Xeroderma pigmentosum

Xeroderma pigmentosum (XP) yanayi ne mai wuya wanda ya wuce ta cikin dangi. XP na sanya fata da kyallen dake rufe ido su zama masu matukar damuwa da hasken ultraviolet (UV). Wasu mutane kuma suna haɓaka matsalolin tsarin damuwa.

XP cuta ce ta maye gurbin mutum. Wannan yana nufin dole ne ku sami kwafi 2 na kwayar halitta ta al'ada don cutar ko halayenku su haɓaka. Rikicin ya gaji mahaifanka ne da mahaifinka a lokaci guda. Kwayar halittar da ba ta da kyau ba safai ake samu ba, don haka damar da iyayen biyu suke da ita na haihuwarsu ba su da yawa. A saboda wannan dalili, da wuya wani ya sami yanayin ya mika shi ga tsara mai zuwa, kodayake yana yiwuwa.

Hasken UV, kamar daga hasken rana, yana lalata kayan halittar (DNA) a cikin ƙwayoyin fata. A yadda aka saba, jiki yana gyara wannan lalacewar. Amma a cikin mutane masu cutar XP, jiki baya gyara lahani. A sakamakon haka, fatar tana samun siriri sosai kuma faci masu launuka daban-daban (launin launuka masu launi) suna bayyana.

Kwayar cutar galibi tana bayyana ne lokacin da yaro ya cika shekaru 2 da haihuwa.

Alamar fata ta hada da:

• Kunar rana a kunne wanda baya warkewa bayan ɗan gajeren hasken rana
• Fusho bayan ɗan gajeren hasken rana
• Gizo-gizo kamar magudanar jini a ƙarƙashin fata
• Manyan launuka masu canza launi waɗanda suke taɓarɓarewa, suna kama da tsananin tsufa
• Kwashe fata
• Fatawar fata
• Fuskantar ɗanyen fata
• Rashin kwanciyar hankali lokacin da kake cikin haske mai haske (photophobia)
• Ciwon kansa na fata a ƙuruciya ƙuruciya (gami da melanoma, ƙananan ƙwayoyin cuta, ƙananan ƙwayoyin cuta)

Alamun ido sun hada da:

• Ido ya bushe
• Girman girji
• Ceunƙun ciki na ƙashin ciki
• Kumburi ko kumburin fatar ido
• Ciwon daji na fatar ido, larura ko ta kwalara

Symptomswayar cututtukan ƙwayoyin cuta (neurologic), waɗanda ke ci gaba a cikin wasu yara, sun haɗa da:

• Rashin hankali
• Rashin jinkiri
• Rashin ji
• Raunin jijiyoyi na kafafu da hannaye

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki, yana ba da kulawa ta musamman ga fata da idanu. Mai ba da sabis ɗin zai yi tambaya game da tarihin iyali na XP.

Gwajin da za a iya yi sun hada da:

• Biopsy na fata wanda ake yin nazarin ƙwayoyin fata a cikin dakin gwaje-gwaje
• Gwajin DNA don kwayar cutar

Gwaje-gwaje masu zuwa na iya taimakawa wajen gano yanayin cikin jariri kafin haihuwa:

• Amniocentesis
• Samfurin Chorionic villous
• Al'adar kwayoyin amniotic

Mutanen da ke da XP suna buƙatar cikakken kariya daga hasken rana. Hatta hasken da ke shigowa ta tagogi ko daga kwararan fitila na da hadari.

Lokacin fitarwa a cikin rana, dole ne a sa tufafin kariya.

Don kare fata da idanu daga hasken rana:

• Yi amfani da hasken rana tare da mafi girman SPF da zaku iya samu.
• Sanye rigunan dogon hannu da dogon wando.
• Sanya tabarau wanda yake toshe hasken UVA da UVB. Koya koyawa yaranku sanya tabarau koyaushe a waje.

Don hana kansar fata, mai bayarwa na iya rubuta magunguna, kamar su mayukan kare, don shafa wa fata.

Idan cutar kansa ta kama, za a yi tiyata ko wasu hanyoyin don kawar da cutar kansa.

Waɗannan albarkatun zasu iya taimaka muku ƙarin sani game da XP:

• NIH Tsarin Gida na Gida - ghr.nlm.nih.gov/condition/xeroderma-pigmentosum
• Xeroderma Pigmentosum Society - www.xps.org
• Supportungiyar Tallafin Iyali ta XP - xpfamilysupport.org

Fiye da rabin mutanen da ke da wannan yanayin suna mutuwa ne saboda cutar kansa a lokacin da suka girma.

Kira don alƙawari tare da mai bada idan ku ko yaranku suna da alamun cutar XP.

Masana sun ba da shawarar ba da shawara kan kwayar halitta ga mutanen da ke da tarihin iyali na XP waɗanda ke son haihuwa.

• Chromosomes da DNA

Bender NR, Chiu YE. Hannun hotuna. A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 675.

Patterson JW. Rashin lafiya na epidermal maturation da keratinization. A cikin: Patterson JW, ed. Ilimin Lafiyar Weedon. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2016: babi na 9.