Gaggioli 2 (2017)

Recessive dystrophic epidermolysis bullosa (RDEB) yana da alaƙa da kumburin fata, tabo mai laushi da nakasar mitten (inda fata ke rufe yatsu da yatsun kafa wanda ke haifar da mitten-kamar bayyanar hannaye ko ƙafa). Kumburi na fata yana faruwa ne saboda rashin furotin fata, wanda ake kira collagen VII, wanda ke haifar da rabuwa daga epidermis (launi na waje) daga dermis da ke ciki kuma tabo ya faru ne saboda rashin lafiyan rauni da kuma sama da samar da nama mai fibrous. Wadannan halayen fata marasa al'ada kuma akai-akai suna haifar da irin ciwon daji na fata da ake kira squamous cell carcinoma (SCC).

An gano cewa a cikin fatar mutanen da ke dauke da RDEB akwai kwayoyin da ke da alaƙa da kumburi a cikin yanki na blister. An san kumburi yana da alaƙa da haɓakar ciwon daji, don haka waɗannan ƙwayoyin cuta na iya ba da gudummawa ga farkon SCC.

Ana samar da wakilai masu mahimmanci, wanda ake kira cytokines, a lokacin kumburi. An nuna su suna shafar ƙwayoyin fata na al'ada (fibroblasts) kuma suna sa su zama Carcinoma Associated Fibroblasts (CAF). Wadannan kwayoyin suna da hannu wajen canza tsarin nama na fata (sake gyarawa) kuma a cikin wannan halin da ake ciki yana inganta ci gaban ciwon daji. CAFs na iya taka rawa a cikin samuwar SCC a cikin RDEB.

Saƙonni tsakanin cytokines suna haifar da juyawa zuwa CAFs. Ana fatan idan za a iya katse wadannan sakonni ko kuma a daina to akwai yiwuwar a daina kamuwa da cutar daji. An gano takamaiman kwayar halitta, Janus kinase (JAK) a cikin wannan hanyar saƙon. Ana iya toshe JAK ta hanyar magani, Ruxolitinib wanda Hukumar Abinci da Magunguna (FDA) ta riga ta amince da ita a cikin Amurka, don wani yanayi.

A ƙoƙarin sake haifar da abin da ke faruwa a cikin mutane a cikin dakin gwaje-gwaje, an nuna cewa fibroblasts da aka bi da su tare da cytokines sun zama CAFs, amma Ruxolitinib na iya hana ayyukan su na lalata. Wannan rukunin bincike don haka yana so ya duba ko Ruxolitinib zai iya zama wakili mai amfani ga marasa lafiya na RDEB da ke fama da kumburi na gida da fibrosis da ke hade da raunin rauni mara kyau kuma a cikin dogon lokaci, yana hana ci gaban SCC mai tsanani.

"Wannan binciken da DEBRA ta ba da tallafi ya ba mu damar gabatar da binciken kimiyya wanda zai iya haifar da haɓaka wata hanyar warkewa madadin maganin ciwon daji na EB."

Dr Cedric Gaggioli

 

Dr Cedric Gaggioli

Dr Cedric Gaggioli babban mai bincike ne a cibiyar IRCAN da ke Nice, Faransa. Abubuwan bincikensa sun fi mayar da hankali kan fahimtar tsarin kwayoyin halitta da tsarin salula na yanayin tumor, juyin halittarsa ​​da kuma musamman, wajen tantance rawar da kwayoyin da ba su da cutar kansa yayin ci gaban kansa da ci gaba. Dr Gaggioli yana da sha'awa mai ƙarfi ga ciwon daji na squamous cell wanda ya taso daga marasa lafiya na RDEB kuma yana ƙoƙari ya gano sababbin hanyoyin warkewa don magance ci gaban irin waɗannan cututtuka masu tsanani.