N immunodeficiencies. N immunodeficiencies: lapaʻau

Immunodeficiency - he 'aʻe' ana o ka aiiay mana o ke kanaka meaola, aie i ka mea hoʻonāwaliwali maoli nō o ka'ōnaehana paleʻea pane i pathogens o kekahi ano. Nauka hōʻike i ka helu ana i kekahi olelo o keia ano keia ano. Kēia pūʻulu o kāna hana wehewehe aku e mahuahua alapine (frequency) a me ka kaumaha papa o infectious ka maʻi. Malfunctions e hoʻonele i loko o keia hihia pili i ka loli i loko o ka qualitative a laupaʻi ano o kona kanaka eiiiiiaiou.

waiwai e hoʻonele

Ka'ōnaehana paleʻea'ōnaehana Radio he pilikia kūlana i loko o ka maʻamau functioning o ke kino, e like me ka mea i manaoia e huai ae, a luku aku i nā antigens i hiki 'ole ke komo iloko, mai ka mea mawaho i hoʻolilo' ia (infectious) paha e kekahi no o kaʻeho ulu iho aeee (endogenous). Ke aiiay kuleana pili i ua kuhikuhi i hoakaka ia ma ka congenital kumumea like me phagocytosis a me ka hoʻokō kahua. No ka mea adaptive pane o ka meaola mākou ke pane lilo 'ōnaehana paleʻea'ōnaehana: ka humoral a me ke kelepona. Palapala e wahi ma ka pau'ōnaehana o kūikawā waiwai - cytokines.

Ke kaumaha ma muli o ka hihia, e māheleʻia ana e hoʻonele kāna hana i loko o iniiaiie a me nā haumāna immunodeficiencies.

He aha mea he iniiaiie immunodeficiency

N immunodeficiencies (PID) - he 'aʻe' ana o ka'ōnaehana paleʻea pane ma muli o ke aaiaoe mā. Ma ka hapanui o na hihia, ka mea i ili mai iā ia, a ua congenital abnormalities. PID ua loa hoikeia mai ma ka kakahiaka nui elemakule, akā, i kekahi manawa, ka mea, i ole diagnosed a adolescence a hiki adulthood.

PID - he pūʻulu o congenital maʻi, heʻano o ka lapaʻau hoike ana. International Mea Maluna o Nā Maʻi 36 ka loaʻa o hoʻokolokolo, a lawa ka ho'ākāka 'ia ma iniiaiie immunodeficiency Hawaii, akā, ka mea, e like me ka' inikua moʻokalaleo, ma laila nō ma kahi o 80. ka mea e ole a pau mai nā'ōewe i ua hōʻike 'kuleana.

Wale i ka'ōewe haku mele 'ana o ka X chromosome ua wehewehe aku ma ka ma ka liʻiliʻi loa eono okoa'ōnaehana paleʻea ia wā, a no ia mea, i ke alapine (frequency) o ka hanana o kēia mau maʻi i loko o nā kāne mea nui oi aku ma mua o ia o nā kaikamahine. Aia ka he mea mahuʻi i ka hooulu ana o congenital immunodeficiency hiki i aetiological ole o intrauterine maʻiʻaʻai, akā, i kēia koi ana i ole i ua scientifically ai.

lapaʻau kiʻi

Ka lapaʻau hoikeana o iniiaiie immunodeficiencies e like like me ka mea, ia lakou iho moku'āina, nō naʻe, he mea hoʻokahi, he pono ole hiʻona - hypertrophic infective (mea koʻohune) maʻi pale.

N immunodeficiencies, like hoʻi me kiʻekiʻe, hoike i ka welelau o ka pinepine relapsing hoʻomanawanui iki mai (recurrence) maʻi o infectious etiology a hiki e kauoha aku e atypical pathogens.

Kēia maʻi ua loa pinepine waiho kokoke ana i bronchopulmonary'ōnaehana a me ka ENT-nā loko. Kekahi pinepine ae aku mucous membranes a me integuments, a e hoike sepsis a me ka abscess. Mea koʻohune pathogens i bronchitis a me ka sinusitis. People me ka'ōnaehana paleʻea deficiency, e pinepine mālama i'ōhule, a eczema, a me kekahi manawa allergic reactions. Pinepine a me ka autoimmune kāna hana, a me ka propensity i ka malignancy. 'ōnaehana paleʻea deficiency i keiki ua aneane mau kumu i kali hou aku; o ka noʻonoʻo 'a me ka physical? Acaeoey.

ʻano papa hana o ka? Acaeoey o iniiaiie immunodeficiencies

Mea Maluna o ka maʻi, e like me keʻano papa hana o ko lākou ulu o ka loa informative i loko o ka hihia study o immunodeficiency Hawaii. Kauka e puʻunaue i ka'ōnaehana paleʻea ano o ka maʻi i loko o ka papa 4 pūʻulu:

- Humoral a B-aeea, i loaʻa Bruton ka maʻi pale (agammaglobulinemia e e hui i ka X chromosome) deficiency IgA a IgG, oi IgM me ka huina immunoglobulin deficiency, hemahema hoi ee iaaanu aey immunodeficiency, haliu ae la ia hypogammaglobulinemia keiki, a me ka helu o nā maʻi pili me ka humoral e hoʻonele .

- T aeea iniiaiie immunodeficiencies, pinepine kapa hui, mahope mai o na kāna hana mua mau wāwahiʻia a me humoral e hoʻonele, e laʻa me hypoplasia (Di George maʻi pale) ole dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies kena ae la ia e mā ma phagocytosis.

- Immunodeficiencies kena ae la ma kāna hana o ka hoʻokō nenoaiu.

susceptibility i ka maʻiʻaʻai

Mai ke kumu o ke immunodeficiency i e ka 'aʻe' ana o nā huahelu likeʻole
ka'ōnaehana paleʻea oihana, a me ka waiū i infectious Luna mea ole ka ia i kēlā me kēia hihia. No ka laʻana, ka wā o nā maʻi humoral ka hoʻomanawanui mea prone i nā mea palahēhē i i ke kumu ma ka streptococci, staphylococci, Haemophilus influenzae. Pela mau microorganisms pinepine ho'āʻo ia'ku i antibiotics. I ka hui immunodeficiency ano hiki ke pili i ka koʻohune nā mea hoʻomaʻi, e like me herpes a fungi i meaʻano nui na poe candidiasis. Phagocytic palapala Ua wehewehe akuʻano nui ma ka hookahi staphylococci a me Gram-io koʻohune.

Ke prevalence o iniiaiie immunodeficiencies

Immunodeficiencies, ili, - muli o kona kulana pohihihi keia kanaka maʻi. e ana kūponoʻia ke alapine (frequency) o ka'ōnaehana paleʻea kāna hana me ka pili ana i kēlā me kēia mau maʻi, no kā lākou prevalence loli aʻe.

Ma awelika, mai o kanalima tausani keiki wale nō kekahi e ae aku congenital hereditary immunodeficiency. Ka loa, he pono ole maʻi i loko o kēia pūʻulu o ka nele o e wae IgA. Congenital immunodeficiency o kēia 'ano ua loaʻa i loko o ka waena o kekahi o nā tausani o nā pēpē. A me ka 70% o nā hihia o IgA deficiency i huina pau ole o keia ke keʻena. I ka Ia manawa, kekahi hou pohihihi keia kanaka mai o ka'ōnaehana paleʻea ano, ili, e e haawi i loko o ka lākiō o ka 1: 1.000.000.

Inā ka mea hiki wale mai o ka maʻi PID ke kaumaha ma muli o kaʻano papa hana, ua noonoo, ka mea, ua haku loa hoihoi 'kulana. B-aeea iniiaiie immunodeficiencies, a me ka mea, e hou mau kapa, he 'aʻe' ana i ka lāʻau lapaʻau kino, oi pono ole ma mua o 'ē aʻe, a e i 50-60% o na hihia a pau. I ka Ia manawa, T-halepaahao a me ka phagocytic ano diagnosed i loko o 10-30% o ka hoʻomanawanui iki mai kēlā me kēia. Noʻonoʻo i ka loa pohihihi keia maʻi o ka'ōnaehana paleʻea'ōnaehana, kena ae la ia e mā ma ka hoʻokō - 1-6%.

Ua E hoi e hoʻomaopopo i ke alapine (frequency) o ka hananaʻikepili PID mea nui okoa ma kekahi'āina, a hiki e pili me ka aaiaoe predisposition o ka aina pūʻulu i kekahi DNA mutations.

ʻike, o ka immunodeficiency

Helu immunodeficiency i keiki ua loa pinepine nānā 'ia e ka untimely, no ka mea,
ka mea e ia i kaʻike, ma ka Apana pediatrician pae ka pilikia.

He IeAUPIIe, IAa IO hualoaʻa ma ke kali hou aku i loko o ka hoʻomaka o ka lapaau a me ka mea hune prognosis o Inc. Inā ke kauka ma muli o ka lapaʻau kiʻi o ka mai, a me ka mau hoao ana hualoaʻa? Aaei immunodeficiency moku'āina, o ka mea mua oia e hana - e hoouna aku i ke keiki i ke kauka-immunologist.
Ma Europa, aia o ka Hui o Immunologists, i epa a me ke ao a me ka hoʻomohala o ki ina hana like o ka hana maikaʻiʻole o ia maʻi, a koho i ka inoa o ka EDI ( 'Eulopa Society no immunodeficiencies). Manawa lākou i hana, a mau ka moʻolelo hōkeoʻikepili PID maʻi, a aponoia PalekanaEND_LINK algorithm no ka lawa kokololioʻike.

Hoʻomakaʻike, ma kaʻohi 'inikua mōʻaukala. E e uku ai i ke kuauhau kēia lākiō mau noonoo, like loa congenital immunodeficiencies i hereditary. Next, ma hope o ka hoʻoponopono ninaninau, a loaa mau lapaʻau noiʻiʻikepili i ka ka muaʻike. I ka wā e hiki mai, e hoʻokūpaʻa ai ke Säkona i ke kuhiakau Kauka, ka hoʻomanawanui e undergo i ka hoʻokolokolo pono ninaninau ma ka hoʻomalu e like me geneticist a me ka immunologist. Wale nō ma hope o nā mea a pau o ka luna manipulations mākou ke kamailio e pili ana i ka? Iecaianoaaiiie o kaʻike, hope loa.

keʻena hoʻokolohua noiʻi

Inā i kaʻike, ua kēia iniiaiie immunodeficiency maʻi pale, i ka i kēia keʻena hoʻokolohua e ho'āʻo ai i pono ai i ka:

- i ke kukuluia'na o ka piha koko helu (kākauʻana ma luna o ka helu ana o lymphocytes);

- hoʻoholo 'o ka lāʻau lapaʻau immunoglobulins;

- laupaʻi i ka ho omaulia o B- a me T-lymphocytes.

hou haʻawina

Ma waho aʻeo ia keʻena hoʻokolohua a me ka lapaʻau 'ana e ho'āʻo ai, a i mua hōʻikeʻia aʻela ma luna, na kanaka hou e ho'āʻo ai e e hāʻawi i kēlā me kēia hihia. Aia He He kamepiula pae, ka poe pono e e ikea no HIV maʻiʻaʻai, a aaiaoe abnormalities. I ke kauka e hoʻolako i ka manawa kūpono i ka makana kanaka immunodeficiency 3 a 4? Ueony, ma lalo o ka mea e koi aku ma luna o ka piha study o ka hoʻomanawanui ma ka phagocytosis ho'āʻo hana anakahi tetrazolinovym uliuli a me ka hōʻoia ke keʻena haku mele 'ana o ka hoʻokō nenoaiu.

PID lapaʻau

Ka mea, ua maopopo i ka mea e pono ai Inc, e hilinaʻi kuhikuhi mai i ka'ōnaehana paleʻea maʻi, akā, Eia naʻe, i ka congenital palapala hiki ole ke eliminated loa, i hiki ole ke olelo mai e pili ana i ka lilo ana immunodeficiency. Ma muli o kaʻikena 'inikua Aey? Acaeoey,ʻepekema e ho'āʻo ana e imi i ke ala, e wehe i ka hihia ma ka aaiaoe kiʻekiʻe. No laila, hiki, kā lākou mau hana i ole i holomua, ua hiki ke olelo aku i ka'ōnaehana paleʻea deficiency - he maʻi kulana. na loina o ka noi Inc noonoo.

hope Inc

Lapaʻau o ka'ōnaehana paleʻea deficiency ana hoemi ia i ka hope Inc. E like me ka olelo mua, i ke kino o ka hoʻomanawanui mea i hiki ia oe ke paka kekahi mau eiiiiiaiou o ka'ōnaehana paleʻea oihana, a me ka nui loa emi i ko lakou mea e like ai koi 'ia. Inc i keia makemake e i ka lapaʻauʻia? Eaia nā mea'aʻaʻa paha immunoglobulins, ma kona ano maoli huahana ua uhaʻiʻia. Loa pinepine, ka lawelaweia ana intravenously ai ', akā, i kekahi manawa, he mea hiki, a me ka subcutaneous ala hele, e kōkua i ke ola o ka hoomanawanui, a ma keia hihia, aole ia i i i kekahi manawa hou kipa i ka' inikua wahi uku paha.

hakahaka rula pinepine e leie aku nā mea maʻi e alakai aneane maʻamau ola: e aʻo, ka hana a me ka pāʻani. O ka holo ana, he hoʻonāwaliwaliʻia'ōnaehana paleʻea kahua maʻi, humoral a me ke kelepona hele aʻe, a me ka ikaika nele no ka hoʻokomo 'ia' o ka pipiʻi ai 'ole e ae i ka hoʻomanawanui i maopopo hoʻonānea, akā, e mea nō maikaʻi ma mua o noho ana ma o ka manaʻo koikoi keena.

Symptomatic lapaau a me ka Kāohi

Ua haawiia i ka mea i kekahi mea uuku no ke ola i ke kanaka mea koʻohune a mea hoʻomaʻi maʻiʻaʻai no ka mea hoʻomanawanui pūʻulu o iniiaiie immunodeficiency maʻi hiki e ona, ka mea i pono, e lawe mai Kāohi competently. Here hele mai i loko o hula antibacterial, antifungal a me antiviral ai '. Key ana e hana ia mea ma luna o kāohi ana, no ka mea, hiki ole i ka hoʻonāwaliwaliʻia'ōnaehana paleʻea'ōnaehana hoʻolimalima i ka i kona mea e like lapaau.

Eia hou, e hoʻomanaʻoʻia ai ia i kēia mau hoʻomanawanui iki mai i prone i allergic, autoimmune, a, a hiki ino, iʻeho Hawaii. A pau keia me ka piha 'inikua hooponopono hiki ole ae i ke kanaka, e alakai i ka piha ola.

transplantation

I ka poe akamai i hooholo i ka hoʻomanawanui, aole ia e noho i waeʻia akā, eʻoki kino, hiki ke hana iwi lolo transplantation. Kēia ke kaʻina hana no ka pili me ka mau ka pilikia no ke ola a me ka ola o ke ahonui, a me ka hana, a hiki i ka hopena o ka holomua hoʻokūkū,ʻaʻole hiki mau ke hoʻoponopono i nā pilikia o ka loaa ana mai o ka'ōnaehana paleʻea kāna hana. Ma ka hooko ana mai ia i ka hana, i ka'ōnaehana holoʻokoʻa ua auou caiaiai ia e ka haumāna haʻawina kālā hematopoiesis e like me i hoakaka ia ma ka pou.

N immunodeficiencies i ka loa pilikia pilikia o ke kālā no lapaau, a, Eia naʻe, aole lakou i noonoo loa. Ilihune prognosis no ka maʻi o keia ano nō ka i lanakila, a me ia meaʻale huli pōʻino, haawiia i ka mea i ka mea, e ae aku ka hapanui keiki. Aka hoi, he nui na ano o ka'ōnaehana paleʻea deficiency mea hana maʻalahi me ka piha ola ina ka mea, ua iʻike, a me ka hoʻohana 'ana i kūpono Inc.