Ngaba umntwana wakho ukhe waba neqhuma elincinci emzimbeni wakhe? Okanye iqhuma lolusu okanye iqhuma elirhawuzelayo elingapholiyo? Ngamanye amaxesha ezi ziqhelekile, kodwa azifane zibe ziimpawu zesifo esingaqhelekanga esibizwa ngokuba yiLangerhans Cell Histiocytosis (LCH). Kuqhelekile ukuziva usoyika xa usiva eli gama, kuba asinto esiyiqhelileyo ukuyiva. Kodwa ungakhathazeki, masithethe ngayo ngokulula.
Ngamafutshane, yintoni i-LCH?
Cinga ngemizimba yethu njengelizwe elikhulu. Kukho umkhosi wokukhusela eli lizwe, kwaye yinkqubo yethu yokuzikhusela . Uhlobo olukhethekileyo lwamajoni kulo mkhosi lubizwa ngokuba ziiseli zeLangerhans . Ezi seli luhlobo lweeseli ezimhlophe zegazi. Umsebenzi wazo ophambili kukulwa neentsholongwane kunye nosulelo olungena emizimbeni yethu kwaye lusikhusele kwizifo. La majoni afumaneka kuwo wonke umzimba wethu, ngakumbi eluswini, emiphungeni, kwii-lymph nodes, kumongo wethambo, kwi-spleen, nakwisibindi.
Kodwa kwimeko ye-LCH, ezi seli zeLangerhans ziyanda ngokungalawulekiyo kwaye ziqokelelana kwiindawo ezahlukeneyo zomzimba. Kufana nomhlambi wamajoni emi nje kwindawo enye ngaphandle kokulwa. Xa ziqokelelana ngolu hlobo, ziqala ukonakalisa izicubu eziphilileyo kwezo ndawo. Izilonda ziyavela kwezo ndawo.
Iindaba ezimnandi ngale meko kukuba uninzi lwabantwana lunokuchacha ngokupheleleyo kwesi sifo ngonyango olufanelekileyo. Ngamanye amaxesha, ingakumbi ukuba sichaphazela ulusu kuphela, sinokuphela ngokwaso ngaphandle konyango. Nangona kunjalo, ukuba ezi seli zichaphazela amalungu abalulekileyo afana nomongo wethambo, ipleen okanye isibindi, kunokufuneka unyango oluqatha ngakumbi.
Ngaba umhlaza we-LCH?
Lo ngumbuzo abantu abaninzi abanawo. Enyanisweni, kusekho ukungavisisani phakathi koogqirha malunga noku. Abaphandi abaninzi bacinga ukuba i-LCH yimeko efana nomhlaza, oko kukuthi, i-neoplasm . Oko kukuthi, ukukhula okungaqhelekanga kweeseli. Kodwa abanye bacinga ukuba sisifo sokudumba kwenkqubo yomzimba yokuzikhusela.
Nangona kunjalo, i-LCH inyangwa ngoogqirha bomhlaza, abagxile kumhlaza nezifo zegazi. Ngamanye amaxesha, unyango oluchasene nomhlaza olufana ne -chemotherapy nalo luyasetyenziswa ukuyinyanga.
Ngubani onokuthi afumane esi sifo?
I-LCH idla ngokubonakala kwiintsana ezisandul’ ukuzalwa kunye nabantwana abaphakathi kweminyaka eli-1 neli-15 ubudala. Abantu abadala banqabile kakhulu, kodwa akunakwenzeka.
Ngokwezibalo, ngumntwana omnye okanye ababini kuphela kwizigidi ezisandul’ ukuzalwa abazalwa benale sifo minyaka le. Oku kuthetha ukuba sisifo esingaqhelekanga kakhulu .
Zithini iimpawu ze-LCH?
I-LCH ayichaphazeli bonke abantwana ngendlela efanayo. Nangona abanye abantwana banokuchaphazeleka yindawo enye kuphela yomzimba wabo, abanye banokuba neendawo ezininzi zomzimba wabo. Ke ngoko, iimpawu ziyahluka ngokuxhomekeke kwinxalenye yomzimba echaphazelekileyo. Makhe sijonge ukuba ziyintoni na.
| Inxalenye yomzimba echaphazelekayo | Iimpawu ezinokubonwa |
|---|---|
| Amathambo | I-LCH ichaphazela amathambo kubantu abamalunga nama-80% abaguli. Iqhuma okanye ukudumba kunokubonakala kwiindawo ezifana nekhakayi, amathambo ajikeleze amehlo, ngasemva kweendlebe, umhlathi, amalungu omzimba, umqolo, kunye namaqaqa. Intlungu isenokuba ikhona okanye ayikho. Ukongeza, iintloko ezibuhlungu, iintlungu zentamo/umqolo, ubunzima bokuhamba, kunye nokuqhwalela kunokubonakala. |
| Ulusu | Eyona ixhaphakileyo yirhashalala yesikhumba. Isiciko se-Cradle sinokuba yimeko engapholiyo ebonakala entlokweni yomntwana. Amabala abomvu, anamaxolo anokubonakala emqolo, emakhwapheni, esiswini, esifubeni, nasemqolo. La mabala anokuvuza aze arhawuzelele okanye abe buhlungu. Izinzipho zinokutshintsha umbala okanye ziwe. |
| Umlomo | Amazinyo akhululekileyo okanye akhululekileyo, amazinyo aphumayo, iintsini ezivuvukileyo, izilonda emilebeni, elulwimini, ngaphakathi ezidleleni, okanye eluphahleni lomlomo. |
| Isibindi okanye i-spleen | Ukwanda kwesibindi okanye ipleyini kunokubangela ukudumba kwesisu, ukutyheli kwamehlo nolusu (i-jaundice), ukurhawuzelela, ukudinwa kakhulu, kunye nokukrweleka okanye ukopha ngokulula. |
| Ummongo wethambo | Ukuphelelwa ligazi, ukuphaphatheka, ukudinwa, kunye nokuphelelwa ngumdla wokutya ngenxa yokuncipha kweeseli ezibomvu zegazi. Usulelo oluqhelekileyo kunye nomkhuhlane ngenxa yokuncipha kweeseli ezimhlophe zegazi. Ukukruquka okulula ngenxa yokuncipha kweeplatelets. |
| Inkqubo ye-Endocrine (Inkqubo ye-Endocrine - iiHormones) | Ukuba i-pituitary gland ichaphazelekile: ukoma kakhulu kunye nokuchama rhoqo (isifo seswekile insipidus), ukukhula okungalindelekanga, ukufikisa kwangethuba okanye ukulibaziseka, ukutyeba. Ukuba i-thyroid gland ichaphazelekile: ukudumba kwe-gland, ubunzima bokuphefumla. |
| Iindlebe | Usulelo oluqhelekileyo lwendlebe, ukuphuma kwamanzi endlebeni, ukuba bomvu kwendlebe, ukurhawuzelelwa kwendlebe, iintlungu zendlebe, kunye nokulahlekelwa kukuva. |
| Amehlo | Amehlo avuvukileyo, ukudumba ngaphezu kwamehlo, ukuphazamiseka kokubona. |
| Ithumba | Ukudumba kunye nentlungu kwiziqhuma (iziqhuma) entanyeni, emakhwapheni, okanye emqolo. |
| Inkqubo yeNervous ephakathi | Intloko ebuhlungu, isiyezi, ukuhlanza, ubunzima bokuhamba, ukulahlekelwa yibhalansi (ataxia), ubunzima bokuthetha okanye bokubona, ukuxhuzula, utshintsho kwindlela yokuziphatha kunye nenkumbulo. |
| Imiphunga | Oku kuqhelekile kubantu abadala, ingakumbi abantu abatshayayo. Iintlungu zesifuba, ukukhwehlela okomileyo, ubunzima bokuphefumla, ukuwa kwemiphunga (pneumothorax). |
| Indlela Yesisu | Intlungu yesisu, ukuhlanza, urhudo, igazi eliphuma endle, kunye nokukhula kakubi ngenxa yokunqongophala kwesondlo. |
Into ebalulekileyo kukuba ezi mpawu zinokubonakala nakwezinye izifo ezininzi eziqhelekileyo. Ngoko ke musa ukoyika i-LCH kuba nje unenye okanye ezimbini kwezi mpawu. Kodwa ukuba ezi mpawu ziyaqhubeka, kubaluleke kakhulu ukubona ugqirha kwaye ufumane ukuxilongwa ngokufanelekileyo.
Yintoni unobangela okhethekileyo we-LCH?
Ngamafutshane, iiseli zethu zinezakhi zofuzo ezizixelela ukuba "zahlule ngoku, ziyeke ngoku." Malunga nesiqingatha sabantwana abane-LCH banotshintsho oluncinci, okanye utshintsho, kwizakhi zofuzo zabo ezibizwa ngokuba yi-'BRAF .' Le zakhi zofuzo yenza iproteni elawula ukukhula kweeseli. Ngenxa yolu tshintsho, loo proteni ayifumani myalelo "wokuyeka ukwahlula." Kufana nokuba iswitshi 'yokuvula' ibambekile. Ngoko ke iiseli zeLangerhans ziyaqhubeka zahlula kwaye zidibana.
Oku akuyonto izuzwa ngabazali ukuya ebantwaneni. Olu tshintsho lwemfuza lwenzeka ngengozi emva kokuba umntwana ekhulelwe esibelekweni sikanina.
Ukongeza kwi-gene ye-'BRAF', abaphandi bafumanise ukuba esi sifo sinokubangelwa kukuguquka kwezinye ii-gene ezifana ne-'MAP2K1`, 'RAS`, kunye ne-'ARAF`.
Uyifumana njani le nto, Gqirha?
Xa usisa umntwana wakho kugqirha, uza kuqala akubuze ngeempawu zomntwana wakho. Emva koko, baza kumhlola ngononophelo umntwana wakho. Ngenxa yokuba i-LCH inokuchaphazela iindawo ezahlukeneyo zomzimba, kunokufuneka iimvavanyo ezininzi ukuqinisekisa ukuxilongwa.
| Uhlobo lovavanyo | Kalula nje... |
|---|---|
| Uvavanyo lwegazi | Uvavanyo lwegazi olupheleleyo (i-CBC) lulinganisa inani leeseli ezibomvu, iiseli ezimhlophe, kunye neeplatelets egazini. Uvavanyo lwegazi lwenziwa ukujonga izinto ezifana nokusebenza kwesibindi. |
| I-Biopsy | Le yeyona ndlela icacileyo yokuqinisekisa i-LCH.Iqhekeza elincinci lesicwili lithathwa kwiqhuma okanye kwisilonda phantsi kwe-anesthesia kwaye lihlolwe nge-microscope ukuqinisekisa ukuba zikhona na iiseli ze-LCH. Ukuba kukho ukurhaneleka kokubandakanyeka komongo wethambo, i-bone marrow biopsy nayo inokwenziwa. |
| Uvavanyo lweMfuzo | Isampuli yethishu ethathwe kwi-biopsy isetyenziselwa ukuvavanya utshintsho kwi-gene ye-'BRAF'. |
| Uvavanyo lokuFanekisa | Uvavanyo olufana ne-X-ray, i-CT scans, i-MRI scans, kunye ne-PET scans lunokumisela ubungakanani bempembelelo ye-LCH kwizitho zangaphakathi ezifana namathambo, ubuchopho kunye nemiphunga. |
Ngokusekelwe kwiziphumo zolu vavanyo, ugqirha wakho uza kuthumela umntwana wakho kwingcali ye-hematologist/oncologist yabantwana.
Ziziphi iindlela zonyango lwe-LCH?
Ayingabo bonke abantwana abane-LCH abafuna uhlobo olufanayo lonyango. Unyango luxhomekeke kwindawo kunye nobunzima besifo.
Oogqirha bahlula i-LCH kwiintlobo ezimbini eziphambili:
1. I-LCH yenkqubo enye: Esi sifo sichaphazela inkqubo yesitho esinye kuphela emzimbeni (umz., amathambo kuphela okanye ulusu kuphela).
2. I-Multi-system LCH: Esi sifo sichaphazela iinkqubo zomzimba ezimbini okanye ngaphezulu (umz., ulusu nesibindi).
Kwakhona, amalungu anjengesibindi, i-spleen, kunye nomongo wamathambo athathwa njengamalungu "asengozini enkulu", ngelixa amalungu anjengesikhumba, amathambo, kunye nemiphunga athathwa "njengasengozini ephantsi." Olu luhlu lumisela isicwangciso sonyango.
Kukho iindlela ezininzi eziphambili zonyango:
- Unyango lwe-steroid: Ingakumbi xa ulusu kuphela luchaphazeleka, amayeza e-steroid afana ne-prednisone asetyenziswa njengeepilisi okanye amafutha.
- Utyando: Utyando olufana ne -curettage lwenziwa ukuze kususwe ithumba le-LCH ethanjeni.
- I-Chemotherapy: Olu luhlobo lonyango olunikwa ukubulala iiseli zomhlaza. Ngenxa yokuba iiseli ze-LCH zahlukana ngokukhawuleza, la mayeza ayawathintela ekukhuleni. La mayeza anokunikwa njengeepilisi, iinaliti, okanye njengekhrimu esetyenziswa eluswini.
- Unyango lwemitha: Isebenzisa imitha enamandla aphezulu ukutshabalalisa iiseli ze-LCH. Oku ngoku akusetyenziswa rhoqo.
- Unyango olujoliswe kulo:Kubantwana abane-'BRAF' gene mutation, kukho amayeza (ii-BRAF inhibitors) ajolise kuloo gene mutation. La mayeza anomonakalo omncinci kakhulu kwiiseli ezisempilweni.
- Unyango lwe-Immunotherapy: Ukuvuselela amajoni omzimba omntwana ukuze alwe neeseli ze-LCH.
- Ukufakelwa kweeseli zesiqu: Ukhetho lonyango oluqwalaselwa kwiimeko ezinzima kakhulu ezinganyangekiyo ngezinye iindlela zonyango.
Injani imeko emva konyango?
Uqikelelo lwe-LCH luxhomekeke kwizinto ezininzi, kuquka iindawo zomzimba ezichaphazelekileyo sesi sifo, ukuba sisasazeke kangakanani, kunye nendlela esisabela ngayo kunyango.
- Izinga lokuphiliswa kwabantwana abanezitho zomzimba "ezingenabungozi" kuphela (zombini inkqubo enye kunye neenkqubo ezininzi) liphantse libe yi -100% . Oku kuthetha ukuba akukho mngcipheko wokufa. Nangona kunjalo, kukho umngcipheko wokuphinda kwenzeke okanye ezinye iingxaki ezihlala ixesha elide.
- Imeko yabantwana abanezitho "ezinobungozi kakhulu", ezifana nesibindi, i-spleen, kunye nomongo wethambo, inokuba yingozi kakhulu.
Kungoko ke, kubaluleke kakhulu ukulandela ugqirha wakho iminyaka emininzi, nokuba unyango lugqityiwe. Kufuneka uhlolwe rhoqo ukuba awuphinde ubuye na.
Umyalezo Wokuya Ekhaya
- I-Langerhans Cell Histiocytosis (LCH) sisifo esingaqhelekanga esibangelwa kukukhula okungalawulekiyo kohlobo lweseli yomzimba yokuzikhusela. Sibonakala kakhulu ebantwaneni.
- Nangona oku kungachazwa njengomhlaza, iingcali zomhlaza ziyawunyanga zisebenzisa iindlela zonyango zokulwa nomhlaza.
- Iimpawu (izilonda zesikhumba, amaqhuqhuva ethambo, usulelo oluqhelekileyo) ziyahluka kakhulu ngokuxhomekeke kwinxalenye yomzimba echaphazelekileyo sesi sifo.
- I-biopsy ibalulekile ukuze kufunyaniswe isifo ngokuqinisekileyo.
- Kwabantwana abaninzi, ingakumbi abo bakwicandelo "elingenabungozi buphezulu", unyango lunokunyanga ngokupheleleyo esi sifo.
- Nokuba unyango lugqityiwe, kubaluleke kakhulu ukuba kubekho uphononongo lwezonyango iminyaka eliqela ukuze kubonwe ukuba isifo siyaphinda na.
- Xoxani ngayo nayiphi na imibuzo okanye iinkxalabo eninazo malunga nemeko yomntwana wenu ngokukhululekileyo nogqirha wenu.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho