BEIJING, Beijing, February 22 (Reporter Yuan Xiuyue) What progress has been made in the management of water ecological environment in China in the past year? What are the key arrangements for the remediation of black and odorous water bodies and the supervision of sewage outlets in 2023? On the 22nd, the Ministry of Ecology and Environment held a regular press conference in February, and the relevant person in charge introduced the water ecological environment management.

　　In 2022, the discharge of major water pollutants in China will continue to decline.

　　Huang Xiaozeng, Director of the Department of Water Ecology and Environment of the Ministry of Ecology and Environment, introduced that in 2022, the Ministry of Ecology and Environment issued and implemented the action plan for the remediation of sewage outlets entering the Yangtze River, and the traceability of sewage outlets in the Yangtze River and Bohai Sea was completed by more than 90%, which promoted the solution of more than 20,000 sewage direct discharge and disorderly discharge. Complete the investigation of sewage outlets in the middle and upper reaches of the Yellow River and 30 cities in 6 provinces of Fenhe River Basin.

　　In 2022, the discharge of major water pollutants in China will continue to decline, and the goal of improving the quality of water ecological environment will be successfully completed. The proportion of excellent surface water quality sections in China was 87.9%, up 3.0 percentage points year-on-year; The proportion of sections with inferior grade V water quality was 0.7%, down 0.5 percentage points year-on-year; For three consecutive years, the main stream of the Yangtze River reached Grade II water quality, and the main stream of the Yellow River reached Grade II water quality for the first time. Chemical oxygen demand and total ammonia nitrogen emissions decreased by 3.7% and 5.5% respectively.

　　Huang Xiaozeng said that although the water ecological environment protection has achieved remarkable results, it still faces many bottlenecks and challenges, and there are still many outstanding problems and shortcomings to be solved urgently. For example, it is difficult to fundamentally eliminate black and odorous water bodies, and the water ecological environment governance capacity needs to be improved. In some places, pollution interception and pollution control are still not in place, and the problems of cyanobacteria bloom and water ecological imbalance still exist.

　　This year, the country plans to complete the renovation of more than 800 large-scale rural black and odorous water bodies.

　　Regarding the problem of black and odorous water bodies, Huang Xiaozeng said that in recent years, all localities have taken responsibility and worked hard to speed up the short-board of urban environmental infrastructure. Judging from the current situation, the black and odorous water bodies in the built-up areas of cities (excluding States and leagues) at the prefecture level and above that were included in the supervision list of black and odorous water bodies in the "Thirteenth Five-Year Plan" have been basically eliminated. He mentioned that in 2022, the elimination ratio of black and odorous water bodies in county-level cities successfully completed the annual target of 40%.

　　On the issue of black and odorous water bodies in rural areas, since the "14th Five-Year Plan", the Ministry of Ecology and Environment has organized all localities to carry out comprehensive investigations on black and odorous water bodies in rural areas, and has included more than 4,000 black and odorous water bodies in rural areas that have been strongly reflected by the masses and have a large area in the national supervision list, and implemented the policy of "posting accounts and canceling the numbers one by one". In 2023, the country plans to complete more than 800 large-scale rural black and odorous water bodies remediation, and related tasks have been broken down and distributed to various provinces.

　　He said that in 2022, the Ministry of Ecology and Environment and the Ministry of Finance launched a pilot project for the treatment of black and odorous water bodies in rural areas, and awarded 200 million yuan, 100 million yuan and 50 million yuan to 15 cities across the country according to the project investment and the declared treatment area. In 2023, the guidance to the pilot areas will be strengthened, and all localities will be urged to further promote the treatment of black and odorous water bodies in rural areas through comprehensive and systematic control measures such as pollution control, dredging, water system connectivity and water ecological restoration. At the same time, this year, the two departments will continue to select and support basic and qualified areas and carry out pilot work.

　　230,000 sewage outlets have been investigated, and about one third of them have been rectified.

　　Regarding the supervision of sewage outlets, Huang Xiaozeng said that according to the dispatching data, 245,000 kilometers of river and lake coastlines have been investigated nationwide, and nearly 230,000 sewage outlets have been found. In the future, with the deepening of the investigation work, the number will surely continue to increase. So far, about one third of the 230,000 sewage outlets have been rectified. In recent years, ecological and environmental departments at all levels have approved more than 8,000 sewage outlets into the river and filed more than 900 sewage outlets into the sea.

　　He said that this year’s target tasks are the main streams and important tributaries of the seven river basins of the Yangtze River, the Yellow River, the Huaihe River, the Haihe River, the Pearl River, the Songliao River and the Taihu Lake. Key lakes and key bays should fully complete the investigation of sewage outlets, and also complete 80% of the traceability and 30% of the remediation tasks.

　　Huang Xiaozeng mentioned that in these typical cases exposed by central environmental protection inspectors, it was found that the centralized collection and treatment rate of sewage in some places was even less than 10%. The places where this happens should be superficial articles on pollution control, and there are still some places where environmental infrastructure construction is lagging behind and sewage treatment capacity is insufficient.

　　"The problem of water pollution is in the water and the roots are on the shore." Huang Xiaozeng said that the reason is that some places have not established a correct view of political achievements, and pollution control "pays more attention to face than lining", which requires real money and silver investment, such as the construction of sewage pipe network and the upgrading of sewage treatment facilities. These aspects are inaction and slow action, and even some places still have the idea of "waiting for what is needed", and there is no fundamental determination to completely solve this problem. This is intolerable.

Reporter Fu Chang and intern Ren Yawen report that the last day of February every year is "International Day of Rare Diseases". On February 23rd, the Rare Diseases Branch of Guangdong Medical Association held the 2019 "International Rare Diseases Day" charity clinic in Guangzhou Hero Square. Experts said that many patients with rare diseases still face problems such as high drug costs, and the public lacks sufficient understanding of rare diseases, calling on the society to pay more attention.

Parents’ voice of children with rare diseases: it is difficult to seek medical treatment and the medicine cost is expensive.

On the morning of the 23rd, there was a light rain in Guangzhou. Although the weather is not beautiful, there are still crowds in front of the charity clinic booth in Hero Square. Many parents came to consult with their children. They frowned slightly and pulled the doctor to ask various questions, as if they had caught the "life-saving straw". In the crowd, the reporter met Mr. Deng and his 4-year-old daughter Jia Jia (a pseudonym).

Mr. Deng told reporters that it was accidental that Jia Jia was diagnosed with a rare disease. "One day, I found that Jia Jia kept blinking when playing with her mobile phone. We thought she had an eye problem, so we quickly took her to the community hospital to see a doctor." He said that as a result, after repeated examinations, ophthalmologists could do nothing about Jiajia’s illness and could not tell why. Seeing that Jia Jia’s symptoms became more and more obvious, Mr. Deng and his wife asked around and visited experts. After many referrals, in November 2017, Jia Jia was finally diagnosed with mucopolysaccharide storage disease in Guangzhou Women and Children Medical Center.

It is understood that mucopolysaccharidosis (MPS) is a rare disease with congenital metabolic abnormality, with an incidence rate of about one in 100,000. Because of the lack of lysosomal enzymes, patients can’t decompose mucopolysaccharides in the body. The accumulated mucopolysaccharides will affect the normal function of cells and destroy many organs of the body such as brain, heart valve, lung and bone. On May 11, 2018, the National Health and Wellness Committee and other five departments jointly formulated the "First List of Rare Diseases", and mucopolysaccharide storage disease was also included.　

Jia Jia, who was diagnosed with this disease, is undoubtedly unfortunate, but compared with other children with mucopolysaccharidosis, Jia Jia may be lucky. Because at the time of diagnosis, Jia Jia’s mother had been pregnant for 30 weeks with her second child, and worried that the second baby would get sick, she immediately went to the hospital for a umbilical cord blood puncture. After confirming the health of the fetus, Jia Jia and her unborn sister were successfully matched, and a bone marrow transplant was successfully performed after her mother gave birth.

"Because of early diagnosis and early treatment, Jia Jia’s current height and intellectual development are normal." Mr. Deng said that in order to help his daughter with treatment, he had previously joined a group of patients with mucopolysaccharide storage disease, many of whom were still struggling on the road of seeking medical treatment and even took many detours. "As far as I know, the average diagnosis time of patients with rare diseases is 7 years, which often misses the ideal treatment period." Mr. Deng said. The reporter also saw at the scene that some children around the age of 10 are only 1 meter tall and have obvious abnormal physical characteristics.

The reporter learned that for the treatment of mucopolysaccharide storage disease, in addition to the bone marrow transplantation surgery accepted by Jia Jia, some experts also proposed gene therapy and enzyme replacement therapy. The former is still in the state of research and development, while the latter refers to the artificial production of enzymes that patients lack and intravenous injection into patients. However, at present, this therapy has not entered the domestic market, and the drug price is expensive, with an annual cost of up to 2 million yuan, which requires lifelong medication. Without the support of medical insurance, ordinary families simply cannot afford it.

Experts appeal: Rare diseases need attention from all walks of life.

It is reported that the World Health Organization (WHO) defines rare diseases as diseases with an incidence of 0.65-1‰, and there are currently about 7,000-8,000 species in the world. "However, due to the large population base in China, rare diseases are not uncommon in terms of the number of patients." Liu Li, chairman of the Rare Diseases Branch of Guangdong Medical Association and chief physician of the Department of Genetics and Endocrinology of Guangzhou Women and Children Medical Center, said. On the occasion of the International Day of Rare Diseases, Liu Li hopes to raise the social attention of rare diseases through public welfare clinics.

She introduced that one of the difficulties in the diagnosis of rare diseases lies in parents’ insufficient understanding of rare diseases. "In real life, a sick child has not been diagnosed and other sick children have been born. This kind of thing often happens." She said that 70%-80% of rare diseases are hereditary diseases, and 70%-80% of rare diseases occur in childhood. Liu Li suggested that parents should have a clear understanding of their children’s development and pay attention to their abnormal conditions in time. If there have been rare cases in the family, prenatal diagnosis must be made before giving birth.

"Rare diseases are rare, doctors who can diagnose rare diseases are even rarer, and drugs that can treat rare diseases are even rarer." This is the true voice of parents of children such as Mr. Deng. Liu Li said that for this reason, the Rare Diseases Branch of Guangdong Medical Association goes to remote places to give academic lectures every year, so that grassroots doctors can understand rare diseases. When patients are found to be abnormal, they can also help to refer them to experts in time, so that patients can seek medical treatment and avoid detours.

She also pointed out that due to the small number of users, high research and development costs, and the lack of relevant laws and regulations in the country, pharmaceutical companies are reluctant to take the initiative to produce rare diseases, which makes it difficult and expensive for patients to buy drugs. However, this situation is gradually improving. With the release of the first batch of rare diseases catalogue in China and the recent joint notice issued by the four departments, the value-added tax policy for rare diseases drugs has been clarified, and the tax rates of some raw materials and imported drugs have been reduced, which will reduce the drug cost of more patients with rare diseases in the future. The patients also called for more drugs to be included in the medical insurance reimbursement.

[Introduction of Several Rare Diseases]

Gaucher’s disease

Gaucher’s disease is a rare monogenic disease and the most common lysosomal storage disease, which is autosomal recessive, with an incidence of about 1/50,000~1/100,000. The mutation of glucocerebrosidase gene (GBA) leads to the lack of glucocerebrosidase activity, which leads to the accumulation of glucocerebrosidase in the lysosomes of macrophages in liver, spleen, bone, lung and even brain, forming a typical storage cell, namely "Gaucher cell", which leads to pathological changes in the affected tissues and organs, and the clinical manifestations are that multiple organs are involved and gradually aggravated. Clinically, Gaucher’s disease can be divided into three types: type ⅰ (non-neuropathy type); Type Ⅱ (acute neuropathy); Type ⅲ (chronic or subacute neuropathy). Caucasians account for about 95% of Gaucher’s disease type I, while Asians account for more than 50% of Gaucher’s disease type II and III.

Patients with Gaucher’s disease are usually characterized by splenomegaly and thrombocytopenia, which are similar to the clinical symptoms of many hematological diseases. Therefore, most patients with Gaucher’s disease will initially go to the hematology department. Hematologists should conduct glucocerebrosidase activity tests to diagnose or rule out Gaucher’s disease after excluding malignant tumors and other diseases.

Gaucher’s disease is an autosomal recessive genetic disease. If the patient’s parents give birth again, the risk of their children getting sick is 25%. For the families and relatives who have given birth to Gaucher’s disease patients, genetic counseling and detection of pathogenic gene carriers should be carried out. Prenatal diagnosis is the most effective way to prevent high-risk families from giving birth to similar children again.

neonatal diabetes

Neonatal diabetes mellitus is a rare special type of diabetes mellitus, which usually refers to persistent and progressive hyperglycemia that occurs within months after birth. The incidence rate is 1/300,000~1/400,000. In the past, the etiology of this type of diabetes was unclear, and it was regarded as an early-onset type of type 1 diabetes for a long time because it needed insulin treatment.

Neonatal diabetes can occur in both men and women, and the onset age ranges from a few days to several weeks. The earliest symptoms that lead to medical treatment are unexplained polyuria and drinking water, often accompanied by feeding difficulties, weight loss or even emaciation, mild to moderate dehydration, mental drowsiness and so on. Some children were diagnosed with elevated blood sugar in routine physical examination, but delayed diagnosis may also lead to symptoms of diabetic ketoacidosis, such as vomiting, shortness of breath, listlessness and even coma. This is a serious manifestation of diabetes, which requires urgent treatment in the hospital. A few children will have motor retardation and occasional convulsions.

Most neonatal diabetes is caused by gene mutation, and the treatment and prognosis of neonatal diabetes caused by different gene mutations are different, so chromosome microarray and gene mutation detection are needed to further clarify the cause diagnosis. Neonatal diabetic children with KCNJⅡ gene and ABCC8 gene mutation can control their blood sugar better by oral sulfonylureas than by exogenous insulin injection.

glycogen storage disease

Glycogen accumulation disease is a kind of glycogen metabolism disorder disease caused by congenital enzyme deficiency, most of which are autosomal recessive inheritance (except for X-linked recessive inheritance of type VI), and its incidence rate is 1/(2 ~ 25 thousand). The process of glycogen synthesis and catabolism requires the participation of various enzymes. There are 12 types of clinical diseases caused by these enzyme defects, among which liver diseases are the main types I, III, IV, VI and VII. Type ⅱ, ⅴ and ⅶ are mainly muscle tissue damage. The common feature of these diseases is glycogen utilization disorder, which increases in liver, muscle, kidney and other tissues.

Glycogen accumulation disease type I is caused by the deficiency of glucose -6- phosphatase system in liver, kidney and other tissues, and it is the most common glycogen accumulation disease, accounting for about 25% of the total. The main manifestations are hypoglycemia, growth retardation, muscle relaxation, abdominal distension, abnormal liver function, metabolic acidosis, increased triglyceride and uric acid in blood tests, and gout and kidney calculi may occur in older children. Most children are prone to infection. Children with the above-mentioned clinical manifestations need to go to a hospital with the conditions for genetic analysis for genetic examination and definite diagnosis and disease classification.

This disease is a rare treatable disease, and diet therapy is the main treatment for glycogen storage disease. The proportions of three nutrients in total calories are: carbohydrate 60-70%, protein 10-15%, and fat < 30%. The carbohydrate is mainly raw corn flour, and raw corn starch releases glucose at a moderate speed in the body, thus maintaining the stability of blood sugar in the body for a period of time, avoiding hypoglycemia and improving metabolic abnormalities in the body. The vast majority of carbohydrates eaten by children in one day must be replaced by raw corn flour. Infant’s diet is fed continuously for 8-10 hours with nasogastric tube at night to maintain blood sugar stability and avoid fasting for more than 4 hours. Pay attention to avoid eating foods containing sucrose, fructose, lactose and galactose, such as fruits, sweets, sugary drinks and commercial products containing milk; In the aspect of fat intake, it is necessary to limit the intake of saturated fatty acids, take more units and polyunsaturated fatty acids. Blood sugar should be maintained between 3.9 and 6.7 mmol/L, so as to ensure the normal growth and development of children.

　　On May 16th, Min County, Dingxi City, Gansu Province suffered from strong convective weather caused by heavy rain and hail. According to the latest report of Minxian Government Information Office, from 16: 00 to 20: 00 on May 16, there was a heavy rain in Minxian County, which caused six townships to be affected to varying degrees. As of 8: 00 on the 17th, the disaster has caused 7 deaths, 1 injured and hospitalized, and now it is out of danger. Beijing Youth Daily learned that at present, more than 3,200 people from 878 households have been evacuated from six affected towns.

　　tell about

　　The heaviest rainfall in more than ten years.

　　Mr. Liu, a villager in Suolongsigou Village, Minxian County, told the reporter of Beiqing Daily that the rain started at 6 pm on the 16th. "In the afternoon, I just got home from the field, and it rained, especially with lightning. It didn’t stop until around 8 pm. " Mr. Liu said that because the village is surrounded by mountains, when it rains, soil keeps flowing down from the mountains and finally collects in the rivers in the village. And this disaster also caused damage to their crops. "Our family planted 6 acres of medicinal materials, and 2 acres were completely flooded."

　　The rain in Min County also left a deep impression on the villager Shi Chuang. Shi Chuang, who lives near the county seat, said that at 4 o’clock in the afternoon, it began to hail. "The hail is particularly big, and it bounces in the car. Before long, the roads were covered with hail, like snow. After the hail, it turned into a heavy rain. "

　　"This is my impression, the biggest rain in more than a decade." Shi Chuang said that although he saw the warning of the weather forecast before the rainstorm, he didn’t expect it to be so big.

　　The reporter of Beiqing Daily learned that after the rain stopped, several villages in Suolong Temple had serious water accumulation. Local fire officers and soldiers and rescue teams, with shovels, are constantly cleaning up the accumulated water from the villagers’ yards.

　　notify

　　Seven people were killed and one injured in the disaster.

　　According to the latest report from the Information Office of Minxian County Government in Dingxi City, Gansu Province, from 16: 00 to 20: 00 on May 16, there was a heavy rain in Minxian County, causing six townships to be affected to varying degrees. As of 8: 00 on the 17th, the disaster has caused 7 deaths, 1 injured and hospitalized, and now it is out of danger.

　　According to the circular, in the afternoon, after receiving the early warning signals from the meteorological and land departments of cities and counties, the local government issued early warning information to the county nine times in time. A total of 878 households with more than 3,200 people were evacuated from six affected towns.

　　After the disaster, the local government immediately organized civil affairs, water affairs, meteorology and other departments to go deep into the affected towns such as Hetuo Town and Suolong Township to check the disaster situation and carry out rescue and relief work. Emergency mobilization of 25 medical personnel and 6 ambulance vehicles (times) to the severely affected towns and villages to carry out the rescue work for the injured. At the same time, more than 300 police officers, fire officers and soldiers, surrounding township cadres and local people were urgently dispatched to search for missing persons.

　　At the same time, quickly repair damaged infrastructure. As of 3: 00 a.m. on the 17th, some villages in Suolong Township resumed power supply, and villages that have not yet been powered on are stepping up repairs; The landslide section of national highway 316 in Suolong has been rushed through, and many water-damaged interrupted roads such as Dadong Village, Yuangengdi Village and Buyers Village in Suolong Township have been unblocked; The village road in Shangou Village, Hetuo Town is being repaired.

　　In order to properly arrange the lives of the affected people, as of 8: 00 on the 17th, the government has urgently mobilized relief tents, cotton-padded clothes, quilts, water shoes, umbrellas, flashlights, instant noodles, mineral water, snacks, candles, generators and other relief materials to the affected towns. At present, it is being distributed in an orderly manner. In addition, the government also distributed solatium 10,000 yuan to the families of the victims who have been identified.

　　affect

　　The direct economic loss is more than 10 million yuan.

　　According to the news released by the National Disaster Reduction Center of the Ministry of Civil Affairs, on May 16, due to the strong convective weather, some areas in Dingxi City and Longnan City of Gansu Province experienced short-term heavy rainfall, with the maximum precipitation of 101.2 mm, accompanied by local hail with the maximum diameter of 2cm, causing floods, winds and hail disasters.

　　The reporter of Beiqing Daily learned that according to preliminary statistics, 75,000 people in Minxian County of Dingxi City and Tanchang County of Longnan City were affected and 7 people died; The affected area of crops is 3.2 thousand hectares, of which 1.5 thousand hectares will be rejected; The direct economic loss is more than 10 million yuan.

　　After the disaster, the local government has organized personnel to guide and help the affected people to carry out disaster relief and self-help. Organize township cadres to go deep into village communities and farmers, quickly carry out disaster verification work, and arrange water affairs and land departments to conduct a comprehensive investigation of hidden dangers of geological disasters in the county to prevent secondary disasters.

　　At present, the work of checking the damage of people’s houses and family property, infrastructure and agricultural production is being carried out in a strong and orderly manner.

　　Text/reporter Ma Jinfeng