Primary immunodeficiencies. Primary immunodeficiencies: magani

Immunodeficiency - wani take hakkin da m ayyuka na mutum kwayoyin, saboda da weakening na rigakafi da martani ga pathogens na daban-daban yanayi. Kimiyya da aka bayyana a yawan takamaiman yanayi na wannan irin. Wannan kungiyar da cuta halin da ya karu mita da kuma weighting shakka daga cututtuka. Malfunctions da wata barã'aa cikin wannan harka hade da wani canji a cikin ingantaccen ko gwada yawa halaye na ta mutum aka gyara.

Properties rigakafi

Na rigakafi da tsarin taka muhimmiyar rawa a cikin al'ada aiki na jiki, kamar yadda aka yi nufi ga gane, kuma ya halaka antigens cewa ya iya ko shiga daga waje yanayi (dauke da kwayar cutar), ko zama a sakamakon ƙari girma nasu sel (na ciki). A m aiki ne da farko ya bayar da nakasar dalilai kamar phagocytosis kuma dace da tsarin. Domin Na'urar martani da kwayoyin amsa samu rigakafi da tsarin: da humoral da kuma salon salula. Communication faruwa ta hanyar dukan tsarin na musamman da abubuwa - cytokines.

Dangane da dalili, jihar rigakafi cuta kasu kashi Firamare immunodeficiencies.

Mene ne wani primary immunodeficiency

Primary immunodeficiencies (PID) - a take hakkin da rigakafi da martani saboda kwayoyin lahani. A mafi yawan lokuta, suna gaji da suke nakasar munanan. PID ne mafi saukar a wani wuri shekaru, amma wani lokacin su ba kamu har samartaka ko ƙarfinsa.

PID - wani rukuni na nakasar cututtuka, da dama na asibiti manifestations. International Nau'in Cututtuka 36 ya hada da binciken, kuma isasshe bayyana farko immunodeficiency jihohi, amma suna bisa ga likita adabi, akwai game da 80. Da cewa ba duk cuta da kwayoyin halittu da aka gano alhakin.

Kawai gene abun da ke ciki na X chromosome ne halin da akalla shida daban-daban na rigakafi ƙarara, sabili da haka da aukuwar wadannan cututtuka a cikin maza ne sosai fi da cewa da 'yan mata. Akwai wani zato cewa ci gaban da nakasar immunodeficiency iya samun aetiological rinjayar intrauterine kamuwa da cuta, amma wannan da'awar ba ta riga aka tabbatar ta hanyar kimiya.

asibiti hoto

The asibiti manifestations na farko immunodeficiencies ne kamar yadda bambancin matsayin su da kansu jihar, duk da haka, akwai daya kowa alama - hypertrophic infective (kwayan) ciwo.

Primary immunodeficiencies, kazalika da sakandare, bayyana hali ya m relapsing marasa lafiya (komawa) cututtuka na dauke da kwayar cutar etiology wanda za a iya lalacewa ta hanyar atypical pathogens.

Wannan cuta aka fi fallasa su bronchopulmonary tsarin da ENT-gabobin. Kuma sau da yawa sha mucous membranes da integuments, wanda zai iya bayyana sepsis da ƙurji. Kwayan pathogens sa mashako da kuma sinusitis. Mutane da rigakafi da kasawa, sukan lura da farkon baldness da eczema, da kuma wani lokacin rashin lafiyan halayen. M da autoimmune cuta, da kuma propensity zuwa malignancy. Rigakafi da karanci a yara ne kusan ko da yaushe yana sa wani bata lokaci da shafi tunanin mutum da kuma ta jiki ci gaba.

Inji na ci gaba farko immunodeficiencies

Nau'in cututtuka bisa ga tsarin ba da su ci gaba ne mafi m, a cikin hali nazarin immunodeficiency jihohi. Doctors raba duk rigakafi yanayin da cuta a cikin 4 babban kungiyoyin:

- Humoral ko B-cell, wanda sun hada da Bruton ta ciwo (agammaglobulinemia da za a guda biyu zuwa ga X chromosome) karanci IgA ko IgG, haddi IgM da total immunoglobulin rashi, sauki m immunodeficiency, juya hypogammaglobulinemia jariri da kuma da dama daga sauran cututtuka dangantawa da humoral rigakafi .

- T cell farko immunodeficiencies, sau da yawa ake kira a hade, tun da farko cuta ko da yaushe karya da kuma humoral rigakafi, msl hypoplasia (Di George ciwo) ko dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies sa da lahani a phagocytosis.

- Immunodeficiencies sa da cuta daga cikin dace da tsarin.

laulayi to kamuwa da cuta

Tun cikin hanyar immunodeficiency iya zama wani take hakkin da daban-daban raka'a
tsarin na rigakafi, da kuma daukan hotuna zuwa dauke da kwayar cutar jamiái ne ba iri daya ba ga kowane hali. Alal misali, sa'ad da humoral cututtuka da mãsu haƙuri ne yiwuwa ga cututtuka da cewa suna lalacewa ta hanyar streptococci, staphylococci, Haemophilus influenzae. Kamar wancan ne wadannan kwayoyin sau da yawa ya nuna juriya ga maganin rigakafi. Lokacin da hada immunodeficiency siffofin za a iya a haɗe zuwa kwayoyin cuta, kamar herpes ko fungi wanda yafi wakilta candidiasis. Phagocytic nau'i ne halin da yafi by guda staphylococci da gram-korau kwayoyin.

Yaduwar na farko immunodeficiencies

Immunodeficiencies, sun gaji, - quite a rare mutum cuta. A mita na wannan rigakafi cuta dole ne a kimanta game da kowane musamman cuta saboda su ruwan dare dabam.

A kan talakawan, kawai daya daga dubu hamsin jariri zai sha wahala nakasar hereditary immunodeficiency. Mafi na kowa cuta a cikin wannan kungiya shi ne rashin zabe IgA. Nakasar immunodeficiency na irin wannan da aka samu a tsakiyar daya daga cikin dubban jarirai. Kuma kashi 70% na duk lokuta na IgA rashi ne total gazawar da wannan bangaren. A daidai wannan lokaci, wasu fiye da rare mutum cuta na rigakafi da yanayi, sun gaji, za a iya rarraba a cikin rabo na 1: 1,000,000.

Idan mun yi la'akari da abin da ya faru na PID cututtuka dangane da inji, shi ne hada da ban sha'awa sosai halin da ake ciki. B-cell farko immunodeficiencies, ko kamar yadda suke more fiye da ake kira, a take hakkin da antibody, fiye da na kowa fiye da wasu, da kuma sa up 50-60% na duk lokuta. A lokaci guda, T-cell da phagocytic form kamu a 10-30% da marasa lafiya kowane. Ya dauki mafi m cututtuka na rigakafi da tsarin sa da lahani a najumla - 1-6%.

Ya kamata kuma a lura da cewa aukuwar data PID ne sosai daban-daban a cikin kasashe daban-daban, wanda za a iya hade da wani kayyade predisposition na kasa kungiyar zuwa takamaiman DNA maye gurbi.

ganewar asali immunodeficiency

Primary immunodeficiency a yara ne mafi sau da yawa m da untimely, saboda
da cewa irin wannan ganewar asali a gundumar pediatrician matakin ne wuya.

Wannan yawanci results a cikin wani bata lokaci ba a farkon magani da kuma talakawa hangen nesa na far. Idan likita dangane da asibiti hoto da cutar da kuma janar gwajin sakamakon shawarar immunodeficiency jihar, abu na farko da ya dole ne ya yi - don aika da yaro zuwa likita-immunologist.
A Turai akwai da Association of Immunologists, wanda ke hulda da nazari da kuma ci gaban hanyoyin da magani daga irin cututtuka, wanda ake kira da sunan da EDI (Turai Society for immunodeficiencies). Aka halitta su kuma kullum updated database PID cututtuka da kuma yarda da bincike algorithm domin a isasshe kulawa da lifiya.

Fara ganewar asali da tara likita tarihi. Musamman hankali ya kamata a biya ga asalinsu al'amari, kamar yadda mafi nakasar immunodeficiencies ne hereditary. Next, bayan wani jiki jarrabawa da kuma samun janar na asibiti bincike data sa da farko ganewar asali. A nan gaba, don tabbatar da ko shanyewa da jarrabawa likita, da mãsu haƙuri ya kamata sha na sosai jarrabawa da kwararru kamar halitta kuma immunologist. Kawai bayan duk na sama jan za mu iya magana game da samar da da karshe ganewar asali.

dakin gwaje-gwaje da bincike

Idan a lokacin da ganewar asali ne da ake zargi da primary immunodeficiency ciwo, wadannan dakin gwaje-gwaje gwaje-gwaje ne zama dole to:

- da kafa cikakken jini count (girmamawa a kan yawan lymphocytes).

- tabbatar da dalilin da magani immunoglobulins.

- gwada yawa lissafi na B- da kuma T-lymphocytes.

ƙarin karatu

Bugu da kari a dakin gwaje-gwaje da kuma bincike gwaje-gwaje, wanda aka riga aka ambata a sama, da mutum ƙarin gwaje-gwaje za a sanya a kowane hali. Akwai hadarin kungiyar, wanda ya bukatar da za a gwada wa HIV, ko kayyade munanan. Har ila yau, likita na bayar da yiwuwar cewa ba mutum immunodeficiency 3 ko 4 jinsunan, a karkashin abin da za su dage a kan wani cikakken nazari na haƙuri da phagocytosis gwajin yi nuna alama tetrazolinovym blue da tabbaci bangaren abun da ke ciki na dace da tsarin.

PID magani

Babu shakka cewa zama dole far zai dogara ne da farko daga rigakafi cuta, amma, da rashin alheri, da nakasar form ba za a iya shafe ta gaba daya, wanda ba za a iya ce game da samu immunodeficiency. Bisa halin yanzu likita aukuwa, masana kimiyya suna kokarin samun wata hanya ta cire dalili a kayyade matakin. Ya zuwa yanzu, da kokarin yi ba nasara, zamu iya cewa da rigakafi da rashi - wani m yanayin. La'akari da ka'idojin amfani da far.

maye far

Jiyya na rigakafi da kasawa yawanci rage wa maye far. Kamar yadda aka ambata a baya, jikin haƙuri ba m, don samar da wani gyara na rigakafi da tsarin, ko muhimmanci runtsẽwar ingancin da ake bukata. Far a wannan zai zama wani magani da liyafar antibodies ko immunoglobulins, halitta kayayyakin sun karye. Mafi sau da yawa, kwayoyi suna gudanar intravenously, amma wani lokacin shi ne zai yiwu, da kuma subcutaneous hanya, don tallafa a rayuwa na haƙuri, wanda a cikin wannan yanayin ba dole ba ne sake ziyarci likita makaman.

canzawa manufa sau da yawa damar marasa lafiya zuwa kai kusan al'ada rayuwa: to koyi, aikin da wasa. Hakika, a raunana na rigakafi da tsarin cuta, humoral da kuma salon salula dalilai da akai bukatar gabatarwar tsada kwayoyi ba zai ba da damar da mãsu haƙuri ga cikakken shakata, amma shi ne har yanzu mafi alhẽri daga da suke zaune a wani matsin lamba jam'iyya.

Symptomatic magani da kuma rigakafin

Ganin cewa a wani kananan ga m adam kwayan ko kwayar cutar ga mãsu haƙuri rukuni na farko immunodeficiency cuta na iya zama m, shi wajibi ne a gudanar da rigakafin competently. Ga zo a cikin play antibacterial, antifungal da antiviral kwayoyi. Key kudi su yi shi ne a kan m matakan, saboda raunana na rigakafi da tsarin ba zai iya iya samar da ingancin magani.

Bugu da kari, ya kamata a tuna da cewa wadannan marasa lafiya ne yiwuwa ga rashin lafiyan, autoimmune, kuma, ko da muni, to ƙari jihohi. Duk wannan ba tare da cikakken likita dubawa ba zai iya ba da damar mutum ya kai cikakken rai.

dasawa

Lokacin da masana yanke shawarar cewa haƙuri ba ya zama wani zabi da tiyata, za a iya yi bargo dasawa. Wannan hanya da ake dangantawa da mahara kasada ga rayuwa da kuma kiwon lafiya na haƙuri, kuma a yi, ko da a cikin taron da ta yi nasara sakamako ba zai iya ko da yaushe warware duk matsaloli na fama da wani rigakafi cuta. A dauke da fitar da irin wannan aiki, da dukan tsarin da aka maye gurbinsu da mai karɓa hematopoiesis kamar bayar da bayarwa.

Primary immunodeficiencies ne mafi wuya matsala na zamani magani, wanda, da rashin alheri, bai warware gaba daya. Poor hangen nesa ga cututtuka irin wannan har yanzu rinjaya, kuma shi ne ninki biyu m, ba da cewa su sha wahala mafi yawa yara. Duk da haka, da yawa siffofin rigakafi rashi jituwa tare da cikakken rai idan sun kasance farkon ganewar asali da kuma yin amfani da isasshen far.