scholarly journals Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis with different changes of pulmonary function tests

2016 ◽  
Vol 15 (3) ◽  
pp. 30-35 ◽  
Author(s):  
V. P. Zolotnitskaya ◽  
V. I. Amosov ◽  
D. V. Dsadsua

Introduction and purpose. Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis and various types of respiratory failure are characterized by different scintigraphic patterns. Aim: to identify features of the circulatory disturbances in the lungs of patients with histiocytosis X, and various forms of bronchial conductivity changes. Material and methods: analysis of changes of microcirculation in the lungs in 54 patients with histiocytosis X, with different types of respiratory insufficiency, assessed by means of single photon emission computed tomography, perfusion scintigraphy and multislice computed tomography. Results and conclusions: in patients with histiocytosis x with obstructive type of respiratory failure, microcirculatory changes were detected at a very early stage of development of the disease. In the terminal stage of the disease, a typical scintigraphic pattern «candle flame» was observed. In patients with combined restrictive and obstructive changes (mixed type), no differences from changes of blood flow in obstructive variant were observed. In the restrictive type of respiratory failure, disorders of microcirculation do not have any typical scintigraphic pattern. The presence of characteristic nodular lesions and increasing fibrotic changes of all structural units were observed in the parenchyma of the lung.

2007 ◽  
Vol 51 (3) ◽  
pp. 480-482 ◽  
Author(s):  
Pio Zeppa ◽  
Immacolata Cozzolino ◽  
Maria Russo ◽  
Pietro Tullio Cataldo ◽  
Antonio Vetrani ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-7
Author(s):  
Carpio Carlos ◽  
Gómez-Carrera Luis ◽  
Álvarez-Sala Rodolfo

Cigarette smoking has been implicated in the development of some uncommon respiratory interstitial diseases. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung diseases are characterized by a diffuse alveolar and peribronchiolar filling with macrophages, respectively. Pulmonary Langerhans' cell histiocytosis is a rare interstitial lung disorder characterized by the proliferation of Langerhans' cell forming interstitial infiltrates and nodules that could progress to cavitary nodules. The treatment of these disorders involves smoking cessation and sometimes the use of steroids. High-resolution computed tomography is essential for the characterization of these smoking-related interstitial lung diseases, but frequently it is necessary to create a workgroup composed by pulmonologists, pathologists, and radiologists to diagnosis and treat patients affected with these pathologies.


2019 ◽  
Vol 20 (-1) ◽  
pp. 328-328
Author(s):  
Mehmet Fatih Elverisli ◽  
◽  
Pinar Yildiz Gulhan ◽  
Salih Ozenc ◽  
Fuat Aytekin ◽  
...  

Breathe ◽  
2020 ◽  
Vol 16 (2) ◽  
pp. 200003
Author(s):  
Mhairi Barclay ◽  
Rebecca Devaney ◽  
Jayesh. M. Bhatt

Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.


2012 ◽  
Vol 40 (4) ◽  
pp. 905-912 ◽  
Author(s):  
Abdellatif Tazi ◽  
Karima Marc ◽  
Stéphane Dominique ◽  
Cédric de Bazelaire ◽  
Bruno Crestani ◽  
...  

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