ALVEOLAR CAPILLARY DYSPLASIA & TREATMENT
Definition: What is "Alveolar Capillary Dysplasia"?
Alveolar capillary dysplasia definition: (ACD) is defined as a lethal evolving anomaly of the pulmonary vasculature. It is normally illustrated as the miscarriage of the normal air-blood diffusion barrier in the newborn lung. It is usually supplementary with the misalignment of the pulmonary veins.
Symptoms & Signs
This pulmonary disease is present in very early infancy. Infants usually become critically sick in the first days of life with harsh hypoxemia & pulmonary hypertension, although presentation has been stated at 6 weeks of life in an infant with a patchy distribution of disease. Majority of the patients with this pulmonary disease will have further linked anomalies of the cardiovascular, gastroinstestinal, urogenital, or musculoskeletal systems.
Treatment: How to Treat "Alveolar Capillary Dysplasia"?
Mechanical ventilation, high concentrations of inspired oxygen, inhalational nitric oxide & ECMO support are included in the standard therapies. These treatments extend life by days to weeks, but haven't led to a long-term survival.
Rare cause of harsh & irreversible pulmonary hypertension usually presents with acute onset respiratory failure during the first 48 hours of life.
The diagnosis of this pulmonary disease should be considered in infants who is present with severe hypoxemia & idiopathic pulmonary hypertension & who do not answer appropriately after 7 to 10 days of neonatal intensive care treatment. The diagnosis can be confirmed by lung biopsy or autopsy. Referring to a pathologist with an experience in making this diagnosis may be helpful.