Cantrell ta pentad: haddasawa, magani, hangen nesa

Akwai yawa malformations kafa a utero. Sun iya shafar wani sashin jiki tsarin. A mafi hatsari ne zuciya lahani, huhu, narkewa kamar tsarin, kodan, da kuma kwakwalwa. Wasu daga cikin su ne m da rai. Sauran amenable ga m magani. Daya daga cikin mafi hadaddun malformations dauke pentalogy Cantrell. Wannan cutar halin a yawan keta da dama gabobin. A wasu lokuta, za ka iya samun wani bai cika pentad Cantrell. Duk da tsananin daga cikin aibi, wani lokacin likitoci suna iya magance da cutar da kuma mayar da m ayyuka na jiki.

Cantrell ta pentad - abin da yake wannan Pathology?

Wannan Pathology aka alaka nakasar zuciya cuta, da kuma sauran gabobin. Kamar yadda sunan pentad Cantrell hada 5 lahani. Daga gare su ne mafi m take hakkin da zuciya da jijiyoyin jini tsarin, wanda entails wani canji a hemodynamics. Har ila yau, wannan aibi ya shafi mummunan kwaskwarima lahani. Gane shi iya zama nan da nan a haifi jaririn, a matsayin zuciya da jariri "sandunansu fita". Cantrell ta ciwo ba ko da yaushe kunshi 5 lahani. Sau da yawa da cewa 1 ko 2 cuta ne ba ya nan (Tetrad triad). Bugu da kari ga zuciya cuta, cuta ya hada da wani nauyi alama - omphalocele. Har ila yau, akwai canje-canje a cikin agara na ciki da kuma kirji bango, diaphragm lahani.

Cantrell ta pentad: Yanã cuta

Yana da muhimmanci a san cewa cutar da ake kafa a karo na biyu trimester ciki. A wannan lokacin, kwanciya duk muhimmanci gabobin da kuma tsarin. Kamar yadda aka sani, ga 14-18 makonni na ciki kafa mesodam. Ƙarƙashin rinjayar muhalli dalilai da kuma daban-daban cututtuka uwa wasu keta embryogenesis na iya faruwa a lokacin wannan lokaci. Daya daga cikin wadannan munanan iya zama pentalogy Cantrell. A daidai hanyar da cutar ba a kafa. Duk da haka, wannan lahani ake dangantawa da chromosomal munanan. Daga cikin su, 18 da kuma trisomy 13, Turner ciwo, X-nasaba da cutar. An sani cewa wannan aibi ne quite rare. A mita na da ya faru shi ne 3-5 jarirai da miliyan. A duniyar kasa da 100 lokuta da aka bayyana. A cuta ne mafi kowa a boys.

Duk da cewa ya gano tushen hanyar cuta ba ta riga ta kasance, kana bukatar ka sani game da hadarin dalilai da kai ga daban-daban cuta da fetal ci gaba. Daga cikin su:

1. A tasiri na cutarwa da kuma guba abubuwa (salts da nauyi karafa, da miyagun kwayoyi, da kuma sunadarai).
2. Radiation daukan hotuna.
3. Jiki tasirin (rauni).
4. Stressful yanayi.
5. Shan taba da kuma shan giya.

Hanyar hemodynamic cuta

Saboda gaskiya cewa pentad Cantrell iya fuskanci daban-daban zuciya lahani, hemodynamic disturbances haka bambanta da juna. Mafi sau da yawa wannan cuta yana tare da na ramin zuciya septal lahani. A ci gaba da hemodynamic cuta ya dogara da mataimakin. Tare da gagarumin lahani faruwa a manyan shunt kananan da'irar, game da shi, abu don na huhu hauhawar jini. A hagu ventricle aka dora kuma compensatory hypertrophy. A sakamakon haka, akwai wani karuwa a duka biyu da RV da LV.

Kadan na kowa atrial septal lahani. Hemodynamic disturbances a lokacin da shi kuma ya dogara da girman da lahani. Tare da babban sadarwa tsakanin atria sallama na jini a cikin dama rabu da aka muhimmanci ƙãra, sakamakon hypertrophy da dilatation na PP. A bar atrial obalodi faruwa.

Babban manifestations Cantrell ta pentad

Kamar yadda aka sani, da na gargajiya Cantrell Syndrome hada 5 lahani. Daga cikin su akwai wadannan keta:

• Omphalocele tayin. A wani hanya shi ne ake kira babba-cibiyarki ciki bango lahani. Wannan take hakkin ne halin da cewa kayan ciki kullum samu a ciki, suna tattara a cibiyarki hernia jakar da tafi a waje.
• Chest bango lahani. An fi halin da rashi ko mai tsaga xiphoid. A sakamakon haka, da zuciya ne kuma ci gaba da tafiya.
• A rashi na pericardium, wanda shi ne ya zama m ga diaphragm.
• Cardiac anomalies ci gaba. A kusan dukkan lokuta da ectopia - ba daidai ba jeri hukumar. A wannan zuciya lahani iya zama a ciki ko wuya. Bugu da kari ectopia, kuma akwai daban-daban lahani. Bugu da kari ga VSD da ASD auku atresia na tricuspid bawul, da na huhu jijiya. A rare lokuta, da cikakken rashi na da zuciya da bẽnãye.
• Diaphragmatic hernia.

Bugu da kari ga Cantrell Syndrome iya zama bai cika ba, kuma shi ne ma ya lura da hade da sauran anomalies. Mafi sau da yawa, wannan cuta na CNS (kwakwalwa hernia, hydrocephalus), gastrointestinal (Pathology na gall mafitsara da kuma baƙin ciki), babu yatsunsu, ko kuma wata gabar jiki.

The asibiti hoto da cutar

A bayyanar cututtuka da cutar zama na fili daga lokacin haihuwa. A farko sa kanta da aka sani gagarumin kwaskwarima aibi a cikin abin da zuciya da kuma na ciki gabobin "tsaya daga" a waje. Bugu da kari, a cikin ta farko hours na rayuwa da kuma shiga mafi tsanani bayyanar cututtuka. Saboda nakasar zuciya lahani jariri samun kadan oxygen. A sakamakon haka, ya yi shortness na numfashi, cyanosis, indrawing na intercostal sarari da kuma ƙananan ɓangare na sternum. Mafi sau da yawa, wadannan jarirai dole a haɗa wani ventilator. Don zargin da cuta, shi wajibi ne don gano inda zuciya tabbatar da daya daga cikin manyan siffofin - ectopia jiki.

Mene ne ganewar asali Cantrell ta pentad?

Cantrell ta ciwo ganewar asali dogara ne da farko a kan waje jarrabawa da jariri. Da farko, likita ya dubi ga gaban omphalocele - wani aibi na ciki da kuma thoracic bango, sa'an nan kayyade inda zuciya ne. A kan tabbatar da ectopic, cuta na numfashi da kuma hemodynamic sigogi, da ganewar asali ne kusan wasu. Domin sanin gaban zuciya lahani, yi auscultation da echocardioscopy. Duban dan na ciki hali impractical saboda farko na dukkan lahani dole ne a shafe.

Jiyya pentad Cantrell

Duk da cewa da pentad Cantrell - Wannan yana hade da mumunar mataimakin, a wasu lokuta yana yiwuwa ya jimre da shi. Wannan ya shafi marasa lafiya da hemodynamic munanan jituwa tare da rayuwa. Babban lahani magani - wani m baki. The aiki kunshi dama, saukarwa kuma an dauki daya daga cikin toughest. A incision aka yi a cikin midline thoracic da kuma na ciki bango. A lokaci guda, likitoci ya kamata gudanar da gyara na zuciya lahani kuma rage daga cikin narkewa kamar tsarin. Ectopia na zuciya da aka yawanci bar. A wasu lokuta, tiyata ga kananan VSD da ASD sa a mafi m shekaru (3 years).

rigakafin malformation

Babban Hanyar rigakafin ne zuwa prenatal ganewar asali. Yawancin lokaci kamu pentalogy Cantrell a cikin tayin za a iya tsĩrar da riga at 20-24 mako na gaba. Mafi sau da yawa, irin wannan ƙarshe shi ne dalilin da karewa daga ciki. Domin farko rigakafin hada da:

• Don kauce wa cutarwa effects.
• Kula da lafiya salon.
• Kauce wa stressful yanayi, musamman ma a farkon rabin ciki.

A hangen nesa da wannan cuta ne shubuha, kamar yadda shi ya dogara da mataki na lalacewar da zuciya. A wasu lokuta, likitoci sarrafa ajiye wani yaro ta rayuwa. Duk da haka, wadannan yara suna bukatar m kula da dubawa na kwararru.