EFFECTS OF THE INFUSION OF INSULIN-LIKE GROWTH FACTOR-I IN A CHILD WITH GROWTH-HORMONE INSENSITIVITY SYNDROME (LARON DWARFISM)

被引:127
作者
WALKER, JL [1 ]
GINALSKAMALINOWSKA, M [1 ]
ROMER, TE [1 ]
PUCILOWSKA, JB [1 ]
UNDERWOOD, LE [1 ]
机构
[1] CHILD HLTH CTR,DIV ENDOCRINOL,WARSAW,POLAND
关键词
D O I
10.1056/NEJM199105233242107
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
THE clinical manifestations of the growth hormone insensitivity syndrome (Laron dwarfism) result from the inability of growth hormone to exert its biologic actions.1,2 Affected children have the severe growth retardation and other physical characteristics of growth hormone deficiency and low serum insulin-like growth factor I (IGF-I) concentrations.1,2 Unlike children with growth hormone deficiency, however, children with growth hormone insensitivity syndrome have high serum growth hormone concentrations, and treatment with growth hormone does not raise their IGF-I concentrations or stimulate growth.1,2 The conclusion that the syndrome is due to abnormal or deficient cellular growth hormone receptors is supported by the finding. © 1991, Massachusetts Medical Society. All rights reserved.
引用
收藏
页码:1483 / 1488
页数:6
相关论文
共 41 条
[11]   ABSENCE OF SERUM GROWTH-HORMONE BINDING-PROTEIN IN PATIENTS WITH GROWTH-HORMONE RECEPTOR DEFICIENCY (LARON DWARFISM) [J].
DAUGHADAY, WH ;
TRIVEDI, B .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1987, 84 (13) :4636-4640
[12]  
DAUGHADAY WH, 1985, WILLIAMS TXB ENDOCRI, P577
[13]   SERUM CONCENTRATIONS OF INSULIN-LIKE GROWTH FACTOR-II ARE NOT CHANGED BY SHORT-TERM FASTING AND REFEEDING [J].
DAVENPORT, ML ;
SVOBODA, ME ;
KOERBER, KL ;
VANWYK, JJ ;
CLEMMONS, DR ;
UNDERWOOD, LE .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1988, 67 (06) :1231-1236
[14]  
DERCOLE AJ, 1976, INT C SERIES, V381, P190
[15]   LARONS DWARFISM - STUDIES ON NATURE OF DEFECT [J].
ELDERS, MJ ;
GARLAND, JT ;
DAUGHADA.A ;
FISHER, DA ;
WHITNEY, JE ;
HUGHES, ER .
JOURNAL OF PEDIATRICS, 1973, 83 (02) :253-263
[16]  
ESHET R, 1984, ISRAEL J MED SCI, V20, P8
[17]   TISSUES OF THE LARON DWARF ARE SENSITIVE TO INSULIN-LIKE GROWTH FACTOR-I BUT NOT TO GROWTH-HORMONE [J].
GEFFNER, ME ;
GOLDE, DW ;
LIPPE, BM ;
KAPLAN, SA ;
BERSCH, N ;
LI, CH .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1987, 64 (05) :1042-1046
[18]   CHARACTERIZATION OF THE HUMAN GROWTH-HORMONE RECEPTOR GENE AND DEMONSTRATION OF A PARTIAL GENE DELETION IN 2 PATIENTS WITH LARON-TYPE DWARFISM [J].
GODOWSKI, PJ ;
LEUNG, DW ;
MEACHAM, LR ;
GALGANI, JP ;
HELLMISS, R ;
KERET, R ;
ROTWEIN, PS ;
PARKS, JS ;
LARON, Z ;
WOOD, WI .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1989, 86 (20) :8083-8087
[19]   SHORT-TERM METABOLIC EFFECTS OF RECOMBINANT HUMAN INSULIN-LIKE GROWTH FACTOR-I IN HEALTHY-ADULTS [J].
GULER, HP ;
ZAPF, J ;
FROESCH, ER .
NEW ENGLAND JOURNAL OF MEDICINE, 1987, 317 (03) :137-140
[20]   RECOMBINANT HUMAN INSULIN-LIKE GROWTH FACTOR-I STIMULATES GROWTH AND HAS DISTINCT EFFECTS ON ORGAN SIZE IN HYPOPHYSECTOMIZED RATS [J].
GULER, HP ;
ZAPF, J ;
SCHEIWILLER, E ;
FROESCH, ER .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1988, 85 (13) :4889-4893