Pulayimale immunodeficiencies. immunodeficiencies zikuluzikulu: chithandizo

Immunodeficiency - kuphwanya ntchito kuteteza thupi la munthu chifukwa cha kufooka Poyankha cha m'thupi ndi tizilombo toyambitsa matenda a chikhalidwe chosiyana. Science anafotokoza zinthu zosiyanasiyana zinthu zenizeni za mtundu uwu. Gulu la matenda yodziwika ndi kuchuluka pafupipafupi ndi weighting Inde matenda opatsirana. Malfunctions chitetezo chokwanira Pankhaniyi kugwirizana ndi kusintha makhalidwe Mkhalidwe kapena kachulukidwe zigawo zake payekha.

katundu chitetezo chokwanira

chitetezo cha m'thupi chimagwira ntchito yapadera mu uziyenda yachibadwa ya thupi, monga cholinga kuti azindikire ndi kuwononga antigens kuti mwina kapena kulowa ku malo Link (matenda) kapena chifukwa chotupa kukula maselo yekha (amkati). Ntchito zoteteza kwenikweni amaperekedwa ndi zinthu kobadwa nako monga phagocytosis ndi dongosolo womuyenerera. Pakuti ayankhe dziphunzitsiranso wa chamoyo Yankho anapeza chitetezo: ndi humoral ndi ma. Communication kumachitika mwa dongosolo lonse la zinthu zapadera - cytokines.

Malinga ndi vuto matenda chitetezo chokwanira boma anawagawa immunodeficiencies pulayimale ndi sekondale.

ndi immunodeficiency chachikulu chimene

immunodeficiencies chachikulu (PID) - kuphwanya ayankhe cha m'thupi chifukwa nthenda ya kumtundu. Nthawi zambiri, iwo anatengera ndi kobadwa nako kuvutika. PID ambiri anaulula ali aang'ono, koma nthawi zina si matenda mpaka aang'ono ngakhale mukadzakula.

PID - gulu la matenda obadwa nawo, zosiyanasiyana mawonetseredwe matenda. Mayiko gulu la Matenda 36 kumaphatikizapo ankafufuza ndi mokwanira anafotokoza immunodeficiency limati chachikulu, koma malinga ndi mabuku a zachipatala, pali 80. Popeza kuti si chibadwa matenda onse akhala kuzindikiridwa udindo.

Only jini zikuchokera kwa X khromozomu amakhala ndi kumalephera osachepera asanu osiyana m'thupi, choncho pafupipafupi zosayembekezereka wa matenda amenewa amuna ndi apamwamba kuposa kuti atsikana. Pali maganizo oti chitukuko cha immunodeficiency kobadwa nako mwina aetiological chikoka cha matenda intrauterine, koma mfundo iyi silinakwaniritsidwe chatsimikiziridwirapo mwasayansi.

chithunzi matenda

Mawonetseredwe matenda a immunodeficiencies chachikulu mosiyanasiyana monga iwo boma Komabe, pali chimodzi Mbali - hypertrophic infective (bakiteriya) syndrome.

immunodeficiencies pulayimale, komanso sekondale, tikhale ndi chizolowezi pafupipafupi odwala relapsing (zisadzachitikenso) matenda a etiology opatsirana kumene kumabwera ndi tizilombo toyambitsa matenda atypical.

matenda nthawi zambiri poyera dongosolo bronchopulmonary ndi ENT-ziwalo. Nthawi zambiri amavutika nembanemba mucous ndi integuments, amene akhoza kusonyeza sepsis ndi abscess. tizilombo toyambitsa matenda bakiteriya chifukwa matenda ndi sinusitis. Anthu amene ali ndi vuto m'thupi, nthawi zambiri anati dazi oyambirira ndi chikanga, ndi zochita zina matupi awo sagwirizana. Pafupipafupi ndi autoimmune matenda, ndi propensity kuti chotupa chija. Timakumana akusowa ana pafupifupi nthawi zonse amachititsa akuchedwa chitukuko maganizo ndi thupi.

Limagwirira chitukuko cha immunodeficiencies chachikulu

Gulu la matenda monga limagwirira wa chitukuko ndi nkhani kwambiri choncho kuphunzira limati immunodeficiency. Madokotala kugawanitsa chikhalidwe zonse m'thupi mwa matenda m'magulu 4 chachikulu:

- Humoral kapena B-selo, monga matenda Bruton a (agammaglobulinemia kuti zolumukizidwa ndi X khromozomu) akusowa IgA kapena IgG, owonjezera IgM ndi okwana akusowa immunoglobulin, yosavuta variable immunodeficiency, kutembenuzira hypogammaglobulinemia wakhanda ndipo chiwerengero cha matenda ena amagwirizana ndi chitetezo chokwanira humoral .

- T selo immunodeficiencies chachikulu, nthawi zambiri amatchedwa zimagwira kuyambira matenda oyamba nthawi wosweka ndi chitetezo chokwanira humoral, mwachitsanzo hypoplasia (Di George syndrome) kapena dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies chifukwa cha zopindika phagocytosis.

- Immunodeficiencies chifukwa cha matenda a dongosolo womuyenerera.

chiwopsezo matenda

Popeza chifukwa cha immunodeficiency angakhale kuphwanya mayunitsi zosiyanasiyana
ndi chitetezo, komanso kukhudzana ndi wothandizila matenda si chimodzimodzi kwa nkhani iliyonse. Mwachitsanzo, pamene matenda humoral wodwala sachedwa matenda amene amayamba ndi streptococci, staphylococci, Haemophilus influenzae. Choncho tizilombo ameneŵa kuonetsa kukana opha tizilombo. Zimagwira mitundu immunodeficiency akhoza Ufumuyo mabakiteriya mavairasi, monga nsungu kapena bowa amene makamaka ankaimira candidiasis. mawonekedwe Phagocytic amakhala makamaka ndi staphylococci chomwecho ndi mabakiteriya galamu-negative.

Kuchuluka kwa immunodeficiencies chachikulu

Immunodeficiencies tinalandira, - matenda ndithu kawirikawiri anthu. Pafupipafupi matendawa m'thupi ayenera ayesedwa ndi kulemekeza aliyense matenda makamaka chifukwa kuwanda zawo zimasiyanasiyana.

Pafupifupi munthu mmodzi yekha zikwi makumi asanu akhanda adzavutika kobadwa nako cholowa immunodeficiency. The matenda ambiri mu gulu ili ndi kupanda IgA kusankha. Kobadwa nako immunodeficiency wa mtundu uwu umapezeka pakati pa imodzi mwa zikwi akhanda. Ndipo 70% milandu onse akusowa IgA ali okwana kulephera kwa chigawo ichi. Pa nthawi yomweyo, ena osowa kwambiri matenda anthu a chikhalidwe m'thupi, wobadwa nawo, akhoza anagawira mu chiŵerengero cha 1: 1,000,000.

Ngati tikulingalira zochitika za matenda PID malingana limagwirira, izo wapangidwa zinthu zosangalatsa kwambiri. B-selo immunodeficiencies chachikulu, kapena ngati iwo ali zambiri amatchedwa, kuphwanya oteteza ndi, ambiri kuposa ena amapanga 50-60% milandu onse. Pa nthawi yomweyo, T-selo ndi mawonekedwe phagocytic anapezeka mu 10-30% odwala lililonse. Akuti matenda ambiri osowa wa chitetezo cha m'thupi chifukwa cha kupunduka mu womuyenerera - 1-6%.

Iwo ayenera kukumbukira kuti pafupipafupi yatsoka deta PID zosiyana kwambiri m'mayiko osiyanasiyana, amene akhoza kugwirizana ndi kutengera za kumtundu wa gulu dziko kuti masinthidwe yeniyeni DNA.

matenda a immunodeficiency

immunodeficiency makamaka ana nthawi zambiri anatsimikiza ndi zosapsa, chifukwa
chakuti akudwala matenda amenewa pa Boma dokotala zovuta.

Izi kawirikawiri zimabweretsa akuchedwa chiyambi cha mankhwala ndi madokotala ananena zawo osauka mankhwala. Ngati dokotala zochokera chithunzi matenda a matenda ndi zotsatira ambiri mayeso adafotokoza immunodeficiency boma, chinthu choyamba chimene iye ayenera kuchita - kutumiza mwanayo kwa dokotala-immunologist.
Ku Ulaya pali Association of Immunologists, amene amachita naye phunziro ndi chitukuko cha njira ya chithandizo cha matenda, lomwe lili ndi dzina la EDI (European Society kwa immunodeficiencies). Iwo analengedwa ndi zonse kusinthidwa matenda Nawonso achichepere PID ndi kuvomereza matenda aligorivimu kwa matenda mokwanira mofulumira.

Yambani matenda posonkhana mbiri zachipatala. chidwi ziperekedwe mbali mibadwo, monga ambiri immunodeficiencies kobadwa nako ndi cholowa. Lotsatira, pambuyo kudzifufuza thupi ndi kupeza ambiri deta kafukufuku matenda kuika matenda waukuluwo. M'tsogolo, kutsimikiza kapena kutsutsa kungoganizira dokotala, wodwala ayenera kukayezetsa bwinobwino ndi akatswiri monga zachibadwa ndi immunologist. Pambuyo onse mwai pamwamba tikhoza kulankhula za ulimi wa matenda komaliza.

kafukufuku zasayansi

Ngati pa matenda ndi achifwamba chachikulu immunodeficiency syndrome, zotsatirazi mayesero zasayansi ndi zofunika:

- kukhazikitsidwa kwa wathunthu magazi okwanira (kutsindika pa chiwerengero cha lymphocytes);

- motsimikiza immunoglobulins seramu;

- kachulukidwe mawerengedwe a B- ndi T-lymphocytes.

maphunziro

Kuwonjezera zasayansi ndi matenda mayesero, kale tatchulazi, munthu mayesero zina nkhani lililonse. Pali chiopsezo gulu, amene ziyenera kuyesedwa kwa kachirombo ka HIV, kapena kuvutika chibadwa. Komanso dotolo amapereka mwayi kuti alipo immunodeficiency anthu 3 kapena 4 mitundu umene afunitsa kuphunzira zambiri za mtima ndi phagocytosis mayeso ntchito chizindikiro tetrazolinovym buluu ndi kutsimikizira chigawo zikuchokera dongosolo womuyenerera.

PID chithandizo

N'zoonekeratu kuti mankhwala zofunika kudzadalira makamaka ku matenda m'thupi, koma, mwatsoka, mawonekedwe kobadwa nako sangathe kuchotsedwa kwathunthu, amene sakhoza anati za immunodeficiency zinazake. Kuchokera pa zimene zikuchitika panopa zachipatala, asayansi akuyesera kuti apeze njira kuchotsa chifukwa pa mlingo chibadwa. Pakadali pano, khama lawo akhala bwino, tikhoza kunena kuti chitetezo akusowa - mkhalidwe osachiritsika. Taonani mfundo za mankhwala ntchito.

m'malo mankhwala

Chithandizo cha akusowa m'thupi zambiri yafupika mankhwala m'malo. Monga tanena kale, thupi la wodwalayo sangathe kubala zigawo zina za chitetezo cha m'thupi, kapena kwambiri m'munsi khalidwe lawo chofunika. Therapy pa zimenezi kungakhale chitetezo mankhwala phwando kapena immunoglobulins, mankhwala achilengedwe wosweka. Nthawi zambiri mankhwala kutumikiridwa m'nsinga, koma nthawi zina n'zotheka, ndi subcutaneous njira, kuti magawowa moyo wa wodwala, amene mu nkhani iyi alibe kamodzinso kukaona malo zachipatala.

M'MALO mfundo zambiri zimathandiza odwala kutsogolera moyo pafupifupi yachibadwa: kuphunzira, ntchito ndi kusewera. Inde, wofooka chitetezo matenda, zinthu humoral ndi ma ndi kufunika mosalekeza kumayambiriro mankhwala okwera mtengo sadzalola kuti wodwala mokwanira kumasuka, koma panopa kuposa ankakhala m'chipinda kuthamanga.

chithandizo Symptomatic ndi kapewedwe

Popeza kuti aliyense kakang'ono ka matenda wathanzi anthu toyambitsa tizilombo gulu wodwala matenda chachikulu immunodeficiency akhoza chakupha, m'pofunika kuchita kupewa mwaluso. Apa amagwira antibacterial, antifungal ndi mankhwala mavairasi oyambitsa. mlingo Ofunika kuchita ndi pa zodzitetezera, chifukwa wofooka chitetezo sangakwanitse kupeza mankhwala khalidwe.

Komanso, tiyenera kukumbukira kuti odwalawo sachedwa awo sagwirizana, autoimmune, ndipo oipitsitsa, m'maboma chotupa. Zonsezi popanda kuyang'aniridwa zonse zachipatala tisalole munthu kukhala ndi moyo wonse.

kupatsidwa zina

Pamene akatswiri kusankha kuti wodwalayo sakhala sangachitire mwina koma opaleshoni, angathe kuchitidwa mafupa kupatsidwa zina. njirayi amagwirizana ndi mavuto angapo kuti moyo ndi thanzi la wodwala ndi kuchita, ngakhale mu mwambo zotsatira bwino sangathe nthawi zonse kuthetsa mavuto onse akudwala matenda ndi m'thupi. Pogwira opareshoni zimenezi, dongosolo lonse m'malo ndi wolandira hematopoiesis monga operekedwa ndi mwiniwake.

immunodeficiencies chachikulu ndi vuto lovuta kwambiri mankhwala amakono, amene, mwatsoka, kodi osatsimikiza kwathunthu. Osauka madokotala ananena zawo matenda a mtundu ichi chichitika, ndipo doubly zolakwika Popeza kuti akudwala makamaka ana. Komabe, mitundu yambiri ya kuperewera kwa m'thupi n'zogwirizana ndi moyo wathunthu ngati ali matenda oyambirira ndi kugwiritsa ntchito mankhwala okwanira.