Adrenoleukodystrophy: menene shi, bayyanar cututtuka da magani

Wadatacce

• Kwayar cututtukan adrenoleukodystrophy
• Yadda ake yin maganin

Adrenoleukodystrophy cuta ce mai saurin yaduwa daga kwayar halitta wacce ke da nasaba da X chromosome, wanda a ciki akwai karancin adrenal da kuma tara abubuwa a cikin jiki wanda ke inganta demyelination na axons, wanda shine ɓangaren jijiyoyin da ke da alhakin gudanar da sigina na lantarki, kuma yana iya kasancewa cikin magana, hangen nesa ko cikin ragi da shakatawa na tsokoki, misali.

Don haka, kamar yadda yake a cikin adrenoleukodystrophy, siginar juyayi na iya lalacewa, yana yiwuwa alamun da alamomin da ke da alaƙa da wannan yanayin na iya tashi tsawon lokaci, tare da canje-canje a cikin magana, wahalar haɗiye da tafiya, da canje-canje a halaye, misali.

Wannan cutar ta fi yawan faruwa ga maza, tunda maza suna da chromosome 1 X kawai, yayin da mata dole ne duk chromosomes da aka canza su kamu da cutar. Bugu da kari, alamomi da alamomi na iya bayyana a kowane zamani, ya danganta da tsananin canjin halittar mutum da saurin abin da ke haifar da lalata mutum.

Kwayar cututtukan adrenoleukodystrophy

Kwayar cututtukan adrenoleukodystrophy suna da alaƙa da canje-canje a cikin aikin gland adrenal da demyelination na axons. Gland din adrenal yana sama da kodan kuma suna da alaƙa da samar da abubuwa waɗanda ke taimakawa daidaita tsarin juyayi na kai, inganta haɓaka wasu ayyuka na jiki, kamar numfashi da narkewa, misali. Sabili da haka, lokacin da akwai lalata ko asarar aiki, ana lura da canje-canje a cikin tsarin juyayi.

Bugu da kari, saboda canjin halittar, yana yiwuwa a tara abubuwa masu guba a cikin jiki, wanda zai iya haifar da asarar jijiyar myelin ta axons, hana yaduwar siginonin lantarki da haifar da alamun halaye da alamun adrenoleukodystrophy.

Don haka, ana fahimtar alamun adrenoleukodystrophy yayin da mutum ya haɓaka kuma ana iya tabbatar dashi:

• Rashin aikin gland na adrenal;
• Rashin ikon magana da ma'amala;
• Canje-canje na hali;
• Strabismus;
• Matsaloli cikin tafiya;
• Wahala a cikin ciyarwa, da ciyarwa ta cikin bututu na iya zama dole;
• Matsalar haɗiye;
• Rashin iyawar fahimta;
• Vunƙwasawa.

Yana da mahimmanci adrenoleukodystrophy an gano shi daidai lokacin haihuwa, saboda yana yiwuwa a rage saurin da alamun ke bayyana, inganta ingancin rayuwar jariri.

Yadda ake yin maganin

Maganin adrenoleukodystrophy shine dashewar kashin kashi, wanda aka bada shawarar lokacin da alamomin suka riga suka ci gaba sosai kuma akwai canjin kwakwalwa mai tsanani. A cikin ƙananan lamuran, likita na iya bayar da shawarar maye gurbin homonin da gland adrenal ya samar, ban da maganin jiki don hana ƙwayar tsoka.