Hutchinson-Gilford syndrome: awohluke zonyango kunye ezibangela sifo

Hutchinson-Gilford syndrome - a sifo esibonakala kwesikhumba ngokukhawuleza kakhulu, nto leyo iqala ukuzalwa komntwana. Kufuneka kuqatshelwe ukuba kulo lonke ihlabathi kukho izigulane ezifana yayincinane kakhulu. Pathology inomnye igama - progeria.

symptomatology kwesifo

Hutchinson-Gilford syndrome na iimpawu ezicacileyo:

• ukunyuka okungephi;
• skull ineenkalo zakhula engaqhelekanga;
• ukungabikho iinwele, ayagqama ngeenkophe;
• "Bird ubuso";
• bone kunye nezinye umzimba iinkqubo likhubazeke;
• ukungabikho fat ongama;
• ukusilela ngamandla kuphuhliso ngokwasemzimbeni, kwaye ukucinga nomqondo kuqhelekile.

Kufanele ukuba abantu babe ethile kweminyaka yokuphila: kwiminyaka eli-14 ubudala, nangona eyaziwa ngokuba kwimeko ekhethekileyo yomntu ngale unesifo waphila ukuya kwiminyaka engama-45. Ukufa ngokuqhelekileyo kwenzeka ngenxa ukusilela intliziyo, kunye needipozithi ezityebileyo kakhulu kwi ijwabu ebuchotsheni.

Izinto ezibangela isifo kwaye unyango oluthile

Hutchinson-Gilford syndrome - a leengxaki ukumqumbisa ukuphazamiseka kwi zofuzo yabantu, okt unyango epheleleyo ngalo mzuzu ayikwazi kube. Intsingiselo unesiphako kuxhomekeke kwinto yokuba inani kumacandelo Iseli yehliswe kakhulu, xa kuthelekiswa nemigangatho.

Kufuneka kuqatshelwe ukuba syndrome hutchinson-Gilford (umntwana form) bayagula nawuphi na umntwana (kungajongwanga budala indlela yobomi abazali). Yena uba lubonakale eneminyaka emibini. Kukho kwakhona sisifo Werner oluchaphazela sele abadala. Ukukhula umzimba zokumisa eneminyaka emithathu.

uxilongo olulodwa kwesi sifo akakho, njengoko ingagunyaziswanga kufundwe ngokupheleleyo. Ukongeza, loo nto ibonakala ngokucacileyo phezu iimpawu kuluhlu. Ngokumalunga unyango lwesifo, ukusebenza iziyobisi nako ostanosit kwaye sibuyele ubomi obuqhelekileyo bomntu, akukho. Noko ke, abantwana abo hutchinson-Gilford syndrome kufuneka babhaliswe kunye nogqirha kunye rhoqo okwenziwa. njani unyango ezithile ukuba wehlise ukuqhubela phambili kwesifo.

isicwangciso sonyango syndrome ingqalelo ukuze isigulane ngasinye. Oku kubandakanya ukwandisa umsebenzi lwemoto womntwana, iinkqubo impilo yomzimba. Ngenxa upotrebelniyu esezantsi-dose aspirin xa umntwana kunciphisa amathuba okuba nestroke. Ngamanye amaxesha izigulane wenziwa utyando, ezilungiselelwe kwi yokucima semithambo, Ukusikwa amazinyo zaseprayimari.

Ukuba unayo bengazi into ikhangeleka ngathi umntwana naso ka hutchinson-Gilford, iifoto ezithunyelwe kweli nqaku, uya kufumanisa ukuba.

Iimpawu zophuhliso lwabantwana nokubandezeleka sifo thaca

Ukuba umntwana ngokomzimba ayikwazi ukukhula, indlela yokucinga iinkolelo khange abachaphazelekayo. Itakane bangafunda ukufunda, unako ukufumana ulwazi. Kakade ke, inkqubo yokufunda iya kuqhubeka ekhaya.

Ke ukuba uqikelelo kwixesha elizayo, kuba yedanisayo. Kangangexesha elide, abantwana abanjalo kuphila. Inyaniso yokuba kulo nyaka mnye bathi kwiminyaka 6-8. Ngokuqhelekileyo, oosonzululwazi asebenza ukuba atyhile indlela ingenzeka kwesifo kunye neendlela ukunqanda oko. Noko ke, kumphakamo wangoku zophuhliso yamayeza unako ukuphucula kancinane kuphela umgangatho yesigulana eso ebomini, kwakunye inciphise inkqubo ukwaluphala.