摘要
血管瘤(HIS)是婴幼儿最常见的良性肿瘤,通常病变可自行消除。血管瘤在新生儿中产生并发症的概率为10-20%,这就需要医学干预和手术治疗。数十年来,口服(或病灶内)皮质类固醇已成为传统的主流治疗方法。2008年,Leaute-Labreze等人发表了论文,首次报道了应用心得安(普奈诺尔)治疗婴儿血管瘤的重大发现。 血管瘤常伴有复杂并发症,本文即报道了一名5个月大 的法洛四联症幼儿病患,因缺氧发作而停止使用心得安,却意外的产生面部和声门下血管瘤,并最终确诊为 PHACES 综合征(颅窝后畸形、血管瘤、动脉异常、主动脉狭窄和(或)心脏缺损、眼部异常及胸骨裂隙)。据我们所知,伴有皮肤和呼吸道婴幼儿血管瘤(PHACES 综合征)在国内外文献中均为首次报告。该病例的发病机制的解释是基于心得安的作用机制 :其首先使血管收缩并下调proangiogenetic 因素、诱导内皮细胞凋亡。长年来,β-受体阻断剂在许多疾病的治疗中得到了广泛应用。尽管β-受体阻断剂的多效性作用机制尚未彻底清楚,但其主要作用于神经激素调节系统并能引起血管再生,在治疗心血管、肿瘤疾病方面有着很好的疗效。
关键词: 呼吸道血管瘤,颌面血管瘤,β-受体阻断剂,PHACES综合征,心得安,声门下血管瘤
Current Medicinal Chemistry
Title:A PHACES Syndrome Unmasked by Propranolol Interruption in a Tetralogy of Fallot Patient: Case Report and Extensive Review on New Indications of Beta Blockers
Volume: 21 Issue: 27
Author(s): G. Bronzetti, A. Patrizi, F. Giacomini, F. Savoia, B. Raone, M. Brighenti, M. Bonvicini, I. Neri and G.D. Gargiulo
Affiliation:
关键词: 呼吸道血管瘤,颌面血管瘤,β-受体阻断剂,PHACES综合征,心得安,声门下血管瘤
摘要: Infantile hemangiomas (IHs) are the most common benign tumors of infancy and usually they don't require specific therapy. In 10-20% of cases IHs are able to generate complication and medical/surgical intervention is needed. For many decades standard treatment consisted in oral or intralesional corticosteroids until Leaute-Labreze and colleagues published the first report on the efficacy of propranolol for cutaneous infantile hemangiomas in 2008. IHs can be sometimes part of complex syndrome. Here we report the case of a patient with tetralogy of Fallot operated at 5 month of age who stopped propranolol treatment for hypoxic spells and unusually developed facial and subglottic IHs configuring the diagnosis of PHACES syndrome (posterior fossa brain malformations, hemangioma, arterial anomalies, cardiac defects and/or aortic coarctation, ocular anomalies and sternal defects). To our knowledge this is the first report in the international literature of a delayed appearance of an infantile hemangioma involving the skin and the airways (PHACES syndrome). The pathophysiological explanation relies on the mechanism of action of propranolol which seems to act initially with vasoconstriction, down-regulating proangiogenetic factors and inducing endothelial cell apoptosis. Many decades since their introduction β-blockers are useful in a growing group of diseases. The pleiotropic effect of β-adrenoceptors antagonists is not yet deeply understood, residing in neurohormonal regulation systems and angiogenesis and proving to be an effective treatment from cardiovascular to oncological illnesses.
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Cite this article as:
Bronzetti G., Patrizi A., Giacomini F., Savoia F., Raone B., Brighenti M., Bonvicini M., Neri I. and Gargiulo G.D., A PHACES Syndrome Unmasked by Propranolol Interruption in a Tetralogy of Fallot Patient: Case Report and Extensive Review on New Indications of Beta Blockers, Current Medicinal Chemistry 2014; 21 (27) . https://dx.doi.org/10.2174/0929867321666140304094345
DOI https://dx.doi.org/10.2174/0929867321666140304094345 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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