Cantrell sika pentad: izimbangela, ukwelashwa, onalesi sifo

Kukhona okuningi kombungu kwakheka utero. Abakwazi nomthelela ohlelweni isitho. I esiyingozi kakhulu kukhona ukukhubazeka inhliziyo, iphaphu, isimiso sokugaya ukudla bungene, izinso, kanye nobuchopho. Ezinye zazo azihambelani ukuphila. Okunye amenable ekwelashweni kuhlinzwa. Omunye kombungu eziyinkimbinkimbi kakhulu kubhekwa pentalogy Cantrell. Lesi sifo libhekene eziningi ukwephula izitho eziningana. Kwezinye izimo, ungase ukwazi ukuthola pentad akuphelele Cantrell. Naphezu kobukhulu isici kulo, ngezinye izikhathi odokotela bayakwazi ukuba babhekane nalesi sifo futhi ubuyisele imisebenzi ebalulekile zomzimba.

pentad Cantrell sika - siyini lesi zokugula?

Lokhu zokugula ihlobene yesifo senhliziyo azalwa naso, kanye nezinye izitho. Njengoba igama pentad Cantrell kuhlanganisa 5 zamehlo. Phakathi kwazo kunane-ukwephulwa eyingozi kakhulu ohlelweni senhliziyo nemithambo yegazi, luhambisana nomthwalo ushintsho hemodynamics. Futhi le nkinga kuhilela isici esibi yezimonyo. Bona kungaba ngokushesha ngesikhathi sokuzalwa ingane, njengoba inhliziyo ngosana 'silandela imithetho out ". Cantrell Syndrome akusho ngaso sonke isikhathi aqukethe 5 zamehlo. Ngokuvamile lokho 1 noma 2 iziyaluyalu bengekho (tetrad triad). Ngaphezu isifo senhliziyo, lesi sifo kuhlanganisa omunye uphawu esindayo - omphalocele. Futhi zikhona izinguquko besisu anterior nasesifubeni odongeni, diaphragm isici.

pentad Cantrell sika: kubangela yesifo

Kubalulekile ukwazi ukuthi kwakhiwa lesi sifo ngo senyanga yokukhulelwa wesibili. Phakathi nale nkathi, ebeka zonke izitho ezibalulekile kanye nezinhlelo. Njengoba ayaziwa amasonto 14-18 yokukhulelwa kwakhiwa mesoderm. Ngaphansi kwethonya yizici zemvelo nonina ahlukahlukene izifo abanye ukwephula embryogenesis kungenzeka phakathi nalesi sikhathi. Enye yalezi kwezinso kungaba Cantrell pentalogy. Imbangela sifo engakaze isungulwe. Nokho, le nkinga lihlotshaniswa chromosomal kwezinso. Phakathi kwabo, 18 futhi trisomy 13, Turner syndrome, X-exhumene isifo. Kuyaziwa ukuthi lo isici singesabo ngempela ezingavamile ezaziwayo. Imvamisa nesenzakalo salo 3-5 izinsana ngamunye million. Emkhakheni amacala ngaphansi kuka-100 kuchazwe. Lesi sifo Kwande ebafaneni yini ukwedlula.

Naphezu kweqiniso lokuthi ukuthola imbangela sifo azikakwazi, odinga ukukwazi mayelana engcupheni eziholela kwempilo wokukhula kwengane esiswini. Phakathi kwabo:

1. Umthelela izinto eziyingozi futhi ezinobuthi (nosawoti of izinsimbi ezisindayo, izidakamizwa, namakhemikhali).
2. Kushisa bhé kule ndawo ukuchayeka.
3. imithelela Physical (ukulimala).
4. ezimweni ezicindezelayo.
5. Ukubhema izinhlobo zophuzo oludakayo.

Mechanism kuphazamiseka hemodynamic

Ngenxa yokuthi i-pentad Cantrell babengayithola ukukhubazeka inhliziyo ezahlukene, ukuphazamiseka hemodynamic ngaleyo ndlela ihluke nomunye. Ngokuvamile lesi sifo kuhambisana isici ventricular septal. Ukuthuthukiswa iziyaluyalu hemodynamic incike okuphambene. Nge ukukhubazeka okukhulu zenzeka ezinkulu shunt indilinga lencane, kanjalo okuholela nomfutho wegazi ophezulu yamaphaphu. I-ventricle engakwesobunxele igcwele kakhulu futhi hypertrophy compensatory. Ngenxa yalokho, kukhona nokwanda zombili RV LV.

Ngaphansi ezivamile atrial septal isici. thuthuva Hemodynamic uma kuphinda kuncike usayizi yokungafaneleki. With zokuxhumana ezinkulu phakathi ukudedelwa Atria wegazi ku ilungelo ahlukaniswe is kakhulu anda, okuholela hypertrophy futhi dilatation we PP. I kwesokunxele sindisa atrial kwenzeka.

I ukubonakaliswa eyinhloko pentad Cantrell sika

Njengoba yaziwa, the classic Cantrell Syndrome kuhlanganisa 5 zamehlo. Phakathi kwazo kunabafundi ukwephulwa lokhu okulandelayo:

• umbungu Omphalocele. Kwenye indlela ibizwa ngokuthi eliphezulu enkabeni besisu odongeni isici. Lokhu ukuhlukumeza libhekene yokuthi izibilini ngokuvamile atholakala esiswini, ziqoqwa e enkabeni isikhwama hernia uhambe ngaphandle.
• Chest odongeni isici. It is kakhulu ngokuvamile ehambisana ukungabi noma ukuhlukaniswa xiphoid. Ngenxa yalokho, inhliziyo futhi uqhubekela phambili.
• Ukungabikho pericardium, okuyinto ukuba ezakhelene diaphragm.
• Senhliziyo ukuthuthukiswa anomalies. Cishe zonke izimo ectopia - ayilungile ukubekwa ejensi. Ngalesi inhliziyo isici kungaba besisu noma entanyeni. Ngaphezu kwalokho ectopia, futhi kukhona ukukhubazeka ahlukahlukene. Ngaphezu VSD futhi ASD kwenzeka atresia of valve tricuspid, umfula yamaphaphu. Ezimweni ezingavamile, ukungabi okuphelele emakamelweni inhliziyo.
• hernia Diaphragmatic.

Ngaphezu Cantrell Syndrome ayiphelele, futhi wabuye wabona ne inhlanganisela nezinye anomalies. Ngokuvamile, lokhu isifo se-CNS (ubuchopho umbhunu, iphimbo), emathunjini (zokugula kwesinye nenyongo futhi ubende), ukungabi bikho kokukhanya iminwe noma nasemilenzeni.

Lwasemtholampilo isithombe lesi sifo

I izimpawu zesifo sobala kusukela ekuzalweni. Eyokuqala izenze eyaziwa isici esibalulekile yezimonyo lapho inhliziyo futhi izitho besisu "eniqhubeka nimkhiphela" ngaphandle. Ngaphezu kwalokho, e amahora yokuqala yokuphila futhi ujoyine izimpawu esabekayo ngaphezulu. Ngenxa azalwa naso inhliziyo yayo yayinezinkinga ezicini ewusana olusanda kuzalwa uthola umoya-mpilo omncane. Ngenxa yalokho, kwakufanele bonyana iphika, cyanosis, indrawing ka intercostal izikhala kanye engxenyeni engezansi sternum. Ngokuvamile, kufanele lezi zingane exhunywe kwi womoya-mpilo. Ukuze usola isifo, kubalulekile ukuthola lapho inhliziyo ukuqinisekisa esinye sezici eziyinhloko - umzimba ectopia.

Kuyini sitholakele pentad Cantrell sika?

syndrome uphethwe Cantrell sika ngokuyinhloko usekelwe emisebenzini yasemahhovisi luhlolo lwangaphandle ngayo lapho usana. Okokuqala, udokotela ecinga khona omphalocele - isici odongeni besisu futhi thoracic, bese inquma lapho inhliziyo eyikho. Lapho isiqinisekiso ethunjini, ukuphazamiseka imingcele zokuphefumula futhi hemodynamic, uphethwe cishe ezithile. Ukuze sithole khona ukukhubazeka inhliziyo, wenziwa auscultation futhi echocardioscopy. Ultrasound wokuziphatha esiswini zingenzeki ngoba lokuqala konke ukushiyeka kumelwe iqedwe.

Ukwelashwa pentad Cantrell

Naphezu kweqiniso lokuthi pentad Cantrell - Lokhu kuhlangene nelesabekayo okuphambene, kwezinye izimo kungenzeka okubhekana nayo. Lokhu kusebenza iziguli kwezinso hemodynamic iyahambisana ukuphila. The main ukwelashwa angelasici - ukungenela kuhlinzwa. Ukusebenza unezigaba eziningana futhi ubhekwa kwezinwele eziqine. I imbobo wenziwa ku thoracic umugcamkhatsi nodonga besisu. Ngesikhathi esifanayo, odokotela abahlinzayo Kumele bente Umsebenti ukulungiswa sesimweni inhliziyo nokwehlisa isimiso sokugaya ukudla bungene. Ectopia wenhliziyo ngokuvamile kwesokunxele. Kwezinye izimo, ukuhlinzwa ukuze VSD amancane ASD yabeka yobudala ekahle (iminyaka engu-3).

Prevention malformation

Indlela eyinhloko zokuvimbela ukuba uphethwe kokubeletha. pentalogy Ngokuvamile kutholakala ukuthi uphethwe yini Cantrell ku-fetus kungaba inikelwe kakade 20-24 ngesonto yentuthuko. Ngokuvamile, isiphetho esinjalo iyona isizathu ukukhipha izisu. Ukuze lokuvikela eyinhloko zihlanganisa:

• Ukuze ugweme imiphumela eyingozi.
• Gcina indlela yokuphila enempilo.
• Gwema izimo ezibangela ukucindezeleka, ikakhulukazi esiwombeni sokuqala sokukhulelwa.

Onalesi sifo yalesi sifo kuyadida, njengoba kuncike izinga lomonakalo enhliziyweni. Kwezinye izimo, odokotela uphathe ukusindisa ukuphila kwengane. Noma kunjalo, lezi zingane zidinga ukunakekelwa njalo futhi kokuqondisa ochwepheshe.