“Luck’s always to blame”: silent wounds of a penetrating gunshot trauma sustained 20 years ago

Gunshot tracheal injuries represent life-threatening events and usually necessitate emergent surgical intervention. We report a case of an exceptional finding of a patient with retained ballistic fragments in the soft tissues of the thorax, proximal to the right subclavian artery and the trachea, carrying silently his wounds for two decades without any medical or surgical intervention. The bullet pellet on the upper part of the trachea seen accidentally in the chest computed tomography, was also found during bronchoscopy. In short “luck’s always to blame”

Diffuse Idiopathic Skeletal Hyperostosis (DISH) and non small cell lung cancer: case presentation and review of the literature

Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier’s disease, is a systemic non inflammatory disease of unknown cause. It is characterized by the presence of osteophytes due to calcification and ossification of spinal ligaments and entheses. Moreover, diffuse idiopathic skeletal hyperostosis has been associated with a variety of metabolic disorders. However, to the best of our knowledge no association with non small cell lung cancer (NSCLC) has been reported so far. In the present study we report a case of a patient with NSCLC and DISH.Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier’s disease, is a systemic non inflammatory disease of unknown cause. It is characterized by the presence of osteophytes due to calcification and ossification of spinal ligaments and entheses. Moreover, diffuse idiopathic skeletal hyperostosis has been associated with a variety of metabolic disorders. However, to the best of our knowledge no association with non small cell lung cancer (NSCLC) has been reported so far. In the present study we report a case of a patient with NSCLC and DISH

Unilateral hypertransparency on chest radiograph: the congenital Poland Syndrome

  Unilateral hypertransparent hemithorax requires a particular diagnostic approach as it can be the result of diverse pulmonary diseases, including pneumothorax, large pulmonary embolus, unilateral large bullae, mucous plag, airway obstruction and contralateral pleural effusion. Congenital syndromes with chest wall abnormalities, are rare, but often underdiagnosed causes. Poland Syndrome consists of such a rare, congenital anomaly and is characterized by the absence of the pectoralis major muscle and upper limb ipsilateral abnormalities. We present a case of a patient with acute exacerbation of chronic obstructive pulmonary disease (COPD) and a unilateral hypertransparency on chest radiology, attributed to the underlying Poland Syndrome.  

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties

CD8+ T lymphocytes in bronchoalveolar lavage in idiopathic pulmonary fibrosis

<p>Abstract</p> <p>Background</p> <p>Recently it was shown that in Idiopathic Pulmonary Fibrosis (IPF) tissue infiltrating CD₈₊T lymphocytes (TLs) are associated with breathlessness and physiological indices of disease severity, as well as that CD₈₊TLs recovered by bronchoalveolar lavage (BAL) relate to those infiltrating lung tissue. Since BAL is a far less invasive technique than tissue biopsy to study mechanisms in IPF we further investigated the usefulness offered by this means by studying the relationship between BAL macrophages, neutrophils, eosinophils, CD₃₊, CD₄₊, CD₈₊, CD_8+/38+TLs and CD₄₊/CD₈₊ratio with breathlessness and physiological indices.</p> <p>Patients and methods</p> <p>27 IPF patients, 63 ± 9 years of age were examined. Cell counts were expressed as percentages of total cells and TLs were evaluated by flow cytometry. FEV₁, FVC, TLC, RV, <it>D</it>LCO, PaO₂, and PaCO₂were measured in all. Breathlessness was assessed by the Medical Research Council (MRC) chronic dyspnoea scale.</p> <p>Results</p> <p>CD₈₊TLs correlated positively (r_s= 0.46, p = 0.02), while CD₄₊/CD₈₊ratio negatively (r_s= -0.54, p = 0.006) with the MRC grade. CD₈₊TLs correlated negatively with RV (r_s= -0.50, p = 0.017). CD_8+/38+TLs were negatively related to the FEV₁and FVC (r_s= -0.53, p = 0.03 and r_s= -0.59, p = 0.02, respectively). Neutrophils correlated positively with the MRC grade (r_s= 0.42, p = 0.03), and negatively with the <it>D</it>LCO (r_s= -0.54, p = 0.005), PaO₂(r_s= -0.44, p = 0.03), and PaCO₂(r_s= -0.52, p = 0.01).</p> <p>Conclusion</p> <p>BAL CD₈₊TLs associations with physiological and clinical indices seem to indicate their implication in IPF pathogenesis, confirming our previous tissue study.</p

Jednostronnie jasne płuco na radiogramie klatki piersiowej — wrodzony zespół Polanda

Zwiększenie przejrzystości jednego płuca wymaga wdrożenia szczególnych procedur diagnostycznych, ponieważ przyczyną takiego stanu może być wiele różnorodnych chorób układu oddechowego, włączając odmę opłucnową, masywny zator płucny, duży jednostronny pęcherz rozedmowy, czop śluzowy, zamknięcie dużego oskrzela oraz płyn w przeciwległej jamie opłucnowej. Wrodzone zaburzenia budowy ściany klatki piersiowej należą do rzadkich, choć często niezdiagnozowanych przyczyn. Zespół Polanda należy do takich rzadkich wrodzonych anomalii i polega na niewykształceniu mięśnia piersiowego większego i nieprawidłowościach budowy kończyny górnej po tej samej stronie. W pracy przedstawiono przypadek chorego z zaostrzeniem przewlekłej obturacyjnej choroby płuc (POChP) i obrazem jednostronnie jasnego płuca na radiogramie klatki piersiowej, spowodowanym zespołem Polanda.Zwiększenie przejrzystości jednego płuca wymaga wdrożenia szczególnych procedur diagnostycznych, ponieważ przyczyną takiego stanu może być wiele różnorodnych chorób układu oddechowego, włączając odmę opłucnową, masywny zator płucny, duży jednostronny pęcherz rozedmowy, czop śluzowy, zamknięcie dużego oskrzela oraz płyn w przeciwległej jamie opłucnowej. Wrodzone zaburzenia budowy ściany klatki piersiowej należą do rzadkich, choć często niezdiagnozowanych przyczyn. Zespół Polanda należy do takich rzadkich wrodzonych anomalii i polega na niewykształceniu mięśnia piersiowego większego i nieprawidłowościach budowy kończyny górnej po tej samej stronie. W pracy przedstawiono przypadek chorego z zaostrzeniem przewlekłej obturacyjnej choroby płuc (POChP) i obrazem jednostronnie jasnego płuca na radiogramie klatki piersiowej, spowodowanym zespołem Polanda

Serum Levels of Surfactant Proteins in Patients with Combined Pulmonary Fibrosis and Emphysema (CPFE)

Introduction Emphysema and idiopathic pulmonary fibrosis (IPF) present either per se or coexist in combined pulmonary fibrosis and emphysema (CPFE). Serum surfactant proteins (SPs) A, B, C and D levels may reflect lung damage. We evaluated serum SP levels in healthy controls, emphysema, IPF, and CPFE patients and their associations to disease severity and survival. Methods 122 consecutive patients (31 emphysema, 62 IPF, and 29 CPFE) and 25 healthy controls underwent PFTs, ABG-measurements, 6MWT and chest HRCT. Serum levels of SPs were measured. Patients were followed-up for 1-year. Results SP-A and SP-D levels differed between groups (p = 0.006 and p= 26 ng/mL) presented a weak association with reduced survival (p = 0.05). Conclusion In conclusion, serum SP-A and SP-D levels were higher where fibrosis exists or coexists and related to disease severity, suggesting that serum SPs relate to alveolar damage in fibrotic lungs and may reflect either local overproduction or overleakage. The weak association between high levels of SP-B and survival needs further validation in clinical trials

The Burden of Progressive Fibrosing Interstitial Lung Disease: A DELPHI Approach

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour. Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients’ quality of life (QoL) and healthcare resource utilisation (HCRU). Annual costs were calculated for progressive and non-/slow-progressive fibrosing ILD for diagnosis, follow-up management, exacerbation management, and end-of-life care based on the survey data. Results: Physicians reported that progression in fibrosing ILD worsens QoL in both patients and their caregivers. Progression of fibrosing ILD was associated with a greater use of HCRU for follow-up visits and maintenance treatment compared with the non-/slow progression. The number of patients who suffered at least one acute exacerbation was reported to be more than three times higher in progressive fibrosing ILD patients than in patients with non-/slow-progressive fibrosing ILD. On average, annual estimated costs of progressive fibrosing ILD per patient were 1.8 times higher than those of the non-/slow-progressive form of the disease.

Survival in Idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach

BACKGROUND: Idiopathic pulmonary fibrosis acute exacerbation (IPF-AE) constitutes IPF’s most devastating event, representing the unexpected superimposition of diffuse alveolar damage of unknown etiology. Guidelines recommend high-dose steroids treatment despite unproven benefit. We hypothesized that previous immunosuppression and the administration of high-dose steroids adversely affect IPF-AE outcome. METHODS: We studied all consecutive patients hospitalized in our department for IPF deterioration from 2007 to June 2013. Our protocol consisted of immediate cessation of immunosuppression (if any), best supportive care, broad-spectrum antimicrobials and thorough evaluation to detect reversible causes of deterioration. Patients were followed-up for survival; post-discharge none received immunosuppression. RESULTS: Twenty-four out of 85 admissions (28 %) fulfilled IPF-AE criteria. IPF-AE were analyzed both as unique events and as unique patients. As unique events 50 % survived; 3 out of 12 (25 %) in the group previously treated with immunosuppression whereas nine out of 12 (75 %) in the group not receiving immunosuppression (p = 0.041). As unique patients 35.3 % survived; 3 out of 6 (50 %) in the never treated group whereas three out of 11 (27.3 %) in the group receiving immunosuppression (p = 0.685). The history of immunosuppression significantly and adversely influenced survival (p = 0.035). Survival was greater in the never treated group compared to the immunosuppressed patients (p = 0.022). Post-discharge, our IPF-AE survivors had an 83 % 1-year survival. CONCLUSIONS: By applying the above mentioned protocol half of our patients survived. The history of immunosuppression before IPF-AE adversely influences survival. Avoiding steroids in IPF patients may favor the natural history of the disease even at the moment of its most devastating event