Prediction of Adverse Pregnancy Outcomes in Women with Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease associated with major obstetrical complications such as gestational loss, preterm delivery, fetal growth restriction (FGR) and preeclampsia. Published literature is not consensual regarding the main risk factors for each of these outcomes. Our goal with this study was to determine the most important predictors for each of the main adverse pregnancy outcomes in this population. We conducted a retrospective cohort study of unifetal pregnancies of women with the diagnosis of SLE followed in our unit between January 1994 and December 2016. We excluded elective terminations of pregnancy and cases lost to follow-up and we analyzed 157 pregnancies (128 women). Multiple logistic regression models for the outcomes gestational loss, preterm delivery, fetal growth restriction, and preeclampsia were built. Two-sided p-values of < 0.05 were used to determine statistical significance, and two-sided confidence intervals of 95% are reported. In our cohort, the main risk factors for gestational loss were maternal age and the presence of antiphospholipid antibodies. Lupic nephritis was predictive of a preterm delivery and preeclampsia. Renal involvement and lupus flares during pregnancy were risk factors for FGR. Overall, the main risk factor for an adverse pregnancy outcome were lupus flares during pregnancy. Despite optimal pregnancy monitoring, women with SLE are still at risk for adverse pregnancy outcomes. Risk stratification for each of these outcomes is crucial for an effective counselling and tailored monitoring.

Lipschütz Genital Ulceration as Initial Manifestation of Primary Sjögren's Syndrome.

Genital ulcers are challenging to any clinician and causes transcend many specialties. Skin ulceration in patients with primary Sjögren's syndrome is infrequent but an established feature of cutaneous involvement. Although gynecological symptoms, such as vulvovaginal dryness, dyspareunia, and pruritus, are common in women with primary Sjögren's syndrome, patients affected by vulvar ulcers are unknown. We describe an exceptional case of necrotic aphthous-type vulvar ulceration as initial presentation of primary Sjögren's syndrome that was possibly triggered by an infectious agent. Successful healing was achieved with oral corticosteroids, despite some loss of labia minora and labia majora as sequelae of the necrotizing process. Reactive acute genital ulcers (Lipschütz ulcers) should be considered as a possible manifestation of many autoimmune/inflammatory disorders, beyond the classic associations such as Behçet's syndrome or Crohn's disease.

Carcinoma renal cromófobo: uma causa rara de hipertensão na gravidez / Chromophobe renal cell carcinoma: a rare cause of hypertension in pregnancy

O carcinoma de células renais, em particular o tipo cromófobo, é uma patologia rara na idade fértil, o que explica a pouca informação disponível na literatura. Os autores apresentam o caso de uma mulher de 37 anos, multípara, com hipertensão arterial de novo, de difícil controle e hematúria às 10 semanas de gravidez, tendo o estudo etiológico do quadro hipertensivo demostrado a existência de um tumor renal. Às 17 semanas de gravidez, a doente foi submetida à nefrectomia esquerda, tendo a cirurgia decorrido sem intercorrências. A histologia demostrou tratar-se de um carcinoma do subtipo cromófobo. O restante da gravidez decorreu sem complicações, com perfil tensional controlado, com um parto vaginal de termo com um recém-nascido saudável.

Renal cell carcinoma, particularly the chromophobe type, is a rare pathology in childbearing age, and consequently, in literature, few cases during pregnancy have been reported. The authors present the case of a 37-year-old, multiparous woman, with de novo high blood pressure of difficult control, and hematuria at 10-week gestation. The etiological study of the hypertensive disorder has demonstrated the existence of a renal tumor. The patient underwent left radical nephrectomy at 17-week gestation. Histology was compatible with chromophobe subtype renal cell carcinoma. The remaining pregnancy period progressed with no complications, with controlled tension profile, and resulted in spontaneous vaginal delivery of a healthy infant at term.

Ten Years' Experience of Pregnancy Outcomes in Women with Cardiac Valvulopathies: Are Valve Prostheses Worst?

BACKGROUND AND AIM OF THE STUDY: The population of pregnant women with valvular heart disease (VHD), and in particular with valvular heart prostheses (VHPs), represents a unique patient group where data are scarce, and where there is an increased risk for adverse maternal and obstetric events. The study aim was to assess the experience of a tertiary center with regards to cardiac and pregnancy outcomes in women with VHD, comparing VHPs with other VHD pathologies. METHODS: A retrospective analysis of 84 pregnancies in women with VHD (mean age 27.5 ± 5.5 years) was carried out over a 10-year period. Twenty-three pregnancies with VHPs (group A) and 61 with other VHD pathologies (group B) were identified and their cardiac, obstetric, and neonatal outcomes evaluated. RESULTS: At the start of pregnancy, group A included more patients with an impaired left ventricular ejection fraction (LVEF) (15.8% versus 3.9%, p = 0.014), with a previous history of cardiac medication (82.6% versus 29.5%, p = 0.000), and with arrhythmic or ischemic events (18.2% versus 4.9%, p = 0.076). A deterioration in NYHA functional class was the most common cardiac complication (8.3%), and in 7.1% of patients it was necessary to initiate some form of cardiac medication. No maternal deaths were recorded. Group A presented significantly more hemorrhagic and thrombotic complications; all of these events were in women receiving low-molecular-weight heparin. There were 95.5% live births, with a medium birth weight of 3068 ± 498 g. In the VHP group there was also a higher incidence of spontaneous abortion (26.1 versus 3.3, p = 0.005), newborns small for gestational age (30.0 versus 0.4, p = 0.07) and mean Apgar score < 7 (16.7 versus 0.0, p = 0.031). Warfarin embryopathy was observed in one case. CONCLUSION: With the multidisciplinary care provided, pregnancy was relatively well tolerated and successful. However, the presence of a VHP remains a challenging condition that is associated with elevated maternal and fetal morbidity. A worse baseline cardiac status of the mother, as well as anticoagulation issues, were determinants for these findings.

Inferior Vena Cava Filter Placement during Pregnancy: An Adjuvant Option When Medical Therapy Fails.

The authors present a case of a 27-year-old multiparous woman, with multiple thrombophilia, whose pregnancy was complicated with deep venous thrombosis requiring placement of a vena cava filter. At 15th week of gestation, following an acute deep venous thrombosis of the right inferior limb, anticoagulant therapy with low-molecular-weight heparin (LMWH) was instituted without improvement in her clinical status. Subsequently, at 18 weeks of pregnancy, LMWH was switched to warfarin. At 30th week of gestation, the maintenance of high thrombotic risk was the premise for placement of an inferior vena cava filter for prophylaxis of pulmonary embolism during childbirth and postpartum. There were no complications and a vaginal delivery was accomplished at 37 weeks of gestation. Venal placement of inferior vena cava filters is an attractive option as prophylaxis for pulmonary embolism during pregnancy.

Acute Sheehan's syndrome presenting as central diabetes insipidus.

Sheehan's syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpartum haemorrhage. Improvements in obstetrical care have significantly reduced its incidence in developed countries, but postpartum pituitary infarction remains a common cause of hypopituitarism in developing countries. We report a case of severe postpartum haemorrhage followed by headache, central diabetes insipidus and failure to lactate, which prompted us to investigate and identify both anterior and posterior pituitary deficiency compatible with Sheehan's syndrome. A timely diagnosis allowed us to implement an adequate treatment and follow-up plan, which are known to improve clinical status and patient outcome.

[Renal oncocytoma in pregnancy--an unusual presentation of secondary hypertension]. / Oncocitoma renal na gravidez--uma forma invulgar de hipertensão secundária.

INTRODUCTION: Renal oncocytoma accounts for 5-7% of primary renal neoplasms. It is usually, diagnosed in asymptomatic patients and is characterized by a benign behavior without invasion of adjacent tissues or metastasis. Diagnosis during pregnancy is uncommon and to date there have been only a few cases reported in the literature. CASE REPORT: The authors present the case of a 32-year-old nulliparous woman with uncontrolled hypertension diagnosed at seven weeks gestation. She was referred to our institution at 24 weeks with superimposed pre-eclampsia complicated by acute pulmonary edema and hemodynamic instability requiring mechanical ventilatory support, fetal growth restriction and stillbirth. Etiological study of the hypertensive disorder performed in the postpartum period was consistent with renal oncocytoma. CONCLUSION: The clinical behavior of renal oncocytoma remains poorly characterized during pregnancy and may lead to an adverse maternal and fetal outcome despite its theoretically benign behavior. It is essential to exclude a possible secondary cause of hypertension in cases that are difficult to control.

[Warfarin hipersensitivity: how to deal with it?]. / Hipersensibilidade à varfarina: que abordagem?

The authors report a clinical case of warfarin hypersensitivity noticed when a patient, with thromboembolic risk, was submitted to warfarin. She was found to be heterozigotic to -1639G>A (gene VKORC1) and homozygotic to 1075A>C (genotype CYP2C9*3/*3). In this article the warfarin pharmacodinamics and its role over Vitamin K is revisited. It includes some suggested indications for investigating genetic polymorphisms, in order to avoid complications associated with warfarin.

Lupus and pregnancy--15 years of experience in a tertiary center.

This retrospective study was designed to evaluate the outcome of pregnancies in women diagnosed with systemic lupus erythematosus (SLE) followed in a tertiary fetal-maternal center. Data were collected from clinical charts between January 1993 and December 2007, with a total of 136 pregnancies (107 patients). Mean maternal age was 29 years, with the vast majority of patients being Caucasian. Most patients were in remission 6 months prior to pregnancy (93%) and the most frequently affected organs were the skin and joints. Renal lupus accounted for 14% of all cases. Twenty-nine percent of patients were positive for at least one antiphospholid antibody (aPL) and nearly 50% had positive SSa/SSb antibodies. All patients with positive aPL received low-dosage aspirin and low-molecular-weight heparin (LMWH). There were no pregnancy complications in more than 50% of cases and hypertensive disease and intrauterine growth restriction were the most common adverse events. There were 125 live births, one neonatal death, eight miscarriages, and three medical terminations of pregnancy. Preterm delivery occurred in 25% of pregnancies. Our results are probably the conjoined result of a multidisciplinary approach together with a systematic management of SLE pregnancies, with most patients keeping their prior SLE medication combined with low-dosage aspirin and LMWH in the presence of aPL.

Primary antiphospholipid syndrome: pregnancy outcome in a portuguese population.

INTRODUCTION: Women with antiphospholipid syndrome (APS) may suffer from recurrent miscarriage, fetal death, fetal growth restriction (FGR), pre-eclampsia, placental abruption, premature delivery and thrombosis. Treatment with aspirin and low molecular weight heparin (LMWH) combined with close maternal-fetal surveillance can change these outcomes. OBJECTIVE: To assess maternal and perinatal outcome in a cohort of Portuguese women with primary APS. PATIENTS AND METHODS: A retrospective analysis of 51 women with primary APS followed in our institution (January 1994 to December 2007). Forty one (80.4%) had past pregnancy morbidity and 35.3% (n=18) suffered previous thrombotic events. In their past they had a total of 116 pregnancies of which only 13.79 % resulted in live births. Forty four patients had positive anticardiolipin antibodies and 33 lupus anticoagulant. All women received treatment with low dose aspirin and LMWH. RESULTS: There were a total of 67 gestations (66 single and one multiple). The live birth rate was 85.1% (57/67) with 10 pregnancy failures: seven in the first and second trimesters, one late fetal death and two medical terminations of pregnancy (one APS related). Mean (+/- SD) birth weight was 2837 +/- 812 g and mean gestational age 37 +/- 3.3 weeks. There were nine cases of FGR and 13 hypertensive complications (4 HELLP syndromes). 54.4% of the patients delivered by caesarean section. CONCLUSIONS: In our cohort, early treatment with aspirin and LMWH combined with close maternal--fetal surveillance was associated with a very high chance of a live newborn.

Acute myocardial infarction in the puerperium.

The authors describe the case of a forty-two-year-old patient with acute myocardial infarction (MI) on the third day of puerperium, who underwent direct angioplasty. The authors review the main physiological cardiovascular and hemostatic changes in pregnancy, which are usually well tolerated but which increase the probability of cardiovascular events when risk factors or previous cardiac disease are associated. Blood volume and cardiac output increase by around 50% during pregnancy, hemoglobin concentration falls, and reduced peripheral vascular resistance leads to a fall in arterial blood pressure during the first half of pregnancy. End-diastolic pressure, stroke volume and cardiac output increase in the immediate puerperium, and cardiovascular risk is highest in the perinatal period. The incidence of MI during pregnancy and puerperium is low; however, it is on the increase due to the growing number of pregnancies in women close to the end of their reproductive age.

[Kidney abscess during pregnancy. A case report]. / Abcesso renal na gravidez.

The development of a kidney abscess is extremely rare despite the fact that urinary tract infections are quite common during pregnancy. The pathogenesis of renal abscess has changed over the years. Nowadays, more than 75% of the renal abscess arises from a previous urinary tract infection. The authors report the case of a 35 year-old woman, pregnant, with fever, nausea, vomits, right lumbar tenderness, admitted in the urgency room. Clinical examination and complementary diagnostic exams led to the diagnosis of renal abscess. It's made a review of the literature about the subject.