Genetic evaluation and management of fetal chylothorax: Review and insights from a case of Noonan syndrome
Journal
Lymphology
Journal Volume
42
Journal Issue
3
Pages
134-138
Date Issued
2009
Author(s)
Chen C.-H.
Chen T.-H.
Kuo S.-J.
Chen C.-P.
Lee D.-J.
Ke Y.-Y.
Yeh K.-T.
Ma G.-C.
Liu C.-S.
MING CHEN
Abstract
Fetal chylothorax is one of a very few syndromes that can be treated in utero with thoracoamniotic shunting or pleurodesis by OK-432 as two major therapeutic modalities. We report on a fetus with Noonan syndrome and a missense mutation c.182A > C (p.Asp61Ala) of PTPN11 who responded poorly to antenatal pleurodesis by OK-432. Based on our previous publication and this case study, we propose that fetal chylothorax of a distinct genetic origin may respond poorly to OK-432 pleurodesis.
Type
review