Isifo se-Nevoid basal cell carcinoma syndrome

I-Nevoid basal cell carcinoma syndrome liqela leziphene ezidlulileyo kwiintsapho. Ukuphazamiseka kubandakanya ulusu, inkqubo yeemvakalelo, amehlo, amadlala endocrine, iinkqubo zokuchama kunye nokuzala, kunye namathambo.

Kubangela ukubonakala kobuso okungaqhelekanga kunye nomngcipheko ophezulu kumhlaza wesikhumba kunye nezidumba ezingenamhlaza.

I-Nevoid basal cell carcinoma nevus syndrome yimeko enqabileyo yemfuza. Uhlobo oluphambili oludityaniswe nesifo saziwa njenge-PTCH ("iipatches"). Uhlobo lwesibini lofuzo, olubizwa ngokuba yi-SUFU, nalo luye lwayanyaniswa nale meko.

Ukungaqheleki kwezi zofuzo kuhlala kudlula kwiintsapho njengeyona nto iphambili kuyo. Oku kuthetha ukuba uhlakulela isifo ukuba ngaba umzali othile udlulisela uhlobo kuhlobo lwakho. Kuyenzeka ukuba uphuhlise esi siphene semfuza ngaphandle kwembali yosapho.

Iimpawu eziphambili zesi sifo zezi:

• Uhlobo lomhlaza wolusu obizwa ngokuba yi-basal cell carcinoma ekhula ngexesha lokufikisa
• Ithumba elingahlanjululwanga lomhlathi, elibizwa ngokuba yi-kerotocystic odontogenic tumor ekhula kwakhona ngexesha lokufikisa

Ezinye iimpawu zibandakanya:

• Impumlo ebanzi
• Inkalakahla ecwengileyo
• Inzima, ibrawuza evelayo
• Umhlathi ophuma ngaphandle (kwezinye iimeko)
• Amehlo abekwe ngokubanzi
• Ukubetha ezintendeni nasezandleni

Imeko inokuchaphazela inkqubo ye-nervous kwaye ikhokelele:

• Iingxaki zamehlo
• Izithulu
• Ukukhubazeka kwengqondo
• Ukuxhuzula
• Ukudumba kwengqondo

Le meko ikwakhokelela kwiziphene zethambo, kubandakanya:

• Ukujika komqolo (scoliosis)
• Ukujika okunamandla komva (kyphosis)
• Iimbambo ezingaqhelekanga

Kunokubakho imbali yosapho kwesi sifo kunye nembali edlulileyo yesifo somhlaza wolusu.

Uvavanyo lunokutyhila:

• Amathumba ebongo
• Ama-cysts emhlathini, anokukhokelela kuphuhliso lwezinyo olungaqhelekanga okanye ukwaphuka kwemihlathi
• Iziphene kwinxalenye enemibala (iris) okanye ilensi yeliso
• Ukudumba kwentloko ngenxa yolwelo kwingqondo (i-hydrocephalus)
• Ukungaqheleki kweRib

Uvavanyo olunokwenziwa lunokubandakanya:

• I-Echocardiogram yentliziyo
• Ukuvavanywa kwemvelo (kwezinye izigulana)
• I-MRI yengqondo
• Ulusu lwe-biopsy yamathumba
• IX-reyi yamathambo, amazinyo, kunye nokakayi
• I-Ultrasound ukujonga amathumba e-ovari

Kubalulekile ukuba uvavanywe ngugqirha wolusu (ugqirha wesikhumba) rhoqo, ukuze umhlaza wolusu unyangwe ngelixa usemncinci.

Abantu abanale ngxaki banokubonwa kwaye banyangwe zezinye iingcali, kuxhomekeka ukuba leliphi na ilungu lomzimba elichaphazelekayo. Umzekelo, ingcali yomhlaza (oncologist) inokunyanga amathumba emzimbeni, kwaye ugqirha wamathambo unokunceda ukunyanga iingxaki zethambo.

Ukulandelwa rhoqo kweentlobo ezahlukeneyo zoogqirha kubalulekile ukuba ube neziphumo ezilungileyo.

Abantu abakule meko banokuphuhlisa:

• Ubumfama
• Ithumba lobuchopho
• Izithulu
• Ukuqhekeka
• Amathumba e-Ovarian
• Iifibromas zentliziyo
• Ukonakala kolusu kunye nokuqunjelwa kakhulu ngenxa yomhlaza wolusu

Nxibelelana nomboneleli wakho wezempilo ukuze ufumane idinga ukuba:

• Wena okanye nawaphi na amalungu osapho ane-basal cell carcinoma syndrome, ngakumbi ukuba ucwangcisa ukuba nomntwana.
• Unomntwana oneempawu zesi sifo.

Izibini ezinembali yosapho yesi sifo zinokuthi ziqwalasele iingcebiso ngemfuzo ngaphambi kokuba zikhulelwe.

Ukuhlala ngaphandle elangeni kunye nokusebenzisa i-sunscreen kunokunceda ukukhusela umhlaza womhlaza wesiseko somzimba.

Gwema imitha efana ne-x-ray. Abantu abakule meko banovakalelo kakhulu kwimitha. Ukubonakaliswa kwimitha kungakhokelela kumhlaza wesikhumba.

Isifo seNBCC; Isifo seGorlin; Isifo se-Gorlin-Goltz; Isiseko se-nevus syndrome (BCNS); Umhlaza wesiseko se-basal-nevoid basal cell carcinoma syndrome

• Isiseko se-nevus syndrome - ukuvala kwesundu
• Isiseko se-nevus syndrome - imingxunya yezityalo
• Isiseko se-nevus syndrome - ubuso kunye nesandla
• Isiseko se-nevus syndrome
• Isiseko se-basal nevus syndrome - ubuso

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ISkelsey MK, iPeck GL. Isifo se-Nevoid basal cell carcinoma syndrome. Ku: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, ii-eds. Unyango lwezifo zolusu: Amacandelo oBuchule oNyango. Umhla we-5. IPhiladelphia, PA: Elsevier; I-2018: isahluko 170.

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