29
WIlE JD::ll'S:EN1S WTIH ~ lHA'i CF rn::wJH /IN) R.ErnlY.
'D1ElWIDl'ICl\L fll1:Cl' CF 'Il:SlCSll'RN':. B2rgili C. ;B2rgili I .
Qntro cE !rM£t:igriae; ~ Y Div:isifu cE
Elrlx:rimlt:xJf, fhpi tal cE Nii'cs R. Qlt.i.&rez , IiHl::S Aires,
l\rg'ntim.
Q::nstillltimal cblay of. groNth an:! p.terty (OXI') is ctancteri.za:l bt sroll stature
an:! cEIay of. lx:re i!£lf' (BI\) an:! se>o.al. a...eJ.qrrent. In 0I:<h:- In a:x:clerate groNth
\elo::il¥ (GI ) an:! se>o.al. cB.elqnmt. 23 I:DjS with
w-o ..ere w:J:l':ia:l fir tlcir
[XXX" groNth an:! p..W::ertal ~ """te treated with nrnthly int::rmuscuJar
testcstenre inj3:ti<rs (41+7.7 lIl)Ihi ) drirg 18 nmtrs (T) . 'Ihirteen Ints with
OXI' w-o v.ere rot. treated served as cmtrol gro.:p (C). fufcxe trmtnmt ttere v.ere
ro diffenn::es tenan gn::q:s in duonk:gical i!£lf' (CA) T:14.6StO.9 vs C:14.31IQ.8
(rrt£), s:s of. tei.<jlt (s:s) T:-3.:ot<J.81 vs C:-2.8SlQ. 62 (rrt£) , s:s of. teiglt foe
IlI\ (~) T:-Q.24tQ.9 vs -Q. Q4-l{).4 (rrt£) , ffi T:11. 81+1. 1 vs 11.59+1. 1 (rrt£),
p.Jbi.c tcir ('Iarrer)(P) T:1.8Ml.7 vs, 1.88t<J.6 (rrt£ ) , testicular \Olure ('IV)
1':7.Ot2.6 vs 6.8+2.1 (rrt£) n:r GI T:4.01+1. 3 vs 4. 9+1. 4 (rrt£) . Patients \..ere
cliniJ:ally e.clwte ~tely e.ert 3.5 nrntns , In resp::r6E! to trmtnmt "'"
dXairai tie foll<:wiIl] dJlO1.cqiaU mta:
=
01 (~)
~
'IV (ml)
visit
T
C
T
C
T
C
4. Ot1.3
4.9+1.4
-Q.24tQ.4 -Q.Q4-l{). 4 7.Ot2.6 6.8+2.1
'P
10.0+1.8
4.2t<l.9
7. 2+2.5 8. 8>2. 8
3°
9.6+1.7
7.1t1.3
-Q.28+0.6 -Q.22+O.3 8.5>3. 2 1O.3t3.2
4°
9.5>1.5
8.3+2.4
9.2t3. 4 12.3+3.3
5°
8.3+1.7
7. 9t2. 4
-Q.23+0.6 -Q.10t0.4 11.5<4. 2 14. 5>2.9
6°
7. 0+1.5
8. 0+1.3
-Q.29t<J.7 -Q.osiil .6 14.1:B.5 14.'€3.3
IrrNa
p; 0. 0001rrt£
p;O.OOl
After 18 m of. trmtnmt there \..ere ro differm:::es in pre:licta:I cdl1t teiglt bt
Iby:t.ey-Pirnxlu (EHl)T:170.5!4.7 C:172.Q±4.7. In!'UlIfEll)' a:lninistmtial of. kw d::ae
te>tn3t.errre a:uld te an <rlq.Hte1:lazlpy in Ints with OXI' In p:ai>::e a
si.gri.ficant a:x:cleratim of. grtwth, irlIIarn! viri.l.i.zatial witlnrt: tie i.rd.>::ti<n of.
e><:essi\€ lx:re rratul:atial n:r cEteriCJ:at.in:.l p:a:lictH:l final reicjlt.
P
30
32
(ZIDricu; p.iarcte (pp) •
In <:J:cEr tD assess tie ~ of. this cEf.ici.a-cy, 26 girls with PP »ere
stufud, duonk:gical i!£lf' (<A) <Xffil) 6.47±2.04 a . lcicjlt, I:rre i!£lf' (BA) ani
se>o.al. a...eJ.qrrent (1lIrIleIc ) ....,." e.clwtHl an:! in ei<jlt cases pelvic an:! a:lrereJ.
ul= r d exanimtim -.ere P"l'famrl. ffi IClH stinulatial test w>s <he in all
p'tients usirg 2SJI given as an TIl !:ol.lE Il"ffiSl.lrirq tasal an:! rest IClH (30 an:! 60
min. ) lUi flIDJ'St.errre (1'UIP) am a:rtis::U Ievels ,'1112 results \..ere aJ1"P'Iffi1 with
tte n::nIJ3t"1IIl s tarduds foe serun l'UIP (N3.v an:! col • .D'M 57:320, 1983) an:! with a
cmtrol gro.:p of. ro:nal. girls (3 prq:utertal an:! 5 p..W::ertal) . B3sal SIffi, Tan:!
SIH7\ lewls \..ere neasured in 10, 24 an:! 12 p'tiEnts ~\€ly. All pataents Ia:I
MI, VPII-Ill . 'Ile reicjlt s:s ;as 0.53j-1.18, tie ratio 1lIVII:;as 0.85:!1.08. In ei.<jlt
p'tiEnts were ul=rd stuY ;as P"l'famrl, tie results """te rcmal , 'Ite lUll'
I:aseline serun lewls \..ere (X§J) 0.65i<J.66 rqoInl an:! tie a:rtis::U lewls 12:!9
uy'loo ml. an:! tie hi.<jEst resp::rre to 1ClH ;as 2. 42t1. 31 an:! 29. 2±7.1 ~\€ly .
N:re of. tte s tulial p3tients stn.o:I 1Xli' lewls tD IClH hig-er than tree:. of.
ro:nal. p:pJlatial of. tie p.t1liIDrl rIJn::qIall, rcc of. rur ro:nal. cmtrols
(p<q>.t:ertal. rqoInl B:O.~ . 31 , rraxiJTal resp::r6E! 2.8IhO.53;p..W::ertal B: 1. ~..:73.
rraxiJTal resp::r6E! 6.48+,].26. '!he blsal serun lewls of. SIffi, 'J' an:! ID£l\ \..ere: X-tID
(mol/L) 94.5O±31. 04, 0.9fI±{l.73 an:! 2300:t2320. OIly tie SHA serun levels "ere
signifi cantly hi<j'er than in ttcse [X"IIia.s l y p..1bliSID cmtrols UQ3±8, 1. 06iQ. 08
an:! 348:!:117) p LO.Ol (EJeleprrsky an:! cnl.• .D'M 67:234, 1988).
In =clusim"'" stufud gro.p of 26 girls witll pre::x:cia1s p..tarcte n::re of. thIn
p:esentHl any l:>ia:haniali evidn::e fir n:n classiml CAH d.e In 2lOIIasa
cEfi.ci.m:y . 'Ite in:rmsa:l ID£l\ lewls cx:nfum a [X'ffiBture rratul:atial of. a:lrereJ.
<rtivil¥ in girls with (ZIDricu; p.t:a.rde.
lUlUJlJllS
QN:FJ\LIZID RfSlSIm:E '10 'UMUlD HJM:l£ IN A (}I]J.ffiEN:lEE CF
IRMXRlPl'll'E (1£) . Chiesa A.;Gnfui= L.
Div:isifu
cE
Eirl::crimloJfa,CElIE,H:Epital
cE
Nii'cs
is a Syrrlrore ctancteri.za:l bt
ele..atHl serun 1'1 an:! T3 lewls, ~tial of. a oml resp::r6E! In 'll1H an:! tte
a!:sEn:e of. nenifestatims of. thyroid h::J:nrre e><:ess . In this <D111U1icatial "'"
rep:rt tie st:ufus in a 6.3 ~ old !:of, with reicjlt an:! Iocicjlt in !.'m
pmBltiJe, b::re i!£lf' 6 ~ , with a
large
g::ri.ter of. 50 gr . '!he p'tiEnt Ia:I
ro:ei-.e::l 1u;pl fir 5 nmtm an:! stqprl it a nmth tefcre. serun 'N 1eI.el ;as 20
rq/dl, T3 339 rq/dl, fIre 'N 5.6 rq/dl, ~ti\€ ant.imi.c:ronIB antih:rlies, 'I9J 1. 0
illAnl. 'I9J rest 'll1H U .5 illAnl, p:olaotire 10.4 rqoInl, p::6t 'll1H 43.1 ~l
tfStn3tercre bin:I.in:l gld:ulin (S1ffi) 68 .5 moI/l, ro:nal. sella tl.II:cic:n 10<. I
Lptake of. 8, 32, 38% at 1. 24 an:! 48 tnJIs ~\€ly. MsatiJe r=tumll 'I9J
oocretim with a ITB'lll r=tumll_'I9J surga (% ir=ffise in 'I9J fran ralir) ;as 105%
(95%cmfuEn:e limits 50-300%) X <by 'I9J 1.8 X nicjlt 'I9J 3.7. 'lhyraid glarrl cliJ:h't
d'al'lJ" drirg 10 nm tm witlnrt: 1:lazlpy. '!he a:lninistmtial of. T3 (80 cqI<by) foe 1
~ p:oinrl a rise in serun T3 In 600 rq/dl , SIffi <fuh ' t d'al'lJ", an:! om! resp::rre
In 'll1H c:k:re3se cnly a 10%. Ebj1t tnJIs after IE 1. 2 ng a:ally blsal 'I9J dnease:l
fran 4. 1 In 0.5 illAnl, ro siglificBnt d'al'lJ" in 'N an:! T3 lewls \..ere d:rerva:I.
'l%yirg tD c:k:re3se g::ri.t:er size tie p'tiEnt ;as given Bro!=iptine 2.5-5 rrg/<by fer
4 nmtm . '!he thyroid glarrl dnease:l In 25 g . , tj;> s ignificant d'al'lJ"s \..ere d:rerva:I
in 'N, T3. fIre 'N, SIffi re:p:mc In 'll1H an:! ......, Lptake. 'Jte Pel resp::r6E!_ tD 'll1H
~ 84%. '!he p.>lsatiJe om l r=tumll ~ <k:rwsfrl X <by 'I9J 36%, X nicjlt
'I9J 38%. '!he p'timt rraintairo:i tie cliniml w1:hyroidisn with a groNth mte of. 7. 3
ury'yr . 'Ih.s 1£ in this p'timt ;as weful in c:l3:::I:ffisin g::ri.ter size Wrile
rraintainirg clinical a.rt:hyroi.disn.
<htmi.c lEe of. this ctpmine<gic <q:nist (1£) nay te weful in certain p'tients
with ~ resistan::>! tD thyroid h::J:nrre.
ffiillICIED FIN\L IEIGll' IN BJlS wrrn =1'lCN'L !nAY CF rn::wJH
/IN) IUl!:RlY {OXI'} . ~ A. ; IErgd'i I. ;/oBrtircI: A. ;le in rich
J .J. ani lErgd'i C.
CElIE.Div:isifu
cE
Elrlx:rimlt:xJf.lI::Epi.tal
cE
Nii'cs R.
Qlti&rez.1iHl::S Aires. l\rg'ntiJa ·
an' is ctancteri.za:l . bt Sttt stature with delaye:l b::re i!£lf' (ffi) , ro..e.er mra
<.O'£Erllirg ac=t:aCj of. p:a:lictH:l fiml rei.<jlt (EHl) in th is p'ttern of. grtwth is
cmtro.e:sial. fer this rees:n "'" Im.e stu:furl a gro.p of. ~ I:DjS with
OXI' 1rF48) (AI with chItn:ilo:.Jial i!£lf' (CA) U.56+2. 1 ~ , s.:s of. reicjlt - 2.67.:!9.64, SA8.92fl.4 ~ an:! a:np3I"t'rl tlcir au><:>iO:p.cal pmsreters with tree:.
at tlcir cmet of p.terty. A an:nj gro.p of. I:DjS with .fanilial Sttt stature (ESS )
(IF16) (U);as use:l fa<:cmp"Iris::n .'IIa:e \..ere rct; diffe!:al::e in'lllr¢ lciglt tenan
gn::q:s. In gro.:p A. 37 to,os starta;I pj:e:ty. 17 at a rnnal <A (All wli.le 20 too
001¥rl P-Jtert¥(A2 ) with its cmet after 13.5 ~ of. a;p.1n gro.p B, 7 I:DjS
starta;I p.terty at a ro:nal. <A (81) Wrile 5 Ia:I cEJa:ro:l p.terty (82 ) . ffi l ;as
assessai bt 1Hyl.ey--Pirn%u in b:Xh gn::q:s d.Irirg prep.b:rty am at tlcir cmet of.
p.Jtei:"l¥,in 16 p3ti£nts "'" dXairai final teiglt.
11'11
I'rep.batal
croet of p.ltert:y
Final Ici<jlt
At
16O.16t6 .3(n=171
160.7 (rF6 )
167. 37.:!.6.0 (rF48)
~.001
PJ.
167. 80+6.9 (n=2O)
166.7 (n=7)
nl
162.86+5.5 (n=7)
162.1 (0=2)
16l.~ .6 (n=16)
~.83
[Q
163. 50t5 . 1 (r>-~)
158.8 (n=2)
ftrty fi\€ pmBlt of ~ Ints with ari'startB:I p.terty at a ro:nal. CA
=i.atHl to a nrlctim of EHl. 'lffise cbta ..ere cmfirnu:l with ttcse p3tiffits w-o
attainal tlei.r aiJlt final rei.cjlt. In cmtmst Ints with
with ~ p.Jtei:"l¥
(55%) Ia:I a similar EHl a:np3I"t'rl tD tlcir pt<di.cti<n, cbta a::nfurra:l with tree:.
wo . a ttairo:i final reicjlt. In !'UlIfEll)' EHl in p'tients with OXI' is rot. a:x:urate
fir p:ep..tErtal 1:DjS, ro..e.er is a ep:;xl lreth:d at tlcir cmet of. p.ltert:y .
=
33
"R.
Qlt.i.&rez" .1iHl::S Aires.l\rg'ntina.
Q:reralize:l resista"re In thyroid h::J:nrre (<RIH)
31
CF Nl'l ClA$lCl\L 2J(In.A5'\ W'ICJ.floCi IN GIRlS wrnl
ffiEIICl!l.5 RJlIIlQE . Gt)rq3rta1 M.;El3o::tar M.E.; Iel.g::n:Eky
A.; IErgd'i C.
Div:isifu
cE
Elrlx:rimlt:xJf.fl:l;pi.t al
cE
Nii'cs
R.
Qlti&rez.l.al:ncab:rio <b In\€sti'Jrifu. fl:l;pi.t al cE Miatria rr. J.P. GnIalan.
Rro3lt rep:rts Im.e d:::6criJ:a:I rrn classiml 2lOIIasa cEfic:i.<n:y in girls with
I'ffiEN:E
~
lloM.NMllIIJ(l"
CF M01FJ(l'1C KlJlI'SSKlI IN HI
MIa CF lUIOJloMN: DIJIIlE:IE5. Arata
M.;Q..rintao>s
C.;Basate JC.
Di.atetes
~tal,Qntro cE
In\€sti~
~
II::Epi.tal cE Nii'cs "R. Qlt.i.&rez" an:! I:E[:artarentD cE Ra:lla1ofta,ClmisiCn
lOCicrel. cE Ererg1a Altrni.ca. IiHl::S Aires, Atgntina.
'De aim of. this wxk ;as tD stl.dj autolop.s imnn:m:d.l1atial (vaa::imtial
na::l-anisls) 01 autoimnre pn::re'itic ~ . ~ fran nultipl.e-l.cwd::ae s u tplI:11ct=in diatetic mi.re ....,." ttansferro:l to ro:nal. ~c mice.
Pa:::ipia1t aninals d?.elqBi al:n:t:nal glu::x:se tolecan::e an:! di.minislal lst. an:! 2rrl.
Iiuses of. irailin socretim. \oh31 ~ froll diatetic d:nxs ""'" i=tnm:i
with MilIJ¥Oin C !ricr In ttansfer, cells rmairaj viable Wt. trey !cale tlcir
pn::re'itic ~ abi.lil¥. ~ fran diatetic d:nxs """'" i=tntHl with
MilrJ¥::in C an:! tlBl in:P::tH:l intD ~ ro:nal. mi.re. 15 d>ys after, mice »ere
in :P::tH:l with an B:jU'l1 d::ae of. ~ fran diatetic d:rors. 15 d>ys after tie
last injectim, nripi.alts sh:J...ej glun;e tn1.eJ:acl:e an:! irailin secretim [ZOfiles
similar In cmtrol gn::q:s in !.'m of. tie in:P::tH:l anirrals. '!his pttXa::ti\€ effect
;as opcifically ird.<Hl bt ~ fran diatetic mi.re irntntHl with
Mill:llliq.n C !ricr tD uansfer (cmtrols:1065tI7 loS . diatetics:l078+2Oill insulin/4
min/loo ng w. t ., rF6 ) . '!his effect ;as -rot. d:rerva:I in at:t¥Uic recipi.alts,
9.lJJ'St.in:.l that an irmrue resp::r6E! sto.Jld te llO.l1tHl in m::ipi.Ent aninals. 'Itese
results show that Mitrn!lcin c-iro.intHl ~ fran diatetic d:rors
spri.fically ird.<Hl a ptiB::ti\€ effect <qUnst :imnre ~ an:! IefreSa1tHl
an eq:erirrEntal ncdll tD stl.dj aut:l:llcgE imnn:m:d.l1atial ll"fffi:lnisn in autI:>imnre
diatetes.
(~DIE) ,
m= CF
_laS
A
~
mrn
IRJIEIN
DIEr
CN
=
==
IN · HI
MIlL CF llL'I£IES INllEl IJ{ SlUNXYJ];S '!RI'Iaffi.
L.;1.cnI:lmb Y.;Basate J .C. Di.atetes ~tal,cmtro cE
!rM£ti~ EW:=in::U"gi.cas
(CElIE) ,1I::Epi.tal cE Nifi:s ''R.
Qlt.i.&rez", IiHl::S Aires, y ra:ulta:l cE ~, lhi.-..ersi.drl eEl Lita:aJ., SvIta
Fe, l\rg'ntim.
In p:eviaJs Wld<s "'" ~ that tie a:lninistmtial of a hi.cjl-pI:t:Xei diet (HID)
tD rats an:! mi.re, atlfnBtHl tie cEJetericus effect of. sutplI:11ct=in (So?) m
irailin ~ (IS ) . en tie <Xter l>rd ttansfer of. ~ (5) fran diaI:etic
tD ra:llnl mi.re, <Bl.5Erl in tte last <res, glw::re-intnlerarce an:! a significant
climiruticn in g1w::re-stinuJatHl IS. In tte p:esent wxk "'" stJ.di.aj if tie
a:lninistmtial of. HID tD mi.re that Ia:I recei.-.e::l diaI:etic 5, m:rli.fy tie d:rerva:I
alt>=tims in IS. C57Bl.16J mi.re v.ere in:P::tH:l with 5 d::aes of. So? (40 11l]/l':!/<by) .
15 d>ys later tlcir spl£ffS ""'" di.sse::ta:lan:! 5 i.s:UatHl. 'Itese "diatetic" 5 """te
in :P::tH:l Lp. in n=ptrr mice k£pt 01 a cmtrol di et (CD) a<: HID. 15 mys after 5
t:rarsfer, P"="'tic glan:I fran reoeptrr mi.re -.ere peri.fu;aj an:! IS p't:t:ems .ere
e.al....t£rl.P€sul ts sha.o:l that mice .tra1sfernrl with "diatetic" 5, k£pt eilter 01 CD
a<: HIIl~ta:l di.minislal lst. an:! 2rrl . Iiuses of. IS (CD: lst. fhase : 1288. 7.!4O.4,
rF6 "" 993.4j:l6.9 ill/6 min/loo ng wt.,r>--5 , p "-O.ool ; 4rl ~ :U76l..Qt201.9, rF6 ""
lJJ7l.2±220.5 ill!32 min/loo ng wt, n=5, p ,,-O.Ol; HIll : lst. fhase: l322.6:!31.2,n=5 , ""
ll34 .0J;15.4,lF9, p£ll.Oi; 2rrl fhase : U 867:!:295.6 "" l Q628±224.6, r>-5 an:! 9, !""0.0l) .
IJ::l..e.ec in mi.re fal Ill) an:! rn.-sfernrl with "diatetic" 5, lst.fhase of. IS ;as
significantly hitjer tren \allES fum mice t.n:.Ier CD (P "<' 0.(01 ) . 'D""", results
S>.I}}'!St that IIID iJqxo.e:l tie B-<:cll sa::retx:xy resp::r6E! in an eq:erirrEntal no:Elaf
diatetEs with anti tela cell imnre ~ .
34
254