Albright maʻi pale. Maʻi pale mc Kyung-Albright-Braitseva. Kumu, lapaʻau

Maʻi pale mc Kyung-Albright i ka inoa i loko o ka hanohano o nā hoʻokamakama kauka, ka mea i oi ma mua o ka hapalua o ka kenekulia aku nei i ho'ākāka 'ia ia. Ka mea, hai aku la i ka lehulehu e pili ana i na keiki, ka hapanui o ka mea, he mau kaikamahine. He nui no ia, he pōkole ke kino o Iesu, a maka, pōkole'ā'ī, pōkole IV a me V metatarsal a metacarpal iwi i Muscle spasms, loli i loko o ka iwi, lohi teething enamel hypoplasia. Aia ka noʻonoʻo retardation, a ua hoi endocrine maʻi, kaheaia i kakahiaka nui puberty me menstrual kahe ana ke koko, e ulu ai o ka umauma, ka ulu ana o ka pubic lauoho a me ka umauma, ka mea ulu i loko o ka uku o ka ulu ana mai o na keiki a me nā loli i ka ili.

Ma ke kālā no lapaau, i ka manawa "Albright maʻi pale" ua hoʻohana 'ana i nā mea maʻi i i pau ole wale kekahi endocrine a me ka ili abnormalities. Aia nā hihia ma kaʻike, i loko o kakahiaka kamaliʻi. Eia naʻe, i loko o typical hoopii, ka mea, ua kau ia na keiki 5-10 makahiki, ma muli ma ka hoailona, i mea ano o keia maʻi. Nui ia mea he pohihihi a me ka ili. Ua koeʻikeʻole i ka etiology a me ka pathogenesis o keia maʻi. E ka nana aku i na hoailona o ka maʻi.

Aʻe 'ana i loko o ka endocrine'ōnaehana

More pinepine ma mua o na kaikamahine, a me Albright maʻi pale ua malama precocious puberty, a ua kauoha aku e estrogens kuu i loko o ke koko, mai ovarian cysts. e mahuahua Cysts ma hope o emi i ka nui i loko o kekahi mau pule paha lā. Me ke kōkua o ke ultrasound kaʻina hana i ka manawa e ike, a, e ana i ka nui o ka hikoko. Cysts ke ulu i hala loa he kohu kūpono nui. Aia he mau hihia, i ka wa a ka mea i ulu ai i ka nui o ke kolepa mea poepoe, i mea oi ma mua o 50 mm o ke anawaena.

Umauma augmentation a menstrual kahe ana ke koko e malama a me ka ulu ana o cysts. Inā ke kaikamahine hoomaka menstruating mua makahiki 2, ka mea, o ka mea mua symptom o Albright maʻi pale. Naʻe, i ka maka o ka ole menstruation a me ovarian cysts hiki e mālama nā ma adolescents a me ka makua wahine. Kēia a pau, aole ia i hiki ole i ka i ola mau keiki.

Iapaau ana o na keiki me ka precocious puberty, he paʻakikī a me ka inefficient. Ina o ka cyst surgically wehe, ia hiki hou-i waho mai. I ka lawe ana i ka hōmona progesterone hiki ke oki menstruating, akā, i ka kokololio uku o ka iwi? Acaeoey a me ka ulu ana o ka ua i ka meheu o ia. Hiki io mea hoʻomaoe no ka hana ana i ka adrenal glands. Ma ka iapaau ana o ia mea haʻilula keimikala no ka 'ai, inu ka paleʻana estrogen hoʻololi kemikala.

thyroid kuleana pili i

50 pakeneka o ka poʻe me Albright maʻi pale, e loaa ana, mai kāna hana o ka thyroid gland. Kēia no laila,-kapa goiter, nodules a me cysts. Aia paha e pohihihi keia hihia maalea mall loli. Ma kēia mau nā mea maʻi, i kaʻilikai hua mai ma ka pituitary TSH mea haʻahaʻa, akā, i ka pae o ka thyroid hormones mea maʻamau nā loina a me iki ke kiekie. Lapaau ana, ma ka mea o ka i emi hoʻololi kemikala o thyroid hormones. Ua Ua hōʻike ma ka hihia ma ka 'ilikai mea kiʻekiʻe lawa, e wehe i ka hōmona.

Excessive secretion o ka ulu hōmona

I ka wā o ka maʻi pituitary hoʻomaka i ka pee nui dala o ka ulu hōmona. Ma na keiki, a ua waiho i kēiaʻike, me Albright maʻi pale, acromegaly i ike. hoomaka ae la na kanaka ui, e hele mai lohi lehalehu t hiʻona, koke ulu mau lima a me nā wāwae, ka mea hiki ke hoomanawanui arthritis. ua hoemiia lapaʻau o nā keiki a me na symptoms i kaʻoki lāʻau lapaʻau ana o ka pituitary māhele, a me ka hoʻohana 'ana o kemikala hōmona somatostatin analogs, inhibits ka? iecaianoaaiiie o ka ulu hōmona.

Other endocrine kāna hana

Aia mau pohihihi a me excessive secretion o adrenal hoʻonui. Oia ka nahae i ka hopena i obesity ke kino a me ka helehelena, he mahuahua ma ke kino paona, cessation o ka ulu a me ka ili fragility. A pau kēia mau symptoms i kapaia o Cushing ka maʻi pale. A hiki aku ua loli i wehe 'ia loli adrenal a lawe lapaʻauʻia e emi ai ka hoʻololi kemikala o Cortisol.

I kekahi manawa, na keiki i Albright maʻi pale, loa haʻahaʻa pae o ka phosphorus ike i loko o ke koko, no o ka lilo nui o posapahate i loko o ka mimi. Kēia kāna hana hiki ia i ke kumu o ka iwi loli pili me rickets. E like me ka lapaʻau a me ku una posapahate a me ka wikamina D i ka hou.

Kāna hana pili i ka ili

Ma kaʻili i hānau 'ole ma hope koke ikea mau kiko o ke ano "kope me ka waiū". Ka mea, ka hapanui o pinepine ana ma luna o ka sacrum, kumu, wāwae, kīkala, hoʻi o ka ai, lae, poo, hoʻi o ke poo. mea no hoi a pau o ia i hōʻailona i ke keiki Albright maʻi pale. Photos o kēia mau pūkoʻa ia e ike hiki ma lalo nei.

Oiai keia maʻi ka neurofibromatosis, kekahi i kina waihoʻoluʻu "kope me ka waiū". Eia naʻe, ua wehewehe aku Albright maʻi pale ma ka nui pūkoʻa ia me ka ole outlines, i poʻeʻuʻuku i loko o ka helu mea. Ka mea, i ke anawaena o ka mai 1 a hiki i kekahi mau kenimika,ʻeleʻele tint. Ke kala a pau o ka ia, ka mea, i ka'ololaha in shape, ka mea i wehewehe aku e ka Kaulu. Ma histological ninaninau pinepine hōʻike i nā epidermis ua ole hoʻololi i loko o kona 'ole, akā, no ka nui o ka melanin i loko o nā keratinocytes mahuahua iki.

Kaʻawale mau kiko o kēia 'ano hiki ke loaʻa i loko o ole ola kanaka. Inā ka mea, e ole bother me ka mai i ulu, alaila, i ka iapaau ana mea i pono. Inā he mea ulu kokololio, eia hoʻokino pūkoʻa irregularly, ka mea, ua? Aeiiaiaoaony e noiʻi ana ia histologically. A laila, wehe surgically.

ka hopena

Pela, ua hiki ke olelo ia no ka Albright maʻi pale ua wehewehe aku ma ka poho o ka iwi a me ka puniu wale no, i ke alo o ka ili pigment wahi lilelile, i kakahiaka nui puberty. 'Oiai, he mea hihia ma laila nō wale nō ka mea mua mau symptoms. I mau, i ke kumu nui hiʻona o ka maʻi pale ka iwi lesions (osteodysplasia). Naʻe, i kēia kaʻina aɃe ka wā puberty. Ma nā mākua, iwi loli ka mea,ʻaʻole i holopono. Nui, o ka ka loaʻaʻana a me ka pono lapaʻau, ka prognosis o Inc o keia maʻi mea he maikaʻi.