{"id":670,"date":"2021-06-09T22:06:09","date_gmt":"2021-06-09T15:06:09","guid":{"rendered":"https:\/\/cdha.edu.vn\/cdha\/?p=670"},"modified":"2021-06-09T22:06:10","modified_gmt":"2021-06-09T15:06:10","slug":"da-u-nang-tuyen-ba-steatocystoma-multiplex","status":"publish","type":"post","link":"https:\/\/cdha.edu.vn\/cdha\/da-u-nang-tuyen-ba-steatocystoma-multiplex\/","title":{"rendered":"\u0110a u nang tuy\u1ebfn b\u00e3  (STEATOCYSTOMA MULTIPLEX)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\"><strong>BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ed9 m\u00f4n Da li\u1ec5u, \u0110\u1ea1i h\u1ecdc Y H\u00e0 N\u1ed9i, Khoa D2, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng.\u00a0<\/strong><br><a href=\"http:\/\/www.nhdv.vn\/chuyen_mon\/chia_se_chuyen_mon_cung_dong_nghiep\/articletype\/articleview\/articleid\/33481\/steatocystoma-multiplex\">Link b\u00e0i g\u1ed1c<\/a><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>\u0110\u1ecbnh ngh\u0129a<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Steatocystoma multiplex (SM) l\u00e0 m\u1ed9t b\u1ec7nh di truy\u1ec1n hi\u1ebfm g\u1eb7p, tr\u00ean da c\u00f3 r\u1ea5t nhi\u1ec1u k\u00e9n tuy\u1ebfn b\u00e3, b\u1eaft \u0111\u1ea7u \u1edf tu\u1ed5i d\u1eady th\u00ec. B\u1ec7nh \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 nh\u01b0 m\u1ed9t bi\u1ebfn d\u1ea1ng h\u00ecnh th\u00e1i ki\u1ec3u hamartoma c\u1ee7a \u0111\u01a1n v\u1ecb nang l\u00f4ng-tuy\u1ebfn b\u00e3. N\u1ebfu ch\u1ec9 c\u00f3 \u0111\u01a1n \u0111\u1ed9c m\u1ed9t k\u00e9n th\u00ec g\u1ecdi l\u00e0 steatocystoma simplex. Th\u01b0\u01a1ng t\u1ed5n da l\u00e0nh t\u00ednh, kh\u00f4ng chuy\u1ec3n sang \u00e1c t\u00ednh nh\u01b0ng g\u00e2y ra c\u00e1c v\u1ea5n \u0111\u1ec1 t\u00e2m l\u00fd cho ng\u01b0\u1eddi b\u1ec7nh. M\u1ed9t s\u1ed1 th\u01b0\u01a1ng t\u1ed5n c\u00f3 th\u1ec3 b\u1ecb b\u1ed9i nh\u1ec5m, g\u00e2y s\u1eb9o. Kh\u00f4ng c\u00f3 s\u1ef1 kh\u00e1c nhau v\u1ec1 t\u1ea7n s\u1ed1 m\u1eafc b\u1ec7nh theo ch\u1ee7ng t\u1ed9c v\u00e0 gi\u1edbi t\u00ednh.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>C\u01a1 ch\u1ebf b\u1ec7nh sinh<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Steatocystoma multiplex di truy\u1ec1n tr\u1ed9i tr\u00ean nhi\u1ec5m s\u1eafc th\u1ec3 th\u01b0\u1eddng. Nh\u01b0ng c\u00f3 nhi\u1ec1u tr\u01b0\u1eddng h\u1ee3p kh\u00f4ng c\u00f3 ti\u1ec1n s\u1eed b\u1ec7nh c\u1ee7a b\u1ed1 m\u1eb9, gia \u0111\u00ecnh, g\u1ecdi l\u00e0 di truy\u1ec1n l\u1ebb t\u1ebb (sporadic). M\u1ed1i li\u00ean quan c\u1ee7a b\u1ec7nh v\u1edbi s\u1ef1 ph\u00e1t tri\u1ec3n c\u1ee7a tuy\u1ebfn b\u00e3 \u1edf tu\u1ed5i d\u1eady th\u00ec g\u1ee3i \u00fd hormon l\u00e0 y\u1ebfu t\u1ed1 kh\u1edfi ph\u00e1t.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Trong th\u1ec3 c\u00f3 y\u1ebfu t\u1ed1 di truy\u1ec1n gia \u0111\u00ecnh, c\u00e1c \u0111\u1ed9t bi\u1ebfn n\u1eb1m tr\u00ean gen\u00a0<em>K17<\/em>\u00a0(keratin 17), \u1edf v\u00f9ng \u0111\u00f3 c\u0169ng c\u00f3 \u0111\u1ed9t bi\u1ebfn c\u1ee7a b\u1ec7nh lo\u1ea1n d\u01b0\u1ee1ng m\u00f3ng b\u1ea9m sinh (pachyonychia congenita) typ 2 (PC-2). PC-2 l\u00e0 r\u1ed1i lo\u1ea1n di truy\u1ec1n tr\u1ed9i tr\u00ean nhi\u1ec5m s\u1eafc th\u1ec3 th\u01b0\u1eddng, c\u00f3 c\u00e1c bi\u1ec3u hi\u1ec7n lo\u1ea1n d\u01b0\u1ee1ng m\u00f3ng, d\u00e0y s\u1eebng khu tr\u00fa l\u00f2ng b\u00e0n ch\u00e2n, l\u00f2ng b\u00e0n tay, nhi\u1ec1u k\u00e9n nang l\u00f4ng tuy\u1ebfn b\u00e3, b\u1ea1ch s\u1ea3n \u1edf mi\u1ec7ng, c\u00e1c tri\u1ec7u ch\u1ee9ng kh\u00e1c c\u1ee7a lo\u1ea1n s\u1ea3n ngo\u1ea1i b\u00ec. Keratin 17 b\u1ed9c l\u1ed9 \u1edf m\u1ed9t s\u1ed1 c\u1ea5u tr\u00fac tuy\u1ebfn, \u0111\u1eb7c bi\u1ec7t l\u00e0 tuy\u1ebfn b\u00e3, l\u1edbp \u00e1o ngo\u00e0i c\u1ee7a nang l\u00f4ng, gi\u01b0\u1eddng m\u00f3ng. S\u1ef1 b\u1ed9c l\u1ed9 c\u1ee7a n\u00f3 t\u01b0\u01a1ng quan ch\u1eb7t ch\u1ebd v\u1edbi l\u00e2m s\u00e0ng c\u1ee7a c\u1ea3 PC-2 v\u00e0 SM. Cho t\u1edbi nay, c\u00f3 14 \u0111\u1ed9t bi\u1ebfn \u0111\u00e3 \u0111\u01b0\u1ee3c m\u00f4 t\u1ea3 \u1edf b\u1ec7nh nh\u00e2n PC-2 ho\u1eb7c SM, t\u1ea5t c\u1ea3 \u0111\u1ec1u n\u1eb1m tr\u00ean domain 1A c\u1ee7a gen\u00a0<em>K17<\/em>. M\u1ed9t v\u00e0i t\u00e1c gi\u1ea3 cho r\u1eb1ng SM ch\u1ec9 l\u00e0 m\u1ed9t th\u1ec3 \u0111\u01a1n gi\u1ea3n c\u1ee7a PC-2 v\u00ec c\u1ea3 hai b\u1ec7nh c\u00f3 nhi\u1ec1u \u0111i\u1ec3m gi\u1ed1ng nhau v\u1ec1 b\u1ec7nh sinh. SM di truy\u1ec1n l\u1ebb t\u1ebb kh\u00f4ng li\u00ean quan t\u1edbi \u0111\u1ed9t bi\u1ebfn c\u1ee7a gen\u00a0<em>K17<\/em>.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Bi\u1ec3u hi\u1ec7n l\u00e2m s\u00e0ng c\u1ee7a b\u1ec7nh<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">C\u00e1c th\u01b0\u01a1ng t\u1ed5n \u0111\u1ea7u ti\u00ean xu\u1ea5t hi\u1ec7n \u1edf tu\u1ed5i d\u1eady th\u00ec, v\u1ecb tr\u00ed hay g\u1eb7p nh\u1ea5t l\u00e0 b\u1ee5ng, ng\u1ef1c, ph\u1ea7n tr\u00ean c\u00e1nh tay, n\u00e1ch, m\u1eb7t. \u1ede m\u1ed9t s\u1ed1 tr\u01b0\u1eddng h\u1ee3p, c\u00e1c th\u01b0\u01a1ng t\u1ed5n c\u00f3 th\u1ec3 xu\u1ea5t hi\u1ec7n kh\u1eafp c\u01a1 th\u1ec3.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">C\u00e1c k\u00e9n tuy\u1ebfn b\u00e3 c\u00f3 k\u00edch th\u01b0\u1edbc 2-20 mm, m\u1eadt \u0111\u1ed9 m\u1ec1m t\u1edbi ch\u1eafc, c\u00f3 m\u00e0u da, m\u00e0u ng\u00e0 v\u00e0ng, ch\u1ee9a ch\u1ea5t d\u1ecbch v\u00e0ng, nh\u1ea7y m\u1ee1. Khi l\u00e0m cho k\u00e9n c\u0103ng l\u00ean c\u00f3 th\u1ec3 th\u1ea5y m\u1ed9t ho\u1eb7c nhi\u1ec1u v\u1ebft l\u00f5m tr\u00ean b\u1ec1 m\u1eb7t, t\u01b0\u01a1ng \u1ee9ng v\u1edbi ch\u1ed7 \u1ed1ng tuy\u1ebfn b\u00e3 \u0111\u1ed5 v\u00e0o nang l\u00f4ng. M\u1ed9t s\u1ed1 k\u00e9n c\u00f3 th\u1ec3 b\u1ecb vi\u00eam, b\u1ed9i nhi\u1ec5m, khi l\u00e0nh \u0111\u1ec3 l\u1ea1i s\u1eb9o, gi\u1ed1ng tr\u1ee9ng c\u00e1 b\u1ecdc ho\u1eb7c vi\u00eam tuy\u1ebfn m\u1ed3 h\u00f4i m\u1ee7.<br>C\u00e1c th\u1ec3 kh\u00e1c nhau c\u1ee7a SM l\u00e0 th\u1ec3 khu tr\u00fa, th\u1ec3 lan t\u1ecfa, th\u1ec3 vi\u00eam m\u1ee7, th\u1ec3 \u0111\u1ea7u c\u1ef1c, th\u1ec3 \u1edf m\u1eb7t.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Ch\u1ea9n \u0111o\u00e1n b\u1ec7nh<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Ch\u1ea9n \u0111o\u00e1n x\u00e1c \u0111\u1ecbnh ch\u1ee7 y\u1ebfu d\u1ef1a v\u00e0o \u0111\u1eb7c \u0111i\u1ec3m th\u01b0\u01a1ng t\u1ed5n da nh\u01b0 \u0111\u00e3 m\u00f4 t\u1ea3 \u1edf tr\u00ean, tu\u1ed5i kh\u1edfi ph\u00e1t. C\u00f3 th\u1ec3 l\u00e0m sinh thi\u1ebft \u0111\u1ec3 kh\u1eb3ng \u0111\u1ecbnh ch\u1ea9n \u0111o\u00e1n. Tr\u00ean h\u00ecnh \u1ea3nh m\u00f4 b\u1ec7nh h\u1ecdc c\u00f3 th\u1ec3 th\u1ea5y c\u00e1c th\u01b0\u01a1ng t\u1ed5n n\u1eb1m gi\u1eefa trung b\u00ec, c\u00e1c tuy\u1ebfn b\u00e3 c\u00f3 th\u00e0nh m\u1ecfng, g\u1ee3n s\u00f3ng, vi\u1ec1n quanh b\u1edfi l\u1edbp bi\u1ec3u m\u00f4 v\u1ea3y, nhi\u1ec1u b\u1ea1ch c\u1ea7u \u00e1i toan \u1edf b\u1ec1 m\u1eb7t, kh\u00f4ng c\u00f3 l\u1edbp h\u1ea1t, c\u00e1c th\u00f9y m\u1ee1 b\u00ean trong l\u1edbn, k\u00edch th\u01b0\u1edbc \u0111a d\u1ea1ng; c\u00e1c k\u00e9n ch\u1ee9a ch\u1ea5t s\u1eebng, ch\u1ea5t b\u00e9o, c\u00e1c s\u1ee3i l\u00f4ng t\u01a1. M\u1ed7i k\u00e9n n\u1ed1i v\u1edbi th\u01b0\u1ee3ng b\u00ec b\u00ecnh th\u01b0\u1eddng b\u1edfi s\u1ee3i d\u00e2y m\u1ea3nh c\u1ee7a c\u00e1c t\u1ebf b\u00e0o bi\u1ec3u m\u00f4 kh\u00f4ng bi\u1ec7t h\u00f3a.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">B\u1ec7nh kh\u00f4ng kh\u00f3 ch\u1ea9n \u0111o\u00e1n nh\u01b0ng trong th\u1ec3 khu tr\u00fa c\u1ea7n ch\u1ea9n \u0111o\u00e1n ph\u00e2n bi\u1ec7t v\u1edbi tr\u1ee9ng c\u00e1, qu\u00e1 s\u1ea3n tuy\u1ebfn b\u00e3.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>\u0110i\u1ec1u tr\u1ecb<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Hi\u1ec7n nay ch\u01b0a c\u00f3 thu\u1ed1c \u0111i\u1ec1u tr\u1ecb kh\u1ecfi b\u1ec7nh. C\u00e1c bi\u1ec7n ph\u00e1p \u0111i\u1ec1u tr\u1ecb mang t\u00ednh ch\u1ea5t lo\u1ea1i b\u1ecf th\u01b0\u01a1ng t\u1ed5n, h\u1ea1n ch\u1ebf vi\u00eam, b\u1ed9i nhi\u1ec5m \u0111\u1ec3 tr\u00e1nh s\u1eb9o g\u00e2y m\u1ea5t th\u1ea9m m\u1ef9.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>C\u1eaft b\u1ecf c\u00e1c k\u00e9n l\u1edbn b\u1eb1ng ph\u1eabu thu\u1eadt, laser, \u0111\u1ed1t \u0111i\u1ec7n, \u00e1p l\u1ea1nh.<\/li><li>S\u1eed d\u1ee5ng kh\u00e1ng sinh n\u1ebfu c\u00f3 vi\u00eam, nhi\u1ec5m khu\u1ea9n.<\/li><li>Isotretinoin \u0111\u01b0\u1eddng u\u1ed1ng l\u00e0m gi\u1ea3m t\u00ecnh tr\u1ea1ng b\u1ec7nh, gi\u1ea3m vi\u00eam t\u1ea1m th\u1eddi.<\/li><li>Ngo\u00e0i ra c\u1ea7n t\u01b0 v\u1ea5n di truy\u1ec1n, h\u1ed7 tr\u1ee3 t\u00e2m l\u00fd cho ng\u01b0\u1eddi b\u1ec7nh.<\/li><\/ul>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>M\u1ed9t s\u1ed1 h\u00ecnh \u1ea3nh l\u00e2m s\u00e0ng c\u1ee7a b\u1ec7nh<\/strong><\/p>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"740\" height=\"555\" src=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image001.jpg\" alt=\"\" class=\"wp-image-671\" srcset=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image001.jpg 740w, https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image001-300x225.jpg 300w\" sizes=\"auto, (max-width: 740px) 100vw, 740px\" \/><figcaption><strong>\u1ea2nh 1. SM khu tr\u00fa \u1edf ng\u1ef1c \u1edf b\u1ec7nh nh\u00e2n nam 27 tu\u1ed5i, b\u1ec7nh b\u1eaft \u0111\u1ea7u t\u1eeb n\u0103m 18 tu\u1ed5i. M\u1ed9t s\u1ed1 th\u01b0\u01a1ng t\u1ed5n b\u1ed9i nhi\u1ec5m, khi l\u00e0nh \u0111\u1ec3 l\u1ea1i s\u1eb9o. Xung quanh l\u00e0 c\u00e1c k\u00e9n tuy\u1ebfn b\u00e3 1-2 mm, m\u00e0u tr\u1eafng, v\u00e0ng, s\u1edd \u0111\u01b0\u1ee3c\u00a0<em>(\u1ea3nh: BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng)<\/em><\/strong><\/figcaption><\/figure><\/div>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"731\" height=\"548\" src=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image003.jpg\" alt=\"\" class=\"wp-image-672\" srcset=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image003.jpg 731w, https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image003-300x225.jpg 300w\" sizes=\"auto, (max-width: 731px) 100vw, 731px\" \/><figcaption><strong>\u1ea2nh 2. SM khu tr\u00fa \u1edf ng\u1ef1c, b\u1ee5ng \u1edf b\u1ec7nh nh\u00e2n n\u1eef 25 tu\u1ed5i, b\u1ec7nh b\u1eaft \u0111\u1ea7u t\u1eeb n\u0103m 16 tu\u1ed5i. C\u00f3 m\u1ed9t k\u00e9n l\u1edbn 3-4 mm, xung quanh l\u00e0 c\u00e1c k\u00e9n nh\u1ecf, 1-2 mm\u00a0<em>(\u1ea3nh: BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng)<\/em><\/strong><\/figcaption><\/figure><\/div>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"720\" height=\"960\" src=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image005.jpg\" alt=\"\" class=\"wp-image-673\" srcset=\"https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image005.jpg 720w, https:\/\/cdha.edu.vn\/cdha\/wp-content\/uploads\/2021\/06\/image005-225x300.jpg 225w\" sizes=\"auto, (max-width: 720px) 100vw, 720px\" \/><figcaption><strong>\u1ea2nh 3. SM lan t\u1ecfa, m\u1eadt \u0111\u1ed9 d\u00e0y \u0111\u1eb7c \u1edf b\u1ec7nh nh\u00e2n nam 28 tu\u1ed5i<\/strong>\u00a0<strong><em>(\u1ea3nh: BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng)<\/em><\/strong><br><strong>B\u00e0i v\u00e0 \u1ea3nh: BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ed9 m\u00f4n Da li\u1ec5u, \u0110\u1ea1i h\u1ecdc Y H\u00e0 N\u1ed9i, Khoa D2, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng.\u00a0<\/strong><\/figcaption><\/figure><\/div>\n\n\n\n<p class=\"wp-block-paragraph\"><\/p>\n","protected":false},"excerpt":{"rendered":"<p>BS. Tr\u1ea7n Th\u1ecb Huy\u1ec1n, B\u1ed9 m\u00f4n Da li\u1ec5u, \u0110\u1ea1i h\u1ecdc Y H\u00e0 N\u1ed9i, Khoa D2, B\u1ec7nh vi\u1ec7n Da li\u1ec5u Trung \u01b0\u01a1ng.\u00a0Link b\u00e0i g\u1ed1c \u0110\u1ecbnh ngh\u0129a<\/p>\n","protected":false},"author":1,"featured_media":671,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[2],"tags":[69,70],"class_list":["post-670","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-doc-sach-cho-vui","tag-da-lieu","tag-steatocystoma-multiplex"],"_links":{"self":[{"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/posts\/670","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/comments?post=670"}],"version-history":[{"count":1,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/posts\/670\/revisions"}],"predecessor-version":[{"id":674,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/posts\/670\/revisions\/674"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/media\/671"}],"wp:attachment":[{"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/media?parent=670"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/categories?post=670"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/cdha.edu.vn\/cdha\/wp-json\/wp\/v2\/tags?post=670"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}