Importance of neurological assessment before bone marrow transplantation for osteopetrosis

被引:24
作者
Abinun, M [1 ]
Newson, T [1 ]
Rowe, PW [1 ]
Flood, TJ [1 ]
Cant, AJ [1 ]
机构
[1] Newcastle Gen Hosp, Childens Bone Marrow Transplantat Unit, Dept Pediat, Newcastle Upon Tyne Hosp NHS Trust, Newcastle Upon Tyne NE4 6BE, Tyne & Wear, England
关键词
osteopetrosis; retinal degeneration; generalised neurodegeneration; bone marrow transplantation;
D O I
10.1136/adc.80.3.273
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Neurological complications of malignant infantile osteopetrosis are well recognised; successful bone marrow transplantation, when performed early in life, can prevent or halt some of them. In a subgroup of infants osteopetrosis is associated with primary retinal degeneration and/or generalised neurodegeneration. Bone marrow transplantation, in spite of being successful in correcting the osseous and haematological abnormalities, does not influence the progressive course of the neurodegenerative disorder. Thus, the recognition of this subgroup of infants with a very poor prognosis is essential before deciding on bone marrow transplantation.
引用
收藏
页码:273 / 274
页数:2
相关论文
共 7 条
[1]  
ALROY J, 1994, ACTA NEUROPATHOL, V87, P594
[2]   CRANIAL IMAGING IN AUTOSOMAL RECESSIVE OSTEOPETROSIS .2. SKULL BASE AND BRAIN [J].
ELSTER, AD ;
THEROS, EG ;
KEY, LL ;
CHEN, MYM .
RADIOLOGY, 1992, 183 (01) :137-144
[3]  
GERRITSEN EJA, 1994, PEDIATRICS, V93, P247
[4]   BONE-MARROW TRANSPLANTATION FOR AUTOSOMAL RECESSIVE OSTEOPETROSIS - A REPORT FROM THE WORKING PARTY ON INBORN-ERRORS OF THE EUROPEAN BONE-MARROW TRANSPLANTATION GROUP [J].
GERRITSEN, EJA ;
VOSSEN, JM ;
FASTH, A ;
FRIEDRICH, W ;
MORGAN, G ;
PADMOS, A ;
VELLODI, A ;
PORRAS, O ;
OMEARA, A ;
PORTA, F ;
BORDIGONI, P ;
CANT, A ;
HERMANS, J ;
GRISCELLI, C ;
FISCHER, A .
JOURNAL OF PEDIATRICS, 1994, 125 (06) :896-902
[5]   ASSOCIATION OF INFANTILE NEUROAXONAL DYSTROPHY AND OSTEOPETROSIS - A RARE AUTOSOMAL RECESSIVE DISORDER [J].
REES, H ;
ANG, LC ;
CASEY, R ;
GEORGE, DH .
PEDIATRIC NEUROSURGERY, 1995, 22 (06) :321-327
[6]   BONE-MARROW TRANSPLANTATION FOR INFANTILE MALIGNANT OSTEOPETROSIS [J].
SOLH, H ;
DACUNHA, AM ;
GIRI, N ;
PADMOS, A ;
SPENCE, D ;
CLINK, H ;
ERNST, P ;
SAKATI, N .
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, 1995, 17 (04) :350-355
[7]   Early VEP and ERG evidence of visual dysfunction in autosomal recessive osteopetrosis [J].
Thompson, DA ;
Kriss, A ;
Taylor, D ;
Russell-Eggitt, I ;
Hodgkins, P ;
Morgan, G ;
Vellodi, A ;
Gerritsen, EJA .
NEUROPEDIATRICS, 1998, 29 (03) :137-144