Neuroendocrine Cell Hyperplasia of Infancy (NEHI)

Neuroendocrine Cell Hyperplasia of Infancy (NEHI) is a rare lung condition primarily affecting infants. It causes an increase in neuroendocrine cells in the lungs, leading to breathing difficulties. These cells regulate the body’s response to stress and control breathing.

Types:

There is only one type of NEHI, which specifically affects infants.

Causes:

  1. Genetic factors: Some cases of NEHI are linked to genetic mutations passed down from parents.
  2. Environmental factors: Exposure to certain substances or pollutants may increase the risk.
  3. Premature birth: Babies born prematurely are more susceptible to NEHI.
  4. Maternal smoking: Smoking during pregnancy can contribute to NEHI.
  5. Respiratory infections: Certain infections may trigger NEHI in susceptible infants.
  6. Immune system issues: Problems with the immune system may play a role.
  7. Air pollution: Exposure to air pollutants can exacerbate NEHI.
  8. Allergies: Allergic reactions may contribute to the development of NEHI.
  9. Gastroesophageal reflux disease (GERD): GERD may increase the risk of NEHI.
  10. Birth complications: Difficulties during birth can be a factor.
  11. Low birth weight: Babies with low birth weight may be at higher risk.
  12. Family history: Having a family member with NEHI increases the risk.
  13. Premature rupture of membranes: When the amniotic sac breaks early, it may increase the risk.
  14. Maternal drug use: Certain medications taken during pregnancy may increase the risk.
  15. Maternal illness: Certain maternal illnesses may increase the likelihood of NEHI in infants.
  16. Airway abnormalities: Structural issues in the airways may contribute to NEHI.
  17. Lung inflammation: Inflammation in the lungs may trigger NEHI.
  18. Oxygen therapy: Prolonged use of oxygen therapy in newborns may increase the risk.
  19. Other lung conditions: Conditions like bronchopulmonary dysplasia may be linked to NEHI.
  20. Unknown factors: In some cases, the cause of NEHI remains unknown.

Symptoms:

  1. Difficulty breathing
  2. Rapid breathing
  3. Wheezing
  4. Cyanosis (bluish discoloration of the skin)
  5. Retractions (pulling in of the chest wall)
  6. Grunting sounds while breathing
  7. Nasal flaring
  8. Poor weight gain
  9. Fatigue
  10. Irritability
  11. Difficulty feeding
  12. Failure to thrive
  13. Frequent respiratory infections
  14. Coughing
  15. Gastroesophageal reflux symptoms
  16. Sleep disturbances
  17. Abnormal chest X-ray findings
  18. Clubbing of fingers or toes
  19. Low oxygen levels in the blood
  20. Abnormal lung function tests

Diagnostic Tests:

  1. History: Detailed information about the infant’s birth, medical history, and family history.
  2. Physical examination: A thorough examination of the infant’s respiratory system, including listening to the lungs and assessing breathing patterns.
  3. Chest X-ray: To look for abnormalities in the lungs.
  4. High-resolution computed tomography (HRCT) scan: Provides detailed images of the lungs.
  5. Pulmonary function tests: Measures lung function, including how well the infant can breathe.
  6. Arterial blood gas analysis: Measures oxygen and carbon dioxide levels in the blood.
  7. Bronchoscopy: A thin, flexible tube with a camera is inserted into the airways to examine them.
  8. Echocardiogram: To assess heart function and rule out heart problems.
  9. Sleep study (polysomnography): Monitors the infant’s breathing and oxygen levels during sleep.
  10. Sweat chloride test: To rule out cystic fibrosis.
  11. Allergy testing: To check for allergies that may contribute to symptoms.
  12. Genetic testing: To look for specific genetic mutations associated with NEHI.
  13. Immunological tests: To assess immune function.
  14. Gastric emptying study: To evaluate for gastroesophageal reflux disease (GERD).
  15. Lung biopsy: Rarely performed but may be necessary to confirm the diagnosis.
  16. Magnetic resonance imaging (MRI) scan: Provides detailed images of the chest.
  17. Electrocardiogram (ECG or EKG): To assess heart rhythm and function.
  18. Nasopharyngeal culture: To check for respiratory infections.
  19. Urine tests: To assess for metabolic disorders.
  20. Blood tests: To evaluate for infections, inflammation, and other potential causes.

Treatments

(Non-Pharmacological):

  1. Oxygen therapy: Providing supplemental oxygen to maintain adequate oxygen levels in the blood.
  2. Mechanical ventilation: For severe respiratory distress, a ventilator may be used to assist with breathing.
  3. Nasal continuous positive airway pressure (CPAP): Helps keep the airways open during sleep.
  4. Oxygen saturation monitoring: Regular monitoring of oxygen levels to ensure they remain within a healthy range.
  5. Nutritional support: Ensuring adequate calorie intake for growth and development.
  6. Chest physiotherapy: Techniques to help clear mucus from the airways.
  7. Positioning: Placing the infant in certain positions to optimize breathing.
  8. Avoiding smoke exposure: Keeping the infant away from tobacco smoke, which can exacerbate symptoms.
  9. Avoiding respiratory irritants: Minimizing exposure to allergens and pollutants.
  10. Humidification: Using a humidifier to keep the air moist, which can help with breathing.
  11. Breastfeeding: Providing breast milk, which can help boost the infant’s immune system.
  12. Monitoring for complications: Regular check-ups to monitor for complications such as infections or respiratory failure.
  13. Occupational therapy: For infants with feeding difficulties, occupational therapy may help improve feeding skills.
  14. Speech therapy: For infants with swallowing difficulties, speech therapy may be beneficial.
  15. Pulmonary rehabilitation: A program of exercises and education to improve lung function.
  16. Nutritional counseling: Guidance on nutrition to support growth and development.
  17. Supportive care: Providing emotional support for both the infant and family members.
  18. Education: Teaching parents about NEHI, its management, and when to seek medical help.
  19. Environmental modifications: Making changes to the infant’s environment to reduce exposure to potential triggers.
  20. Palliative care: For infants with severe or life-limiting symptoms, palliative care may focus on comfort and quality of life.

Drugs:

  1. Bronchodilators: Medications that relax the muscles around the airways to improve breathing.
  2. Inhaled corticosteroids: Reduce inflammation in the airways.
  3. Systemic corticosteroids: Oral or intravenous steroids may be used for severe symptoms.
  4. Montelukast (Singulair): Helps prevent asthma symptoms.
  5. Leukotriene receptor antagonists: Medications that block the action of leukotrienes, which can cause inflammation.
  6. Antibiotics: To treat bacterial infections if present.
  7. Antifungal medications: To treat fungal infections if present.
  8. Antiviral medications: To treat viral infections if present.
  9. Proton pump inhibitors: To reduce stomach acid and help manage GERD.
  10. Mucolytics: Medications that help thin and loosen mucus in the airways.

Surgeries:

  1. Lung biopsy: Rarely performed but may be necessary to obtain a tissue sample for diagnosis.
  2. Tracheostomy: In severe cases, a surgical opening in the neck may be created to assist with breathing.
  3. Fundoplication: Surgery to prevent acid reflux in infants with severe GERD.
  4. Adenoidectomy: Removal of the adenoids if they are contributing to airway obstruction.
  5. Tonsillectomy: Removal of the tonsils if they are contributing to airway obstruction.
  6. Repair of airway abnormalities: Surgery to correct structural issues in the airways.
  7. Lung transplantation: In very severe cases, a lung transplant may be considered.
  8. Thoracoscopic lung biopsy: Minimally invasive surgery to obtain a tissue sample from the lungs.
  9. Laryngotracheal reconstruction: Surgery to repair or reconstruct the larynx and trachea.
  10. Lobectomy: Removal of a lobe of the lung if it is severely affected by NEHI.

Prevention:

  1. Avoiding smoke exposure during pregnancy and after birth.
  2. Breastfeeding, if possible, to boost the infant’s immune system.
  3. Minimizing exposure to respiratory irritants and pollutants.
  4. Keeping the infant up to date on vaccinations to prevent respiratory infections.
  5. Prompt treatment of respiratory infections to prevent complications.
  6. Following safe sleep practices to reduce the risk of sudden infant death syndrome (SIDS).
  7. Regular prenatal care to monitor the health of both mother and baby.
  8. Maintaining a healthy lifestyle during pregnancy, including eating a balanced diet and staying physically active.
  9. Avoiding unnecessary use of medications during pregnancy unless recommended by a healthcare provider.
  10. Seeking medical attention promptly if any concerning symptoms develop in the infant.

When to See Doctors:

  1. Difficulty breathing that does not improve with home remedies.
  2. Rapid breathing or shortness of breath.
  3. Wheezing or noisy breathing.
  4. Cyanosis (bluish discoloration of the skin).
  5. Retractions (pulling in of the chest wall).
  6. Grunting sounds while breathing.
  7. Nasal flaring.
  8. Poor weight gain or failure to thrive.
  9. Fatigue or lethargy.
  10. Irritability or difficulty feeding.
  11. Frequent respiratory infections.
  12. Coughing that persists or worsens.
  13. Abnormal chest X-ray findings.
  14. Clubbing of fingers or toes.
  15. Low oxygen levels in the blood.
  16. Any concerns about the infant’s breathing or respiratory health.
  17. Symptoms of gastroesophageal reflux disease (GERD), such as spitting up frequently or arching the back during feeding.
  18. Sleep disturbances or abnormal breathing patterns during sleep.
  19. Developmental delays or regression.
  20. Family history of lung disease or respiratory issues.

In summary, Neuroendocrine Cell Hyperplasia of Infancy (NEHI) is a rare lung condition that primarily affects infants. It can cause breathing difficulties and other symptoms. Diagnosis involves a thorough medical history, physical examination, and various tests. Treatment may include oxygen therapy, medications, and non-pharmacological interventions. In severe cases, surgery may be necessary. Prevention strategies focus on avoiding potential risk factors and seeking prompt medical attention for any concerning symptoms. If parents notice any respiratory symptoms or other concerns in their infant, they should seek medical advice promptly.

 

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. Thank you for giving your valuable time to read the article.

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References