Kodi mwana wanu wayamba kutupa pang'ono pathupi lake? Kapena kutupa pakhungu kapena kutupa komwe sikuchira? Nthawi zina izi zimakhala zachilendo, koma nthawi zambiri zimakhala zizindikiro za matenda osowa otchedwa Langerhans Cell Histiocytosis (LCH). Ndizachilendo kuchita mantha mukamva dzinali, chifukwa si chinthu chomwe timachizolowera kumva. Koma musadandaule, tiyeni tikambirane mwachidule.
Mwachidule, kodi LCH ndi chiyani?
Taganizirani za thupi lathu ngati dziko lalikulu. Pali gulu lankhondo loteteza dziko lino, ndipo ndilo chitetezo chathu chamthupi . Msilikali wapadera m'gululi amatchedwa maselo a Langerhans . Maselo amenewa ndi mtundu wa maselo oyera amagazi. Ntchito yawo yayikulu ndikulimbana ndi majeremusi ndi matenda omwe amalowa m'thupi lathu ndikutiteteza ku matenda. Asilikali amenewa amapezeka m'thupi lathu lonse, makamaka pakhungu, m'mapapo, m'malo olumikizirana mafupa, m'mafupa, m'ndulu, ndi m'chiwindi.
Koma pankhani ya LCH, maselo a Langerhans awa amachulukana mosalamulirika ndipo amasonkhana m'malo osiyanasiyana a thupi. Zili ngati gulu la asilikali omwe akungoyima pamalo amodzi popanda kumenyana. Akamasonkhana motere, amayamba kuwononga minofu yathanzi m'malo amenewo. Mabala amapangika m'malo amenewo.
Nkhani yabwino yokhudza vutoli ndi yakuti ana ambiri amatha kuchira kwathunthu ku matendawa ngati atalandira chithandizo choyenera. Nthawi zina, makamaka ngati zimakhudza khungu lokha, zimatha zokha popanda chithandizo chilichonse. Komabe, ngati maselowa akhudza ziwalo zofunika kwambiri monga mafupa, ndulu kapena chiwindi, chithandizo champhamvu chingafunike.
Kodi khansa ya LCH ndi yotani?
Funso limene anthu ambiri ali nalo ndi ili. Ndipotu, madokotala akadali kusagwirizana pang'ono pankhaniyi. Ofufuza ambiri amaona kuti LCH ndi vuto lofanana ndi khansa, kutanthauza kuti, chotupa . Izi zikutanthauza kukula kosazolowereka kwa maselo. Koma ena amaganiza kuti ndi matenda otupa a chitetezo chamthupi.
Komabe, LCH imachiritsidwa ndi madokotala a khansa, omwe ndi akatswiri pa matenda a khansa ndi magazi. Nthawi zina, mankhwala oletsa khansa monga chemotherapy amagwiritsidwanso ntchito pochiza.
Ndani amene ali ndi mwayi waukulu wopeza matendawa?
LCH imapezeka kwambiri mwa makanda obadwa kumene ndi ana azaka zapakati pa 1 ndi 15. Akuluakulu ndi osowa kwambiri, koma sizingatheke.
Malinga ndi ziwerengero, mwana mmodzi kapena awiri okha mwa ana obadwa kumene miliyoni iliyonse amabadwa ndi matendawa chaka chilichonse. Izi zikutanthauza kuti ndi vuto losowa kwambiri .
Kodi zizindikiro za LCH ndi ziti?
Matenda a LCH sakhudza ana onse mofanana. Ngakhale ana ena angakhudzidwe ndi gawo limodzi lokha la thupi lawo, ena angakhudzidwe ndi madera angapo a thupi lawo. Chifukwa chake, zizindikiro zimasiyana malinga ndi gawo la thupi lomwe lakhudzidwa. Tiyeni tiwone zomwe zili.
| Chiwalo cha thupi chomwe chakhudzidwa | Zizindikiro zomwe zingawonekere |
|---|---|
| Mafupa | LCH imakhudza mafupa mwa odwala pafupifupi 80%. Chotupa kapena kutupa kungawonekere m'malo monga chigaza, mafupa ozungulira maso, kumbuyo kwa makutu, fupa la nsagwada, miyendo, msana, ndi chiuno. Ululu ukhoza kukhalapo kapena kusakhalapo. Kuphatikiza apo, mutu, kupweteka kwa khosi/msana, kuvutika kuyenda, ndi kufooka kungawonekere. |
| Khungu | Matenda ofala kwambiri ndi kutupa pakhungu. Chivundikiro cha mwana chingakhale vuto losachira lomwe limawonekera pamutu pa mwana. Mabala ofiira komanso otupa amatha kuwoneka pa khosi, m'khwapa, m'mimba, pachifuwa, ndi kumbuyo. Izi zitha kutuluka madzi ndikuyabwa kapena kupweteka. Misomali ingasinthe mtundu kapena kugwa. |
| Pakamwa | Mano omasuka kapena omasuka, mano obwerera m'mbuyo, mkamwa wotupa, zilonda pamilomo, lilime, mkati mwa masaya, kapena padenga la pakamwa. |
| Chiwindi kapena Nkhumba | Kutupa kwa chiwindi kapena ndulu kungayambitse kutupa m'mimba, chikasu cha maso ndi khungu (jaundice), kuyabwa, kutopa kwambiri, komanso kuvulala kapena kutuluka magazi mosavuta. |
| Mafuta a Bone | Kuchepa kwa magazi m'thupi, kufooka, kutopa, komanso kusowa chilakolako cha chakudya chifukwa cha kuchepa kwa maselo ofiira a m'magazi. Matenda opatsirana pafupipafupi komanso kutentha thupi chifukwa cha kuchepa kwa maselo oyera a m'magazi. Kutupa kosavuta chifukwa cha kuchepa kwa ma platelet. |
| Dongosolo la Endocrine (Dongosolo la Endocrine - Mahomoni) | Ngati pituitary gland yakhudzidwa: ludzu lochuluka ndi kukodza pafupipafupi (shuga insipidus), kukula movutikira, kutha msinkhu msanga kapena kuchedwa, kunenepa. Ngati chithokomiro chakhudzidwa: kutupa kwa chithokomiro, kuvutika kupuma. |
| Makutu | Matenda a m'khutu pafupipafupi, kutuluka magazi m'khutu, kufiira kwa khutu, kuyabwa m'khutu, kupweteka m'khutu, komanso kutaya kumva. |
| Maso | Maso otupa, kutupa pamwamba pa maso, kulephera kuona bwino. |
| Ma Lymph Nodes | Kutupa ndi kupweteka kwa ziphuphu (zotupa) m'khosi, m'khwapa, kapena m'mimba. |
| Dongosolo Lapakati Lamitsempha | Mutu, chizungulire, kusanza, kuvutika kuyenda, kulephera kukhazikika bwino (ataxia), kuvutika kulankhula kapena kuona, khunyu, kusintha kwa khalidwe ndi kukumbukira. |
| Mapapu | Izi zimachitika kwambiri pakati pa akuluakulu, makamaka osuta fodya. Kupweteka pachifuwa, chifuwa chouma, kupuma movutikira, kufooka kwa mapapo (pneumothorax). |
| Njira Yopangira Mimba | Kupweteka m'mimba, kusanza, kutsegula m'mimba, magazi m'chimbudzi, komanso kusakula bwino chifukwa cha kusowa zakudya m'thupi. |
Chofunika kwambiri ndichakuti zizindikirozi zitha kuwonekanso m'matenda ena ambiri ofala. Choncho musaope LCH chifukwa chakuti muli ndi chimodzi kapena ziwiri mwa zizindikirozi. Koma ngati zizindikirozi zikupitirira, ndikofunikira kwambiri kuonana ndi dokotala kuti mudziwe bwino matendawa.
Kodi chifukwa chenicheni cha LCH ndi chiyani?
Mwachidule, maselo athu ali ndi majini omwe amawauza kuti "agawani tsopano, siyani tsopano." Pafupifupi theka la ana omwe ali ndi LCH ali ndi kusintha pang'ono, kapena kusintha, mu jini yawo yotchedwa `BRAF .' Jini iyi imapanga puloteni yomwe imayang'anira kukula kwa maselo. Chifukwa cha kusintha kumeneku, puloteniyo silandira lamulo loti "siyani kugawa." Zili ngati kuti 'pa' switch yatsekedwa. Choncho maselo a Langerhans amapitiriza kugawa ndi kusakanikirana.
Ichi si chinthu chomwe chimabadwa kuchokera kwa makolo kupita kwa ana. Kusintha kwa majini kumeneku kumachitika mwachisawawa mwana akangobadwa m'mimba mwa mayi ake.
Kuwonjezera pa jini ya `BRAF`, ofufuza apeza kuti matendawa amathanso kuyambitsidwa ndi kusintha kwa majini ena monga `MAP2K1`, `RAS`, ndi `ARAF`.
Kodi mwapeza bwanji izi, Dokotala?
Mukapita ndi mwana wanu kwa dokotala, iye adzakufunsani kaye za zizindikiro za mwana wanu. Kenako, adzamuyang'ana mwana wanu mosamala. Popeza LCH ingakhudze ziwalo zosiyanasiyana za thupi, mayeso angapo angafunike kuti atsimikizire matendawa.
| Mtundu wa mayeso | Mwachidule... |
|---|---|
| Mayeso a Magazi | Kuwerengera magazi konse (CBC) kumayesa kuchuluka kwa maselo ofiira, maselo oyera, ndi ma platelet m'magazi. Kuyezetsa magazi kumachitikanso kuti aone ngati chiwindi chikugwira ntchito bwino. |
| Kufufuza za thupi | Iyi ndiyo njira yotsimikizika kwambiri yotsimikizira LCH.Kachidutswa kakang'ono ka minofu kamatengedwa kuchokera ku chotupa kapena chotupacho pansi pa opaleshoni yoletsa ululu ndikuwunikidwa pogwiritsa ntchito maikulosikopu kuti atsimikizire ngati maselo a LCH alipo. Ngati pali kukayikiridwa kuti mafupa akhudzidwa, kuyezetsa mafupa kungachitikenso. |
| Kuyesa Majini | Chitsanzo cha minofu yotengedwa kuchokera ku biopsy chimagwiritsidwa ntchito kuyesa kusintha kwa majini a `BRAF`. |
| Mayeso Ojambula | Mayeso monga X-ray, CT scans, MRI scans, ndi PET scans amatha kudziwa momwe LCH imakhudzira ziwalo zamkati monga mafupa, ubongo, ndi mapapo. |
Kutengera ndi zotsatira za mayesowa, dokotala wanu adzatumiza mwana wanu kwa dokotala wa matenda a magazi/oncologist wa ana.
Kodi mankhwala a LCH ndi ati?
Si ana onse omwe ali ndi matenda a LCH omwe amafunika chithandizo chofanana. Chithandizo chimadalira malo ndi kuopsa kwa matendawa.
Madokotala amagawa LCH m'magulu awiri akuluakulu:
1. LCH ya dongosolo limodzi: Matendawa amakhudza chiwalo chimodzi chokha m'thupi (monga mafupa okha kapena khungu lokha).
2. Kutupa kwa ziwalo za m'thupi (multi-system LCH): Matendawa amakhudza ziwalo ziwiri kapena zingapo za thupi (monga khungu ndi chiwindi).
Komanso, ziwalo monga chiwindi, ndulu, ndi mafuta am'mafupa zimaonedwa kuti ndi ziwalo "zoopsa kwambiri", pomwe ziwalo monga khungu, mafupa, ndi mapapo zimaonedwa kuti ndi "zoopsa zochepa." Gulu ili limapanga dongosolo la chithandizo.
Pali njira zingapo zazikulu zochizira:
- Mankhwala a steroid: Makamaka khungu lokha likakhudzidwa, mankhwala a steroid monga prednisone amagwiritsidwa ntchito ngati mapiritsi kapena mafuta odzola.
- Opaleshoni: Opaleshoni monga curettage imachitika kuti ichotse chotupa cha LCH m'fupa.
- Chemotherapy: Uwu ndi mtundu wa mankhwala omwe amaperekedwa kuti aphe maselo a khansa. Chifukwa chakuti maselo a LCH amagawikana mofulumira, mankhwalawa amawaletsa kukula. Mankhwalawa angaperekedwe ngati mapiritsi, jakisoni, kapena ngati kirimu wopaka pakhungu.
- Chithandizo cha radiation: Chimagwiritsa ntchito ma radiation amphamvu kwambiri kuti chiwononge maselo a LCH. Izi sizikugwiritsidwa ntchito kawirikawiri.
- Chithandizo cholunjika:Kwa ana omwe ali ndi kusintha kwa majini a `BRAF`, pali mankhwala (BRAF inhibitors) omwe amalimbana ndi kusinthako. Mankhwalawa sawononga maselo athanzi kwambiri.
- Chithandizo cha Immunotherapy: Kulimbikitsa chitetezo cha mthupi cha mwana kuti chizimenyana ndi maselo a LCH.
- Kusamutsa maselo oyambira: Njira yothandizira yomwe imaganiziridwa pazochitika zoopsa kwambiri zomwe sizingachiritsidwe ndi mankhwala ena.
Kodi zinthu zili bwanji mutalandira chithandizo?
Kuyezetsa kwa matenda a LCH kumadalira zinthu zingapo, kuphatikizapo ziwalo za thupi zomwe matendawa akhudza, momwe afalikira, komanso momwe amayankhira bwino akalandira chithandizo.
- Chiŵerengero cha machiritso a ana omwe ali ndi ziwalo zochepa zokha (zonse za dongosolo limodzi ndi zambiri) ndi pafupifupi 100% . Izi zikutanthauza kuti palibe chiopsezo cha imfa. Komabe, pali chiopsezo chobwereranso kapena mavuto ena a nthawi yayitali.
- Matenda a ana omwe ziwalo zawo "zoopsa" monga chiwindi, ndulu, ndi mafupa zimakhudzidwa angakhale oopsa kwambiri.
Choncho, ndikofunikira kwambiri kukaonana ndi dokotala kwa zaka zambiri, ngakhale chithandizo chitatha. Muyenera kufufuzidwa nthawi zonse kuti muwone ngati matendawa abwereranso.
Uthenga Wopita Nawo Kunyumba
- Matenda a Langerhans Cell Histiocytosis (LCH) ndi matenda osowa omwe amayamba chifukwa cha kukula kosalamulirika kwa mtundu wina wa maselo oteteza thupi. Amaonekera kwambiri mwa ana.
- Ngakhale kuti izi sizimayikidwa m'gulu la khansa, madokotala a khansa amachichiza pogwiritsa ntchito mankhwala oletsa khansa.
- Zizindikiro (mabala a pakhungu, timibulu ta mafupa, matenda opatsirana pafupipafupi) zimasiyana kwambiri kutengera mbali ya thupi yomwe yakhudzidwa ndi matendawa.
- Kuyeza magazi m'thupi (biopsy) n'kofunika kwambiri kuti mudziwe bwinobwino matendawa.
- Kwa ana ambiri, makamaka omwe ali mgulu la "osatetezeka kwambiri", chithandizo chingachiritse matendawa kwathunthu.
- Ngakhale chithandizo chitatha, ndikofunikira kwambiri kukhala ndi dokotala kwa zaka zingapo kuti muwone ngati matendawa abwereranso.
- Kambiranani momasuka ndi dokotala mafunso kapena nkhawa zilizonse zomwe muli nazo zokhudza matenda a mwana wanu.
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