UNASSIGNED: Alveolar @entity8025 with @entity33711 ( @entity13685 / @entity6688 ) is a rare neonatal pathology that combines refractory @entity519 with severe @entity120 , and leads to @entity204 every time. Histologic examination of lung tissue confirms the diagnosis and is characterized by a decreased number of @entity358 , immature lobular development, and abnormal proximity between pulmonary arteries and veins. This abnormal proximity is responsible for an arteriovenous shunt. We report five cases confirmed by postmortem histology, which occurred over 14 years in Lower Normandy (France). The cumulative incidence is therefore of 1.8 for 100,000 births. In these five cases, the first symptoms appeared during the first hour of life and @entity204 occurred before 24h in four of five cases. The @entity1 with the longest survival had mild histological lesions and delayed emergence of the first symptoms. Genitourinary and @entity344 were associated with @entity13685 / @entity6688 in two cases, and bilateral @entity358 in three cases. Optimized invasive ventilation, pulmonary vasodilators, vasoactive drugs, and pulmonary surfactant did not improve survival. Extracorporeal membrane oxygenation (ECMO) was not used. We present a review of the literature on @entity13685 / @entity6688 , a clinical and histological entity little known to both clinicians and pathologists, whereas a premortem diagnosis is possible and genetic counseling in affected families can be suggested.

[Alveolar XXXX with @entity33711 : A fatal cause of refractory neonatal cyanosis.]

@entity1 :: ('9606', 'Species') :: ['patient'] @entity8025 :: ('MESH:C536590', 'Disease') :: ['capillary dysplasia'] @entity120 :: ('MESH:D006976', 'Disease') :: ['pulmonary arterial hypertension'] @entity344 :: ('MESH:D005767', 'Disease') :: ['gastrointestinal anomalies'] @entity33711 :: ('MESH:D010547', 'Disease') :: ['misalignment of pulmonary veins'] @entity519 :: ('MESH:D000860', 'Disease') :: ['hypoxemia'] @entity358 :: ('MESH:D008171', 'Disease') :: ['pulmonary capillaries', 'pulmonary hypoplasia'] @entity13685 :: ('MESH:C535474', 'Disease') :: ['ACD'] @entity6688 :: ('10244', 'Species') :: ['MPV'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity8025 :: (MESH:C536590,Disease) :: ['capillary dysplasia']
