Although NEHI is clearly rare, its incidence and prevalence remain unknown. Since the original report of 15 cases from two large referral centers, the published literature tallies an additional 116 cases.
What causes NEHI?
Neuroendocrine cell hyperplasia of infancy (NEHI), initially described as persistent tachypnea of infancy, is a rare lung disease first defined in 2005 [1]. The etiology is unknown, but genetic mechanisms may play a role.
What is NEHI syndrome?
Neuroendocrine hyperplasia of infancy (NEHI) is a type of rare lung disease that falls under the umbrella term of Interstitial Lung Disease. Babies with NEHI often present with fast breathing and retractions even when they are not sick.
What is diffuse idiopathic pulmonary neuroendocrine cell hyperplasia?
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare and poorly understood lung condition that is characterized by the abnormal overgrowth of certain cells in the lung (called pulmonary neuroendocrine cells) that receive signals from nerve cells (neurons) and produce hormones .
How common is NEHI?
NEHI is a relatively rare disorder of the lungs that was first classified and described in 2005. As a result, it is difficult to estimate the number of children with this disorder in the US. However, this is one of the most common forms of chILD and is still underdiagnosed.
How is DIPNECH treated?
Currently, the management and treatment options for DIPNECH have included clinical observation, oral and inhaled steroids, chemotherapy, surgical lung resection, and even lung transplantation [3,4,9,10].
Is DIPNECH fatal?
The clinical course however, is characterized by slowly progressive, unspecific pulmonary symptoms such as long-lasting dry cough, wheezing and exertional dyspnoea [2,4-8]. Nonetheless, DIPNECH can also cause severe airflow obstruction and respiratory failure which can prove fatal [2,3,7,9].
What are neuroendocrine cells in lungs?
Pulmonary neuroendocrine cells (PNECs) are one of the epithelial cell types lining large and small airways that form a tiny cellular population (0.4% of total airway epithelial cells) and are evolutionarily preserved among air-breathing vertebrates. Their chemosensitivity features have previously been demonstrated.
How common is DIPNECH?
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature.
What is the life expectancy of a person with interstitial lung disease?
The average survival for people with this type is currently 3 to 5 years . It can be longer with certain medications and depending on its course. People with other types of interstitial lung disease, like sarcoidosis, can live much longer.
What is the most common cause of interstitial lung disease?
A bacteria called Mycoplasma pneumoniae is the most common cause. Idiopathic pulmonary fibrosis. This makes scar tissue grow in the interstitium.Aug 8, 2017
What lung diseases can you be born with?
- Apnea of prematurity. external link.
- Bronchopulmonary dysplasia.
- Childhood interstitial lung disease.
- Meconium aspiration syndrome. external link.
- Persistent pulmonary hypertension of the newborn.
- Pleural disorders.
- Pneumonia.
- Transient tachypnea of the newborn.