Bruton Nthenda: Etiology osiyanasiyana, zizindikiro ndi mbali chithandizo

matenda majini - ndi chosowa kobadwa nako matenda ovuta kulosera pasadakhale. Zidzachitika ngakhale pa nthawi imene pali mapangidwe Ndisanaumbidwe lapansi. Nthawi zambiri, iwo adutsa kuchokera ku makolo, koma sikuti kuchitika. Nthawi zina, kuyan'anila zimachitika majini okha. Mmodzi wa pathologies zimenezi akuti X-zogwirizana agammaglobulinemia. Zikutanthauza immunodeficiency chachikulu. Matendawa posachedwapa lotseguka m'ma 20. Choncho si bwino bwino kwa madokotala. Kumachitika kawirikawiri, kokha anyamata.

matenda Bruton akuti: mbiri ya

kudwala izi zokhudzana ndi X-zogwirizana chromosomal anomalies opatsirana pa mlingo chibadwa. matenda Bruton a amakhala ndi kuvutika mu chitetezo chokwanira humoral. The chizindikiro chachikulu ndi chiwopsezo ake kuti njira opatsirana. Kutchula koyamba nkhani kwa 1952 wa kudwala izi. Pa nthawi imeneyo, American wasayansi Bruton kuphunzira mbiri ya mwana kukhala kudwala nthawi zoposa 10 mu zaka 4 zakubadwa. Mwa njira opatsirana anakumana mnyamata septicemia, chibayo, matenda oumitsa khosi, chapamwamba kupuma thirakiti kutupa. Pamene kufufuza ana, anapezeka kuti chitetezo ku matenda amenewa ali kwina. M'mawu ena, Akayankha ndi chitetezo silinkachitidwa matenda.

Kenako, cha m'ma 20 mochedwa, X-zogwirizana agammaglobulinemia madokotala anafufuza kachiwiri. Mu 1993, madokotala anali kudziwa jini zosalongosoka zimene zimachititsa chitetezo chokwanira mkhutu.

Zimayambitsa X-zogwirizana agammaglobulinemia

Agammaglobulinemia (matenda Bruton a) Kawirikawiri ndi cholowa. Chilema imatengedwa kuti ndi recessive khalidwe, kotero Mwina ndi mwana ndi kudwala ndi 25%. Onyamula jini ZOOPSA ndi akazi. Ichi ndi chifukwa chakuti chilema isanafalikire pa X khromozomu. Komabe, matenda imafalikira yekha kwa chiwalo chachimuna. chifukwa chachikulu agammaglobulinemia zolakwika mapuloteni, mbali ya 'kala' ikusonyeza jini kwa tyrosine kinase. Komanso, matenda Bruton akhoza kukhala idiopathic. Izi zikutanthauza kuti chifukwa cha pakuwonekera kwake amakhalabe bwinobwino. Zina mwa zinthu chiopsezo zimene zimakhudza malamulo majini mwanayo, kuonetsetsa:

1. Kumwa mowa ndi mankhwala pa mimba.
2. Psycho-maganizo kupsyinjika.
3. Ndinakumana ionizing cheza.
4. irritants mankhwala (sichikupindulitsani kupanga akumane malo).

ndi pathogenesis matenda chiyani?

Limagwirira matenda kugwirizana ndi puloteni zosalongosoka. Mwachibadwa, jini udindo kabisidwe tyrosine kinase, kumatanthauza mapangidwe B-lymphocytes. Iwo maselo mthupi amene ali ndi udindo odziteteza humoral. Chifukwa insolvency wa tyrosine kinase B lymphocytes sakhwimitsa zipatso zamphumphu kwathunthu. Chifukwa, iwo analephera kupanga immunoglobulins - chitetezo. The pathogenesis matendawa Bruton ndi kutsekereza wathunthu chitetezo humoral. Chifukwa, anakumana ndi wothandizila opatsirana mu chitetezo cha thupi thereto si opangidwa. Mbali yapadera ya matenda ndi kuti chitetezo cha m'thupi akhoza kulimbana ndi kachilombo, ngakhale pakalibe B-lymphocytes. Nature wa kuphwanya chitetezo humoral zimadalira kuopsa kwa chilema.

Bruton Nthenda: zizindikiro matenda

Kudwala kwa nthawi yoyamba zimapangitsa lokha anamva ali wakhanda. Nthawi zambiri, matenda kumaonekera mwezi 3-4-TH wa moyo. Ichi ndi chifukwa chakuti pa m'badwo uno thupi la mwanayo Kusiya kuteteza chitetezo cha mayi. Zizindikiro zoyambirira za matenda angakhale opweteka anachita izi katemera, tizilonda ta pakhungu, matenda a thirakiti chapamwamba kapena kuchepera kupuma. Komabe, yoyamwitsa kumateteza mwana kuti njira yotupa, popeza mkaka wa m'mawere ali mu kapangidwe kake wa immunoglobulins.

matenda Bruton kuwawonetsera pafupifupi 4 zaka. Panthawi imeneyi mwana amabwera mu kukhudzana ndi ana ena ku sukulu ya mkaka. Pakati zotupa opatsirana chogwidwa meningo, streptococci ndi microflora staphylococcal. Motero, ana akhoza poyera kuti purulent kutupa. The matenda zambiri monga chibayo, sinusitis, media otitis, sinusitis, kakhosi, conjunctivitis. Pa mankhwala yosayembekezereka ya onse a pasukuluyi mukhoza kupita mu sepsis. Ndi chiwonetsero cha matenda mwina Bruton dermatological kudwala. Chifukwa yafupika m'thupi ayankhe tizilombo kuchulukitsa mofulumira pa malo mabala zimakhalapo.

Kuwonjezera pa kuwonekera kwa matenda monga bronchiectasis - kusintha pathological mu mapapo. Zizindikiro monga kufupika kwa mpweya, kupweteka m'chifuwa, nthawizina hemoptysis. N'zothekanso zikamera wa zotupa yotupa mu ziwalo m'mimba thirakiti mkodzo, zikopa mucous. Nthawi anati kutupa ndi mwachikondi malo.

akugwirizana ndi matenda matendawa

Woyamba i matenda muyenera kukhala zochitika pafupipafupi. Ana akuvutika kudwala Bruton ndi kunyamula matenda oposa 10 pa chaka, komanso kangapo pa mwezi. Matenda uyenera kubwerezedwa kapena taphunzira (otitis TV, zilonda zapakhosi, chibayo). Kuzionetsetsa, kulibe pharyngeal tonsil hypertrophy. N'chimodzimodzinso palpation a mwanabele zotumphukira. Muyeneranso kulabadira zimene mwana pambuyo katemera. kusintha kwakukulu anali anamuona pa kafukufuku zasayansi. The KLA zizindikiro za kutupa (chinawonjezeka chiwerengero cha leukocytes inapita ESR). chiwerengero cha maselo mthupi yafupika. Izi zikupezeka mu leukocyte chilinganizo: ochepa lymphocytes ndi neutrophils kuchuluka okhutira. kafukufuku n'kofunika immunogram. Izo zimanyezimiritsa kuchepetsa kapena kusapezeka kwa chitetezo. Mbali imeneyi zimathandiza inu kuti matenda. Ngati dotolo akukayikira, n'zotheka kuchita kuyezetsa chibadwa.

Kusiyana Bruton matenda kuchokera pathologies ofanana

kudwala Izi kusiyanitsidwa ndi zina pulayimale ndi immunodeficiencies sekondale. Pakati pawo - Swiss mtundu agammaglobulinemia, syndrome Di Dzhordzhi ndi HIV. Mosiyana pathologies awa matenda Bruton a amakhala ndi kuphwanya chitetezo chokwanira humoral. Izi ziri kuwonetseredwa ndi chakuti thupi ndi zakutha kulimbana wothandizila tizilombo. Chinthu Ichi ndi chosiyana kuchokera Swiss mtundu agammaglobulinemia, amene wosweka pamene onse humoral ndi ma ayankhe m'thupi. Kupanga masiyanidwe matenda ndi matenda Di Dzhordzhi, muyenera kuchita pachifuwa X-ray (thymic aplasia) ndi kudziwa zili calcium. Kuti achotse HIV imachitika palpation a mwanabele, Elisa.

Njira ya chithandizo cha agammaglobulinemia

Mwatsoka, kwathunthu kugonjetsa matenda Bruton ndi zosatheka. The njira ya mankhwala monga agammaglobulinemia m'malo ndi mankhwala symptomatic. Cholinga cha Kupambana akuti ndi milingo wabwinobwino wa immunoglobulins m'magazi. Kuchuluka kwa chitetezo ayenera approximated 3 g / L. Chifukwa chaichi, Popanga madzi a gamma globulin mlingo wa 400 mg / kulemera makilogalamu thupi. Oteteza ndende ayenera ziwonjezeke pa matenda pachimake matenda, monga thupi sangathe kulimbana nawo paokha.

Komanso anachita mankhwala symptomatic. The kaŵirikaŵiri Kwalamulidwa mankhwala "Ceftriaxone", "Penicillin", "Ciprofloxacin". Pamene mawonetseredwe khungu ayenera mankhwala apakhungu. Analimbikitsanso kutsuka mucosal njira antiseptic (pakhosi ndi ulimi wothirira m'mphuno).

Kuti madokotala ananena zawo kwa agammaglobulinemia Bruton a

Ngakhale kuti moyo wonse m'malo mankhwala kuti madokotala ananena zawo kwa agammaglobulinemia yabwino. mankhwala mosalekeza ndi prophylaxis njira opatsirana kuchepetsa odwala sanamvere. Childs, odwala ndi wamphamvu ndi akuchitachita. Pa njira yolakwika mankhwala angathe mavuto mpaka sepsis. Pankhani ya akuthamanga matenda madokotala ananena zawo osauka.

Kupewa X-zogwirizana agammaglobulinemia

Pamaso pa matenda mu achibale kapena amaganiziridwa m'pofunika kuchita kuyezetsa majini ngakhale pa trimester mimba. Komanso zodzitetezera pazikhalanso kukhudzana ndi mpweya, pakalibe matenda aakulu ndiponso mavuto. Woyembekezera, mayi ndi contraindicated nkhawa. Kupewa yachiwiri ali vitamini mankhwala, utsogoleli wa Popanga madzi a gamma globulin, moyo wathanzi. M'pofunikanso kuti musayandikire anthu kachilombo.