MISALIGNMENT OF PULMONARY VEINS WITH ALVEOLAR-CAPILLARY DYSPLASIA - AFFECTED SIBLINGS AND VARIABLE PHENOTYPIC-EXPRESSION

被引:72
作者
BOGGS, S
HARRIS, MC
HOFFMAN, DJ
GOEL, R
MCDONALDMCGINN, D
LANGSTON, C
ZACKAI, E
RUCHELLI, E
机构
[1] CHILDRENS HOSP PHILADELPHIA, DEPT PATHOL, PHILADELPHIA, PA 19104 USA
[2] UNIV PENN, SCH MED, DIV NEONATOL, PHILADELPHIA, PA USA
[3] UNIV PENN, SCH MED, DIV GENT, PHILADELPHIA, PA USA
[4] UNIV PENN, SCH MED, DEPT PATHOL, PHILADELPHIA, PA 19104 USA
[5] BAYLOR COLL MED, DEPT PATHOL, DALLAS, TX USA
[6] CHILDRENS HOSP, HOUSTON, TX USA
关键词
D O I
10.1016/S0022-3476(94)70267-5
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Misalignment of pulmonary veins with alveolar capillary dysplasia is recognized as a rare cause of persistent pulmonary hypertension of the neonate. Until now, misalignment of pulmonary veins was thought to be a random occurrence, but its appearance in siblings at our institution suggests that there may be a familial predisposition. There have been reports of variable expression and variable severity in this disease; our report describes this variability in family members.
引用
收藏
页码:125 / 128
页数:4
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