Alveolar capillary dysplasia with misalignment of pulmonary veins

Common Name(s)

Alveolar capillary dysplasia with misalignment of pulmonary veins

Congenital alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is characterized histologically by failure of formation and ingrowth of alveolar capillaries that then do not make contact with alveolar epithelium, medial muscular thickening of small pulmonary arterioles with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated pulmonary veins running alongside pulmonary arterioles and sharing the same adventitial sheath. Less common features include a reduced number of alveoli and a patchy distribution of the histopathologic changes. The disorder is associated with persistent pulmonary hypertension of the neonate and shows varying degrees of lability and severity ({2:Boggs et al., 1994}). Affected infants present with respiratory distress resulting from pulmonary hypertension in the early postnatal period, and the disease is uniformly fatal within the newborn period ({11:Vassal et al., 1998}). Additional features of ACDMPV include multiple congenital anomalies affecting the cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems, as well as disruption of the normal right-left asymmetry of intrathoracic or intraabdominal organs ({6:Sen et al., 2004}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Alveolar capillary dysplasia with misalignment of pulmonary veins" for support, advocacy or research.

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Scientific Literature

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Remodulin as Add-on Therapy for the Treatment of Persistent Pulmonary Hypertension of the Newborn
 

Status: Not yet recruiting

Condition Summary: Persistent Pulmonary Hypertension of the Newborn

 

Last Updated: 6 Oct 2014

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Early iNO for Oxidative Stress, Vascular Tone and Inflammation in Babies With Hypoxic Respiratory Failure
 

Status: Not yet recruiting

Condition Summary: Persistent Fetal Circulation Syndrome; Hypertension, Pulmonary, of Newborn, Persistent; Persistent Pulmonary Hypertension of Newborn

 

Last Updated: 3 Mar 2014

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Pharmacokinetics of Sildenafil in Premature Infants
 

Status: Recruiting

Condition Summary: Persistent Pulmonary Hypertension of the Newborn

 

Last Updated: 8 Sep 2014

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