ABNORMALITIES OF THYROID-FUNCTION AND GLUCOSE CONTROL IN SUBJECTS WITH RETT-SYNDROME

被引:20
作者
COOKE, DW [1 ]
NAIDU, S [1 ]
PLOTNICK, L [1 ]
BERKOVITZ, GD [1 ]
机构
[1] JOHNS HOPKINS UNIV,DEPT NEUROL,BALTIMORE,MD 21287
关键词
RETT SYNDROME; THYROID FUNCTION; ORAL GLUCOSE TOLERANCE TEST;
D O I
10.1159/000184309
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
We have identified subtle abnormalities of thyroid function and glucose control in patients with Rett syndrome. The mean serum total thyroxine (T-4) concentration was significantly lower in a group of subjects with Rett syndrome (6.9 +/- 1.5 mu g/dl, n = 34; p < 0.0001) than the adult reference range (8.5 +/- 1.75 mu g/dl, n = 200). This difference remained significant even for the 17 subjects not taking anticonvulsants (7.6 +/- 1.5 mu g/dl; p < 0.05 vs. adult reference). The difference was more marked when compared to age-adjusted normals, with 10 subjects having a serum total T-4 concentration below normal for age including 3 of 17 of the subjects not taking anticonvulsants. This decrease in serum total T-4 concentration was not due to changes in binding proteins as measured by 3,5,3'-triiodothyronine resin uptake, and was associated with a decreased concentration of thyroid-stimulating hormone (1.7 +/- 1.6 mU/l, n = 23 vs. 2.5 +/- 1.0 mU/l, n = 200; p < 0.01). Oral glucose tolerance tests were performed in 10 of the subjects with Rett syndrome. They had a delay in the peak glucose and insulin concentrations. Glucose levels were elevated at 1 and 2 hours (p < 0.05), and insulin levels were elevated at 1, 2, and 3 hours (p < 0.05). Two subjects fulfilled criteria for impaired glucose tolerance.
引用
收藏
页码:273 / 278
页数:6
相关论文
共 26 条
[1]   RETT-SYNDROME - AN UPDATE AND REVIEW FOR THE PRIMARY PEDIATRICIAN [J].
BRADDOCK, SR ;
BRADDOCK, BA ;
GRAHAM, JM .
CLINICAL PEDIATRICS, 1993, 32 (10) :613-626
[2]   REDUCED CONCENTRATIONS AND INCREASED METABOLISM OF BIOGENIC-AMINES IN A SINGLE CASE OF RETT-SYNDROME - A POSTMORTEM BRAIN STUDY [J].
BRUCKE, T ;
SOFIC, E ;
KILLIAN, W ;
RETT, A ;
RIEDERER, P .
JOURNAL OF NEURAL TRANSMISSION, 1987, 68 (3-4) :315-324
[3]   A REVIEW OF THE BIOCHEMICAL PATHWAYS STUDIED AND ABNORMALITIES REPORTED IN THE RETT SYNDROME [J].
BURD, L ;
KEMP, R ;
KNULL, H ;
LOVELESS, D .
BRAIN & DEVELOPMENT, 1990, 12 (04) :444-448
[4]   EFFECTS OF ANTICONVULSANT DRUGS ON THYROID-HORMONES IN EPILEPTIC CHILDREN [J].
FICHSEL, H ;
KNOPFLE, G .
EPILEPSIA, 1978, 19 (04) :323-336
[5]   SERUM T4, TBG, T3 UPTAKE, T3, REVERSE T3, AND TSH CONCENTRATIONS IN CHILDREN 1 TO 15 YEARS OF AGE [J].
FISHER, DA ;
SACK, J ;
ODDIE, TH ;
PEKARY, AE ;
HERSHMAN, JM ;
LAM, RW ;
PARSLOW, ME .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1977, 45 (02) :191-198
[6]   RETT SYNDROME - CRITERIA FOR INCLUSION AND EXCLUSION [J].
HAGBERG, B ;
GOUTIERES, F ;
HANEFELD, F ;
RETT, A ;
WILSON, J .
BRAIN & DEVELOPMENT, 1985, 7 (03) :372-373
[7]   THYROID-FUNCTION IN EPILEPTIC PATIENTS TREATED WITH CARBAMAZEPINE [J].
ISOJARVI, JIT ;
PAKARINEN, AJ ;
MYLLYLA, VV .
ARCHIVES OF NEUROLOGY, 1989, 46 (11) :1175-1178
[8]   THYROTROPIN-RELEASING-HORMONE [J].
JACKSON, IMD .
NEW ENGLAND JOURNAL OF MEDICINE, 1982, 306 (03) :145-155
[9]   NEUROPATHOLOGY OF RETT SYNDROME [J].
JELLINGER, K ;
ARMSTRONG, D ;
ZOGHBI, HY ;
PERCY, AK .
ACTA NEUROPATHOLOGICA, 1988, 76 (02) :142-158
[10]  
KOZINETZ CA, 1993, PEDIATRICS, V91, P445