Journal of medical genetics · 2005
Multiple meningiomas: differential involvement of the NF2 gene in children and adults.
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Evans D G R, Watson C, King A, Wallace A J, Baser M E
Аннотация
Congenital diaphragmatic hernia (CDH) results from abnormal development of the diaphragm during fetal life, allowing abdominal organs to herniate through the defect into the thorax. Stunted lung growth is associated with pulmonary hypoplasia and pulmonary hypertension, which are the primary sources of morbidity and mortality for this population. Despite strides in neonatal and surgical care, the management of neonates with CDH remains challenging. Optimal treatment strategies are still largely unknown. Many centers utilize gentle ventilation, permissive hypercapnia, and pulmonary hypertension treatment inclusive of nitric oxide, sildenafil, or epoprostenol, delayed surgical repair, and extracorporeal membrane oxygenation (ECMO). Evidence-based guidelines are needed to enhance CDH care practices and better outcomes. The successful management of CDH is a collaborative team effort from the prenatal to the postnatal period and beyond.