Sirolimus in blue rubber bleb naevus syndrome: A systematic review

被引：32

作者：

Wong, Xin Lin ^{[1
]}

Phan, Kevin ^{[1
,2
]}

Rodriguez Bandera, Ana I. ^{[3
]}

Sebaratnam, Deshan F. ^{[1
,2
,4
]}

机构：

[1] Univ New South Wales, Sydney, NSW, Australia

[2] Sydney Childrens Hosp Network, Liverpool Hosp, Dept Dermatol, Sydney, NSW, Australia

[3] Sydney Childrens Hosp Network, Dept Dermatol, Sydney, NSW, Australia

[4] Hosp Univ La Paz, Dept Dermatol, Madrid, Spain

来源：

JOURNAL OF PAEDIATRICS AND CHILD HEALTH | 2019年 / 55卷 / 02期

关键词：

blue rubber bleb naevus syndrome; mTOR; sirolimus; vascular malformation; venous malformation; RECURRENT IRON-DEFICIENCY; PATIENT; MALFORMATION; RAPAMYCIN; CHILD;

D O I：

10.1111/jpc.14345

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

The aim of this study is to review sirolimus as a treatment for blue rubber bleb naevus syndrome (BRBNS). A literature search of Medline, Embase, CINAHL, SCOPUS and Google Scholar was conducted for publications reporting treatment of patients with BRBNS with sirolimus. Of 46 articles identified, 17 studies reporting 23 patients met inclusion criteria. Sirolimus was well tolerated in all but one patient who required treatment cessation; 17/18 patients noted an improvement in gastrointestinal disease where this was reported; 21/22 patients noted an improvement in cutaneous disease where this was reported. Based on these results, sirolimus may be considered a first-line treatment of BRBNS depending on patient morbidity.

引用

页码：152 / 155

页数：4

共 31 条

[1]

Abusin G, 2015, PEDIATR BLOOD CANCER, V62, P58

[2]

Aksu AÜ, 2017, J PEDIAT HEMATOL ONC, V39, P147, DOI 10.1097/MPH.0000000000000681

[3]

Akyuz C, 2014, PEDIATR BLOOD CANCER, V61, pS338

[4] Blue rubber bleb nevus syndrome: Promising response to sirolimus [J].

Akyuz, Canan ;

Susam-Sen, Hilal ;

Aydin, Burca .

INDIAN PEDIATRICS, 2017, 54 (01) :53-54

[5]

[Anonymous], PEDIAT DERMATOL S1

[6]

[Anonymous], PEDIAT BLOOD CANC S1

[7]

[Anonymous], 2018, ANN DERMATOL VENER

[8]

[Anonymous], PEDIAT DERMATOL S1

[9]

[Anonymous], J PEDIAT GASTROEN S1

[10]

[Anonymous], PEDIAT DERMATOL S1

← 1 2 3 4 →