Monochorionic-diamniotic twin pregnancy complicated by spontaneous septostomy and cord entanglement. a systematic review, evaluation of complication rates and presentation of an additional case

Background: Our purpose is to describe the ultrasound findings, both with bi-dimensional and three-dimensional imaging, suggestive of spontaneous septostomy in monochorionic-diamniotic twin pregnancies. Methods: PubMed, Medline and reference lists were searched using “Spontaneous septostomy and twin pregnancy” as keywords. Seventeen articles reporting a total of 25 cases, adding our own, were included in the systematic review. Only English full text articles, the main purpose of which was to describe spontaneous septostomy in twin pregnancies, were included. Results: In our sample the major ultrasound sign arousing suspicion of spontaneous septostomy was found to be an absent or disrupted inter-twin membrane (79% of cases). Twins close to each-other were described in 33% of cases, while cord entanglement was suspected only in 27% of cases. We reported a lower antenatal detection of entanglement when compared with intrapartum evaluation (27% vs 59%). Adverse fetal outcomes occurred in 12% of cases, while 88% of cases were born alive. Conclusions: Spontaneous septostomy represents a diagnostic and clinical challenge for obstetrics providers. Clinicians must focus on ultrasound findings to close surveil fetal wellness and reduce both fetal and neonatal impairment

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Early Systemic Sclerosis: Marker Autoantibody Positive Patients Have A Faster Pace Of The Disease.

Early Systemic Sclerosis: Marker Autoantibody Positive Patients Have A Faster Pace Of The Disease. Background/Purpose: To investigate whether patients affected with any of the 3 subsets of early systemic sclerosis (SSc) i.e. Raynaud’s Phenomenon (RP) with SSc marker autoantibody (ACA or anti-Scl70 or anti-RNA polymerase III or anti-fibrillarin or anti-Th/To) and typical capillaroscopic findings (megacapillaries and/or avascular areas) (subset I); or autoantibody positive only (subset II); or capillaroscopy positive only (subset III) (1) and unsatisfying the 2012 ACR/EULAR classification criteria for SSc (2) at admission differ in the lag time to satisfy the new SSc classification criteria. Methods: Early SSc patients consecutively admitted to a Rheumatology and an Angiology center and unsatisfying the 2012 ACR/EULAR classification criteria for SSc at admission, were subdivided into the 3 above referred subsets and followed-up for 7–101 months (median 45). S288 Sunday, October 27 They were re-evaluated six-monthly by history, clinical examination, B-mode echoDopplercardiography and Lung functional study including DLCO evaluation and yearly by lung HRCT to assess whether and when each of them satisfied new ACR/EULAR classification criteria i.e. developed a disease score 9 (2). Results: During the follow-up, 11 out of 21 subset I patients (52.3%) (baseline score 8); 10 out of 15 subset II patients (66.6%) (baseline score 6) and 0 out of 24 subset III patients (baseline score 5–7) satisfied the criteria; the difference being significant between each of the 2 autoantibody positive (subsets I and II) and the capillaroscopic positive-autoantibody negative subset (subset I versus III: X2 by log rank test17.45, p0.0001; subset II versus III: X2 11.04, p0.0009), no difference being detected between the 2 autoantibody positive subsets (X2 0.55, p0.454). The 11 subset I patients satisfied the criteria because of the development of teleangectasias in 5 cases; puffy fingers in 3 cases; lung fibrosis in 2 cases; digital ulcers in 1 case. The 10 subset II patients did it because of the development of at least 2 of the following manifestations: scleroderma capillaroscopic pattern in 5 cases, teleangectasias in 5 cases, puffy fingers in 5 cases, digital ulcers in 3 cases, pulmonary hypertension in 1 case and lung fibrosis in 1 case. Despite the unfulfillment of the criteria, among the 24 subset III patients, 21 of whom already presented puffy fingers at baseline, 2 developed telangectasias, 1 digital ulcers, 4 a DLCO 80%. Conclusion: We have recently pointed out that autoantibody positive early SSc patients differ from subset III patients in the pattern of activation markers (increased serum concentration of procollagen I carbossipropeptide versus increased serum concentration of E-selectin) and preclinical internal organ involvement (higher prevalence of decreased DLCO) (3). Here we point out that autoantibody positive patients present a faster pace of the disease. References: 1) Koenig M et al. Arthritis Rheum. 2008;58:3902–12 2) Van den Hoogen F et al. Eular Congress 2013, OP0033 3) Valentini G et al. Arthritis Res Ther. 2013; 29;15:R63 Disclos

Chronic abdominal pain associated with intermittent compression of the celiac artery

Recurrent abdominal pain (RAP), surely one of the most frequent causes of medical intervention, is frequently present in many gastrointestinal disease. Usually no structural and/or biochemical alterations can be demonstrated. This condition is, therefore, considered to be due to functional disorders such as irritable bowel syndrome (IBS) or functional dyspepsia. Previous observations suggest the presence of a rare alteration of celiac vessels among the possible causes of RAP. This pathological condition was known as Dunbar syndrome. We report 2 cases of chronic abdominal pain. The former reported weight loss and the latter anemia with iron deficiency. It is remarkable that patients with initial diagnosis of IBS can be affected by celiac disease (CD), which is the cause of their abdominal pain. Our patients were tested for CD; the former was negative and IBS was diagnosed, the latter was positive and a gluten free diet was prescribed. The presence of an epigastric bruit, accentuated during expiration, suggested a possible vascular alteration known as tripod celiac artery compression syndrome. Duplex Doppler sonography suggests the diagnosis of celiac arterial constriction due the diaphragmatic ligament. These cases show that tripod celiac artery compression syndrome might be a cause of RAP and that it may be evaluated and investigated when the clinical examination discloses an abdominal systolic bruit

Vascular Diseases in Women: Do Women Suffer from Them Differently?

According to the World Health Organization, cardiovascular disease (CVD) is the leading cause of death among women worldwide, yet its magnitude is often underestimated. Biological and gender differences affect health, diagnosis, and healthcare in numerous ways. The lack of sex and gender awareness in health research and healthcare is an ongoing issue that affects not only research but also treatment and outcomes. The importance of recognizing the impacts of both sex and gender on health and of knowing the differences between the two in healthcare is beginning to gain ground. There is more appreciation of the roles that biological differences (sex) and sociocultural power structures (gender) have, and both sex and gender affect health behavior, the development of diseases, their diagnosis, management, and the long-term effects of an illness. An important issue is the knowledge and awareness of women about vascular diseases. The risk of cardiovascular events is drastically underestimated by women themselves, as well as by those around them. The purpose of this review is to draw attention to improving the medical care and treatment of women with vascular diseases