Congenital diaphragmatic hernia (CDH) is one of the most common and devastating conditions known to pediatric surgeons. Worldwide, a baby with CDH is born every 10 min. The estimated annual cost of CDH in the United States alone is more than $230 million. While most cases of CDH are surgically correctable, these patients suffer significant morbidities including pulmonary hypertension in the neonatal period and long-term ventilatory insufficiency due to pulmonary hypoplasia and abnormal lung development. Furthermore, despite significant advancements in supportive neonatal critical care, mortality in the most severe CDH cases is unacceptably high. Thus, mechanistic studies to help develop better interventions to improve the outcomes of these patients are needed.
Developing normal and CDH fetal lungs might temporarily look like a dynamically changing topologic mosaic with varying cell types with dissimilar proliferation/differentiation rates, diverse patterns of lung tissue damage, variable metabolic landscape, and different structures. The local interaction between these factors and their accompanying processes in addition to the potential role of other systemic factors might lead to formation of a common vector of biological response unique to each zone. CDH developing lungs have altered parenchymal morphology, which results in different from normal lungs local topological zone, different cell types distribution, cell-cell interactions and signaling. The main goal is to have studies of topological, signaling and metabolic changes in normal and CDH lungs to induce functional lungs development.
Contributing manuscripts should address aspects of normal and altered lung development, the interaction between various networks forming during normal lung development and what is altered in CDH (action of mechanical forces, activation of mucosal mast cells, production and secretion of damage-associated molecular pattern substances, cell-cell communications, extracellular matrix remodeling, low-grade local pulmonary inflammation, and cardiac contraction-induced periodic agitation of lung tissue, among others) as well as the effect of different treatment modalities.
Congenital diaphragmatic hernia (CDH) is one of the most common and devastating conditions known to pediatric surgeons. Worldwide, a baby with CDH is born every 10 min. The estimated annual cost of CDH in the United States alone is more than $230 million. While most cases of CDH are surgically correctable, these patients suffer significant morbidities including pulmonary hypertension in the neonatal period and long-term ventilatory insufficiency due to pulmonary hypoplasia and abnormal lung development. Furthermore, despite significant advancements in supportive neonatal critical care, mortality in the most severe CDH cases is unacceptably high. Thus, mechanistic studies to help develop better interventions to improve the outcomes of these patients are needed.
Developing normal and CDH fetal lungs might temporarily look like a dynamically changing topologic mosaic with varying cell types with dissimilar proliferation/differentiation rates, diverse patterns of lung tissue damage, variable metabolic landscape, and different structures. The local interaction between these factors and their accompanying processes in addition to the potential role of other systemic factors might lead to formation of a common vector of biological response unique to each zone. CDH developing lungs have altered parenchymal morphology, which results in different from normal lungs local topological zone, different cell types distribution, cell-cell interactions and signaling. The main goal is to have studies of topological, signaling and metabolic changes in normal and CDH lungs to induce functional lungs development.
Contributing manuscripts should address aspects of normal and altered lung development, the interaction between various networks forming during normal lung development and what is altered in CDH (action of mechanical forces, activation of mucosal mast cells, production and secretion of damage-associated molecular pattern substances, cell-cell communications, extracellular matrix remodeling, low-grade local pulmonary inflammation, and cardiac contraction-induced periodic agitation of lung tissue, among others) as well as the effect of different treatment modalities.
