Ebook các hội chứng nhãn khoa với bệnh lý toàn thân phần 2

H
301. H A A B (Thoai hoa hodng diem)
Thodi hod hodng diem lao suy Haab (1885) ttfang ting
vdi nhtfng tdn thucfng kho, khong dich ri, gay ra su ngtfng
dong sdc to' khd cd dinh or vung hodng diem. Mdi ddu chi
Id mot it hat sdc to', tdn thtfong hodng diem lan rong dan
vd cuo'i cung tao ra mot vung teo vdnh trdn d trung tam,
de lo ro mang mach hdc mac d ngay dtfdi dd. Doi khi, do
vifc khdm mot cdch cd h f thong md phdt hifn ra trang
thdi thodi hod, nhtfng thong thtfdng do gidm thi ltfc, ddc
biet Id khi doc sdch, md ddn den viec khdm mdt, ttf dd
ma ta phdt hifn ra mpt dm diem trung tam dtfcmg tinh, doi
khi ngtfdi bfnh nhin vat thay bien dang. Benh thodi hod
hodng diem lao suy, kho, teo Id do xo hod ldp mach hdc mac.
Vd hinh dnh ddy mdt, the 1dm sdng ndy ddi lap vdi the thodi
hod hodng diem lao suy dich ri Coppez vd Danis.
Cung vdi cdch suy nghl nhtf vay, ngtfdi ta ndi Id cd thodi
hod hodng diem Haab sau chd'n thtfong neu xdy ra teo
hodng diem trong qud trinh diln bien cua m ft hoi chtfng
dung dap d mdt.

302. HAAB (Kf van)
Ke vdn Haab (1889) Id nhtfng dtfdng van nhd d mdt sau
gidc mac, hinh cong n&m ngang; d£y Id nhtfng vung rdch
d mdng Descemet gdp trong bfnh glocom b£m sinh.

181


303. H A A B (Phan xa dong tiif vo nao - thj giac)
Duoc Kestenbaum neu len, phan xa dong ttf vo nao thj giac Haab bieu hien bang su co dong tu khi m it van

0 tu the bat dong va benh nhan thi chu y ve mot cham
sdng d chu bien thi trudng.
304. HAAB - DIMMER (Loan dudng gidc mac)
Loan dudng mang ltfdi (dystrophie grillag6e) gidc mac
dtfgc Haab va Dimmer nghien ctfu, ndm 1889. Day 1& m0t
loan dtfdng gidc mac d nhu mo, di truyln trQi, c&

2

gidi

deu bi va doi khi cd stf phan bd' theo dia ly. B$nh xuit
hien ttf ndm 20 tuoi, c6 the b it dlu b ln g mdt stf gilm thi
ltfc va mai den khoang 40 tuoi mdi giam nhieu, bdi vi
trong qua trinh tien trien cua benh c6 nhtfng quang cdch
cd dau nhtfc do loet giac mac tai diln.
Soi sinh hien vi thay ro bo mat lam sdng cua loai loan
dtfdng nay thtfdng la ca 2 m it; nhtfng tdn thtfang theo
dtfdng thing deu d trung tam gidc mac, khu tru a 2/3 trtfdc
nhu mo, khong dung den vung chu bien gidc mac. Hinh
anh tdn thtfong ddc trtfng, nhtfng soi dng dnh ttf vung sdt
ria di vao theo htfdng trung tam gidc mac, bd sgi 1dm chom
nhtfng gai nho. Cdc soi tdch ddi mQt cdch khong deu va
khong chip noi vao nhau.
Thong thtfdng thi cdc ldp sau gidc mac cung nhtf vung chu
bien vin trong. Cam gidc gidc mac co the bi tdn hai; tan mach
vd seo gidc mac la nhtfng bien chtfng thtf phdt sau cdc dgt loet
Loan dtfdng gidc mac Haab - Dimmer, d mQt giai doan
tien trien ndo dd, cd chi dinh r£t tot de 1dm ghep gidc
mac, ddc b ift id ghep nong.

182


305. HAGEN (Dau hieu)
Dugc Hagen mo ta nam 1921 trong van de bong hac
mac. Soi xuyen cung mac thay doi dien vdi vung bi bong,
ddi nau the mi bi durt doan va d vung bong thi cung mac
lai thay qua sdng, dong tuf cung vay.

306. HALLERMANN - STREIFF
(Loan phdt xuong dau)
(Xem FRANCOIS J.)
307. HALLGREN (Hpi chtfng)
Hoi chtfng Hallgren (1959) bao gom m§t viem vong mac
sdc to', diec bam sinh, mat dieu hoa tien dinh - tieu nao
va suy thoai tam than.
H0i chtfng c6 vd do chi mot gen 16p vOi do tham nhap
b&ng nhau d cd 2 gidi va cd khd nang gay roi loan phtfc
tap nhtf gdy ra viem vong mac sdc to', diec bdm sinh va
mat diiu hod d hdu het cac trtfdng hop. Benh sdc to v5ng
mac la kinh dien va thtfdng ddn den mtf; doi khi cdn c6
them due the thuy tinh vdi rung giat nhan cdu. Diec rat
nang. Suy thoai tam than la dac di£m cua hoi chtfng va
cd the ddn den mot tam than phdn li^t thtfc thu.

308. HAND - SCHULLER - CHRISTIAN (B?nh)
Ldn ltfot dtfgc Hand (1893), Schuller (1915), vd Christian
(1919) nghi&n ctfu, b$nh ndy Id mQt b§nh u vdng cd
cholesterin binh thtfdng (xanthomatose normocholesterinique) (b

183


tinh gia dinh, xay ra d tre em it tuoi. Trieu chung bieu
hien mot benh tich lipid mo ludi gom cd:
- Nhdng trieu chiing a xuong vdi hinh anh hoc khuyet
trong xtfong so thay ro khi chup X quang (u vdng xtfong);
- U vang noi tang, gan - ldch to;
- Nhdng dau hieu trung nao - tuyin y in nhtf ddi thdo
nhat, ngtfng phat trien, nhi hod, hQi chtfng suy mdn hodc
phi sinh due.

Tri$u chtfng d mdt khd ddc trtfng, g6 m chu y£u mQt
loi nhan cau Id dau hieu thtfc stf cua benh cdnh 1dm sdng.
Bo mdt Id mot "bo mdt nhdi" (faci&s de grenouille); m$t
ntfa so' cac trtfOng hop co loi mdt, do tham lau u vdng vdo
thanh hoc mdt.
Cac lOp mo cua mdt deu co the bi tham ngam u vang
nhtf: ban vang d ket mac va cung mac dtfdi dang nhtfng
chat ngtfng dong mdu vang nhat, loan dtfdng gidc mac do
qua tai gom nhtfng not mdu xdm - vdng nhat tham vdo
nhu mo; ton thtfong d ddy mdt cd kha ndng gay xuat huyet
va dich ri hoac dong chat u vdng d vung hodng diem. Ton
thtfong gai thi (phu ne, viem thi than kinh hodc teo) Id
hau qua cua loi nhan cau.
309. HANHART (Hoi chtfng)
La mQt tap hop nhilu di dang cd nguon goc ngoai bi,
dtfac Hanhart mo ta ndm 1947. Gom c6 day stfng o gan
ban tay, gan bdn chan, tam than tri d

hdm vd viem gidc mac loan dtfdng nong.

HQi chtfng ndy rdt gdn vdi hQi chtfng do Schafer mo ta.

184


310. HANSEN (Benh)
La ten goi kha thong dung cua benh phong, do trtfc
khuan Hansen. Thtfdng hay gay ton thtfong d mat (50 den
80%) va co the d khSp cac bo phan cua m£t.

1. Ton thtfdng mi - ket mac: ton thtfang

d da, vet cham

nhtf ban do, u phong, c6 kha nang xam lan va tham nhap
vao cac mo d mi, tao ra mot ve doi khi that khung khiep.
Hau qua thudng thay la rung long mi, long may va Ion bd
mi. Viem ket mac khong phai 16 dac hieu cua benh, mac dau
ngudi ta c6 mo ta nhUng u phong nho d ket mac sun. Chay
nude mat la do t6c cac dng dan thoat nUdc mat ra ngoai.
2. Ton thtfdng giac mac rat hay xay ra, do mat cam
giac giac mac va do cac tdn thtfang d mi. Ngudi ta phan biet
ra viem giac mac tang san vdi u phong d giac mac va viem
gi6c mac kieu nhu mo hoac c6 loet vdi tan mach phat trien.
3. Ton thtfcfng mang bo dao rat dac trtfng. Viem mong
m6t thtfdng cd nhtfng not nho mau tr6ng nhat d nhu mo
va din den hau qua la bit quanh dong ttf va tang nhan
ap thtf phat. Trong mot so' trtfdng hop, cac ndt d mong tu

tap lai, tao ra mot dam gia u, ttfc la u phong. Cac mang
sau cua m6t doi khi cung bi nhtf viem h6c - vong mac hoac
cd nhtfng not rdi rac d day m6t.
Tdn thtfong th£n kinh m6t hiem tha'y hon: hd mi do
li^t mat, liet v6n nhan ngoai lai v4 nkinh rat hiem xay ra.
311. HARADA (Benh)
Hpi chtfng dtfdc Harada m6 ta ndm 1926, n&m trong
khuon khd nhtfng vi€m m&ng bd d6o - m6ng nao, v6 rat

185


gan vdi hoi chufng Vogt - Koyanaghi. Co the do cdn nguyen
nhiem khuan cd tinh chat virus.
Trieu chiifng gom co:

1. Nhtfng dau hi«?u mang nao - nao co phan ting
mang nao, doi khi cd hon me thtfc stf va nhtfng con co
giat. Dich nao - tuy cd tang albumin nhieu. Dudng di£n
nao ky thudng roi loan, ca d the benh nhe cung nhtf nang.
2. Nhtfng dau hi£u ve thinh giac kha kin dio, bieu
hien bang nghe k6m do nguon goc oc tai, can phit hi^n
bang thinh giac do.
3. Nhtfng dau hi$u d da: bac long tdc, rung ldng t6 c,
lang tr^ng da. Nhtfng trieu chting nky 16m cho b#nh inch
lai gan vdi nhtfng the viem mang bo dao - mang nao khac
(benh Vogt - Koyanaghi).
4. M o t hQi chtfng ve m at. Cac dau hieu d m it co the
la d hang dau cua benh canh lam sang. Bieu hipn mdi dau

bang mot viem thi than kinh thtfc stf cd giam thi ltfc, va
doi khi cd viem vong mac phu ne d ctfc sau. Ve sau, xuat
hien bong vdng mac kinh dien d cd 2 m6t, cd tinh chat
viem vdi cac dau hipu viem mang bo dao trtfdc. Bong vdng
mac cd dich ri, mdi dau d phia dtfdi, sau toan bp, khong cd
chd rach, cd ton thtfdng viem quanh tinh mach va due dich
kinh nang. Tien triln thtfdng la thuan loi va bong vdng mac
se dinh trd lai, de lai sac to rai rac vdi teo hac - vong mac
312. HARRIS (Hoi chtfng)
Chting nhtic ntia dau than kinh kich phat Wilfred Harris
(1920) cd dac diem la dau day than kinh mat va hdc mat
d mQt ben, cd k&m theo cac tri§u chting giao cdm vk d m it

186


nhu do mdt, chay nude mdt. Nhtfng con dau dau, dau day
thftn kinh the mi thudng xay ra v i dem cung gio trong
n hiiu tuan, va co xu the bien dan mot cdch tu nhien.
313. H A R T (Di tat)
Tat mu mau do khong hoan toan (protanomalie) hay di
tat Hart, la mot ton hai bam sinh ve cam giac mau sdc
n&m trong khuon kho nhUng di tat ve nhan thtfc 3 mau
co ban (trichromasies anormales). Benh nhan c6 stf suy
kem ve nhan thtfc thanh phan mdu d6, vd trong phuong
trinh so mau Rayleigh (do + xanh luc = vdng) thay mdu
do nhiiu qud. DuOng bieu diln dQ nhay cdm d mtfc trung
gian gitfa ngudi mu mdu do Dalton vdi ngtfdi binh thudng.
Pho so mdu cd stf suy sup d mdu do. Thuong s6' Rayleigh
la 0,7. Test Farnsworth 100 Hue cho biet cu the di tat ndy

theo true mdu do (gdn true n&m ngang). Stf chenh lech it
se giup phdn bipt di tat Hart hay tdt mu mdu dd khong
hoan toan vdi di tat Dalton hay tat mil mdu do.
314. H A S S A L - H EN LE (Tieu the)
Tieu the Hassal (1846) vd Henle (1866) Id nhtfng to
chtfc kinh loi to, to hoac nhd, phdt trien d chu bien gidc
mac, do su thoai hod thong thudng cua ldp n§i mo Desce­
met. Soi d&n khe thdy nhtfng not ddy d mang Descemet
nhu nhtfng 16 nho sdm toi n&m d phia sau gidc mac. Neu
nhtfng to chtfc loi ndy khu tru d trung tam gidc mac mot
cach tap trung thi se tao ra trang thdi nhtfng giot ntfdc d
gidc mac (cornea guttata), la thu pham gay ra nhtfng roi
loan v i th lm nhap vdo noi mo vd mdng Descemet (loan
dtfdng ngi mo Fuchs).

187


315. H A U ST R A TE
(Xem FRANCESCHETTI - ZW A H L E N )
316. H EB R A (Ban do da dang c6 bong ntfdc).
Duoc Hebra mo ta nSm 1866, benh ban do da dang c6
bong ntfdc la mot thac the lam sang rat rong, vdi d ie diem
la len ban do co phu ne va dich ri 6 mfit da cdc chi, c<$,
than minh va doi khi d m$t. Niem mac c6 th i bi; nhilu
the' 16m sang da dtfoc mo ta tuy theo dinh khu v& mtfc d$
cua bpnh nhtf cac benh ngoai bi trot (ectodermose Erosive),
hOi chtfng Stevens - Johnson.v.v... Vi vfiy, c6 c bieu hi§n d
mat. neu co thi thtfOng la d cac the b e n h nftng (1101 chtfng
Stevens - Johnson, hoi chtfng Fiessinger - Rendu).

317. H E E R F O R D T
(Xem B E SN IE R - BOECK - C H A U M A N N )
318. H E ID E N H A IN (Hoi chtfng)
Hoi chtfng phoi hop cac bieu hien cua ti£n sa sut tri tue lao
suy (predemence senile) vdi noi kho, mat dieu ho6, nm ray.
ctfng dO, vdi mu mat do nguon goc vo nao co day mit binh
thtfong. Stf thoai hoa nao nay chu yeu la d thuy cham va cho
thay trtfOc la se ket thuc bi tham trong thOi gian ngftn

319. HEINE • MEDIN (Benh)
Ngudi ta thtfdng goi dtfdi cai t£n b$nh Heine ( 1840 ) va
Medin (1890) d l chi b$nh viem tuy x£m trtfdc cap tinh
kinh diln (poliomyelite antdrieure aigue).

188


Chung toi k hong muon nhdc lai d day cac trieu churng
lam sang dtfc/c rat nhieu ngtfOi biet cua cac benh nhiem
khuan, dich te va hay lay do virus nay.
Trong trieu chufng hoc cua benh, liet van nhan ngoai
lai cd vi tri khiem tdn thoi. Tuy nhien, liet van nhan ngoai
lai cd the dung den ca 2 day than kinh van nhan cung
nhu day than kinh mat, dan tdi hd khe mi, gay viem giac
mac thu" phdt. L iet van nhan trieu chufng trong the benh
d cao thudng xuat hien d giai doan cap cua benh. Hay co
rung giat nhan cau, nhung thudng chi thoang qua. d tre it
tuoi, cd the chuyen thanh lac co nang. Ngudi ta cung co
mo ta liet cdc dong tac nhin vd nhufng tdn thucfng dr dong
tuf (gian ddng tuf do kich thich giao cdm hay hoi chufng

Claude Bernard - Horner). Soi ddy mdt thay trong mot so
trUdrng hop cd phu gai 2 ben tiep theo sau phu nao. Sau
cung, cdn luu y Id cd the cd benh phoi mdt do virus viem
tuy xdm: cdn luu y la the benh d cao cua viem tuy xdm cd
the gay ra b£nh lipt len cap Landry.
320. H E N C Y (Dau hieu mi)
Duoc Kestenbaum ndi tdi, ddu hieu mi Hency cd the xdc
dinh ro trong liet mdt; benh nhan khong the nhdm mi
ben mdt li#t nhe (paresie) md khong bdt buQC phai nhdm
ca mdt ben kia. Gia tri cua dau hieu nay ddng nghi ngd,
vi nd cd the thay d ngudi binh thudng trong trang thai
sinh ly khi d mot mdt cd tfu the troi ve phan bo' than kinh.
321. H EN LE
(Xem HASSAL - HENLE)

189


322. HENOCH
(Xem SCHONLEIN - HENOCH)
323. H E N N E B E R T
(Xem MYGIND)
324. H E R R E R T (Tieu hoa hong)
Tir do Herbert (1907) d&t ra de chi nhtfng hot d ria hofic
d chu bien giac mac trong benh mdt hot va khu tru d dien
mang mau phia tren. Day 14 nhtfng not nho due Id md,
xung quanh co he thong tan mao mach nho.
325. H E R B E R T (Viem giac mac cham)
D t fg c

Herbert mo ta n&m 1900, va thay d An Do, loai

viem giac mac cham, nong va nhiet ddi nay thtfdng xay
ra a m ot ben mfit va khong lay.
Ton thtfong la nhtfng ch6 loet nho, nong, b it mau, c6
ve nhtf nhtfng cham nho tron, lom d gitfa. Phan tfng viem
quanh giac mac khong nhieu.
Loai viem giac mac nay chu yeu d ngtfOi ldn.
326. H E R R IC K (Benh)
Benh Herrick (1910), mot benh thieu mau co hong cau
hinh liem, hoac benh hong cau hinh liem (drepanocytose)
la mot benh thieu mdu tan huyet di truyln va gia dinh,
dac biet xay ra d gio'ng ngtfOi da den hoftc co td tien da
den. Dac diem chu yeu la trong hong cau co huyet cau to
bat binh thtfdng la huyet cau to' S. Benh bieu hien dtfdi 2
hinh thdi:

190


- Hinh thai n an g a ngUcfi dong hop tuf (homozygote)
co vang da, thieu mau nang, gan - ldch to, nhufng con dau
a noi tang va cac chi do cac vi huyet khoi gay ra boi "sir
tao hong cau hinh liem", khi giam bao hoa oxy trong mau
Luc bay gia benh chuyen bien den nguy kich d tuoi thanh
nien hoac trudng thanh.

- Hinh thai nhe d ngtfdi di hgp tiif, thtfdng la tiem
tang, phdi phat hien bdng cdch tim cau tao hong cau hinh
l iim . Cac bieu hien d mdt, ngay nay da biet ro va thtfdng

tuy theo mdc do nang, nhe cua benh, gom co:
. Cdc cLdu lucu a ket mac nhtf xodn vdng (boucles) mao
mach, gian mao mach d ket mac nhan cau;
. Huyet khoi a dong mach hoac tinh mach vong mac
doi khi gay ra viem thi than kinh thtfc stf;
. Benh a vong mac do huyet cau td' S

bat thudng

(Hannon, 1956); tinh mach cong queo, tdc cac dong mach
nho v& tinh mach nho, seo sdc to hdc - vdng mac, can dich
ri dng dnh, xuat huyet dich kinh, viem vdng mac t&ng sinh
va sau cung la bong vdng mac. NgUdi ta cho rdng benh
vdng mac trong thieu mau hong cau hinh liem tien trien
qua nhieu giai doan:
Giai doan 1: tinh mach gian nd, vung phu ne khu tru;
Giai doan 2: vi phinh mach cd tan mach va chdp noi
dong - tinh mach;
Giai doan 3: xuat hien chay mau, dich ri va nhieu huyet
khdi;
Giai doan 4 :16 xuat hien cuoi cung, co chay mau dich kinh,
viem vdng mac t&ng sinh, bong vdng mac vh teo nhan cau.

191


-

Cdc van mach: (stries angioides): Ngtfdi ta c6 n£u len

nhilu l§n vi su phoi hcfp them nhtfng van mach vong mac
do dtft mang Bruch.
Chan do£n benh Herrick ph&i dtfa v4o stf c6 mfit cua
ckc h6ng c6u hinh lilm trong m4u vk dtfa v6o k§t qua di#n
di cho thay c6 cham hong diu S d4c tnmg.

327. HERTWIG - MAGENDIE (H$i churng)
Hien tuong hofic hpi chtfng Hertwig - Magendie (1833)
14 sir mat song h&nh gitfa 2 m6 t; 2 m6 t l§ch sang bin v i
khong can xtfng; mat d ben tin thuong thi nhin xutfng vk
vao trong, cdn m it kia thi nhin l§n v4 ra ngo4i. Stf l§ch
nhan cau co cung nay van ton tai mac cho mat nhin ve
htfdng nao. Hien tuong nay bieu lo stf doc lap cua cac dong
tac mdt, tuong Ung voi mot ton thuong noi so pham vao
cac dudng tieu nao va me dao, bo doc sau, dau nguyen
nhan la chan thuong, u ung thu lan rong hay ton thuong
mach mau. NguOi ta co mo ta nhtfng the bam sinh. Hoi
chtfng co the tao ra o sue vat thtfc nghiem b&ng cach cat
cuong tieu nao gitfa.

328. HIGASHI
(Xem CHEDIAK - HIGASHI)

329. HILDING (Hoi chUng)
Duoc Stephan Von G rosz noi tdi, hoi chung Hilding phoi
hop nhtfng ton thuong sun - khdp (sun stfdn. sun tai. trat
khdp nhe) vOi nhung trieu chtfng viem m ang bo dao man
tinh (hoi chtfng m ang bo dao - sun - khop).

192

330. H ILLE M A N D
(Xem FOIX - HILLEM AN D)
331. H IPPEL (VON) - LIN D AU (Benh)
B enh u mach vong mac - tieu nao Von Hippel (1904)
va Lindau (1926) thuoc khuon kho nhurng hoi churng than
kinh - da (phacomatose) va nhtfng benh u mach thdn kinh
- mat. Benh co tinh gia dinh trong 20% so trudng hop va
t ru y ln theo kieu troi vdi tinh tham nhap khong hoan chinh
va do bieu hien thay doi.
Ve phuong dien giai phau benh, u mach la mot u mach
mdu (hemangiome) chuyen dang sang u nang. Trieu chufng
g om cd:

1. U mach v on g mac, duoc phat hien nhan mQt tinh
trang gidm thi luc hoac nhan mot hoi chting than kinh.
Trong mot ntia so cac trudng hop, bi ca 2 mdt: soi ddy
m6t, d giai doan ddu cua benh, thay gian mach d ca doi
dong mach vd tinh mach tti gai thi ra, ducfng di ngodn
ngoeo, ra den chu bien thi tan cung b&ng mot not u mach
nho, mdu do, loi ra phia trudc vong mac. Doi khi thay co
nhilu not u mach d vong mac.
Ve sau, bo mdt lam sdng rat goi y ta thay ro mot u mach
thuc sU, trdn, nhu hinh qua trting, trong dd cd doi dong vd
tinh mach chui vdo, mdu slm vd gian rat to. Xuat huyet vd
dich ri vong mac vay quanh u mach. Cuoi cung xay ra nhurng
bien co ndng tai chd nhu chay mdu dich kinh, viem vdng
mac tdng sinh vd glocom thti phdt tuyet ddi.
2

. MQt u mach tieu nao ra't hay phoi hop theo, hodc

cdn phdi phdt hien bdng cac x6t nghiSm thdn kinh vd

193


chup dong mach Luc bay giO u mach se din den mpt hpi
chiing tang ap luc noi so (ton thucfng nao that IV), cac dau
hieu tieu nao va doi khi xuat huyet a mang nao. U mach
nang hoa hiem khi o than nao va tuy. NhCifng con dpng
kinh va roi loan tam than c6 dupe neu len.
3.

C ac nang u npi tang thinh thoang cd phoi hop vdi

u mach than kinh - vong mac, thudng khu tru a tuy, 16ch,
gan va than.

Tat ca nhurng bieu hien 16m sang khde nhau n£y hiem
khi dupe day du, nhimg can ph6 i tim ki£m m$t c6 ch cd h#
thong d ngtfdi benh.
Ngdy nay, phtfOng phdp quang dfing cho phgp ta giai
quyet duoc sOm u mach vong mac mot cach co hieu qua.
332. H IR SC H B E R G (Viem vong mac trung tarrn
Do nhtfng cong trinh nghien ctfu cua Hirschberg 1 1 & 9 0
- 1891) ma doi khi ttf nay dupe dung de chi benh viem
vong mac trung tam, co cham, vi xuat huyet va dich n , 6
benh dai thao. Do la bieu hien vong mac thudng hav co

trong benh dai th6o dudng.
333. H IR SC H P R U N G (Benh)
Dupe Hirschprung mo ta n&m 1888, chtfng to ruot ket
hoac to va dai ruot ket bam sinh (megacolon, megadolichocolon congenital) la mot benh cua tre tho au. ma trieu
chtfng co nang va X quang rat goi y.
Di dang ruot nay doi khi thay phoi hop vOi nhtfng di
dang mat kieu bam sinh, nhu viem vong mac sdc to co
rung giat nhan cau va due the thuy tinh bam sinh iWorster

194


Drought), trail dich nao co teo thi than kinh (Shafar) va
khuyet tat cua day than kinh thi giac (Giirich).
334. H O D G K IN (Benh)
N am

1932, Hodgkin da lay ten minh dat cho benh

lym pho hat ac tinh. Paltauf va Sternberg (1897 - 1898) da
thuc sU biet lap benh nay ra khoi cac benh bach cdu
(leucemies), cac benh viem va ung thu. Benh lympho hat
ac tinh kha hay gap va thudng xay ra d ngUOi tre. Benh
Hodgkin, ve dang lam sang thi dinh vdi ung thu, ve cau
tao md hoc thi dinh vdi viem. Trieu chufng chung co dac
diem chu yeu la noi hach nhieu, d nong va sau, gio'ng nhu
nhurng mo tan tao thuc sU, gay ra cdc trieu chufng chen dp.
Hach d nhieu noi; lach to, phoi, xuong, mang phoi, gan, da
lam cho trieu chufng hoc da dang. Lam sinh thiet, dem va
lam cong thufc mau se cho phep xac dinh chan doan benh.

Ndi chung thi ft thay cd bieu hien d mdt, vd nhdng bieu
hien nay cd le la do sU tdng san mo u hat.
- U hoc mdt boc lo bdng loi nhan cau, thudng d mot
ben, tien trien man tinh, cham vd tuan tien; chup X quang
thay nhufng cho ne cua xuong.
- Cdc bieu hien ve thdn kinh - mdt Id do cdc the benh
gia u gay ra nhufng trieu chdng chdn dp vdo cdc dudng thi
giac va van nhan. Vi vay md ngudi ta da mo ta cdc teo
thi than kinh, phu gai, hoi churng khe budm, hoi chdng gdc
cau - tieu nao, hoi chdng Claude Bernard - Horner, tdn
thuong day than kinh sinh ba,v.v...

- Trong qua trinh bien chuyen cua benh, soi ddy mdt
cd the thay tinh trang cdc mach mdu gian rong ra va

195


ngo&n ngoeo, xuat huyet vong mac dudi nhieu ve khdc
nhau. Cuoi cung, nguOi ta cung co noi den nhurng tdn thtfong
khu tru cu the hcfn nhung rat hiem thay nhu not d da mi,
hoi chtfng Mickulicz, viem gidc mac nong vd cdc tri#u chtfng
mang bo dao nhu u d mong mdt, viem mong mdt th£ kin
dao, viem hdc mac trung tam hodc chu bien.
335. HOEVE (VAN DER) (dd'u hi^u)
Benh viem nhiim d mui - xoang lan r§ng dfi'n htfc mdt,
doi khi gay nhufng trieu chufng ton thuong d ddy t.h4n kinh
thi. Diem mu to ra cd phoi hop hay khdng vdi mpt dm
diem trung tam (dau hieu Van Der Hoeve, 1910) 16 ddc
diem cua viem thi than kinh hdu nhan cdu.

336. HOEVE (VAN DER) (Hoi chdng)
Benh duoc nhiiu ngudi biet hon vdi cai ten Id benh
nhuyen cung mac thung (scleromalacie perforante), do Van
Der Hoeve mo ta ndm 1921. Ld mot benh ky di, doc ddo,
rat hiem thay, vdi dac diem 1a hoai tuf mo cung mac gav
ra nhtfng lo rd thtfc stf d cung mac. Nhuyln cung mac
thudng hay xay ra trong qua trinh viem da khdp ndng do
thap cd kem theo nhtfng not thap dudi da. Chu yeu la benh
tien trien mot cdch dm y, thdm ldng, khong cd mot dau
hieu co ndng vd viem nao, It nhat cung la trong nhtfng
giai doan ddu. Benh khdi phat bdng nhtfng vung gian mong
cung mac, mau xanh, xuat hien tren nhtfng mang hoac not
mau vang nhat 0 phdn trUOc cung mac, gitfa ria giac mac va
vung xich dao nhan cau. Qua trinh viem se lan vao sau, dan
dan gay ra mot ch6 thung d cung mac; cuoi cung hinh thdnh
mot dam hd rong da giac xung quanh nhan cau, cdch ngoai

196


giac mac mot khoang. O ngay dudi do, hdc mac bi lo ra,
va chi con duoc bao phu bdi ldp ket mac nhan cdu.
Kinh dien thi hSc mac khong bi gian loi ra. Rat hiem
thay nhan cau bi thiing thuc sU, neu co thi se kem theo
cac phan dng viem.
337. H O E V E (VAN DER)
(Xem LOBSTEIN)
338. H O L T E N M U L L E R - W IE D E R M A N N
(Hoi chdng)
Duoc cac tac gia mo ta ndm 1960, 14 hoi chdng da di

dang, xay ra d tre rat it tuoi, gom co di dang so hinh 3
la (en trefle), cd tran dich nao, chi ngdn va ban tay dinh
ngdn. Cdc di dang d mdt di kem theo vd cd the gay ra teo
thi than kinh nhu trong moi roi loan xuong so. Sau cung
can neu len nhufng di dang d xuong mdt, mui va ham.
339. H O L T H O U S E - B A TTE N
(Thoai hoa hoang diem)
Loai thodi hoa hoang diem nay, duoc Holthouse va
Batten mo ta ndm 1897, ndm trong khuon kho nhtfng thoai
hod kinh vung gai - hoang diem. Hinh anh day mdt g6m
nhieu cham nho, trdng, nhieu d xung quanh gai va phia
vung hoang diem. Nhufng cham nho trdng - vdng nay hinh
cham tron, hodc chieu ngang rong hon (dudng kinh gap 3
- 4 ldn dudng kinh mach mdu vong mac), cd ve khong dung
den vung phia mui cua ddy mdt, vd khong cd sdc to kem
theo. Gai thi mdu sdc vdn binh thudng va mach mdu vong

197


mac k hong thay doi. Chufc nang thi giac it thay ddi
(Holthouse va Batten, Bickerton). The loai l§m sang nky
co ve giong nhUng thoai hoa vong mac khac nhu vifem hdc
mac giot choroiditis guttata) va thoai h6a hoang diim
Doyne theo kieu "tang to ong".

340.

HORNER

(Xem CLAUDE BERNARD - HORNER)
341. HORTON (Benh)
Duoc Horton tach biet ra vi mat lSm sang nam 1932, bgnh
nay thuoc nhufng benh chat tao keo, co dac diem la viem dong
mach dau, nhieu nhat la o vung dong mach thai duong.
Benh thuong xuat hien o nguOi Ion tuoi, gom nhung con
dau viem dong mach thai duong vdi nhufc dau duf doi, nhat
la o thai duong va co the lan len dau. The trang chung co
bi ton hai, sot nhe, toe do ldng mau tang va thieu mau.
SO nan, doi khi thay co sU biih d6i d dong mach thai
duong nong, nhu mpt soi day thifng rdn, cufng, Ion nhon
nhufng not. Lam sinh th iet d dong mach thai duong nong
cho thay nhufng ton thuong dac trung cua viem dong mach
dang u hat co te bao khong lo.
Bien chufng d mdt thay co khoang 50% cac truOng hop.
va lam cho benh nang them; bien chufng la do viem dong
mach khu tru or cac mach mau nhan cdu va hoc mdt. Doi
khi, cac bieu hien 0 mdt md dau benh canh lam sang.
Loi mdt tam thcJi va han che dong tac van nhan thudng
la vo hai. Bien chufng thi giac mdi dang so va co tinh chat

198


bi tham. Mu dot ngot m ot mdt va ca hai mdt la bieu hien
thuong gap. Co the mu don thuan hoac phoi hop vOi nhufc
dau xay ra trong can tien trien hoac sau con. Doi khi co
nhung can nhin ma ch oc lat (crises d’amblyopie transitoire)
xay ra trudc khi bi mu.
Hinh anh day mdt co nhilu ve lam sang; thuong hay

thay nhat la phu ne thieu mau dr gai thi (gia viem gai
mach) vOi dla thi bi phu va tai nhat, co k&m theo cac ke
xuat huyet doc theo mach mau. Doi khi thay co mot tdc
nghen d dong mach trung tam vong mac hoac a mot nhdnh
cua no, tao ra phu vong mac kinh dien vOi hoang diem do
noi bat. Cung co the co benh canh lam sang cua mot viem
thi than kinh cap. Dau the ndo thi cung chi trong it ngdy
hoac it tudn, se ket thuc bdng teo thi thdn kinh vlnh viln
vk mil. Ngoai nhufng dau hieu ve teo thi than kinh, soi ddy
mdt cdn thay nhufng ddu hieu c6 lien quan den mot xo
cufng dong mach vong mac.

Dieu tri lau dai bdng Corticosteroid c6 the phan nao
phOng ngCra dtfgc cac hien tuong thieu mdu cuc bo d vong
mac vd thi thdn kinh.
342. HUDSON - STAHLI (Dtfdng)
Duoc Hudson mo ta ddu tien ndm 1911, sau dd 14 Stahli
sinh 1918, day la mot dudng sdc to d gidc mac thay duoc
bdng soi sinh hien vi, thudng gap cr ngtfdi gid tren 50 tuoi.
Day la mot dudng vien cd sdc to', doi khi mau vang nhat
hodc xdm nhat, d cdc ldp nong giac mac, chay theo chilu
ngang d doan tiep gidp giufa 1/3 dudi va 1/3 gitfa cua gidc
mac, tufc la d di$n khe hd cua mi mdt. Stf tham nhap vao

199


cac ldp nong nhat cua nhu mo bdi cdc chat Idng dong
hemosiderin hoac sSc to ttf ria vao, c6 th# 14 do ttf nhtfng
chd ntft ne cua mang Bowmann. Cdn thdy stf tuong ttf vdi

vong Fleischer gap trong gidc mac hinh chdp.

343. HUGHLINGS - JACKSON (Dau hieu)
Nho cocain cho phdp chan dodn phan biet gitfa sup mi
do liet (ton thuong day III) vdi sup mi giao cam (ton thuong
co sun tren); trong sup mi giao cdm, nho cocain khong gfiy
gian dong ttf (dau hieu Hughlings - Jackson).

344. HUNT
(Xem TOLOSA - HUNT)

345. HUNTER
(Xem HURLER)
346. HURLER (Benh)
Benh nay duoc Hunter mo ta trudc nSm 1917, den nSm
1919 duoc Von Pfaundler vd Gertrud Hurler nghien ctfu
sau hon. Cho nen benh mang ten khdc nhau cua nhtfng
tac gia nay; ngodi ra, benh cdn dUdc goi bdng nhtfng ten
khac nhu loan phdt nhieu xuong, loan dudng md - sun (lipo
- chondro - dystrophie) va da loan dudng (gargoihsme
Hoi chtfng nay ndm trong khuon khd rong ldn va phuc tap
cua cac benh mucopolysacarit. Thuc ra, benh Pfaundler Hurler co ve tuong tfng vdi mot roi loan chuyen hoa giong
nhu co tinh chat enzym cua cac mucopolysacarit acid dan
tdi mot sU xam lan thuc thu vao nhidu co quan va md nhu
gan, hach bach huyet, lach, sun, bach cau, giac mac va he

200


than kinh. Stf tham nhap nay xay ra trong noi te bao. Cac

xdt nghiem sinh hoc cho thay co nhufng hat Alder - Reilly
va Gasser trong bach cdu, co tuang bao hoc (plasmocytes
vacuoles) trong tuy (te bao Buhot). Dinh ltfcmg mucopolysa­
carit trong ntfdc tieu cho thay ty le t.&ng cao. Do d6 co the
chdn dodn benh da loan dtfdng bdng xet nghiem sinh hod
hoc vd cho phdp phat hien ra nhtfng the benh chtfa ro rang
(formes frustes).
B enh Hurler xay ra d ca nam va ntf vdi tdn so bdng
nhau. Cdc b en h dn lam sang doi khi cho thay tinh chat
dan phat ma k h on g co m ot yeu td gia dinh nao. Tuy nhien,
cdc cdng trinh nghien ctfu vd di truyln hoc nhd'n manh
den tinh cha't di truyen cua benh, vd thudng thay di truyen
theo phtfong thtfc ldp autosom.
Su tdng hdm ltfong mucopolysacarit trong ntfdc tieu cd
vd chi ra tinh trang dd c6 di san xd'u (tare) d ngudi di hop
tuf (porteur hdtdrozygote) trong thd' he trtfdc ho&c d con cdi
md tren lam sdng v in binh thtfdng. Thtfdng thdy cd tinh
trang bd' me cung huyet thdng. Cung cd trtfdng hop di
truyen theo kilu ldp lidn quan vdi gidi tinh, chi ddnh vdo
nam gidi vd do me truyen lai. Cd nhtfng cdng trinh nghien
ctfu ve di truydn hoc vd sinh - hod hoc cho bid't cd nhilu
kieu sinh - hod hoc trong benh da loan dtfdng. Trang thdi
mucopolysacarit nUdc tieu c6 the’ thay doi d ngtfdi di hop
tuf, tuy nhien neu ho lay nhau thi cd the ddn den mot da
loan dtfdng hoan chinh.
-

C ac tr ie u chufng chinh, ddi khi xuat hien rd't sdm

d 1 tudi, vdi nhtfng ddc diem Id di dang xuong so - mdt:

ddu hinh thuyIn (scaphocephalie), 2 mdt cdch nhau qud

201


xa, tran do, tf6n song mui, moi ddy va tfdn treu ra. ltfdi
to, loan dudng r&ng (ddu nhu mieng dng mdng). Mat khac,
co them nhdng roi loan ve phat trien xuong vdi chung lun,
dlin den gu lung - cot sdng cd det dot sdng (platispondylie)
va di dang d cac dau xuong, nhat la xuong co tay v4 xuong
ban tay. Suy giam tri tue, din den mot stf tri dpn thtfc stf.
Sd nan bung thudng thay gan to, ldch to. Sau cung 14 nen
luu y den tinh trang gian long co - da cua v4nh dai quanh
bung, tao ra thodt vi ron, thodt vi ben.v.v.... Chup X quang
cho thay ao yen rong ra, cham cot hod d cdc diem dau
xuong dai, det va lech lac cac dot xuong song.
-

Benh Hurler tien trien khdc nhau. (J m$t s6 the b#nh,

co tien luong nang, td vong vao khoang 10 tuoi trong mot
benh canh suy tim hodc tf tic ho hip vdi nhiim khuan
xay den. Cd nhtfng the b|nh khdc, tien trien ttf ttf va hoan
chinh dan qua nhilu nfim. Benh nhan 14 nhtfng ngudi tan
phe nghiem trong, tri dpn v4 cd th i keo dai den tuoi ldn
> Tri^u chting d mdt, chu yeu 14 tich lipid md ltfdi gidc
mac d cA 2 m it, trong hon 70% cac trtfdng hop; gidc mac md
due tin man, nhtf ddm may, thtfdng nhin bing mit cung
thay. Ddi khi, chi soi b&ng sinh hien vi mdi phat hien ra
dtfgc nhtfng chd md due hinh cham hodc dam md hcri tring

chiem ca dien tich giac mac, v4 thtfdng troi hon a 1/3 trtfdc
giac mac. Nhtfng cham nay doi khi rat tinh vi, dac biet hay
d vung chu bien ngay sat ria giac mac. Ve nguyen t i c thi
khong co tan mach va khong roi loan cam giac giac mac
Giam thi luc tuy trudng hgp nhtfng thtfdng khong nhieu
Xet nghiem mo hgc cho thay ton thtfong d phan trtfdc
cua nhu mo nhieu hon. Khoang gitfa cdc 14 (espace in-

202


terlam ellairej rong ra, hi tach rdi va gom mot dam hat co
nhieu cham nho; bieu mo giac mac thudiig nguyen lanh. Thay
co ton thtfcfng d cac te bao day gan mang Bowman, nhtf co
hoc (vacuolisation), phu ne va cang phong do mot cai mang
co mau s&c khac. Cho nen cac to chtfc gia u nang cua te bao
day d bieu mo cung vdi stf pha huy mang day cf mot vai cho
co ve nhtf la mot dac diem cua ton thucfng giac mac.
- C on thay nhUng bieu hien khftc of mftt nhu glocom
bam sinh, giac mac to, khuyet m ong mdt, due the thuy
tinh bam sinh, lac mat va ton thuong d mi. Sau cung la
nhufng phoi hap vdi ton thuang thoai hoa vong mac, dac
b iet la vie m vong mac s&c to.
Ve trieu chufng hoc, co mot vfti benh gin gio'ng vdi hoi
chufng Hurler nhu:
- Benh Morquio (1929). Hoi chufng nfty rat gan gui vdi
benh Hurler, truyen theo kieu ldp autosom, thudng 111 loan
dudng sun thuoc cftc loai benh mucopolysacarit. Di dang
hinh thfti nghiem trong nhu lun nhieu ngay ttf luc 1 - 2
tuoi vdi xuang uTc nho ra trUdc, det dot song, gii lung - cot

sdng, goi veo vfto trong (genu valgum), bftn tay veo, dui
cong vao (coxa vara), nhao cftc khdp co tay vft ngdn tay.
Di dang d mat it hem trong hoi chufng Hurler vft tri tue
van duac nguyen ven. Thudng bi thinh gidc kem. Ton
thuang noi tang it hoac khong cd; tham nhap giac mac
cung vay. Tuy nhien cung cd mdt so' b£nh an duac mo ta
(benh Morquio - Ullrich, 1943) la cd hoi chufng loan dudng
giac mac kinh dien cua benh Hurler.
- Maroteaux (1961) co mo ta chufng lun da loan dudng
lft mot the benh di truyen khac. Day 1ft stf loan san dot

203


song - dau xtfcmg muon (dysplasie spondylo - epiphysaire
tardive) phdt hien vao khoang 10 tuoi, trong do cdc chi
ttfang doi nguyen lanh so vdi cdc dot song. The benh ndy
di truyen, lien quan vdi the n h i i m s i c X.
Cuoi cung, chung toi neu len mpt vdi bi£n th§ ldm sdng
khdc cua hoi chting Hurler vd Morquio, phdt hien bdng x6t
nghifm h6a hoc ntfdc tieu.
- Hoi chdng Sanfilippo (1961): di truyln 16p autosom,
cd tdng heparitin sulfat trong ntfdc tilu.
- H6i chiing Scheie (1962): di truyen I6 p autosom, c6
tdng chondroitin sulfat B trong ntfdc tilu.
347. H U T C H IN SO N (Viem gidc mac)
T en goi benh viem gidc mac khe nhu mo kinh dien do
nguon goc giang mai thudng la bam sinh, vd han htfu mdi
la hau phdt (Hutchinson, 1858 - 1863). Chung toi khong
cdn phai mo ta lai cai benh nhieu ngtfdi biet nay, ma ngay

nay ft con thay. Benh tien trien trong nhieu thdng vdi
tham lau, tdn mach d gidc mac vd ve sau tieu bien di, de
lai nhting ton thtfang viem d bdn phan trtfdc. Nhtfng bien
doi d gidc mac do di chtfng de lai dtfdi dang nhting day
mach t&c nghen d ldp sdu gidc mac, ket hop vdi test chan
doan benh giang mai, cho ph£p ta chan dodn hoi ctfu nhurng
the benh cu vd da lam seo. Can chu y, hay thay co tat
can thi vd cdc tdn hai hdc - vong mac, cung lam cho thi
ltfc giam. Hien nay, vdi cdc phtfang phdp dieu trj va du
phdng benh giang mai, cdc thuoc cortico - steroit da 1dm
bien doi sau sdc tdn so ngtfdi mile benh vd dien bien lam

sdng cua loai viem gidc mac ke nhu mo ndy.

204


Chung toi nhSc lai a day cac nhan to tao thanh bo ba
trieu chufng H utchinson cd lien quan vdi benh giang mai
di truyen va cac di dang ciia no:
1. Viem giac mac nhu mo ot ca 2 mat, co tan mach;
2. T on hai d rang (rang Hutchinson); cac rSng ciifa tren,
giufa va vlnh cufu co hinh tuocncfvi't (dung cu van dinh vit),
cd rang rong hon d l u rang, va dau rang co the bi lem
khuyet. Cac rang cufa cung m oc xa nhau, cam cheo va khong
co men rang. Soi xuyen co the thay ro tdn thucmg cr cac
rang vlnh ciifu khac.
3. Diec tir tu-, nang do tdn thucfng me dao.
Ve cac dau tfch khac cua giang mai bam sinh, khong
thuoc pham vi mo ta cua chung tdi of day.

348. H U T C H IN S O N (Hoi chiing)
Hoi chiirng Hutchinson tuong ting vdi nhting di can vao
hoc m it cua nhting u thuang than d tr£ em. Nam 1907
Hutchinson cd bao cao ve 10 trudng horp u thuong than cd
di can vao xuong va so. Ng&y nay, ngudi ta thtia nhan ring
nhting u l y la nhting u nguyen bio than kinh thuong than
(neuroblastome surrenalien) gap d tre rat it tuoi, vao
khoang 2-3 tuoi, han htiu d tre ldn hcfn. Ung thu thuong
than cd th i gfiy ldi nhan clu do u khu tru d thai dtfcfng
va hoc m it, trieu chting ldi m it nay ddi khi cd gia tri phat
hien benh. Doi khi ldi nhan cau manh or mot ben m it,
han htiu 2 m it, mdi dau cd xuat huyet d mi. Chup X quang
xac dinh sU xam lan vao xuong. Di can thai duong - hdc
m it cua hoi chting Hutchinson bieu hien su lan rong nhanh
cua u nguyen b&o th in kinh thuong than.

205