title: [Alveolar capillary dysplasia: a case report and review of literature].
abstract: OBJECTIVE
  To report a newborn infant who died of alveolar capillary dysplasia (ACD). The literature on about 20 cases of ACD was reviewed.
  
  
  METHODS
  A retrospective review of records of infants from Medline with a diagnosis of ACD was carried out.
  
  
  RESULTS
  The case was a newborn female infant who developed respiratory distress 5 hours after an uncomplicated delivery. She died at the fourth day after birth despite full ventilatory support. The lung autopsy provided a diagnosis of ACD. In the 21 infants, 7 were male and 14 were female; 19 infants were born full-term and 2 were born pre-term. The birth weight of 19 infants and Apgar score of 15 infants were normal; 16 infants developed progressing tachypnea and cyanosis within 24 hours of age, 5 developed cyanosis at 1 day to 19 days. Echocardiography demonstrated a right to left shunt in the hearts of all the 21 infants, and pulmonary hypertension in 20 infants. Twenty infants were treated with conventional mechanical ventilation, 7 infants with high-frequency oscillatory ventilation and 12 infants with extracorporeal membrane oxygenation (ECMO). Fourteen infants were also treated with inhaled nitric oxide therapy and 4 with exogenous surfactant. Diagnostic open lung biopsy was performed in 6 infants. The chest radiography showed normal findings in 3 infants, pneumothoraces in 9 infants, reticular markings, granular, patchy or diffuse opacity in lungs of 7 infants, and decreased pulmonary vascular markings in two infants. All the 21 infants died; 8 of them died within 10 days of age, 7 within 30 days of age, and one died at the age of 4 months who was the longest survivor. Fourteen infants were associated with congenital malformations, such as cardiovascular, gastrointestinal, and genitourinary systems, including one infant associated with chromosomal abnormalities, two infants of familial genetic predisposition.
  
  
  CONCLUSIONS
  At present, ACD is still a disease with poor prognosis, significant medical expenses and no specific treatment. When respiratory failure or persistent pulmonary hypertension (PPHN) is persistent after routine treatment in an infant, ACD should be highly suspected and conventional open-lung biopsy should be preformed to confirm the diagnosis.
authors.affiliation: Department of Neonatology, Southern Medical University, Guangzhou 510515, China.
authors.forename: Ning
authors.initials: N
authors.lastname: Li
authors.forename: Xin-hua
authors.initials: XH
authors.lastname: Zhou
authors.forename: Hong-wu
authors.initials: HW
authors.lastname: Chen
authors.forename: Wei-min
authors.initials: WM
authors.lastname: Huang
authors.forename: Xiao-yan
authors.initials: XY
authors.lastname: Song
authors.forename: Ying-min
authors.initials: YM
authors.lastname: Yao
mesh_terms: D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis* / Q000473:pathology; D011650:Pulmonary Alveoli / Q000002:abnormalities / Q000473:pathology