Overproduction of vascular endothelial growth factor - Vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome
Overproduction of vascular endothelial growth factor - Vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome
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DOI：
10.1002/(sici)1097-4598(199811)21:11
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发表时间：
1998-11-01
期刊：
影响因子：
3.4
通讯作者：
Osame, M
中科院分区：
文献类型：
--
作者：
Watanabe, O;Maruyama, I;Osame, M
Crow-peptidase或POEMS综合征的多发性神经病，器官肿大，内分泌病，M蛋白，皮肤变化是一种罕见的多系统疾病，发病机制不明，与微血管病，新血管形成，血管通透性加快。我们检测了10例该综合征患者血清和脑脊液中血管内皮生长因子/血管通透性因子（VEGF）的水平。血清VEGF水平约为对照受试者或格林-巴利综合征（GBS）、慢性炎性脱髓鞘性多发性神经病（CIDP）和其他神经系统疾病患者的15-30倍。CSF中VEGF水平与GBS和CIDP相似。7例Crow-dopase综合征患者经常规治疗后血清中VEGF水平下降。Crow-peptide综合征患者VEGF的主要亚型为VEGF_（165），VEGF的升高不依赖于M蛋白的表达。(C)John Wiley & Sons，Inc. Muscle Nerve 21：1390-1397，1998.
Crow-Fukase or POEMS syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes is a rare multisystem disorder of obscure pathogenesis that is associated with microangiopathy, neovascularization, and accelerated vasopermeability. We examined the levels of the vascular endothelial growth factor/vascular permeability factor (VEGF) in the serum and cerebrospinal fluid (CSF) from 10 patients with this syndrome. Serum VEGF levels were about 15-30 times those in control subjects or patients with Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and other neurological disorders. The CSF VEGF levels, however, were similar to those found in GBS and CIDP, Elevated VEGF levels in the serum decreased in 7 patients with Crow-Fukase syndrome after conventional therapy. The principal isoform of VEGF in Crow-Fukase syndrome was VEGF165, Elevated VEGF was independent of M-protein, Our results suggest that the overproduction of VEGF is important in the pathogenesis of this disorder. (C) 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1390-1397, 1998.