Lub cev tiv thaiv kab mob ua haujlwm tiv thaiv tib neeg. Nws tiv thaiv nws ob sab hauv thiab tawm kom nws lub cev thiab cov ntaub so ntswg ua haujlwm zoo.
Tab sis, zoo li txhua lub cev lub cev, lub cev tiv thaiv kab mob yog raug rau cov txheej txheem pathological. Ib lossis ntau qhov txuas hauv cov kab mob tiv thaiv kab mob yuav ploj los yog tsis muaj peev xwm. Qhov tshwm sim yog immunodeficiency xeev, thawj lossis theem nrab immunodeficiencies.
YPrimary immunodeficiencies
Cov kab mob no, uas yog raws li kev hloov pauv hauv cov qauv thiab kev ua haujlwm ntawm lub cev tiv thaiv kab mob, muaj ntau heev. Lawv tshwm sim los ntawm kev ua txhaum loj ntawm kev tiv thaiv kab mob. Ntau cov tsos mob tau txuas nrog X chromosome, yog li lawv tshwm sim ntau zaus hauv cov tub. Lwm qhov muaj ib qho autosomal recessive qauv ntawm qub txeeg qub teg thiab tshwm sim sib npaug ntawm cov ntxhais.
Feem ntau, pawg no muaj ntau dua 100 yam kab mob sib txawv, qhov tshwm sim ntawm ib tus neeg mobrau 1,000,000 tus neeg mus rau ib tug ntawm 100,000. Lawv yuav luag ib txwm tshwm sim nyob rau hauv thaum yau, txij li thaum ib tug tseem ceeb feem ntawm cov neeg mob no muaj ib tug loj tus naj npawb ntawm immunodeficiency thiab tsis nyob ntev tshaj 20 xyoo. Nyob rau hauv cov ntaub ntawv me me, kev tiv thaiv kab mob tsis zoo tuaj yeem raug them nyiaj ib feem ntawm lub hnub nyoog thiab tsis ua rau muaj kev pheej hmoo rau lub neej ntawm tus neeg nqa khoom, thaum mob hnyav, ntawm qhov tsis sib xws, ua rau tuag txawm tias thaum menyuam mos.
Kev faib tawm
Primary immunodeficiencies yog subdivided raws li theem ntawm kev puas tsuaj rau hauv:
Cellular immunodeficiencies:
- qhov tsis txaus ntawm CD4 hlwb (pom thaum menyuam yaus hauv daim ntawv ntawm cryptococcal meningitis thiab chronic oral candidiasis);
- qhov tsis txaus ntawm CD7 hlwb (ib kis tau piav qhia):
- interleukin deficiency ntawm ob lossis ntau dua interleukins;
- tsis txaus ntawm ib lossis ntau dua cytokines;
- DiGeorge's Syndrome (thaum ntxov cev xeeb tub, thymus caj pas ntawm lub embryo tsis tau txais T-cell precursors, cov qog parathyroid tseem tsis tau tsim los ntawm tetany, convulsions, nrog rau lub plawv tsis xws luag, cov qauv tsim. mob ntawm lub ntsej muag nyob rau hauv daim ntawv ntawm cleft di ncauj thiab palate, anomalies nyob rau hauv kev loj hlob ntawm cov pob txha ntawm lub pob txha, paj hlwb, ob lub raum).
2. Humoral immunodeficiencies
- Hyper-IgM-syndrome: T-hlwb pib synthesize immunoglobulin ntawm ib hom M. Qhov no, muaj qhov tsis txaus ntawm lwm hom Ig. Kev tshwm sim thaum muaj hnub nyoog ntxov los ntawm neutropenia, pneumocystis pneumonia, thaum thawj xyoolub neej, nquag purulent sinus-pulmonary kab mob yog pom. Yog hais tias tus me nyuam muaj sia nyob mus txog rau hnub nyoog laus laus, mob siab rau daim siab lossis B-cell lymphomas feem ntau tshwm sim.
- IgA deficiency. Txij li qhov no immunoglobulin muab kev tiv thaiv hauv zos rau ntawm daim tawv nqaij thiab cov mucous daim nyias nyias, bronchitis, conjunctivitis, raws plab, sinusitis, mob ntsws, thiab furunculosis ntawm daim tawv nqaij lesions ua manifestation ntawm deficiency. Lactose intolerance, ntau yam ua xua tshwm sim, autoimmune pathologies kuj ua tau.
- IgG deficiency. Kev tshwm sim nyob ntawm seb qhov twg G-subclass raug kev txom nyem. Yeej, cov no yog cov kab mob otitis mus tas li, sinusitis, bronchitis, conjunctivitis.
- Bruton's disease (X-linked agammaglobulinemia) - tshwm sim los ntawm cov kab mob purulent ntawm plab hnyuv, ENT kab mob, musculoskeletal system, abscesses thiab furunculosis, nquag mob - meningitis thiab sepsis.
- Tsis muaj cov tshuaj tiv thaiv kab mob nrog cov qib immunoglobulins ib txwm muaj. Nws yog manifested los ntawm recurrent sino-pulmonary kab mob, nrog rau cov kab mob atopic (asthma, rhinitis, dermatitis). Tsis tshua pom ua ntej hnub nyoog ob xyoos.
3. Combined immunodeficiencies
- Louis Bar Syndrome (ataxia telangiectasia), ntau lub luag haujlwm raug cuam tshuam: underdeveloped thymus gland, T-cell deficiency, IgG, IgE, IgA, ataxia, vascular txhab, pigmentation disorders, sinusitis, kab mob ua pa.
- Kev tiv thaiv kab mob sib xyaw ua ke (kev tshwm sim hnyav, ntau qhov txhab, tsis zoo).
- Kev tsis txaus ntawm tus kheej enzymes (purine nucleotide phosphorylase, adenosine deaminase). Los ntawm-Nyob rau hauv thawj rooj plaub, T-cells raug kev txom nyem, nyob rau hauv lub thib ob - T-cells thiab B-lymphocytes. Clinically, nws yog kev loj hlob qeeb, neurological ntshawv siab - spasms, hlwb retardation, thyroiditis, systemic lupus erythematosus.
- CD3 deficiency thiab 8 - txawv ntawm cov qauv kev tshwm sim ntawm kev tiv thaiv kab mob.
- Bald lymphocyte Syndrome - tus naj npawb ntawm T-tus pab cuam raug kev txom nyem, tshwm sim nws tus kheej li kev tiv thaiv kab mob nrog rau kev puas hlwb thiab raws plab tas li.
- Wiskott-Aldrich syndrome - thrombocytopenia nrog hemorrhagic syndrome, neoplasms, eczema thiab ua ke tiv thaiv kab mob.
4. Deficiencies nyob rau hauv tej yam tiv thaiv kab mob
- Insufficiency ntawm lub complement system. Nyob ntawm cov khoom uas cuam tshuam, daim duab kho mob txawv. Qee tus yog vasculitis, lymphomas, sepsis, sinusitis, otitis, meningitis, thaum lwm tus yog mob ntsws, tawv nqaij, kab mob autoimmune.
- Kev tsis zoo hauv phagocytosis - neutropenia (ntau yam txawv), mob ntsws tsis tu ncua los ntawm cov kab mob hauv lub cev lossis kab mob fungal.
Clinic
Kho mob, thawj thiab theem nrab immunodeficiency xeev tau tshwm sim los ntawm kev ua txhaum ntawm kev tiv thaiv kab mob thiab kab mob sib kis. Txo cov tshuaj tiv thaiv kab mob, tsis tsuas yog cov kab mob, tab sis kuj suav nrog hauv cov microflora ib txwm muaj (piv txwv li, Candida, Pneumocystis, cytomegalovirus, staphylococcus, enteroviruses, protozoa).
Qhov tshwm sim ntawm kev tiv thaiv kab mob tiv thaiv kab mob yog txiav txim siab los ntawm qhov chaw ntawm qhov txhab hauvKev tiv thaiv kab mob thiab/lossis kev sib xyaw ua ke ntawm cov teeb meem cuam tshuam.
- Muaj cov kab mob ntev ntawm cov kab mob ua pa sab saud, pob ntseg, paranasal sinuses, plab hnyuv, daim tawv nqaij thiab cov mucous membranes. Cov kab mob kis tau yooj yim rau kev nthuav dav thiab septicemia, tsis tuaj yeem ua raws li kev kho tus qauv.
- kab mob autoimmune - scleroderma, thyroiditis, kab mob siab, mob caj dab thiab lwm yam.
- Anemia, txo cov leukocytes thiab lymphocytes, thrombocytopenia.
- Kev loj hlob qeeb thiab kev loj hlob ntawm tus menyuam.
- Feem ntau muaj qhov ua rau muaj kev tsis haum tshuaj nyob rau hauv daim ntawv ntawm kev tsis haum tshuaj tam sim - Quincke's edema, eczema, ua xua rau tshuaj thiab khoom siv.
- digestive disorders, malabsorption, raws plab syndrome.
- Cov tshuaj tiv thaiv tsis txaus ntawm lub cev rau kev qhia txog sera thiab tshuaj tiv thaiv, nrog rau kev qhia txog tshuaj tiv thaiv nyob, sepsis yuav tshwm sim.
- Predisposition rau mob qog noj ntshav, tshwj xeeb tshaj yog cov qe ntshav.
Diagnosis
Ob lub xeev kev tiv thaiv kab mob hauv nruab nrab thiab theem nrab muaj cov qauv zoo sib xws ntawm cov kab mob sib kis. Kev kuaj mob thiab tshuaj tiv thaiv kab mob yuav pab tsim kom muaj qhov tseeb dua. Yog tias qhov tsis xws luag nyob hauv ib cheeb tsam, piv txwv li, tsis muaj T lossis B lymphocytes, lossis qhov txo qis hauv cov ntsiab lus ntxiv, cytokines, lossis qee yam immunoglobulins tuaj yeem kuaj pom.
Kev kho mob
Vim qhov ua rau thawj qhov kev tiv thaiv kab mob yog qhov tsis xws luag hauv genome, kev kho etiotropic yog kev kho noob (yog tias cov noob muaj lub luag haujlwm rau kev tiv thaiv kab mob tshwj xeeb). Cov noob tuaj yeem txheeb xyuas taulos ntawm polymerase saw cov tshuaj tiv thaiv. Lwm txoj hauv kev yog kev hloov kho (kev hloov pob txha pob txha, kev hloov pauv ntawm neutrophils thiab lymphocytes, kev tswj hwm cov enzymes thiab cytokines. Thiab kev kho mob - kev kho kab mob sib kis, immunomodulators, vitamins.
Secondary immunodeficiencies
Tau txais cov tshuaj tiv thaiv kab mob thib ob tau tsim los ntawm kev ua ntawm lwm yam lossis sab hauv thiab tsis cuam tshuam nrog cov cuab yeej caj ces. Qhov tseeb, cov no yog cov xwm txheej cuam tshuam nrog cov kab mob paub lossis kev ua ntawm kev puas tsuaj.
Cov kab mob tiv thaiv kab mob thib ob: kev faib tawm
Raws li kev txhim kho muaj:
- mob (vim raug mob, phais, mob kis mob);
- mob ntev (nrog rau cov neoplasms malignant, kab mob ntev, helminthiass, txheej txheem autoimmune).
Severity:
- them nyiaj (lub teeb, nrog tsis tiav kev tiv thaiv kab mob txuas);
- subcompensated (mob mob hnyav, qee qhov txuas ntawm kev tiv thaiv kab mob cuam tshuam tag nrho);
- decompensated (feem ntau systemic, mob hnyav).
Raws li theem ntawm cov txheej txheem pathological: thawj thiab theem nrab immunodeficiency xeev. Lawv pathophysiology zoo sib xws:
- ua txhaum ntawm T-cell tiv thaiv kab mob;
- ua txhaum ntawm B-cell tiv thaiv kab mob;
- pathology ntawm phagocytosis system;
- pathology ntawm kev sib ntxiv.
Secondaryimmunodeficiency xeev, ICD 10:
D50-D89. Cov kab mob ntawm cov ntshav, cov kab mob hematopoietic thiab qee yam kab mob cuam tshuam nrog lub cev tiv thaiv kab mob.
D80-D89. Xaiv cov kab mob cuam tshuam nrog lub cev tiv thaiv kab mob.
D84. Lwm yam kev tiv thaiv kab mob:
- ntxiv qhov tsis xws luag;
- immunodeficiencies;
- theem nrab immunodeficiencies.
D84.9 Immunodeficiency, unspecified.
Yog vim li cas
Qhov ua rau ntawm cov tshuaj tiv thaiv kab mob theem nrab tuaj yeem ua rau exogenous thiab endogenous.
Cov ua rau sab nrauv - tag nrho cov kev puas tsuaj ib puag ncig - tsis zoo ecological teeb meem, mob lom ntawm lub cev, teeb meem hluav taws xob (ionizing, microwave, thiab lwm yam), teeb meem ntawm lub suab nrov, plua plav, noj qee yam tshuaj tiv thaiv kab mob thiab tshuaj hormonal.
Internal ua rau - theem nrab immunodeficiency thiab immunosuppressive xeev nyob rau hauv cov ntaub ntawv no muaj ntau ntau thiab ntau yam:
- cov menyuam hnub nyoog, txog li 1 xyoos, tshwj xeeb tshaj yog thaum yug los muaj lub cev hnyav, thaum tsis muaj zaub mov noj (lossis pub mis ntxiv) ntxiv rau lub cev tiv thaiv kab mob;
- laus;
- cev xeeb tub thiab lactation - ua rau lub cev tiv thaiv kab mob, feem ntau ua ke nrog cov tsis muaj hlau tsis muaj ntshav liab;
- mob tsis txaus ntawm kev noj zaub mov, protein, kab kawm, vitamins lossis dej;
- kev raug mob, kev ua haujlwm, kev rov qab los ntev tom qab lawv;
- kab mob ntev (kab mob, kab mob, fungal) yuav luag txhua yamcuam tshuam rau kev tiv thaiv kab mob (mob siab ntev, glomerulonephritis, tuberculosis, rubella, thiab lwm yam. Tshwj xeeb tshaj yog, tau kawg, HIV);
- helminthiases - ua rau thiab ua kom muaj zog tiv thaiv kab mob theem nrab (ascariasis, trichinosis, toxoplasmosis);
- plasma poob - ntshav poob, kub hnyiab, raum puas;
- malignant oncological formations;
- mob ntshav qab zib mellitus, hyper- thiab hypothyroidism;
- autoimmune pathologies (rheumatoid mob caj dab, scleroderma, kab mob lupus erythematosus, thiab lwm yam), uas tus kheej lub cev tiv thaiv kab mob tsom nws lub cev thiab lub cev;
- noj qee yam tshuaj (cyclosporine, carbamazepine, valproate, azathioprine, corticosteroids, cytostatics, tshuaj tua kab mob);
- ntshav poob ntev (piv txwv li, nrog rau peptic ulcer ntawm txoj hnyuv);
- mob raws plab;
- kev nyuaj siab.
Raws li peb tuaj yeem pom, cov kab mob tiv thaiv kab mob thib ob muaj keeb kwm sib txawv kiag li. Lawv tshwm sim los ntawm ob qho tib si exogenous thiab endogenous yam. Lawv yog cov heev dav thiab nrog rau ob qho tib si physiological thiab ntau yam txheej txheem pathological. Yog li, raws li kev kis tus kab mob, kev ntxhov siab, qhov tsis zoo ntawm ib puag ncig, thiab tshwj xeeb tshaj yog lawv cov kev sib xyaw ua ke, cov kev tiv thaiv kab mob thib ob tshwm sim.
Pathophysiology: lub hauv paus ntawm kev tshwm sim ntawm kev tiv thaiv kab mob theem nrab yog kev tuag ntawm cov hlwb ntawm lub cev tiv thaiv kab mob, uas tshwm sim ob txoj hauv kev. Thawj - raws li hom necrosis, thaum lub hlwb tuag vim kev puas tsuaj rau daim nyias nyias, thiab qhov thib ob - raws li hom apoptosis, tom qab ntawd tuag.tshwm sim los ntawm DNA degradation nyob rau hauv qhov kev txiav txim ntawm nws tus kheej enzymes. Tsis tas li ntawd, feem ntau cov kev tiv thaiv kab mob hauv nruab nrab tshwm sim vim qhov tsis txaus ntseeg hauv cov hlwb ntawm lub cev tiv thaiv kab mob, xws li cov pab cuam thiab cov hlwb suppressor.
Diagnosis
- Anamnesis, kev tsis txaus siab, kev kawm txog kev xeeb tub.
- Kev txiav txim siab ntawm T-lymphocytes hauv cov ntshav, kev ua haujlwm thiab tus lej ntawm phagocytes, spectrum ntawm immunoglobulins.
- Kuaj HIV, kab mob siab, helminths, thiab lwm yam.
- YProteinogram.
- Kev kuaj kab mob ntev.
Txhua qhov kev kawm raug muab los ntawm tus kws tshaj lij.
Kev kho mob
Kev kho mob tactics nyob ncaj qha rau ntawm qhov ua rau ua rau muaj kev tiv thaiv kab mob theem nrab. Cov piv txwv kho mob:
- Nyob rau hauv qhov kev ua ntawm qhov tsis zoo (piv txwv li, ionizing hluav taws xob), tsuas yog lawv tshem tawm thiab tiv thaiv kab mob yuav pab tau.
- Yog tsis muaj zaub mov noj, protein lossis vitamins - ntxiv rau lawv noj.
- Thaum cev xeeb tub thiab lactation - noj cov vitamins ntxiv thiab kab kawm, kho ntshav qab zib (yog tias muaj).
- Rau cov kab mob ntev thiab helminthiasis, ua ntej ntawm tag nrho, kev huv huv ntawm cov kab mob foci thiab tom qab ntawd immunotherapy.
- Thaum muaj kab mob autoimmune, lawv qhov kev tso tawm ruaj khov yog qhov tsim nyog, yog li, kev kho tshuaj hormone yog ua tiav.
- Raws li kev kho mob - hloov kho. Piv txwv li, interferons, interleukins, cytokines, plasma.
In xaus
Pib thiabtheem ob immunodeficiency xeev muaj keeb kwm sib txawv kiag li, thiab yog li ntawd tshwm sim ntawm cov hnub nyoog sib txawv.
Tib lub sijhawm, lawv cov txheej txheem pathophysiological zoo sib xws thiab ua raws li ob peb txoj hauv kev. Thiab yog tias thawj cov tshuaj tiv thaiv kab mob nyuaj kho tau vim muaj qhov tsis xws luag hauv genome, tom qab ntawd cov neeg thib ob tuaj yeem kho tau qhov tseeb. Txhawm rau ua qhov no, nws tsuas yog tsim nyog los tsim qhov laj thawj vim li cas qhov txuas ntawm kev tiv thaiv kab mob poob tawm. Kev hloov pauv tshwj xeeb, hauv qhov kev hwm no, yog lub xeev kev tiv thaiv kab mob hauv nruab nrab hauv tus menyuam - nrog kev kho raws sijhawm, qhov kev mob tshwm sim feem ntau yog qhov zoo heev.
