Duchenne murdede dystrophy da Becker: magani

Kwayoyin cututtuka kasance a cikin rukuni na nauyi pathologies da magani daga wanda a yau shi ne wuya. Daga cikin wadannan chromosomal munanan ake samu daban-daban take hakki. Da yawa daga cikinsu suna da neurologic bayyanar cututtuka. Misalai ne Duchenne murdede dystrophy, Becker. Wadannan cututtuka ci gaba a cikin shimfiɗar jariri, kuma suna da m hanya. Duk da ci gaba na neuroscience, irin pathologies ne da wuya mu bi. Wannan shi ne saboda chromosomal canje-canje da cewa an kwantar da lokacin da samuwar jiki.

Description of Duchenne murdede dystrophy

Duchenne murdede dystrophy - a kayyade cutar halin m tsoka cuta naúrar. A Pathology ne rare. Yaduwar na anomaly ne kamar 3 persons da 10 000 maza. Cuta a kusan dukkan lokuta, rinjayar boys. Duk da haka, ci gaban Duchenne murdede 'yan mata ba su cire. Wannan Pathology bayyana kanta a farkon yara.

Sauran cututtuka da cewa suna da wannan haddasawa da kuma bayyanar cututtuka ne myodystrophy Becker. Shi yana da mafi m hanya. tsoka nama lalacewar auku yawa daga baya - a samartaka. A wannan yanayin, cututtuka ci gaba sannu a hankali, kuma cikin haƙuri rike da ikon yin aiki a cikin 'yan shekaru. Kamar yadda da Duchenne murdede dystrophy, wannan Pathology ne na kowa daga namiji yawan jama'a. A abin da ya faru shi ne 1 person for 20 dubu boys.

Duchenne murdede dystrophy: neuroimmunology cuta

A dalilin duka biyu pathologies ta'allaka a take hakkin da X chromosome. Canjin da faruwa a Duchenne da Becker murdede dystrophy, karatu a cikin 30-ies karni na karshe. Duk da haka etiological far ya ba tukuna aka samu. anomalies recessive yanayin na gādo. Wannan yana nufin cewa idan mahaukaci gene ne ba a daya daga cikin iyaye, yiwuwar samun wani rashin lafiya yaro haihuwa ne 25%. A X chromosome ne mafi tsawo a cikin jiki. Tare da iri dystrophies warwarewarsu auku a wanda kuma wannan wuri (p21). Wannan lalacewa take kaiwa zuwa wani karu a gina jiki kira, wanda shi ne wani ɓangare daga cell membranes na tsoka nama. Duchenne murdede dystrophy a lokacin da ya ke gaba daya ba a nan. Saboda haka, keta faruwa yawa a baya. Becker murdede dystrophy a lokacin da mai gina jiki da aka hada a adadi kaɗan ko ne pathological.

The asibiti hoto na murdede dystrophy

Duchenne murdede dystrophy ne halin da raunuka na neuromuscular tsarin. A cuta iya zargin yana da shekaru 2-3 shekaru. A wannan lokacin, shi ya zama na fili da cewa yaro ~ uka komai, a cikin jiki ci gaban da takwarorinsu, ke bad, gudanar da tsalle. Saboda haka da yara wuya su yi hau matakala, ta sau da yawa fada. Da shan kashi na tsokoki fara da ƙananan extremities. Daga baya, ya rufe duk proximal tsokoki. Degeneration faruwa a cikin sama kafada abin ɗamara, quadriceps. A wadannan wurare akwai thinning na tsokoki. A tsawon shekaru, myodystrophy ci gaba. Da shan kashi na tsokoki da kuma akai kaya a kan su kai ga contractures - m hargitsi wata gabar jiki. Bugu da kari, marasa lafiya da Duchenne murdede dystrophy lura da cututtukan zuciya, wanda lokaci zuwa lokaci sa kansu ji. Har ila yau, domin wannan cutar halin a rage na iyawa ta hankali (ba sosai furta).

Becker murdede dystrophy yana da guda bayyanar cututtuka, amma ci gaba daga baya. A farko manifestations faruwa a 10-15 shekaru. Akwai sauka a hankali canji a Gait unsteadiness ya bayyana daga baya ci gaba contracture. Take hakki na zuciya da jijiyoyin jini tsarin ne m. Intelligence a wannan cuta da aka yawanci ba a rage.

Yadda za a gane asali murdede dystrophy?

Ganewar asali "Duchenne murdede dystrophy" (ko Becker) na iya sa wani gogaggen neurologist. A farko wuri shi dogara ne a kan na asibiti hoto na wadannan cututtuka. Da hankali ne kõma zuwa bayyanar cututtuka irin su thinning na proximal tsokoki, da gastrocnemius tsoka hypertrophy ƙarya (auku saboda fibrosis kuma mai adibas). Wadannan cututtuka su ne kusan ko da yaushe a hade tare da zuciya da jijiyoyin jini pathologies. A ECG za a iya gani dysrhythmia, hagu na ramin zuciya hypertrophy.

Har ila yau, marasa lafiya da Duchenne murdede dystrophy dan kadan wanzuwa da takwarorinsu a cikin ilimi ci gaba. Don sanin wannan, da yara yi aiki a matsayin psychologist. Lokacin da cutar da ake zargin kwashe myography (definition na lantarki m tsoka) da kuma EhoKS - jarrabawa na zuciya da bẽnãye. Domin a daidai ƙayyade gaban cuta, kwayoyin ganewar asali aka yi. Lokacin da Duchenne da Becker murdede dystrophy marasa lafiya ya kamata a lura a dama masana. Daga cikin su - a neurologist, a psychologist da wani likitan zuciyar.

magani

Abin baƙin ciki, etiological magani daga Duchenne murdede dystrophy da Becker ba a ci gaba. Duk da haka, marasa lafiya nuna symptomatic da kuma taimaka far. A farkon matakai da cutar suna da za'ayi darussa na physiotherapy, tausa. Tare da gagarumin tawaya bukatar yin m ƙungiyoyi daga cikin wata gabar jiki. Don rage da ci gaban na tsawo contractures, koma ga kayyade kafafu a lokacin barci. Maintenance far iya tsawanta rayuwar marasa lafiya da kuma rage bayyanar cututtuka. Yi amfani da alli kari, magunguna "galantamine" da "Neostigmine". A wasu lokuta, rubũta hormonal jamiái, yafi "Prednisolone". Lokacin da wani m cuta daga cikin zuciya nada cardioprotectors.

Duchenne murdede dystrophy da Becker Forecast

Duchenne murdede dystrophy hangen nesa ne ya fi karfinsu. A farkon cin gaban cututtuka da kuma m ci gaban da cutar take kaiwa zuwa tawaya a cikin shimfiɗar jariri. Marasa lafiya tare da wannan cuta bukatar m kula. A tsawon rayuwar jama'a da marasa lafiya ne game da shekaru 20. Becker murdede dystrophy halin da m hanya. Karkashin m dubawa na likitoci da kuma aiwatar da su umarnin operability da marasa lafiya da kiyaye har zuwa 30-35 shekaru.