Vascular Perfusion Abnormalities in Infants with Spinal Muscular Atrophy

被引:94
作者
de Queiroz Campos Araujo, Alexandra Prufer [1 ]
Araujo, Mario [2 ]
Swoboda, Kathryn J. [3 ]
机构
[1] Univ Fed Rio de Janeiro, BR-21941912 Rio De Janeiro, Brazil
[2] Univ Estado Rio De Janeiro, Rio De Janeiro, Brazil
[3] Univ Utah, Sch Med, Pediat Motor Disorders Res Program, Salt Lake City, UT USA
关键词
AMYOTROPHIC-LATERAL-SCLEROSIS; SYMPATHETIC SKIN-RESPONSE; DISEASE; TYPE-1; MUSCLE;
D O I
10.1016/j.jpeds.2009.01.071
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type 1, also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report 2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all play a role in the development of this complication. (J Pediatr 2009,155:292-4)
引用
收藏
页码:292 / 294
页数:3
相关论文
共 13 条
[1]   Finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation in patients with progressive in spinal muscular atrophy [J].
Arai, H ;
Tanabe, Y ;
Hachiya, Y ;
Otsuka, E ;
Kumada, S ;
Furushima, W ;
Kohyama, J ;
Yamashita, S ;
Takanashi, JI ;
Kohno, Y .
JOURNAL OF CHILD NEUROLOGY, 2005, 20 (11) :871-875
[2]   Medical considerations of long-term survival of Werdnig-Hoffmann disease [J].
Bach, John R. .
AMERICAN JOURNAL OF PHYSICAL MEDICINE & REHABILITATION, 2007, 86 (05) :349-355
[3]   SYMPATHETIC SKIN-RESPONSE ABNORMALITIES IN AMYOTROPHIC-LATERAL-SCLEROSIS [J].
DETTMERS, C ;
FATEPOUR, D ;
FAUST, H ;
JERUSALEM, F .
MUSCLE & NERVE, 1993, 16 (09) :930-934
[4]  
Kamiya A, 2000, AM J PHYSIOL-REG I, V278, pR445
[5]   VEGF at the neurovascular interface: Therapeutic implications for motor neuron disease [J].
Lambrechts, Diether ;
Carmeliet, Peter .
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 2006, 1762 (11-12) :1109-1121
[6]   Expanding the phenotypes of the Pro56Ser VAPB mutation:: Proximal SMA with dysautonomia [J].
Marques, Vanessa D. ;
Barreira, Amilton A. ;
Davis, Mary B. ;
Abou-Sleiman, Patrick M. ;
Silva, Wilson A., Jr. ;
Zago, Marco A. ;
Sobreira, Claudia ;
Fazan, Valeria ;
Marques, Wilson, Jr. .
MUSCLE & NERVE, 2006, 34 (06) :731-739
[7]   COMPARISON OF STRUCTURE OF SMALL BLOOD-VESSELS IN NORMAL, DENERVATED AND DYSTROPHIC HUMAN MUSCLE [J].
MUSCH, BC ;
PAPAPETROPOULOS, TA ;
MCQUEEN, DA ;
HUDGSON, P ;
WEIGHTMAN, D .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1975, 26 (02) :221-234
[8]   Sustained Improvement of Spinal Muscular Atrophy Mice Treated with Trichostatin A Plus Nutrition [J].
Narver, Heather L. ;
Kong, Lingling ;
Burnett, Barrington G. ;
Choe, Dong W. ;
Bosch-Mare, Marta ;
Taye, Addis A. ;
Eckhaus, Michael A. ;
Sumner, Charlotte J. .
ANNALS OF NEUROLOGY, 2008, 64 (04) :465-470
[9]   The changing natural history of spinal muscular atrophy type 1 [J].
Oskoui, M. ;
Levy, G. ;
Garland, C. J. ;
Gray, J. M. ;
O'Hagen, J. ;
De Vivo, D. C. ;
Kaufmann, P. .
NEUROLOGY, 2007, 69 (20) :1931-1936
[10]   DECREASED HEART-RATE-VARIABILITY IN AMYOTROPHIC-LATERAL-SCLEROSIS [J].
PISANO, F ;
MISCIO, G ;
MAZZUERO, G ;
LANFRANCHI, P ;
COLOMBO, R ;
PINELLI, P .
MUSCLE & NERVE, 1995, 18 (11) :1225-1231