scholarly journals Tubular aggregate myopathy presenting with acute type II respiratory failure and severe orthopnoea

Thorax ◽  
2005 ◽  
Vol 61 (1) ◽  
pp. 89-90 ◽  
Author(s):  
N Shahrizaila
2006 ◽  
Vol 26 (3) ◽  
pp. 1109-1123 ◽  
Author(s):  
Daniela S. Bassères ◽  
Elena Levantini ◽  
Hongbin Ji ◽  
Stefano Monti ◽  
Shannon Elf ◽  
...  

ABSTRACT The leucine zipper family transcription factor CCAAT enhancer binding protein alpha (C/EBPα) inhibits proliferation and promotes differentiation in various cell types. In this study, we show, using a lung-specific conditional mouse model of C/EBPα deletion, that loss of C/EBPα in the respiratory epithelium leads to respiratory failure at birth due to an arrest in the type II alveolar cell differentiation program. This differentiation arrest results in the lack of type I alveolar cells and differentiated surfactant-secreting type II alveolar cells. In addition to showing a block in type II cell differentiation, the neonatal lungs display increased numbers of proliferating cells and decreased numbers of apoptotic cells, leading to epithelial expansion and loss of airspace. Consistent with the phenotype observed, genes associated with alveolar maturation, survival, and proliferation were differentially expressed. Taken together, these results identify C/EBPα as a master regulator of airway epithelial maturation and suggest that the loss of C/EBPα could also be an important event in the multistep process of lung tumorigenesis. Furthermore, this study indicates that exploring the C/EBPα pathway might have therapeutic benefits for patients with respiratory distress syndromes.


2018 ◽  
Vol 37 ◽  
pp. S20 ◽  
Author(s):  
A. Bond ◽  
A. Teubner ◽  
M. Taylor ◽  
C. Cawley ◽  
J. Varden ◽  
...  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Chun-Yu Lin ◽  
Tao-Hsin Tung ◽  
Meng-Yu Wu ◽  
Chi-Nan Tseng ◽  
Feng-Chun Tsai

Abstract Background The DeBakey classification divides Stanford acute type A aortic dissection (ATAAD) into DeBakey type I (D1) and type II (D2) according to the extent of acute aortic dissection (AAD). This retrospective study aimed to compare the early and late outcomes of D1-AAD and D2-AAD through a propensity score-matched analysis. Methods Between January 2009 and April 2020, 599 consecutive patients underwent ATAAD repair at our institution, and were dichotomized into D1 (n = 543; 90.7%) and D2 (n = 56; 9.3%) groups. Propensity scoring was performed with a 1:1 ratio, resulting in a matched cohort of 56 patients per group. The clinical features, postoperative complications, 5-year cumulative survival and freedom from reoperation rates were compared. Results In the overall cohort, the D1 group had a lower rate of preoperative shock and more aortic arch replacement with longer cardiopulmonary bypass time. The D1 group had a higher in-hospital mortality rate than the D2 group in overall (15.8% vs 5.4%; P = 0.036) and matched cohorts (19.6% vs 5.4%; P = 0.022). For patients that survived to discharge, the D1 and D2 groups demonstrated similar 5-year survival rates in overall (77.0% vs 85.2%; P = 0.378) and matched cohorts (79.1% vs 85.2%; P = 0.425). The 5-year freedom from reoperation rates for D1 and D2 groups were 80.0% and 97.1% in overall cohort (P = 0.011), and 93.6% and 97.1% in matched cohort (P = 0.474), respectively. Conclusions Patients with D1-AAD had a higher risk of in-hospital mortality than those with D2-AAD. However, for patients who survived to discharge, the 5-year survival rates were comparable between both groups.


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