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NEONATAL LUPUS ERİTEMATOZUSA SEKONDER KONJENİTAL ATRİOVENTRİKÜLER TAM BLOK

CONGENITAL COMPLETE ATRIOVENTRICULAR BLOCK SECONDARY TO NEONATAL LUPUS ERYTHEMATOSUS

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Abstract (2. Language): 
Neonatal lupus erythematosus (NLE), which is a disorder caused by the passage of maternal anti-Ro/SSA and anti-La/SSB autoantibodies through the placenta. It is clinically characterized mainly by cutaneous eythematous rash, congenital atrioventricular block (AVB), haematologic and hepatic disorders. Congenital AVB is the most important and serious symptom. In this case, we report a premature newborn which was delivered by emergent c-section due to sustained fetal bradicardia. The newborn diagnosed as complete AVB and he treated with surfactant due to respiratory distress syndrome. But unfortunately, the baby died on 5th postnatal day. The maternal history was remarkable for four recurrent abortus. Anti-Ro and anti-La antibodies were positive for antibodies and infant diagnosis was establieshed as NLE. In conclusion, NLE should be keept in mind, if persistant bradicardia diagnosed on antenetal ultrasonography evaluation of women with recurrent abortuses and/or newborn with persistant complete AVB . Complete AVB in the fetus is irreversible, although maternal steroids can prevent progression to complete AVB.
Abstract (Original Language): 
ÖZET Neonatal lupus eritematozus (NLE) maternal anti SS-A/Ro ve anti SS-B/La antikorlarının transplasental geçiĢi sonucu oluĢan, eritematöz cilt lezyonları, doğumsal atrioventriküler (AV) blok, hepatik fonksiyon bozukluğu ve hematolojik bozukluklar ile seyreden kazanılmıĢ bir otoimmün hastalıktır. En önemli ve ciddi bulgusu doğumsal kalp bloğudur. Bu yazıda annedeki antikor pozitifliği bilinmeyen, fetal bradikardi nedeniyle acil sezaryen ile 34 haftalık prematüre olarak doğan, AV tam bloğu olan, respiratuar distres sendromu nedeniyle iki kez sürfaktan tedavisi uygulanan ve beĢ günlükken kaybedilen olan bir olgu sunulmuĢtur. Dört kez abortusu olan anneden doğumdan sonra ve bebekten postnatal ilk gününde gönderilen anti SS-A/Ro ve anti SS-B/La antikorlarının pozitif olduğu postmortem olarak öğrenilmiĢtir. Sonuç olarak tekrarlayan düĢükleri olan bir gebede ve sebat eden fetal bradikardi varlığında etyoloji araĢtırılırken anti-Ro ve anti-La antikorlarının da bakılması düĢünülebilir. Risk grubunun önceden bilinmesi ile birinci derece AV blok geliĢimi tespit edilebilir ve geri dönüĢümsüz AV tam bloğa ilerlemeyi durdurabilen maternal steroid uygulaması imkanı sağlanabilir.

REFERENCES

References: 

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