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Diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) in adults and neuroendocrine hyperplasia of infancy (NEHI) in children are rare lung diseases characterized by marked increases in neuroendocrine cells.
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DIPNECH is a progressive disease found in middle-aged women who present with chronic cough and dyspnea. Imaging reveals air trapping, pulmonary nodules, and bronchial carcinoids or tumorlets.
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DIPNECH is associated with obstructive lung disease, thought to be owing to the secretion of
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy
Section snippets
Key points
Diffuse idiopathic neuroendocrine epithelial cell hyperplasia
The prevalence of DIPNECH in the adult population is not known and a lack of specific diagnostic criteria has led to a confusing literature composed of a heterogeneous patient population. Although rare, this disease is becoming more commonly recognized. In 1976, Churg and Warnock4 reported the finding of pulmonary carcinoid tumorlets in a review of indexed autopsy and surgical pathology cases. Seven cases of multiple pulmonary carcinoid tumorlets, a nodular proliferation of neuroendocrine cells
Neuroendocrine hyperplasia of infancy
Neuroendocrine cell hyperplasia of infancy (NEHI), previously termed “persistent tachypnea of infancy,” is a rare form of childhood interstitial lung disease that has characteristic clinical, radiographic, and histologic features.19, 20 NEHI typically presents in otherwise healthy infants with chronic tachypnea and intercostal retractions in the first few months to year of life. Most patients with NEHI are born at term after uncomplicated pregnancies, but cases occurring in late preterm infants
Summary and Discussion
The presence of neuroendocrine cell hyperplasia in small airways is the defining feature of DIPNECH in adults and NEHI in young children. The underlying etiology of NEC hyperplasia is unknown in either of these rare lung diseases. Although both disorders manifest with dyspnea, obstruction and severe air trapping, symptoms in NEHI typically improve over time, whereas DIPNECH is progressive in nature. In addition, DIPNECH has a gender bias toward women, whereas NEHI is found in both genders
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Cited by (14)
Early onset children's interstitial lung diseases: Discrete entities or manifestations of pulmonary dysmaturity?
2019, Paediatric Respiratory ReviewsCitation Excerpt :However, new studies cast doubt on the existence of NEHI as a discrete clinical entity. Large numbers of data points for normal values of bombesin positive cells, and formal criteria for defining neuroendocrine cell excess are lacking [23]. NEHI cells were counted in 73 biopsies from children with a wide range of chILD [24].
Updates on lung neuroendocrine neoplasm classification
2024, HistopathologyPediatric pulmonology 2021 year in review: Rare and diffuse lung disease
2023, Pediatric PulmonologyCongenital interstitial lung diseases: What the anesthesiologist needs to know
2022, Paediatric Anaesthesia
Disclosure Statement: Dr J.A. Kern is a consultant to Gensignia Life Sciences, Inc, and serves on the scientific advisory boards of Strand Life Sciences, and Uptake Medical, Inc.
© 2016 Elsevier Inc. All rights reserved.