Ingabe izingxenye zomzimba wakho zihlelwe ngendlela ehlukile? Ake sixoxe nge-Heterotaxy Syndrome!

Izitho ezibalulekile emizimbeni yethu, njengenhliziyo, amaphaphu, nesibindi, zibekwe ngokulandelana okufanayo nasezindaweni ezifanele wonke umuntu, akunjalo? Yileyo indlela evamile. Kodwa cabanga ngakho, ngezinye izikhathi lezi zitho azikho lapho kufanele zibe khona, kodwa zingatholakala ngendlela ehlukile kancane, ezindaweni ezahlukene. Yilokho esizokhuluma ngakho namuhla, isimo esingavamile kodwa esibalulekile okufanele usiqaphele. Lokhu kubizwa ngokuthi i-heterotaxy syndrome.

Iyini le heterotaxy syndrome?

Kalula nje, i-heterotaxy syndrome yisimo lapho izitho zangaphakathi esifubeni sakho nasesiswini sakho zitholakala ezindaweni ezahlukene kunezivamile. Cabanga ngakho, lapho wonke umuntu ezalwa, izitho zakhe zitholakala ezindaweni ezithile emzimbeni wakhe. Isibonelo, umuntu one-heterotaxy angase abe nenhliziyo yakhe kanye ne-spleen ohlangothini lwesokudla esikhundleni sesobunxele. Lezi zinguquko ngezinye izikhathi zingabangela izinkinga ezinkulu zempilo, futhi zingaba yingozi ngisho nokuphila.

Igama elithi "heterotaxy" livela olimini lwesiGreki. Igama elithi "heteros" lisho "okuhlukile" kanti elithi "taxis" lisho "ukuhleleka, ukuhlela". Lokhu kusho ukuhlela okuhlukile . Lokhu ngezinye izikhathi kubizwa ngokuthi "Heterotaxia" noma "Atrial Isomerism".

Yiziphi izitho zomzimba ezingathinteka yi-heterotaxy syndrome?

Lesi simo singathinta isikhundla kanye nokusebenza kwezitho ezilandelayo emzimbeni wakho:

• Inhliziyo
• Amaphaphu
• Isibindi
• Ubende
• Amathumbu

Ingabe lokhu kwehlukile kokuthi `(Situs Solitus)` kanye `(Situs Inversus)`?

Yebo, lezi yizimo ezintathu ezahlukene.

• (Situs Solitus): Lokhu kubhekisela endaweni evamile, elindelekile yezitho zethu zangaphakathi. Lena yindlela iningi lethu elinazo ngayo izitho zethu.
• (Situs Inversus): Lokhu kwenzeka lapho izitho zangaphakathi zibekwe ngendlela ephambene nendawo yazo evamile, njengokungathi ubheka esibukweni . Isibonelo, inhliziyo ingakwesokudla esikhundleni sesobunxele. Ezimweni eziningi, `(Situs Inversus)` akuvamile ukubangela izinkinga ezinkulu zempilo.
• (Heterotaxy Syndrome): Lokhu akuyona nje indaba yokuthi izitho zomzimba ziyashintshwa. Ezinye izitho zingase zingakheki kahle, noma kungase kube nezinkinga ezinkulu ngokusebenza kwazo. Lesi yisimo esingabangela izinkinga zempilo eziyinkimbinkimbi nezingathi sína kune-`(Situs Solitus)` noma `(Situs Inversus)`.

Ubani ongahlakulela lesi simo?

I-Heterotaxy syndrome iwukuhlukahluka kwezakhi zofuzoNoma ubani angaba nesifo esibangelwa yisifo senhliziyo sokuzalwa. Ngokuvamile, lesi simo senzeka ngezikhathi ezithile, okusho ukuthi akekho emndenini oke waba naso ngaphambili, futhi sibangelwa ukuguquka kwezakhi zofuzo okusha (`(sporadic noma de novo mutation)`). Kodwa-ke, uma othile emndenini wakho eke waba nezinkinga zenhliziyo zokuzalwa , ingozi yakho yokuba nengane enalesi simo inganda kancane.

Sivame kangakanani lesi simo?

Emhlabeni jikelele, kulinganiselwa ukuthi cishe ingane eyodwa kweziyi-10,000 ezisanda kuzalwa ihlushwa yi-heterotaxy syndrome. Kodwa-ke, ezinye izifundo ziphakamisa ukuthi lesi simo singase sibe sivame kakhulu ngoba ngezinye izikhathi asihlolwa kahle.

Cishe u-3% wezifo zenhliziyo ezizalwa nazo zihlobene nalesi sifo se-heterotaxy.

Ziyini izimpawu zalokhu?

Uphawu oluyinhloko ukuthi izitho zangaphakathi esifubeni nasesiswini azikhuli njengoba kulindelekile. Ngezinye izikhathi ezinye izitho zingase zingabi khona, noma zingase zingakheki kahle ngesikhathi sokukhulelwa. Izimpawu ezibangelwa yilokhu yilezi:

• Izitho zangaphakathi (inhliziyo, amaphaphu, isibindi, ubende, amathumbu) azisebenzi kahle.
• Ukuba nesakhiwo esingavamile senhliziyo (isimo senhliziyo yokuzalwa).
• Ukuwohloka kwamathumbu.
• Ukungabikho kwe-spleen (Asplenia) noma ukuhlukaniswa kwe-spleen kube yizingxenye (i-Polysplenia).

Izimpawu ezinjengalezi zingase zivele ngoba izitho zangaphakathi azisebenzi kahle:

• Ubunzima bokuphefumula.
• Ukwehliswa kokumelana nezifo.
• Isikhumba esiluhlaza okwesibhakabhaka noma esimpunga (i-Cyanosis).
• Ubuhlungu besisu noma besisu.
• Ubunzima bokudla, ukukhuluphala, noma ukugaya ukudla.
• Ukushaya kwenhliziyo okungajwayelekile.
• Ukunqwabelana kwe-mucus noma uketshezi emaphashini.

Cabanga nje, kukhona umntwana osanda kuzalwa, unobunzima bokuphefumula, umzimba wakhe uluhlaza okwesibhakabhaka kancane. Lapho odokotela bemhlola, bathola ukuthi kukhona umehluko omncane enhliziyweni yengane, mhlawumbe udende lutholakala kolunye uhlangothi. Lezi zinto zingaba izimpawu ze-`(Heterotaxy Syndrome)`.

Yini ebangela lokhu?

Kunezici eziningana ezingabangela i-heterotaxy syndrome.

Imbangela enkulu ukuguquka kwezakhi zofuzo . Izinguquko ezakhiweni zofuzo ezingaphezu kuka-60 zingathinta lokhu. Kunezindlela ezahlukene ongazuza ngazo lesi simo sofuzo:

• Ukuzuza ikhophi yesakhi sofuzo esiguquliwe kumzali oyedwa (`(Autosomal Dominant)`).
• Ukuzuza ikhophi yofuzo eguquliwe evela kubazali bobabili (`(Autosomal Recessive)`).
• Uguquko lwezakhi zofuzo olusanda kwenzeka (`(Sporadic noma De Novo)`) olungenawo umlando womndeni.
• Ukuba noshintsho lwezakhi zofuzo ku-chromosome yakho ye-X, okungenye yama-chromosome akho ocansi (`(X-linked)`). Lokhu kuvame kakhulu kwabesilisa, ngoba bane-chromosome eyodwa ye-X kuphela.

Ngaphezu kwezimbangela zofuzo, izici zemvelo nazoIsibonelo, ukuchayeka kukamama entweni yamakhemikhali noma enobuthi (isib. izibulala-zinambuzane, izinto eziqukethe umthofu) ngesikhathi sokukhulelwa nakho kungabangela lesi simo embungwini osakhula.

Ngisho namanje, kwenziwa izifundo ezengeziwe ezimweni lapho lesi simo senzeka khona ngaphandle kwezici zofuzo noma ezemvelo.

Ukuthola kanjani lokhu?

I-Heterotaxy syndrome ihlolwa udokotela ngemva kokukuhlola bese enza ukuhlolwa okukodwa noma ngaphezulu kwezithombe, okufana nalokhu:

• Ukuhlolwa kwe-MRI (i-Magnetic Resonance Imaging).
• Ukuskena kwe-`CT` (ukuskena kwe-Computed Tomography)`.
• I-echocardiogram (i-ultrasound scan yenhliziyo).

Uma, ngemva kwalezi zivivinyo zezithombe, udokotela esola i-heterotaxy syndrome, uzoyala ukuhlolwa okwengeziwe ukuhlola ukusebenza kwezitho zakho zangaphakathi. Lokhu kufaka phakathi:

• Ukuhlolwa kwegazi ukuhlola impilo ye-spleen.
• I-Endoscopy (ukwethulwa kwepayipi elinekhamera)
• Ukuhlolwa okuhlola ukusebenza kwezinso.
• I-Ultrasound Yezinso.

Itholakala nini i-heterotaxy syndrome?

Lesi simo singatholakala ngaphambi kokuzalwa nge-ultrasound yangaphambi kokubeletha noma i-echocardiography (ukuhlolwa kokuhlola inhliziyo yengane esanda kuzalwa). Iningi labantu litholakala lapho lizalwa. I-Heterotaxy ivame ukutholakala lapho kunezimpawu zesifo senhliziyo esizalwa naso. Abanye abantu abangenazo izimpawu ezibucayi bangase batholakale kamuva ebuntwaneni. Akuvamile kakhulu, lesi simo sitholakala ngengozi ngesikhathi sokuhlolwa kwesinye isimo lapho umuntu emdala.

Yiziphi izindlela zokwelapha? `(Ukwelashwa)`

Ukwelashwa kwe-heterotaxy syndrome akufani kuwo wonke umuntu. Kuya ngokuthi yiziphi izitho zomzimba wakho ezithintekile nokuthi zithinteke kangakanani .

Ukwelashwa okuvamile kakhulu ukuhlinzwa . Lokhu kuhlinzwa kwenziwa ukuze kulungiswe ukukhubazeka ekukhuleni noma ekusebenzeni kwezitho esifubeni nasesiswini. Ukwelashwa kwalesi simo kungadinga ukuhlinzwa okuningi kuyo yonke impilo yomuntu, ngezinye izikhathi kusukela ebuntwaneni . Kunezinhlobo eziningana zokuhlinzwa:

• Ukuhlinzwa kwenhliziyo: Ukuhlinzwa ukuze kuthuthukiswe ukusebenza kwenhliziyo noma kulungiswe ukukhubazeka kwenhliziyo okuzalwe nakho.
• Inqubo yeFontan : Lokhu futhi kuyindlela yokuhlinzwa kwenhliziyo. Kwakha igumbi elilodwa (i-ventricle) lokupompa igazi emaphashini nasemzimbeni.
• Inqubo Yokuhlinza : Ukuhlinzwa kokulungisa ukusonteka kanye nokuvinjelwa kwamathumbu.
• Ukufakelwa inhliziyo`(Ukufakelwa Inhliziyo)`: Ukufaka inhliziyo yakho esikhundleni senhliziyo yomnikeli. Lokhu kungadingeka kubantu abadala abaye bahlinzwa inhliziyo kaningi empilweni yabo yonke.

Izinketho zokwelapha ezengeziwe zifaka:

• Ukufakelwa kwe- pacemaker ukulawula isigqi senhliziyo.
• Ukuthatha imithi yokwehlisa umfutho wegazi.
• Ukuthatha ama-antibiotic (ama-antibiotiki okuvimbela) ukusiza ubende ukulwa nokutheleleka.

Ngizolulama ngokushesha kangakanani ngemva kokwelashwa?

Isikhathi esithathayo ukuze ululame ngemva kokwelashwa siyahlukahluka, kuye ngohlobo lokuhlinzwa okwenzile . Ngemva kokuhlinzwa, umzimba wakho udinga ukuphumula kahle ukuze ululame. Ngemva kokuhlinzwa kwenhliziyo okuningi, uzoba sesibhedlela ngaphansi kokubhekwa kwezokwelapha amahora angama-24 izinsuku eziningana kuya kwamasonto ukuze ubone ukuthi ukuhlinzwa kuphumelele yini nokuthi kukhona yini imiphumela emibi. Ngemva kokwelashwa okuthile, kungathatha izinyanga eziningana ukuthi umzimba wakho ululame ngokugcwele. Ngaphambi kokuhlinzwa, udokotela wakho uzokuchazela ukuthi ungawunakekela kanjani umzimba wakho ngemva kokuhlinzwa nokuthi yini ongayenza ukuze usize ukululama kwakho.

Ingabe lokhu kungavinjelwa?

Ngenxa yokuthi ezinye izimbangela ze-heterotaxy syndrome zibangelwa izinguquko zofuzo , lesi simo asinakuvinjelwa ngokuphelele. Ungakhuluma nodokotela wakho mayelana nokuhlolwa kofuzo ukuze ufunde ngezingozi zakho ngesikhathi sokukhulelwa.

Uma ukhulelwe, ukugwema ukuchayeka kumakhemikhali noma ubuthi (isib., izibulala-zinambuzane, imikhiqizo equkethe umthofu) engabangela lesi simo kumntwana okhulayo kuzosiza ekuqinisekiseni impilo yomntwana okhulelwe.

Ingakanani iminyaka yokuphila komuntu ophila nalesi simo?

Isikhathi sokuphila somuntu one-heterotaxy syndrome sincike ebunzimeni bokuxilongwa. Izinhlobo ezinzima zalesi simo, ngisho noma sezelashwe, zingaba yingozi empilweni yezinsana nezingane. Kodwa-ke, uma lesi simo singesibi kakhulu, ngokwelashwa nokuqashwa njalo kwezokwelapha, kungenzeka ukuphila impilo evamile ngaphandle kwezinkinga eziningi zempilo . Uma uzwa ukushaya kwenhliziyo okungajwayelekile noma ubuhlungu besifuba noma besisu, bheka udokotela ngokushesha.

Udinga ukubona udokotela ngasiphi isikhathi?

Bona udokotela wakho uma unezinye zalezi zimpawu:

• Uma isikhumba sakho siphenduka sibe luhlaza okwesibhakabhaka noma sibe mpunga okhanyayo.
• Uma ungakwazi ukudla noma ukuphuza.
• Uma unenxeba elingapholi, noma inxeba elivuvukele, eliphuma ubomvu obuphuzi, noma elinoqweqwe.

Kunini lapho kudingeka uye ekamelweni lezimo eziphuthumayo?

Kulesi simo, iya ekamelweni eliphuthumayo ngokushesha, noma ushayele u-1990:

• Ubuhlungu obukhulu besifuba noma ubuhlungu besisu.
• Ukushaya kwenhliziyo okungajwayelekile.
• Ubunzima bokuphefumula.

Yimiphi imibuzo okufanele uyibuze udokotela?

Uma kutholakala ukuthi une-heterotaxy syndrome, ungase ufune ukubuza udokotela wakho imibuzo efana nalokhu:

• Kubaluleke kangakanani ukuxilongwa kwami?
• Ingabe ngidinga ukuhlinzwa noma ukuhlinzwa okuningana?
• Ulungiselela kanjani ukuhlinzwa?
• Kuthatha isikhathi esingakanani ukululama ngemva kokuhlinzwa?
• Ingabe ikhona imiphumela emibi ekwelashweni okukhuthazayo?

Kuyini 'i-Isomerism'?

Igama elithi `Isomerism` lisetshenziswa kumakhemikhali ukuchaza ama-compounds anefomula efanayo yamakhemikhali kodwa izakhiwo ezahlukene. I-Heterotaxy syndrome ibizwa nangokuthi `(Isomerism)` ngoba izitho zakho zangaphakathi azikho ezindaweni zazo ezifanele, kodwa ngezinye izikhathi zingenza imisebenzi yazo eyisisekelo. Okusho ukuthi, umqondo uwukuthi noma isimo noma isikhundla sihlukile, imvelo eyisisekelo iyafana .

Iyini ikhodi ethi `ICD` yalesi simo?

I-International Classification of Diseases (ICD) iyithuluzi elisetshenziswa odokotela ukuhlukanisa izimo zezokwelapha ezindaweni zezokwelapha. Ikhodi ye-ICD-10-CM ye-heterotaxy syndrome yi-Q89.3.

Umyalezo obalulekile kubazali kanye nabazali besikhathi esizayo

Uma uthola ukuthi ingane yakho esanda kuzalwa ine-heterotaxy syndrome, ungase ube nemizwa ehlukahlukene. Kuyaqondakala ukuthi kunzima kakhulu. Kodwa ungakhathazeki. Odokotela bakho, kanye nochwepheshe, bazokwenza konke okusemandleni abo ukuqinisekisa impilo yengane yakho nokuphatha izimpawu ukuze zingabe zisongela impilo. Izinkinga nezimpawu zalesi simo zidinga ukwelashwa okuqhubekayo nokuqapha ukuqinisekisa ukuthi intuthuko yengane nokuphila kwayo okugcwele akuphazanyiswa.

Uma ukhulelwe umntwana futhi ufuna ukuqonda ingozi yokuba ingane yakho ibe nesimo sofuzo njenge-heterotaxy syndrome, kubalulekile ukukhuluma nodokotela wakho mayelana nokuhlolwa kofuzo noma ukwelulekwa ngofuzo . Lolu lwazi luzokusiza ukuthi wenze izinqumo ezinolwazi. Khumbula, kungcono ngaso sonke isikhathi ukufuna iseluleko sezokwelapha nganoma iyiphi inkinga yezempilo.

I- Heterotaxy Syndrome, I-Heterotaxy Syndrome, Izitho Zangaphakathi, Isifo Senhliziyo, Amaphutha Okuzalwa, Ukuguqulwa Kwezakhi Zofuzo, Impilo Yengane