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 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 mot vung teo vdnh trdn d trung tam, de lo ro mang mach hdc mac d dtfdi dd Doi khi, vifc khdm mot cdch cd h f thong md phdt hifn trang thdi thodi hod, nhtfng thong thtfdng gidm thi ltfc, ddc biet Id doc sdch, md ddn den viec khdm mdt, ttf dd ma ta phdt hifn mpt dm diem trung tam dtfcmg tinh, doi ngtfdi bfnh nhin vat thay bien dang Benh thodi hod hodng diem lao suy, kho, teo Id 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 teo hodng diem 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 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 m it van 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& gidi deu bi va doi cd stf phan bd' theo dia ly B$nh xuit hien ttf ndm 20 tuoi, c6 the b it dlu b ln g mdt stf gilm thi ltfc va mai den khoang 40 tuoi mdi giam nhieu, bdi vi qua trinh tien trien cua benh c6 nhtfng quang cdch cd dau nhtfc loet giac mac tai diln Soi sinh hien vi thay ro bo mat lam sdng cua loai loan dtfdng thtfdng la ca m it; nhtfng tdn thtfang theo dtfdng thing deu d trung tam gidc mac, khu tru a 2/3 trtfdc nhu mo, khong dung den vung chu bien gidc mac Hinh anh tdn thtfong ddc trtfng, nhtfng soi dng dnh ttf vung sdt ria di vao theo htfdng trung tam gidc mac, bd sgi 1dm chom nhtfng gai nho Cdc soi tdch ddi mQt cdch khong deu va khong chip noi vao Thong thtfdng thi cdc ldp sau gidc mac cung nhtf vung chu bien vin Cam gidc gidc mac co the bi tdn hai; tan mach vd seo gidc mac la nhtfng bien chtfng thtf phdt sau cdc dgt loet Loan dtfdng gidc mac Haab - Dimmer, d mQt giai doan tien trien ndo dd, cd chi dinh r£t tot de 1dm ghep gidc mac, ddc b ift id ghep nong 182 305 HAGEN (Dau hieu) Dugc Hagen mo ta nam 1921 van de bong hac mac Soi xuyen cung mac thay doi dien vdi vung bi bong, ddi nau the mi bi durt doan va d vung bong thi cung mac lai thay qua sdng, dong tuf cung vay 306 HALLERMANN - STREIFF (Loan phdt xuong dau) (Xem FRANCOIS J.) 307 HALLGREN (Hpi chtfng) Hoi chtfng Hallgren (1959) bao gom m§t viem vong mac sdc to', diec bam sinh, mat dieu hoa tien dinh - tieu nao va suy thoai tam than H0i chtfng c6 vd chi mot gen 16p vOi tham nhap b&ng d cd gidi va cd khd nang gay roi loan phtfc tap nhtf gdy viem vong mac sdc to', diec bdm sinh va mat diiu hod d hdu het cac trtfdng hop Benh sdc to v5ng mac la kinh dien va thtfdng ddn den mtf; doi cdn c6 them due the thuy tinh vdi rung giat nhan cdu Diec rat nang Suy thoai tam than la dac di£m cua hoi chtfng va cd the ddn den mot tam than phdn li^t thtfc thu 308 HAND - SCHULLER - CHRISTIAN (B?nh) Ldn ltfot dtfgc Hand (1893), Schuller (1915), vd Christian (1919) nghi&n ctfu, b$nh ndy Id mQt b§nh u vdng cd cholesterin binh thtfdng (xanthomatose normocholesterinique) (b[...]... chtfng co the bieu hien d phtfong thtfc nhe hon nhtf sau: - Hodc la chi rung giat d mdt ben mit dtfa ra; 21 2 - Hoac la rung giat a ca 2 mdt khi diTa m i t nhin ngang, bien do cac dong tac rung giat khong can xtfng gitfa 2 m it ma troi ro hon a ben m at dua ra Nhtf vay thi hoi chtfng Jayle va Ourgaud co 2 dac diem cc( ban la: liet nhin ngang troi hon d mat dua vao va rung giat bien do rong hcfn a mat dua... 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) 1 92 330 H ILLE M A N D (Xem FOIX - HILLEM AN D) 331 H IPPEL (VON)... 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- 20 2 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),... 1dm bien doi sau sdc tdn so ngtfdi mile benh vd dien bien lam sdng cua loai viem gidc mac ke nhu mo ndy 20 4 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... 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... than kinh 366 JAHNKE (Xem STURGE - WEBER - KRABBE) 367 JANSKY - BIELSCHOWSKY (Benh) Chtfng ngu va mil muon Jansky (1910) va Bielschowsky (1914 - 1 921 ) xay ra d tre ttf 1 den 2 tuoi, vk khong phai chi xay ra d gidng ngtfdi Israel nhtf trong benh Tay - Sachs 21 1 Mat khac benh tien trien chain hem k6o dai trong nhiiu nftm va tuy vay, v i n dan den mu vd ttf vong Ndi bat nhfi’t la nhdng trieu chufng tieu... 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... 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 20 3 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... 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 20 1 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... 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 ... 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 Herbert... phia gai thi v i cic mach mau 22 8 L 4 12 LAFFER - ASCHER (Hoi chtfng) DtfOc Laffer (1909) nghien cufu, va sau la Ascher (1919 - 1 921 ), hoi chufng di dang rat hiem thay (khoang 30 trtfdng hop y van,... theo chieu doc vd ca be ngang cua chi den tan xtfcfng va phdn mem Thdm dd mach mdu cho thay ro 22 2 c a c 16 ro d o n g cf t i n h n g a i m d i c o n h t fn g N a m c h u fn g m a c h n h tf tr