NEHI Lung Disease

NeuroEndocrine Hyperplasia of Infancy (NEHI), also known as Persistent Tachypnea of Infancy, is a form of rare infant pulmonary diseases. The disease inhibits the lung's ability to undergo gas exchange, which is the process in which red blood cells receive oxygen from the lungs and expel carbon dioxide. The disease causes a condition called hypoxemia, which means oxygen is deprived from the blood and tissue cells are unable to receive enough oxygen for cellular reactions.

Causes

• NEHI occurs when the infants resistance in the blood vessels does not lower after birth. Normally, lungs and vessels expand after birth, and the newborn's blood pressure lowers. In infants with NEHI, the high blood pressure persists. Resistant especially persists in the lungs, blocking proper oxygen flow. The heart has an increased mechanical responsibility, and the strain may lead to cardiac arrest.

Symptoms

• The most common symptom of NEHI is the child's difficulty in breathing. The infant will use accessory muscles to work for air. The infant will also have fast breathing. When listening to the infant breathe, the mother will hear wheezing or crackling. Infants with NEHI are also more prone to respiratory invasion of pathogens. NEHI infants are more susceptible to pneumonia and bronchiolitis.

Diagnosis

• Doctors will first observe the infant's breathing. He may then listen to the child's lungs to detect any abnormal sounds. The best way for the doctor to diagnose a baby with NEHI is through chest X-rays. The doctor may also take a lung biopsy. The lung biopsy is sent to a lab where the tissue is observed under a microscope. This also helps the doctor rule out any other possible conditions.

Treatment

• The first treatment given to a patent with NEHI is supplemental oxygen. This is required to reverse the effects of the hyoxemia, which is the main symptom accompanying the disease. The vasodilator tolazoline is used to expand the diameter of the blood vessels, lowering the blood pressure and improving blood flow through the body. Nitric oxide is also used as a vasodilator and helps relax the smooth muscle in the blood vessels. Prostacyclin is a drug that targets the relaxation of the lung blood vessels specifically.

Adults

• Although NEHI is primarily an infant's disease, the disease can also affect adults or children in later years. NEHI in adults causes scarring of the lungs, and it can be asymptomatic. Even though the disease is the same, symptoms for adults can be presented much differently than in the infant form of NEHI.