Ciwon Hunter: menene shi, ganewar asali, alamomi da magani

Wadatacce

• Kwayar cututtukan cututtukan maharbi
• Yadda ake ganewar asali
• Jiyya don cutar Hunter

Ciwon Hunter, wanda aka fi sani da Mucopolysaccharidosis type II ko MPS II, cuta ce ta kwayar halitta wacce ba a cika samun ta ba ga maza wanda ke da karancin enzyme, Iduronate-2-Sulfatase, wanda ke da mahimmanci ga aikin jiki daidai.

Saboda raguwar aikin wannan enzyme, akwai tarin abubuwa a cikin sel, wanda ke haifar da mummunan bayyanar cututtuka da ci gaba mai saurin ci gaba, kamar ƙarfin gwiwa, haɗin zuciya da canjin numfashi, bayyanar cututtukan fata da canje-canje na jijiyoyin jiki, misali .

Kwayar cututtukan cututtukan maharbi

Alamomin cututtukan Hunter, saurin ci gaban cuta da tsananin su sun bambanta daga mutum zuwa mutum, babban halayen cutar shine:

• Canje-canje na jijiyoyi, tare da yiwuwar karancin tunani;
• Hepatosplenomegaly, wanda shine faɗaɗa hanta da baƙin ciki, wanda ke haifar da faɗaɗa ciki;
• Starfin haɗin gwiwa;
• Fuskar da ba ta da kyau kuma ba ta dace ba, tare da babban kai, hanci mai fadi da lebe mai kauri, misali;
• Rashin ji;
• Rushewar kwayar ido;
• Matsalar motsi;
• Yawan cututtukan numfashi;
• Matsalar magana;
• Bayyanar raunin fata;
• Kasancewar hernias, akasarin cibiya da inguinal.

A cikin mafi munanan yanayi kuma za a iya samun canje-canje na zuciya, tare da rage aikin zuciya, da canje-canje na numfashi, wanda na iya haifar da toshewar hanyar iska da haɓaka damar kamuwa da cututtuka na numfashi, wanda na iya zama mai tsanani.

Saboda gaskiyar cewa bayyanar cututtuka suna bayyana kuma suna canzawa daban tsakanin marasa lafiya da cutar, tsawon rai kuma yana da canji, tare da mafi girman damar mutuwa tsakanin shekarun farko da na biyu na rayuwa lokacin da alamun suka fi tsanani.

Yadda ake ganewar asali

Ganewar cutar Hunter Syndrome ana yin ta ne daga likitan halittu ko babban likita bisa ga alamun cututtukan da mutum ya gabatar da kuma sakamakon takamaiman gwaje-gwaje. Yana da mahimmanci cewa ba a yin binciken ne kawai bisa ga alamun asibiti, saboda halayen suna da kamanceceniya da na sauran mucopolysaccharidoses, kuma yana da mahimmanci likita ya ba da umarnin ƙarin takamaiman gwaje-gwaje. Ara koyo game da mucopolysaccharidosis da yadda ake gano shi.

Don haka, yana da mahimmanci a auna glycosaminoglycans a cikin fitsari kuma, galibi, a kimanta matakan aiki na enduma Iduronate-2-Sulfatase a cikin fibroblasts da plasma. Bugu da kari, sauran gwaje-gwajen galibi ana ba da shawarar ne don duba tsananin alamun, kamar su duban dan tayi, gwaje-gwajen don tantance karfin numfashi, na’urar sauraren sauti, gwaje-gwajen jijiyoyin jiki, gwajin ido da yanayin kwanyar da kashin baya, misali.

Jiyya don cutar Hunter

Maganin cutar Hunter Syndrome ya bambanta gwargwadon halaye da mutane ke gabatarwa, duk da haka yawanci likita yana ba da shawarar yin aikin maye gurbin enzyme don hana ci gaban cutar da bayyanar rikitarwa.

Bugu da ƙari, likita ya ba da shawarar takamaiman magani don bayyanar cututtukan da aka gabatar da maganin aiki da maganin jiki domin motsa magana da motsin marasa lafiya da ke fama da cutar don hana haɗarin mota da magana, misali.