CASE REPORT Gunm Dnd by A Smun nd Vnu Smpn (ASVS) Yukyh Okuh 1, Tk Nmm 1, Hm Iwhh 1, Tkh Kzu 1, I Hyh 1, Kh Ok 1, Kzuy Ym 1,SUn 1, Fum Kub 1, Munhd Muh 1, Kn K 1, Kuyuk Az 2, Tnk Km 3, K Ou 3, Shj Nkm, Yuh Tmk 5, Thu Funhh 1, Jun-h Myw 6 nd Ih Shmmu 1 Ab T dnfy h n f pn ndn um, mun nd vnu mpn (ASVS) knwn b ufu f nunm nd nm, bu ufun f unm h n bn vfd d. H w p f unm h w dnd by ASVS wh um dn, n whh n ppxmy 6-fd n f un w bvd n h pn y y. MEN1 n ny vfd h pn f mun nd h unm w nfmd f pn. In nun, ASVS ud b ufu f h dn f unm. Ky wd: mup ndn np yp 1, mun nd vnu mpn, unm () () Indun Mup ndn np yp 1 (MEN1) mup ndn np mpx n whh hypphydm ud by phyd hypp d wh pn ndn um nd puy dnm. Th uv n MEN1, whh xhb n um dmnn pn f nhn. Pn ndn um fund n ppxmy 65-75% f MEN1 pn (1). Rdn pn ndn um, pn pyppd (PP)- n um h m mmn pn ndn um n MEN1 pn, un n 8% m. Thy uuy mpnd by nm, wh nunm, unm, nd VIPm fund n ppxmy 2%, 3%, nd 1%, pvy (2). Amn h pn ndn um h u n MEN1 pn, unm m fn mnn nd hu qu y dn nd y mn. Hwv, n h um n wy y. Uund, CT, nd MRI h mmny ud dn mn mhd f pn ndn um, wh nphy ndd h m ffv mhd (huh m nvv) f dn uh um n h n fquny hypvu (3). A mun nd vnu mpn (ASVS) knwn b ufu f zn nunm nd nm, wh hh dn nvy f nunm bn pd (-8), bu ufun f dn unm h n bn dmnd. H, w p f MEN1 n whh unm w d by ASVS wh um dn. C Rp Th pn w 66-y-d wmn wh n fmy hy f ndn um. Sh nd wn n h h n v n h f 38 y, bu f und. A h f 52, hypm, hypphphm, Dpmn f Mb Mdn, Gdu Sh f Mdn, Ok Unvy, Su, Dpmn f Phy, Gdu Sh f Mdn, Ok Unvy, Su, Dpmn f Rdy, Gdu Sh f Mdn, Ok Unvy, Su, Dpmn f Suy, Ok Nn Hp, Ok, Dpmn f B nd Endn Suy, Gdu Sh f Mdn, Ok Unvy, Su nd Dpmn f Inn Mdn, Dvn f Db nd Mbm, Hy C f Mdn, Nhnmy Rvd f pubn Spmb 8, 28; Apd f pubn Mh 13, 29 Cpndn D. Yukyh Okuh, kuh@md2.md.k-u..jp 125
Tb1. LbyTnAdmn RBC Hb WBC P FPG HbA1 IRI CPR T-h TG HDL-C LDL-C TP Ab T-B AST ALT γ-gtp ALP LDH 31 1 /µl 13.8 /dl 656 /µl 18.7 1 /µl 17 m/dl 6.6 % 2 µu/ml 5.5 n/ml 22 m/dl 191 m/dl 8 m/dl 163.8 m/dl 6.6 /dl 3.9 /dl. m/dl 13 U/L 9 U/L 32 U/L 118 U/L 28 U/L C BUN UA N K C C P CRP Uny ph Pn Su Ou An C Ab CPR 1.2 m/dl 26 m/dl 5.7 m/dl 1 meq/l.1 meq/l 112 meq/l 5.2 meq/l 2. meq/l <.2 m/dl 7. (+/-) (-) (-) (-) 29.9 ml/mn 29.6 m/dy 137.7 µ/dy nd hh pm PTH v w dd by n, nd h pn vd u hp h fwn y. Hypphydm w dd nd ub phydmy (vn 1/2 f h upp f nd) w pfmd. A h m, MEN1 w upd. Endny nvn nd mn ud w pfmd, bu n ddn bnmy w fund, xp f PTH v. Af uy, h C nd PTH v dd, nd h pn w dhd. Fm h f 5 y, h pn bn xpn bkh nd bn pn, nd h C nd PTH v bm hy vd. A h f 58, h 75 OGTT w pfmd whn h pn w dmd f nvn f h bk pn, nd dmnd db pn. H bn pn nnud wn, nd h pn w fwd up by n. Whn h pn vd u hp h f 66 y, uund vd 1 8 mm w h n n h d p f h f b f h hyd nd, nd hh-nny w n n T2-whd MR m. Bd vd h fwn bnm fndn: C w 5.2 meq/l, P w 2.6 meq/l, nd PTH w 99. p/ml. Th pn w dmd u hp n h m y f nvn nd mn. Sh w 153 m nd whd 52.8 k. Bd pu w 118/6 mmh. Phy xmnn vd ddn f vby nn f nf mb nd numbn f upp nd w mb. Sh hd hh C nd PTH v, w P v, nd mpd n funn (Tb 1). 99 mt- MIBI nphy vd n f nd upk n h phyd bhnd h f b f h hyd nd. Sn h um v f C nd PTH w pny vd nd n dyfunn w bvd, w ndd d ndn f uy. Endn ud (Tb 2) vd vn f pn ndn hmn nd bdmn mn hwd ndu (5 mm nd 25 mm n dm) n h f h pn, un h pn f pn um (F. 1). Tmn f h hypphydm w vn py, nd h pn w nfd h Dpmn f Endn Suy f mv f h mnn phyd nd. Ph xmnn f h d nd vd hypp, bu n bvu mnny. Th PTH v w 157.5 p/ml p uy, nd f 55.1 p/ml 15 mnu f mv f h nd. Th d phyd nd mud 1 9 mm. P f h nd w unpnd n h f fm f hd bn u n m p. H um um v mpvd.8 meq/l f uy. Th pn w ubquny nfd u dpmn f fuh n bu MEN1 w upd. Pn MRI f h hd dd n v ny fndn uv f puy dnm. Ahuh h b v f GH w hh, h 75 OGTT ud nhbn f GH n, un u h pby f GHm. Pn CT f h bdmn vd w-dny m (5 mm n dm) n h h dn nd, un h pn f n dnm. Adn nd dnmduy hmn udy hwd vd nn nd dn v, bu h um nd uny v f, dnn, nd ndnn w whn h nm n ny hy vd. Cmbnd wh h f h uppn f w nd n h pd dxmhn uppn, h m w nudd b nn-funnn dn um. H pn ndn um w ny upd b n nunm bd n h vd v f IRI nd CPR. Whn CPR uppn nd uym/hypym mp w pfmd, uppn f CPR w nuffn. Thn w pnnd ASVS mk dn nd z h nunm. In ddn, bu u nun hd md dn f unm un ASVS bf (9), w pnnd u u h pby f unm. ASVS w pfmd dn pdu dbd pvuy (), nd w ud h pd by Hyh (1). W ud C (Dnppn-Summ C. Ld., Ok, Jpn) n h h um un 8.5%. C w njd bu d f.25 meq C 2+ /k bdy wh n h v hzd y. Whn ASVS w pfmd, v n f nun w n dd, bu n ppxmy 6-fd n f un w bvd n h pn y y, ny un h pn f unm (F. 2). Thu, dn mn vd mup um n h pn nn fm 5 25 mm n dm, nd h w vn f h b v f nun, un, mn, nd pn pyppd (PP). In ddn, ASVS dmnd vn f un n h pn y y, whh nud h f h pn. Bd n h fndn, dn f unm w upd nd pp d pnmy w pfmd. Th d f h pn nnd um mun 5 mm nd 2 mm n dm, pndn h n dnfd by mn ud (F. 3). P hpmy w pfmd bu wh ndu, v- 126
Tb2. HmnExmnnfhPnPn n dmn p pn p Op 6 mnh nm n GH IGF-1 ACTH PRL LH FSH TSH FT FT3 PTH nn IR-un FPG IRI CPR PP n mn uny nn vy dn dnn ndnn 6.3 n/ml 3.7 n/ml < 6. n/ml 1 n/ml 121-36 n/ml 38. p/ml 29 p/ml < 6 p/ml 16.9 n/ml 12. n/ml < 27 n/ml 22.3 miu/ml 39. miu/ml.2-79.6 miu/ml 8.3 miu/ml 112.6 miu/ml 12.6-235.7 miu/ml 2.8 µiu/ml.38 µiu/ml.-3.8 µiu/ml 1.1 n/dl 1. n/dl 1.-1.6 n/dl 2.2 p/ml 2. p/ml 2.1-3.8 p/ml 113 p/ml 62 p/ml 111 p/ml 1-66 p/ml 77. p/ml 2 p/ml 21.6-5. p/ml 271 p/ml 19 p/ml 21 p/ml -1 p/ml 17 m/dl 11 m/dl 1 m/dl 7-11 m/dl 2 µu/ml 1 µu/ml 6 µu/ml < 12 µu/ml 5.5 n/ml 3. n/ml 2. n/ml 1.-2. n/ml 87 p/ml < 326 p/ml 93. p/ml 3-15 p/ml 18. p/ml 1.-12. p/ml 25.2 µ/dl 1.9 µ/dl.5-2. µ/dl 2.1 µ/dy 35-16 µ/dy.2 n/ml/h 12.1 n/ml/h.2-2.7 n/ml/h 19.7 n/dl 12.1 n/dl 2.-13. n/dl.2 n/ml.2 n/ml <.17 n/ml.35 n/ml.38 n/ml.15-.52 n/ml Fu1. AbdmnMR mnhwdndunh pn(dm5mm nbdy[nwhd],25 mm n[ndwhd]),unhpnfpn um. mm n z, w fund n h uf f h f b f h v dun uy. Th d pn um w dmnd b um by ph xmnn. Bh um w mpd f wh bu bbn-k pfn, nd w npud wh n n f nvn n h uundn u. Immunnn vd numu un-pv, w m h w pv f nun, mn, PP n bh um (F. -d). On ph xmnn, h wh ndu n h uf f h f b f h v w hwn b n f fb nd pd, un u h pby f v m. Whn ndn ud w pfmd h wk f uy, h v f pn ndn hmn w w (un w 19 p/ml, IRI w 1 μu/ml, CPR w 3. n/ ml, nd mn w 1 p/ml) hn bf h pn. In ddn, w xmnd MEN1 n, f wn nfmd nn w bnd fm h pn, nd.73 dc w fund xn (F. 5). Dun Gunm w f pd n 1966 by MGvn (11). Th bnm pdun f un d db nd hypmndm, n wh ympm uh wh, mpd u n, kn h, muh u, nd nm. Mnn fd h ympm h unm yndm (12). In h p- 127
8 7 Sum IR-Gun (p/ml) Sum IRI (µu/ml) 6 5 3 2 1 1 12 1 8 6 3 6 9 12 SMA pn A GDA hp A SMA pn A GDA hp A 2 3 6 9 12 Fu2. Rufmunndvnumpn(ASVS)nhpn.Eh npnnunndunvnhhpvnfnjnfum un 8.5% wnjdbudf.25meqc 2+ /kbdywhnhdudn y(gda),upmny(sma),pny(sa),ndhpy(ha).a vnfnunwndd,bunppxmy6-fdnfunw bvdnhpnyy. Fu3. Mpfndnfhum.Thd fhpnnndummun5mm nd 2mm ndm,pndnhndnfdby mnud. n pn, un f hypm nd phyd hypp w nd wh h pn w bn fwd up f ub phydmy pfmd 13 y. Fuh nvn vd h pn f pn ndn um nd n dn um, dn h dn f MEN1. Sn pn um fund n 65-75% f MEN1 pn nd unm fquny mnn mn h ndn um f h pn, dffn dn f pn ndn um v f y dn nd y mn. Th pn f v m ndd b h hf pn f f unm. In h pn, ndu w fund n h f b f h v dun d pnmy, bu m w ud u by ph xmnn. In ddn, w ndn pn um w dd n h pn nd w xmnd h mnny f h um un K-67 ndx. W xmnd K-67 ndx, un mhd pvuy pd (13), nd fund h h K-67 ndx w qu w. Thu h pby f mnny f bh um w huh b w (13). Nvh, h pn w nd b fwd up wh pd mn ud. Th vu f pvv un u f dnn nd n ndn um w knwn. Immu nd Dppmn pfmd v nfun f mun n ndn um by dnfn f h fdn y, nd pd h nm nd nunm ud b zd by dn wh n nd um, pvy (-8), bu h 128
F u. M p f nd n f h um ( 2 mm nd m )( 2 ).I mmun h h m n nf u n( ), n u n-( b), m n-( ), p n p y p p d( PP)( d). I mmun h n n v dnum u u np v, w m h w p v f n u n, m n, PP. kn ympm w kn. In ddn, h pn hd n-ndn mpd u n, whh md ndy hypunm nd hypnunm pby. Thu, h dn f unm w dffu fm ny h b vu f hmn. Hwv, ASVS vd vn f h un v n h pn y y, wh nun dd n hw pn uv f nunm. Ou xpn u h ASVS wh um dn ufu f unm, puy f ppv dn n pn wh ny h vn f b un vu. Whn h uv n w xmnd n h pn,.73dc w fund xn. Th w h m mun h S pd 82 dc n 2 (1). F u 5. DNA qu n fmen1 nw x m n d n Aknwdmn h p n.. 7 3 d Cw f und x n. W hnk mmb f md ff n h Dpmn f Mb Mdn nd Dpmn f Gn Suu f ASVS f unm h n bn pd xp y, Ok Unvy Hp f h vub hp wh h f n u hp (9). In h pn, h f- wk. n un v w 271 p/ml (n xmy hh) nd Rfn 1. Bh DK, Fndh V, Ln P, Ck I, Knn PH, Rhmund M. Oum f dudnpn n n pn wh mup ndn np yp 1. Ann Su 22: 757-766, 25. 129 2. Dhy GM. Mup ndn np Typ 1. J Su On 89: 13-15, 25. 3. D R, Km I, Nkmu Y,. Pn ndn um
n Jpn. Pn 28: 27-252, 2.. Dppmn JL, Chn R, Fk DL,. Lzn f nunm n f h pn by n- mun wh um. Ann Inn Md 123: 269-273, 1995 (Eum n: Ann Inn Md 123: 73, 1995). 5. Immu M, Tkhh K, Adh H,. Ufun f v n njn f nm n pn wh Zn-En yndm. Ann Su 25: 23-239, 1987. 6. Immu M, H Y, Nhd O,. Unpnvn f nunm n: nfn f h n n pn wh nunm. Pn 5: 67-73, 199. 7. Bnd M, Pfmm T, Spn GA, Lhmnn R, Shmd C. Amn f v um mun nd hp vnu mpn z nun-n um. Cn Endn (Oxf) 55: 357-362, 21. 8. Tn F, Fn G, N G,. Pnmy n mup ndn np Typ 1-d nm nd pn ndn np. Ann Su 2: 61-7, 26. 9. Yh N, Kjm K, Oh H,. A f pn unm f dm 15 mm, dvd wh md xmnn nd um n n v y w ffv n ppv dn. Hmn T Rnh (Cn Endny) 6: S156-S161, 1998 (n Jpn). 1. Hyh T, Hnd H, Yum K,. Sv n- njn f um f zn f nunm: ppd nw. Nppn Iku Hhn Gkk Zh 55: 952-956, 1995 (n Jpn, Ab n Enh). 11. MGvn MH, Un RH, Rn L, Pk HC, K C, Lvn ME. A un-n ph- nm f h pn. N En J Md 27: 18-113, 1966. 12. Mnn CN, Bm SR, Wn AP, Smn PR, Cx B. A unm yndm. Ln 2: 1-5, 197. 13. P G, B ME, Bn CG,. Endn um f h pn: K-67 mmunvy n pffn n n ndpndn pd f mnny: A mpv udy wh pfn- nu nn nd pn p pn mmunnn, m ndx, nd h nph vb. Hum Ph 27: 112-113, 1996. 1. S M, Kh M, Nhn A,. L nd ympm pn um n pn wh mup ndn np yp 1. Endn 13: 263-266, 2. 29 Th Jpn Sy f Inn Mdn hp://www.nk..jp/mndx.hm 13