Neuroendocrine Cell Hyperplasia of Infancy (NEHI)

What is Neuroendocrine Cell Hyperplasia of Infancy (NEHI)?

NEHI causes trouble with breathing in babies. The exact cause of NEHI is not fully understood. Neuroendocrine cells are normal in the body, but in NEHI there are extra neuroendocrine cells in the lungs. Researchers believe it might be due to both genetics and the environment. We are working to learn more about NEHI and what causes it.

Babies with NEHI usually have fast and heavy breathing. They may have low oxygen levels and “crackles” on lung exam. Babies usually have trouble gaining weight and growing. This is because they have to use a lot of energy to breathe. They may need the hospital for respiratory illness.

Diagnosis

NEHI is possible when babies have trouble breathing, low oxygen levels, and trouble growing. Usually clinical history and imaging tests are enough to diagnose NEHI. It is important to rule out infection, heart problems, and other causes of trouble breathing.

• A NEHI Clinical Score can be used to see if the baby has NEHI signs and symptoms.
• A chest x-ray might show areas of pneumonia.
• A lung scan (chest CT) shows areas of grayish-white in a certain pattern suggesting NEHI.
• Some children’s hospitals may be able to do infant pulmonary function tests.
• A lung biopsy is sometimes needed for the right diagnosis. This will show extra neuroendocrine cells in the tiny airways. It will also rule out other causes of lung problems.

Treatment

Treatment of NEHI is to support breathing and nutrition. Children usually need oxygen to help keep oxygen levels normal. Oxygen can also help them use more energy to grow. Sometimes babies and children with NEHI need help with extra calories. This might mean using a feeding tube to help with nutrition.

What Does NEHI Mean for My Child?

Most babies and children with NEHI do well with oxygen and nutrition support. As the child grows, their lungs get stronger. Children are usually able to come off of oxygen as they grow. Older children and teenagers with NEHI may only need oxygen when sleeping or during illness.

It is important to protect your child’s lungs from infection by making sure they get the recommended vaccines. We are still learning about how NEHI impacts young adults.

What to Watch Out For

• If your child is working hard to breathe, talk to a health care provider. If possible, ask to see a lung specialist who is experienced with these conditions.
• Try to prevent infections from common childhood illnesses. Wash your hands often and ask your friends and family to let you know if they are sick before they are around your child.
• Get your child’s vaccines and yearly flu shot.

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This information is for educational purposes only. It should not be used as a substitute for the medical advice of one’s healthcare provider.