Ingabe ingane yakho ivame ukuba nomkhuhlane, ukukhwehlela, izifo zendlebe, noma izifo zesikhumba? Ingabe isifo esisodwa siyathuthuka ngaphambi kwesinye? Ngokuvamile, sicabanga ukuthi amasosha omzimba wengane aphansi kancane nokuthi ithola izifo esikoleni nasezindaweni zokudlala. Kodwa-ke, ezimweni ezingavamile kakhulu, lokhu kungabangelwa yisimo esithile sofuzo esibizwa ngokuthi i-WHIM Syndrome . Nakuba lokhu kungakhulunywa ngakho kakhulu, kubaluleke kakhulu ukukuqaphela.
Iyini le-WHIM syndrome?
Kalula nje, i-WHIM syndrome iyisifo esingavamile kakhulu esithinta amasosha omzimba wethu. Igama elithi WHIM lisuselwa ezincwadini zokuqala zezimpawu ezine eziyinhloko zalesi sifo.
- Ama-warts (ama-warts)
- I-Hypogammaglobulinemia (amazinga aphansi ama-antibodies egazini)
- Izifo (izifo ezivame ukwenzeka)
- Ukopha kwe -Myeloalveolar (ukuminyana kwamangqamuzana amhlophe egazi emnkantsheni wethambo)
Cabanga nje ukuthi umzimba wethu unebutho lamangqamuzana amhlophe egazi. Umsebenzi wala masosha ukulwa nezitha ezifana namagciwane namagciwane abangela izifo. Kumuntu onesifo se-WHIM syndrome, amasosha abaluleke kakhulu kuleli butho, amangqamuzana amhlophe egazi abizwa ngokuthi ama-neutrophils , abhajwa esisekelweni sawo, umnkantsha wamathambo. Awakwazi ukuphuma, aye egazini, ukuze alwe nezifo. Ngakho-ke, uhlelo lokuzivikela lomzimba luba buthaka, futhi aqala ukugula kaningi.
Kuyivelakancane kakhulu kangangokuthi kukholakala ukuthi kuthinta umuntu oyedwa kuphela kwabayizigidi ezi-5 abazalwa, ngakho-ke kungenzeka ukuthi awukaze uzwe ngakho ngaphambili.
Ziyini izimpawu ze-WHIM syndrome?
Lezi zimpawu zingahluka kakhulu kumuntu nomuntu. Abanye abantu banezimpawu ezincane kakhulu. Abanye bangaba nezifo ezinzima ezingasongela impilo. Lezi zimpawu zivame ukuqala ebuntwaneni, kodwa ngezinye izikhathi lesi sifo singagcina singatholakali kuze kube yilapho umuntu ekhula.
| Uhlobo oluyisici | Izinto ovame ukuzibona |
|---|---|
| Izimpawu zokuqala ebuntwaneni |
|
| Izimpawu ezingaba khona kamuva |
|
Ikakhulukazi mayelana nama-warts kanye nengozi yomdlavuza
Abantu abaningi abasizungezile bangangenwa yi-human papillomavirus (HPV), ebangela izinhlungu. Kodwa-ke, kumuntu onesimiso sokuzivikela somzimba esinempilo, leli gciwane liyanyamalala cishe ngokuzenzakalelayo emzimbeni. Kodwa-ke, ngenxa yokuthi isimiso sokuzivikela somzimba somuntu one-WHIM syndrome sibuthakathaka, kunzima ukulwa naleli gciwane le-HPV. Ngakho-ke, igciwane lihlala emzimbeni isikhathi eside, okubangela izinhlungu, futhi kwezinye izimo, kwandisa ingozi yomdlavuza.
Kubangelwa yini lokhu? Ingabe lokhu kuyathelelana?
Imbangela eyinhloko ye-WHIM syndrome ukuguquka kwezakhi zofuzo okubizwa ngokuthi `CXCR4` emzimbeni wethu. Cabanga ngalezakhi zofuzo njengohlelo olufundisa amangqamuzana ethu. Lapho le zakhi zofuzo zishintsha, amangqamuzana amhlophe egazi (ama-Neutrophils) ashiwo ngenhla ayayeka ukuphuma emnkantsheni wethambo.
Ngenxa yokuthi lesi yisimo sofuzo, asinakusabalala kusuka kumuntu kuya komunye ngokuthimula noma ngokuthinta . Kodwa-ke, singadluliselwa kusuka kubazali kuya ezinganeni. Uma noma yimuphi umzali enalesi shintsho sofuzo, ingane ngayinye abanayo inethuba elingu-50% lokuthola lesi simo njengefa .
Uyithola kanjani ngokunembile lesi sifo?
Ngenxa yokuthi lesi yisifo esingavamile, kunzima ukusithola ngokuhlolwa okukodwa nje. Udokotela wakho uzokuhlola ngokucophelela izimpawu zakho nengane yakho, ukuthi ithola kangaki izifo, bese encoma ukuhlolwa okuningana.
- Ukubalwa Kwegazi Okuphelele (i-CBC): Lokhu kuhlola amangqamuzana amhlophe egazi aphansi, ikakhulukazi ama-neutrophil, egazini.
- Ukuhlolwa kwe-antibody (ukuhlolwa kwegazi kwe-Immunoglobulin G - IgG):Lokhu kuhlolwa kwenziwa ukuze kubonakale ukuthi amazinga ama-antibodies alwa nokutheleleka aphansi yini.
- Ukuhlolwa Kwe-Bone Marrow: Kule nqubo, kuthathwa isampula encane yomnkantsha ethanjeni lesinqe ngaphansi kwe-anesthesia emnene bese ihlolwa ngaphansi kwe-microscope. Lokhu kunganquma ngokunembile ukuthi amaseli amhlophe egazi abhajwe yini emnkantsha wethambo (myelokathexis).
- Ukuhlolwa Kwezakhi Zofuzo: Lokhu kuhlolwa kungaqinisekisa ukuthi kukhona yini ukuguquka kwezakhi zofuzo ze-`CXCR4` okubangela i-WHIM syndrome.
Uma lesi sifo sisheshe sitholakale, mancane amathuba okuba nezinkinga ezifana nokulaliswa esibhedlela njalo, ukulahlekelwa ukuzwa, kanye nokulimala kwamaphaphu.
Yiziphi izindlela zokwelapha i-WHIM syndrome?
Akukho ukwelashwa kwalesi simo okwamanje. Kodwa-ke, kunezindlela eziningana zokwelapha ezingasiza ukulawula izimpawu, ukuvikela ezifweni, futhi zikusize uphile impilo evamile.
Izinhlobo zemithi
- I-Xolremdi: Lona umuthi omusha owavunywa ngo-2024. Usiza ekukhululeni amaseli amhlophe egazi abhajwe emnkantsheni. Lokhu kunganciphisa ingozi yokutheleleka cishe ngo-40%.
- Ukwelashwa kwe-G-CSF: Lokhu kuwumjovo. Kuvuselela umongo wamathambo futhi kusize ekukhiqizeni amaseli egazi amhlophe engeziwe.
- Ukwelashwa kwe-IgG: Kulabo abanamazinga aphansi e-antibody, isimiso somzimba sokuzivikela siqiniswa ngokunikeza ama-antibodies avela ngaphandle.
- Ama-antibiotic: Lapho kuvela izifo ezibangelwa amagciwane (njenge-pneumonia, izifo zesikhumba), udokotela wakho uzokunikeza lezi ukuze azilawule.
Ukwelashwa kwama-warts
Uma amaqhubu evele njalo, ungabonana nodokotela wesikhumba ukuze asuswe. Kunezindlela eziningana zokwenza lokho.
- Ukuhlinzwa kwe-Cryosurgery
- Ukuhlinzwa ngogesi
- Ukwelashwa nge-laser
- Izinhlobo zezingubo ezikhethekile
Ungaphila kanjani uphilile une-WHIM syndrome?
Uma uphila nesimo esingavamile kangaka, kufanele ucabange kakhulu ngempilo yakho.
- Ukudla okuhle: Ukudla okunomsoco kubaluleke kakhulu ukugcina amasosha omzimba wakho eqinile. Faka imifino eminingi, izithelo, okusanhlamvu okuphelele, inhlanzi, kanye nenkukhu ekudleni kwakho.
- Ukuzivocavoca: Ukuzivocavoca kusiza ukugcina umzimba uqinile futhi kukhulisa amasosha omzimba. Yenza into oyithandayo, njengomdlalo, ukuhamba, noma ukubhukuda, nsuku zonke.
- Ukuvikelwa ezifweni:
- Gwema izindawo ezinabantu abaningi ngangokunokwenzeka.
- Geza izandla zakho njalo ngensipho.
- Khuluma nodokotela wakho bese unquma ukuthi yimiphi imijovo yokugoma oyidingayo wena nomndeni wakho.Khuluma ngqo ngezinto ezifana nomuthi wokugomela i-HPV, umuthi wokugomela i-pneumonia, kanye nomuthi wokugomela umkhuhlane waminyaka yonke.
Ngaphezu kodokotela womndeni wakho, ungase udinge usizo lodokotela abahlukahlukene, njengodokotela wezifo zomzimba, udokotela wezifo zegazi, kanye nodokotela wesikhumba, ukuze bakwelaphe.
Umlayezo Wokuya Nawe Ekhaya
- I-WHIM syndrome iyisifo esingavamile kakhulu, esibangelwa ufuzo. Akulona iphutha lakho noma lengane yakho.
- Uma ingane yakho ithola izifo ezivame kakhulu, kubaluleke kakhulu ukukhuluma nodokotela ngakho ngaphandle kokunganaki .
- Lesi sifo asithelelani, kodwa singadluliselwa kubazali kubantwana.
- Nakuba kungekho ikhambi eliphelele lalokhu okwamanje, izindlela zokwelapha zamanje zingalawula izimpawu, zinciphise izinkinga, futhi zilondoloze izinga lempilo elihle.
- Khuluma nodokotela wakho ngokukhululekile nganoma yiziphi izinkinga noma ukukhathazeka ngempilo ongase ube nakho.
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