Pneumothorax in Cystic Fibrosis

Flume, Patrick A.; Strange, Charlie; Ye, Xiaobu; Ebeling, Myla; Hulsey, Thomas C.; Clark, Leslie L.

doi:10.1378/chest.128.2.720

Cited by 139 publications

(97 citation statements)

References 46 publications

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“…The differential diagnosis for a patient with a history of familial spontaneous pneumothorax and diffuse pulmonary cystic changes includes TSC (25), ␣ 1 -antitrypsin deficiency (21), Marfan syndrome (22), Ehlers-Danlos syndrome (23), LAM (24), LCH (26), CF (27), primary spontaneous pneumothorax (15), and BHDS. The distribution of cystic lung changes in radiologic studies may be helpful in distinguishing these diseases.…”

Section: Discussionmentioning

confidence: 99%

“…However, most cases of familial SP are inherited in an autosomal dominant pattern with incomplete penetrance (19,20). Familial SP may be a complication of various inherited disorders, such as ␣ 1 -antitrypsin deficiency (21), Marfan syndrome (22), Ehlers-Danlos syndrome (23), primary lymphangioleiomyomatosis (LAM) (24), tuberous sclerosis (TSC) (25), Langerhans cell histiocytosis (LCH) (26), cystic fibrosis (CF) (27), and BHDS. Therefore, understanding and defining the pulmonary features of BHDS are important for the diagnosis as well as for the treatment of patients.…”

Section: Birt-hogg-dubé Syndrome (Bhds; Omim [Online Mendelianmentioning

confidence: 99%

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Lung Cysts, Spontaneous Pneumothorax, and Genetic Associations in 89 Families with Birt-Hogg-Dubé Syndrome

Toro

Pautler

Stewart

et al. 2007

Am J Respir Crit Care Med

305

315

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Rationale: Birt-Hogg-Dubé syndrome (BHDS) is an autosomal, dominantly inherited genodermatosis that predisposes to fibrofolliculomas, kidney neoplasms, lung cysts, and spontaneous pneumothorax. Objectives: We evaluated 198 patients from 89 families with BHDS to characterize the risk factors for pneumothorax and genotypepulmonary associations. Methods: Helical computed tomography scans of the chest were used to screen for pulmonary abnormalities. BHD mutation data were used for genotype-pulmonary associations. We examined the relationship of pneumothorax with categorical parameters (sex, smoking history, and lung cysts) and continuous parameters (number of cysts, lung cyst volume, and largest cyst diameter and volume). Logistic regression analyses were used to identify the risk factors associated with pneumothorax. Measurements and Main Results: Twenty-four percent (48/198) of patients with BHDS had a history of pneumothorax. The presence of lung cysts was significantly associated with pneumothorax (p ϭ 0.006). Total lung cyst volume, largest cyst diameter and volume, and every parameter related to the number of lung cysts were significantly associated (p Ͻ 0.0001) with pneumothorax. A logistic regression analysis showed that only the total number of cysts in the right parenchymal lower lobe and the total number of cysts located on the pleural surface in the right middle lobe were needed to classify a patient as to whether or not he or she was likely to have a pneumothorax. Exon location of the BHD mutation was associated with the numbers of cysts (p ϭ 0.0002). Conclusions: This study indicates that patients with BHDS have a significant association between lung cysts and spontaneous pneumothorax.

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Section: Discussionmentioning

confidence: 99%

Section: Birt-hogg-dubé Syndrome (Bhds; Omim [Online Mendelianmentioning

confidence: 99%

Lung Cysts, Spontaneous Pneumothorax, and Genetic Associations in 89 Families with Birt-Hogg-Dubé Syndrome

Toro

Pautler

Stewart

et al. 2007

Am J Respir Crit Care Med

305

315

View full text Add to dashboard Cite

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“…It may be treated conservatively in presence of small pneumothorax (<2 cm) or with hospitalisation and intercostal tube for large pneumothoraces (≥2 cm). The average annual incidence of pneumothorax is 0.64% [20] and approximately 3.4% of individuals with CF will experience a pneumothorax during their lifetime [20].…”

Section: Complicationsmentioning

confidence: 99%

Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

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“…Esophageal rupture 43 Iatrogenic-lung biopsy, pleural biopsy, or fine-needle biopsy; mechanical ventilation; thoracotomy; placement of subclavian vein catheter, chemotherapy [44][45][46][47][48][49][50] Primary lung diseases Asthma 51 Respiratory bronchiolitis 21 Cystic fibrosis 52,53 Usual interstitial pneumonia 54 Pulmonary infarction 55,56 Hypersensitivity pneumonitis [34][35][36]57 Infections Spontaneous pneumothorax usually occurs in young, thin men, particularly smokers, who have no obvious underlying lung diseases. In these patients, pneumothorax is presumed to result from rupture of bullae and blebs.…”

Section: Specific Findingsmentioning

confidence: 99%

Approach to Lung Biopsies From Patients With Pneumothorax

Schneider

Murali

Veraldi

et al. 2014

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Patients with pneumothorax occasionally require limited lung resections to control persistent air leaks. In some patients, especially smokers, histopathologic findings suggest that a ruptured bulla or bleb caused the pneumothorax. Other patients only exhibit histopathologic changes related to the physical trauma of acute, and likely occult recurrent, peripheral lung injury in the setting of “spontaneous,” or idiopathic, lung rupture. Sometimes, pneumothorax occurs secondary to an underlying localized or diffuse parenchymal lung disease. A comprehensive description of the morphologic findings that may be seen in these specimens will help the surgical pathologist distinguish patients with more common and indolent occurrences of pneumothorax from those requiring additional workup or treatment. Objective.—To develop a diagnostic approach for surgical pathologists encountering lung specimens obtained in the context of pneumothorax repair. Data Sources.—Literature review and consultation experience of the authors. Conclusions.—Two general categories of histopathologic changes can be identified: (1) nonspecific changes, reflecting the lung's acute and chronic response to localized injury, and (2) changes suggesting an underlying lung disease that may have played an etiologic role in the development of pneumothorax. The latter changes are important to recognize because they may require additional workup or treatment of clinically occult lung disease. Difficulty arises when nonspecific histopathologic changes overlap with those of an underlying lung disease. Awareness of these diagnostic challenges and pitfalls, together with clinicoradiographic correlation, is essential in these situations and will help guide the surgical pathologist toward an accurate diagnosis and the appropriate management of clinically occult disease.

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Pneumothorax in Cystic Fibrosis

Cited by 139 publications

References 46 publications

Lung Cysts, Spontaneous Pneumothorax, and Genetic Associations in 89 Families with Birt-Hogg-Dubé Syndrome

Lung Cysts, Spontaneous Pneumothorax, and Genetic Associations in 89 Families with Birt-Hogg-Dubé Syndrome

Adult cystic fibrosis care in the 21st century

Approach to Lung Biopsies From Patients With Pneumothorax

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