Underdevelopment or underdevelopment of the lungs

　　One, Etiology

　　During the process of embryonic development, a stage of lung bud development produces a barrier, leading to underdevelopment and underdevelopment of the lungs. Most of them are accompanied by other developmental defects, common ones include underdevelopment and absence of the trachea, bronchi, and pulmonary artery, and abnormalities in vertebral development. Abdominal visceral organs pass through the diaphragm and diaphragmatic peritoneum into the pleural cavity, these malformations are all competing for space in the pleural cavity with the lungs. 50% to 80% of infants with congenital diaphragmatic hernia die of pulmonary dysfunction, mainly due to congenital underdevelopment of the lungs.

　　Two, Pathogenesis

　　Underdeveloped or underdeveloped lungs can be divided into the following pathological types:

　　1. Pulmonary hypoplasia, which is the developmental disorder of the lung substance, common types include underdeveloped lung tissue with congenital diaphragmatic hernia, absent pulmonary artery, spheroid lung with bronchial malformation.

　　2. Lung lobe absence: One or more lobes are absent, commonly the right middle lobe and right lower lobe. The mediastinum shifts towards the affected side, and the remaining lung on the affected side often has other malformations.

　　3. Unilateral lung absence is due to developmental disorders of one lung bud, which can be bronchial atresia, with no lung tissue and pulmonary vessels at the distal end; another part is manifested as bronchial stenosis and emphysema of the distal lung tissue, which is due to the absence of the main bronchus formed by the gas entering the lung tissue lacking pulmonary vessels through the lateral bronchus, leading to secondary lung absence. The left side is significantly more than the right side, due to the inability of the remaining lung to compensate for ventilation, most children die in infancy, and there are reports that a few can live to over 60 years old without obvious clinical symptoms.

　　4. Both lungs absent: The lung bud does not develop in the embryonic stage, the disease is rare, the fetus often has malformations of the heart and other organs, most of them die and are aborted in utero, and a few can have respiratory struggles after birth but cannot maintain life.

　　Survivors often have growth and development delays, residual chronic asthma, pulmonary fibrosis, poor lung function, pulmonary heart disease, and complications of the nervous system such as intellectual disability and cerebral palsy.

　　1. Intellectual disability:During the period of development, the general intellectual function is significantly lower than that of peers, accompanied by a group of diseases with adaptive behavior defects. The intelligence quotient (IQ) is below 2.0 standard deviations from the population mean (the population's IQ mean is set at 100, and the IQ value of one standard deviation is 15). Generally, an IQ below 70 (or 75) indicates significantly lower than the average level.

　　2. Cerebral palsy:It usually refers to central motor disorders caused by non-progressive brain injury or brain maldevelopment due to various reasons within one month before and after birth

　　Repeated respiratory tract infections are the main reason for seeking medical attention. Patients with unilateral lung underdevelopment often have slight dyspnea and poor physical and endurance. Some patients may cough blood due to collateral circulation from the systemic circulation. Patients with respiratory tract infection have worsening dyspnea, cyanosis, rough breathing sounds, delayed growth and development, and may have symptoms of heart, skeletal, or other organ malformations. The chest of patients is often without malformation, bilateral symmetrical or nearly symmetrical. The respiratory movement on the affected side is weak, the breathing sounds are weakened or disappeared, and percussion can be dull or very light, without specificity. Patients with associated chest malformations often have corresponding signs. Patients with absent pulmonary lobe have fewer clinical symptoms, and the condition is concealed. Physical examination only shows reduced respiratory sounds on the affected side. Without X-ray and other examinations, it is easy to miss the diagnosis. The above types may have rough breathing sounds and rales on the affected side if accompanied by lung infection.

　　Repeated respiratory tract infections should consider the existence of congenital pulmonary underdevelopment. Diagnosis must be very cautious. X-ray examination is the first choice. If it cannot be diagnosed, bronchography or pulmonary artery perfusion scan can generally clarify the cause.

　　Most congenital malformations are generally untreatable once they are born, so preventing the birth of patients is very important. It mainly relies on premarital physical examination and prenatal diagnosis. This is a comprehensive and systematic health check-up, mainly including a comprehensive physical examination, genetic health inquiry, and post-marital sex education. Among them, genetic counseling and genetic testing are important contents.

　　1. X-ray examination:On the X-ray chest film, one lung underdevelopment shows uniform and dense density on the affected side of the thoracic cavity, lacking aerated lung tissue, bronchial shadows, and blood vessel streaks. The heart and mediastinal structures are shifted towards the affected side, and the diaphragmatic surface on the affected side is unclear. The opposite normal lung shows varying degrees of compensatory emphysema, leading to the descent of the diaphragm and flattening of the diaphragmatic surface. Overly expanded lungs can form mediastinal hernias. If there is also a hemivertebra畸形 in the spine, it is very helpful for diagnosis. Some part of lung maldevelopment can be shown on the X-ray chest film as aerated lung tissue, but with sparse pulmonary vessels, which sometimes may be mistaken for bronchitis or bronchiectasis on the healthy side. Special attention must be paid to this.

　　2. Bronchography:It can show the absence of the main bronchus on the affected side, as if the trachea is directly connected to the main bronchus on the other side, or the main bronchus is malformed due to underdevelopment, or the number of bronchial branches is sparse. Pulmonary angiography examination can show underdevelopment or absence of the main pulmonary artery on the affected side, which is helpful for diagnosis.

　　3. Pulmonary artery perfusion scan:The affected side shows reduced or significantly reduced pulmonary blood flow.

　　One, Dietotherapy for the underdevelopment or maldevelopment of the lung

　　1. Fritillary Bulb and Pear Pork Lung Soup

　　Take 120 grams of pork lung, clean and slice, boil in water for 5 minutes, then rinse with cold water. Clean and crush 9 grams of fritillary bulb; clean the pear with the skin, remove the stem and core, and cut the pear meat into small pieces with the skin. Put all the materials in a pot of boiling water, simmer over low heat for 2 hours, season to taste, and drink as desired.

　　2. Almond and Pear Yam Paste

　　Take 10 grams of almond, one pear, appropriate amounts of yam powder and brown sugar. First, soak the almond in boiling water, remove the skin, and clean it; peel, clean, and cut the pear into pieces. Then put the almond and pear pieces in a blender, blend into a paste. Mix the almond paste, pear paste, yam powder, and brown sugar with an appropriate amount of water to form a paste, pour it into a pot of boiling water (about 100 milliliters of boiling water), stir continuously, and cook until done. Eat as desired.

　　3. Winter Mushroom and Silver Ear Pork Pancreas Soup

　　4. Other:Take one pig pancreas, 60 grams of lean pork, 15 grams of winter mushroom, and 9 grams of silver ear. First, clean the winter mushroom; soak, rinse, and pick small pieces of silver ear; clean and slice the pig pancreas and lean pork. Then put the winter mushroom and silver ear in the pot, add an appropriate amount of water, boil over high heat, then simmer over low heat for 20 minutes, add the pig pancreas and lean pork, boil again, and season to taste. Drink as desired.

　　2. What to eat for pulmonary hypoplasia or hypoplasia that is good for the body

　　1. Eat more fresh fruits and vegetables. For example: pear, grape, jujube, pomegranate, tangerine, sugarcane, persimmon, lily, radish, water chestnut, silver ear, etc.

　　2. Eat more traditional Chinese medicine that promotes lung fluidity, expectoration, nourishing Yin and benefiting Qi, such as ginseng, schisandra, American ginseng, lily, almond, and fritillaria.

　　3. It is best not to eat anything for pulmonary hypoplasia or hypoplasia.

　　1. Avoid spicy foods such as chili.

　　2. Avoid smoking and drinking.

　　Treatment

　　Pulmonary hypoplasia without obvious clinical symptoms does not require any treatment. If there are recurrent hemoptysis or pulmonary infection, even developmental delay, and if there are bronchial or vascular malformations in the residual lung, lobectomy or pneumonectomy must be performed. However, pneumonectomy should be done very cautiously, and it must be confirmed that the healthy lung function is completely normal, otherwise it may cause disability or even death. Pay special attention to anatomical variations during surgery and do not damage surrounding organs. If there is a congenital abnormality of the heart or large blood vessels, a full preoperative evaluation is required, and correction may be performed during surgery if necessary.