Neuroendocrine Hyperplasia of Infancy- Causes, Symptoms, Diagnosis, and Treatment

Creator: Dr. K. M. Tarannum
Published: 2023-01-13T17:58:57

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Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare interstitial lung disorder that usually manifests itself in the first year of life. Explore to know more.

Written by

Dr. K. M. Tarannum

Medically reviewed by

Dr. Jeffrey Aaron

January 13, 2023

July 13, 2023

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Contents

What Is the Cause of NEHI?
What Are the Sign And Symptoms of Neuroendocrine Hyperplasia?
What Is the Diagnosis of Neuroendocrine Hyperplasia?
What Is the Treatment of Neuroendocrine Hyperplasia?
What Are the Differential Diagnosis of NEHI?
What Is the Prognosis Of NEHI?
What Is the Cause of NEHI?
What Is the Treatment of Neuroendocrine Hyperplasia?
What Is the Prognosis Of NEHI?

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.

Conclusion

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.

Frequently Asked Questions

1. What Is Neuroendocrine Cell Hyperplasia of the Lungs?

Neuroendocrine cell hyperplasia of the lungs is a disease in which the number of neuroendocrine cells in the lung tissue is higher than normal. It is common in babies with pulmonary hypertension, a disease that makes it hard for blood to flow through the lungs.

2. Does Neuroendocrine Cell Hyperplasia of Infancy Pulmonary Hypertension Involve?

Cancer of the neuroendocrine cells in the stomach is called neuroendocrine carcinoma gastric. It is a rare type of cancer that can have different effects, from slow-growing tumors to ones that spread quickly.

3. What Is Neuroendocrine Carcinoma Gastric?

The best way to treat a neuroendocrine tumor depends on several things, such as the size and location of the tumor and whether or not it has spread to other parts of the body. Surgery, chemotherapy, radiation treatment, and "targeted therapies" are all possible ways to treat cancer.

4. What Treatment Option Is Considered the Most Effective for a Neuroendocrine Tumor?

Small nodules composed of neuroendocrine cells can be found throughout the lung tissue in a person with nodular neuroendocrine hyperplasia. In most cases, these nodules are painless and harmless and do not require any treatment.

5. How Would Nodular Neuroendocrine Hyperplasia Be Described?

Nodular Neuroendocrine Hyperplasia (NNH) is a rare lung disease in which neuroendocrine cells form a number of small nodules or tumors in the lungs. These lumps are usually smaller than 5 mm and are found over the lung tissue. NNH is considered a harmless condition, and it is often found by accident during imaging studies or histopathological exams. Even though no one knows for sure what causes NNH, it is thought to have something to do with the abnormal growth of neuroendocrine cells.

6. What Is the Significance of Gastric Neuroendocrine Cell Hyperplasia?

Gastric neuroendocrine cell hyperplasia is significant because it can develop into a more lethal neuroendocrine carcinoma. In order to detect any signals of malignant transformation and administer the necessary treatment, it is crucial to monitor and properly manage this illness.

7. Are Neuroendocrine Tumors Always Malignant?

Malignant neuroendocrine tumors are rare but possible. Some neuroendocrine tumors are malignant and can spread to other organs, whereas others are non-cancerous and do not cause any harm to the surrounding tissues or organs.

8. How Can the Benign or Malignant Nature of a Neuroendocrine Tumor Be Determined?

Neuroendocrine tumors can be diagnosed as benign or malignant with the help of imaging techniques (such CT scans), biopsies, and pathological evaluation of the tumor tissue. The characteristics and behavior of the tumor can be evaluated with the use of these tests.

9. What Information Does a CT Scan Provide for Diagnosing Neuroendocrine Cell Hyperplasia of Infancy?

A CT scan can provide valuable information for diagnosing Neuroendocrine Cell Hyperplasia of Infancy (NEHI). It allows for a detailed evaluation of the lungs, including their structure, size, and appearance. CT scans may reveal characteristic findings such as ground-glass opacities, which are areas of increased density in the lung tissue. The distribution, pattern, and severity of lung abnormalities observed on CT imaging can aid in confirming the diagnosis of NEHI and ruling out other potential lung disorders.

10. On Average, What Is the Duration of Oxygen Therapy Required for Children With NEHI?

On average, the duration of oxygen therapy required for children with NEHI (neuroendocrine cell hyperplasia of infancy) can vary depending on the severity of their condition. It typically lasts for several months to a few years. However, it's important to note that each child's case is unique, and the duration of oxygen therapy will be determined by their specific needs and the recommendations of their healthcare, provider.

11. What Diagnostic Methods Are Used for Identifying Interstitial Lung Disease in Children?

Several diagnostic methods can be used to identify Interstitial Lung Disease (ILD) in children. These may include a physical examination, medical history review, lung function tests, chest X-rays, computed tomography (CT) scans, and sometimes a lung biopsy. These tests help doctors assess lung function, identify abnormalities in the lungs, and determine the type and extent of the ILD present in the child.

12. Can Interstitial Lung Disease Be Cured?

Interstitial lung disease (ILD) encompasses a group of lung disorders that involve inflammation and scarring of lung tissue. While there is no universal cure for ILD, the outcome depends on the specific type and underlying cause. Some ILDs are chronic and progressive, without a known cure. Treatment mainly focuses on managing symptoms, slowing disease progression, and improving quality of life through medications, oxygen therapy, and pulmonary rehabilitation. In severe cases, lung transplantation may be considered a treatment option, but it does not provide a definitive cure.

13. What Does the Presence of Interstitial Lung Markings Indicate?

Most of the time, Interstitial Lung Disease (ILD) is present when imaging tests show interstitial lung markings. Interstitial lung marks are thin, linear, or reticular opacities that can be seen on chest X-rays or CT scans. They show that there are problems in the interstitial tissue of the lungs. ILD is a group of lung diseases in which the interstitial tissue becomes inflamed and scarred. These diseases include idiopathic pulmonary fibrosis, sarcoidosis, and connective tissue diseases. To figure out the exact reason for the interstitial lung markings, it is often necessary to do more tests, such as a clinical evaluation, pulmonary function tests, and sometimes a lung biopsy.

14. Which Autoimmune Disease Is Associated With Interstitial Lung Disease?

Rheumatoid arthritis (RA) is an autoimmune disease often linked to Interstitial Lung Disease (ILD). RA is a long-term disease that causes inflammation. It mostly affects the joints but can also affect other organs, like the lungs. When people with RA get ILD, they may have symptoms like shortness of breath, coughing, and less ability to handle activity. ILD can also be linked to autoimmune diseases like systemic sclerosis (scleroderma) and Systemic Lupus Erythematosus (SLE). ILD must be caught early and treated correctly in these autoimmune conditions to stop further lung damage and improve results.

15. Is Interstitial Lung Disease a Permanent Condition or Reversible?

Interstitial Lung Disease (ILD) is a broad term for a number of lung diseases, and the outlook and whether or not it can be cured depends on what's causing it. Some types of ILD, like idiopathic pulmonary fibrosis, get worse over time and can't be fixed. This causes lasting lung damage and makes it hard for them to work. But other kinds of ILD, like those caused by certain diseases of the connective tissues, by drugs, or by certain infections, may be reversed or get better with the right treatment.

Sources

Dr. Jeffrey Aaron

Pediatrics

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