????The Pentagon, located in Arlington, southwest of Washington, D.C., is the headquarters of the United States Department of Defense, the highest military command organ in the United States, with geographical coordinates of 38 52 ′ 15.00 ″ n and 77 03 ′ 21.00 ″ w.. Seen from the air, the building is pentagonal, hence the name "Pentagon".

????Original title: The Department of Defense born in the "Air and Sea War"-The little-known inside story of the struggle when the US Department of Defense was founded

????On August 10th, 2009, the US Department of Defense held a grand celebration ceremony to celebrate the 60th anniversary of the official establishment of the Department of Defense. However, what is little known is that the establishment of the US Department of Defense 60 years ago was the product of struggle and compromise among the three major services of the US military, and one of the events called "Admiral Rebellion" became the direct "fuse" for the birth of the US Department of Defense. Then, in the United States, which flaunts democracy and freedom and pursues the system of "civilian leading", why did the admiral, as an important minister in the army "rebel"? Why is the birth of the Ministry of National Defense linked to this incident? Let’s break through the fog of history and appreciate the thrilling infighting within the US military 60 years ago.

????The Prelude of Conflict —— The National Security Law of 1947

????After World War II, with the rapid improvement of American military strength and international status, the organizational form of the US military, which used to focus on the two independent departments of the sea and land, obviously could not meet the needs of the development of the situation, so a new round of military institutional reform is imperative. In an article entitled "Our armed forces must be unified", Truman once wrote with emotion when talking about the experience and lessons of World War II, "The military institutions we hastily pieced together have always been the cause of disasters … For the sake of national security, a more solid foundation must be established. The first step is obviously to unify the army and navy, so that all our defensive and offensive forces are under the leadership of an institution and an authoritative and responsible headquarters. " Under this background, it has become the top priority of the US military’s military reform work to concentrate on solving the deep-rooted contradictions between Lu Haijun, solving the repetitive construction problems in national defense construction, and establishing a unified military organization to effectively integrate the two major military departments of the sea and land.

????In October, 1945, a motion led by the army with the main content of establishing a unified military organization was submitted to the National Assembly, and the focus of the struggle was mainly on the ownership of the Marine Corps and the Naval Air Force. However, judging from the comparison of the forces of the three major services of the US military at that time, the Army and the Air Force played a decisive role in World War II, so they also mastered the "right to speak" in the new round of national defense institutional reform. The army, headed by Marshall and Eisenhower, spared no effort to publicize the benefits of the alliance, hoping to merge the Marine Corps into the army and form its own amphibious combat force to further strengthen its leading position in the national defense institutions. At the same time, after the baptism of the two world wars, especially in the course of the war against Japan, the Army Air Force (the predecessor of the Air Force), which was "resident" in the army, has been transformed into a veritable "third service" step by step. Therefore, after the war, fanatical air force elements advocated the establishment of an independent air force everywhere, demanding that the aircraft of the army and navy be unified under the command of the Air Force. Based on the affection of "the same ancestry and the same origin", the Army and the Army Air Force reached a tacit agreement on the issue of dividing up the navy’s "heritage", that is, acquiring the Marine Corps and the Naval Air Force. Therefore, a large number of senior army generals, such as Eisenhower, all held a positive attitude towards the independence of the Air Force. Although the navy also played an important role in the Pacific War, the humiliation of Pearl Harbor incident, the nuclear monopoly advantage of the United States after the war and the serious lack of maritime strategic threats,Coupled with the strong pressure of "alliance" between land and air, these factors make the navy in a very embarrassing situation in the new round of national defense institutional reform. In order to publicize the benefits of air force independence and integration, and to retaliate against the numerous obstacles set by the navy on the issue of air force independence in the past, especially the persecution of Billy Mitchell, the "father of the air force", air force elements used their influence to belittle the role of the navy as much as possible everywhere. Frank Armstrong, the backbone of the Air Force, publicly demanded that the functions and functions of the navy be reduced to the level of escort, transport and honor guard, because the navy "will only have a few aircraft carriers, which will have no fighting capacity in any case … and will probably be sunk in the first battle", so "the navy will be useless except providing materials needed by the army, air force and ground forces." Karl spaatz, the first Air Force Chief of Staff, also attacked the Navy in public speeches, which made the Navy faction angry. He pointed out, "Why should we continue to keep the navy? The only reason why we keep the navy may be that many countries still keep the navy, but the Soviet Union has only a small navy, or there is no navy at all, so there is absolutely no need for us to waste money on the navy. "

????Under the dual pressure of "alliance" between land and air, although the navy is well aware of the significance and truth of unified operational command, it has to oppose it for its own interests, because the navy generals are particularly worried that the integration system and the formation of the air force will make them lose the two core arms of naval aviation and marine corps, thus damaging the overall interests of the navy. Therefore, chester nimitz, then Minister of Naval Operations, expressed strong opposition to the idea of integration. He pointed out that "practice has proved that the two departments with separate responsibilities and powers can provide different arms according to the requirements of modern warfare, so it is not only useless but also harmful to the proper division of labor and the effective use of maritime forces to merge the War Department and the Navy Department into a single department." At the congressional hearing, a large number of admirals and members of the navy expressed strong dissatisfaction with Truman’s bill, so they have not reached a consensus on the issue of integration.

????Finally, in order to calm the disputes between the various military forces, especially the dissatisfaction of the navy, Truman had to adopt a compromise plan and make necessary compromises and concessions on some key issues: first, adopt the opinions of the army and set up a unified Ministry of National Defense; Secondly, in response to the demands of air force activists, an independent air force was formed, and Lu Haijun did not keep land-based aircraft; Third, support the opinion of the navy, carrier-based aviation and marine corps will remain in the navy, and the establishment and tasks of the marine corps will remain unchanged. In this context, the US Army and Navy Ministers finally reached an agreement on the issue of unified jurisdiction. On July 26, 1947, President Truman signed the National Security Law, and established the National Security Council, the National Military Department (the predecessor of the Ministry of National Defense), the Air Force Department and the Joint Chiefs of Staff, and established a national military system composed of the Minister of National Defense, the Joint Chiefs of Staff and independent air force, army and navy departments. On September 15th, 1947, JamesVincentForrestal, a former Secretary of the Navy, took office and became the first Secretary of Defense in American history. Three days later, the US Air Force was formally established, and Stuart Semin, a leading figure who made great contributions to the independence of the Air Force, became the first air force minister. However, because the National Security Law of 1947 was established on the basis of a temporary compromise between various military services, compared with the rank and status of the Cabinet Departments of the three major military services, the National Military Department is not a Cabinet Department, but a loose alliance in name only, with only general coordination rights.In addition, with only a small number of staff, it is impossible to fully exercise the functions and powers of the commander-in-chief, and the bill does not clearly stipulate the specific tasks and division of roles of various military species. This congenital deficiency has also laid the groundwork for the outbreak of the largest dispute within the US military after the war.

????The war is rekindled-B-36 VS "Super Carrier"

????At the end of World War II, with the successful development of nuclear weapons and their decisive role in the war against Japan, the US military believed that nuclear weapons would become the "killer weapon" to determine the outcome of future wars, and strategic nuclear bombing would also become a decisive new operational mode in the future, thus making nuclear weapons and the newly formed Strategic Air Force (SAC) the priority targets of the US military’s strategic deployment and military funding. At the same time, the two major services, the air force and the sea force, have launched a new "infighting" for the dominance of the strategic air force. The focus of this struggle is mainly on the weapons development plans of the two major services: the navy’s CV-58 "United States" super aircraft carrier plan and the Air Force’s B-36 "flying arsenal" strategic bomber plan.

????With the help of their supporters, the navy and air force used their influence to attack and slander each other in some public and secret occasions. The navy, represented by Nimitz, believes that the "United States" is of great significance to the navy, because it is the first super aircraft carrier equipped by the navy after the war. This aircraft carrier has the ability to take off and land large aircraft such as strategic bombers, so it is also the navy to maintain its own survival in the nuclear age and compete with the air force for budgetary capital. The Air Force, represented by Stuart Semin and curtis lemay, believes that the US military can achieve a "cheap victory" only by launching nuclear air strikes, without having to carry out heavy casualties on the ground, and without wasting huge sums of money to build a "living coffin at sea"-an aircraft carrier with poor maneuverability and weak viability. As the world’s first strategic bomber with intercontinental long-range bombing capability, the B-36 will replace the old B-29 and B-56.

????However, because the Ministry of National Military Affairs is only a loose alliance, it is impossible to effectively stop the jealousness and infighting between the two ministries. In addition, the Truman administration greatly reduced the military budget after the war, so it is impossible for the two major R&D projects to be launched at the same time, and they can only choose one of them. Therefore, this inevitably intensifies the already tense contradiction between the two services. At first, because Forrester served as the secretary of the navy and was a typical naval faction, the navy gained the upper hand in the initial competition. The CV-58 project was successfully passed and started in Newport News on March 18, 1949. Many navy generals could not help but be complacent, but the navy was too happy. Stuart Semin, the eloquent air force minister, launched a comprehensive propaganda campaign, which gradually won the support of the press, Congress and the army. Bradley, then chairman of the Joint Chiefs of Staff, also expressed support for the Air Force, arguing that "using the navy to carry out nuclear bombing missions will only greatly reduce the ability and suddenness of the Air Force to carry out large-scale explosive missions." Things are unpredictable. Just a few days later, the "United States" was forced to stop construction and dismount because of the fall of the Navy and the coming to power of the Air Force. Due to the opposition to the Truman administration’s policy of reducing military spending, and the constant exposure of media reports about his poor mental condition, Forrester gradually lost Truman’s trust and had to resign on March 28, and the new defense minister was replaced by Louis Johnson, an activist from the Air Force, so the navy became a "super carrier."The plan was inevitably stillborn.

????"Admiral rebelled"

????In April 1949, after the new Secretary of Defense, Louis Johnson, took office, he not only canceled the "super carrier" plan without the approval of Congress, but also repeatedly suppressed the navy’s requirements at the Joint Chiefs of Staff, drastically reduced the navy’s funds, and even canceled the navy and the Marine Corps. Johnson pointed out, "There is no reason to keep the Navy and Marine Corps, because General Bradley told me that amphibious operations have become history, and we will never do amphibious operations again, so there is no need to keep the Marine Corps. In addition, the air force can do everything that the navy can do, so there is no need to keep the navy. " A stone stirred up a thousand waves, which caused an uproar in the United States and became the fuse of the famous "Revolt of the Admirals" in American history.

????A large number of famous naval generals protested and resigned in anger, and many retired naval generals, government and business people related to the navy, and officials and people from all walks of life who lost the location of the shipyard were extremely angry at the Truman administration’s capricious policy. Admiral Daniel Garrett, headed by Major General, began to publish a series of fierce articles and speeches in the United States, attacking Johnson for belittling the navy’s national defense policy and the Air Force’s B-36 bomber plan. Garrett even published a highly inflammatory rebellion statement in an article entitled "Don’t let them sink the navy", calling Johnson an "out-and-out criminal and villain" and demanding that he immediately take the blame and step down. This has caused an uproar in the United States, causing a storm in the city and chaos in Congress, the press, the government and the military. Subsequently, the senior navy generals represented by Admiral Arthur Redford, commander of the Pacific Fleet, began to set off a bigger storm again.

????In order to go all out to attack the Air Force’s B-36 bomber plan by hook or by crook, the Navy once appointed arleigh burke to secretly set up a special office called OP-23, which was mainly responsible for making plans to win the support of the US Congress and the public. In June 1949, an anonymous document not only recorded that Denfield and other senior naval generals expressed strong dissatisfaction with Johnson’s national defense policy, but also accused the relevant officials of the Air Force Department and senior air force generals of fraud and bribery in the B-36 plan, exposing that Johnson and Symington benefited personally and politically from the owner of the aircraft manufacturer, Odlum, and believed that the Air Force had unfair competition in the bidding stage of aircraft, and received defective aircraft to make personal profits at the expense of national defense interests. This accusation makes the Air Force extremely angry, because it has done great damage to the honor of Air Force officers and the reputation of the Air Force Department. Congress launched a series of hearings to investigate the so-called "B-36 procurement fraud case". At the congressional hearing, Air Force Minister Semin Clinton and Air Force Chief of Staff arthur vandenberg refuted Radford’s point of view one by one. After investigation, Congress also thought that many accusations of the navy lacked rationality and appropriateness and were untenable. Bradley, the general of the army, also refuted the accusation of the navy, and pointed out that the naval disturbance was an "open rebellion against the civilian leadership system", and its essence was to resist the unified command and plan of the US military "in spirit and action".And "the constant quarrel in the supreme command organization is like a drop of poison dripping into wine, which makes all the sharers suffer." After an in-depth investigation, the congressional investigation report pointed out that "there is not a bit, even a little bit of evidence to prove the allegations of premeditated, fraudulent, bribery, bullying or favoritism in the procurement of B-36 bombers" and proved that the "documents" provided by the navy were forged, and the initiator behind it was Colonel John G. Grommelin, deputy director of the OP-23 office. The report also pointed out that "the Ministry of National Defense has made great efforts to pursue the’ unification’ of the services, and the pace is too fast." Moreover, in this struggle, "the navy that refused the’ marriage’ of the services, the army that was too proud of itself, and the somewhat unruly air force made the Committee not find any’ Puritans’ in the Ministry of National Defense who fully supported reunification and had pure motives."

????In the end, this "air-sea war" ended with the navy’s breach of trust in the world and the air force’s victory: the B-36 plan continued, but the navy’s "super carrier" plan was forced to dismount, and an article in Time magazine of the United States also dubbed the ups and downs of the service struggle within the US military as the "Admiral Rebellion" incident. The "Admiral Rebellion" incident not only shocked the whole United States, but also made the top officials of the United States realize the importance of strengthening the authority of the Ministry of National Defense. In this context, the US Congress passed the amendment to the National Security Law on August 10th, 1949, which renamed the loosely structured "National Military Department" as the "Ministry of National Defense", upgraded it to the Cabinet Department, and further strengthened the power of the Minister of National Defense, reducing the ranks of the three major military departments of land, sea and air from the Cabinet Department to the directly affiliated department of the Ministry of National Defense, and the Minister of Military Services is no longer a member of the National Security Council and the Cabinet. In addition, in order to limit the competition between services, the post of Chairman of the Joint Chiefs of Staff was established with the approval of Congress. As the chief military adviser to the President and the Secretary of Defense, it was held by senior military generals, which also laid the foundation for the modern defense commander system in the United States.

????Silent ending

????Finally, the navy became a complete loser in this struggle: Navy Minister Sullivan resigned; Naval Operations Minister Denfeld was forced to "retire early"; Garrett was tried by a military court, which completely ruined his future; Truman also chose the mediocre "good guy" Matthews and the more obedient forrest sherman as the secretary of the navy and the minister of operations respectively, regardless of the strong opposition of the navy generals. Forester, the former secretary of defense who was born as the secretary of the navy, also committed suicide because of the mental disorder caused by the intensified service struggle. Although the personal endings of these senior naval officers were not very good, they were regarded as heroes by the navy because they sacrificed their lives and future to safeguard the survival and honor of the navy in this struggle. After the "Super Carrier" plan died, the US Congress decided to build a smaller aircraft carrier for the navy in order to appease the navy’s mood, and this aircraft carrier was named "Forrester". In addition, although the "Admiral Rebellion" incident ended in the failure of the navy, the subsequent "counterattack" by the Marine Corps also made the navy regain some face. In August 1950, under the strong pressure of the navy faction in Congress, the Truman administration was finally forced to agree to set up a representative of the Marine Corps in the Joint Chiefs of Staff. Although the Marine Corps is still under the jurisdiction of the Navy, it has a place in the Joint Chiefs of Staff, so it has become the fourth largest service on an equal footing with the army, navy and air force.

????At the same time, another protagonist in the "naval rebellion", Defense Minister Johnson of the Air Force faction, also suffered a bad result because of his arrogant and tough treatment of the navy. After the "Admiral Rebellion" incident, Truman, as Johnson’s immediate superior, was also humiliated in Congress, government and army, so he gradually lost his trust in Johnson. He wrote in his private diary, "Everything that happened made me begin to believe that Potomac fanaticism and a morbid state have made Louis show a mental disorder and selfish arrogance trying to drive the whole government, because he offended everyone in the cabinet … and never missed every opportunity to speak ill of people around me." Secretary of State Acheson also said in his memoirs that Johnson’s behavior was "very hateful, and it was not enough to just swear." Bradley hit the nail on the head and pointed out that "Truman inadvertently appointed a mental patient to replace another mental patient." Because Johnson suppressed the navy too hard, the navy did not have enough ships to carry out the task of sea blockade in the subsequent Korean War. A year later, in August 1950, Truman dismissed Johnson and appointed george marshall, who was low-key, well-balanced and highly respected in the military, as the new Secretary of Defense. At this point, the "Admiral Rebellion" incident, which caused a lot of trouble in the city, finally came to an end.

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.

Things seem a little sudden.

At 4: 30 pm on May 8, 2015, Peng Lianghao, vice mayor of jurong city, made an appointment with Ren Ding (a pseudonym), president of Nanjing University, at a tennis court near the exit of Jurong City on Shanghai-Nanjing Expressway. But it’s not playing ball.

Peng lianghao

Unlike the usual meeting, Peng Lianghao looked a little nervous. He told Sting Ren that if anyone investigates his daughter’s going to college, it should be in accordance with the agreed terms.

A few hours later, Peng Lianghao was taken away by the procuratorate.

What the procuratorate wants to talk to him is far more than the fact that his daughter goes to school. The vice mayor of jurong city, who had been in charge of planning approval and engineering construction for a long time, came to an abrupt end on this day.

From May 24 to June 17, 2016, after four days of trial, the case is about to enter the sentencing period.

Recently, The Paper (www.thepaper.cn) learned the above details according to the relevant materials of Peng Lianghao’s case. With the public hearing of this case, more details about Peng Lianghao’s bribery case have gradually surfaced.

"lightning falls"

On the day of being taken away, Peng Lianghao also attended a public event in Jurong normally, which can be described as "lightning falling".

According to public reports, on May 8, 2015, jurong city Industry and Commerce, Quality Inspection and Food and Drug Administration were formally merged and listed, and jurong city Market Supervision Administration was established. Jurong Mayor Xu Wen and Vice Mayor Peng Lianghao attended the inaugural meeting and unveiled it.

Jurong is a county-level city managed by Zhenjiang City, Jiangsu Province. As the southeast gateway of Nanjing, Jurong is known as "the new eastern suburb of Nanjing and the Royal Garden of Jinling".

Peng Lianghao is a cadre who grew up in Jurong. According to public information, Peng Lianghao graduated from Shenyang Institute of Civil Engineering and Architecture, and worked in the planning and urban construction of Jurong for a long time.

In 2001, Peng Lianghao became the deputy director of jurong city Construction Bureau, in 2004, he was transferred to the planning director of jurong city, and in 2007, he became the deputy mayor of jurong city until he was dismissed.

As a deputy mayor, Peng Lianghao was mainly in charge of tourism bureau, health bureau and education bureau before his fall. Before that, Peng Lianghao had been in charge of urban and rural planning, housing and urban and rural construction for a long time.

The Paper reporter noted that Peng Lianghao was directly taken away by Zhenjiang City Procuratorate that day, and did not pass the usual "pre-processing" procedure of Zhenjiang City Commission for Discipline Inspection.

Pulled out by another vice mayor

Chen shanle

Although Peng Lianghao is a "lightning horse", the outside world seems very sudden, but in fact there are signs.

On January 4th, 2015, Chen Shanle, then vice mayor of jurong city, was put on file for investigation on suspicion of accepting bribes. According to the relevant materials of the Anti-Corruption Bureau of Zhenjiang Procuratorate about Peng Lianghao’s case, it was in the process of checking a briber in Chen Shanle’s bribery case on April 24, 2015 that Zhenjiang People’s Procuratorate accidentally discovered that Peng Lianghao, the deputy mayor of jurong city, was also suspected of a major crime and accepted bribes from him many times.

At the same time, the prosecution also found that Peng Lianghao was also suspected of taking bribes of RMB 1 million in the process of his female "trick" entering Nanjing University.

Two weeks later, Zhenjiang City Procuratorate officially filed a case against Peng Lianghao for suspected bribery.

According to the relevant materials of Peng Lianghao’s case, Ren Ding, the person involved and director of Nanjing University, confessed that Peng Lianghao called Sting Ren at four o’clock in the afternoon when he was taken away, and asked him to meet him at the tennis center in front of the exit of Jurong City on Shanghai-Nanjing Expressway for about 20 minutes.

Peng Lianghao told Sting Ren that a boss had an accident in Jurong recently, and several city leaders were very nervous.

1 million "Nanda point recruitment fee"

Peng Lianghao specifically mentioned his daughter’s "little trick" to go to school in Nantah. "He reminded me again that his daughter’s recruitment to Nantah should be in accordance with the agreed statement." Sting Ren said.

The so-called "point-and-click" means that as long as the score of the college entrance examination reaches a line, as long as there are some outstanding experts such as the school board, and a certain "point-and-click fee" is paid, a candidate will be guaranteed to enter a famous school.

According to Peng Lianghao’s account to the procuratorate, her daughter took the college entrance examination in June 2012, and her score reached one line. After that, she successfully entered Nantah through the recommended quota of Sting Ren, and Gao Mouyun, a local real estate owner in Jurong, paid a "point recruitment fee" of 1 million yuan for her, and directly paid the money to Sting Ren.

Peng Lianghao met Sting Ren at a dinner organized by Yang Ju, then head of Jurong City Investment Company.

In May 2013, Yang Ju was taken away for investigation. In order not to affect himself, Peng Lianghao called Gao Mouyun, the chairman of the developer, home and told him. If anyone asks, he said that the 1 million yuan that Gao Mouyun called Sting Ren was borrowed from Gao Mouyun by Yang Ju.

When he went to the prison to see Yang Ju, Peng Lianghao told Yang Ju that the money that Yang Ju owed Gao Mouyun had been paid back. "This is to unify the caliber with Yang Ju and cope with possible investigations." Peng Lianghao confessed.

One of Peng Lianghao’s immediate family members told The Paper that according to his understanding, Peng knew that he had to pay 1 million yuan, but he never received a notice to pay the money. It was not until after Yang cited it in 2013 that he realized that the money had already been paid by Gao Mouyun, so he was very nervous at that time.

Accused of "eating all the developers"

Jurong

Although Jurong is a county-level town in the south of the Yangtze River, it is close to Nanjing, the provincial capital. In recent years, urban construction projects have blossomed everywhere.

As an official who has been deeply involved in Jurong urban construction and planning, Peng Lianghao was accused of accepting bribes from several developers. According to the prosecution’s allegations, Peng Lianghao accepted bribes from 25 developers or builders, large and small, totaling over 10 million yuan.

According to the prosecution’s allegations, from the end of 2005 to January 2013, Peng Lianghao illegally accepted RMB 2,403,500 from Fang Moufu, the chairman of a tourism development company in Jiangsu, nine times, providing assistance for the planning approval of some projects of the company where Fang Moufu worked. From 2003 to 2012, Peng Lianghao received 1.47 million yuan in cash and gift cards from Gao Mouyun, the project contractor, for 13 times to help Gao Mouyun undertake the hydropower installation project.

During the construction of a residential area, Peng Lianghao even personally came forward to coordinate the demolition work of several nail households.

"In this regard, we are grateful to Peng Lianghao. He did not deliberately set a threshold for us in the planning plan we reported." Li, the person in charge of the contractor of the community, explained that since then, they have adjusted the plot ratio of the community, and the construction area has increased by about 20,000 square meters, so that they can earn more than 30 million yuan. "With Peng Lianghao’s concern, the floor area ratio adjustment was successfully approved." Li said.

According to the investigation by the prosecution, from 2010 to 2013, Li paid bribes to Peng Lianghao four times, totaling 190,000 yuan.

There is also a religious figure in Jurong, Yang, who paid a bribe of 50,000 yuan to Peng Lianghao in order to thank him for his help in the renovation of the top palace of Maoshan Daoyuan.

According to the religious personage, at first he prepared 100,000 yuan, but Peng Lianghao refused to accept it anyway. "I told him that this is the money to receive wealth, and it must be collected, so he took half." The source said.

The Paper reporter noted that among the 25 developers or builders who paid bribes, more than 20 were the chairmen or general managers of various construction and real estate development companies in Jurong. Many reasons for bribery are to provide convenience for project planning and approval, not to be difficult, and to do a good job in daily relations.

The responsible person of jurong city Urban Construction Bureau confessed that,As the deputy mayor in charge of urban construction and planning, Peng Lianghao holds the power of life and death. The implementation of a project must finally be signed by the deputy mayor in charge. For major and special projects, the deputy mayor in charge may ask the secretary and mayor for instructions, but in the end, the deputy mayor in charge will sign the approval..

"self-evaluation"

Peng Lianghao’s place to receive property is mainly the government office.

According to the prosecution’s allegations, the briber Wang paid a total of 750,000 yuan for 11 times, and 10 times he sent the money in a paper bag to Peng Lianghao’s deputy mayor’s office. The briber Lei paid a total of 750,000 yuan for 8 times, all of which happened in Peng Lianghao’s office.

These bribers are familiar people, but they are not "iron buddies".

Peng Lianghao’s residence (home) is another major place for bribery. The prosecution said that Gao Mouyun, a developer friend whom Peng Lianghao had known for many years, paid bribes 12 times in Peng Lianghao’s home. Of Peng Lianghao’s tens of millions of assets, five million were placed in a developer who was familiar with him, and more than five million were placed in his younger brother.

In May 2015,Peng Lianghao made a self-evaluation when he was tried. He said that he was in a hot post and had rejected the temptation of many possessions. "If I let go, it will not be a problem to charge six or seven hundred thousand yuan a year for the Spring Festival and Mid-Autumn Festival."

Peng Lianghao said that he had no special hobbies, didn’t take a shower outside, didn’t have a driver’s license, never drove alone for more than 10 years, had no valuables at home, had an average decoration grade, and didn’t speculate in real estate. However, in terms of collecting cash, "I can’t help myself. I have collected huge bribes for many years. Since I filed an investigation, I have learned from a painful experience and only tried to make a complete me."

Like many officials under investigation, Peng Lianghao did not plead guilty quickly. According to the relevant materials of Peng Lianghao’s case, during the examination, Peng Lianghao did not "tell the whole story" about his behavior, but also had some struggles.

In July 2015, Peng Lianghao once confessed that all the criminal facts he had previously confessed were false. "I have never taken any money from others." He changed his mind and said that the assets at home were normal. At the same time, he also accused the procuratorate of "extorting a confession from him", which led him to be forced to account for taking bribes of more than 10 million.

The old man drowned

In the bribery list, Peng Lianghao once accepted four bribes totaling 420,000 yuan from Ding Moshun, the head of an architectural design company in Jurong, all in Peng Lianghao’s municipal government office.

Unlike ordinary developers, Ding Moshun previously served as the deputy director of jurong city Planning Bureau, and was an old subordinate of Peng Lianghao.

In 2009, Ding resigned from the Planning Bureau and set up a design company. At first, he was mainly engaged in architectural design, and three years later, his business scope was increased by planning and design.

According to Ding Moshun, in the planning and architectural design process of a resettlement housing project, Ding Moshun once looked for Peng Lianghao, hoping to do these businesses. Finally, Ding Moshun also successfully won the business.

Ding Moshun confessed that during the three years from 2011 to 2013, Ding Moshun went to the office of Vice Mayor Peng Lianghao three times and sent a total of 400,000 yuan in cash.

Surprisingly, after accepting the investigation by the prosecution and explaining the bribery, Ding Moshun drowned.

The Paper reporter learned from many insiders that Ding Moshun drowned in Gexian Lake, a famous scenic spot in Jurong, in the early morning of October 10, 2015, and was found at 6: 00 the next morning.

There is no relevant public report on Ding Moshun’s death. Two people familiar with the relationship with Ding Moshun told the The Paper reporter that more than a month before his death, Ding Moshun had told them angrily that he blamed himself for Peng Lianghao’s case and "wanted to stand up and sacrifice himself".

Two people familiar with the matter said that they didn’t care at the time, but I didn’t expect Ding Moshun to really die.

The Paper has not confirmed that Ding Moshun’s death is related to Peng Lianghao’s case or Zhenjiang City Procuratorate. However, according to the materials of Peng Lianghao’s case, on the afternoon of October 10th and 11th, that is, shortly after Ding Moshun’s death, a member of Zhenjiang CPPCC and a representative of Zhenjiang Municipal People’s Congress, who had been questioned by prosecutors before, were once again called in for questioning.

The testimony of the two witnesses is roughly the same as the previous two, but in the last part of the transcript, two questions were added.

One question is, was the process of questioning you by the procuratorate standardized? Both witnesses gave a positive answer.

Another question is, did you strictly abide by the time limit for questioning witnesses during the inquiry?

Both witnesses replied that they had not been questioned for more than 12 hours. After discussing the problem clearly within a few hours, they signed it and went back without delaying the work and life of the witnesses.

Suddenly "postponed the trial"

Peng Lianghao’s bribery trial site. Oriental IC data

On June 17, 2016, Peng Lianghao’s bribery case was heard by Zhenjiang Intermediate People’s Court for the last time. Previously, the case was heard on May 24th, May 25th and June 8th of the same year.

The indictment of Zhenjiang People’s Procuratorate alleges that from 1999 to 2014, Peng Lianghao took advantage of his position as deputy director of jurong city Construction Bureau, director of Planning Bureau and deputy mayor of jurong city Municipal People’s Government to seek benefits for others in planning approval, project undertaking and project payment, and illegally accepted other people’s property totaling RMB 11,953,500.

Due to the seriousness of the case, the court announced an adjournment after the trial on June 17.

On June 20th this year, Zhenjiang Intermediate People’s Court decided to postpone the trial of the defendant Peng Lianghao’s bribery case.

According to the personnel involved in the trial, Peng Lianghao had made a final statement at the trial site, and according to the process, he "waited for the verdict".

People in the legal profession told The Paper that it is not uncommon for the court to postpone the trial when hearing a case, but it is still rare to postpone the trial on the eve of the final statement made by the defendant. "It may be that the case is extremely complicated, the prosecution may find some flaws or mistakes, or the court may find it extremely difficult." The reason for this is still unknown.

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　　　　Hot models are a hit.

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　　　　The appearance is bright and cross-border

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