International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts.

BACKGROUND Congenital portosystemic shunts (CPSS) are rare vascular malformations associated with the risk of life-threatening systemic conditions, which remain underdiagnosed and often are identified after considerable diagnostic delay. CPSS are characterized by multiple signs and symptoms, often masquerading as other conditions, progressing over time if the shunt remains patent. Which patients will benefit from shunt closure remains to be clarified, as does the timing and method of closure. In addition, the etiology and pathophysiology of CPSS are both unknowns. This rare disorder needs the strength of numbers to answer these questions, which is the purpose of the international registry of CPSS (IRCPSS). METHOD A retrospective and prospective registry was designed using secuTrial® by the ISO certified Clinical Research Unit. Given that a significant number of cases entered in the registry are retrospective, participants have the opportunity to use a semi-structured minimal or complete data set to facilitate data entry. In addition, the design allows subjects to be entered into the IRCPSS according to clinically relevant events. Emphasis is on longitudinal follow-up of signs and symptoms, which is paramount to garner clinically relevant information to eventually orient patient management. The IRCPSS includes also three specific forms to capture essential radiological, surgical, and cardiopulmonary data as many times as relevant, which are completed by the specialists themselves. Finally, connecting the clinical data registry with a safe image repository, using state-of-the-art pseudonymization software, was another major focus of development. Data quality and stewardship is ensured by a steering committee. All centers participating in the IRCPSS have signed a memorandum of understanding and obtained their own ethical approval. CONCLUSION Through state-of-the-art management of data and imaging, we have developed a practical, user-friendly, international registry to study CPSS in neonates, children, and adults. Via this multicenter and international effort, we will be ready to answer meaningful and urgent questions regarding the management of patients with CPSS, a condition often ridden with significant diagnostic delay contributing to a severe clinical course

International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations associated with the risk of life-threatening systemic conditions, which remain underdiagnosed and often are identified after considerable diagnostic delay. CPSS are characterized by multiple signs and symptoms, often masquerading as other conditions, progressing over time if the shunt remains patent. Which patients will benefit from shunt closure remains to be clarified, as does the timing and method of closure. In addition, the etiology and pathophysiology of CPSS are both unknowns. This rare disorder needs the strength of numbers to answer these questions, which is the purpose of the international registry of CPSS (IRCPSS). METHOD: A retrospective and prospective registry was designed using secuTrial® by the ISO certified Clinical Research Unit. Given that a significant number of cases entered in the registry are retrospective, participants have the opportunity to use a semi-structured minimal or complete data set to facilitate data entry. In addition, the design allows subjects to be entered into the IRCPSS according to clinically relevant events. Emphasis is on longitudinal follow-up of signs and symptoms, which is paramount to garner clinically relevant information to eventually orient patient management. The IRCPSS includes also three specific forms to capture essential radiological, surgical, and cardiopulmonary data as many times as relevant, which are completed by the specialists themselves. Finally, connecting the clinical data registry with a safe image repository, using state-of-the-art pseudonymization software, was another major focus of development. Data quality and stewardship is ensured by a steering committee. All centers participating in the IRCPSS have signed a memorandum of understanding and obtained their own ethical approval. CONCLUSION: Through state-of-the-art management of data and imaging, we have developed a practical, user-friendly, international registry to study CPSS in neonates, children, and adults. Via this multicenter and international effort, we will be ready to answer meaningful and urgent questions regarding the management of patients with CPSS, a condition often ridden with significant diagnostic delay contributing to a severe clinical course

Evaluation de la pratique de l'amniocentèse sur le réseau Sud-Ouest francilien pendant l'année 2003

L'objectif est d'évaluer la pratique de l amniocentèse sur un réseau pendant une année. Pour cela, notre étude a été rétrospective multicentrique sur le réseau sud-ouest francilien. Elle a inclu 2400 patientes ayant eu une amniocentèse, du 1er janvier 2003 au 31 décembre 2003. 170 patientes (7,5%), dont nous n avions pas les issues de grossesse, ont été classées perdues de vue. Finalement, le taux d amniocentèses a été de 9,5%. L indication la plus fréquente était un dépistage par marqueurs sériques maternels positif (44%). 4,12% d anomalies chromosomiques ont été décelées, la plus fréquente étant la trisomie 21. Les issues de grossesses sont marquées par un taux de pertes fœtales (FCS et MIU) global de 1,4%. Après exclusion des fœtus porteurs d une aneuploïdie ou d une pathologie létale, un taux de pertes fœtales (MIU et FCS) potentiellement imputables à l amniocentèse a été évalué à 1,21%. Le taux de ruptures prématurées des membranes a été de 1,12% et la prématurité a été 6,5% des naissances vivantes. En définitive, notre étude a révélé une variété des pratiques de l amniocentèse au sein du réseau. Au global, celle-ci induit potentiellement 1,21 % de pertes fœtales (FCS et MIU). Les tests de dépistage sont actuellement utilisés de manière séquentielle ce qui aboutit à une inflation du nombre d amniocentèses et donc du nombre de pertes de fœtus à priori sains. Avec l utilisation d un dépistage combiné, la fréquence des amniocentèses pourrait être diminuée sans que cela ne diminue le taux de détection des anomalies chromosomiques. Le taux de rupture prématurée des membranes et de prématurité ne semblent pas augmentés par la pratique de l amniocentèse.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Le dépistage de la trisomie 21 et ses conséquences

LE KREMLIN-B.- PARIS 11-BU Méd (940432101) / SudocPARIS-BIUP (751062107) / SudocSudocFranceF

Evaluation de la pertinence du diagnostic échographique dans une maternité de niveau III (Antoine Béclère)

LE KREMLIN-B.- PARIS 11-BU Méd (940432101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

[Progesterone and preterm delivery: Back to the future?].

International audienceProgesterone was widely used in France during the 1980s and 1990s to prevent preterm birth until some published cases of cholestasis suddenly stopped its prescription. Since then, multiple randomized controlled trials have emerged and demonstrated the efficiency of the treatment but also its safety at low doses. In order to clarify its indications, we performed a current literature review. We analyzed literature data according to different categories of risk and different routes of administration. Results confirm that progesterone is an efficient treatment to prevent preterm birth in singleton gestation with short cervical length, and in singleton gestation with prior preterm birth with or without short cervical length. Apart from these indications, progesterone, especially 17-alpha-hydroxyprogesterone (17OHP), should not be used outside research protocols

[Could we perform quality second trimester ultrasound among obese pregnant women?].

International audienceOBJECTIVE: To compare the quality of second trimester ultrasound images and their anatomical quality scores among obese women and those with a normal body mass index (BMI). MATERIALS AND METHODS: This prospective study, which took place from 2009 to 2011, included every obese pregnant woman (prepregnancy BMI greater than 30 kg/m(2)) who had an ultrasound examination at 20 to 24 weeks in our hospital and a control group with a normal BMI (20-24.9kg/m(2)) who had the same examination. A single operator evaluated the quality of all images, reviewing the standardized ultrasound planes - three biometric and six anatomical - required by French guidelines and scoring the quality of the six anatomical images. Each image was assessed according to 4-6 criteria, each worth one point. We sought excellent quality, defined as the frequency of maximum points for a given image. RESULTS: The obese group included 223 women and the control group 60. The completion rate for each image was at least 95 % in the control group and 90 % in the obese group, except for diaphragm and right outflow tract images. Overall, the excellence rate varied from 35 % to 92 % in the normal BMI group and 18 % to 58 % in the obese group and was significantly lower in the latter for all images except abdominal circumference (P=0.26) and the spine (P=0.06). Anatomical quality scores were also significantly lower in the obese group (22.3 vs. 27.2 ; P=0.001). CONCLUSION: Image quality and global anatomical scores in second trimester ultrasound scans were significantly lower among obese than normal-weight women