Neuroendocrine hyperplasia | |
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Other names | Neuroendocrine cell hyperplasia of infancy |
Specialty | Pulmonology |
Neuroendocrine hyperplasia is rare and poorly understood lung condition which is characterized by an abnormal growth of pulmonary neuroendocrine cells in the lungs. It is a non-progressive disease of the interstitial tissues of the lungs. Prior to the findings of the hyperplasia of neuroendocrine cells it was known as tachypnea of infancy, as most children outgrow the need for oxygen supplementation within two to seven years. It is characterized by tachypnea, hypoxemia, and retractions. [1] It is typically diagnosed in infants and children younger than one year of age. [2] There is no currently recognized treatment, infants and children are given oxygen supplementation until they outgrow the need; since neuroendocrine cells do not multiply or get larger in size while the lungs continue to grow. This allows the lung disease to have less effect on lung function with age, although they will always have the same amount of neuroendocrine cells as they were born with.
People with this diagnosis may have no obvious symptoms, they may present with shortness of breath or wheezing. Infants and children present with symptoms of heavy breathing at a rate greater than 20 breath/min. Oxygen levels are lower due to hypoxia, and chest x-rays show signs of pneumonia. NEHI typically presents in otherwise healthy infants during the first months to year of life. Severe cases may risk permanent damage to the lungs and death from severe tachypnea.
There is not cause of Neuroendocrine hyperplasia, however some known causes are a rapid increase of pulmonary endocrine cells in the lungs in children under the age of 2. An increase in pulmonary endocrine cells is usually seen in adults with a history of smoking, COPD, or cystic fibrosis. Children under the age of 2 may present with signs of interstitial lung disease and be diagnosed with NEH following severe progression. [2]
Neuroendocrine hyperplasia is a rare condition amongst chILD. This condition is characterized as an overgrowth of pulmonary endocrine cells in the lungs. These cells receive signals from neurons to produce hormones. With this rapid increase of PNC (pulmonary endocrine cells), this can affect the airways of children. [3] Furthermore, this increase can be a precursor of pulmonary carcinoid tumors. [4]
To diagnose neuroendocrine hyperplasia after a referral is made for fast breathing (tachypnea) or need for extra oxygen. There are several tests that are commonly performed to confirm the diagnosis. [2] Chest CT gives a better look at the lungs to see signs of pneumonia. [5] With a bronchoscopy, a scope (small camera) is passed from the mouth or nose, through the windpipe, and into the lungs to check for other causes of breathing problems. [5] A lung biopsy may be the only way to diagnose the disease if the chest CT does not show the characteristic findings. In a biopsy, a small portion of lung tissue is removed to determine if lung disease is present.
There is no consensus on the therapy for NEHI, and management generally consists of supportive care: supplemental oxygen for chronic hypoxemia, adequate nutrition, proper immunization, avoidance of environmental pollutants, and treatment of recurrent infections . [1] To relieve symptoms of NEH, there are not any methods yet proven effective in infants. [2] Supportive care and adequate nutrition may be considerate in improve quality of life as NEH in most cases is not treatable. [2]
Most outcomes in neuroendocrine hyperplasia leads to failure to thrive due to the restrictions of oxygen flow in lungs. [5] The long-term outcome of NEHI is generally favourable with most patients gradually improving over time, although persistent airway obstruction mimicking severe asthma and relapse with respiratory infection. [4]
The incidence and prevalence of NEHI are unknown, but it is clearly rare. Available data derive from small to moderate sized case series. The original report of this disorder in 2005 included 15 cases. [4] A study from a large referral center identified 19 cases (14 percent) from among 138 lung biopsy cases accrued over a 10-year period. Twenty-three NEH cases were included in a separate study testing chest CAT scan. The largest report to date includes 37 cases in a manuscript focusing on infant pulmonary function testing (PFT)
The research being done on neuroendocrine hyperplasia consists of a criterion to distinguish its characteristics from similar cHILD cases. [6] A recent study in November, 2020 helped identify pathologic features of NEH, and used clinical patients to support their data. [7] Another study reviewed the various supplemental oxygen use in NEH patients. They identified factors in NEH to help in clinical course. As well as, reviewed failure to thrive patients who would have an increased use of supplemental oxygen. [8] Other than its associations with chILD, researchers do not know much about this condition. [1]
Diffusing capacity of the lung (DL) measures the transfer of gas from air in the lung, to the red blood cells in lung blood vessels. It is part of a comprehensive series of pulmonary function tests to determine the overall ability of the lung to transport gas into and out of the blood. DL, especially DLCO, is reduced in certain diseases of the lung and heart. DLCO measurement has been standardized according to a position paper by a task force of the European Respiratory and American Thoracic Societies.
Asbestosis is long-term inflammation and scarring of the lungs due to asbestos fibers. Symptoms may include shortness of breath, cough, wheezing, and chest tightness. Complications may include lung cancer, mesothelioma, and pulmonary heart disease.
Transient tachypnea of the newborn is a respiratory problem that can be seen in the newborn shortly after delivery. It is caused by retained fetal lung fluid due to impaired clearance mechanisms. It is the most common cause of respiratory distress in term neonates. It consists of a period of tachypnea (rapid breathing. Usually, this condition resolves over 24–72 hours. Treatment is supportive and may include supplemental oxygen and antibiotics. The chest x-ray shows hyperinflation of the lungs including prominent pulmonary vascular markings, flattening of the diaphragm, and fluid in the horizontal fissure of the right lung.
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium and space around the alveoli of the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.
A chest radiograph, called a chest X-ray (CXR), or chest film, is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in medicine.
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Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a syndrome caused by the repetitive inhalation of antigens from the environment in susceptible or sensitized people. Common antigens include molds, bacteria, bird droppings, bird feathers, agricultural dusts, bioaerosols and chemicals from paints or plastics. People affected by this type of lung inflammation (pneumonitis) are commonly exposed to the antigens by their occupations, hobbies, the environment and animals. The inhaled antigens produce a hypersensitivity immune reaction causing inflammation of the airspaces (alveoli) and small airways (bronchioles) within the lung. Hypersensitivity pneumonitis may eventually lead to interstitial lung disease.
Pneumonitis describes general inflammation of lung tissue. Possible causative agents include radiation therapy of the chest, exposure to medications used during chemo-therapy, the inhalation of debris, aspiration, herbicides or fluorocarbons and some systemic diseases. If unresolved, continued inflammation can result in irreparable damage such as pulmonary fibrosis.
Hypoxemia is an abnormally low level of oxygen in the blood. More specifically, it is oxygen deficiency in arterial blood. Hypoxemia has many causes, and often causes hypoxia as the blood is not supplying enough oxygen to the tissues of the body.
Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia (BOOP), is an inflammation of the bronchioles (bronchiolitis) and surrounding tissue in the lungs. It is a form of idiopathic interstitial pneumonia.
Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.
Bronchopulmonary dysplasia is a chronic lung disease in which premature infants. Premature (preterm) infants who require treatment with supplemental oxygen or require long-term oxygen are at a higher risk. The alveoli that are present tend to not be mature enough to function normally. It is also more common in infants with low birth weight (LBW) and those who receive prolonged mechanical ventilation to treat respiratory distress syndrome. It results in significant morbidity and mortality. The definition of bronchopulmonary dysplasia has continued to evolve primarily due to changes in the population, such as more survivors at earlier gestational ages, and improved neonatal management including surfactant, antenatal glucocorticoid therapy, and less aggressive mechanical ventilation.
Idiopathic pulmonary fibrosis (IPF), or (formerly) fibrosing alveolitis, is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism.
Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the pulmonary interstitium. UIP is thus classified as a form of interstitial lung disease.
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Rheumatoid lung disease is a disease of the lung associated with RA, rheumatoid arthritis. Rheumatoid lung disease is characterized by pleural effusion, pulmonary fibrosis, lung nodules and pulmonary hypertension. Common symptoms associated with the disease include shortness of breath, cough, chest pain and fever. It is estimated that about one quarter of people with rheumatoid arthritis develop this disease, which are more likely to develop among elderly men with a history of smoking.
Equine multinodular pulmonary fibrosis is a chronic lung disease of horses. There is evidence that the disease is caused by infection with a gammaherpesvirus, equine herpesvirus 5. The disease affects usually adult horses reducing the ability to exercise as a result of the formation of nodular lesions in the lungs.
Pulmonary neuroendocrine tumors are neuroendocrine tumors localized to the lung: bronchus or pulmonary parenchyma.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a diffuse parenchymal lung disease which often presents with symptoms of cough and shortness of breath. The pathological definition published by the World Health Organization is “a generalized proliferation of scattered single cells, small nodules, or linear proliferations of pulmonary neuroendocrine (PNE) cells that may be confined to the bronchial and bronchiolar epithelium.” The true prevalence of this disease is not known. To date, just under 200 cases have been reported in the literature. However, with an increase in recognition of this disease by radiologists and pulmonologists, the number of cases has been increasing. DIPNECH predominantly affects middle-aged women with slowly progressive lung obstruction. DIPNECH is usually discovered in one of two ways: 1) as an unexpected finding following a lung surgery; or 2) by evaluation of a patient in a pulmonary clinic with longstanding, unexplained symptoms.
Childhood interstitial lung disease, sometimes abbreviated as ChILD, is a family of rare chronic and complex disorders that affect the lungs of children. In the lungs, these disorders affect the interstitium, which is the space around the alveoli. The alveoli are the air sacs of the lungs. For these disorders, the alveoli are typically impaired by inflammatory and fibrotic changes which can lead to dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests.