scholarly journals Year in review 2014: Interstitial lung disease, physiology, sleep and ventilation, acute respiratory distress syndrome, cystic fibrosis, bronchiectasis and rare lung disease

Respirology ◽  
2015 ◽  
Vol 20 (5) ◽  
pp. 834-845 ◽  
Author(s):  
Toby M. Maher ◽  
Amanda Piper ◽  
Yuanlin Song ◽  
Marcos I. Restrepo ◽  
Neil D. Eves
Keyword(s):  
Cystic Fibrosis ◽  
Acute Respiratory Distress Syndrome ◽  
Interstitial Lung Disease ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Distress Syndrome ◽  
Review 2014

scholarly journals Serum heme oxygenase-1 measurement is useful for evaluating disease activity and outcomes in patients with acute respiratory distress syndrome and acute exacerbation of interstitial lung disease

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Ryo Nagasawa ◽  
Yu Hara ◽  
Kota Murohashi ◽  
Ayako Aoki ◽  
Nobuaki Kobayashi ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Acute Respiratory Distress Syndrome ◽  
Interstitial Lung Disease ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Heme Oxygenase ◽  
Distress Syndrome ◽  
Heme Oxygenase 1

Abstract Background Oxidative stress plays an important role in acute lung injury, which is associated with the development and progression of acute respiratory failure. Here, we investigated whether the degree of oxidative stress as indicated by serum heme oxygenase-1 (HO-1) is clinically useful for predicting prognosis among the patients with acute respiratory distress syndrome (ARDS) and acute exacerbation of interstitial lung disease (AE-ILD). Methods Serum HO-1 levels of newly diagnosed or untreated ARDS and AE-ILD patients were measured at diagnosis. Relationships between serum HO-1 and other clinical parameters and 1 and 3-month mortality were evaluated. Results Fifty-five patients including 22 of ARDS and 33 of AE-ILD were assessed. Serum HO-1 level at diagnosis was significantly higher in ARDS patients than AE-ILD patients (87.8 ± 60.0 ng/mL vs. 52.5 ± 36.3 ng/mL, P <  0.001). Serum HO-1 correlated with serum total bilirubin (R = 0.454, P <  0.001) and serum LDH (R = 0.500, P <  0.001). In both patients with ARDS and AE-ILDs, serum HO-1 level tended to decrease from diagnosis to 2 weeks after diagnosis, however, did not normalized. Composite parameters including serum HO-1, age, sex, and partial pressure of oxygen in arterial blood/fraction of inspired oxygen (P/F) ratio for prediction of 3-month mortality showed a higher AUC (ARDS: 0.925, AE-ILDs: 0.892) than did AUCs of a single predictor or combination of two or three predictors. Conclusion Oxidative stress assessed by serum HO-1 is persistently high among enrolled patients for 2 weeks after diagnosis. Also, serum HO-1 levels at the diagnosis combined with age, sex, and P/F ratio could be clinically useful for predicting 3-month mortality in both ARDS and AE-ILD patients.

scholarly journals Serum heme oxygenase-1 measurement is useful for evaluating disease activity and outcomes in patients with acute respiratory distress syndrome and acute exacerbation of interstitial lung disease

2020 ◽  
Author(s):  
Ryo Nagasawa ◽  
Yu Hara ◽  
Kota Murohashi ◽  
Ayako Aoki ◽  
Nobuaki Kobayashi ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Acute Respiratory Distress Syndrome ◽  
Interstitial Lung Disease ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Heme Oxygenase ◽  
Distress Syndrome ◽  
Heme Oxygenase 1

Abstract Background: Oxidative stress plays an important role in acute lung injury, which is associated with the development and progression of acute respiratory failure. Here, we investigated whether the degree of oxidative stress as indicated by serum heme oxygenase-1 (HO-1) is clinically useful for predicting prognosis among the patients with acute respiratory distress syndrome (ARDS) and acute exacerbation of interstitial lung disease (AE-ILDs).Methods: Serum HO-1 levels of newly diagnosed or untreated ARDS and AE-ILD patients were measured at diagnosis. Relationships between serum HO-1 and other clinical parameters and 1 and 3-month mortality were evaluated. Results: Fifty-five patients including 22 of ARDS and 33 of AE-ILD were assessed. Serum HO-1 level at diagnosis was significantly higher in ARDS patients than AE-ILD patients (87.8 ± 60.0 ng/mL vs. 52.5 ± 36.3 ng/mL, P < 0.001). Serum HO-1 correlated with serum total bilirubin (R = 0.454, P < 0.001) and serum LDH (R = 0.500, P < 0.001). In both patients with ARDS and AE-ILDs, serum HO-1 level tended to decrease from diagnosis to 2 weeks after diagnosis, however, did not normalized. Composite parameters including serum HO-1, age, sex, and partial pressure of oxygen in arterial blood/fraction of inspired oxygen (P/F) ratio for prediction of 3-month mortality showed a higher AUC (ARDS: 0.925, AE-ILDs: 0.892) than did AUCs of a single predictor or combination of two or three predictors. Conclusion: Oxidative stress assessed by serum HO-1 is persistently high among enrolled patients for 2 weeks after diagnosis. Also, serum HO-1 levels at the diagnosis combined with age, sex, and P/F ratio could be clinically useful for predicting 3-month mortality in both ARDS and AE-ILD patients.

scholarly journals First Wave of COVID-19 in French Patients with Cystic Fibrosis

2020 ◽  
Vol 9 (11) ◽  
pp. 3624 ◽  
Author(s):  
Harriet Corvol ◽  
Sandra de Miranda ◽  
Lydie Lemonnier ◽  
Astrid Kemgang ◽  
Martine Reynaud Gaubert ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Transplant ◽  
Distress Syndrome ◽  
Rt Pcr ◽  
Respiratory Complications ◽  
Short Term ◽  
Transplant Patients

Viral infections are known to lead to serious respiratory complications in cystic fibrosis (CF) patients. Hypothesizing that CF patients were a population at high risk for severe respiratory complications from SARS-CoV-2 infection, we conducted a national study to describe the clinical expression of COVID-19 in French CF patients. This prospective observational study involves all 47 French CF centers caring for approximately 7500 CF patients. Between March 1st and June 30th 2020, 31 patients were diagnosed with COVID-19: 19 had positive SARS-CoV-2 RT-PCR in nasopharyngeal swabs; 1 had negative RT-PCR but typical COVID-19 signs on a CT scan; and 11 had positive SARS-CoV-2 serology. Fifteen were males, median (range) age was 31 (9–60) years, and 12 patients were living with a lung transplant. The majority of the patients had CF-related diabetes (n = 19, 61.3%), and a mild lung disease (n = 19, 65%, with percent-predicted forced expiratory volume in 1 s (ppFEV1) > 70). Three (10%) patients remained asymptomatic. For the 28 (90%) patients who displayed symptoms, most common symptoms at admission were fever (n = 22, 78.6%), fatigue (n = 14, 50%), and increased cough (n = 14, 50%). Nineteen were hospitalized (including 11 out of the 12 post-lung transplant patients), seven required oxygen therapy, and four (3 post-lung transplant patients) were admitted to an Intensive Care Unit (ICU). Ten developed complications (including acute respiratory distress syndrome in two post-lung transplant patients), but all recovered and were discharged home without noticeable short-term sequelae. Overall, French CF patients were rarely diagnosed with COVID-19. Further research should establish whether they were not infected or remained asymptomatic upon infection. In diagnosed cases, the short-term evolution was favorable with rare acute respiratory distress syndrome and no death. Post-lung transplant patients had more severe outcomes and should be monitored more closely.

scholarly journals Acute Interstitial Pneumonia (Hamman-Rich Syndrome) as a Cause of Idiopathic Acute Respiratory Distress Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Jackrapong Bruminhent ◽  
Shahla Yassir ◽  
James Pippim
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Distress Syndrome ◽  
Glucocorticoid Therapy ◽  
Unknown Etiology ◽  
Upper Respiratory Tract ◽  
Acute Interstitial Pneumonia

Hamman-Rich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. It should be considered as a cause of idiopathic acute respiratory distress syndrome. Confirmatory diagnosis requires demonstration of diffuse alveolar damage on lung histopathology. The main treatment is supportive care. It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. We report the case of a 77-year-old woman without pre-existing lung disease who initially presented with mild upper respiratory tract infection and then progressed to rapid onset of hypoxic respiratory failure similar to acute respiratory distress syndrome with unknown etiology. Despite glucocorticoid therapy, she did not achieve remission and expired after 35 days of hospitalization. The diagnosis of acute interstitial pneumonia was supported by the histopathologic findings on her lung biopsy.

scholarly journals Hydroxychloroquine, a successful treatment for lung disease in ABCA3 deficiency gene mutation: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Waleed Shaaban ◽  
Majeda Hammoud ◽  
Ali Abdulraheem ◽  
Yasser Yahia Elsayed ◽  
Nawal Alkazemi
Keyword(s):  
Interstitial Lung Disease ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Distress Syndrome ◽  
Complex Mixture ◽  
Gene Mutations ◽  
Poor Response ◽  
Severe Form ◽  
Severe Condition

Abstract Background Pulmonary surfactant is a complex mixture of lipids and specific proteins that stabilizes the alveoli at the end of expiration. Mutations in the gene coding for the triphosphate binding cassette transporter A3 (ABCA3), which facilitates the transfer of lipids to lamellar bodies, constitute the most frequent genetic cause of severe neonatal respiratory distress syndrome and chronic interstitial lung disease in children. Hydroxychloroquine can be used as an effective treatment for this rare severe condition. Case presentation We report a late preterm Bosnian baby boy (36 weeks) who suffered from a severe form of respiratory distress syndrome with poor response to intensive conventional management and whole exome sequencing revealed homozygous ABCA3 mis-sense mutation. The baby showed remarkable improvement of the respiratory condition after the initiation of Hydroxychloroquine, Azithromycin and Corticosteroids with the continuation of Hydroxychloroquine as a monotherapy till after discharge from the hospital. Conclusion Outcome in patients with ABCA3 mutations is variable ranging from severe irreversible respiratory failure in early infancy to chronic interstitial lung disease in childhood (ChILD) usually with the need for lung transplantation in many patients surviving this rare disorder. Hydroxychloroquine through its anti-inflammatory effects or alteration of intra-cellular metabolism may have an effect in treating cases of ABCA3 gene mutations.

scholarly journals In defence of extrapolation but not improvisation in SARS-CoV-2 lung disease

Breathe ◽  
2020 ◽  
Vol 16 (2) ◽  
pp. 200113
Author(s):  
Oleg Epelbaum ◽  
Irene Galperin
Keyword(s):  
Clinical Trials ◽  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Distress Syndrome ◽  
Point Of View ◽  
Evidence Based ◽  
Alternative Perspective ◽  
Based Medicine

Recently, the struggle against COVID-19 by respiratory and intensive care clinicians worldwide was punctuated by the sound of calls from a number of influential publications for an end to, as it were, improvisation and a return to principles of evidence-based medicine. The message was that management of SARS-CoV-2 lung disease needed to be guided strictly according to established dogma in acute respiratory distress syndrome unless supplanted by clinical trials specific to COVID-19. This position is predicated on the assumptions that knowledge about acute respiratory distress syndrome, and only about that entity, is directly translatable to SARS-CoV-2 lung disease, and that clinical trials enrolling COVID-19 patients will be completed in a sufficiently timely and rigorous fashion to influence empirical practice during the current pandemic. Clearly, there is room for an alternative perspective. In this Viewpoint, we aim to articulate a contrary point of view by resorting to arguments that are likely to resonate with frontline clinicians battling COVID-19.

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