Al-Toubah Taymeyah, Strosberg Jonathan, Halfdanarson Thorvardur R, Oleinikov Kira, Gross David J, Haider Mintallah, Sonbol Mohamad Bassam, Almquist Daniel, Grozinsky-Glasberg Simona
Department of GI Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL.
Department of GI Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL.
Chest. 2020 Jul;158(1):401-405. doi: 10.1016/j.chest.2020.01.031. Epub 2020 Feb 12.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare lung disease associated with proliferation of neuroendocrine cells in the lung and multifocal neuroendocrine tumorlets/tumors. Although usually considered an indolent condition, DIPNECH causes chronic, progressive cough and dyspnea which can adversely impact quality of life. There is very limited information on the treatment of this condition. The objective of this study was to assess changes in symptoms and pulmonary function tests (PFTs) in response to somatostatin analog (SSA) treatment.
Patients with clinical and/or pathologic diagnosis of DIPNECH and chronic respiratory symptoms were treated with SSAs at the H. Lee Moffitt Cancer Center and Research Institute, Hadassah-Hebrew University Medical Center, and Mayo Clinic Cancer Center. Their charts were reviewed to assess changes in symptoms and PFTs.
Forty-two patients were identified who had either chronic cough or dyspnea because of proven or suspected DIPNECH and who had received treatment with an SSA. Thirty-three patients experienced symptomatic improvement. Additionally, 14 of 15 patients in whom PFTs were checked were noted to have an improvement in FEV after treatment.
SSA treatment can improve chronic respiratory symptoms and PFTs in patients with DIPNECH.
弥漫性特发性肺神经内分泌细胞增生症（DIPNECH）是一种罕见的肺部疾病，与肺内神经内分泌细胞的增殖以及多灶性神经内分泌微瘤/肿瘤有关。尽管通常被认为是一种进展缓慢的病症，但DIPNECH会导致慢性进行性咳嗽和呼吸困难，这会对生活质量产生不利影响。关于这种病症的治疗信息非常有限。本研究的目的是评估生长抑素类似物（SSA）治疗后症状和肺功能测试（PFT）的变化。
在H. Lee Moffitt癌症中心及研究所、哈达萨希伯来大学医学中心和梅奥诊所癌症中心，对临床和/或病理诊断为DIPNECH且有慢性呼吸道症状的患者使用SSA进行治疗。对他们的病历进行回顾，以评估症状和PFT的变化。
确定了42例因已证实或疑似DIPNECH而患有慢性咳嗽或呼吸困难且接受过SSA治疗的患者。33例患者症状有所改善。此外，在检查了PFT的15例患者中，有14例在治疗后FEV有所改善。
SSA治疗可改善DIPNECH患者的慢性呼吸道症状和PFT。
