Clinical Disorders of Primary Malfunctioning of the Lymphatic System

被引：19

作者：

Bellini, Carlo ^{[1
]}

Hennekam, Raoul C. M. ^{[2
]}

机构：

[1] Gaslini Inst, Emergency Dept, Neonatal Intens Care Unit, Genoa, Italy

[2] Univ Amsterdam, Acad Med Ctr, Dept Pediat & Translat Genet, NL-1105 AZ Amsterdam, Netherlands

来源：

DEVELOPMENTAL ASPECTS OF THE LYMPHATIC VASCULAR SYSTEM | 2014年 / 214卷

关键词：

INTESTINAL LYMPHANGIECTASIA; VASCULAR MALFORMATIONS; HEREDITARY LYMPHEDEMA; TRANSCRIPTION FACTOR; MUTATIONS; CHILDREN; CHYLOTHORAX; LINKAGE; FAMILY; GENE;

D O I：

10.1007/978-3-7091-1646-3_14

中图分类号：

R602 [外科病理学、解剖学]; R32 [人体形态学];

学科分类号：

100101 ;

摘要：

Primary lymphedema is defined as lymphedema caused by dysplasia of the lymph vessels. This complex group of diseases is discussed in detail from a clinical perspective. A review of the epidemiology and classification of lymphedema on the backdrop of its clinical presentation reveals weaknesses of the present classification system, which, however, is the basis for the choice of optimal patient care. Non-syndrome and syndrome types of primary lymphedema are presented in detail and related molecular findings are summarized.

引用

页码：187 / 204

页数：18

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