گزارش یک مورد سندرم لوپوس نوزادی

Neonatal lupus syndrome is a rare disease. The manifestations of this disease in neonatal period include congenital heart block, cutaneous leasions, liver disease, thrombocytopenia, Neutropenia, pulmonary and nurologic derangements. Most of the clinical manifestations ofter disappear spontaneously. Although congenital heart block is usually permanent, and often require pacemakers. Congenital heart block is dangerous and life theratening. It may cause fetal death but more commonly results in neonatal cardiac morbidity, or neonatal death. A typical antenatal presentation is a fixed fetal heart rate of 50 to 60 beats per minute. Neonatal lupus syndrome is due to maternal IgG antibodies, directed against the ribonuclear protein of cardiac antigens. Which can be detected in 20-25% of serum samples. At the present time there is no intrauterine successful treatment. Although in utero pacemake placement has been attemped, no successful cases have been reported. The incidence of congenital heart block is reported between 15 to 30% and 50-70% of them need pacemaker placement. In this article, a neonatal lupus syndrome with congenital heart block is presented. This is the first child of a 25 years old mother, who is a known case of SLE pericardial effusion and cardiomegaly were reported is hersonographies and the fetus shoued fixed bradycardia, A term 3450 gram neonate was born with fixed heart rate of 70 perminute. He showed grade II congenital heart block and was referred to another hospital for pacemaker placment but he died because of complete heart block which did not respond to CPR attempts.