Ngaba unamabala angaqhelekanga eluswini lwakho? Ngaba kungenxa ye-(Incontinentia Pigmenti)?

Ngaba wakha weva ngale meko ibizwa ngokuba yi-`(Incontinentia Pigmenti)` okanye `(IP)`? Mhlawumbi xa usiva eli gama, lisenokuvakala liyinkimbinkimbi kwaye lingaqhelekanga. Kodwa le yingongoma ebalulekileyo ekufuneka sithethe ngayo, kuba yimeko engaqhelekanga yemfuza enokuchaphazela abanye abantwana, ingakumbi amantombazana. Ngoko ke namhlanje siza kuthetha ngezinto ezininzi ngokulula, njengokuthi yintoni i-`(Incontinentia Pigmenti)`, zithini iimpawu zayo, kunye nendlela enyangwa ngayo.

Yintoni i-`(Incontinentia Pigmenti)`? Masiyiqonde ngokulula!

Ngamafutshane, i-Incontinentia Pigmenti sisifo semfuza esidityaniswe ne-X. Usenokucinga, "Owu, gqirha, andiyiqondi le nto." Kulungile, masiyenze ibe lula ngakumbi. Yimeko edluliselwa ngee-genes, oko kuthetha ukuba idluliselwa kumzali ukuya emntwaneni. Ikwiqela lezifo ezibizwa ngokuba zii-neurocutaneous disorders. Oku kuthetha ukuba ezi ntlobo zezifo zinokuchaphazela inkqubo yethu ye-nervous system (oko kukuthi, ingqondo kunye nomqolo), ulusu, amehlo, amazinyo, kunye nenkqubo yamathambo .

Kwimeko ye-`(IP)`, utshintsho oluthile (utshintsho) kwiijini zethu lubangela ukubonakala kancinci kancinci kwamabala kunye namabala eluswini. Olu luphawu oluphambili noluqhelekileyo lwesi sifo. La mabala eluswini anokubonwa xa umntu ezalwa, kwaye ngamanye amaxesha anokuvela nakwiiveki ezimbalwa emva kokuzalwa.

Kukho iintlobo ezine zamabala eluswini!

La mabala esikhumba anokuvela elinye ngexesha, ngamanqanaba amane. Ngamanye amaxesha la manqanaba anokulandelana, okanye elinye inqanaba linokuvela emva kwelinye. Cinga ngathi amaxesha onyaka ayatshintsha, kodwa ngamanye amaxesha elinye ixesha lonyaka liphela ngaphambi kokuba elinye liqale.

1. Inqanaba le-Vesicular: Kulapho kuvela khona amaqhuqhuva amancinci, abomvu, azele ngamanzi eluswini. Afana namaqhuqhuva avela ngexesha le-chickenpox. Angavela njengemicu emzimbeni wonke, ingakumbi emalungeni.

2. Inqanaba lokuqaqamba: Ekuhambeni kwexesha , amabala aphakamileyo, afana neentsumpa aqala ukuvela apho kwakukho khona amaqhuqhuva. Ngamanye amaxesha la maqhuqhuva anokuhlala iinyanga ezininzi.

3. Inqanaba lokutshintsha umbala: Okulandelayo, kuvela amabala angwevu, aluhlaza okwesibhakabhaka-mdaka eluswini. La mabala avela ngokungacwangciswanga emzimbeni nasemalungeni, afana neepateni ezimabula okanye imigca egobileyo . La mabala yenye yezizathu zokuba igama elithi `(Incontinentia Pigmenti)` liqanjwe. Igama elithi "Pigmenti" lithetha umbala. Eli nqanaba linokuhlala iminyaka.

4. Inqanaba lokutshintsha umbala: Ekugqibeleni, amabala amnyama aza kuthathelwa indawo ngamabala amhlophe, ngamanye amaxesha afana namabala . La maxesha amaninzi anokuhlala ubomi bonke.

Into ebalulekileyo kukuba ayinguye wonke umntu oza kuba nazo zonke ezi zihlandlo zine ngokulandelelana kwazo. Abanye abantu banokubona umzuzu omnye kuphela ngokucacileyo, ngelixa abanye banokubona ezininzi ngaxeshanye.

Ixesha elininzi, i-IP ibangelwa kukuguquka kwe-gene ebizwa ngokuba yi-NEMO (NF-kappaB essential modulator). Le gene ye-NEMO yi-gene ebalulekileyo enceda ukulawula ukusebenza kweeseli emzimbeni wethu kwaye ikwanceda ekufeni kweeseli okucwangcisiweyo. Ngelishwa, akukho nyango luthile lwe-IP okwangoku.

Ziziphi iingxaki ezinokubakho ze-`(Incontinentia Pigmenti)`?

I-IP ayisiyongxaki yesikhumba nje kuphela. Ngamanye amaxesha inokubangela nezinye iingxaki zempilo. Kubaluleke kakhulu ukuba sikwazi nokuyiqonda loo nto.

Iingxaki ze-neurological

• Ukuwohloka kobuchopho.
• Imingxunya emincinci kwi-white matter embindini wengqondo.
• Ukutshatyalaliswa kweeseli zemithambo-luvo kwi-cerebellar cortex.

Ngenxa yezi zizathu, malunga nama-20% abantwana abane `(IP)` banokuba nezi meko zilandelayo:

• Izakhono zokunyakaza ezicothayo (umz., ukukhasa okulibazisekileyo, ukuhamba okulibazisekileyo).
• Ukukhubazeka kwengqondo (njengokukhubazeka kokufunda).
• Ubuthathaka bemisipha.
• Ukuba nesithuthwane (ukuxhuzula).

Iingxaki zokubona

Abanye abantwana abane `(IP)` banokuba neengxaki zokubona. Umzekelo:

• Amehlo anqamlezileyo (strabismus).
• IiCataracts.
• Kwezinye iimeko ezinzima, ukulahleka ngokupheleleyo kombono kunokwenzeka .

Iingxaki zamazinyo

Iingxaki zamazinyo zinokubonwa nakwi-`(IP)`.

• Amanye amazinyo awekho.
• Amazinyo "amile okwesikhonkwane"
• Ukulibaziseka kokuqaqanjelwa kwamazinyo.

Yingakho kubaluleke kakhulu ukufuna ingcebiso kugqirha wamazinyo.

Yintoni `(Incontinentia Pigmenti Achromians)`?

Enye imeko efana nale yi-`(incontinentia pigmenti achromians)`. Kule meko, ulusu luba nemibimbi ekhanyayo, ekhanyayo (engenambala) kunye nemigca . Ngokungafaniyo namabala amnyama kwi-`(IP)`, le meko inamabala akhanyayo kuphela. Nangona kunjalo, njenge-`(IP)`, le meko `(incontinentia pigmenti achromians)` inokunxulunyaniswa neengxaki kwinkqubo yemithambo-luvo.

Iphathwa njani `(Incontinentia Pigmenti)`?

Njengoko besitshilo ngaphambili, akukho nyango luthile lwe-`(IP)` okwangoku. Nangona kunjalo, kukho iindlela ezahlukeneyo zonyango ezikhoyo zokulawula iimpawu kunye neengxaki ezibangelwa yile meko. Unyango lumiselwa ziimpawu zomntu ngamnye.

• Amabala esikhumba: Uninzi lwexesha, la mabala esikhumba, ingakumbi amaqhuqhuva kunye namabala arhabaxa avela kwizigaba zokuqala, ayaphela kancinci kancinci ngaphandle konyango, nokuba kukwiminyaka yokufikisa okanye kubudala.Ezo ndawo zimnyama (hyperpigmentation) kunye neendawo ezikhanyayo (hypopigmentation) zinokuhlala ixesha elide, ngamanye amaxesha ubomi bonke. Kodwa azisoloko zibangela ntlungu.
• Iimpawu zenkqubo yemithambo-luvo: Ukuba unesifo sokuxhuzula, ubuthathaka bemisipha, okanye ukuqaqamba kwemisipha okungaqhelekanga, ingcali yemithambo-luvo inokukunika amayeza kunye nezinye izixhobo zonyango .
• Iingxaki zokubona: Ukuba ubona kakubi, ugqirha wakho unokucebisa iiglasi, amayeza, okanye, kwiimeko ezinzima, utyando .
• Iingxaki zamazinyo: Iingcali zempilo yamazinyo zinokunyanga iingxaki zamazinyo. Zingenza izinto ezifana nokugcwalisa amazinyo kunye namazinyo okufakelwa.

Buza kuba njani ubomi xa kukho imeko ethi `(Incontinentia Pigmenti)`? (Ingxelo yokuxela kwangaphambili)

Le yingxaki kubantu abaninzi. Asinguye wonke umntu one-IP ofumana iingxaki ezinkulu. Abantu abaninzi, ingakumbi abo bangabi neengxaki ezinkulu ngexesha lobuntwana okanye ebuntwaneni, banokuphila ubomi obuqhelekileyo nobunempilo. Amabala e-pigment avela eluswini adla ngokunyamalala ngokuhamba kwexesha, kwaye ngamanye amaxesha anyamalale ngokupheleleyo.

Nangona kunjalo, abanye abantu banokuqhubeka beneengxaki zemithambo-luvo nezomzimba. Ngoko ke, ukuba wena okanye umntwana wakho une-IP, kungcono ukuthetha nogqirha wakho okanye umcebisi wezemfuza ukuze amisele isicwangciso sempilo esifanelekileyo kuwe.

Abaphandi okwangoku benza izifundo zokudibanisa i-genetic ukuze bafumane apho ii-genes ezinxulumene ne-neurocutaneous disorders ezifana ne-IP zikhoyo. Bakwafunda nendlela ezi nguqu ze-genetic ezichaphazela ngayo uphuhliso kunye nomsebenzi wengqondo kunye nenkqubo ye-nervous. Uphando olufana nolu luya kusinceda siqonde ngakumbi ngezifo ze-genetic kwaye lunokusivulela indlela yonyango lwexesha elizayo.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

• I-Incontinentia Pigmenti yimeko engaqhelekanga, yemfuza echaphazela kakhulu ulusu, kodwa inokuchaphazela nenkqubo yemithambo-luvo, amehlo, namazinyo.
• Amabala esikhumba angavela ngamanqanaba amane. La mabala adla ngokuphela ngokuhamba kwexesha.
• Asinguye wonke umntu ofumana iingxaki ezinkulu. Uninzi lwabantu lunokuphila ubomi obuqhelekileyo.
• Unyango lumiselwa ziimpawu. Ungacela uncedo kwiingcali ze-neurology, i-ophthalmology, kunye ne-dentistry.
• Ukuba wena okanye umntwana wakho ninenkxalabo malunga nale meko, funa ingcebiso kagqirha ngoko nangoko. Eyona nto ibalulekileyo kukungaxhalabi, ukufumana ulwazi olufanelekileyo, kunye nokufuna unyango olufunekayo.

Ndiyathemba ukuba olu lwazi luza kukunceda. Ukuba unemibuzo, ungabi nazintloni ubuze ugqirha. Sonke silapha ukukunceda.

` Incontinentia Pigmenti, IP, izifo zesikhumba, izifo zemfuza, i-NEMO gene, amabala esikhumba, izifo zemithambo-luvo, izifo zabantwana