Cantrell kaThixo pentad: oonobangela, unyango, abantu abane

Kukho ezininzi malformations kwasekwa Esibelekweni. Ezichaphazela ngayo nayiphi na inkqubo omzimba. Kuyingozi kakhulu iziphako ngentliziyo, lung, inkqubo yokucola ukutya, izintso, nobuchopho. Ezinye zazo awahambisani nobomi. Okunye iSADC unyango ngotyando. Enye malformations ezininzi ezintsonkothileyo ingqalelo pentalogy cantrell. Esi sifo waphawulwa ngezinto eziliqela kwephulwa yimibutho eyahlukeneyo. Kwezinye iimeko, unako ukufumana i pentad ezingaphelelanga cantrell. Nangona ubungqongqo unesiphako, maxa wambi oogqirha bakwazi ukumelana nesi sifo nokubuyisela imisebenzi ebalulekileyo umzimba.

pentad cantrell kaThixo - yintoni na le zokugula?

Le ngezifo buhlobene yesifo senhliziyo, kunye namanye amaziko. Njengoko igama pentad cantrell iquka iziphene-5. Phakathi kwawo ke kukwaphula ingozi inkqubo sentliziyo, obuquka utshintsho hemodynamics. Kwakhona kule isiphene ibandakanya siphene ezoyikekayo izithambiso. Ukuyibona kunokuba ngokukhawuleza ekuzalweni usana, njengoko intliziyo usana "isihange phandle". Cantrell engqondweni ayisoloko kuqulatha iziphene-5. Amaninzi ukuba ukugula 1 okanye 2 abangekhoyo (tetrad triad). Ukongezelela isifo sentliziyo, isifo ibandakanya enye uphawu enzima - omphalocele. Kwakhona kukho utshintsho esiswini sobuKristu nodonga esifubeni, uvalo siphene.

pentad cantrell esithi: ebangela izifo

Kubalulekile ukwazi ukuba esi sifo senziwa ezintathu yesibini yokukhulelwa. Ngeli xesha, ngokubekwa onke amalungu abalulekileyo kunye neenkqubo. Njengokuba kubhaliwe iyaziwa, iiveki 14-18 zokukhulelwa kwakhiwa mesoderm. Phantsi ngempembelelo yokusingqongileyo kunye nomama ezahlukeneyo zizifo ezithile na ukunyhashwa embryogenesis zingenzeka ngeli xesha. Enye yezi makhwiniba kunokuba pentalogy cantrell. Oyena nobangela ngqo sifo iye ayasekwa. Noko ke, kule isiphene inxulunyaniswa abanala makhwiniba chromosomal. Phakathi kwazo, 18 kunye trisomy 13, Turner syndrome, X-ezinxulumene nesifo. Yinto eyaziwayo ukuba lo isiphako kakhulu inqabile. Le rhoqo ukwenzeka kwayo iintsana 3-5 lezigidi zeetoni. Kwihlabathi amatyala ngaphantsi kwe 100 ezichazwe. Esi sifo ixhaphake kakhulu amakhwenkwe.

Ngaphandle kokuba ukufumanisa nobangela wesifo ungekabikho, kufuneka ukwazi malunga ubungozi ezikhokelela zezigulo ezahlukeneyo Palsy. Phakathi kwabo:

1. Ifuthe izinto ezinobungozi kunye ityhefu (iityuwa zesinyithi enzima, iziyobisi, kunye namachiza).
2. exposure imisebe.
3. Iimpembelelo Physical (ukwenzakala).
4. iimeko ezibangela.
5. Ukutshaya kunye neziselo ezinxilisayo.

Indlela ukuphazamiseka hemodynamic

Ngenxa yokuba le pentad cantrell ungajongana iziphene ngentliziyo ezahlukeneyo, ukuphazamiseka hemodynamic ngaloo kwahluka enye kwenye. Kaninzi oku sifo lukhatshwa siphene ventricular septal. Uphuhliso ukuphazamiseka hemodynamic kuxhomekeke kamongameli. Abaneziphene ezibalulekileyo kwenzeka shunt isangqa esikhulu encinane, nto leyo ekhokelela wegazi miphungeni. Le ventricle eliseleyo ngumthwalo yaye hypertrophy zembuyekezo. Ngenxa yoko, kukho ukwanda zombini RV kunye LV.

Azixhaphakanga septal siphene atrial. ukuphazamiseka Hemodynamic xa oko kuxhomekeke kubungakanani siphene. Ekubeni unxibelelwano enkulu phakathi ukumpompoza nomthambo igazi ekunene ezahlulwe sisithuba yandiswa ngokubonakalayo, kubangele hypertrophy kunye dilatation le PP. I overload atrial ngasekhohlo kwenzeka.

Eyona engundoqo zokubonisa pentad cantrell kaThixo

Njengoko yaziwa, i-classical cantrell Syndrome iquka iziphene-5. Phakathi kwazo kukho ezi kwephulwa zilandelayo:

• umbungu Omphalocele. Kwenye indlela ibizwa eliphezulu-enkabeni esiswini udonga siphene. Le ukuhlukunyezwa luphawulwa into yokuba amalungu zangaphakathi ngokuqhelekileyo zifumaneka esiswini, ziqokelelwe engxoweni Ithemba yosana uye phandle.
• Chest udonga siphene. Kukho ezininzi eziqhele esibonakala ngokungabikho okanye liqhekeke xiphoid. Ngenxa yoko, intliziyo nayo ihamba phambili.
• Nokungabikho pericardium, eliza kuba kufuphi uvalo.
• uphuhliso okungahambi inhliziyo. Ngazo zonke iimeko ectopia - arhente nokubekwa ezingachanekanga. Kule intliziyo sifo kuba esiswini okanye emqaleni. Ukongeza ectopia, kwaye kukho iziphene ezahlukeneyo. Ukongezelela VSD kunye ASD yenzeka atresia ka vana tricuspid, kwemithambo miphungeni. Kwiimeko ezinqabe kakhulu, ukungabikho ngokupheleleyo nasemagumbini intliziyo.
• Ithemba Diaphragmatic.

Ukongezelela cantrell Syndrome kungenzeka ingapheleli, kwaye kananjalo yabonakala ukuhlanganiswa kwezinye okungahambi. Amaninzi, esi sifo le CNS (Ithemba ingqondo, hydrocephalus), zesisu (ngezifo lwesinye inyongo kunye udakada), ukungabikho ngeminwe okanye omzimba.

Lo mfanekiso kwezonyango sifo

Iimpawu zesifo kwabonakala ukususela kwixesha lokuzalwa. Okokuqala yenza kwaziwe siphene ebalulekileyo izithambiso apho intliziyo amalungu esiswini "inamathele phandle" ngaphandle. Ukongeza, kwiiyure yokuqala yobomi kwaye bazibandakanye iimpawu ezimbi ngakumbi. Ngenxa ngenhliziyo ukukhubazeka naso kuzalwa ufumana ioksijini encinane. Ngenxa yoko, uphelelwa ngumphefumlo, cyanosis, indrawing of izithuba intercostal kunye ezantsi kwentamo. Amaninzi, ezi iintsana kufuneka adityaniswe kwi mpilo. Ukuze usola sifo, kuyimfuneko ukuba ukufumanisa apho intliziyo ukuqinisekisa omnye iimpawu eziphambili - umzimba ectopia.

Yintoni lokufunyaniswa pentad cantrell kaThixo?

Cantrell kaThixo syndrome uphethwe ezisekelwe ubukhulu becala kwiziphumo zoviwo lwangaphandle ye usana. Okokuqala, ugqirha ukhangela ubukho omphalocele - isici kodonga esiswini kunye thoracic, uze igqiba apho intliziyo. Phezu isiqinisekiso pregnancy, ukuphazamiseka yemida bokuphefumla hemodynamic, uxilongo bayilwele. Ukuze ubone ubukho ngenhliziyo ukukhubazeka, wenza auscultation kunye echocardioscopy. Ultrasound ukuziphatha isisu ayisebenzi kuba kuqala zonke iziphene kufuneka kuvalwe.

Treatment pentad cantrell

Ngaphandle kwento yokuba pentad cantrell - Lo vice buhlanganiswe eyoyikekayo, kwezinye iimeko kunokwenzeka ukuhlangabezana nayo. Oku kusebenza kuzo izigulane ukungaqheleki hemodynamic ehambelanayo nobomi. Unyango eligqibeleleyo eziphambili - ungenelelo yotyando. Utyando iqulathe izigaba ezininzi kwaye igqalwa omnye wemisebenzi enzima. Le liqatywe lwenziwa kwi thoracic umgca kunye nodonga esiswini. Kwangaxeshanye, oogqirha kufuneka enze kukulungiswa iziphene intliziyo kunye nokucuthwa kwinkqubo yokwetyisa. Ectopia entliziyweni sidla ngasekhohlo. Kwezinye iimeko, utyando ukuze VSD amancinci ASD ndibeke yobudala efanelekileyo (iminyaka emi-3).

Prevention malformation

Eyona ndlela iphambili yothintelo ukuba isifo siswini. pentalogy Ngokuqhelekileyo bene cantrell kwi Ingane ziziswe sele kwiveki 20-24 yophuhliso. Amaninzi, kwisigqibo onjalo isizathu ukupheliswa yokukhulelwa. Kuba Uthintelo lokuqala ziquka:

• Ukuze siphephe iingxaki.
• Gcina indlela yokuphila esempilweni.
• Ziphephe iimeko ndixinezeleke kakhulu, ingakumbi kwisiqingatha sokuqala yokukhulelwa.

I-ALS kwesi sifo luyathandabuzeka, oko njengoko kuxhomekeke kwi iqondo umonakalo entliziyweni. Kwezinye iimeko, oogqirha ukulawula ukusindisa ubomi bomntwana. Noko ke, aba bantwana kufuneka inkathalo rhoqo kunye kweliso labezempilo.