Neuroendocrine hyperplasia of infancy (NEHI) is a rare and complex respiratory disorder that affects newborns and infants. It is characterized by excessive growth of the neuroendocrine cells in the airways, resulting in difficulty breathing. They can also lead to coughing, wheezing, and other respiratory problems.
Neuroendocrine cell hyperplasia of infancy was previously known as persistent tachypnea until histopathology of lung biopsies revealed that the disease was associated with an increased number of neuroendocrine cells.
What Is the Cause of NEHI?
There is no known cause of neuroendocrine hyperplasia, but some causes include a rapid increase in pulmonary and endocrine cells in children under two. Adults with a history of smoking COPD or cystic fibrosis have an increase in pulmonary and endocrine cells. Environmental factors may also play a role in the development of NEHI.
What Are the Sign And Symptoms of Neuroendocrine Hyperplasia?
The signs and symptoms of neuroendocrine hyperplasia of infancy (NEHI) vary depending on the severity of the condition. Generally, the most common symptoms include difficulty breathing, shortness of breath, wheezing, cyanosis (a bluish discoloration of the skin and mucous membranes due to lack of oxygen), recurrent chest infections, and poor growth.
Other less common symptoms may include persistent coughing, recurrent pneumonia, recurrent apnea (periods of stopped breathing), recurrent episodes of airway obstruction, and chronic obstructive pulmonary disease (COPD). Due to the low oxygen level, a chest x-ray may also show signs of pneumonia. In addition to respiratory difficulties, infants with NEHI may also experience tachypnea (fast breathing), hypoxemia (lack of oxygen in the tissue), crackles in the lungs, and exercise intolerance.
What Is the Diagnosis of Neuroendocrine Hyperplasia?
Diagnosis of neuroendocrine hyperplasia of infancy (NEHI) is typically based on a combination of clinical, radiologic, and histopathologic findings. A biopsy of the lungs may be necessary for definitive diagnosis and may also use tissue samples from the other organ. Imaging studies such as infant pulmonary function tests (PFTs) and computed tomography (CT) scans may also be performed to evaluate the extent and severity of NEHI.
1. Chest CT: It shows signs of pneumonia. A high-resolution computed tomography (CT) scan of the lungs, which offers a characteristic pattern known as ground glass opacities, is frequently helpful in diagnosing NEHI.
2. Infant Pulmonary Function Test (PFT): PFTs are an essential diagnostic tool for NEHI as they can help provide insight into a patient's pulmonary health. PFTs measure the amount and rate of airflow into and out of the lungs and assess how well oxygen is absorbed. This test may be performed several times over days or weeks to monitor any changes in lung function.
3. Lung Biopsy- During this procedure, a small sample of lung tissue is taken and examined under a microscope to look for abnormalities associated with NEHI. This test can provide valuable information about the extent and severity of the disease, as well as any underlying metabolic or immune disorder that might be present.
4. Bronchoscopy- Another diagnostic test for NEHI is bronchoscopy, in which a thin, flexible tube containing a light and camera is inserted into the airways. The camera allows the doctor to look at the walls of the airways and take pictures of tissue samples to be examined under a microscope. Bronchoscopy can also provide information about how well a patient's airways are functioning and whether any blockages need to be addressed.
What Is the Treatment of Neuroendocrine Hyperplasia?
The primary goal of treatment for neuroendocrine hyperplasia of infancy (NEHI) is to provide adequate oxygen to the patient, good nutrition, and prevent recurrent infections. Supplemental oxygen is usually the first step in treating NEHI. Other treatments may include corticosteroids to help reduce airway inflammation and proper immunization to protect against diseases.
1. Oxygen Supplementation: The amount of oxygen required by NEHI patients varies. Some people require oxygen 24 hours a day, while others only need it at night and during illness, and others do not.
2. Adequate Nutrition: Adequate nutrition is also essential for NEHI patients. A healthy diet should be rich in protein, vitamins, minerals, carbohydrates, and fats. Patients may need to be supplemented with formula or breast milk if they cannot eat enough solid food. It is essential to discuss nutritional needs with a pediatrician or nutritionist.
3. Steroids: Corticosteroids can also be used in some cases of NEHI to reduce inflammation and improve breathing. However, these drugs can have serious side effects, so discussing their use with a doctor before taking them is essential.
4. Treatment of Infections: Common colds and flu can be more severe in NEHI patients; treating recurrent infections is also an essential part of NEHI. Antibiotics may be prescribed to treat bacterial infections, and antiviral medications can be used for viral infections.
5. Proper Immunization: Proper immunization is also essential for NEHI patients, as this can help prevent future infections.
What Are the Differential Diagnosis of NEHI?
Infective lung diseases.
Cystic fibrosis-Inherited disorder that damages the lungs and digestive system.
Childhood interstitial lung disease.
Pulmonary hypoplasia-Incomplete development of the lungs that may affect the child's overall growth and development.
Pulmonary interstitial glycogenosis-Rare and Interstitial lung disease of unknown cause.
Chronic neonatal lung disease of prematurity.
Surfactant dysfunction disorder-A lung disorder that causes breathing problems.
What Is the Prognosis Of NEHI?
The prognosis for NEHI is dependent on severity. Many children experience a complete resolution of symptoms, but in severe cases, children need lifelong management and treatment. Early diagnosis and treatment can lead to better outcomes. Many children have been successfully treated and no longer require long-term care or medication. With proper management and treatment, most children can reach adulthood without significant issues related to NEHI.
Neuroendocrine hyperplasia of infancy (NEHI) is a rare, severe condition that can cause medical problems. It is essential to diagnose and treat NEHI early, as it can have lasting effects on a child's development and quality of life. While there is no cure for NEHI, proper treatment can help manage symptoms and reduce the risk of complications.