Ola kinoKa noʻonoʻo '

Maʻi pale "pebaldizm": He aha ia mea, e hookomo ana, symptoms,ʻike, a me ka lapaʻau

Kekahiʻano o ka pigment pūnao kāna hana - pohihihi keia maʻi pale "pebaldizm". Gnozdnym wehewehe aku ma ka discoloration o ka lauoho ma luna o kona poʻo. Kēia mea,ʻaʻole he hewa ole mea hoʻonani kina, akā, he symptom o ka helu nui o nā maʻi kaumaha.

Ma keia 'atikala, ua noonoo ma ka au mamuli "pebaldizm". Kumu, symptoms,ʻike, lapaʻau, e e ho'ākāka 'ia ma kekahi.

Kumu o ka lauoho Hekikai

'Aʻe' ana o pigment pūnao mea he depigmentation - pigment poho, hyperpigmentation - amplification i hookohu aku i.

Ka lauoho waihoʻoluʻu pigment i ka waiwai 2 - eumelinin, pheomelanin. Ka mea, i loko o ka cortex o ka lauoho. Eumelanin mea oho i eleele, a me kaʻeleʻele pheomelanin - yellow-ʻulaʻula. Ke me ka hui pu ana o kēia mau pigments i loko o ke kanaka, ka helu, a me ka AaOIeIOUIeXAOAaIN me ka lewa i hāʻawi mai i ka lauoho i kekahi waihoʻoluʻu. Hair waihoʻoluʻu ua genetically hanaʻia.

maʻi pale "pebaldizm" Pehea pinepine e hoʻomaka ai, i ka i kēia maʻi?

I 'aʻe' ana o nā melanocytes, ke pigment-synthesizing lauoho, graying me ka makahiki e hoʻomōhala ai. Inā ke ano hoʻomaka ma kekahi manawa'ōpio wale nō - keia mea hoike o ka aaiaoe maʻi, a me ka maʻi kaumaha.

Maʻi i loko o ka mea i poino pigmentary mail

No ke aha lā i loaʻa ka "pebaldizm" maʻi pale, aha mea keia haiahū kūmau?

ʻO kēia mau Hawaii e ulu ana ma 'aʻe ana i nā melanocytes:

  • pebaldizm ma kaʻawale palapala;
  • albinism;
  • pebaldizm hui me ka maka maʻi, pepeiao,ʻili, ko mākou hopohopo nenoaiu, musculoskeletal nenoaiu.

lapaʻau kiʻi

Pehea e hoike mai ai ia ia iho, "pebaldizm" maʻi pale, aha 'aʻole ia ke ano o?

I ka lilo o ka huluhulu pigment i loko o ka palapala o ka helehelena o ke keʻokeʻo hoʻohuli akula ma luna o ke poo ke'ākoʻakoʻa, mai ka hanau ana i ka adulthood.

Ke koke, aia no ka 'aʻe' ana o pigment pūnao, i ka oi nui ke kumu kaumaha hopena.

Kaʻawale discoloration hoʻohuli akula hui me ka hapa o ka discoloration ma luna o kou lae. Pu ma ke ano o ka foci o hyperpigmentation, 'ole. E. Gnozdnogo poeleele hoi o ka ili i ka mua ili o ka abdomen, uha, wāwae, umauma, i na mea kaua Ana mai 1 mm o ke 6 knm.

Maʻi i pee ma hope o pebaldizmom

Aia mea he nui pūʻulu o nā maʻi a me nā anomalies o? Acaeoey, hoʻohuiʻia pu me ka loloʻa lauoho 'inoʻino loa nā loko, a nenoai. Undiagnosed pathology e malama i ke ola o ke keiki, a me nā mākua, e hāʻawi mai i ka hailona o ka pōpilikia.

Waardenburg-Klein maʻi pale

Ka maʻi o ka autosomal Hoʻohaku kumu o ka noho ana, oa. E. Na ia pinepine ia lŘlŘ i loko o nā keikikāne a me nā kaikamāhine. Ke aaiaoe kina hiki e ili mai kekahi makua.

Ma waho aʻeo i ka loloʻa aʻe o ka lauoho kaula makalii e ae nānā nui a me ka ocular hoike ana mai o ka maʻi.

Ocular symptoms o keia maʻi:

  • hypoplasia o na lihilihi;
  • oho like ma luna o ka pā kuemaka;
  • ololi maka slit;
  • okoa i nā pōpō mau maka;
  • hyperopia;
  • hoʻomano kaulike maka.

Common symptoms paha e loli iki ana degere o ka oolea:

  • hapa albinism;
  • ka laulā nasal alahaka;
  • pōkole kiekie;
  • anomalies o ka niho;
  • kuli-mutism a kaʻawale lilo hālāwai hoʻolohe manaʻo;
  • anomalies o ka mea na nā loko;
  • hoʻololi i ka nui o ka iwipoo.

Ua haawiia ia i mua i ke oolea o ka maʻi, a ia lŘlŘ wale palena iki pebaldizmom Pono e kekahi mea loiloi, e hookaawale ia mea pathology. Ia ka leo o ka piebaldism.

Wolfe ka maʻi pale

Kaʻawale heterochromia, 'ole. E. Aʻokoʻa maka waihoʻoluʻu ua ka hapanui i loaʻa i loko o na holoholona. Ma kānaka, i ke alapine (frequency) i waena o ka mea a pau heluna mea 1%.

Famous kanaka me kekahi maka waihoʻoluʻu: Mila Kunis ( "oz Ka nui a ikaika hoi", "Bloodlines", "Dia e piʻi"), Pelekāne mea hana keaka Dzheyn Seymur, e pono ike no ka ho film "Live a E Make", Devid Boui - Pelekāne pohaku mea hoolea, songwriter, ka hoʻohua, kani lunaʻenehana, artist, Mea Keaka.

Kaʻawale heterochrony mea i ka maʻi, a ma ka hui pu ana, me ka pebaldizmom maʻi pale i kapaia o Wolfe, a hiki aku i ka hoʻokēʻai-ulu pepeiao kuli.

E differentiate i kēia maʻi e ia me ka retinitis pigmentosa - pigment epithelium dystrophy maka. Pinepineʻano papa o kēia maʻi - retinitis pigmentosa me pigment.

Mom a me ke kaikamahine i ka maʻi pale o "pebaldizm"

Ka maʻi Ua recessive, chromosome-ua hoʻopili wahine ka hooilina. A wahine ae ke'ōewe i kāna keiki, aʻo ia i ka maʻi. Kaikamahine o kēia poʻe kānaka e ola, ina ka mea, i ke ola i ke makuwahine. He mea e ka wale hoike ana o ka pebaldizm.

Inā ka makuahine mea ola clinically, akā, i ka lawe ana o ka'ōewe, i ke keiki e e maʻi.

E diagnose i ka maʻi hoʻohana ophthalmoscopy, a e hōʻike ana i nā kiko e like mu-ai fundus pigmentation.

Gipomelanoz Ito

I ka maʻi e hoʻomōhala ai, mai ka hanau ana. E ae aku i nā mamo a nā sexes me ka like alapine (frequency). 'Aʻole i maopopo hereditary EYI, mai na makua i ka hua, akā, i ka maʻi ua mau hui me congenital mā o ka na nā loko.

I kaʻike, ua i clinically ma luna o ke kumu o ka kēia mau symptoms:

  • hou ma luna o kaʻili amelanotic haʻawina bizarre ma ka palapala curls, snowflakes;
  • Ma ka palmar, plantar surfaces a me mucous membranes na loli mai i hiki mai ana;
  • i loko o ke kino māhele o ka maloo lauoho noaioee - pebaldizm;
  • makahiki depigmentation? eou e emi i nanaia;
  • poino i ka'emi'ōnaehana i mau hoike noʻonoʻo retardation, convulsive kāna hana, ua hoʻemi Muscle leo;
  • pathology o ka maka akaka strabismus e loli iki degere o ka oolea;
  • Ukali aku scoliosis, umauma deformationʻaoʻao i loko o 25% o nā hihia;
  • me ke alapine (frequency) o 10% hui ia me ka naau mā.

E differentiate i kēia maʻi, mai ka mea e pono ai ke ano i kapaia o urinary pigment. Ka mea, i like lapaʻau symptoms, akā, nei ili.

pigment incontinence

Ua pohihihi keia maʻi pale ua ho'īli "pebaldizm"?

I ka maʻi ua pū i hiki mai ka makuwahine i ke kaikamahine. Ke kaumaha ma muli o ka oolea o ka lapaʻau kiʻi ua hoikeia aku ai e na kēia symptoms:

  • Skin poʻe noʻonoʻoʻole i loko o ka palapala o ka wehe 'loa a, ma hope o ka maloʻoʻana a me ka wehe' ana o crusts haʻalele'āpana pigmentation. Pele i ka undulating papa me ka papa'āweʻaweʻa kemika helehelena o ka hou ili keena. Mai kēia mau mea e kukaʻi maturation keiki lilo pigment, a lilo lenalena.
  • Dysplasia a me ka niho hoʻonohonohoʻia, kui, lauoho depigmentation like alopecia a me ka'ōhule.
  • Neurological kāna hana i hoikeia hoʻopaneʻe 'ia ana psycho-olelo ulu, a mahuahua nui intracranial puʻe, ke kāʻili' ana, nawaliwali i ka lima a me nā wāwae.
  • Eye kāna hana i lolelua ka, a akaka strabismus, nystagmus, cataracts, hoemiia hihio, makapo.

Gristselli maʻi pale

Kēia mea he pūʻulu o ka maʻi i ke hana i ka ekolu 'ano apau loa, i ka papa kuhikuhiE similarity o ke alo o discolored loʻi o ka lauoho ma luna o kona poʻo. ʻO kēia ka hōʻike 'ana i loaʻa:

  • Neurological kāna hana me ka hypopigmentation.
  • Ka noho pilikia loa 'ano o ka maʻi i loko o i "pebaldizm" maʻi pale hui me kaʻole hematopoiesis. Lapaʻau hoike ana: lōpū ke kuni, a me ka paranormal lapaau. Pinepine 'wela i nā maoli, pili ana i ho'ākea akepaa, ka spleen, ka ka ho'ēmiʻana o erythrocytes, leukocytes, platelets, fibrinogen i loko o ke koko. Ka hope ua komo i loko o ka keʻano o ka hanaʻana o ka ua'emi ko kahua.
  • hypopigmentation o ka ili a me ka lauoho o ka loli iki ana oolea, i a laila puka mai me ka make ana o ke koko keena, a me ka ua'emi nenoaia. No ka mea 'eleuʻike, o keia mea maʻi e nīnau i ka hematologist dermatologist, aaiaoe?. Mai poina e lawe i ka mooolelo o ka noho ana o ka ohana o kekahi mau kāna hana o ka pigment pūnao, ke Ka Ikepili e hookaawale ia'ōewe mutations.

Urgency a me ka pilikia o ka pau no i ke alapine (frequency) kiʻekiʻe hoʻohuiʻia ia me Down maʻi pale, Edwards, Turner.

E kuhikuhi i depigmentation o ka ili a me ka lauoho hiki ia i hanau, akā, no ka hookupaa aku i ke alo o ko Turner ka maʻi pale hiki e like me ka kanaka e ulu ana i wale ia ia pōkole ke kino o Iesu, lymphostasis, naau maʻi, kiʻekiʻe ke koko, underdeveloped mammary glands, haʻahaʻa kulana o ka nipple, hakahaka o na wahine nā loko connective kapa. Manao i loko o kēia mau nā mea maʻi aneane, aole i ae aku, kāʻei kua naʻau i loko ua kokoke mau ke kiekie, he mea kekahi maikaʻi euphoria, a wehewehe i ka pono nohona ka hoʻololiʻana o kēia mau hoʻomanawanui iki mai.

ka hopena

Ma kānaka, i ka'ōewe o maʻamau pigmentation o ka ili i mau Hoʻohaku ma ka pili ana i ka'ōewe kuleana no ka 'aʻe' ana o pigment pūnao.

Ka walaʻauʻanaʻano o ka pilikia me ka pigmentation o ia mea, i pilikia hōʻailona o ka chromosomal maʻi, mau congenital malformations.

Kekahi 'aʻe' ana o ka ili pigment pūnao, mucous membranes, ka lauoho e nīnau i ka hoʻomalu. Ua noonoo mākou "pebaldizm" maʻi pale. He aha ka mea, e lilo kālaʻe.

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