Bruton ta cuta: etiology, Yanã, cututtuka da kuma magani siffofin

Kwayoyin cuta - shi ne mai rare nakasar cutar da cewa da wuya a hango ko hasashen a gaba. Da suka faru ko da a wani lokaci a lokacin da akwai wani samuwar amfrayo. Mafi sau da yawa, su sun shige daga iyaye, amma shi ba ya ko da yaushe faru. A wasu lokuta, take hakki da faruwa a cikin halittar da kansu. Daya daga cikin wadannan pathologies aka dauke X-nasaba agammaglobulinemia. Yana nufin wani primary immunodeficiency. Da cutar ta kwanan nan ya buɗe a tsakiyar karni na 20th. Saboda haka, an ba da cikakken gane abin da likitoci. Yana auku sosai da wuya, kawai a boys.

Bruton ta cuta: tarihin

Wannan Pathology alaka X-nasaba chromosomal anomalies daukar kwayar cutar a cikin kwayoyin matakin. Bruton ta cutar halin munanan a humoral rigakafi. Babban alama ne da laulayi dauke da kwayar cutar tafiyar matakai. A farko ambaci asusun ga shekara 1952 wannan Pathology. A wannan lokacin, da American masanin kimiyya Bruton karatu tarihi na yaro kasancewa da rashin lafiya fiye da sau 10 a cikin 4 shekara. Daga cikin cututtuka matakai sun hadu da wannan yaro septicemia, ciwon huhu, meningitis, babba numfashi fili ƙonewa. Yayin da muke yara, an gano cewa, antibodies to wadannan cututtuka ne ba ya nan. A wasu kalmomin, bayan da wani rigakafi martani ba ya lura cututtuka.

Daga baya, a cikin marigayi 20th karni, X-nasaba agammaglobulinemia likitoci nazari sake. A shekarar 1993, likitoci sun iya gano m gene da cewa yana sa illa rigakafi.

Sanadin X-nasaba agammaglobulinemia

Agammaglobulinemia (Bruton ta cuta) sau da yawa ne hereditary. A aibi aka dauke su a recessive hali, don haka yiwuwar ciwon yaro da Pathology ne 25%. Dako na mutant gene mata ne. Wannan shi ne saboda gaskiyar cewa aibi ne sarrafa a kan X chromosome. Duk da haka, cutar da ake daukar kwayar cutar kawai ne da ɗa namiji. Babban dalilin agammaglobulinemia ne m gina jiki, wani ɓangare na gene coding for tyrosine kinase. Bugu da kari, da Bruton ta cutar na iya zama idiopathic. Wannan yana nufin cewa a cikin hanyar da ya faru da ya rage m. Daga cikin hadarin abubuwan da zai shafi da yaro ta kayyade code, ware:

1. Abuse barasa da kwayoyi a lokacin daukar ciki.
2. Psycho-wani tunanin iri.
3. Exposure zuwa ionizing radiation.
4. Chemical irritants (tareda žata samar, unfavorable yanayi).

Mene ne pathogenesis da cutar?

Hanyar cutar dangantaka da m gina jiki. Yadda aka saba, da gene alhakin tsarinsa tyrosine kinase, aka hannu a cikin samuwar B-lymphocytes. Su rigakafi Kwayoyin da suke da alhakin humoral tsaron gida. Saboda da insolvency na tyrosine kinase B lymphocytes ba girma sosai. A sakamakon haka, suna iya samar da immunoglobulins - antibodies. A pathogenesis da cutar Bruton ne cikakken tarewa na humoral kariya. A sakamakon haka, a lamba tare da cututtuka jamiái a cikin jiki antibodies cikinta ba samar. A musamman alama ce ta wannan cuta ne cewa tsarin na rigakafi ne ke iya yin yaƙi da cutar, duk da babu B-lymphocytes. Yanayin da take hakkin humoral kariya ya dogara da tsanani daga cikin lahani.

Bruton ta cuta: cuta cututtuka

Pathology karo na farko da ke sa kanta ji, a jarirai. Mafi sau da yawa, cutar bayyana kanta a 3-4-th watan rayuwa. Wannan shi ne saboda cewa a wannan shekaru jikin yaro gushe kare mahaifiyarsa antibodies. A farko alamun cutar na iya zama mai raɗaɗi dauki wadannan lamba, fata rashes, cututtuka daga cikin manya ko ƙananan numfashi fili. Duk da haka, shayarwa kare jariri daga kumburi tafiyar matakai, tun nono madara yana a cikin abun da ke ciki na immunoglobulins.

Bruton cuta bayyana ta game da 4 years. A wannan lokaci yaro ya zo a cikin lamba tare da sauran yara a kindergarten. Daga cikin cututtuka raunuka mamaye meningo, streptococci da staphylococcal microflora. A sakamakon haka, yara za a iya fallasa su surkin jini kumburi. A mafi m cututtuka sun hada da ciwon huhu, sinusitis, otitis kafofin watsa labarai, sinusitis, meningitis, conjunctivitis. A untimely lura da duk wadannan matakai za su iya tafiya a sepsis. Shi ne kuma wani buɗi na cuta na iya zama Bruton dermatological Pathology. Saboda da rage rigakafi martani kwayoyin ninka hanzari a site da raunuka da kuma scratches.

Bugu da kari ga manifestations da cutar sun hada da bronchiectasis - pathological canje-canje a cikin huhu. Alamun sun hada da nakasa daga 'ya'yan numfashi, zafi a cikin kirji, wani lokacin hemoptysis. Shi ne kuma zai yiwu fitowan da kumburi raunuka a cikin narkewa kamar gabobin, urinary fili, mucous membranes. Lokaci da Lokaci lura kumburi da kuma taushi a gidajen abinci.

A bincike da sharudda da cutar

A farko bincike sharudda kamata hada da m abin da ya faru. Yara fama da Bruton ta Pathology, kawo fiye da 10 cututtuka a kowace shekara, kazalika da sau da yawa a lokacin watan. Cututtuka iya maimaita ko m (otitis kafofin watsa labarai, tonsillitis, ciwon huhu). A jarrabawa, akwai wani rashin pharyngeal tonsil hypertrophy. Haka ya shafi palpation na gefe Lymph nodes. Ya kamata ka ma kula da jariri ta dauki bayan alurar riga kafi. Muhimmanci canje-canje da aka lura a dakin gwaje-gwaje kima. A KLA ãyõyin kumburi (ƙãra yawan leukocytes kara ESR). Yawan rigakafi Kwayoyin an rage. Wannan aka nuna a cikin leukocyte dabara: karamin yawan lymphocytes da kuma neutrophils ya karu ciki. Muhimmanci bincike ne immunogram. Yana nuna raguwa ko babu antibodies. Wannan yanayin ba ka damar yin ganewar asali. Idan likita yana da shakku, yana yiwuwa a gudanar da kwayoyin gwaji.

Bambance-bambance Bruton cuta daga irin wannan pathologies

Wannan Pathology da aka bambanta daga sauran makarantun firamare da sakandare immunodeficiencies. Daga cikin su - da Swiss irin agammaglobulinemia, Di Dzhordzhi ta ciwo, HIV. A bambanci ga wadannan pathologies for Bruton ta cutar halin kawai take hakkin humoral rigakafi. Wannan yana bayyana da cewa jiki ne iya yaƙi kwayar jamiái. Wannan factor ne daban-daban daga Swiss irin agammaglobulinemia, wanda aka karya a lokacin da duka biyu humoral da kuma salon salula rigakafi martani. Don yin bambanci ganewar asali da ciwo Di Dzhordzhi, kana bukatar ka yi kirji X-ray (thymic aplasia) da kuma sanin ko alli abun ciki. Don ware HIV gudanar palpation na Lymph nodes, Elisha.

Hanyoyin da magani daga agammaglobulinemia

Abin baƙin ciki, gaba daya kayar Bruton ta cuta ne ba zai yiwu ba. The hanyoyin da magani hada agammaglobulinemia maye da symptomatic far. Babban manufar nasara aka dauke su zama al'ada matakan immunoglobulins a cikin jini. The adadin antibodies ya kamata a approximated zuwa 3 g / l. A saboda wannan dalili, gamma globulin cikin kudi na 400 MG / kg jiki nauyi. Antibody taro kamata a karu a lokacin m cututtuka, kamar yadda jiki ba zai iya jimre da su da kansa.

Bugu da kari yi symptomatic magani. A mafi akai-akai wajabta maganin rigakafi "Ceftriaxone", "penicillin", "Ciprofloxacin". Lokacin da fata bayyananen bukatar Topical jiyya. Har ila yau, shawarar wanka mucosal antiseptic mafita (makogwaro da kuma hanci ban ruwa).

A hangen nesa ga Bruton ta agammaglobulinemia

Duk wani lifelong sauyawa far, da hangen nesa ga wani m agammaglobulinemia. Cigaba da magani da kuma prophylaxis na dauke da kwayar cutar matakai rage wajen kisa da aka rage girmanta. Yawanci, marasa lafiya ne iya-bodied da kuma aiki. A daidai ba tsarin kula da magani zai iya ci gaba da rikitarwa har sepsis. A cikin hali na gudãna cututtuka matalauta hangen nesa.

Rigakafin X-nasaba agammaglobulinemia

A gaban cuta a cikin dangi ko zargin shi wajibi ne su gudanar da kwayoyin gwaji ko a lokacin farko da trimester ciki. Har ila yau, to m matakan kamata hada da daukan hotuna da iska, babu mai dagewa cututtuka da kuma cutarwa effects. A lokacin daukar ciki, uwar shi ne contraindicated danniya. Domin na biyu rigakafin ne bitamin far, gwamnati na gamma globulin, da lafiya salon. Yana da muhimmanci a kauce wa lamba tare da kamuwa da mutane.