Lamellar ichthyosis: menene, alamu da magani

Wadatacce

• Sanadin lamiclar ichthyosis
• Babban bayyanar cututtuka
• Yadda ake ganewar asali
• Jiyya don lamellar ichthyosis

Lamellar ichthyosis cuta ce mai saurin yaduwar kwayar halitta wacce ke da alaƙa da canje-canje a cikin samuwar fata saboda maye gurbi, wanda ke ƙara haɗarin kamuwa da cututtukan jiki, baya ga akwai kuma canjin ido, raunin hankali da rage samar da zufa.

Saboda yana da alaƙa da maye gurbi, ichthyosis na lamellar ba shi da magani kuma, don haka, ana yin magani da nufin sauƙaƙa alamomi da haɓaka ƙimar rayuwar mutum, yana buƙatar amfani da mayukan shafawa wanda likitan fata ya ba da shawarar don kauce wa taurin fata da kiyayewa ya danshi.

Sanadin lamiclar ichthyosis

Lamellar ichthyosis na iya haifar da maye gurbi a cikin kwayoyin halitta da yawa, duk da haka maye gurbi a cikin kwayar halittar TGM1 shine mafi alaqa da aukuwar cutar. A karkashin yanayi na yau da kullun, wannan kwayar halitta tana inganta samuwar cikin adadi mai yawa na transglutaminase 1, wanda ke da alhakin samuwar fata. Koyaya, saboda maye gurbi a cikin wannan kwayar halitta, adadin transglutaminase 1 ya lalace, kuma ƙila za a sami ƙarami ko ƙarancin wannan furotin, wanda ke haifar da canjin fata.

Tunda wannan cutar ta kasance koma baya ne ga mutum, don mutum ya kamu da cuta, ya zama dole iyaye biyu su dauki wannan kwayar domin yaro ya sami maye gurbi kuma cutar ta auku.

Babban bayyanar cututtuka

Lamellar ichthyosis shine mafi tsananin nau'in ichthyosis kuma ana nuna shi da saurin ɓaɓɓewar fata, yana haifar da bayyanar ɓarkewar fata da yawa a cikin fata wanda zai iya zama mai raɗaɗi ƙwarai, yana ƙara haɗarin kamuwa da cututtuka da kuma tsananin bushewar jiki da rage motsi, tunda a can Hakanan yana iya zama taurin fata.

Baya ga yin baje-baje, yana yiwuwa ga masu cutar lamellar ichthyosis su fuskanci alopecia, wanda yake shi ne asarar gashi da gashi a sassan jiki daban-daban, wanda hakan na iya haifar da rashin haƙuri da zafi. Sauran cututtukan da za a iya gano su ne:

• Canjin ido;
• Juyawa daga fatar ido, wanda aka sani a kimiyance yadda yake;
• Kunnen manne;
• Rage cikin samar da gumi, wanda ake kira hypohidrosis;
• Microdactyly, wanda ƙarami ko ƙaramin yatsu ke samuwa;
• Lalacewar kusoshi da yatsu;
• Gajere;
• Rashin hankali;
• Rage ƙarfin ji saboda tarin ma'aunin fata a cikin mashigar kunne;
• Thicknessara kaurin fata a hannaye da ƙafa.

Mutanen da ke dauke da lamellar ichthyosis suna da tsammanin rayuwarsu ta al'ada, amma yana da muhimmanci a dauki matakai don rage barazanar kamuwa da cutar. Bugu da kari, yana da mahimmanci su kasance tare da masana halayyar dan adam, tunda saboda nakasassu da yawa da kuma yawan awo za su iya fuskantar wariya.

Yadda ake ganewar asali

Ganewar ƙwayar lamellar ichthyosis yawanci ana yin sa ne a lokacin haihuwa, kuma yana yiwuwa a tabbatar cewa an haife jaririn da fata mai launin rawaya da fasa. Koyaya, don tabbatar da ganewar asali, jini, kwayar halitta da gwajin immunohistochemical sun zama dole, kamar kimanta aikin enzyme TGase 1, wanda ke aiwatar da tsarin samuwar transglutaminase 1, tare da raguwar aikin wannan enzyme a cikin lamellar ichthyosis.

Bugu da kari, ana iya yin gwaje-gwajen kwayoyin don gano kwayar halittar TGM1, duk da haka wannan gwajin yana da tsada kuma babu shi ta Hukumar Kiwan lafiya (SUS).

Haka kuma yana yiwuwa a gudanar da bincike har ma a lokacin daukar ciki ta hanyar nazarin DNA ta amfani da amniocentesis, wanda shine jarrabawa wacce a ke dauke samfurin ruwan mahaifa daga cikin mahaifa, wanda ke dauke da kwayoyin jarirai kuma wanda za a iya kimanta dakin gwaje-gwaje don gano kowane canjin halittar. Koyaya, ana ba da shawarar irin wannan gwajin ne kawai idan akwai yanayin lamellar ichthyosis a cikin iyali, musamman dangane da alaƙar da ke tsakanin dangi, saboda iyaye suna iya zama masu ɗauke da maye gurbin kuma don haka su miƙa shi ga ɗansu.

Jiyya don lamellar ichthyosis

Maganin cutar lamellar ichthyosis na da nufin sauƙaƙe alamomin da inganta ƙimar rayuwar mutum, tunda cutar ba ta da magani. Don haka, yana da mahimmanci a gudanar da maganin bisa ga likitan fata ko kuma babban likitan da ke jagorantar, ana ba da shawarar shaƙuwa da amfani da wasu ƙwayoyi masu alhakin kula da bambancin kwayar halitta da kula da kamuwa da cuta, tunda a matsayin fata, wanda shine farkon shinge na kariya daga kwayar halitta, ta lalace a cikin lamellar ichthyosis.

Kari akan haka, ana iya bada shawarar amfani da wasu mayuka don sanya fatar jiki danshi, cire busassun yatsun fatar kuma hana ta zama mai tauri. Fahimci yadda za a yi maganin ichthyosis.