Alveolar Capillary Dysplasia

Below you will find more information about Alveolar Capillary Dysplasia from Medigest. If you believe that you are suffering from any of the symptoms of Alveolar Capillary Dysplasia it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Alveolar Capillary Dysplasia and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Alveolar Capillary Dysplasia comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

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.

Diagnosis

The diagnosis of this pulmonary disease should be considered in infants who is present with severe hypoxemia and idiopathic pulmonary hypertension, and 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.

Treatment

Mechanical ventilation, high concentrations of inspired oxygen, inhalational nitric oxide and 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.

Symptoms and Signs

This pulmonary disease is present in very early infancy. Infants usually become critically sick in the first days of life with harsh hypoxemia and 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.

Causes

Rare cause of harsh and irreversible pulmonary hypertension usually presents with acute onset respiratory failure during the first 48 hours of life.

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