8 research outputs found
Congenital acinar dysplasia: a lethal entity
Congenital acinar dysplasia is a lethal, developmental lung malformation resulting in neonatal respiratory insufficiency. This entity is characterized by pulmonary hypoplasia and arrest in the pseudoglandular stage of development, resulting in the absence of functional gas exchange. The etiology is unknown, but a relationship with the disruption of the TBX4-FGF10 pathway has been described. There are no definitive antenatal diagnostic tests. It is a diagnosis of exclusion from other diffuse embryologic lung abnormalities with identical clinical presentations that are, however, histopathologically distinct
The anatomic substrate of irreversible airway obstruction and barotrauma in a case of hurricane-triggered fatal status asthmaticus during puerperium: Lessons from an autopsy
Non-fully reversible airway obstruction in fatal asthma is often seen in association with profound structural changes of the bronchial wall, termed airway remodeling. Evidence suggests that heavy precipitation events can trigger epidemics of severe asthma. We present a case of fatal asthma in a young woman with no prior near-fatal exacerbations and postulate that the patient's extensive airway remodeling and puerperal state (susceptibility factors), in combination with a massive allergen challenge during a hurricane landfall (triggering factor), played a central role in her death. The autopsy revealed diffuse obstruction of proximal and distal bronchi by mucous plugs together with transmural chronic inflammation, tissue eosinophilia, extensive goblet cell hyperplasia with MUC-5 expression and airway smooth muscle (ASM) thickening. The observed distribution of airway remodeling was heterogeneous with sparing of the lingula, which exhibited hyperinflation and expansion of perivascular spaces indicative of dissecting air. The massive stagnation of mucus and significant inter-airway structural heterogeneity created an anatomical substrate for unequal airflow distribution facilitating the development of barotrauma. Although not considered conventional risk factors for fatal asthma, we believe that in this case, the patient's puerperal state in conjunction with an extreme environmental event dispersing aeroallergens were major contributors to the development of a fatal asthma attack. Our autopsy findings suggest that effective strategies to evacuate stagnated mucus and induce relaxation of thickened ASM are crucial in the management of life-threatening asthma exacerbations. Keywords: Fatal asthma, Airway remodeling, Barotrauma, Thunderstorm, Hurricane, Autops
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How might non nutritional sucking protect from sudden infant death syndrome
Epidemiology has identified an association between the use of pacifiers and protection from sudden infant death syndrome (SIDS). The use of pacifiers for SIDS prevention fails to gain adoption partly because there is no widely accepted physiologic mechanism to explain the epidemiologic association. Additionally, the scientific literature available on pacifier use focuses largely on the probable adverse effects. We hypothesize that pacifier use and all other forms of non-nutritional sucking (specifically digit sucking, also known as thumb sucking) is a life saving defense mechanism meant to splint open and stabilize the collapsible portion of the upper airway in infants.The main objective of this review article is to propose a mechanism to explain how pacifiers might help prevent SIDS. If the medical community accepts this mechanism, it can help promote pacifier use by the public and potentially reduce the incidence of SIDS
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Safety and efficacy of holmium laser enucleation of prostate as salvage procedure for persistent or recurrent lower urinary tract symptoms secondary to bladder outlet obstruction after prior prostate artery embolization: a match analysis
Transplant outcome for patients with acquired aplastic anemia over the age of 40: has the outcome improved?
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