Ectodermal a fibrous dysplasia. hakuʻala dysplasia

Ma lalo o dysplasia (unuhi mai Greek me dys - hōʻole; wplasis - hoʻonaʻauao) pili i ka hewa a me ka paha ka nānaina i ka ulu ana o kekahi hui,. Kēia mea - he like kau e uhi ana i kekahi 'aʻe'.

Fibrous dysplasia pili i ka maʻi o ka skeletal oihana, a ua wehewehe aku i loko i ka iwi'aʻaʻa UAIAaIN e fibrous'aʻaʻa. Kēia kaʻina deforms na iwi. I ka papa kuhikuhiE kumu o keia maʻi o keʻano o kaʻeho kaʻina, a hoʻomaka i ke keiki, a laila, hoʻomaka e holomua. Fibrous dysplasia mea IeAUPIIe, IAa IO i loko o nā wāhine.

maʻi kekahi mau Mea Maluna. Ka loa, he pono ole pa alima mea, ke kaumaha ma muli o ka nui o ka hoʻonuiʻana o ka pathological kaʻina:

• monoossalnaya fibrous dysplasia e loli wale kekahi iwi;
• poliossalnaya - nā loli i malama i mau iwi (IeAUPIIe, IAa IO hohola ma kekahiʻaoʻao o ke kino, a me ka pinepine ia lŘlŘ ma kamaliʻi).

Ka mua ano o ka maʻi e loli ai ka cranial iwi, i ka maka, omo iwi (lōʻihi) lihi. Ka lua - i ka mea ia manawa oi aku mamua o kanalima pakeneka o ka holoʻokoʻa iwi iwi.

I ka papa kuhikuhiE complications nā pinepine fractures, i koke coalesce, akā, Hoʻonui i ke kanana nei i loko o ka iwi, e like me ka pono me sarcoma.

Fibrous dysplasia i kekahi lapaau hiki ole ia, e hiki orthopedic wale kaʻina hana (transplant iwi curettage, osteotomy), a me ka hookohu o ka ai 'i relieves eha.

Ectodermal dysplasia encompasses he nui helu o nā maʻi a me nā syndromes (ma kahi o 100), i ka i loko o i ka ke alo o'āno'ē? Acaeoey o nā mea loaʻa ectoderm. Ka mua hoailona - i ke alo o ka hyperthermia, i ka wā i olelo ia, a me ka pinepine ho'āhewa wale ke alakai i ka hahau ana i kekahi 'imi naʻauao? Acaeoey, a me ka make.

Oe ke koho i ke kēia mauʻano a me kā lākou mau symptoms.

Angidroticheskaya hoʻomaka e hoike mai ai ia ia iho i loko o na kamalii uuku, he keiki, a me ka mau ma ke ola. I ka papa kuhikuhiE hiʻona i:

• hereditary maʻi;
• ikaika deformation o na niho;
• ke alo aku o ka slowdown i loko o nā kui;
• mucosal atrophy (puu, waha, ihu);
• ka mea mawaho helehelena mea i 'ano like me ke keiki e hoomanawanui i ka congenital syphilis.

Symptomatic kanaka pono i haawiia mai. Hiki ole: icai ma ka hema'āina, ka hana i ka wela a me ka Nine Inch mea kanu.

Gidroticheskaya ekzodermalnaya dysplasia ua wehewehe aku e loli i loko o na poho, na puupuu wawae, lauoho ulu. E like me ka lapaʻau ua ana lawelawe wikamina "A" mawaho, a ma loko, i hoopalupaluia, holika a me keratolytics. Ma waho aʻeo ia keia, hoʻohana: enzymatic 'o ia hoʻi Methyluracilum, anabolic steroid, kiniki,'amino nāʻakika a me biostimulants.

ʻaʻa puʻuwai dysplasia loaʻa paha kekahi mau ano e OAOOUIEeE:

• morphological hoikeana (cystic a me ka poe noonoo ole);
• IIeEeAIEUAA (cortico-medullary, medullary a me ka cortical);
• prevalence - huina, segmental, mea nui).

I ka papa kuhikuhiE kumu o keia maʻi e aaiaoe aʻe, a teratogenic na, a mawaho keiki ma embryo. No laila, i ke kumu o ka maʻi i congenital malformations, i, ka nānā 'ana i ka' ano, e alakai i ka emi ma ka hakuʻala nui, vicious ulu o ka parenchyma, he nui ka ho'ēmiʻana o kona hana.

Iapaau ana o keia maʻi mea symptomatic a me ka pinepine hihia ia he mea i PAaIEN no transplantation o ka hui,.

I mau, dysplasia maupaʻa i ka pae o ka maʻi i Komo'āno'ē ulu ana o ka hui, a me ka mea a pau nenoaiu. E like me ka rula, i ka papa kuhikuhiE ke kumu - aaiaoe a me ka congenital aʻe.

Lapaʻau mea symptomatic, a koi e no ka hui, transplant paha iwi.