[23] Immunoexpression of CD117 and CD34 in Human Fetal and Neonatal Lungs Focusing on Alveolar Capillary Dysplasia

H-S Hwang, S-A Kim, S-J Jang, Y Lee. University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea

Background: Alveolar capillary dysplasia(ACD) is a rare lethal pulmonary disorder. Many infants with ACD have additional malformations, such as intestinal malrotation, duodenal atresia, imperforate anus, aganglionosis/hypoganglionosis of colon, absent gallbladder(GB), tracheoesophageal fistula with esophageal atresia, asplenia, arteriovenous malformation(AVM) of liver and trisomy 21. Recently, there are few manuscripts that 16q24.1 microdelition is associated with VATER, ACD and CD117 immunostaining is helpful to diagnose ACD.
Design: We selected several cases of autopsied fetal and infant lung tissue with variable malformations ; duodenal atresia(DA) with cardiac anomaly and single umbilical artery(2 cases), DA with Down syndrome(3 cases), VATER with/without DA(10 cases), absent GB(2 cases), asplenia(2 cases), CCAM(3 cases), Single umbilical artery(SUA,1 case), AVM of liver(1 case), neonatal ACD(2 cases) and normal control tissue(9 cases). Immunostaining of CD117 and CD34 are performed to evaluate the expression pattern, semiquantitatively.
Results: In two cases of ACD, there is uniform CD117 immunonegativity in the septal interstitial cells and decreased densities of CD34 positive capillaries. Relatively decreased densities of CD34 positive capillaries and markedly decreased numbers of CD117 positive septal interstitial cells are found in following cases ; DA with cardiac anomaly and SUA(2 cases), DA with Down syndrome(2 cases), VATER with DA(2 cases), each one case of VATER with asplenia, VATER, asplenia and CCAM. Remaining cases do not reveal any remarkable different expression patterns comparing with control cases.
Conclusions: DA with other malformation and asplenia are disorders that we may search for coexisting ACD in cases of persistent pulmonary hypertension in newborn.
Category: Autopsy

Wednesday, March 24, 2010 9:30 AM

Poster Session V # 24, Wednesday Morning

 

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