EBSTEM (2015)

Recessive dystrophic epidermolysis bullosa (RDEB) yana da asali da raƙuman fata mai saurin lalacewa don amsa ko da ƙananan rauni. Duk da cewa shigar da fata ita ce mafi bayyananniyar sigar asibiti, wasu sun haɗa da anemia mai zurfi, gazawar haɓakawa, jinkirin girma, tabo na nama da ke rufe tsarin narkewar abinci (wani lokaci yana haifar da 'rufewa' na esophagus), da zazzagewar corneal (ido) tabo. Ko da yake ba a fahimci gaba ɗaya yawancin waɗannan tasirin ba saboda ƙarar kumburi. A halin yanzu babu ingantaccen magani ga RDEB kuma ba za mu iya hana rikitarwa ba.

Mesenchymal stromal Kwayoyin (MSCs) su ne sel waɗanda ke ware daga jinin cibiya, bargon kashi da sauran tushe. A wasu lokuta ana kiran su ƙananan ƙwayoyin cuta kuma suna iya girma cikin nau'in tantanin halitta daban-daban, amma mahimmanci a nan, suna iya haifar da amsawar anti-mai kumburi kuma an nuna su don amfanar mutane da dama masu kumburi.

Wannan binciken zai kimanta ko MSCs na iya samar da fa'ida a cikin fata na yara masu RDEB. Ƙirƙirar sababbin jiyya na warkewa tsari ne mai sauƙi kuma bayan kimantawar dakin gwaje-gwaje, matakin farko na ci gaba a cikin mutane shine tabbatar da cewa maganin yana da lafiya kuma baya haifar da wani sakamako maras so. Wannan binciken shine farkon kimantawa a cikin marasa lafiya. Mahalarta binciken goma suna karɓar intravenous guda uku (a cikin rafin jini) infusions na MSC sama da wata ɗaya tare da ƙwayoyin da aka ɗauka daga kasusuwan kasusuwa na masu ba da gudummawa lafiya. Ana bin mutanen da ke cikin binciken har tsawon watanni 12.

Waɗanda ke shiga ana tantance su don duk wani tasiri na gaggawa da ke da alaƙa da jiko na MSCs kuma daga baya yanayin fatar su (ƙumburi da ƙarfin fata), ƙwarewar zafi, barci, gajiya da ingancin rayuwa. Za a bincika alamun da ke da hannu a cikin martanin kumburi a cikin kyallen takarda.

Har zuwa yau an yi duk infusions ba tare da wani mummunan tasiri ba. Wasu iyaye sun ba da rahoton samun ci gaba a yanayin fatar ɗansu, da kuma tasiri mai kyau ga rayuwar iyali. Duk da haka, waɗannan rahotannin anecdotal ne na wucin gadi kuma binciken yana ci gaba da faɗaɗa kimar kimiyya na sakamakon. Wannan binciken zai taimaka wajen fahimtar hanyoyin kumburi a cikin RDEB da kuma samar da zaɓuɓɓukan hanyoyin warkewa.

"A cikin shekaru 25 na bincike na akan RDEB, wannan shine kawai lokacin da na ga wani magani ya canza yanayin yanayin. Sakamakon yana da ban sha'awa da gaske kuma allurar MSCs ba ta haifar da damuwa na aminci ba. Har ila yau, yana da mahimmanci a lura cewa wannan ba magani bane kuma amfanin ya ƙare bayan kimanin watanni 6."

"Ci gaba, ba za mu fara gabatar da maimaita jiko na MSCs cikin kulawar asibiti na NHS na yau da kullun ba. Amma muna kuma fara sabbin gwaje-gwaje na asibiti - hada kwayoyin halitta da maganin kwayoyin halitta don baiwa marasa lafiya da RDEB, yara da manya, har ma da ingantattun jiyya yayin da muke neman maganin wannan mummunan yanayin fata da aka gada."

Farfesa John McGrath

Farfesa John McGrath

John McGrath MD FRCP FMedSci Farfesa ne na Kwayoyin cututtukan fata a Kwalejin King London kuma Shugaban Sashin Cututtukan Fatar Halittu, da kuma mai ba da shawara ga likitan fata a St John's Institute of Dermatology, Guy's da St Thomas' NHS Foundation Trust a London. A baya ya kasance ɗan ƙaramin ɗan bincike na ED mai tallafin DEBRA kuma ya yi aiki akan binciken EB sama da shekaru 35. Yanzu yana jagorantar da haɗin kai akan ayyuka da yawa na ƙasa da ƙasa don haɓaka kwayoyin halitta, tantanin halitta, furotin da magungunan ƙwayoyi waɗanda zasu iya haifar da ingantattun jiyya ga mutanen da ke zaune tare da EB.