Work

Alveolar capillary dysplasia: an example of health system data to understand rare conditions

Year: 2026

Type: article

Abstract: Congenital lung disorders are rare but commonly present with severe early respiratory failure. Diagnosis is difficult as this presentation mimics common conditions such as respiratory distress syndrome (RDS), neonatal acute RDS, primary pulmonary hypertension (PPHN), and cardiac disease. 1 , 2 Confirming a congenital lung disorder is often complex, requiring multiple investigative layers including echocardiography, computerised tomographic imaging, genetic testing and surgical biopsy. 1 , 3 Predominantly, these invasive investigations occur in a critically unwell infants needing high level neonatal intensive care unit (NICU) support. Traditionally, severe congenital lung disorders have proven fatal in early life with long-term treatment limited to transplantation. One of the more common severe congenital lung disorders is alveolar capillary dysplasia (ACD), especially when associated with misalignment of pulmonary veins (MPV). 1 , 3 , 4 , 5 ACD/MPV is characterised by multiple structural abnormalities of lobular development, alveolar septal thickening and abnormal pulmonary capillaries and pulmonary veins. 1 , 3 Difficulties establishing diagnosis and lack of data on clinical treatments hampers clinicians in decision making around suitable management pathways and caregiver counselling at a time of great uncertainty.

Source: Pediatric Research

DOI: 10.1038/s41390-026-05357-x

Authors: Samuel B. Axford, Ruth K. Armstrong, Anastasia Pellicano, David G. Tingay

Institutions: Royal Children's Hospital, The University of Melbourne, Murdoch Children's Research Institute

Language: English

Topic: Neonatal Respiratory Health Research

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,390