|
KMID : 0918520170170010024
|
|
Journal of the Korean Society of Inherited Metabolic Disease
2017 Volume.17 No. 1 p.24 ~ p.30
|
|
|
A Case of Gorham-Stout Disease with Life-threatening Chylothorax Successfully Treated with the Combined Therapy of mTOR Inhibitor and Beta-blocker
|
|
Ryu Kyung-Guk
Seo Go-Hun
Kim Yoon-Myung
Choi Jin-Ho
Yoo Han-Wook
Lee Beom-Hee
|
|
|
Abstract
|
|
|
|
Gorham-Stout disease is a rare disorder characterized by lymphovascular proliferation and destruction of osseous matrix. The etiology of this condition remains poorly understood. Chylothorax as a consequence of lymphatic leakage in thoracic cage may cause a severe life-threatening complication, accompanying respiratory difficulty. Currently, there is no standard management for this extremely rare condition. Here we describe a patient affected by Gorham-Stout disease successfully managed by the combined treatment of mTOR inhibitor and beta-blocker. A previously healthy 11-year-old female developed dyspnea and chest pain with a massive pleural effusion. The ligation of right thoracic duct and bilateral pleurodesis temporarily decreased her pleural effusion, which was aggravated repetitively and required frequent admission and tube thoracotomies. Along with bilateral pleural adhesiolysis with thoracotomy, the combined treatment of oral beta-blocker and mTOR inhibitor was commenced. After 1 month of oral medication, her pleural effusion was not increased and she was free of respiratory difficulty on room air without chest tubes. Over eleven months of treatment, no serious adverse reaction was noted and her condition has been stable with no further admission required.
|
|
|
KEYWORD
|
|
|
Gorham-Stout disease, Chylothorax, beta-Blockers, mTOR Serine-Threonine kinases, Thoracotomy
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
|