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- W1971477827 abstract "Alveolar capillary dysplasia (ACD) is a uniformly lethal lung disorder found in newborns that is characterized by severe pulmonary hypertension and hypoxemia. Histologically, it is identified by inadequately capillarized alveoli. 1 Janney C.G. Askin F.B. Kuhn III, C. Congenital alveolar capillary dysplasia An unusual cause of respiratory distress in the newborn. Am J Clin Pathol. 1981; 76: 722-727 Crossref PubMed Scopus (125) Google Scholar The association of ACD with abnormalities in other organ systems, notably the genitourinary and gastrointestinal tracts, has been established and is seen in approximately 60% of cases. 2 Khorsand J. Tennant R. Gillies C. Phillips A.F. Congenital alveolar capillary dysplasia a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn. Pediatr Pathol. 1985; 3: 299-306 Crossref PubMed Scopus (37) Google Scholar , 3 Chelliah B.P. Brown D. Cohen M. Talleyrand A.J. Shen-Schwarz S. Alveolar capillary dysplasia—a cause of persistent pulmonary hypertension unresponsive to a second course of extracorporeal membrane oxygenation. Pediatrics. 1995; 96: 1159-1161 PubMed Google Scholar , 4 Sirikin W. O’Hare B.P. Cox P.N. Perrin D. Cutz E. Silver M.M. Alveolar capillary dysplasia lung biopsy diagnosis, nitric oxide responsiveness, and bronchial generation count. Pediatr Pathol Lab Med. 1997; 17: 125-132 PubMed Google Scholar The association of ACD with structural heart disease, however, has only just recently been reported. 5 Garola R.E. Thibeault D.W. Alveolar capillary dysplasia, with and without misalignment of pulmonary veins an association of congenital anomalies. Am J Perinatol. 1998; 15: 103-107 Crossref PubMed Scopus (45) Google Scholar From 1991 to 1998, we identified 4 patients with histologically proven ACD who underwent treatment at our institution. Two of the 4 patients had associated structural heart disease, which consisted of an atrioventricular septal defect (AVSD), mild left ventricular hypoplasia, and coarctation of the aorta. In addition to the 4 patients described in the following, 2 additional patients with histories suggestive of ACD, including 1 with an AVSD and aortic arch hypoplasia, were noted, but not included in this report because requests for autopsy or lung biopsy were declined, and a definitive diagnosis was unavailable. Although many initial clinical findings and problems might have been attributed to their anatomic heart disease, the superimposition of ACD resulted in an atypical and labile clinical course, which became explainable once the diagnosis of ACD had been established. Importantly, based on previous experience and clinical suspicions, we were able to prospectively diagnose ACD in 1 of our most recent patients, thereby avoiding futile and prolonged clinical interventions. Such a prospective diagnosis of ACD in a patient with congenital heart disease has, to our knowledge, not previously been reported. In this report, we review our institutional experience with this disorder, emphasizing the clinical characteristics that might suggest the presence of ACD in infants with associated cardiac disease." @default.
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- W1971477827 date "1999-09-01" @default.
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- W1971477827 title "Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease" @default.
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- W1971477827 doi "https://doi.org/10.1016/s0002-9149(99)00395-1" @default.
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