Pulmonary and pleural lymphatic endothelial cells from pediatric, but not adult, patients with Gorham-Stout disease and generalized lymphatic anomaly, show a high proliferation rate
(2016) In Orphanet Journal of Rare Diseases 11(1).- Abstract
Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or... (More)
Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or Gorham-Stout disease were studied using an immunohistochemical approach, targeting lymphendothelial markers (D2-40/Prox-1) and a proliferation marker (Ki-67). Results: We found significant proliferation and growth in these lesions in pediatric patients but not in adults. Furthermore, the data may suggest that the disease process is at least partly reversible. Conclusions: These malformations of the lymphatic system proliferate at a significant rate long after birth, which could suggest that the clinical approach for children should be different from adults.
(Less)
- author
- Mori, Michiko LU ; Dictor, Michael LU ; Brodszki, Nicholas LU ; López-Gutiérrez, Juan Carlos ; Beato, María ; Erjefält, Jonas S. LU and Eklund, Erik A. LU
- organization
- publishing date
- 2016-05-18
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Chylothorax, Generalized lymphatic anomaly, Gorham-Stout disease, Lymphatic malformation, VEGF-C
- in
- Orphanet Journal of Rare Diseases
- volume
- 11
- issue
- 1
- article number
- 67
- publisher
- BioMed Central (BMC)
- external identifiers
-
- scopus:84975060426
- pmid:27194137
- wos:000377508900003
- ISSN
- 1750-1172
- DOI
- 10.1186/s13023-016-0449-4
- language
- English
- LU publication?
- yes
- id
- b38b302f-76ed-4a9c-95f2-3358814e31b3
- date added to LUP
- 2016-07-06 15:04:14
- date last changed
- 2024-04-05 03:29:42
@article{b38b302f-76ed-4a9c-95f2-3358814e31b3,
abstract = {{<p>Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or Gorham-Stout disease were studied using an immunohistochemical approach, targeting lymphendothelial markers (D2-40/Prox-1) and a proliferation marker (Ki-67). Results: We found significant proliferation and growth in these lesions in pediatric patients but not in adults. Furthermore, the data may suggest that the disease process is at least partly reversible. Conclusions: These malformations of the lymphatic system proliferate at a significant rate long after birth, which could suggest that the clinical approach for children should be different from adults.</p>}},
author = {{Mori, Michiko and Dictor, Michael and Brodszki, Nicholas and López-Gutiérrez, Juan Carlos and Beato, María and Erjefält, Jonas S. and Eklund, Erik A.}},
issn = {{1750-1172}},
keywords = {{Chylothorax; Generalized lymphatic anomaly; Gorham-Stout disease; Lymphatic malformation; VEGF-C}},
language = {{eng}},
month = {{05}},
number = {{1}},
publisher = {{BioMed Central (BMC)}},
series = {{Orphanet Journal of Rare Diseases}},
title = {{Pulmonary and pleural lymphatic endothelial cells from pediatric, but not adult, patients with Gorham-Stout disease and generalized lymphatic anomaly, show a high proliferation rate}},
url = {{http://dx.doi.org/10.1186/s13023-016-0449-4}},
doi = {{10.1186/s13023-016-0449-4}},
volume = {{11}},
year = {{2016}},
}