Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation. A clinical morphologic assessment--Part I. 816 31

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discussed stenotic aortic valves and Part II discusses causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, and rheumatic. Other rare causes included active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and disease affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal defect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing purely regurgitant aortic valves.
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PMID:Pathology of aortic valve stenosis and pure aortic regurgitation: a clinical morphologic assessment--Part II. 816 82

Aortic valve replacement in the child and young adult is often delayed, and multiple operations or invasive procedures are performed to avoid valve replacements. Prosthetic valves, bioprosthetic valves, or allograft valves have been associated with significant complications or early failure and have been a disappointing solution for the patient requiring aortic valve replacement. The pulmonary autograft replacement (PAR) of the aortic valve in children has been shown to be safe and effective with a low incidence of late valve dysfunction. The absence of thromboembolism, the avoidance of anticoagulants, and its viability with the potential for growth and repair strongly support its use for the potential parent, patients of age 35 or less. The experience with 112 patients, 32 females and 80 males, ages 1.5 to 35 years (average 16.1) are reviewed. Twenty-four had aortic insufficiency, 34 had aortic stenosis, and 54 had both aortic stenosis and insufficiency. Actuarial survival was 95.4% +/- 2.0% at 7 years and freedom from reoperation or significant aortic insufficiency of the autograft valve was 92.7% +/- 3.7%. Freedom from all valve related complications of the autograft valve and the homograft replacement of the pulmonary valve was 90.0% +/- 4.0%. Reoperation for the autograft valve was related to limited experience in one, leaflet prolapse and adherence to a VSD patch in one, associated lupus erythematosus in one, and annular and sinotubular dilatation in one. Reoperation of the homograft valve in two patients was secondary to early homograft stenosis, probably due to rejection.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pulmonary autograft replacement of the aortic valve in the potential parent. 818 67

It is very difficult to identify pregnant asymptomatic mothers carrying anti-SSA/SSB antibodies. We report two cases of neonatal lupus erythematosus, born to asymptomatic mothers with anti-SSA/Ro antibody, who developed isolated complete congenital cardiac heart block and transient second degree conduction defect associated with cardiac abnormalities, respectively. The first died suddenly of acute myocarditis at the age of 20 months, while the second underwent surgery at the age of 10 years for a ventricular septal defect, after two episodes of second degree atrioventricular block in infancy. We believe that in both cases the diagnosis could have been made in utero after correct heart beat analysis. We propose careful monitoring of fetal heart beat in all pregnant mothers. The occurrence of heart beat modification should prompt clinicians to test the mother for antibody positivity. This approach may permit early diagnosis and in utero treatment in order to spare the child from cardiac conduction defects. We provide the evidence of these cases and propose a flow chart for all physicians dealing with pregnancy.
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PMID:Congenital conduction defects in children born to asymptomatic mothers with anti-SSA/SSB antibodies: report of two cases. 985 74

Systemic lupus erythematosus is a disease in which inflammatory process provoked by different antibodies affects many organs and systems. The circulatory system is one of them. In patients with systemic lupus erythematosus cardiac disorders are generally known and well proved. It is known that this disease has heritage background. Thus, the offsprings of patients suffering from systemic lupus erythematosus belong to a risk group. Moreover, it is thought that maternal antibodies crossing transplancentally to the fetus cause damages of tissues including the heart. The aim of this study was to evaluate cardiological status of 38 children whose mothers suffered from systemic lupus erythematosus. In all sick mothers the diagnosis fulfilled criteria of the American Rheumatism Association. The mothers have been remaining under medical treatment while the children have been under control and simultaneously prophylaxis of lupus has been undertaken. The study was undertaken in 17 girls and 21 boys aged 3 to 18 years (average: 12 +/- 4.5 years). Physical development of presented children was satisfactory. During cardiological examination all subjects were in good general condition, without any clinical evidence of collagen disease and infection. Obtained results were compared with the ones found in control group of 38 children of healthy mothers, being at the same age. Study methods were: physical examination, arterial blood pressure measurement, standard and 24 hours according to Holter method ECG record, echocardiographic and Doppler examination, and physical performance test according to Bruce's protocol. In children of sick mothers examined laboratory parameters were within the normal limits excluding the presence of antinuclear antibodies (controlled by indirect immunofluorescence test), result of which was positive in 15 studied children (39%). In the group of the children of sick mothers the abnormalities of sinus node function were detected in 12 cases (32%), significantly more often than in the control. There were found abnormalities of atrio-ventricular and intraventricular conduction in 15 subjects (40%). The premature beats of ventricular origin were noticed in 3 cases (8%). These disturbances were significantly different from the control group. In addition, correlation between the presence of antinuclear antibodies and the cardiac abnormalities was taken into consideration. So, significant correlation between antinuclear antibodies and heart rhythm disorders was proved. During echocardiographic examination structural and functional abnormalities were found. They were: ventricular septal defect (muscular part) (1), pericarditis effusion (1), prolapse of the mitral valve anterior leaflet (2), mitral valve regurgitation of the second degree (2) and increased diameter of left atrium (8). One girl from the studied group, suffering from atrio-ventricular block of III* was operated on because of persistent ductus arteriosus still in the newborn's period. At the same time the permanent pace-maker was implanted. After 1 year of age this girl was operated on because of atrial septal defect (ASD II). In studied group of children echocardiographic global indices of left ventricular systolic function were normal. The subclinical impairment of diastolic left ventricle function was found in 8 children with increased left atrium-aorta index. Both the theoretical knowledge and the results of the studies suggest that the offsprings of mothers suffering from SLE need a careful cardiological observation.
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PMID:[Cardiological state of offsprings of the mothers suffering from systemic lupus erythematosus]. 1105 11

Lamotrigine (LTG) is a widely used second-generation antiepileptic drug for long-term therapy of epileptic patients. Although LTG monotherapy during pregnancy is assumed to be relatively safe, teratogenic effects related to LTG has been reported previously. The presence of fetal malformations and maternal drug-induced lupus erythematosus concurrently in a pregnant women using LTG have not been reported before. We herein report a term infant with coarctation of aorta and ventricular septal defect, who was born to a mother treated with LTG for epilepsy before conception and throughout pregnancy. The mother was diagnosed with drug-induced lupus erythematosus at the 36th gestational week, and the symptoms resolved after discontinuation of the drug. Fetal cardiac anomalies should be searched in mothers who were exposed to LTG during pregnancy.
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PMID:Coexistence of Fetal Cardiac Malformation and Maternal Drug-Induced Lupus: Is Lamotrigine Safe? 2641 83