Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A cohort study was undertaken of 18 consecutive pregnancies in 16 patients with systemic lupus erythematosus without significant renal impairment. Of these, eight each had at least two previously unexplained pregnancy failures, 10 had elevated anticardiolipin antibodies, nine had the lupus anticoagulant and five had antibodies to Ro. Twelve live births resulted but a Downs syndrome baby died in the perinatal period. Fetal morbidity was confined to four cases of fetal growth retardation, all in mothers positive for anticardiolipin antibodies, and fetal distress in another two. There were no cases of the neonatal lupus syndrome. There was one maternal death. An obstetric history of at least two previously unexplained pregnancy failures and the presence of maternal anticardiolipin or anti-Ro antibodies accurately predicted fetal loss in six of seven cases. No other serological risk factors for fetal loss were identified. We were unable to show that suppression of antibodies in those mothers with at least two previously unexplained pregnancy failures improved fetal outcome.
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PMID:Pregnancy and lupus: prognostic indicators and response to treatment. 317 25

Anti-IgG antibodies were detected in 69% of 235 sera tested. A simple and reproducible method was developed to measure the avidity index of anti-IgG antibodies. It was found that subjects with systemic lupus erythematosus and progressive systemic sclerosis and normal controls had antibodies with lower avidity index than those with rheumatoid arthritis and Down's syndrome. The difference in avidity seems to be disease specific. Avidity does not correlate with antibody titers, but all sera with high avidity antibody are positive in the latex test. The anti-IgG as measured by this method was shown to be mainly of IgM class. The avidity indices of whole sera and purified IgM fractions were similar.
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PMID:Avidity indices of anti-IgG antibodies in diseases. 645 18

The clinical, hematologic, and cytogenetic findings are described in a patient who developed clinical and hematologic features of acute myelogenous leukemia (AML) after a three-year period of observation with unexplained thrombocytopenia. Five months before the diagnosis of AML she developed hepatosplenomegaly and a lupus-like syndrome. At this time she was also found to have trisomy 21 in all bone marrow cells studied, in addition to trisomy 8 in a few cells. The finding of trisomy 21 in all of the bone marrow cells examined could reflect a nonrandom alteration in the leukemic stem line or it might indicate that mosaic patients with trisomy 21 cells in their bone marrow share the increased risk of AML that has been documented for trisomy 21 patients.
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PMID:Trisomy 21 in bone marrow cells of a patient with a prolonged preleukemic phase. 693 5

An 8-year-old male, who had Down syndrome associated with systemic lupus erythematosus (SLE), is described. He also had a partial complement 4 deficiency. This case is a reminder that the physician should be aware of the possibility of an immune defect in a male presenting with SLE at a young age. The question of whether the association of Down syndrome with SLE is coincidental or whether there is a predilection for autoimmune disorders in Down syndrome is discussed.
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PMID:Down syndrome associated with systemic lupus erythematosus: a mere coincidence or a significant association? 783 1

Moyamoya disease is characterized by bilateral involvement of the internal carotid arteries. The etiology of this involvement is unknown. However, we previously reported two pediatric cases of moyamoya disease that progressed from unilateral to bilateral involvement. Some cases of unilateral occlusion in the carotid fork seem to have occurred at an early stage of definite moyamoya disease. In the present study, we examined five pediatric patients showing bilateral and/or unilateral occlusion of the internal carotid artery. In each case, the etiology was known. They included Apert syndrome, radiation therapy for pilocytic astrocytoma, systemic lupus erythematosis, von Recklinghausen disease and Down syndrome. The clinical manifestations, EEG, CT, MRI, PET, and angiographic findings in these patients were presented in comparison with those of definite moyamoya disease. This comparison led us to suggest that definite moyamoya disease might be included in these cases, and we emphasize the importance of precise examination in each case.
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PMID:[Akin moyamoya disease in children]. 842 89

An association is described between women with lupus anticoagulant and abnormal prenatal serum screening results. Three cases of positive second-trimester serum screening for Down syndrome, with karyotypically normal fetuses, in women demonstrated to have lupus anticoagulant are presented. Serum screening positivity was principally due to a disproportionately elevated maternal serum human chorionic gonadotrophin (hCG) level. In each case, early, severe intrauterine growth restriction was documented, with only one fetus surviving the neonatal period. As maternal lupus anticoagulant may have a profoundly adverse effect on the course of pregnancy, we suggest that an elevated hCG level on prenatal screening prompt consideration of maternal lupus anticoagulant testing if ultrasonography demonstrates an otherwise normal singleton gestation and the fetal karyotype is normal.
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PMID:Elevated mid-trimester hCG and maternal lupus anticoagulant. 860 82

Our study describes the presence of antineutrophil cytoplasmic antibodies (ANCA) in a group of different pathologies comprising 101 patients. Rheumatoid arthritis, systemic lupus erithematosus, idiopatic neutropenia, acute post-streptoccocal glomerulonephritis, minimal change nephrotic syndrome, Downs syndrome, adult periodontitis, tumoral calcinosis, monoartheritis and lipodystrophy were investigated for ANCA, through indirect immunofluorescence and an indirect solid-phase immunoassay (ELISA). Our results show the pattern of distribution of ANCA in the diseases investigated, and allowed us to make the first description of ANCA in diseases such as Downs syndrome, acute post-streptococcal glomerulonephritis and adult periodontitis. The high percentage of reactivity for ANCA detected in adult periodontitis, raise important questions about the possibility of reporting inaccurate percentages of positivity for some diseases, due to the presence of a concurrent disease such as adult periodontitis.
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PMID:[Antineutrophil cytoplasmic antibodies (ANCA): study of their presence in diseases not associated with arteritis]. 871 20

Elastosis perforans serpiginosa can arise as a rare complication in patients with Down syndrome. It is one of the primary perforating disorders characterized by transepithelial elimination of abnormal elastic fibers. This may reflect an immunological reaction aquist elastic fibers. We describe a 40-year-old woman with Down's syndrome whose skin lesions were histologically mistaken for lupus vulgaris for a long time.
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PMID:[Elastosis perforans serpiginosa in Down syndrome]. 948 66

Maternal serum inhibin A levels are increased on average in pregnancies affected by Down syndrome (DS). However, some reports have found increased serum levels in women with pre-eclamptic toxaemia as well. In the current study, maternal serum inhibin A was retrospectively measured in a series of 32 serum samples from pregnant women previously diagnosed as having either systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (APS). For comparison, normal medians were calculated from 57 unaffected control pregnancies together with a total of 854 samples tested at 13-19 weeks of gestation as part of the routine antenatal DS screening. All results were expressed in multiples of the gestation specific normal medians (MoM). A cubic regression formula was fitted, weighting for the number of women tested at each gestation. The median MoM value in the 16 cases of SLE and the 16 cases of primary APS is 0.60 (95% confidence interval 0.40-0.91) and 0.88 (95% confidence interval 0.66-1.17), respectively. For primary APS this was not statistically significant, whereas the SLE patients had a highly statistically significant reduction of serum inhibin A (p<0.002, Wilcoxon Rank sum Test, 2 tailed). Six pregnancies in the SLE group had a complicated obstetric outcome, i.e. missed abortion, placental abruption, exacerbation of the underlying disease which necessitated delivery, and severe postpartum haemorrhage. In 85% of this subgroup, serum inhibin A levels were below the normal 10th centile. The current data suggest that serum inhibin A is decreased on average in SLE patients. Those preliminary results might have various obstetric implications such as antenatal DS screening of SLE patients, identification of pregnant women at risk of developing SLE, who have presented for routine DS screening and for monitoring SLE patients throughout their pregnancy.
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PMID:Serum inhibin A levels in pregnant women with systemic lupus erythematosus or antiphospholipid syndrome. 1070 44

Maternal serum human chorionic gonadotrophin (hCG) levels were measured during the second and the third trimesters of pregnancy in patients with either systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (APS). All results were expressed in multiples of the gestation-specific normal medians (MoM). The median MoM level in 17 samples from SLE patients was 1.48 compared with 0.79 MoM in 99 controls of similar gestation (p < 0.002, Wilcoxon Rank sum test). In contrast the median MoM level in 19 samples from primary APS patients was only 1.14. These preliminary findings should be further studied to evaluate the implications for Down syndrome screening, detection of SLE cases during pregnancy and the prediction of adverse outcome in SLE gestations.
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PMID:Maternal serum human chorionic gonadotrophin levels in systemic lupus erythematosus and antiphospholipid syndrome. 1124 44


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