P1‐35: A case of chronic type II respiratory failure due to Bethlem myopathy

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.

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Efficacy of naloxone combined with non-invasive ventilator in the treatment of COPD complicated with type II respiratory failure

Minerva Surgery ◽

10.23736/s2724-5691.21.09291-1 ◽

2021 ◽

Author(s):

Xin XU ◽

Danfeng MA ◽

Feng ZHOU

Keyword(s):

Respiratory Failure ◽

Type Ii ◽

Non Invasive

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Assessment of Nonallelic Genetic Heterogeneity of Chronic (Type II and III) Spinal Muscular Atrophy

Human Heredity ◽

10.1159/000154164 ◽

1993 ◽

Vol 43 (6) ◽

pp. 380-387 ◽

Cited By ~ 10

Author(s):

L.M. Brzustowicz ◽

C. Mérette ◽

P.W. Kleyn ◽

T. Lehner ◽

L.H. Castüla ◽

...

Keyword(s):

Spinal Muscular Atrophy ◽

Genetic Heterogeneity ◽

Muscular Atrophy ◽

Type Ii ◽

Chronic Type

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Intravenous Sedation in Arnold-Chiari Malformation With Respiratory Failure

Anesthesia Progress ◽

10.2344/anpr-66-01-06 ◽

2019 ◽

Vol 66 (1) ◽

pp. 37-41 ◽

Cited By ~ 2

Author(s):

Yoshiki Shionoya ◽

Eishi Nakamura ◽

Takahiro Goi ◽

Kiminari Nakamura ◽

Katsuhisa Sunada

Keyword(s):

Respiratory Failure ◽

Respiratory Depression ◽

Chiari Malformation ◽

Dental Treatment ◽

Anesthetic Management ◽

Cranial Nerves ◽

Intravenous Sedation ◽

Type Ii ◽

Neck Extension ◽

Arnold Chiari Malformation

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.

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Isolation and identification of ‘Mycobacterium angelicum’ from a patient with type II respiratory failure: suggested reporting guidelines to molecular clinical laboratories

British Journal of Biomedical Science ◽

10.1080/09674845.2012.12069140 ◽

2012 ◽

Vol 69 (3) ◽

pp. 134-136 ◽

Cited By ~ 1

Author(s):

E. Davies ◽

J. Wieboldt ◽

T. Stanley ◽

Y. Maeda ◽

M. Smyth ◽

...

Keyword(s):

Respiratory Failure ◽

Reporting Guidelines ◽

Type Ii ◽

Isolation And Identification ◽

Clinical Laboratories

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Benign gastrobronchial fistula following oesophagectomy in a patient presenting with respiratory failure

BMJ Case Reports ◽

10.1136/bcr-2018-228537 ◽

2019 ◽

Vol 12 (9) ◽

pp. e228537 ◽

Cited By ~ 1

Author(s):

Kasper Favere ◽

Klaas Vanderbiest ◽

Jan Bresseleers ◽

Pieter Depuydt

Keyword(s):

Respiratory Failure ◽

Minute Ventilation ◽

Muscle Flap ◽

Causative Factor ◽

Type Ii ◽

Ivor Lewis ◽

Gastrobronchial Fistula ◽

Life Threatening ◽

Intercostal Muscle Flap

Benign gastrobronchial fistula (GBF) is a rare but potentially life-threatening complication of oesophagectomy for malignancy. We present a case of GBF post Ivor-Lewis surgery manifesting as pulmonary sepsis and type II respiratory failure. Clues to the diagnosis were persistent hypercapnia despite high minute ventilation, aspiration of gastric content through the endotracheal tube and accumulation of air in the nasogastric drainage bag. Flexible bronchoscopy confirmed the diagnosis. Surgical exploration identified necrosis of the proximal stomach as causative factor. Despite reconstruction of the oesophagogastric anastomosis and interposition of an intercostal muscle flap, the patient developed a new episode of type II respiratory failure. Bronchoscopy revealed in situ recurrence of the fistula. Patency of the fistula was proven through application of methylene blue with subsequent gastroscopy. A conservative, symptom-based, management was conducted. The patient died 6 hours later.

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