Overlap syndrome and its implications in pregnancy. Case report

Article Abstract:

The case report is presented of a 29-year-old pregnant woman with a history of juvenile chronic arthritis whose fetus was diagnosed with heart block, a disorder of the electrical conducting system. Ultrasound examination of the fetus at the age of 30 gestational weeks indicated complete heart block (where the atria and ventricles beat independently of one another); tests on the mother revealed immunological abnormalities associated with autoimmune disease (in which the body manufactures antibodies against its own proteins). The infant was delivered by cesarean section because of hemorrhage after 34 weeks gestation. The baby had complete heart block and a ventricular rhythm of 70 to 80 beats per minute, but required no special treatment. The mother and infant were discharged 21 days after delivery. Complete heart block, which occurs in 1 of every 20,000 births, is known to be associated with diseases of maternal connective tissue (tissues that support and partially compose the organs and bones). Overlap syndrome is a term that describes diffuse connective tissue diseases (DCTDs) with overlapping symptoms. It is possible for fetuses of mothers with such disorders to develop scarring of the heart muscle, in which areas of the heart's electrical pacing system are replaced with fibrous tissue. In such cases, defects of the fetal heart are noted during the second trimester of pregnancy. Information concerning the immunological characteristics of mothers of infants with congenital complete heart block is presented. Treatment for heart defects in such cases consists of decreasing the concentrations of maternal autoantibodies that cross the placenta or reducing inflammation in the fetal heart. These treatment approaches are described. The babies of women with anti-Ro (SS-A) and anti-La (SS-B) antibodies are particularly at risk for complete heart block. Even when DCTDs such as juvenile chronic arthritis are inactive, associated factors may contribute to complications in pregnancy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Abnormalities, Complications and side effects, Rheumatoid arthritis in children, Juvenile rheumatoid arthritis, Pregnancy, Complications of, Pregnancy complications, Fetal heart

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Fetal heart block associated with maternal anti-Ro (SS-A) antibody - current management; A review

Article Abstract:

Complete congenital heart block (CCHB) is a birth-related heart disorder characterized by poor conduction of the electrical impulses that cause the heart to contract. CCHB has been associated with the presence of connective tissue disease in the mother. In addition, infants with CCHB and their mothers have autoantibodies, or abnormal immune proteins which are directed against the body's own tissues and cell components. Ro (SS-A) antigen is a ribonucleoprotein, that is, a compound containing both ribonucleic acid and protein, which is capable of triggering an immune response. It is believed that Ro antigen in the heart activates the production of anti-Ro antibodies. The attachment of anti-Ro antibodies to Ro antigen may cause inflammation and the formation of fiber-like tissue in the heart's conduction system. The factors that increase the risk of CCHB include a previous infant with CCHB, elevated levels of anti-Ro antibodies or the additional presence of anti-La antibodies (another type of autoantibody directed against ribonucleoprotein) in the infant, and the presence of a specific human leukocyte antigen (HLA), DR3, in the mother. The HLA are tissue antigens present on most cells and are controlled by a specific set of genes called the major histocompatibility complex. They are capable of provoking immune responses, and specific HLA are increased in certain diseases. Pregnant women with anti-Ro antibody can be given preventive treatments to reduce levels of autoantibodies and inflammation of the fetal heart. When fetal heart block with inflammation resulting from anti-Ro antibodies is identified, the fetus must be continually monitored throughout the rest of the pregnancy and during delivery. (Consumer Summary produced by Reliance Medical Information, Inc.)

Analysis, Autoantibodies, Congenital heart disease, Congenital heart defects

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Raised maternal serum alpha-fetoprotein levels during pregnancy following treatment of an endodermal sinus tumor: case report

Article Abstract:

A healthy 23-year-old woman had surgery to remove a right ovarian cyst which did not extend past the capsule of the ovary; her right ovary and fallopian tube were removed. The tumor was diagnosed as an endodermal sinus tumor. Eleven months later the patient developed a tender nodule on the surgical scar; it was found to be a second tumor that matched the cell-type of the original endodermal sinus tumor. Blood tests detected an increase in alpha-fetoprotein (AFP), a protein that is increased in the blood of mothers carrying abnormal fetuses and in patients with certain types of cancer. Endodermal sinus tumors of the ovary are known to produce AFP. The AFP level decreased after the tumor was removed, and the patient received chemotherapy. Four months later the patient became pregnant. Routine blood screening revealed an increase in AFP. Since it was difficult to ascertain whether the source of the increased AFP was a recurrent endodermal tumor or a fetal abnormality, the type of AFP was characterized using chromatographic analysis. This laboratory method was able to determine the source of the AFP. It was found that the alpha-fetoprotein was not originating from the fetus, and ultrasonography, the use of high frequency sound to visualize the fetus, found no structural abnormalities. The AFP level declined rapidly after a normal full-term infant girl was delivered. It is not known why this patient had elevated AFP during her pregnancy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Measurement, Surgery, Ovaries, Ovarian tumors, Alpha fetoproteins

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