Acro-cardio-facial syndrome

Acro-cardio-facial syndrome (ACFS) is a rare genetic disorder characterized by split-hand/split-foot malformation (SHFM), facial anomalies, cleft lip/palate, congenital heart defect (CHD), genital anomalies, and mental retardation. Up to now, 9 patients have been described, and most of the reported cases were not surviving the first days or months of age. The spectrum of defects occurring in ACFS is wide, and both interindividual variability and clinical differences among sibs have been reported. The diagnosis is based on clinical criteria, since the genetic mechanism underlying ACFS is still unknown. The differential diagnosis includes other disorders with ectrodactyly, and clefting conditions associated with genital anomalies and heart defects. An autosomal recessive pattern of inheritance has been suggested, based on parental consanguinity and disease's recurrence in sibs in some families. The more appropriate recurrence risk of transmitting the disease for the parents of an affected child seems to be up to one in four. Management of affected patients includes treatment of cardiac, respiratory, and feeding problems by neonatal pediatricians and other specialists. Prognosis of ACFS is poor

Methemoglobinemia presenting in a circumcised baby following application of prilocaine: a case report

<p>Abstract</p> <p>Introduction</p> <p>Local anesthesia with prilocaine has become a routine part of ambulatory circumcision procedures. Methemoglobinemia is a rare but potentially lethal complication of local anesthetics.</p> <p>Case presentation</p> <p>We report the case of a 40-day-old Turkish boy who presented with cyanosis after receiving local anesthesia with prilocaine. His methemoglobin level revealed severe methemoglobinemia (methemoglobin = 44%). His cyanosis resolved after intravenous administration of methylene blue.</p> <p>Conclusion</p> <p>Although the association between prilocaine use and methemoglobinemia has generally restricted the use of prilocaine in babies, it is still widely used in ambulatory procedures, especially during circumcision in the neonatal period. Prilocaine should not be used in babies who are less than 3 months old because of the risk of methemoglobinemia; other local anesthetics may be used for this age group. Furthermore, general anesthesia by mask ventilation may be favored for babies less than 3 months of age instead of local anesthetics.</p

La situation des réfugiés espagnols en Poitou-Charentes

Malgré les drames vécus dans nombre de régions françaises, la situation des réfugiés de la guerre d'Espagne fut moins tragique dans certains contextes. Ce fut le cas, notamment, en Poitou-Charentes, où les autorités et la population locales ont satisfait, au moins partiellement, à leur devoir d'humanité.Sivasli Nermin. La situation des réfugiés espagnols en Poitou-Charentes. In: Hommes et Migrations, n°1249, Mai-juin 2004. Médiations et travail social. pp. 127-133

La gestion des connaissancesdans un guide qualité des projetspilotes de systèmes d\u27informationâ France Télécom

Dans un contexte de plus en plus turbulent, gérer les ressources classiques de l\u27entrepri-se ne suffit plus. Il faut également gérer les savoirs distribués dans l\u27organisation. Notre tra-vail a pour but de présenter une démarche pragmatique de construction et de co-construc-tion d\u27outils de gestion. Nous examinons le processus majeur de gestion des connaissancesau travers de la conception, de la réalisation et du déploiement d\u27un Guide Qualité de laMise en Place Pilote et de ses outils types. Ce papier relate notre expérience menée au seinde la Division Système d\u27Information de France Télécom sur la période courant de 1995 à2000. Nous répondons aux questions du comment acquérir, organiser, capitaliser et pro-pager les connaissances afin de soutenir la stratégie de mémoire d\u27une organisationconfrontée à la concurrence et qui vit des mutations organisationnelles importantes

La gestion des connaissances dans un guide qualité des projets pilotes de systèmes d'information à France Télécom

In a turbulent context, managing classical resources of the organization is not sufficient. The knowledge distributed into the organization require to be managed. Our work consist to present a pragmatic approach in constructing management tools in collaboration with key people of the organization. We examine the major process of knowledge management by designing, constructing and diffusing a quality guide with deliverable templates for pilot implementation. This paper recounts our experience in the Information Systems Division of France T&eacute;l&eacute;com from 1995 to 2000. How to acquire, organize, capitalize and spread knowledge of an organization that is confronted to competition and is living important organizational changes? We address this question in the following article.Dans un contexte de plus en plus turbulent, g&eacute;rer les ressources classiques de l'entreprise ne suffit plus. Il faut &eacute;galement g&eacute;rer les savoirs distribu&eacute;s dans l'organisation. Notre travail a pour but de pr&eacute;senter une d&eacute;marche pragmatique de construction et de co-construction d'outils de gestion. Nous examinons le processus majeur de gestion des connaissances au travers de la conception, de la r&eacute;alisation et du d&eacute;ploiement d'un Guide Qualit&eacute; de la Mise en Place Pilote et de ses outils types. Ce papier relate notre exp&eacute;rience men&eacute;e au sein de la Division Syst&egrave;me d'Information de France T&eacute;l&eacute;com sur la p&eacute;riode courant de 1995 &agrave; 2000. Nous r&eacute;pondons aux questions du comment acqu&eacute;rir, organiser, capitaliser et propager les connaissances afin de soutenir la strat&eacute;gie de m&eacute;moire d'une organisation confront&eacute;e &agrave; la concurrence et qui vit des mutations organisationnelles importantes

Which Local Anesthesia Should Be Used In Neonatal Circumcision In Newborns?

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Spontaneous Neonatal Chylothorax Treated With Octreotide In Turkey: A Case Report

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in Gaziantep urban area, Turkey

Thalassemia is one of the most common hereditary disorders in the Mediterranean region and studies have shown that the prevalence of beta-thalassemia trait is high in the southern part of Turkey. Gaziantep is a city located near this region and, therefore, the authors investigated the prevalence and hematological characteristics of the beta-thalassemia traits in primary school students in Gaziantep. Sixty primary schools were selected from a list of all primary schools using a systematic sampling method. Data were collected by a face-to-face questionnaire. Osmotic fragility testing (OFT) using single-tube 0.36% NaCl solution was used for the screening of beta-thalassemia. Students who were positive in regard to OFT went through a series of testing, including a complete blood count, serum ferritin levels, serum iron, and hemoglobin electroforesis. Chi-square test was used in statistical analysis. Of the 2439 students enrolled to the study from the selected 60 classrooms, 1353 (55.5%) were male and 1086 (44.5%) were female. The OFT was positive in 115 (4.7%) of the participants. CEA and confirmatory HPLC results of the students who were positive OFT indicated that 70 (60.8%) had normal results, 33(28.7%) showed high HbA2 levels, 7 (6.1%) showed high HbA2 and HbF levels, 5(5.2%) showed high HbA2 and Fe-deficiency anemia, and none showed increased HbF levels. The overall prevalence of beta-thalassemia trait was 1.84%. No gender differentials and highest rates among the Kahramanmaras (3.5%) and Sanliurfa (1.7%) born students were the other significant findings of this study. Implementation of a routine carrier-screening program offering genetic counseling, prenatal diagnosis, and selective termination of affected fetuses would be a wise approach to eliminate this disease from the region