Bruton ka maʻi: Etiology, ke kumu, symptoms a me ka hana hiʻona

Aaiaoe maʻi - ia mea he pohihihi congenital maʻi i mea ole, e kilokilo ma mua. Ka mea, i hoʻomaka ai, a hiki i kekahi manawa, i ka wa laila mea i ka ho okumu ana o ka embryo. Loa pinepine, ka mea, i hele mai na makua, akā, ka mea aole e mau hana. Ma kekahi mau hihia, 'aʻe' ana i loko o nā nā'ōewe lakou iho. I kekahi o kēia mau pathologies ua noʻonoʻo X-ua hoʻopili agammaglobulinemia. Ua pili ia i ka iniiaiie immunodeficiency. I ka maʻi i nei wehe i loko o ka hapalua like-20th kenekulia. No laila, ka mea, ua i maopopo maopopo ia e kauka. Ua lŘlŘ loa kākaʻikahi, wale nō i loko o nā keikikāne.

Bruton ka maʻi: i ka mōʻaukala o

Kēia pathology e pili pū ana i ka X-ua hoʻopili chromosomal anomalies pū i hiki i ka aaiaoe kiʻekiʻe. Bruton ka maʻi ua wehewehe aku ma abnormalities ma humoral e hoʻonele. I ka papa kuhikuhiE symptom o kona susceptibility i infectious keʻano o ka hanaʻana. Ka mua iki o ka mooolelo no ka 1952 o keia pathology. I ia manawa, ai ka American nä känaka 'epekema Bruton aʻo mōʻaukala o ke keiki i maʻi oi ma mua o 10 manawa i loko o 4 makahiki o ka makahiki. I waena o ua hālāwai infectious keʻano o ka hanaʻana i kēia keiki septicemia, pneumonia, meningitis, luna naehana'āpana li nui. Ka wā keiki ina hookolokolo, ka mea i loaʻa i nā mea'aʻaʻa i kēia mau maʻi ma kahi e. Ma na olelo e ae, ma hope o ka'ōnaehana paleʻea muli o ia i mālama nā mea palahēhē.

Hope, ma ka hopena o ke kenekulia 20th, X-ua hoʻopili agammaglobulinemia kauka hookolokolo hou. I ka makahiki 1993, ua hiki ke kuhikuhi i ka hemahema'ōewe i ke kumuʻole e hoʻonele kauka.

Kumu o ka X-ua hoʻopili agammaglobulinemia

Agammaglobulinemia (Bruton ka maʻi) pinepine mea hereditary. Ke kina Ua manaoia e e he recessive kāna moʻolelo, no laila, i ka pahiki o ka ke keiki me ka pathology o 25%. Nā moku halihali mokulele o ka mutant'ōewe i na wahine. O ke kumu ho i ka mea i ke kina ua IIeEeAIEUIeXAIIUE ma luna o ka X chromosome. Aka hoi, i ka maʻi ua pū i hiki wale i ka kane wahine. Ke kumu nui agammaglobulinemia mea hemahema kumuʻiʻo, hapa o ka'ōewe helu kuʻina no tyrosine kinase. Eia hou, i ka Bruton ka maʻi hiki e idiopathic. Kēia 'o ia hoʻi i ka aoao o kona hanana noho unclear. Mawaena o ke kamepiula kumumea i aloha ia i ke keiki ka aaiaoe kivila, līkaia no:

1. Hainā o ka waiʻona, a ai 'i hāpai keiki.
2. Psycho-mea naʻau ke kanana nei.
3. Waiū i ionizing pāhawewe.
4. Nine irritants (ʻino wale i? Iecaianoaaiiie, noho lakou malaila 'ia).

He aha ka ka pathogenesis o ka maʻi?

ʻano papa hana o ka maʻi pili i ka hemahema kumuʻiʻo. Maʻamau, ka'ōewe kuleana no ka hoʻopā'ālua tyrosine kinase, ua komo i loko o ke kahua o ka B-lymphocytes. Ka mea, e'ōnaehana paleʻea aeee i nā kuleana no humoral pale. Aie i ka insolvency o tyrosine kinase B lymphocytes mai i oo loa. E like me ka hopena, e hiki i ka paka immunoglobulins ka mea, - nā mea'aʻaʻa. Ke pathogenesis o ka maʻi Bruton o ka piha wahi o humoral hoomalu. E like me ka hopena, ma ka hui 'ana me infectious Luna i loko o ke kino nā mea'aʻaʻa ana e i lawe. A kūikawā hiʻona o keia maʻi mea i ka'ōnaehana paleʻea nenoaiu mea hiki ke kaua aku i ka mea hoʻomaʻi, hoʻowahāwahā i ka wa e kaawale aku o B-lymphocytes. Nature o ka 'aʻe' ana o humoral hoopakele hilinaʻi nui ma luna o ka oolea o ke kina.

Bruton ka maʻi: maʻi symptoms

Pathology no ka manawa muaʻo ia ia iho i manao ai ma kaʻuʻuku mai. Loa pinepine, i ka maʻi hoike mai ai ia ia iho i loko o 3-4-la mahina o ke ola. O ke kumu ho i ka mea e ma keia makahiki i ke kino o ke keiki oki nā mea'aʻaʻa i ka makuahine o ka mea e pono ai. Ka mua hoailona o ka maʻi hiki e ke ola naoh kēia vaccination, ili rashes, nā mea palahēhē o ka luna a lalo naehana'āpana. Eia naʻe, breastfeeding i malama i ke kamaiki mai ka hoʻoʻino keʻano o ka hanaʻana, mai ka umauma waiu i loko o kona haku mele 'ana o immunoglobulins.

Bruton maʻi hoike mai ai ia ma kahi o 4 makahiki. I keia manawa i ke keiki ke hele mai i loko o hui 'ana me nā keiki i loko o kindergarten. Iwaena o infectious lesions Kīwala'ō meningo, streptococci a me staphylococcal microflora. E like me ka hopena, na keiki i ke kokoke ana i purulent li nui. Ka loa pinepine mai nā pneumonia, sinusitis, otitis Media, sinusitis, meningitis, conjunctivitis. Ma untimely lapaʻau no a pau o kēia mau keʻano o ka hanaʻana ke hele ma ka sepsis. Ua mea no hoi i ka hoike ana o ka maʻi i ia Bruton dermatological pathology. Aie i ka hoemi'ōnaehana paleʻea muli o microorganisms mahuahua hoʻomaka koke i ka kahua o ka eha a me ka scratches.

Ma waho aʻeo i ka hoike ana mai o ka maʻi nā bronchiectasis - pathological loli ma ka ia maʻiʻaʻai. Symptoms nā pokole o ka hanu,ʻeha i loko o ka pahu, i kekahi manawa hemoptysis. Ua mea no hoi e hiki i ka muli o hoʻoʻino lesions i loko o ka digestive nā loko, urinary'āpana, mucous membranes. Kekahi manawa mālama nani a me ka palupalu i loko o na ami.

Ke PalekanaEND_LINK ana hoʻohālike no ka maʻi

Ka mua PalekanaEND_LINK ana hoʻohālike e loaʻa i ka pinepine mea hiki wale mai. Children loaa mai Bruton ka pathology, e lawe aku oi ma mua o nā mea palahēhē 10 no ka makahiki, e like me kekahi mau manawa iloko o ka mahina. Maʻi hiki e hai 'ole' ē aʻe (otitis Media, tonsillitis, pneumonia). Ma keia hookolokolo ana, aia no he nele i pharyngeal tonsil hypertrophy. ʻO ia pili i ka palpation o kaʻike ākea lymph aka wahi. Oe E hoi e lohe i ka bebe ka naoh ma hope o vaccination. Mea hou i malamaia ma ka keʻena hoʻokolohua assays. Ka KLA na hoailona o ka li nui (hoonuiia ka helu o leukocytes hōʻeleu i ESR). ua hoemi ia ka helu o ka'ōnaehana paleʻea keena. Kēia i ka lewa i loko o ka leukocyte haʻilula: he uuku helu o lymphocytes a me neutrophils mahuahua maʻiʻo. Nui noiʻi mea immunogram. Ua Hōʻike 'ka ka ho'ēmiʻana a me ka wa e kaawale aku o nā mea'aʻaʻa. Kēia hiʻona apono 'oe e hana i kaʻike,. Inā ke kauka he mea pohihihi, he mea hiki, e lawe mai aaiaoe ikea ai.

Oko Bruton maʻi, mai like pathologies

Kēia pathology ua differentiated mai nā iniiaiie a me nā haumāna immunodeficiencies. Iwaena o lakou - o ka Hawaiian type agammaglobulinemia, Di Dzhordzhi ka maʻi pale, HIV. Akä naÿe i mau pathologies no Bruton ka maʻi ua wehewehe aku ma wale i ka 'aʻe' ana o humoral e hoʻonele. Keia ua hoikeia aku ai e na mea ana i ke kino mea hiki ke kaua mea hoʻomaʻi konohiki. Kēia ololi mea okoa mai ka Hawaiian type agammaglobulinemia, a ua uhaʻiʻia ka wā nā humoral a me ke kelepona hele'ōnaehana paleʻea pane. E hana i ka palena pauʻike, a me ka maʻi pale Di Dzhordzhi, e pono e hana umauma X-Ray (thymic aplasia) a, e hooholo i ka kalipuna maʻiʻo. E hookaawale ia HIV alakai aku ia palpation o lymph aka wahi, Elisa.

Nā Papahana O Ka lapaʻau o agammaglobulinemia

Eia naʻe, loa lanakila Bruton ka maʻi mea hiki ole. Ke ki ina hana like o ka lapaʻau nā agammaglobulinemia hope a me ka symptomatic Inc. I ka papa kuhikuhiE manao o ka e piʻi like ua manaoia e e mahana maʻamau o immunoglobulins i loko o ke koko. I ka nui o nā mea'aʻaʻa E e He kokekau wale nō i ka g. 3 / ho'āo ai. No keia mea, gamma globulin i ka uku o ka 400 mg / kg kino ke kaumaha. E e mahuahua lāʻau lapaʻau kino lehulehu ma huʻi infectious maʻi, me ka kino hiki ole Makuakane me ia kū kaʻawale.

Eia hou kekahi hana symptomatic lapaau. Ka loa pinepine hoakaka antibiotics "Ceftriaxone", "Penicillin", "Ciprofloxacin". I ka ili hoike ana pono i topical lapaau. I paipai holoi mucosal antiseptic pāʻoihana (puʻu, a me ka nasal nenoaio i?).

Ke prognosis no ka Bruton ka agammaglobulinemia

I loko nō o ka lifelong hope Inc, ka prognosis no ka maikaʻi agammaglobulinemia. Ana lapaau a me ka prophylaxis o infectious keʻano o ka hanaʻana emi morbidity minimized. Nō kāu, nā mea maʻi i hiki-kino puʻipuʻi a me kaʻeleu. Ma ka hewa kokoke i ka lapaʻau hiki hoʻomōhala complications a sepsis. Ma ka hihia o ka holo nā mea palahēhē hune prognosis.

Kāohiʻana o ka X-ua hoʻopili agammaglobulinemia

Ma ke alo o ka maʻi i loko o poe hoahanau, a me kēia mea o pono ai a hiki i ka trimester mua o ka hāpai keiki, e hoʻomaka aaiaoe ikea ai. No hoi i kāohi ana e hoʻohui i waiū i ka lewa, i ka wa e kaawale aku o ka maʻi nā mea palahēhē a me nā palapala 'ole. Ma hāpai keiki, ka makuahine o contraindicated stress. No ka haumāna Kāohiʻana i wikamina Inc, hooko ana o ka gamma globulin, he olakino Aloha Hawaii. Ua mea i nui, e pale aku me ka hui 'iho mailoko o kanaka.