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Aplasia Cutis Congenita and Lymphangiectasia-Reply

Am J Dis Child. 1985;139(12):1178. doi:10.1001/archpedi.1985.02140140012005.
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In Reply.—In both Turner's and Noonan's syndromes, the severe and sometimes persistent edema of hands and feet is probably the most generally recognized lymphatic abnormality.1 The article by Benson and co-workers,2 regretfully misquoted in our report, described hypoplasia or aplasia of superficial lymph tracts of the lower limbs in adult patients with genotype XO who had persistent peripheral edema. These observations and the reported association between intestinal lymphangiectasia and Noonan's syndrome in patients with peripheral edema, like that observed in Turner's syndrome,1,3 may have generated the erroneous claim of an association between Turner's syndrome and intestinal lymphangiectasia. As Gleason rightly points out, this association has not yet been reported, although it is plausible.


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