Maternal and sibling microchimerism in twins and triplets discordant for neonatal lupus syndrome-congenital heart block

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Maternal and sibling microchimerism in twins and triplets discordant for neonatal lupus syndrome-congenital heart block.

OBJECTIVE Neonatal lupus syndrome-congenital heart block (NLS-CHB) is an acquired autoimmune disease in which maternal autoantibodies are necessary but not sufficient for disease. Maternal myocardial cells have been found in the hearts of patients with NLS-CHB, suggesting that maternal microchimerism may also play a role. In this study we asked whether levels of microchimerism in the blood are ...

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treatment of congenital complete atrioventricular heart block with permanent epicardial pacemaker in neonatal lupus syndrome

conclusions mothers with sle should be screened and closely followed up during pregnancy for the development of fetal atrioventricular (av) block. case presentation a term infant with fetal bradycardia, detected at the 23rd gestational age, was diagnosed with chb due to nls and was successfully treated with a permanent epicardial pacemaker. the patient was reported here due to rarity of the pro...

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Familial congenital complete heart block and maternal systemic lupus erythematosis.

A family is reported in which two siblings had congenital complete heart block with resultant congestive heart failure, the father and paternal grandfather show adult-onset conduction defects, and the mother has systemic lupus erythematosis. The interaction of heredity and environment is discussed in this context. A review of the literature on familial complete heart block suggests that so-call...

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Discordant disease expression of neonatal lupus erythematosus in twins.

Neonatal lupus erythematosus is an autoimmune disease resulting from the trans-placental passage of maternal anti-SSA/Ro, anti-SSB/La, and less frequently anti-RNP antibodies to the foetus. At the time of diagnosis 50% of mothers are asymptomatic. Neonatal manifestations of this multisystem disease may include congenital heart block, cutaneous lesions and haematological abnormalities. We presen...

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Neonatal congenital heart block.

Congenital Heart Block (CHB) is the most serious complication of neonatal lupus erythematosus. Transplasental transfer of maternal anti SSA/Ro or antiSSB/La antibodies around 12th week of gestation is associated with development of CHB. This may lead to inflammation, fibrosis and scarring of fetal conduction system in the early second trimester. Different degrees of atrioventricular (AV) block ...

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ژورنال

عنوان ژورنال: Rheumatology

سال: 2004

ISSN: 1462-0332,1462-0324

DOI: 10.1093/rheumatology/keh453