| Literature DB >> 24000203 |
Vered Nir1, Ludmila Guralnik, Galit Livnat, Ronen Bar-Yoseph, Fahed Hakim, Anat Ilivitzki, Lea Bentur.
Abstract
Gorham-Stout syndrome is a very rare syndrome characterized by progressive angiomatosis and lymphangiomas involving multiple organs. We describe herein a girl with progressive Gorham-Stout syndrome since the age of 13 years. Her disease involved the mediastinum, pericardium, vertebrae, ribs, and skull and did not respond to interferon and bisphosphonates. Propranolol administration was initiated at the age of 18 years and was associated with improvement in pulmonary function tests and involution of the mediastinal and hilar hemangiomatous lesions. The possible beneficial effect of propranolol in Gorham-Stout syndrome should be further investigated.Entities:
Keywords: Gorham-Stout syndrome; propranolol
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Year: 2013 PMID: 24000203 DOI: 10.1002/ppul.22869
Source DB: PubMed Journal: Pediatr Pulmonol ISSN: 1099-0496