Ciwon daji: Sanadin, magani

An fara binciken cututtuka na tsarin rigakafi a cikin kwanan nan, tun da yake wannan kimiyya tana daya daga cikin ƙaramin magani. Duk da haka, saboda girman ci gaba, a yau akwai abubuwa da yawa a cikin wannan masana'antu. Ana kira cututtuka na tsarin tsaro na jiki a cikin jihohin rashin daidaituwa, wanda aka raba zuwa raunuka na farko da sakandare. Misalin irin wannan cututtukan kwayar cutar ne Di-Georgi ciwo, wanda aka lura da rashin lafiya na T-lymphocytes. Bugu da kari ga rashin m sunadaran, cutar aka bayyana da mahara munanan na gabobin da kuma tsarin ne riga m, a cikin neonatal lokaci, kuma a wasu lokuta - a utero.

Hanyar ci gaban cututtuka

Di Dzhordzhi ciwo ne halin da cikakken ko m hypoplasia na da thymus (thymus). Wannan gadon yana samuwa a cikin kogin thoracic na yara kuma wajibi ne don samuwar amsawa ta hanyar salula. A mafarki, da thymus fara ji ƙyama a size da kuma canza zuwa cikin kitsen nama. Wannan tsari na al'ada ne kuma ba ya nufin alakar ilimin pathology. Tare da ciwo na Di Georgi yana da tambaya game da rashin ingancin wannan jikin ko kuma rashin ci gaba (ciwon hypoplasia). A sakamakon haka, Kwayoyin rigakafi sun zama marasa dacewa kuma basuyi aikin su. Bugu da ƙari, lokacin da cuta ne sau da yawa wani rashin na parathyroid gland, ke da alhakin alli da phosphorus metabolism. Hanyar ci gaban abubuwan da ke tattare da nau'o'i na tsarin daban-daban yana hade da haɗuwa da ci gaban intrauterine na tayin da ke faruwa a farkon matakan ciki.

Ciwon daji na cuta: Sanadin cututtuka

A wannan lokacin babu cikakkiyar bayani game da dalilin da yasa akwai alamar wutsiyar glandan thymus. A mafi yawan lokuta, tare da wannan ilimin halitta, akwai nakasar kwayoyin cuta a cikin 22-chromosome, wanda ya ƙunshi asarar daya daga cikin sassansa. An yi imani cewa irin wannan cin zarafin yana hade da haɗin kai, duk da haka, wannan ka'ida ba a tabbatar ba. Bugu da ƙari, ba a lura da nakasar a cikin 22 na chromosome a duk marasa lafiya, amma a cikin 80% na lokuta. Sanadin cututtuka na Di Georgi ma suna hade da haukacin tayi na tayi na tayi na tasowa daga mahaifa da ke cikin uwa. Kwayoyin cututtuka (rubella, measles, herpes), ciwon sukari, ciwon kwakwalwa, da dai sauransu, wanda aka sauya a lokacin daukar ciki, za a iya danganta su.Anada wasu cututtukan cututtukan Di Georgi ne (shan barasa, kwayoyi) da kuma yaduwa ga sunadarai yayin lokacin intrauterine Ƙaddamarwa.

Bayanin asibiti na kamus

Haka kuma cutar ta zama sananne a farkon kwanakin jariri, saboda baya ga rashi na tsarin rigakafi, yana nuna kanta a cikin rashin daidaituwa. Mafi mahimmanci shine suturar zuciya, tun da wasu daga cikinsu basu dace da rayuwa ba (tetralogy na Fallot). Abubuwa masu haɓaka ta haɓaka zasu iya rinjayar duk wani sifofi da tsarin, amma yawancin ciwo na Di Georgi yana nunawa ta hanyar wadannan alamu:

1. Rashin lafiyar fuska: raguwar kasusuwan kwanyar da kuma jaw, idanu masu yawa, ƙaddamar da kwayoyin halitta, "gothic" palate, "lakabi", da dai sauransu.
2. Sauran cututtukan respiratory da esophagus.
3. Lahani na ci gaba da tsarin mai juyayi - atrophy na cizon ƙwayar cuta ko cerebellum. A sakamakon wadannan cututtuka, gait, paresis da paralysis, sauye-sauye a hankali suna kiyaye. Babban bayyanuwar CNS anomalies aka dauke hankulansu retardation, wanda ya zama m, a cikin farkon shekaru na yaro ta rayuwa.
4. Malformations na sarkar kwayar halitta: stenosis da atresia.
5. Abubuwa na cututtuka na kasusuwa, karuwa a yawan yatsunsu ko yatsun kafa.
6. Abubuwa na kwayoyin halitta: strabismus, cututtuka na kwakwalwa.
7. Malformations na tsarin urinary.

Rashin lafiya zai iya faruwa ne kawai ko hada tare da juna. A wasu lokuta, haɗarin rashin ci gaba ba su da shi, kuma ciwon Di Georgi ya ƙunshi kawai gajiyar hanyoyin da ba su da nasaba. Rashin rashin lafiya na T-lymphocytes an nuna shi ta hanyar karuwanci ga cututtukan kwayoyin cuta da cututtukan kwayoyin cutar. A wannan yanayin, cututtuka da suka haifar da rashin daidaituwa, ba su da kyau ga maganin cutar antibacterial da anti-inflammatory. Aplasia na parathyroid gland yana nunawa ta hanyar yanayin damuwa.

Ka'idojin bincike na cutar

An gane ganewar asali lokacin da aka hada wadannan alamun cututtuka: ƙananan hali ga cututtukan cututtuka, ciwo masu ci gaba da haɗari. A cikin gwaje-gwaje na gwaje-gwaje, zaka iya ganin yawan ƙananan lymphocytes da ragewa a cikin matakin calcium. Domin tabbatar da ganewar asali, yana da muhimmanci don gudanar da nazarin halin da ake ciki. Ana ganin waɗannan canje-canje: babu amsa ga maganin alurar riga kafi da gwajin gwaji don hypersensitivity da canjin lymphoblastic. Yawan adadin B-lymphocytes ba a canza ba. Yayin da ake nazarin tsarin tsarin rigakafin kwayoyin halitta, karuwar su, rage yawan aiki. Hanyoyin dan tayi na kogin thoracic ya nuna babu ko rage yawan kamus da parathyroid. Binciken tsarin DNA ya ba ka damar koyi game da canje-canje a cikin 22-chromosome.

Syndrome Di Georgi: magani na pathology

Jiyya na pathology ya dogara da nauyin rikici. A cikin cikakken rashi na thymus nuna Gabar dasawa. Yin magani na wajibi ne don ƙananan lahani na gabobin da tsarin. A cikin yanayin rashin lafiya na rashin lafiya da kuma rashin mummunan cututtuka, an ba da umarnin tallafi. Magunguna masu mahimmanci sune marasa amfani. A cikin rayuwar, dole ne a maye gurbin rashi na jiki a jiki. A cikin cututtukan cututtuka, ana amfani da farfadowa na cututtuka: maganin rigakafi, maganin maganin rigakafi. Baya ga shan magunguna, wajibi ne don kauce wa cututtukan mahaukaci, yanayi masu damuwa da sauran abubuwan da ba su da kyau.

Sakamakon kamus na thymus

Ciwo na Di Georgi yana da haɗari saboda yana haddasa matsaloli da yawa. Magunguna da wannan cuta suna da saukin kamuwa da tsarin ciwon tumo, matakai mai tsanani. Abinda ya haifar da ciwon ciwo shi ne ci gaban ƙwayoyin cuta. Wannan shi ne saboda gaskiyar cewa marasa lafiya suna ganin kwayoyin jikinsu ne ga ma'aikatan waje kuma sun fara yakar su. A sakamakon haka, akwai cututtuka irin su lupus erythematosus, dermatomyositis, rheumatoid arthritis. A cikin mummunan cututtuka na ci gaba, ana iya lura da matsalolin da ake biyowa: jinkirin tunanin mutum, nakasa na gabar jiki, makanta. Lokacin da convulsive cuta na iya ci gaba numfashi gazawar saboda asphyxia.

Sanin ganewa ga ciwo na Di Georgi

Lokacin da ake haɗuwa da raunin ganyayyaki da kuma cututtukan da ake gani a ci gaba ta fuskar gashin kansa an gano shi: Di Georgi's syndrome. Hotuna na mutanen da ke shan wahala daga wannan cuta za a iya gani a littattafan likita na musamman. Tare da wannan haɗuwa da waɗannan bayyanar cututtuka, ƙin ganewar rayuwa shine yawanci maras kyau, tun da cikewar haɓakawa suna nuna alamun cututtuka. Yawancin lokaci, yara tare da Di Georgi ciwo ba su rayu da shekaru 10 ba saboda cututtuka mai tsanani ko cututtuka. A cikin yanayin sauƙi, babu rashin daidaituwa da kuma sauyawa maye gurbin, alamar na iya zama mai kyau.

Yin rigakafi na ciwo Di Georgi

Musamman maganin wannan cuta bata wanzu. Don hana ƙetare cin zarafin tayi na mahaifa, mahaifiyar da ta gaba zata ƙayyade abubuwan da ke damuwa, watsi da mummunan dabi'u, kuma hana rigakafi da cututtuka na kwayan cuta da kwayoyin cutar. Ana iya gano alamun ci gaba a wani shirin da aka tsara, wadda aka yi a kowace shekara ta ciki.