Syndrome pachimake akuti: Zoyambitsa, Zizindikiro ndi Chithandizo

Pachimake matenda - ndi chosowa kobadwa nako matenda, mmene maselo a anthu kuonetsa kutengera mapangidwe Kusakhazikika. Iwo tinatengera mu autosomal unsembe recessive.

Kulakwila kumene anapezeka ndi anafotokoza mu 1954 ndi dermatologist American chiyambi Devid Blum. M'malo mwa wasayansi ndi linali dzina la matenda. Lofanana - kobadwa nako teleangiektaticheskaya erythema.

syndrome pachimake kwambiri ambiri amakhudza anthu a mtundu wachiyuda (1 mwa 100 anthu). Matendawa akhoza kukhala amuna ndi akazi, koma zizindikiro masika ndi yapadera kwambiri. Ichi ndi chifukwa chake akazi ndi matenda nthawi zambiri sazindikira msanga.

A mwana amene syndrome odwala pachimake, makolo ali ndi kusintha zobisika mu allele mmodzi wa jini BLM. Amaganiza kuti zizindikiro zosiyanasiyana zimadalira wotani kusintha chili mwa chibadwa wodwalayo. Komabe, si koma kutsimikiziridwa.

chithunzi matenda

Odwala matenda pachimake pa kubadwa ndi kulemera pang'ono (pafupifupi 1900-2000 g). M'tsogolo, iwonso akuchedwa kukula ndi bwino kupeza kulemera. Unamwali anachedwa, ndipo ngakhale chitadutsa, ndi zosalongosoka. Mu amuna, osabereka Sizachilendo, ndi akazi - abnormally oyambirira kusintha. Ngakhale izi, chitukuko maganizo limafanana ndi malamulo zaka.

Mu masabata loyamba la moyo la odwala pa masaya, makutu, mphuno ndi kumbuyo kwa manja muli thovu, erythema ndi kutumphuka. Nthawi zambiri pali kuchuluka kudziwa UV kuwala. Ngakhale yochepa kupeza odwala pansi dzuwa kungachititse kuti mapangidwe mtima maukonde ndi kuwonongeka kwa khungu la Kusinthasintha mwamphamvu. Pambuyo ayambe khungu irradiated pa izo akhoza kupanga mawanga kwambiri mdima kapena kuwala malo ndi manja.

Odwala ndi chitetezo chokwanira maola ndipo motero zambiri anakumana ndi matenda opatsirana, amenenso anawathetsa akuyambiranso.

Pachimake syndrome amaganizira ndi asymmetry ya makosi a ntchafu fupa ndi obadwa nawo a mtima matenda.

maonekedwe

Maonekedwe odwala zachilendo. Ali m'malo yopapatiza Chigaza, ang'ono chibwano ndi otchuka mphuno ( "mbalame nkhope"). Izi zimaonekera makamaka ngati inu muyang'ana pa chithunzi cha matenda pachimake.

Odwala zambiri otsika kukula, ndi mawu mkulu. Odwala ena, pali deformities phazi ndi kuvutika kwa mano. Odwala Kawirikawiri kutupa ndi kutupa kwa milomo, ukuchoka iwo. Nthawi zina, pali kuphwanya dongosolo la keratinization wa khungu ndi blockage ake (zikuwoneka ngati "tokhala tsekwe").

diagnostics

Matendawa "pachimake Syndrome" amaponya dokotala, zochokera chithunzi matenda a zotsatira wodwalayo kuchipatala deta ndiponso zasayansi.

Kuzionetsetsa koyenera kuona udindo wa chitetezo cha m'thupi. Mu Khadili odwala matenda pachimake ndi Amowabu kuchepetsa kuchuluka kwa immunoglobulin ndi T-lymphocytes. Komanso, tikulimbikitsidwa kuti amayang'anitsitsa kuphana mlongo chromatid.

Pamene matenda n'kofunika kuti tisasokoneze ndi pachimake syndrome lupus erythematosus syndrome Nile-Dingwall syndrome Rotmunda-Thomson ndi cutaneous porphyria.

Oncology

Low chitetezo chokwanira ndi kukhalapo kwa ambiri masinthidwe osiyana kutsogolera chakuti wodwala kwambiri kumachititsa khansa. Lolani nthawi yomweyo ukhoza kukhala ziwalo ndi magazi, lymph, fupa minofu.

The pathologies ambiri kunachitika odwalawo, ndi:

• myeloid khansa;
• lymphoma;
• lymphocytic khansa;
• Zilonda zotupa za kum'mero, lilime, ndi matumbo;
• m'mapapo khansara;
• carcinoma ya bere.

Zambiri zokayikitsa kuti matenda medulloblastoma ndi khansa impso.

chithandizo

A mtima mavuto pachimake matenda, mankhwala adzakhala symptomatic. Kuchepetsa zovuta zotsatira zosasangalatsa chingapezeke ndi thandizo la mankhwala ndi chamankhwala. Kusankha awo opangidwa ndi dokotala kupezeka pa maziko a zimene zizindikiro kusokoneza mtima. Mwachitsanzo, mu Oncology angafunike Morning, cheza mankhwala kapena opaleshoni, ndi matenda mano - .. pochotsedwa mano, etc. Chiritsani kwathunthu ndi kudwala si panopa zotheka.

Mulimonsemo, odwala nthawi zonse tiyenera kugwiritsa ntchito njira kuti molondola kuteteza khungu ku cheza ultraviolet, ntchito vitamini ndi mchere zowonjezera (iwo ayenera kukhala mbali ya vitamini E), carotenoids (monga mwa mawonekedwe a chakudya zina, ndipo chakudya) ndi mankhwala, kukonza ntchito ndi chitetezo. Nthawi zina, akhoza kuchititsa mankhwala m'thupi.

Odwala amene pachimake matenda, kuphunzira moyo wonse kuzioneka ndi dermatologist ndi oncologist kudziwa zizindikiro zoyambirira za khansa ya pakhungu. Ngati kusintha kulikonse Muzisamala zofunika nthawi yayifupi zotheka kuwona dotolo.

Odwala, pa thupi pamene gawo la timadontho-timadontho, m'pofunika kukhala mthunzi ndi kupewa dzuwa, amavala zovala ngati kuli kotheka kubisala thupi.

kaonedwe

Kuti madokotala ananena zawo odwala ndi matenda pachimake kumadalira pa lotani pathological nawo limodzi. Nthawi zambiri, odwala kufa chifukwa cha njira pachimake oncological kapena chibayo.

Tikumbukenso kuti odwala amene mankhwala symptomatic ndipo kuyang'aniridwa ndi dokotala, ali wamkulu moyo amayembekezeka poyerekezera ndi odwala amene satero.

kupewa

syndrome Kuteteza pachimake pa ana MUKACHULUKIDWE kupewa ukwati ndi achibale. Ndi mwa amitundu, mu mwambo umene mabanja yogwirizana, nthendayo kaŵirikaŵiri kufalikira.

Komanso, achinyamatawo pamaso lingaliro tikulimbikitsidwa kuti alandire wokwanira. zachipatala bwino.