Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Forty-two babies with different congenital cardiac conduction defects, and in 12 cases the mothers, were tested for autoantibodies to Ro, La, U1RNP and Sm. Ro-specific antibodies were detected most frequently. They were to be found in 16 sera from infants and in 8 maternal serum samples. The occurrence of anti-Ro was associated preferentially with several atrioventricular conduction blocks. The sex relation of anti-Ro associated congenital heart block did not show a typical preference (6 male/10 female). At the time of giving birth, 5 anti-Ro-positive mothers did not have any clinical symptoms of rheumatic autoimmune diseases. Three of them had a first degree atrioventricular block. Our findings indicate that all pregnant women at risk for anti-Ro like connective tissue disease or cardiac conduction defects should be tested for these autoantibodies because of the suspicion of cardiac conduction abnormalities in the offspring. Anti-Ro-positive infants should be examined for structural heart disease by echocardiography.
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PMID:Investigations into Ro-specific antibody-associated congenital cardiac conduction defects. 139 17

Sinus node dysfunction (SND) is a rare cause of bradycardia in children without structural heart disease. The clinical and diagnostic findings in 4 children with this condition are described. Two of them presented with symptoms, in one arrhythmias had been noted before birth, and a routine physical examination had revealed bradycardia in another. Age at onset of either clinical symptoms or bradycardia ranged from 0 to 11 1/2 years. Routine and 24-h-electrocardiograms showed atrioventricular junctional rhythms with minimal rates of 25/min and episodes of asystole with a maximal duration of 10.3 s. Other electrocardiographic abnormalities such as first degree atrioventricular block, ventricular extrasystoles or tachycardia were common findings. Electrophysiological studies were performed in 3 cases and confirmed the diagnosis of SND. A permanent pacemaker was inserted in 2 children; medical treatment did not have any long-term effect. During a follow-up period of 5 to 13 years there were no complications. In summary, SND in childhood can be assessed by Holter monitoring with high reliability. Electrophysiological studies are not necessary and of limited value. Therapeutic policies and prognostic statements are difficult to establish due to the small number of cases so far described. Permanent cardiac pacing, however, is unavoidable in symptomatic children.
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PMID:[Sinus node dysfunction in children without heart defect]. 752 46