properties, meaning it can increase urine production and help eliminate waste products from the body. This can help relieve the burden on the kidneys and prevent the buildup of toxins. By promoting diuresis, cistanche may also help Reduce high blood pressure, a common complication of kidney disease.

Moreover, cistanche has been shown to have antioxidant effects. Oxidative stress, caused by an imbalance between the production of free radicals and the body's antioxidant defenses, plays a key role in the progression of kidney disease. ies help neutralize free radicals and reduce Oxidative stress, thereby protecting the kidneys from damage. The phenylethanoid glycosides found in cistanche have been particularly effective in scavenging free radicals and inhibiting lipid peroxidation.

Additionally, cistanche has been found to have anti-inflammatory effects. Inflammation is another key factor in the development and progression of kidney disease. Cistanche's anti-inflammatory properties help reduce the production of pro-inflammatory cytokines and inhibit the activation of inflammation mandatory pathways, thus alleviating inflammation in the kidneys.

Furthermore, cistanche has been shown to have immunomodulatory effects. In kidney disease, the immune system can be dysregulated, leading to excessive inflammation and tissue damage. Cistanche helps regulate the immune response by modulating the production and activity of immune cells, such as T cells and macrophages. This immune regulation helps reduce inflammation and prevent further damage to the kidneys.

Moreover, cistanche has been found to improve renal function by promoting the regeneration of renal tubes with cells. Renal tubular epithelial cells play a crucial role in the filtration and reabsorption of waste products and electrolytes. In kidney disease, these cells can be damaged, leading to damaged renal function. Cistanche's ability to promote the regeneration of these cells helps restore proper renal function and improve overall kidney health.

In addition to these direct effects on the kidneys, cistanche has been found to have beneficial effects on other organs and systems in the body. This holistic approach to health is particularly important in kidney disease, as the condition often affects multiple organs and systems. che has been shown to have protective effects on the liver, heart, and blood vessels, which are commonly affected by kidney disease. By promoting the health of these organs, cistanche helps improve overall kidney function and prevent further complications.

In conclusion, cistanche is a traditional Chinese herbal medicine used for centuries to treat kidney disease. Its active components have diuretic, antioxidant, anti-inflammatory, immunomodulatory, and regenerative effects, which help improve renal function and protect the kidneys from further damage. , cistanche has beneficial effects on other organs and systems, making it a holistic approach to treating kidney disease.