Werdnig-Hoffmann: kwifomu iimpawu eziphambili

Werdnig-Hoffmann - kakhulu ngezifo okukhulu ihamba amyotrophy, ngokuthe ngcembe ukutshatyalaliswa luvo engundoqo ye umnqonqo, kwaye, ngenxa yoko, yoma kwezihlunu. Ngoko nangoko ke kubalulekile ukuqaphela ukuba le sisifo genetic ilifa, nto leyo ezidluliselwa ngendlela sofuzo autosomal.

Yintoni Werdnig-Hoffmann?

Sisifo luvo sikhatshwe intshabalalo chu izakhiwo ezisisiseko innervating luvo. Umzekelo, ngokufuthi kukho demyelination beengcambu Icipande umnqonqo. Ukongeza, esi sifo sichaphazela ezinye luvo cranial.

Kakade ke, lo umonakalo luvo kuchaphazela imo izihlunu. Nangona kunjalo, esi sifo luphawulwa ekuthiwa-ilahleko umqadi kwezihlunu, apho inxalenye esihlunwini ugcina ubuchule nesivumelwano, ngoxa eyahlukileyo "iinyanda" yome.

yoma spinal Verdniga-Hoffmann ebonakaliswa ebuntwaneni. Namhlanje wahlulwe iintlobo ezintathu ezingundoqo zesi sifo.

yesifo azalwa Verdniga-Hoffmann kunye neempawu zayo

Ngokuqhelekileyo, iimpawu yokuqala yale fomu kwesi sifo sele olubonakalayo kwiintsuku zokuqala ezimbalwa emva kokuzalwa. Umntwana asondeze ngababuthuntu paresis kweqondo. Ukubhomboloza abantwana abagulayo abaswele amandla, ayifiki oluvakalayo, ukongeza, kuba inkqubo amandla yaphulwe.

Njengoko umntwana wakho ekhula, ungenza uqaphele ukuqhwalela ngokomzimba. abantwana abagulayo musa entloko, akukho ukuhlala Ongemiyo. Kuphela kukwiimeko ezinqabileyo, usana uyakwazi ukubamba umzimba kwindawo tye, kodwa eli khono liyalahleka ngokukhawuleza njengoko ukutshatyalaliswa luvo.

Ukongeza, kukho ezinye izinto ezingaqhelekanga kuphuhliso skeletal, dysplasia ingakumbi unyonga, zokufota, ukuyilwa ifanele esifubeni, kokukhubazeka, hydrocephalus.

Ngexesha esibulalayo, yaye angakwazi kwalapho luqhuba ngokukhawuleza. Yoma kungachazwa kuphela izihlunu skeletal, kodwa ke Imicu izibilini. Kudla wathinta uvalo, nto leyo ekhokelela ukusilela sokuphefumla. Ngelishwa, abantwana kunye nesifo efanayo baphile (nge-avareji) ukuya kwiminyaka elithoba.

kusekwangoko isifo Verdniga-Hoffmann

Iimpawu eziphambili zale sifo azibonakalisa kwisiqingatha sesibini ebomini. Kwiinyanga ezimbalwa ukukhula komntwana idlule eqhelekileyo - itakane ufunda ukubamba intloko uhlale, maxa wambi na ukuhlala up lenyama. Ke zonke ezi zakhono balahlekile emva kokuba uqalile okanye ipoti ye sifo. Hi ndlela leyi, ngokufuthi omnye afune usulelo syndrome.

Lafa leminwe imicu contracture - Iimpawu zokuqala yokutshatyalaliswa luvo. Kamva iphuhlisa yoma kunye ukushwabana izihlunu. Ubude avareji yobomi lwezigulane iphakathi kwiminyaka eli-14 ukuya ku-16.

isifo kade Verdniga-Hoffmann

isifo okunjalo zamva. Njengoko umthetho, umntwana iphuhlisa kakhulu ngokuqhelekileyo de kweminyaka emibini. Itakane ufunda ukuhlala, nime, uhambe. Kuphela kwexesha abazali baqale uqaphele kutenxa kokumiselweyo.

Okokuqala, ukutshintsha umlenze womntwana egulayo - lihambe, kakhulu ayeguqa ngamadolo, kudla ngokuwa, musa ukubabeka tyala imali eseleyo. Njengoko ngezifo inokuba uqaphele utshintsho abathile kuphahla, ezifana deformation kwesifuba. Verdniga-Hoffman syndrome iphawulwa nokungcangcazela nkqi, sinciphise kwezihlunu, yokuduka engundoqo ingqondo unconditioned.

Kwiimeko ezininzi iminyaka 10-12 umntwana ithe ngokupheleleyo indlela yokuhamba elizimeleyo. Noko ke, kule meko izigulane kusinda ukuya ku-20, kwaye ngamanye amaxesha ukuya kwiminyaka engama-30.