ichthyosis

Chidule cha ichthyosis

Ichthyosis ndi gulu la zikhalidwe zapakhungu zomwe zimapangitsa khungu louma, loyabwa lomwe limawoneka losalala, lolimba komanso lofiira. Zizindikiro zimatha kukhala zofatsa mpaka zovuta kwambiri. Ichthyosis imatha kukhudza khungu kokha, koma mitundu ina ya matendawa imatha kukhudzanso ziwalo zamkati.

Anthu ambiri amatengera ichthyosis kuchokera kwa makolo awo kudzera mu jini yosinthika (yosinthidwa). Komabe, anthu ena amapanga mawonekedwe a ichthyosis (osati chibadwa) chifukwa cha matenda ena kapena mankhwala ena. Ngakhale kuti pakali pano palibe mankhwala a ichthyosis, kafukufuku akupitirira ndipo mankhwala alipo kuti athetse zizindikiro.

Malingaliro a anthu omwe ali ndi ichthyosis amasiyana malinga ndi mtundu wa matenda ndi kuopsa kwake. Anthu ambiri omwe ali ndi ichthyosis amafunikira chithandizo chamoyo wonse kuti matendawa athe kutheka.

Ndani amatenga ichthyosis? 

Aliyense akhoza kutenga ichthyosis. Matendawa nthawi zambiri amafalitsidwa kuchokera kwa makolo; komabe, anthu ena angakhale oyamba m’banja kukhala ndi ichthyosis chifukwa cha kusintha kwa jini kwatsopano. Anthu ena amapanga mawonekedwe (osakhala a chibadwa) a ichthyosis, omwe amayamba chifukwa cha matenda ena kapena zotsatira za mankhwala.

Mitundu ya ichthyosis

Pali zizindikiro zoposa 20 za ichthyosis, kuphatikizapo zomwe zimachitika ngati gawo la matenda ena kapena chikhalidwe. Madokotala amatha kudziwa mtundu wa ichthyosis pofufuza:

• Kusintha kwa gene.
• Chitsanzo cha cholowa posanthula mitengo ya mabanja.
• Zizindikiro, kuphatikizapo kuuma kwawo komanso ziwalo zomwe zimakhudza.
• Zaka pamene zizindikiro zinayamba kuonekera.

Mitundu ina ya matenda omwe amatengera kwa makolo ndipo sali mbali ya matendawa ndi awa:

• Ichthyosis vulgaris ndi mtundu wofala kwambiri. Nthawi zambiri amapita mofatsa ndipo amawonekera m'chaka choyamba cha moyo ndi khungu louma, lophwanyika.
• Harlequin ichthyosis nthawi zambiri imawoneka pa kubadwa ndipo imayambitsa masamba okhuthala akhungu kuti aphimbe thupi lonse. Matendawa amatha kusokoneza mawonekedwe a nkhope ndikuchepetsa kuyenda kwamagulu.
• Epidermolytic ichthyosis ilipo kuyambira kubadwa. Ana ambiri amabadwa ndi khungu losalimba komanso matuza ophimba matupi awo. Pakapita nthawi, matuza amatha, ndipo peeling imawonekera pakhungu. Itha kukhala ndi nthiti pamagawo amthupi omwe amapindika.
• Lamellar ichthyosis alipo kuyambira kubadwa. Mwana amabadwa ndi nembanemba yolimba, yowoneka bwino yomwe imaphimba thupi lonse yotchedwa collodion membrane. M’milungu yochepa chabe, nembanembayo imang’ambika, ndipo mamba akuluakulu akuda kwambiri amaonekera m’thupi lonse.
• Congenital ichthyosiform erythroderma ilipo pakubadwa. Ananso nthawi zambiri amakhala ndi nembanemba ya collodion.
• Ichthyosis yolumikizidwa ndi X nthawi zambiri imayamba mwa anyamata ndipo imayamba pafupifupi miyezi itatu mpaka 3 yakubadwa. Peeling amapezeka pakhosi, m'munsi nkhope, torso, ndi miyendo, ndipo zizindikiro zimatha kuipiraipira pakapita nthawi.
• Erythrokeratoderma yosinthika nthawi zambiri imayamba miyezi ingapo mutabadwa ndipo imakula ali mwana. Zigamba zapakhungu zolimba, zokhuthala, kapena zofiira zimatha kuwoneka pakhungu, nthawi zambiri kumaso, matako, kapena malekezero. Madera okhudzidwawo amatha kufalikira pakhungu pakapita nthawi.
• Progressive symmetric erythrokeratoderma nthawi zambiri imakhala ndi khungu louma, lofiyira, lotupa makamaka malekezero, matako, kumaso, akakolo, ndi manja.

Zizindikiro za ichthyosis

Zizindikiro za ichthyosis zimatha kukhala zofatsa mpaka zovuta. Zizindikiro zodziwika kwambiri ndi izi:

• Khungu louma.
• Kuyabwa
• Kufiira kwa khungu.
• Kusweka kwa khungu.
• Mamba pakhungu loyera, imvi kapena bulauni ali ndi mawonekedwe awa:
  • Chaching'ono ndi chophwanyika.
  • Akuluakulu, akuda, mamba a lamellar.
  • Mamba olimba, ngati zida.

Malingana ndi mtundu wa ichthyosis, zizindikiro zina zingaphatikizepo:

• Matuza omwe amatha kuphulika, zomwe zimapangitsa mabala.
• Kutha tsitsi kapena kuwonongeka.
• Kuwuma kwa maso komanso zovuta kutseka zikope. Kulephera kutuluka thukuta (kutuluka thukuta) chifukwa zotupa pakhungu zimatsekereza zopangitsa thukuta.
• Kuvutika kumva.
• Kukhuthala kwa khungu m'manja ndi m'miyendo.
• Khungu kumangitsa.
• Kuvuta kupindika mafupa ena.
• Tsegulani mabala chifukwa choyabwa pakhungu.

Chifukwa cha ichthyosis

Kusintha kwa majini (kusintha) kumayambitsa mitundu yonse ya cholowa cha ichthyosis. Kusintha kwa majini ambiri kwadziwika, ndipo chikhalidwe cha cholowa chimadalira mtundu wa ichthyosis. Anthu akumakula khungu latsopano nthawi zonse ndikuchotsa khungu lakale m'moyo wawo wonse. Kwa anthu omwe ali ndi ichthyosis, majini osinthika amasintha kakulidwe kabwino ka khungu ndi kutsetsereka kwa khungu, zomwe zimapangitsa maselo a khungu kuchita chimodzi mwa izi:

• Amakula mofulumira kuposa momwe amagwetsera.
• Amakula pamlingo wabwinobwino koma amakhetsa pang'onopang'ono.
• Amakhetsa mwachangu kuposa momwe amakulira.

Pali mitundu yosiyanasiyana ya cholowa cha ichthyosis, kuphatikizapo:

• Dominant, zomwe zikutanthauza kuti mumalandira kopi imodzi yabwinobwino komanso jini losinthika lomwe limayambitsa ichthyosis. Kope losazolowereka la jini limakhala lamphamvu kapena "likulamulira" mawonekedwe abwinobwino a jini, zomwe zimayambitsa matenda. Munthu amene ali ndi kusintha kwakukulu amakhala ndi mwayi wa 50% (1 mwa 2) wopatsira matendawa kwa mwana aliyense.
• Zowonjezereka, zomwe zikutanthauza kuti makolo anu alibe zizindikiro za ichthyosis, koma makolo onse amanyamula jini imodzi yokha yachilendo, yomwe siili yokwanira kuyambitsa matendawa. Makolo onse akakhala ndi jini yokhazikika yofanana, pamakhala mwayi wa 25% (1 mwa 4) pamimba iliyonse yokhala ndi mwana yemwe adzalandira majini osinthika onsewa ndikudwala matendawa. Pali mwayi wa 50% (2 mwa 4) woti mimba ikhale ndi mwana yemwe adzalandira jini imodzi yokha yosinthika, zomwe zimamupangitsa kukhala chonyamulira cha jini ya matendawa popanda zizindikiro zowonekera. Ngati kholo limodzi liri ndi mawonekedwe a ichthyosis omwe ali ndi majini awiri osinthika, ana awo onse amanyamula jini imodzi yachilendo koma nthawi zambiri sadzakhala ndi zizindikiro za ichthyosis.
• X-olumikizidwa, zomwe zikutanthauza kuti masinthidwe amtundu ali pa X sex chromosome. Munthu aliyense ali ndi ma chromosome awiri ogonana: akazi nthawi zambiri amakhala ndi ma X chromosome (XX) ndipo amuna amakhala ndi X chromosome imodzi ndi Y chromosome (XY). Mayi nthawi zonse amadutsa X chromosome, pamene abambo amatha kupatsira X kapena Y chromosome. Njira ya cholowa cha X-yolumikizidwa ichthyosis nthawi zambiri imakhala yochulukirapo; Izi zikutanthauza kuti amuna omwe poyamba amakhala ndi X chromosome imodzi amadutsa X chromosome yosinthidwa. Chifukwa cha kachitidwe kameneka, akazi amakhudzidwa kaŵirikaŵiri, ndipo nthaŵi zambiri amakhala ndi X chromosome yosinthidwa ndi yachibadwa.
• Modzidzimutsa, zomwe zikutanthauza kuti kusintha kwa jini kumachitika mwachisawawa popanda mbiri yabanja ya matendawa.