Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Difference between revisions
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{{Infobox medical condition | |||
| name = Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia | |||
| synonyms = DIPNECH | |||
| image = | |||
| caption = | |||
| field = [[Pulmonology]] | |||
| symptoms = [[Cough]], [[dyspnea]], [[wheezing]] | |||
| complications = [[Obstructive lung disease]], [[carcinoid tumors]] | |||
| onset = Middle-aged adults | |||
| duration = Chronic | |||
| causes = Unknown | |||
| risks = | |||
| diagnosis = [[High-resolution computed tomography]], [[lung biopsy]] | |||
| differential = [[Asthma]], [[chronic obstructive pulmonary disease]], [[bronchitis]] | |||
| treatment = [[Corticosteroids]], [[bronchodilators]], [[surgery]] | |||
| medication = | |||
| prognosis = Variable, can be progressive | |||
| frequency = Rare | |||
}} | |||
{{Short description|A rare lung condition involving neuroendocrine cell proliferation}} | {{Short description|A rare lung condition involving neuroendocrine cell proliferation}} | ||
'''Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia''' (DIPNECH) is a rare pulmonary disorder characterized by the proliferation of [[neuroendocrine cells]] within the [[lungs]]. This condition is considered a precursor to [[neuroendocrine tumors]] and is often associated with [[obstructive lung disease]]. | '''Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia''' (DIPNECH) is a rare pulmonary disorder characterized by the proliferation of [[neuroendocrine cells]] within the [[lungs]]. This condition is considered a precursor to [[neuroendocrine tumors]] and is often associated with [[obstructive lung disease]]. | ||
==Pathophysiology== | ==Pathophysiology== | ||
DIPNECH involves the diffuse proliferation of neuroendocrine cells in the [[bronchiolar epithelium]]. These cells can form small nodules known as [[tumorlets]], which are less than 5 mm in diameter. The condition is thought to be a precursor to [[carcinoid tumors]], which are a type of [[neuroendocrine tumor]]. The exact cause of DIPNECH is unknown, but it is considered idiopathic, meaning it arises spontaneously without a known cause. | DIPNECH involves the diffuse proliferation of neuroendocrine cells in the [[bronchiolar epithelium]]. These cells can form small nodules known as [[tumorlets]], which are less than 5 mm in diameter. The condition is thought to be a precursor to [[carcinoid tumors]], which are a type of [[neuroendocrine tumor]]. The exact cause of DIPNECH is unknown, but it is considered idiopathic, meaning it arises spontaneously without a known cause. | ||
==Clinical Presentation== | ==Clinical Presentation== | ||
Patients with DIPNECH often present with symptoms of [[chronic cough]], [[dyspnea]] (shortness of breath), and sometimes [[wheezing]]. These symptoms are due to the obstruction of airways by the proliferating neuroendocrine cells and tumorlets. In some cases, patients may be asymptomatic, and the condition is discovered incidentally during imaging studies for other reasons. | Patients with DIPNECH often present with symptoms of [[chronic cough]], [[dyspnea]] (shortness of breath), and sometimes [[wheezing]]. These symptoms are due to the obstruction of airways by the proliferating neuroendocrine cells and tumorlets. In some cases, patients may be asymptomatic, and the condition is discovered incidentally during imaging studies for other reasons. | ||
==Diagnosis== | ==Diagnosis== | ||
The diagnosis of DIPNECH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. [[High-resolution computed tomography]] (HRCT) of the chest may reveal multiple small nodules and areas of air trapping. A [[lung biopsy]] is often required to confirm the diagnosis, showing the characteristic proliferation of neuroendocrine cells and the presence of tumorlets. | The diagnosis of DIPNECH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. [[High-resolution computed tomography]] (HRCT) of the chest may reveal multiple small nodules and areas of air trapping. A [[lung biopsy]] is often required to confirm the diagnosis, showing the characteristic proliferation of neuroendocrine cells and the presence of tumorlets. | ||
==Treatment== | ==Treatment== | ||
There is no standard treatment for DIPNECH, and management is often tailored to the individual patient. Options may include observation for asymptomatic patients, [[bronchodilators]] and [[corticosteroids]] for symptomatic relief, and in some cases, surgical resection of larger lesions. The use of [[somatostatin analogs]] has been explored in some cases to control symptoms and slow disease progression. | There is no standard treatment for DIPNECH, and management is often tailored to the individual patient. Options may include observation for asymptomatic patients, [[bronchodilators]] and [[corticosteroids]] for symptomatic relief, and in some cases, surgical resection of larger lesions. The use of [[somatostatin analogs]] has been explored in some cases to control symptoms and slow disease progression. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for patients with DIPNECH varies. Some patients may experience stable disease with minimal symptoms, while others may progress to develop more significant obstructive lung disease or [[neuroendocrine tumors]]. Regular follow-up with pulmonary function tests and imaging studies is often recommended to monitor disease progression. | The prognosis for patients with DIPNECH varies. Some patients may experience stable disease with minimal symptoms, while others may progress to develop more significant obstructive lung disease or [[neuroendocrine tumors]]. Regular follow-up with pulmonary function tests and imaging studies is often recommended to monitor disease progression. | ||
==Related pages== | ==Related pages== | ||
* [[Neuroendocrine tumor]] | * [[Neuroendocrine tumor]] | ||
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* [[Obstructive lung disease]] | * [[Obstructive lung disease]] | ||
* [[Chronic cough]] | * [[Chronic cough]] | ||
[[Category:Pulmonary diseases]] | [[Category:Pulmonary diseases]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Neuroendocrine tumors]] | [[Category:Neuroendocrine tumors]] | ||
Latest revision as of 23:28, 3 April 2025
| Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia | |
|---|---|
| Synonyms | DIPNECH |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Cough, dyspnea, wheezing |
| Complications | Obstructive lung disease, carcinoid tumors |
| Onset | Middle-aged adults |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown |
| Risks | |
| Diagnosis | High-resolution computed tomography, lung biopsy |
| Differential diagnosis | Asthma, chronic obstructive pulmonary disease, bronchitis |
| Prevention | N/A |
| Treatment | Corticosteroids, bronchodilators, surgery |
| Medication | |
| Prognosis | Variable, can be progressive |
| Frequency | Rare |
| Deaths | N/A |
A rare lung condition involving neuroendocrine cell proliferation
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterized by the proliferation of neuroendocrine cells within the lungs. This condition is considered a precursor to neuroendocrine tumors and is often associated with obstructive lung disease.
Pathophysiology[edit]
DIPNECH involves the diffuse proliferation of neuroendocrine cells in the bronchiolar epithelium. These cells can form small nodules known as tumorlets, which are less than 5 mm in diameter. The condition is thought to be a precursor to carcinoid tumors, which are a type of neuroendocrine tumor. The exact cause of DIPNECH is unknown, but it is considered idiopathic, meaning it arises spontaneously without a known cause.
Clinical Presentation[edit]
Patients with DIPNECH often present with symptoms of chronic cough, dyspnea (shortness of breath), and sometimes wheezing. These symptoms are due to the obstruction of airways by the proliferating neuroendocrine cells and tumorlets. In some cases, patients may be asymptomatic, and the condition is discovered incidentally during imaging studies for other reasons.
Diagnosis[edit]
The diagnosis of DIPNECH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. High-resolution computed tomography (HRCT) of the chest may reveal multiple small nodules and areas of air trapping. A lung biopsy is often required to confirm the diagnosis, showing the characteristic proliferation of neuroendocrine cells and the presence of tumorlets.
Treatment[edit]
There is no standard treatment for DIPNECH, and management is often tailored to the individual patient. Options may include observation for asymptomatic patients, bronchodilators and corticosteroids for symptomatic relief, and in some cases, surgical resection of larger lesions. The use of somatostatin analogs has been explored in some cases to control symptoms and slow disease progression.
Prognosis[edit]
The prognosis for patients with DIPNECH varies. Some patients may experience stable disease with minimal symptoms, while others may progress to develop more significant obstructive lung disease or neuroendocrine tumors. Regular follow-up with pulmonary function tests and imaging studies is often recommended to monitor disease progression.
