Nevoid basal cell carcinoma syndrome

Nevoid basal cell carcinoma syndrome iboka rehurema hwakapfuura kuburikidza nemhuri. Iko kusagadzikana kunosanganisira ganda, tsinga system, maziso, endocrine gland, urinary uye kubereka masisitimu, uye mapfupa.

Zvinokonzera kutaridzika kwechiso zvisina kujairika uye njodzi yakakwira yegomarara reganda uye mamota asina cancer.

Nevoid basal cell carcinoma nevus syndrome ndiyo isingawanzoitika magene. Iyo huru geni yakabatana neiyo syndrome inozivikanwa sePTCH ("chigamba"). Yechipiri geni, inonzi SUFU, yakabatanidzwawo neichi chiitiko.

Kujairika mune aya majini kunowanzo pfuudzwa kuburikidza nemhuri seyakazvimirira hunhu hunhu. Izvi zvinoreva kuti unovandudza chirwere kana chero mubereki achikupfuudza jeni. Izvo zvakare zvinokwanisika kukudziridza iri geni kuremara isina nhoroondo yemhuri.

Zviratidzo zvikuru zvechirwere ichi ndezvi:

• Rudzi rwegomarara reganda unonzi basal cell carcinoma inokura panguva yekuyaruka
• Bundu risingakonzere shaya, rinodanwa kuti kerotocystic odontogenic bundu rinokurawo panguva yekuyaruka

Zvimwe zviratidzo zvinosanganisira:

• Mhino yakafara
• Yakachena muromo
• Inorema, yakabuda pahuma
• Shaya inonamira kunze (mune dzimwe nguva)
• Wide-set maziso
• Kugadzika pamichindwe uye pasi

Mamiriro acho anogona kukanganisa huta uye kutungamira ku:

• Matambudziko eziso
• Matsi
• Urema hwekuziva
• Kufenda
• Tumota dzehuropi

Mamiriro acho anotungamira mukukanganisa kwemapfupa, kusanganisira:

• Kukakavara kweshure (scoliosis)
• Kukomba kwakakomba kwemusana (kyphosis)
• Mbabvu dzisina kujairika

Panogona kuve paine nhoroondo yemhuri yechirwere ichi uye yakapfuura nhoroondo yemasalal cell ganda kenza.

Miedzo inogona kuratidza:

• Mamota ehuropi
• Cysts mushaya, izvo zvinogona kutungamira mukusanzwisisika kwemazino kukura kana shaya kutsemuka
• Zvinokanganisa muchikamu chine mavara (iris) kana lens yeziso
• Musoro kuzvimba nekuda kwemvura pauropi (hydrocephalus)
• Rib kusagadzikana

Miedzo inogona kuitwa inosanganisira:

• Echocardiogram yemoyo
• Genetic kuyedza (mune vamwe varwere)
• MRI yeuropi
• Ganda biopsy yemamota
• X-ray yemapfupa, mazino, uye dehenya
• Ultrasound yekutarisa mamota eovarian

Izvo zvakakosha kuti uongororwe nachiremba weganda (dermatologist) kazhinji, kuitira kuti kanzuru dzeganda dzinogona kurapwa dzichiri diki.

Vanhu vane chirwere ichi vanogona zvakare kuonekwa nekurapwa nevamwe vanamazvikokota, zvichienderana nekuti chikamu chipi chemuviri chakakanganiswa. Semuenzaniso, nyanzvi yekenza (oncologist) anogona kurapa mamota mumuviri, uye chiremba wemapfupa anogona kubatsira kurapa matambudziko emapfupa.

Kakawanda kuteedzera kwakasiyana-siyana kwehunyanzvi vanachiremba kwakakosha pakuva nemhedzisiro yakanaka.

Vanhu vane mamiriro aya vanogona kukura:

• Bofu
• Bundu rebongo
• Matsi
• Kutsemuka
• Ovarian mamota
• Cardiac fibromas
• Kukuvara kweganda uye kukuvara kwakakomba nekuda kwegomarara reganda

Bata wako mupi wezvehutano kuti uwane musangano kana:

• Iwe kana chero nhengo dzemhuri mune nevoid basal cell carcinoma syndrome, kunyanya kana uri kuronga kuva nemwana.
• Iwe une mwana ane zviratidzo zveichi chirwere.

Vakaroora vane nhoroondo yemhuri yeiyi syndrome vanogona kufunga nezvemajini kurairiswa vasati vaita nhumbu.

Kugara kunze kwezuva uye kushandisa sunscreen kunogona kubatsira kudzivirira nyowani basal cell ganda kenza.

Dzivisa radiation sezvakaita x-ray. Vanhu vane chirwere ichi vane hanya nemwaranzi. Kujekesa kune radiation kunogona kutungamira kumagomarara eganda.

NBCC chirwere; Gorlin chirwere; Gorlin-Goltz chirwere; Basal cell nevus chirwere (BCNS); Basal cell cancer - nevoid basal cell carcinoma syndrome

• Basal cell nevus syndrome - padyo nemuchindwe
• Basal cell nevus syndrome - makomba ekudyara
• Basal cell nevus syndrome - kumeso uye ruoko
• Basal sero nevus chirwere
• Basal cell nevus syndrome - kumeso

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Skelsey MK, Peck GL. Nevoid basal cell carcinoma syndrome. Mune: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Kurapa kweGanda Rezvirwere: Dzakakwana Therapeutic Strategies. 5 ed. Philadelphia, PA: Elsevier; 2018: chits. 170.

Walsh MF, Cadoo K, Salo-Mullen EE, Dubard-Gault M, Stadler ZK, Offit K. Genetic zvinhu: nhaka yekenza inotangira syndromes. Mune: Niederhuber JE, Armitage JO, Kastan MB, Doroshow JH, Tepper JE, eds. Abeloff's Clinical Oncology. 6th ed. Philadelphia, PA: Elsevier; 2020: chits. 13.