Disease Bruton kuti: Etiology, hunokonzera, zviratidzo uye kurapwa mamiriro

Genetic chirwere - iri chisingawanzowaniki vachiberekwa chirwere kuti kwakaoma kufanotaura pachine. Zvaitika kunyange panguva pane paumbwe embryo. Kakawanda, zviri vakapfuura vachibva vabereki, asi haugari kuitika. Mune zvimwe zviitiko, kutyorwa kuitika mune majini pachavo. Rimwe pathologies izvi anonzi X-chokuita agammaglobulinemia. Rinoreva chikuru immunodeficiency. The chirwere achangobva akazarura pakati pezana remakore rechi20. Saka, hazvina aizvinzwisisa navanachiremba. It kunoitika chaizvo kashoma, chete vakomana.

Bruton nehosha: nhoroondo

zvokurwara ichi chine chokuita X-chokuita chromosomal anomalies chinopfuudzwa panguva majini chechetere. chirwere Bruton inozivikanwa nokuva abnormalities mu humoral kutapata. The chikuru chiratidzo chiri susceptibility kune noutachiona kwendangariro. Kutaurwa kutanga 1952 kubva zvokurwara ichi maakaundi. Panguva iyoyo, American musayendisiti Bruton akadzidza nhoroondo mwana ari kurwara anopfuura 10 kanomwe 4 makore. Pakati zvirwere pezvakaitwa vanosangana ichi mukomana septicemia, mabayo, yomumuzongoza, rokumusoro kufema turakiti kuzvimba. Paanoongorora vana, kwakaonekwa kuti antibodies zvirwere izvi hatisi. Nemamwe mashoko, pashure zvirwere mhinduro haina kuchengetwa zvirwere.

Gare gare, mukupera remakore rechi20, X-chokuita agammaglobulinemia vanachiremba aongororwe zvakare. Muna 1993, vanachiremba vaikwanisa kuziva chakaremara Gene hunokonzera havachanyatsogoni kutapata.

VaBennett X-kwakabatana agammaglobulinemia

Agammaglobulinemia (Bruton nehosha) kazhinji ndiyo nhaka. Urema anoonekwa kuva recessive unhu, saka mukana wokuva mwana zvokurwara ndiyo 25%. Vatakuri mutant Gene vakadzi. Izvi imhaka yokuti ndiko kuremara vemunyika pamusoro X chromosome. Kunyange zvakadaro, maperembudzi inoparadzirwa chete kune vanhurume. Chikonzero chikuru agammaglobulinemia riri chakaremara mapuroteni, chikamu majini Coding kuti tyrosine kinase. Mukuwedzera, chirwere ari Bruton anogona kuva idiopathic. Izvi zvinoreva kuti mhosva parinowanika anoramba kujeka. Pakati zvinokonzera njodzi chokuita majini Mubumbiro wemwana, govera:

1. Abuse doro uye zvinodhaka panguva yepamuviri.
2. Psycho-Mupfungwa.
3. Kuonana ionizing nemwaranzi.
4. Chemical irritants (kukuvadza kugadzira, vanoshorwa zvakatipoteredza).

Chii pathogenesis chirwere?

Mashandiro chirwere chokuita chakaremara mapuroteni. Kazhinji, majini anokonzera yeEncoding tyrosine kinase, rinobatanidzwa paumbwe B-lymphocytes. Ndivo masero zvirwere kuti vane basa humoral kudzivirira. Nokuda insolvency kuti tyrosine kinase B lymphocytes regai vakuru zvakakwana. Somugumisiro, havakwanisi kubereka immunoglobulins - antibodies. The pathogenesis chirwere Bruton ndiye ichivharira yakakwana humoral kudzivirirwa. Somugumisiro, uchikurukura zvirwere vamiririri kupinda mumuviri antibodies kwazviri vasiri akabudiswa. Chimwe chinhu chinokosha chirwere ichi ndechokuti zvirwere anokwanisa kurwisa utachiona, pasinei kusavapo B-lymphocytes. Nature of kutyora humoral kudzivirirwa kunoenderana kwokuomerwa ndiko kuremara.

Bruton kuti Disease: chirwere zviratidzo

Zvokurwara kekutanga inoita pacharo sokuti ari mucheche. Kakawanda, hosha kunooneka pacharo muna 3-4-fung mwedzi upenyu. Izvi imhaka yokuti pazera ichi muviri mwana kurega kudzivirira antibodies waamai. Zviratidzo chirwere wokutanga inogona chinorwadza maitiro majekiseni anotevera, ganda rashes, zvirwere vaikudzwa kana nechepazasi kufema turakiti. Zvisinei, achiyamwa inodzivirira mwana kubva anogumbura urongwa, sezvo mukaka ane ayo Zvinoumba immunoglobulins.

Bruton chirwere kunoratidza munenge 4 makore. Panguva iyi mwana anouya kuonana nevamwe vana kukireshi. Pakati zvirwere maronda akatonga meningo, streptococci uye staphylococcal microflora. Somugumisiro, vana vanogona chavakaona purulent kuzvimba. Zvikuru kakawanda zvirwere zvinosanganisira nemabayo, sinusitis, otitis nhau, sinusitis, yomumuzongoza, conjunctivitis. At kukurumidza kurapwa zvose azvaiitwa anogona kupinda sepsis. Ndizvo zvinoratidza chirwere chingava Bruton dermatological zvokurwara. Nokuda wakaderedzwa zvirwere norudaviro tusingaoneki ikawanda nokukurumidza pane nzvimbo maronda uye anokwenya.

Kuwedzera zvinoratidza chirwere zvinosanganisira bronchiectasis - Kumwe kuchinja kunotanga mumapapu. Zviratidzo zvinosanganisira kupfupika mweya, kurwadziwa muchipfuva, dzimwe nguva hemoptysis. Zvinogonekawo kuti kwaita anogumbura maronda ari zvomudumbu zvomukati, Urinary turakiti, mucous membranes. Nguva nenguva akacherechedza kuzvimba uye unyoro majoini.

The chirwere dzekuziva kuti chirwere

Yokutanga chirwere zvinodiwa zvinofanira kusanganisira kakawanda kuitika. Vana vachitambura kubva Bruton kwakaita zvokurwara, takura anopfuura 10 Zvirwere pagore, uyewo kanoverengeka mwedzi. Zvirwere zvingadzokororwa kana humwewo (otitis zvenhau, tonsillitis, mabayo). On ongororo, pane kushayikwa pharyngeal ndimbo hypertrophy. Izvi zvinoshandawo palpation pamusoro peripheral node nodes. Unofanirawo kuteerera mwana ichatii pashure kubaya. Kuchinja vakanga kuchengetwa murabhoritari assays. The KLA Zviratidzo kuzvimba (nhamba yakawedzera kubva leukocytes wakakurumidza ESR). Kuwanda masero masoja ipfupi. Izvi zvinoratidzwa leukocyte payakavakirwa: vashoma lymphocytes uye neutrophils kwakawedzera achigutsikana. Kutsvakurudza chinokosha immunogram. Zvinoratidza rutapudzo kana kusavapo antibodies. Nyaya iyi kunoita kuti vaone chirwere. Kana chiremba ane chokwadi, zvinokwanisika kuita magene yokuedzwa.

Kusiyana Bruton chirwere kubva zvakafanana pathologies

zvokurwara Izvi kusiyaniswa nevamwe zvepuraimari yechipiri immunodeficiencies. Pakati pavo - neSwiss mhando agammaglobulinemia, Di Dzhordzhi kuti chirwere, HIV. Kusiyana pathologies izvi nokuda Bruton nehosha kunoratidzwa chete kutyora humoral kutapata. Izvi kunoratidzwa ndechokuti muviri anokwanisa kurwa nemavhairasi nevamiririri. chinhu ichi akasiyana Swiss mhando agammaglobulinemia unomedurirwa apo vose humoral uye cellular masoja norudaviro. Kuti differential chirwere pamwe chirwere Di Dzhordzhi, unofanira kuita chipfuva X-ray (thymic aplasia) uye kuziva karusiyamu kugutsikana. To siya HIV inoitwa palpation pamusoro node nodes, Elisa.

Nzira Mabatirwo agammaglobulinemia

Zvinosuruvarisa chose kukunda chirwere Bruton kuti hazvibviri. Nzira yokurapa zvinosanganisira agammaglobulinemia yokutsiva uye symptomatic kurapa. Chinangwa chikuru kubudirira anoonekwa kuti zvinoitika kwevari immunoglobulins muropa. Huwandu antibodies anofanira approximated 3 g / l. Nokuti chinangwa ichi, gamma globulin mwero 400 MG / makirogiramu muviri uremu. Antibody wevasungwa inofanira ngazviwedzerwe panguva chakasimba zvinotapukira, sezvo muviri haagoni kurarama navo tisingatungamirirwi.

Mukuwedzera akaita symptomatic kurapwa. Inonyanyoshandiswa ushandiswe mishonga "Ceftriaxone", "Penicillin", "Ciprofloxacin". Kana ganda kuratidzwa vanofanira kukamura uchishandisa kurapwa. Dzakamukurudzirawo Washing mucosal akambozvishandisa kuuraya utachiona mhinduro (huro uye nasal kudiridza).

The fungidziro nokuti Bruton kuti agammaglobulinemia

Pasinei woupenyu hwose yokutsiva mushonga, asi fungidziro kuti zvakanaka agammaglobulinemia. Kuramba kurapwa uye prophylaxis nezvirwere pezvakaitwa kuderedza hwevanhu vanorwara kushoma. Kazhinji, varwere vari akagwinya uye tichishingaira. Panguva isiri nzira kunokwanisa kuva matambudziko kusvikira sepsis. Kana uchimhanya zvirwere murombo fungidziro.

Kudzivirira X-kwakabatana agammaglobulinemia

In pamberi zvirwere hama kana vanofungidzirwa zvakafanira kuti kuitisa majini yokuedzwa kunyange panguva trimester akazvitakura rokutanga. Uyewo kune matanho inofanira kusanganisira nezvemidzimu mumhepo, kusavapo chisingaperi noutachiona uye zvinokonzerwa. Panguva pamuviri, mai contraindicated kunetseka. Nokuti dziviriro yechipiri vari vhitamini kurapa, kwokubatsira gamma globulin, hwakanaka mararamiro. Zvinokoshawo kuti usagunzva utachiona vanhu.