期刊
PEDIATRIC BLOOD & CANCER
卷 69, 期 -, 页码 -出版社
WILEY
DOI: 10.1002/pbc.28985
关键词
generalized lymphatic anomaly; Gorham– Stout disease; kaposiform lymphangiomatosis; lymphatic malformation; sirolimus; vascular malformation
CLAs are congenital diseases of the lymphatic circulation system associated with significant morbidity and early mortality. This article presents clinical features of four CLAs and provides perspectives on diagnostic testing and disease management, including supportive care, medical therapies, and other interventions, based on three cases from the authors' practice.
Complex lymphatic anomalies (CLA) are congenital diseases of the lymphatic circulation system that are associated with significant morbidity and early mortality. While guidelines for the comprehensive evaluation of the CLA were recently published, the diagnostic approach and medical management are not standardized. This article presents the clinical features of four CLA: Gorham-Stout disease, generalized lymphatic anomaly, kaposiform lymphangiomatosis, and central collecting lymphatic anomaly. We also offer three cases from the authors' practice and our views on diagnostic testing and disease management including supportive care, medical therapies, and other interventions.
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