Ingabe unawo amangqamuzana abomvu afana nameva egazini lakho? Ake sifunde nge-(Acanthocytosis)!

Wake wezwa ngamaseli abomvu egazini lakho? Ngokuvamile, ayindilinga, njengamabhola amancane asicaba. Kodwa ngezinye izikhathi, lawa maseli abomvu egazi angashintsha isimo futhi abe nesimo esingajwayelekile, esinameva, nesinamathe. Yilokho igama lezokwelapha lalesi simo elibizwa ngokuthi `(Acanthocytosis)`. Cabanga nje, igama lesiGreki elithi "acan" lisho iva, into efana neva. Igama elithi "Cyte" lisho iseli. Ngakho-ke, "amangqamuzana afana nameva" yincazelo elula yalokhu.

Empeleni, sonke sinawo ambalwa ala maseli anameva (ama-Acanthocyte) egazini lethu. Ngokuvamile akuyona inkinga enkulu. Kodwa, uma unawo amaningi kakhulu ala maseli anameva egazini lakho, yilapho kuqala khona izinkinga ezincane. Uyazi ukuthi kwenzekani? Sinesitho esibizwa ngokuthi i-spleen . Sisebenza njengesihlungi esihlanza igazi lethu. Lokho okwenziwa yi-spleen ukuhlonza amangqamuzana amadala, alimele egazini bese siwabhubhisa. Ngakho-ke, lapho lawo maseli abomvu egazi anameva edlula e-spleen, i-spleen icabanga ukuthi, "O, lawa abukeka engcolile kancane, awabonakali njengamaseli ajwayelekile." Bese i-spleen iqala ukubhubhisa la maseli anameva ngesivinini esikhulu. Uma lokho kwenzeka, inani lamangqamuzana abomvu egazi aphilile emzimbeni wakho lingancipha. Lokhu kubizwa ngokuthi i-anemia noma i-"hemolytic anemia". Okusho ukuthi, ukuntuleka kwegazi okubangelwa ukuwohloka kwamangqamuzana abomvu egazi. Akukhona lokho kuphela, lesi simo esibizwa ngokuthi "i-Acanthocytosis" singaba uphawu lwezinye izifo nezinkinga eziningana. Ngakho-ke, uma la mangqamuzana anameva etholakala egazini, kungasiza odokotela ekuxilongeni ezinye izifo eziyisisekelo.

Ngakho-ke, hlobo luni lwezimpawu esingazithola ngenxa yalokhu `(i-Acanthocytosis)`?

Uma i-spleen yakho ibhubhisa ngokushesha amangqamuzana abomvu egazi ngenxa yesifo se-sickle cell, kuvela isimo esibizwa ngokuthi "i-hemolytic anemia". Ungase ube nezimpawu ezifana nalezi:

• Ukuphuzi kwesikhumba namehlo: Siphinde sikubize ngokuthi "i-jaundice", njengoba kwenzeka lapho abanye abantu benomkhuhlane ophuzi.
• Ukuzizwa ukhathele kakhulu: Hhayi nje ukukhathala, kodwa nomuzwa wokukhathala kungakhathaliseki ukuthi ulala kangakanani.
• Ubunzima bokuphefumula: Ukuzizwa uphelelwa umoya ngisho noma usebenza kancane (ukuphelelwa umoya).
• Ukushaya kwenhliziyo okusheshayo: Umuzwa wesifuba sakho sishaya ngokushesha kakhulu ngisho noma umile nje (i-tachycardia).
• Igazi emchameni: Ngezinye izikhathi umchamo ungaba bomvu noma ube nsundu ngokumnyama (hematuria).
• Ubuhlungu besisu: Lokhu kuvame ukubangelwa ukuvuvukala kwe-spleen.

Ngaphezu kwalezi zimpawu, ezinye izimpawu zingase zivele kuye ngesifo esiyisisekelo esibangele ukwakheka kwala maseli e-scaly emzimbeni wakho. Cabanga, kwabanye abantu:

• Ukwehla kwesisindo: Uma ungumntwana omncane, ukukhula kungase kuhambe kancane.
• Izinkinga zokuxhumanisa umsebenzi wemisipha: Njengokulahlekelwa ibhalansi (i-ataxia).
• Ukunyakaza okungahleliwe kwezingxenye zomzimba umuntu angenakuzilawula: isibonelo, ukucwayiza noma ukuphendula intamo (ukudlikiza).
• Ubunzima bokucabanga nokukhumbula: Ukuzizwa sengathi ubuchopho bakho buyehla (ukukhubazeka kwengqondo).
• Izinkinga zokugaya ukudla.
• Indle ephaphathekile: Lokhu kungenzeka uma amafutha engagaywa kahle (i-steatorrhea).
• Umchamo onombala omnyama.

Uma unesinye noma ngaphezulu salezi zimpawu, kungcono ukubona udokotela ngokushesha okukhulu.

Kungani lezi zinhlobo zamaseli anama-spiky zakheka egazini lethu? Yiziphi izizathu?

Kalula nje, imbangela eyinhloko ye-`(Acanthocytosis)` umphumela olwelwesini lwangaphandle lwamaseli abomvu egazi, okungukuthi, i-`(ilwelwesi leseli).` Lolu lwelwesi lwenziwe ngamafutha `(lipids)` namaprotheni . Ngakho-ke, uma kukhona isifo noma isimo esithinta lezi zingxenye ezibalulekile, isimo esijwayelekile esiyindilinga samaseli abomvu egazi siyashintsha bese eqala ukuthatha leso simo esifana nesameva.

Lawa maseli okuqala aguquliwe, njengoba kushiwo ngaphambili, awahlungwa kalula uma edlula ku-spleen. Ngakho-ke, i-spleen iyawabhubhisa. Ngezinye izikhathi, esikhundleni sokubhubhisa lawa maseli, i-spleen ithuntubeza eminye yemisipha yawo bese iwabuyisela egazini. Lawa maseli okuqala aguquliwe ngezinye izikhathi abizwa ngokuthi `(amaseli e-spur)`.

Nazi ezinye zezimbangela eziyinhloko ze-acanthocytosis:

• Isifo sesibindi esibi kakhulu: Amaseli e-spur avame ukuba uphawu lwesifo sesibindi esibi kakhulu, ngezinye izikhathi esisongela ukuphila. Abanye abantu abanesifo sesibindi baba nesimo esibizwa ngokuthi i-spur cell anemia (SPA). Lokhu kusho ukuthi banamaseli e-spur egazini labo, futhi banenani eliphansi lamaseli abomvu egazi.
• I-Abetalipoproteinemia: Lesi yisifo esingavamile kakhulu, esibangelwa izakhi zofuzo. Okusho ukuthi, sitholwa ngabazali kubantwana. Lesi sifo sibangela ukuthi umzimba ungakwazi ukumunca kahle amafutha namavithamini ancibilikayo emafutheni (amavithamini A, D, E, K).
• I-Neuroacanthocytosis: Lokhu futhi kuyisifo esingavamile sofuzo. Lokhu kubangela ukungakwazi ukulawula ukunyakaza komzimba. Izibonelo zifaka phakathi i-chorea-acanthocytosis, i-McLeod syndrome, i-pantothenate-kinase associated neurodegeneration (PKAN), kanye ne-Huntington's disease-like 2.

Ngaphezu kwalezi zimbangela eziyinhloko, ezinye izimo zingadala "i-Acanthocytosis":

• I-Anorexia nervosa yisimo esibangelwa ukuntuleka kwesifiso sokudla.
• Ukungasebenzi kahle kwe-thyroid gland (i-Hypothyroidism).
• I-Myelodysplastic syndrome yisifo somnkantsha wamathambo.
• Isifo sezinso.
• Ukususwa kobende ngokuhlinzwa (i-splenectomy).
• Imithi ethile (isibonelo, imithi yokwehlisa i-cholesterol efana "nama-statin").

Odokotela bakuthola kanjani i-acanthocytosis?

Udokotela wakho angaxilonga i-acanthocytosis ngokwenza i-peripheral blood smear (PBS) . Lokhu kuhilela ukuthatha isampula encane yegazi lakho bese ulibheka ngaphansi kwe-microscope. Lokhu kuzobonisa ngokucacile ukuthi kukhona la maseli angajwayelekile, anama-spikes.

Bese, ukuze uthole ukuthi yini ngempela ebangela lawa ma-cyst e-sebaceous, kuzodingeka wenze ukuhlolwa kwegazi okwengeziwe. Lokhu yilokhu:

• Inani eliphelele legazi (i-CBC)
• Ukuhlolwa kokusebenza kwesibindi
• Ukuhlolwa kokusebenza kwezinso
• Ukuhlolwa kwamahomoni e-thyroid
• Ukuhlolwa kwezinga lamafutha egazini `(Iphaneli ye-Lipid)`
• Ukuhlolwa kwezakhi zofuzo: Hlola izinguquko zezakhi zofuzo ezihlobene nezifo ezithile ezizuzwe njengefa.

Ngaphezu kwalokho, udokotela wakho angase futhi ancome ukuhlolwa okufana nokuskena izithombe ukuze abone ukuthi kukhona yini umonakalo ezicutshini noma ezithweni zomzimba wakho.

Yiziphi izindlela zokwelapha lokhu?

Ukwelashwa kwe-acanthocytosis kuncike embangela. Ezimweni eziningi, uma isimo esiyisisekelo selashwa kahle, amangqamuzana abomvu egazi abuyela esimweni esijwayelekile.

Cabanga ngakho, ukwelashwa kwesifo sesibindi kanye nezimo ezifana ne-"Neuroacanthocytosis" kuhloselwe kakhulu ukulawula izimpawu. Lezi zifo ngokuvamile kunzima ukuzelapha ngokuphelele. Zivame ukuba zimbi kakhulu ngokuhamba kwesikhathi. Kodwa-ke, ezifweni zesibindi ezinzima, kunezimo lapho ukufakelwa kwesibindi kuyikona kuphela okwelaphayo.

Uma unesifo sofuzo esibizwa ngokuthi i-abetalipoproteinemia, kungadingeka unciphise ukudla kwakho amafutha angenampilo futhi uthole amakhalori anele. Kungadingeka futhi ukuthi uthathe izithasiselo zamavithamini ukuqinisekisa ukuthi uthola amavithamini anele (A, D, E, K) ukwakha amaseli egazi abomvu anempilo.

Uma i-acanthocytosis ibangelwa yi-anorexia noma i-hypothyroidism, lesi simo sivame ukuthuthuka uma isimo esiyisisekelo selashwa. Futhi, uma kutholakala ukuthi umuthi ubangela i-acanthocytosis, lesi simo sivame ukuphela uma umuthi umisiwe.

Uma nginalesi simo, yini okufanele ngiyilindele?

Kwezinye izimo, isimo esibizwa ngokuthi i-acanthocytosis singalapheka ngokuphelele ngokwelashwa. Lokhu kusho ukuthi amaseli abomvu egazi abuyela esimweni esijwayelekile.

Kodwa-ke, isikhathi esiningi, imiphumela yempilo yakho incike ekutheni yini ebangele ukukhula kwalezi zindlala ze-sebaceous nokuthi uqala ngokushesha kangakanani ukwelashwa kwazo.

Isibonelo, izifo ezifana ne-`(Neuroacanthocytosis)` kanye ne-`(Abetalipoproteinemia)` zingabangela umonakalo wesikhathi eside ezicutshini zomzimba. Kodwa-ke, uma lesi sifo sitholakala futhi selashwa kusenesikhathi,, kunganciphisa ukuqhubeka kwezimpawu.

Ngokuvamile, uma une-`(Acanthocytosis)` enesifo sesibindi, kungaba yingozi kakhulu. Abantu abaningi abane-`(spur cell anemia)` eshiwo ngenhla bafa zingakapheli izinyanga ezimbalwa. Ngakho-ke, indlela engcono kakhulu yokwazi ngesimo sakho ivela kudokotela okwelaphayo. Uzobheka impilo yakho iyonke kanye nembangela yala maseli e-spur, bese echaza ukuthi kwenzekani kuwe.

Ingabe ikhona indlela yokuvimbela lokhu `(i-Acanthocytosis)` ukuthi kungenzeki?

Empeleni, uma i-acanthocytosis ibangelwa yisifo sofuzo, ayikho indlela yokuyivimbela. Ngoba yinto esiyizuza njengefa. Kodwa-ke, kunezinto esingazenza ukunciphisa ingozi yokuthola lesi simo ngenxa yezinye izimbangela.

• Gwema noma unciphise ukusetshenziswa kotshwala: Lokhu kungasiza ekuvimbeleni izifo zesibindi ezihlobene notshwala.
• Yidla ukudla okunomsoco: Umzimba wakho udinga ukuthola izakhamzimba ezifanele ukuze wakhe amangqamuzana abomvu egazi. Uma udinga usizo lokukhetha ukudla okufanele, xhumana nochwepheshe wezokudla.
• Thola ukwelashwa kusenesikhathi: Ezinye izimo ezibangela ama-cyst e-sebaceous zingaba zimbi kakhulu uma zingelashwa. Ngakho-ke, kubaluleke kakhulu ukubona udokotela ngokushesha nje lapho kuvela izimpawu, uxilonge isimo, bese uqala ukwelashwa.

Ngingayinakekela kanjani impilo yami?

Into ebaluleke kakhulu ukulandela ukwelashwa okunqunywe udokotela wakho ngesimo esiyisisekelo esibangela i-acanthocytosis yakho.

Uma unesifo sofuzo esibizwa ngokuthi i-acanthocytosis, cabanga ukuthola ukwelulekwa ngofuzo . Umeluleki wezakhi zofuzo angakweluleka ngokuthi lesi simo singakuthinta kanjani wena nomndeni wakho, kanye namathuba okuthi uzodlulisela ukuguquka kwezakhi zofuzo okubangela lokhu ezinganeni zakho.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Uma uya kudokotela, ungangabazi ukubuza imibuzo efana nale:

• Ingabe kufanele ngikhathazeke ngokuba nala maseli anameva (ama-Acanthocyte) egazini lami?
• Yiziphi ezinye izivivinyo engizodinga ukuzenza ukuze ngithole imbangela yalokhu?
• Hlobo luni lokwelashwa olungicebisayo?
• Hlobo luni lwemiphumela engingayilindela kulezi zindlela zokwelapha?
• Ingabe kufanele ngibone umeluleki wezakhi zofuzo?

Ekugcineni, kufanele ngithi... (Umyalezo Wokuya Ekhaya)

Uma odokotela bekuxilonga, babheka nokubonakala kwamaseli akho abomvu egazi. Uma uxilongwa ukuthi une-`(Acanthocytosis)` kusho ukuthi unamaseli abomvu egazi angajwayelekile egazini lakho. Ungakhathazeki. Ngezinye izikhathi, ukwelashwa kungabuyisela amaseli akho abomvu egazi esimweni esijwayelekile. Noma, kungasiza ekulawuleni isifo esiyisisekelo. Kodwa-ke, ngezinye izikhathi, ukuba nama-acanthocytes egazini lakho kungaba uphawu lwesimo esibucayi, njengesifo sesibindi esibi. Ngakho-ke, udokotela wakho ungumuntu ongcono kakhulu ukuchaza kahle ukuthi ukuba ne-`(Acanthocytosis)` kuyithinta kanjani impilo yakho. Uma ulalela lokho akushoyo futhi ulandela ukwelashwa ngqo, lesi simo singalawulwa kaningi.

I- Acanthocytosis, Amaseli egazi abomvu, i-Scutellaria, i-Spleen, i-Anemia, isifo sesibindi