Isifo seCreutzfeldt-Jakob sachazwa ngabososayensi ababili baseJalimane, abanikazi babo ababizwa ngokuthi lesi sifo, ekuqaleni kwekhulu lama-20. Nakuba kusukela ngaleso sikhathi kudlule isikhathi esingaphezu kwekhulu, ukwelashwa kwalesi sifo akukaze kutholakale. Ososayensi bakwazi ukukhomba umthombo wesifo - i-prion enenzondo, kodwa ayikwazanga ukubhekana nendlela yokubhekana nayo.
Isifo se-Creutzfeldt-Jakob - siyini?
Isifo se-Creutzfeldt-Jakob prion siqala ngenxa yokuguquka kwesakhi sofuzo esibangelwa isintu esiyingozi, i-protein prion. Kukholelwa ukuthi umthombo walolu protheyini yizinkomo, kodwa ucwaningo olusha luveza ukuthi lesi sifo senziwa ngokuzenzekelayo ngaphandle kwesizathu sangaphandle. Abacwaningi bakholelwa ukuthi isifo se-KBH (isifo sofuba sezinkomo) siyaqhubeka, futhi kuphawulwa amacala ezinhlobo ezintsha zezifo. Ngawo-1990, amacala e-subspecies yalesi sifo alotshwe, okuthiwa yi-cow cow.
Ngaphambilini, lesi sifo sabathinteka abantu asebekhulile kuneminyaka engama-65, kodwa manje kukhona amacala okulimala kubantu abasha. Igciwane le-Prion lithinta ubuchopho, ngenxa yalokho izinqubo zokucabanga kanye nomlingiswa kuqala ukuhlupheka kubantu. Ukuthuthukiswa kwe-lesion kuholela ekukhuleni kwezimpawu, ukuphazamiseka kwenkulumo, ukuhlukunyezwa kanye ne-paresis yamalungu. Ukuphakama kwalesi sifo kuwukuthi i-coma nokufa. Ngemuva kokutheleleka, umuntu uhlala engaphezu kweminyaka emibili. Isikhathi sokuphila esiphezulu sokulimala kwe-prion yizinyanga ezingu-8.
Isifo se-Creutzfeldt-Jakob - i-agent causative
Igciwane lesandulela ngculazi libangelwa iphrotheni ye-mutant prion. Ama-prions akhona emzimbeni womuntu, kodwa abe nesakhiwo esihlukile. Iprotheyini enonya evela ngaphandle ayifi emzimbeni womuntu, kodwa ivela ekusakazeni kwegazi kuya ebuchosheni. Lapho, uqala ukuxhumana nabantu, okuholela ekuguqulweni kwesakhiwo sabo. I-prion ezithathelwanayo yenza ama-plac on neurons, emva kwalokho i-neuron iphela.
Isifo se-Creutzfeldt-Jakob - indlela yokutheleleka
Ososayensi bahlukanisa izindlela ezinjalo zokutheleleka kwesifo seCreutzfeldt-Jakob:
- ukufakelwa kwezitshalo ezithintekile ezikhulelwe ezinezifo ezithathelwanayo;
- ukumpontshelwa kwegazi elingcolile;
- ukudluliselwa kokutheleleka ngokusebenzisa izinsimbi zokuhlinzeka ezingcolile ezingelapheki;
- bedla izinyosi, izimvu, izimbuzi ezinesifo esithintekile kabi.
Izifo zeCreutzfeldt-Jakob - izimbangela
Isizathu sezotiological se-Creutzfeldt-Jakob sifo asisungulwe kahle. Nakuba inguqulo ye-ingress ye-prion alien evela ngaphandle (ngokuvamile evela esilwaneni) ivunywa ngokujwayelekile, kunezinye izinkolelo. Enye yezinkolelo ukucabanga ukuthi i-prion yomuntu, ishintshwe ngesizathu esithile, iqala ukushintsha amakhondoni angomakhelwane, okuholela ekunqotjweni kwezakhiwo ezahlukene zobuchopho.
Iziqu ze-Mutagenic ne-Creutzfeldt-Jakob sifunda ukuqala ukusebenza ngokumelene nemvelo. Ivimbela iseli ukuthi ingenzi umsebenzi wayo, ivimbela izinqubo ezenzeka kuyo. Ngenxa yamabhonasi, iseli lifa. Emangqamuzaneni afile, izinqubo zokuvuvukala zikhula, lapho ama-enzyme asebenzayo ahlanganyela khona. Lezi zinto ziphazamisa umsebenzi wamaseli aphilile, ngaleyo ndlela zandisa umonakalo ezakhiweni zobuchopho.
I-Creutzfeldt-Jakob isifo - izimpawu
Imilenze yezinyosi kubantu abanezimpawu zabo ezixhomeke endaweni yesikhumba esiteji sokuqala kubonakala ngezibonakaliso ezinjalo:
- ukuphazamiseka, ukucasula;
- ikhanda, isizungu ;
- izinkinga zokulala;
- ukuwohloka kombono;
- ukunganakwa, ukungabi nesinyathelo;
- ukukhubazeka kwememori ;
- kunciphise libido;
- ukuzwakalisa ama-episodic noma ukwesaba;
- i-delirium noma i-hallucinations;
- izinkinga ngenkulumo;
- i-paresis yemilenze, ukungaqiniseki ngenkathi kuhamba.
Esigabeni sesibili, isifo sofuba sezinkomo, izimpawu zokukhula, sibonakaliswa yizibonakaliso ezinjalo:
- ukuhlukunyezwa kwesisu ;
- ukukhubazeka;
- ukulahleka kokulawulwa kwemisipha, ukuthuthumela;
- i-dementia;
- ukukhubazeka okukhulu kwenkulumo;
- izifo zengqondo.
Isigaba sokugcina sibonakala ngezimpawu ezilandelayo:
- isimo sokuzibulala;
- ukulahlekelwa kokulawulwa kwezitho zomzimba;
- ukugwinya ukugwinya;
- ukuqeda ukukhubazeka;
- i-coma.
Isifo se-Kreutzfeldt-Jakob - ukuxilongwa
Ukucacisa ukuxilongwa kudinga isithombe esiphelele somtholampilo, esiqinisekisiwe ngemininingwane yendiza. Kulokhu, udokotela, lapho eqoqa i-anamnesis, uthola ukuthi uhlala kuphi isiguli, kungakhathaliseki ukuthi kukhona oxhumana nabo ngezinkomo. Kubalulekile ukuthola zonke izimpawu isiguli esibhekene nazo. Ukuqaphela ikakhulu kubhalwa izinkinga zombono, amakhono engqondo nezithuthuthu.
Idatha ye-Instrumental ihlanganisa imiphumela yocwaningo olunjalo:
- I-EEG (i-electroencephalogram) - iyobe yanciphisa imisebenzi enezikhathi ezithile noma ama-pseudoperiodic acute waves.
- I-PET yobuchopho.
- I-Creutzfeldt-Jakob isifo, i-MRI lapho i-T2-mode yenziwa khona, itholakala ekuhlolweni yi-okuthiwa "uphawu lwezinyosi" - izindawo ezinezimpawu eziphakeme.
- I-Lumbar iphoqa ukutadisha uketshezi lwe-cerebrospinal.
- Biopsy stereotoxic yobuchopho, okuvumela ukuthola isiprotheni esithathelwanayo.
Isifo se-Creutzfeldt-Jakob - ukwelashwa
Njengoba kusenaso isizathu esiqondile saleso sifo, akukho mithi etholakale ngokumelene nayo. Ukugonywa kwezinkomo nabantu akuzange kube nomphumela oyifunayo. Ungenzi ezinkampanini ezinobuthi kanye nezidakamizwa ze-antiviral. Abacwaningi bakwazi ukuqonda indlela yokwandisa impilo yamaseli anegciwane, kodwa lokhu kuyisinyathelo esincane kuphela ekufuneni umuthi osebenzayo. Okwamanje, isifo sezinkomo samademoni kubantu siphathwa kuphela ngesifo. Isiguli sinqunywe izidakamizwa ze-anticonvulsant kanye nezidakamizwa.