( CIP ) /. :,2004. 10 ISBN 7-5331-3800-7......... : : :. R725. 8 CIP ( 2004 ) 093314 : : 16 :250002 : ( 0531 ) 2098088 : w w w. lkj. c om. c n : s dkj @ jn- public. s d. c ninfo. n et : : 16 :250002 : ( 0531 ) 2098071 : : 753 :261041 : ( 0536 ) 2116928 : 787 m m 1092 mm 1/ 16 : 17 : 370 : 2004 10 1 1 ISBN 7-5331-3800-7 : 40.00 R 1118
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,,,,,,,, ( ) ( ),, ( ),,,,,,,,!,,
1 1 2 6 8 8 18 32-35 35 ( G HD) 43 61 67 83 83 89
99 104 109 111 113 113 114 116 119 130 137 142 145 148 150 150 160 174 184
198 D 204 204 D 207 209 220 228 230 245 245 248 252 252 260
262 Bartter 262 Liddle 265 267 267 280 288 290 305 318 321 329 330 336 345
( 1-1 ),,,,,,, 1-1 : ( ),
, ;,, ( ) 20 60,,,, - D, ( IGF ), ( EG F ), ( FGF ), ( NGF ),, ( ) 10,, DNA ;,,,,,,,,, ( hormone),, ( telecrine),, ; ( paracrine) ; ( autocrine),,,,, ( neurohormone ), ( neurocrine) ( ),,, ( 1-1 ) : 1. ( 1) ( 2)
1-1 T R H GnR H ( ) SRI H ( SS) G H R H CR H ( ) MR F ( ) MI F P RF P IF ( ) A V P ( AD H ) OX T AC T H TS H FS H L H ( ) -MS H P RL G H ( ) T 4 T 3 C CT P T H IN S : ( ) F ( ) A L D E N E : T ( ) E 2 E 3 P HCG - CCK-PZ
( ) A N P M T P G 1, 25, D 3 1, 25- ( O H ) 2 - VD 3 2. ( ), 1, 25- D, ( ),,, : 1.,,,,,, 2.,,,, 3., nmol/ L, pmol/ L,,,,,, 0.1 g, 1 gact H, 40 g, 400 4.,,,,, ;,,,,,,,,, ( permissive action ),,,,,,, cam P ( ) 1.,,
( 1) :, ( ),, :, ;, G,,, G ( prot ein tyrosine kinase, P TK),, P TK,,,, -,,,, DNA,,,,, 1, 25- D3 ( 2) ( affinity ),, ( up regulation ), ( down regulation) 2.,,, cam P, camp, camp ( PK A),, cam P, cgmp Ca 2 +,, PK A, C ( P KC ) G ( PKG ), ( C ) ( G ), ( 1) G ( guanine nucleotide-binding regulatory protion) G G,, G GTP, G GTP GDP, G,, G G ( Gs ) G ( Gi) Gs, camp ; Gi, camp, ( Rs ) ( Ri), ( Hs ) ( Hi), Gs Gi,, camp
( 2), (inositol-1,4,5, triphosphate, IP3 ) ( diacylglycerol, DG), Ca 2 + IP3 Ca 2 + Ca 2 + Ca 2 + ( calmodulin, CaM ),, DG C ( prot ein kinase C, PKC ), PKC Ca 2 + P KC PK A,, 3.,, ( ), -,, -,,, DN A, mrna,, ( ),,, ( ) ( 1-2 ) : ; / ; 1-2 ; ( ) ;, - - CR H, ACT H, ACT H, CRH AC TH,,,,,, - - :
,,,,,,, IL-1-2 ACT H PR L, T RH T SH ; T IL-2, T NK, ( superoxide anion, ), T ; ; ( GnR H ) ACT H,,,, ( binding assay ) ( RIA ) ( IRMA ) ( ELISA) ( FIA) ( CLIA), DNA B CT MRI,, ( )
, ( ), 7 :,,,, 1., 40 3 : ) ( 1) 12 ( 2) 16 ( 13 28 ( 3) 12 ( 29 40 ) 2. 28 3. 28 1 ( 0 ) 4. 1 3 ( 1 2 ) 5. 3 5 6, 6 7 (3 5 ) 6. 6 7 12 ( ), 13 ( ) ( 6 13) 7. 11 12 17 18, 13 14 18 20, ( ) 1. ( Intraut erine Growth ), (intrauterine growth retardation, IUGR ), ( Birth body weight, BBW ), < 2 500 g IU GR IU GR IU GR catchup growth, 10% 30% IUGR ( 1) ( The Placenta ), ( 2) ( Classic H ormones Of Growth & Fetal Growth ), G H,, ( placental
lactogen), IGF-1 ( 1 ), ( G H V gene ) ( Laron dwarfism ) IGF-1 IUGR IGF-1, IGF IGFBP-1, IU GR, IGFBP-1, IUGR,, IGFBP-1, IGFBP-1 ( 3) ( Ins ulin ),, ( small for gest ational age, SGA) IU GR ( 4 ) ( Epider mal Growth Factor, EGF ) EGF, EGF-Rs, IU GR IU GR EGF, EGF ( 5 ) ( Fibroblast Growth Factor, FGF ) ( FGF-R ) FGF 3 FG F- R ( 6) ( Genetic, Maternal & U terine Factors ), IUGR TORCH ( toxoplasmosis, rubella, cytomegalovirus, herpes and others), IU GR ( 7) ( Chromosomal Ab normalities & M alform ation Syndrome), T ur ner 2. ( Postnat al Growth ), GH- IGFs,, ( growth spurt) : -, ( 2-1) ( 1) ( GH- IGFs Axis) ( G H ) 17 ( q22-24 ), 5, 191-, 22 kda,, ( G H RH ) ( G HIH ),, G H ( 2) ( T hyroid Hormone ),,,, ( untreated CH ),,,,, ( US LS ), 2 3 Koc-
, ( 3) ( Sex St eroid ) IGF-1,, IG F-1 ( 4) ( Glucocorticoids ), 2-1,, G H ;, ;, ;,,,,, CA H ( 5) (INSULIN ),
IGF ( 6) ( Genetic Factor s ),,, ( Socioeconomic Factors), ( Nutritional Factors ), G H, IGF-1 ; G H, IGF-1, IGFBP-1 ( IGF-1 ) ( vegan diets) ( Psychologic Factors ),,, G H R H, ( p sychosocial dwarfism ), ( Chronic Disease), IGF-1 ( Catch-Up Growth ) : ( ) 1. ( ), ( ) ( ) 2, 2,, 1 3.1 kg 9 kg, 6 kg; 2 11.5 kg, 2.5 kg ( ) 50.1 cm; 1 74 75 cm, 24 25 cm, ; 2 87.9 cm, 13.9 cm 4 ( ) ; 5 7,,, ; 8 10, ( ), ; 11 15,,, ;, 2.,,,,, 1/ 4, 1/ 8 3., 2, 5,, ( ) 1., ;,,
, 3 4,, 7 10, 7% 8 %,, 1 3 300 200 g, 4 6 200 100 g,7 9 100 80 g, 10 12 80 50 g 600 800 g; 250 g 2, 2.5 3.0 2, 2, 4 5 kg, 2 3 2, 1, 2, 4 5 2, 1 3, 2 4, 4 5 7 1 2, 10 3 : 1 6 ( kg ) = ( kg) + 0.7 ( kg) 7 12 ( kg ) = 6 ( kg ) + 0.25( kg) 2 ( k g) = ( ) 2 + 7( 8) ( kg) 2. ( ) ( ),, 1 50 cm, 1 25 cm, 2 10 cm, 5 7.5 cm 2, 1 1.5, 4 2, 13 14 3 ( ) : 2 12 ( ) ( cm ) = ( ) 7 + 70( cm) : ( 1),,, 0.67 14 0.53 ( 2) ;,,,,, 12 ( 3), 1, 6, 12 ( 4), 12 ( 5),, 4 ; 2 5 ; 6 6 ; 8 3. 34 cm, 9 cm, 3 cm, 2 2 cm, 3 1 2 cm 2 48 cm, 5 50 cm 2,
,, 3 6 ; 2.5 cm, 12 18 ;, 2 4 ; ; ;, 370, 1/ 4; 6 700, 1/ 2; 1 2.5, 60% ; 2 3, 3/ 4; 6 90 %, 4., 1 2 cm; 1, 1,,,, 5.,,, 1 6.,,, ;, ( bone age),, ;, X, : ( 4 6 ) ; ( 1 ) ; ( 2 2.5 ) ; (3 ) ; ( 3.5 5 ) ; ( 5 6 ) ; ( 6 7 ) ; ( 9 10 ) 10, 10 1 9 : ( ) + 1, 1,, 3, 1, ; 6, 2, ; 1, 3,, 7.,, ( 20 ) ( 32 ), 4 10, 12, 2.5 2 : 4 6, 6 1, 12 2, 17 3 ( ), 8. - - ( H PG ), ( Gn ) H PG 2, H PG, H PG, GnR H Gn
, ; 2 3 ( 6 8 ), ( adrenarche), Gn, ( pubarche),, 10,, GnR H Gn, GnR H Gn,, 6 7, 3 : 10 13,,, ( ) ;, 14 16,, ;,17 20, ;,, ( 1), 10%, 1,, 3 ml, 2 cm, 5 cm 10 14,, > 2.5 cm, 4 ml,,,,, > 5 cm ; 10 11, 12 13, 14 15, 16,, ; 5 (2),, ;,,, ( ) : B1 B3 ( 3 4 mm), ( B4 7.4 mm B5 10 mm),, ( ), :, /,, 3 cm 5 cm 1 ml 2 10 ml,,, B > 2.5 ml, > 1 ml, 9 10 ( thelarche), ; 10 11 ( pubarche) ;13, B4 ( menarche) ( ) ( ) 1. ( 1) :, 10 kg, 10 g; 1 7 50 kg, 50 g; 7 100
kg, 100 g ( 2) : ;,,,, 1 3, 3,,,,,, ( 3),,, 1 1, 2. ( 1) 3 3 ( 2) 3,,,,,,,,,, cm, 1, 0.1 cm 3,,,,,,,, 60,,,,,, 0.1 cm ( 3) ( ),, ( 2 m, ), (2 m ) 0.5 cm 3. ( 1) ( 2),,,, 0.1 cm, ( 3), 0.1 cm,, 10,,,, 4. ( 1) ( 2) 3, 3,,,,,, 0.1 cm ( 3) ( 1, ) 5. ( 1),
0.6 cm 15 cm,, 15 g/ cm 2 0.5 mm ( 2) :,,, 45,,, 3 cm, ( ),, : 3, 0 1 4 1 2 2, 3 6 9 ( 2 5 8 )12 18 24 30 36 ; 3 6 1 ; 1,,, ;,,,,, 9 ( 7 ) ( 7 18 ) ;,, 2-1 1. ( 1),, ( X) ( SD) X 1SD 68%, X 2SDX 95%, X 3SDX 99.7% ( 2-1, 2 ) X - 2SD X - ( 1S 2 S) X 1SD X + (1S 2 S) X + 2SD 2-2 X - 2SD X - ( 1S 2S ) X - 1SD X + 1SD X + ( 1S 2S ) X + 2SD - + X 2SDX, X-2SD, X + 2SD ( 2),
, 3 10 25 50 90 97 P3 3, X - 2SD; P97 97, X + 2SD; P3 P97 95% ; P50 ( 3), ( ),,,, 2. ( 1) ( ) 3, ( ), ( 2) Kaup = ( )/ 2 ( m 2 ), (, BMI ),, 6 8, 1 < 12, 12 13.5, 13.5 18, 18 20, > 21 = ( )/ ( ) 100,, 64, 3 53 ( 3) ( ),,, 3. ( 1) ( SDS ), SDS, SDS = ( X - X)/ SD, SDS 2 ; 2 3 ; 3 ( 2) ( T H ) T H = + 6.5, - 6.5-2SD, TH, SDS, T H, ( GV ) 4,,, SDS, TH + 2SD, ( ), 1. (cm ) 2SD( ) 2, 95% 2.,,, 3 97, 95% 2,,, 3. ( GV )
, ( cm/ ) 25 cm, 15 cm, 5 7 cm, 8 12 cm ( height velocity chart),,,, 25 cm, 3,,,,,, ( 2-2) 2-2 ICPP 4. ( U S ) ( LS ) US: LS : 1.6, 1 1.4, 6 1.12, 12 1 5.,,,, G HD, 6. G H D 7., 3 X ( ) 2 ( - 2SD) 3, ( short stature)
,, 3 7 cm, 3 5 cm, 6 cm, 1 %, ( ),,,, 1. ( ),,,, ( 5 ) (,, ), ( ),, 2 ( ), 2 3,, 2 3, 16, 14, 30%, 90% G H,, 12 hch,,,, hch, 2., ;, 3 5,, 3,,,, GH,,, < 160 cm, < 150 cm,,,,, 3. IU GR, 10 % 30% IU GR, 1 2, 800 g( ), 3 IUGR GV,, R ussell-silver,,,, IUGR, ( ) ( ) IU GR IU GR, G H GV 4.
, : ( 1) Turner 45, XO T ur ner ( ),, T urner,, ( 2 ) Noonan ( Pseudo-T urner ) T ur ner, T urner, Noonan 46, XX, 46, XY, T urner, Turner, Nooan Pseudo- Turner, 12q24 ( 3) P rader-willi ( ), ( ),, 15 ( q11-13 ) N ( SNRPN ), 15, 15 15.1 ( 4) Laurence-Moon Biedl-Bardet Biedl-Bardet 16 ( q21), ( ) Laurence-Moon, ( 5),,,,, US LS, 21 ( ), ( 6) 100,,,,,,, ( F GFR3 4p 16.3), 132 cm, 123 cm, G H 5.,, GV,,,,,,,, 1 GV, 25,,
,,,,,, IG F-1,, IG F-1, IGF-1,, G H Crohn IGF-1, IGF-1, GV,,,,, G H G V, D( VitD), 1.25- (O H ) 2 -VitD3 ( ),,, GH GV, ;,,,, ph < 5.5,,,,,,,,, ( ) ( ) T urner 6. ( ),,,,,,,,, G H, IGF-1 7. ( ), ( ),, ( ) ( ) 1., G H 1 4 000 1 3 500, 3, 5
cm/, 114 881 5%,, 2.5 1 G H G H R H G H, G H ; G H, G H R H G H,, GH, G H P IT1 ( ) G H T S H ( 1) (-1 SD),, 1 2 G H,,, G H G H, GH, LDL, G H HDL G H G H R H ( 2 cm) GH ; ACT H,, ( ),,, G H G H, G H ( TR H ), TR H G H,,, C T MRI, 7%, G H,,, G H G H N ( 17q22-24) IA G H, GH ; G H, G H, hg H,,,,,, IG F-1 GH IB G H, GH,, X- G H, ( GRF )
, G H ( 2) G H G H,,,,, -,, G H,,, G H, G H G H, G H, 12 18 G H, ( ),, / ( US/ LS ) Wilms ( 1 10 cm, 5 7 cm ) ( ), ( 3) G H G H G H Laron ( G H IGF-1 ) G H, G H G HBP, G HBP RNA G H, IGF-1 G H, IGF- 1, IGF-1, 1/ 3, DNA IGF-1, IGF-1 G H G H, GH IGF-1 IGF-1 GH, G H, G H,,, G H IGF-1,, G H, G H, G H Pygmies G H, IGF-1 IGF-2 GH IGF-1, IGF-1, IG F-2, IGF-1 Efe,,,,, IGF-1, G H, G H, G H G H ( 4) G H G H G H,
G H G H, G H ( G H > 10 ng/ ml, 6 ng/ ml) G H GH, G H G H, G H GH, G H G H, G H 10% G H, G H, G H, G H 10 ng/ ml, G H G H 10,, G H (0.5 g/ kg, 20 g, 30 ) ( 10 mg/ kg ) ( 0.1 0.15 mg/ m 2 ),, GH,, 10 % 25%,,, ( ), 0.075 0.1 U/ kg, 20 40, 50%, G H ACT H,,,,, 50% ( G H RPs ) G H G H RPs GRF, GRF (, ) ( ),, 24 hg H ( ), GH G H G H G H, IGF-1 IGFBP-3 GH G H IGF-1,, IGF-1 G H, IGF-1, G H - GH IGF-1,, G H, IGF-1,, GH, IGF-1 G H - IGFBP-3 G H, IG F-1 G H G H IGF-1 IGFBP-3 G H
,, GH, ( 5 2.5 3.5 SD), ( 5 ), G H, hg H,, G H,,, G H, GH, 3 6 GnR H G H, G H G H, T urner, G H, ( 5) 1986, G H G H ( hg H ) 1985, Creutzfeldt-Jakob, 10 15 hg H GH,, G H G H, 191 ( ), 192 ( ), G H,,,, G H G H R H, G H G H R H G H, G HR H GH G H IGF-1 G H R H GH G H IG F-1 DNA, IGF-1, Laron,, GH ( GH ),, 0.3 mg/ ( kg w),, 6 7,, FDA, G H G H 50 G H G H, 1.4 SD, 2.9 SD,, G H FDA G H G H, GH
,, G H G H,,, G H G H G H,,,, G H,,, G H, G H,, G H GH IUGR T urner, G H T urner G H, 150 cm, 144 cm G H G H IGF-1 IGFBP-3, G HBP, G H,,,, : LDL, H DL BMI, G H G H,,,, :,,, GH,,,,,,