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1/116. Successful pregnancy and delivery in a case of systemic lupus erythematosus treated with immunoadsorption therapy and cyclosporin A.

    A 32-year-old woman diagnosed as systemic lupus erythematosus (SLE) became pregnant. During pregnancy she was treated with a daily dosage of prednisolone 15 mg. However, because the exanthema became worse, she was hospitalized on January 14, 1997 in order to receive immunoadsorption therapy. Before delivery we implemented the immunoadsorption therapy twice and cyclosporin A (CsA) was administered simultaneously. She gave birth in her 37th week. The baby weighed 2260 g at the time of delivery and had no deformities. The mother also had no side effects. The success of pregnancy and childbirth in our case, without any side effects, shows the possibility that the combination of CsA and immunoadsorption therapy may be considered safe to control a pregnancy complicated by SLE.
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keywords = pregnancy
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2/116. Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus.

    Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.
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ranking = 0.85714285714286
keywords = pregnancy
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3/116. Immunological abnormalities in primary APS evolving into SLE: 6 years follow-up in women with repeated pregnancy loss.

    We have performed a prospective study to determine the prevalence of immunological abnormalities and the evolution from primary antiphospholipid syndrome (APS) into systemic lupus erythematosus (SLE) in women who had had unexplained repeated pregnancy loss (PL) and APS. Of 105 women with abortions or fetal deaths, 33(31%) fulfilled criteria for APS. Among these patients with primary APS, 24% had antinuclear antibodies (ANA), 91% had elevated circulating immune complexes (CIC), 70% had low total haemolytic complement (CH100), 52% had low levels of complement 4 (C4) and 30% had low levels of complement 3 (C3), in a significantly higher prevalence than women whose pregnancies were successful (control group). Through out a 6 y follow-up, 3 (9%) of the patients with APS who had autoimmune related abnormalities when entered into the study developed features of lupus like disease (LLD) or fullblown SLE. Our findings suggest that women with unexplained repeated PL with APS who presented with positive ANA, high levels of CIC, low levels of CH100, C3 and C4, may define a subset of patients exhibiting immunological alterations similar to those of SLE. These parameters may help in the assessment of prognosis in APS patients with PL. Those patients should be carefully surveyed with regard to the development of connective tissue diseases.
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ranking = 0.71428571428571
keywords = pregnancy
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4/116. Apparent cyclophosphamide (cytoxan) embryopathy: a distinct phenotype?

    cyclophosphamide (CP) is an alkylating agent widely used in treating cancer and autoimmune disease. CP is classified as a pregnancy risk factor D drug and is teratogenic in animals, but population studies have not conclusively demonstrated teratogenicity in humans. Six isolated reports of prenatally exposed infants with various congenital anomalies exist, but to date no specific phenotype has been delineated. The purpose of this report is to document a new case of in utero CP exposure with multiple congenital anomalies and to establish an apparent CP embryopathy phenotype. The mother had systemic lupus erythematosus and cyclophosphamide exposure in the first trimester. She also took nifedipine, atenolol, clonidine, prednisone, aspirin, and potassium chloride throughout pregnancy. The infant had growth retardation and multiple anomalies including microbrachycephaly, coronal craniosynostosis, hypotelorism, shallow orbits, proptosis, blepharophimosis, small, abnormal ears, unilateral preauricular pit, broad, flat nasal bridge, microstomia, high-arched palate, micrognathia, preaxial upper limb and postaxial lower limb defects consisting of hypoplastic thumbs, and bilateral absence of the 4th and 5th toes. chromosomes were apparently normal. The reported cases of in utero exposure to cyclosposphamide shared the following manifestations with our patient: growth deficiency, developmental delay, craniosynostosis, blepharophimosis, flat nasal bridge, abnormal ears, and distal limb defects including hypoplastic thumbs and oligodactyly. We conclude that (a) cyclophosphamide is a human teratogen, (b) a distinct phenotype exists, and (c) the safety of CP in pregnancy is in serious question.
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ranking = 0.42857142857143
keywords = pregnancy
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5/116. Hypocomplementemia correlates with intrauterine growth retardation in systemic lupus erythematosus.

    PROBLEM: The aim of this study was to elucidate fetomaternal risks in systemic lupus erythematosus (SLE)-complicated pregnancy. METHOD OF STUDY: pregnancy course, complications, and fetal outcome in 82 pregnancies of 55 patients with SLE were investigated. RESULTS: These 82 pregnancies resulted in 14 fetal losses and 66 live births. Without clinical manifestation of SLE-flare, 4 of 8 patients who had low serum complement activity during the pregnancies delivered small-for-date neonates. The rate of the intrauterine growth retardation was significantly higher than that observed in pregnancies with normal complement activity. The frequency of premature deliveries (60%) in patients who received more than 15 mg/day of prednisolone was significantly high when compared with pregnancies maintained by 0-15 mg/day (13.1%). CONCLUSIONS: These data demonstrate the preconceptional and perinatal management necessary in SLE and suggest that the pregnancy with hypocomplementemia, the disease activity, and/or a relatively high maintenance dose of corticosteroid should be carefully managed and monitored.
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ranking = 0.28571428571429
keywords = pregnancy
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6/116. Management of heparin-associated thrombocytopenia in pregnancy with subcutaneous r-hirudin.

    heparin-induced thrombocytopenia type II is a serious, immune-mediated complication of heparin therapy. Due to its low cross-reactivity with heparin-associated antibodies (10-20%), danaparoid has successfully been administered in these patients. In recent studies, r-hirudin as a potent and specific thrombin inhibitor, was demonstrated to be a safe and effective anticoagulant. We report a pregnant woman with systemic lupus erythematosus and recurrent venous thromboembolism who suffered from heparin-induced thrombocytopenia type II while treated with dalteparin sodium. Positive cross-reactivities with danaparoid were found. Anticoagulation with 15 mg subcutaneous r-hirudin was performed twice daily from the 25th week of pregnancy until delivery. No thromboembolism or bleeding or fetal toxicity of r-hirudin was detected. Recombinant hirudin is a potent and specific thrombin inhibitor that can be used as a safe and effective anticoagulant in pregnancy.
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ranking = 0.85714285714286
keywords = pregnancy
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7/116. Libman-Sacks endocarditis in a pregnant woman with acute respiratory distress syndrome.

    BACKGROUND: Sterile fibrinous vegetations on the mitral valve (Libman-Sacks endocarditis) might be found in one-third of patients with antiphospholipid antibodies. Usually of minor hemodynamic significance, these vegetations might complicate acute respiratory distress syndrome in pregnancy. CASE: Despite delivery and aggressive medical therapy, a 17-year-old primigravida with pyelonephritis and acute respiratory distress syndrome suffered rapid decompensation. echocardiography showed mitral valve vegetations with severe regurgitation. blood cultures were negative, but antinuclear antibody test and lupus anticoagulant were positive. The patient died of massive cerebral infarction and brainstem herniation. autopsy found a patent foramen ovale and Libman-Sacks endocarditis. CONCLUSION: With rapid decompensation of acute respiratory distress syndrome in pregnancy, despite aggressive medical therapy, complicating processes must be considered, especially with antiphospholipid antibodies, which can be associated with sterile heart vegetations and subsequent fatal thromboembolism.
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ranking = 0.28571428571429
keywords = pregnancy
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8/116. ovulation induction and early pregnancy loss in a woman susceptible to autoimmune diseases: a possible interrelationship.

    We report a 34-year-old woman with sequentially occurring autoimmune diseases that are possibly triggered by numerous ovulation inductions. At the ages of 26-32 years, she experienced 27 uncontrolled ovulation induction cycles using clomiphene citrate (CC) or CC plus human menopausal gonadotropin plus human chorionic gonadotropin. She became pregnant at the ages of 27, 30 and 31 with subsequent pregnancy loss in the 28th, 8th and 10th week of gestation, respectively. insulin-dependent diabetes mellitus (IDDM) developed at the age of 28. During the second year of ovulation induction, at the age of 27, she developed arthralgia that worsened and became migratory from the age of 31. thrombocytopenia appeared at the age of 33. The diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) was established at the age of 34. To the best of our knowledge, this is the first case of concurrent IDDM, SLE and APS in a patient associated with ovulation inductions. Excessive levels of estradiol achieved during the ovulation inductions could play a role in the expression of multiple autoimmune diseases in the susceptible woman.
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ranking = 0.74756040323367
keywords = pregnancy, gestation
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9/116. Mitral valve plasty in systemic lupus erythematosus in the setting of antiphospholipid syndrome.

    The first case was of a 27-year-old female, who was diagnosed as having mitral valve stenosis with regurgitation, systemic lupus erythematosus and antiphospholipid syndrome at her previous pregnancy. We performed mitral valve plasty, which included open mitral commissurotomy and Kay's annulo plasty. The second case was of a 53-year-old female, who was diagnosed as old myocardial infarction, mitral regurgitation, systemic lupus erythematosus and antiphospholipid syndrome. She underwent mitral valve plasty and coronary artery bypass grafting. Both cases were treated by administration of methylpredonisolone and heparin perioperatively to avoid thrombosis and aggravation of systemic lupus erythematosus. Both patients showed good postoperative outcome without complications. We consider that it is important to perform the plasty as far as possible, and to administer effective anticoagulation treatment to prevent complications for patients in the setting of systemic lupus erythematosus and antiphospholipid syndrome.
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ranking = 0.14285714285714
keywords = pregnancy
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10/116. A case report on the perinatal management of a 30-week preterm baby with congenital complete heart block.

    INTRODUCTION: Congenital complete heart block is an uncommon condition in the newborn, but is known to occur with maternal systemic lupus erythematosus. CLINICAL PICTURE: This paper presents one such baby with complete heart block who was born premature (after a gestation of 30 weeks) and weighing 759 g. TREATMENT: Continuous isoprnaline infusion was initially used to support the baby while her other neonatal problems were treated. A Medtronics VV1 pacemaker was subsequently inserted to maintain a heart rate that would be more physiologically acceptable for the patient. OUTCOME: This baby is currently thriving well, having been followed up for one year. CONCLUSIONS: The management issues, encompassing maternal and neonatal problems, and a review of current literature on this condition are discussed.
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ranking = 0.03327468894796
keywords = gestation
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