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Neural Tube Defects are a group of related but individual pathologies that are the most common congenital anomalies of the Central Nervous System. The importance of this condition has generated a large body of literature that seldom was written for the clinician in a comprehensive and didactic manner. In this book, each chapter is richly illustrated and dedicated to one disease with subheadings that encompass etiology, molecular biology, clinical anatomy, symptoms, rationale for surgical procedures, natural history and transition into adulthood.
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Neural Tube Defects --- Neural Tube Defects --- etiology --- prevention & control
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Neural tube --- Abnormalities --- Prevention. --- Embryology --- Nervous system
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alpha-Fetoproteins --- Neural Tube Defects --- analysis --- Netherlands.
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The book Neural Tube Defects - Role of Folate, Prevention Strategies and Genetics has several eminent international authors and the book is a resource for anybody who is interested in this very important subject. The authors are distinguished and the chapters are a product of their extensive research.
Neural tube --- Abnormalities. --- Dysraphia --- Dysraphic states --- Dysraphism --- Dysrhaphia --- Dysrhaphism --- Neural tube defects --- Status dysraphicus --- Clinical psychology
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Abnormalities, Human --- Neural Tube Defects --- Neural Tube Defects --- prevention & control --- therapy
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This retrospective study assesses the outcome after intensive chemotherapy followed by stem cell transplantation compared to conventional chemotherapy in patients suffering from acute myeloid leukemia or myelodysplastic syndrome with poor-risk karyotype, i.e. monosomy 5 or 7 and/or deletion of the long arm of chromosome 5 or 7.
We compare 3 groups of patients: the first contains 16 patients with poor-risk karyotype who were treated with stem cell transplantation. The second contains 11 patients with poor-risk karyotype treated with conventional chemotherapy. The third group contains 14 patients with intermediate risk (normal) karyotype treated with stem cell transplantation.
The results of intensive chemotherapy followed by stem cell transplantation are compared to those of conventional chemotherapy in terms of response and survival (overall and disease-free) in patients with poor-risk karyotype : the remission rate is significantly higher in the transplantation group. The relapse rate, the overall survival and the disease-free survival are not different between the 2 groups.
The results of intensive chemotherapy followed by stem cell transplantation are compared in terms of response and survival (overall and disease-free) in patients with poor-risk vs intermediate-risk karyotype : the remission rate is not different between the 2 groups but the relapse rate is significantly higher in the poor-risk patients. The overall and disease-free survival are better in intermediate-risk group. By multivariate analysis, the disease-free survival is influenced by the age at the time of treatment.
In summary, this study does not demonstrated improved survival in poor-risk patients treated with intensive chemotherapy followed by stem cell transplantation. However the remission rate was improved after intensive chemotherapy followed by stem cell transplantation. The intermediate-risk patients have abetter survival and a lower probability of relapse than poor-risk patients Cette étude rétrospective évalue les résultats de traitements chimio thérapeutiques intensifs avec greffe de cellules souches par rapport à des traitements conventionnels chez des patients à haut risqué atteints de leucémie myéloïde aiguë ou de myelodysplasie, portant une anomalie du caryotype défavorable : monosomie ou délétion d’un segment du bras long des chromosomes 5 et/ ou 7.
Trois groupes sont étudiés : le premier est composé de 16 patients à caryotype défavorable traités par greffe, le deuxième comporte 11 patients à caryotype défavorable traités par chimiothérapie conventionnelle et le troisième comprend 14 patients à pronostic intermédiaire (caryotype normal) traité par greffe de cellules souches.
Les résultats des traitements intensifs avec greffe de cellules souches sont comparés à la chimiothérapie conventionnelle en terme de réponse et de survie (globale et sans maladie) chez les patients à caryotype défavorable : le taux de rémission est significativement meilleur chez les patients greffés ; quant au taux de rechute, à la survie globale et à la survie sans maladie, ils ne diffèrent pas d’un groupe à l’autre.
Les résultats des traitements intensifs avec greffe de cellules souches sont comparés en terme d’impact du type d’anomalie cytogénétique sur la réponse au traitement et la survie chez les patients à caryotype défavorable et des patients à caryotype intermédiaire : le taux de rémission ne diffère pas d’un groupe à l’autre, mais le taux de rechutes est significativement plus important dans le groupe de patients à caryotype défavorable. La survie globale et la survie sans maladie sont meilleures chez les patients à caryotype intermédiaire. L’analyse multivariée indique que la survie sans maladie est influencée par l’âge au traitement.
En résumé, les traitements intensifs avec greffe de cellules souches ne confèrent pas d’avantage significatif de survie chez les patients à caryotype défavorable. Le taux de rémission est cependant supérieur après traitement intensif.
Les patients à caryotype intermédiaire traités par chimiothérapie intensive suivie de greffe de cellules souches ont moins de rechutes et une meilleure survie que les patients à caryotype défavorable ayant reçu le même type de traitement
Leukemia, Myeloid --- Neural tube defects --- Drug Therapy --- Cell Transplantation
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Neural Tube Defects Chairwoman: Professor Judith Hall 1994 Neural tube defects are major human congenital malformations. They are the second most common cause of perinatal death owing to birth defect in the UK and lead to considerable childhood morbidity. This book contains descriptions of normal neurulation in birds, amphibians and mammals, including humans. The anatomy of neural tube defects is discussed, with emphasis on the underlying molecular genetics, particularly in mouse strains with heritable neural tube defects. There is evidence for both genetic and environmental factors in the development of neural tube defects. Possible risk factors include anticonvulsant agents, diabetes and alcohol: the current status of epidemiological studies on these is assessed and the relative contributions of potential causative elements are critically reviewed. Clinical trials in humans have shown that folic acid consumption before and immediately after conception significantly reduces the risk of having a child with a neural tube defect. The book contains a detailed description of folic acid metabolism and discussion of how it may act during neural tube closure. The book describes methods for antenatal detection and diagnosis of neural tube defects. The feasibility of treatment, both in utero and after birth, is considered. Finally, the book presents the case for implementing a programme to ensure adequate consumption of folic acid by all women of child-bearing age. Possible objections to, or disadvantages of, such a programme are discussed fully and solutions to such problems are proposed. Related Ciba Foundation Symposia: No. 156 The childhood environment and adult disease Chairman: David Barker 1991 ISBN 0 471 92957 3 No. 165 Postimplantation development in the mouse Chairwoman: Anne McLaren 1992 ISBN 0 471 93384 8 No. 182 Germline development Chairwoman: Anne McLaren 1994 ISBN 0 471 94264 2
Neural Tube Defects. --- Neural tube --- -Embryology --- Nervous system --- Acrania --- Developmental Neural Tube Defects --- Iniencephaly --- Neural Tube Developmental Defects --- Neuroenteric Cyst --- Occult Spinal Dysraphism --- Occult Spinal Dysraphism Sequence --- Tethered Spinal Cord Syndrome --- Craniorachischisis --- Developmental Defects, Neural Tube --- Diastematomyelia --- Exencephaly --- Neurenteric Cyst --- Spinal Cord Myelodysplasia --- Tethered Cord Syndrome --- Acranias --- Craniorachischises --- Cyst, Neurenteric --- Cyst, Neuroenteric --- Cysts, Neurenteric --- Cysts, Neuroenteric --- Defect, Neural Tube --- Defects, Neural Tube --- Diastematomyelias --- Dysraphism, Occult Spinal --- Dysraphisms, Occult Spinal --- Exencephalies --- Iniencephalies --- Myelodysplasia, Spinal Cord --- Myelodysplasias, Spinal Cord --- Neural Tube Defect --- Neurenteric Cysts --- Neuroenteric Cysts --- Occult Spinal Dysraphisms --- Spinal Cord Myelodysplasias --- Spinal Dysraphism, Occult --- Spinal Dysraphisms, Occult --- Tethered Cord Syndromes --- Abnormalities --- -Congresses --- Neural Tube Defects --- -Abnormalities --- Neural tube defects
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