Mate wheua Marble: take, tohu, maimoatanga, te ārai

Marble Ko te māuiuitanga mate congenital whakakitea e te e osteosclerosis E ruia ana te nuinga o wheua kōhiwi me huringa hohonu i roto i te konupūmā-ūkuikui pākia. Ko tino onge te mate. I whakaahuatia tuatahi te reira i roto i 1904 i te taote Tiamana me radiologist Albers-Schoenberg (Albers-Schonberg NE). puta mate Marble tino i roto i ngā kāwai tau katoa, i roto i nga whenua katoa i runga i te ao, he ōrite i roto i tane e rua.

osteosclerosis congenital, osteopetrosis, me dysplasia giperostaticheskaya mate Albers-Schonberg - he maha o taurite te mate kua. Kaua e whakapoauau te mate mapere me te syndrome i huaina "kiri mapere." A, no te tīmata te mate wheua mapere i roto i te kōhiwi ki te whakaputa i taonga kiato anō, me te i roto i te awa medullary tīmata ki te whakawhanake i te mātotoru wheua. He mapere huaina congenital oko vazonevroz kiri i roto i te kiri, tino maha tupu i roto i ngā tamariki, me te faaite ake wahi reticular i runga i te kiri i te tae kikorangi i muri i tinei. I muri e painaina ana i te tohu riro. Ka puta mai hoki i roto i te kiri pakeke mapere ki te kiri marama kikokore e whai raruraru ki te oko toto.

He unknown te etiology o tenei mate. I roto i wā o te mate tino mohiotia kua reira he pūāhua mapere whānau me te tuku i te whakatupuranga ki whakatupuranga. Tona mate ingoa mapere au he pikitia motuhake o te X-hihi o te kōhiwi, denoted nei e te homogeneity rerekē, me te mātotoru wheua koi, me te te kore o te hanganga wheua. Te āhua e kua hanga o mapere wheua.

E rua ngā momo o te mapere mate:

- rerengia ana e te kore tohu haumanu kitea, pāngia anake i te whakamātautau X-ray;

- faaite ake i roto i te kōhungahunga, kua korerotia tohu.

Ko te āhuatanga matua o te mate ko te pakeke tonu te kanohi ranei o te wheua trabecular. I te wāhanga tuatahi o te mate sclerotized anake wheua tubular i roto i nga metaphyses wheua, me te flat i roto i te wahi piringa. I tua atu, kitea taurite hiri wheua cranial, te nuinga pā kōhao paranasal, rawa tuatahi, me te frontal. Kei te kitea Hinganga ano me paparinga e, e haere tahi ai i ofatiraa o te whanaketanga hū me te niho - te huri to ratou hanganga, iti rawa whakawhanakehia pakiaka, te obliterating nga awa me kōwhao o te niho. Nuinga o ngā wā e rapu tūroro aro hauora mamae take i roto i te wahi me te rohirohi, no te haere ana. Marble mate wheua ano pēhia pā te whanaketanga o te pūnaha iaia, rite tupu nui o te kiko wheua rerekē āta whakakapi te kiko hematopoietic.

i whiwhi Best mate hua rongoā mapere i roto i ngā nganatanga huaranga (huaranga) wheua wheua. maimoatanga pono whai hua o tenei mate e kore te tīariari i tēnei wā. Kei te nuinga hāngai maimoatanga o tenei mate i te whakapakari i te pūnaha uaua, me te manavasii, ka kawea i roto i te nuinga o te wā rite outpatients. whakaatu tūroro i tua, mirimiri me te kaukau. kia waiho Nutrition he tonu, taonga i roto i ngā āhuatanga wahi me huaora, kia reira i roto i te tahua, te tīhi, hua hou me huawhenua, hua tūturu me he inu huawhenua. wā tamariki ki tenei tātaritanga tonoa ki te maimoatanga hōhipera.

Ko te tikanga taketake mō te ārai mate mapere pā pupuri gestation tātaritanga perinatal 8 wiki. Kia kore ai te takanga o deformities wheua, me te kia kawea piere pathological i roto i whakamātautau hihiri i roto i orthopedics.