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Company Law (Core Texts Series) [Alan Dignam, John Lowry] on * FREE* shipping on qualifying offers. The Core Text series takes the reader. Company Law (Core Texts Series) [John Lowry, Alan Dignam] on * FREE* shipping on qualifying offers. Aiming to demystify company law for. : Company Law (Core Texts Series) (): Alan Dignam, John Lowry: Books.

Corporate Governance Third Edition R. It discusses the current debates surrounding company law, including the reviews by the English and Scottish Law Commissions, the consultation papers issued by the Company Law Review Steering Group and the ensuing White Papers and Draft Companies Bill.

Get to Know Us. Alan DignamJohn Lowry. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Written by leading academics and renowned for their clarity, these concise texts explain the intellectual challenges of each area of the law.

Company Law

Top Reviews Most recent Top Reviews. The text also includes valuable coverage of corporate governance and theory, including the current debates surrounding these areas. Concentrate Questions and Answers Company Law: Company Law provides the perfect balance between depth, succinctness, and accessibility. Lifting the veil 4. Company Law gives an authoritative and accurate account of the key principles and demystifies this complex area of the law without oversimplification.

Company Law (Core Texts Series): John Lowry, Alan Dignam: : Books

Chapter introductions will orientate readers with the various aspects of company law and further reading will provide the tools for further research and study. Learn more about Amazon Prime. Oxford University Press; 4 edition November 9, Language: Aiming to demystify company law for today’s student, dignm concise textbook offers an overview of the subject, covering most undergraduate company law syllabi.

Oxford University Press is a department of the University of Oxford.

Company Law provides the perfect balance between depth, concision, and accessibility. Company Law Core Texts Series. Read, highlight, and take notes, across web, tablet, and phone. Amazon Second Chance Pass it on, trade it in, give it a kowry life. Academic Skip to main content. My library Help Advanced Book Search. I’d like to read this book on Kindle Don’t have a Kindle?

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The Core Text series takes the reader straight to the heart of the subject, providing a reliable and invaluable guide for students of law at all levels. Account Options Sign in. Contents Table of secondary legislation. Previous publication dates AugustAugustOctober Amazon Drive Cloud storage from Amazon. Particular emphasis is given to corporate governance and the theoretical bases underlying company law.

Company Law Alan J.

The Core Text Series takes the reader straight to the heart of the subject, providing a reliable and invaluable guide for students of law at all levels.

Issues of Corporate Authority Written by leading academics and renowned for their clarity, these concise texts explain the intellectual challenges of each area of the law.

Company Law Alan J. Would you like to tell us about a lower price?

The Core Text series takes the reader straight to the heart of the subject, providing a reliable and invaluable guide for students of law at all levels. DignamJohn Lowry Snippet lowrj – Read more Read less.

LEGO Recycle Truck Set Instructions. Theme: LEGO Town. Item No, Set Number, BOID, Year Released, 30cm x 42cm x. 35 Items ItemName: Lego Recycle Truck, ItemType: Set, ItemNo: , Buy and sell complete No box or instructions brand new band no recycle stickers. LEGO set database: Recycle Truck. Set number: ; Name: Recycle Truck; Also known as: Envirotruck; Set type: Normal; Theme group: Modern.

Hey, you’re really churning out a whole bunch of models now.

instructikns About the red technic bush, it will fit if you rotate the “fuel tank” assembly upwards a bit which forces you to move some other bits and lebo as well, so it’s a bit awkward.

About the technic long pin, I see you’ve bumped in to that problem. Yeah, it’s because the designers at TLG are using illegal techniques, even if they are not supposed to. These kind of problems were worse a few years ago, but it seems TLG has gotten better laterly. Still it’s interesting you instructiobs one as late as I’ve also included a posed version that shows the walker walking, just for fun.

Click on the image for larger version. Image of straight-leg version. I read this and thought for a moment, “LDD has the ability to animate? I feel like maybe you mean to say “posed” or “articulated” unless there is yet another layer to LDD that we can unlock with enough knowledge of assembly and hex. Had nothing to do, so I did the flexi-stuff. A bit tricky it was. I had this finished on Friday, but didn’t levo time to post it before heading out for the weekend What trickery did you do to place that “conical wheel z12” in the 66668 back?

In my model it would not fit due to a collision with the cross axle above it, and while it fits in your model I can still see the collision. The most time i spend on this model was aligning all gears so they would levo. At first i planned to do the back tilted like in the instructions, but after like 2 hours i gave up and it sill took me like 1 hour or so that get it like it’s displayed now.

Sounds exactly like my experience while building this model. I had just built the in LDD and I had same problem of placing on its proper position.

Would you mind telling me the way to place it? Hm, not sure I remember since I was fiddling around a lot. First Innstructions took away all the bricks so that I could position the flex-rod in i.

Then I positioned the other end of the flex-bar in the up in instrudtions roof. This tooks some moving the mouse around and finaly it snapped. I can’t really remember, but I think I nudged it slowly in place in the Use an external file hosting service to host the files, such as brickshelf.

The old intercoastal seaport, with crane which readily falls over. Instructinos this up was not so easy, as many parts do not instructione and had to be replaced with alternatives see list below. Errors also include wrong stickers and mini-fig decals.

I was kind of wondering how others did this But like described above, I also delete most of the surrounding bricks to minimize things getting in the way – but I do this using a “copy” of the entire structure.

This allows me to make all sorts of changes that would most likely screw up the surrounding structure – but on a copy, who cares?

LEGO 6668 Recycle Truck / Dust Cart C/w Box And Instructions 1992

Once the flexible piece is in the proper shape, it can easily be moved into place. Another seaport, shares a lot of similarities withbut many strange differences in the harbour and boat designs. Obviously a time of ‘reinvent the wheel’. An old classic, only two hooklift vehicles were made; the recycle truck, and the container truck in train Here is the little recycle truck. My name is Anders Gaasedal and I am the designer of the A-model.

I have just seen the LDD model you created and must say you’ve done a magnificent job.

Instructions for – Recycle Truck |

I hope you had as much fun recreating it in LDD as I had designing it. What a nice surprise. No errors in legoo, I don’t think, and all the part numbers are correct too. You need to be a member in order to leave a comment. Sign up for a new account in our community. Already have an account?

Prev 73 74 75 76 77 78 79 80 81 82 83 Next Page 78 of Posted August 18, Technic – Tractor [Vers 02] – Theme: Share this post Link to post Share on other sites. Posted August 18, edited. Posted August 19, I’ve also included an animated version that shows the walker walking, just for fun.

LEGO Recycle Truck Instructions , City – Adrisa

Posted August 21, edited. Edited Inztructions 21, by P4trickvH. Posted August 21, You can find all the rules of the topic in the first page. I rotated the shaft above it 45 degrees and that did the trick. Posted August instrcutions, A bit tricky it was You can download the file and update your post if you want.

Posted August 25, Posted August 26, edited. Posted August 26, Hi vean, welcome on EB and in this topic. To post a new set in this topic, you have to read the rules you can find in the first page. About your post you should: Forum’s attachment function is not much suitable for this kind of use.

Posted August 27, edited. LXF File Errors The crane supports are custom made from bricks, and not the single Big Ugly Piece The tyres for the truck and tractor are wrong; but alternatives are used. The motorbike in the container is just plain wrong; and even then had to be hacked to make a legal fit. The crane jib does fit together with green highlights, but may not be future proof. The rear window of the truck is a custom made piece with a 1×4 hinge plate vertical.

The plate with ball vertical, down does not seem to exist, so is a custom hack. The top of the boat bow does not exist so is made of custom plates, shown in red. The jib of the boat crane does not seem to exist, so is made of custom plates, but coloured white for now. Probably more, but the general gist is correct. Truck Edited September 4, by roamingstudio.

Posted August 27, Posted August 28, Posted August 28, edited. No hose pipe which flexes for the cargo engine No correct tyres for the truck, tractor and forklift. No correct forklift piece – the modern replacement is used.

However it requires additional plates to complete the submodel, highlighted in green. No correct 2×2 plate with single wheel underneath modern replacement used. Probably some more, but it is as complete as possible.

Diesel Engine Container Reefer Refrigerated Container Posted August 30, edited. Town An old classic, only two hooklift vehicles were made; the recycle truck, and the container truck in train Incorrect minifig decals as always Edited August 30, by roamingstudio. Posted August 31, Got a mail today that I was asked to post here: Posted September 1, edited. Edited September 2, by prateek. Train Duplicate No building errors or decals, but only 1 substituted part road plate.

Edited September 1, by Calabar “Duplicate” tag added. Create an account or sign in to comment You need to be a member in order to leave a comment Create an account Sign up for a new account in our community. Register a new account. Sign in Already have an account?

George Modelski, Professor Emeritus of our department, passed away at his home in Washington, DC on February 21, Modelski was on. Seapower in Global Politics, – Authors: Modelski, George, Thompson, William R. Buy this book. eBook 53,54 €. price for Spain (gross). Buy eBook. Long Cycles in World Politics. Authors: Modelski, George. Buy this book. eBook 51,16 €. price for Korea, Republic of (South Korea) (gross). Buy eBook.

Dana was my friend and mentor for more than 25 yea Principles of world politics more. After his retirement, he moved to Washington, DC where he remained active in research, conferences, and publishing. Skip to main content.

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Although never widely cited or known in IR circles, his contributions were always distinctively different and original. He was a major contributor to international relations theory, the study of multinational corporations as actors in international relations, and the study of leadership in international relations.

Get to Know Us. Amazon Second Chance Pass it on, trade it in, give it a second life. Responding to the destabilized international system of the s, George constructed an interpretation of world politics that was based on the emergence of lead economies, their rise and fall, and implications for global war and order.

Political Science and Organization. Remember me on this computer. There’s a problem loading this menu right now. Leading sectors and world powers: II is an interdisciplinary journal with a particular interest in research that focuses upon the broad range of relations and interactions among the actors in the global system.

In he was awarded the Bronze Kondratieff Medal, which is provided by the Kondratieff Foundation and the Russian Academy of Sciences in citing his outstanding contributions to the development of the social sciences. Heisler on “Farewell to our friend, Dana Larsen”: Another major venture in this vein was his effort to develop a better empirical and theoretical understanding of historical urbanization processes, as reflected in World Cities, to Endeavoring to create communities of scholars dedicated to international studies, ISA is divided into 6 geographic subdivisions of ISA Regions29 thematic groups Sections and 4 Caucuses which provide opportunities to exchange ideas and research with local colleagues and within specific subject areas.

This appeared among other works in a co-edited book Globalization as Evolutionary Process: William R Thompson Publisher: That work appeared in a book Long Cycles in Global Politics Macmillan and was further developed in a book Seapower in Global Politics Macmillan with William Thompson along with numerous articles and book chapters.

Be Boundless for Washington For the World. Are you an author? How can this process of morphogenesis be explained They also underlined his very early interest in comparing types of international georg.

His main contribution to the study of international relations, nonetheless, has to be founding a research program on leadership long cycles.

Like an untold number of moselski, I claim that Dana Remembering Chandra Sriram, a dedicated human righ During his academic career, Modelski held a number of positions in the profession and received a number of honors. Chase-Dunn, University of California-Riverside.

His dissertation, completed in and published in as a Theory of Foreign Policywas a very early attempt to treat foreign policy issues theoretically, as opposed to the then standard reliance on descriptive accounts. Free Pr Publication Date: Modelski’s work has been widely cited and is a standard reference in textbooks on international relations.

Amazon Music Stream millions of songs. Amazon Restaurants Food delivery from local restaurants. A theory of foreign policy more. ComiXology Thousands of Digital Comics. Hire a UW Ph. Comparative Studies in Society and History.

Amazon Advertising Find, attract, and engage customers. In addition to his lasting scholarly imprint on the field of international relations, Modelski spurred two generations of graduate students to work on various aspects of long-cycle theory.

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George Modelski

Multimedia 22 Awards Groups Enter the email address you signed up with and we’ll email you a reset link. Throughout a long and distinguished career, George Modelski emphasized the modelwki to bring together theory, evidence, and history in the unraveling of world political processes.

Seapower in global politics, more.

Modelski began developing this original perspective around first conference paper and published the first article infollowing a slightly earlier effort to begin developing a systemic interpretation of world politics Principles of World Politics Amazon Renewed Refurbished products with a warranty. Denemark, University of Delaware Christopher K.

English Choose a language for shopping. IPS is an interdisciplinary journal gorge to the diversification of both scholarly interests and regional concerns in contemporary international studies.

George Modelski – Wikipedia

Southall on “In Memoriam: Comments There are currently no comments, be the first to post one. Categories All Conferences Log In Sign Up. Help Center Find new research papers in: Another generation of global warfare was required before

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Discussion threads can be closed at any nnetgear at netbear discretion. I have mine setup in an enclosed entertainment center yes, it vented and cannot hear it. A simple way to provide segmentation of the network with internal routing through the switch — reserving the router for external traffic routing only, making the entire network more efficient. Amazon Music Stream millions of songs.

Should your order go missing before it arrives, Amazon may register the serial number with loss and theft databases gs274t prevent fraudulent use or resale of the item. Why we ask for your product’s serial number: In fact, the Linksys router just did not work the way I had expected it to work, so I eventually ended up swapping out the router for a Ubiquiti USG.

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This Guide takes you through the Dedication and the first two hundred or so lines of Luis de Góngora’s Soledad Primera. The poem is presented by pages. A Poetics Sacralized: Luis de Góngora s ” Soledades ” 5 living in the Americas who led this revival of gongorism; it was the crio- llos and mestizos, the men. Among scholars of Spanish Baroque poetry, the dominant opinion has been that the writer Luis de Góngora was silent, indifferent, or even.

Soledades by Luis de Góngora

Probably a little over-ambitious for my first book-length poem in Spanish. Given the length, I expected a narrative poem, however there is no story to be found here. The Solitudes were designed to be difficult at a moment when decorous rhetorical obscurity and conceited poetics were in vogue.

Ele rated it liked it Jan 14, GAKO rated it it was amazing Jun 28, Most are very helpful, as it often not remotely obvious what Gongora is referring to. A prerequisite is a thorough education in Greek mythology, for the poet repeatedly alludes to that substantial body of work. The second Soledad’s closing image is incredible and probably my favorite part of either poem. Footnotes are required for both Solitudes, but I also recommend reading through them once without consulting the footnotes, just for the pleasure of being lost — sometimes literally, in the case of the second poem — in the text.

The poem’s hyper-baroque syntax forces you to process its imagery in such a way that, in order to understand Gongora’s meaning, you have to engage with his language in extremely unusual ways.

Some seemed unnecessary – Sisyphus and Bacchus are pretty well known, right? Contact Contact Us Help. Adoro muchos de sus sonetos. He also belongs in every age, as elements can be found in nearly each style. On approval, you will either be sent the print copy of the book, or you will receive a further email containing the link to allow you to download your eBook. Great metaphorical reflections coupled with an infusing of the quotidian world with mythological reference.

Published January 1st by Ediciones Catedra S.

Wonder is mute, it speaks by being silent, and, blind, it follows a river, that-shining child of those precipices– in convoluted discourse, and digressive, benevolent it tyrannizes the fields; with its gongoea ornamented by fruit trees, Copia herself desires it for her horn -if the creature of Amalthea had been armed with crystal transparency-; setting edifices in its silver, crowning itself with walls, it embraces, imprisons islands, from the high grotto where it first breaks vongora to the opaque jasper waters deep where pride is soledadse and memory hides away.

Mapping words to their translations was rather fun, although I didn’t attempt it systematically. This is definitely the translation to read; I tried Wilson’s first, and it was impossible. But Edith Grossman’s facing translation is a masterful lifeline.

There’s some great lines about owls too!! Gongora loves crystals and nymphs from Greek myth, both of which recur constantly.

Las Soledades de Luis de Gongora Argote

Ian Lepine rated solrdades it was amazing Jan 05, Return to Book Page. Carolina rated it liked it Dec 15, This website uses cookies to ensure you get the best experience on our website. Feb 04, Francisco rated it it was amazing Shelves: Ultimately, Gongora’s syntax performs the process of poetic perception itself in all its chaotic excitement, and in aoledades so transforms his readers into visionary poets, exulted yet burdened with poetic inspiration and imagery.

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Aspects of Góngora’s ‘Soledades’ | John R. Beverley

An extremely difficult yet marvelous poem that rewards multiple re-readings. His Solitudes stands as his artistic gonglra and testament. He has inherited the vestments of the ancient Greek poets. This is required reading for lovers of the written word.

Soledqdes sings throughout nearly ever stanza. Project MUSE promotes the creation and dissemination of essential humanities and social science resources through collaboration with libraries, publishers, and scholars worldwide. And this is my impression by reading it in English without the rhyme scheme in Spanish! The second Solitudewhich I only read once in aoledades Edith Grossman translation, was admittedly more abstract and slightly more difficult for all the allusions to myth.

I read the first Solitude twice in a row in different translations just to experience the journey again. Fusing epic and pastoral, the poem describes a journey through the countryside and contrasts the beauty of nature and of the rural crafts and customs with the vices of court, commercial greed and colonial exploitation.

In lieu of an abstract, here is a brief excerpt of the content: Let me start off by saying that well before finishing even the first Soledad there are twoI knew I would have to go back and re-read these poems which I intend to do. It is hypnotic, adventurous, peaceful, and sublime. Quevedo, Lope de Vega, and other writers first cultivated gongorism in order to mock it, but later wound up dabbling in it for many other literary uses.

At its most abstract, it remains centered on the most concrete human experiences:

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AMX AXB-REL8 Relay Controller

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: Design of Piping Systems () by M. W. Kellogg Company and a great selection of similar New, Used and Collectible Books. Design of Piping Systems by M W Kellogg Company, , available at Book Depository with free delivery worldwide. Find Design Of Piping Systems by Kellogg, M W, Company at Biblio. Uncommonly good collectible and rare books from uncommonly good booksellers.

Design of Piping Systems

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Design Of Piping Systems

Description reprint of the second edition. A very handsome copy. Ships from the UK. Russell Books Ltd Condition: Advanced Book Search Browse by Subject.

Design of Piping Systems : M W Kellogg Company :

Visit our Beautiful Books page and find lovely books for kids, photography lovers and more. Near Fine Condition Edition: Kellogg stamp on top of first page along with previous owner’s neatly inked name, head and tail of spine lightly bumped, otherwise lightest shelf and edge wear only. Sign up to receive offers and updates: Dispatched from the UK in 3 business days When will my order arrive? Check out the top books of the year on our page Best Books of Book ratings by Goodreads.

This title made available for the first time an adequately organized, comprehensive analytical method for evaluating the stresses, reactions and deflections in an irregular piping system in space, unlimited as to the character, location or number of concentrated loadings or restraints.

Design of Piping Systems. Profusely illustrated and meticulously detailed. Your purchase also supports literacy charities. Showing best matches Show all copies. Shows some signs of wear, and may have some markings on the inside. Kellogg Company John Wiley, We’re featuring millions of their reader ratings on our book pages to help you find your new favourite book. Better World Books Ltd Condition: No interior marks except as noted above.

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Design Of Piping Systems by Kellogg, M W, Company

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Falli soffrire Gli uomini preferiscono le stronze. La versione aggiornata: Sherry Argov: Books – There is a newer edition of this item: Falli Soffrire Gli Uomini Preferiscono Le Stronze / Why Men Love Bitches – Italian Edition CDN$ Falli soffrire has ratings and reviews. Thea said: I picked up a used copy of this book as a gag gift, and my fascination with the intensive.

I do like the fact that it forces women to be true to who they are first and foremost and that they should always act with dignity, it becomes a bit overbearing.

Don’t get me wrong–I’m all about making my man prove himself and toil a bit for me. You learn how to basically con sogfrire way into and through a relationship here. Il terzo tipo sono regole americane, per cui o sono incomprensibili per l’italiana che non conosca nulla degli USA o forse possono servire se avete preferizcono che fare con Tom, Dick o Harry.

You will learn so many things, even things that we already know but just ignore.

Falli soffrire: Gli uomini preferiscono le stronze

If you are dead set on reading this book, I sofrrire urge you to just forgo reading the Dumb Fox chapter. I know tons of girls that go out with guys who like to treat women like a doormat. Comparing men to seals clapping their fins for salmon salmon being sex, obviously was discouraging. Many books on this preferiscoho are a bit lame.

Reading the book makes me pay more attention to my boyfriend. Why it will end up in that dangerous spiral of hurt feelings and nagging. In my opinion, it’s not practical to go follow the long list of guidelines if you’re on a long term relationship.

Don’t get me wrong–I’m all about making my man prove himself and toil a bit for me. This review has been hidden because it contains spoilers. Return to Book Page. Is it worth reading?

Women with attitude and not push overs. This book is perfect. But there never seemed to be…well, anything about love. And I have to say, I do like the story about the woman who leaves for the weekend to spend time with her friends and family whenever her husband is being a little distant. Does this method work?

I pride myself on wearing my heart on my sleeve, which is certainly a turn off to many people, nevermind men. I could cite many other examples of bad advice in this stfonze but I think it’s best to say that if you want to read some poor literature and be entertained then this book may be for you; however, if you actually need better advice than this, I recommend “If I’m So Wonderful, Why Am I Still Single? Also, if you need a book to tell you that owning your power and guarding it jealously is the only way to have a life free of foot marks on your back, well, you’re basically fucked.

Una guida alle relazioni di coppia rivolta alle donne “troppo premurose”. Those are the gist of the whole book and the rest is just fluff and repetition in my opinion.

It will save you a stronxe of head-scratching and a lot of time and in all you’ll enjoy this book more.

Argov is right when she stressed that women should have their own lives outside of their relationship. View all 3 comments. According to Argov, a woman shouldn’t even change a lightbulb on her own because then her man will feel emasculated and unappreciated around the house.

Being passive aggressive to your partner to do what you want to do talks a lot about the relationship. Sherry Argov gives a lot of good details and examples and they are very entertaining.

But the last time I checked, no man I know has ever said he wanted a high maintenance bitch. Why Men Love Bitches is also a 1 first-class live theater production in Latin America, and her bestselling books are currently published throughout the world in over thirty languages. I got a man and this book made appreciate him even more Of course, she does take a moment to point out that any man who describes himself as a feminist is likely to quit his job and take up residence on the couch while you support him.

I’m in a long term relationship And I basically followed one of the steps here. Chapter after chapter recommends what to look out for in terms of your own and your love interest’s behavior and how to best use this to your advantage. I know it’s hard to admit that your any of this things but come on every woman has experienced if not one all of this traits at one point in their lives and if you still don’t believe me then ask yourself right now So if a relationship book which never talks about love but talks about sustaining the relationship brings in a lot of questions.

Maybe next time, when I’m done with my reading list. This book teaches these kind of women how to be hard and let them know they don’t need a man.

Read falli-soffrire-gli-uomini-preferiscono-le-stronze

To visit my websites: I couldn’t give it full stars because there are a couple areas that are not addressed. Want to Read saving…. To follow me on Instagram: This book is not yet featured on Listopia. And if I got nothing else out of it, her book showed me that much. Goodreads helps you keep track of books you want to read.

Place yourselves above the need to sustain the relationships and many other ideas totally pissed me off. It talks about how women need to stop throwing themselves at men when they don’t want them. Book reviewer that I am, I couldn’t just “not” read them. My friend gave me a stack of these books after she got married.

With saucy detail on every page, this no-nonsense guide reveals why a strong woman is much more desirable than a “yes woman” who routinely sacrifices herself.

4 years ago. Escala Abreviada De Desarrollo Unicef Colombia. Explicit content. Escala Abreviada De Desarrollo Unicef Co 4 years ago. GUIAS ALAD Trabajo presentado con los auspicios de UNICEF, en el Curso Internacional sobre Escala Abreviada de Desarrollo (Ministerio de Salud – Nelson Ortiz. Escala Abreviada del Desarrollo Psicosocial. [Internet]. Disponible en: http:// 8. Silvestre N.

This study was approved by the ethics committee of the Faculty of Medicine of Universidad Nacional. A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease.

Saray Esther Polo Polo

All patients were male, 6 months to 16 years of age, one of them died by the age of 5 due to complications of a respiratory infection. University of Washington, Seattle; https: Pelizaeus Merzbacher Disease PMD is a chronic pediatric leukoencephalopathy caused by disorders of the axonal myelination and the myelin metabolism in the oligodendrocytes, reported for the first time on by doctor Friedrich Pelizaeus 1 and revisited on by Knicef Merzbacher 2.

Pelizaeus-Merzbacher disease clinical and nosological study.

Ile47IlefsX4 and resulted in a truncated protein product, 4 aminoacids downstream Among other differential diagnosis we can count Krabbe desarrolli, Canavan disease, other leukodystrophies and cerebral palsy.

Abgeviada PMD is characterized in the first stages of disease by nystagmus, hypotonia and tremor in male affected patients, joint progressively by ataxia and spastic quadriparesis in the school age. Laboratory endpoints are shown in Table 2. Q99X affects the cytoplasmic domain while c. Q99X and missense mutation c. Neuroimaging of patients with classic PMD showed evidence of T2 hyperintensities both diffuse desargollo periventricular in the supratentorial withe matter.

Patients desarrlolo from a connatal form of PMD, the most severe phenotype, have histopathological studies revealing complete absence of myelination in the brain, explaining the rapid clinical deterioration and suggesting tan death of this patients may respond to nervous conduction alterations in brain control centers.

PLP1-related inherited dysmyelinating disorders Pelizaeus-Merzbacher disease and spastic paraplegia type 2. PLP1 molecular analysis Complete duplication. To our knowledge, this is not only one of first Latin-American case series but the larger one, presenting the main characteristics of the clinical diagnosis and molecular signatures of PMD male affected patients, being the classical form overall more frequent than the connatal form.

Connatal form of PMD is more severe than the classical form, a verifiable fact in our series, and with a reported expectancy of life lower than the first decade of life. In the physical examination, Mean age at diagnosis was esccala years 5 months, being classic PMD most frequently diagnosed, as compared to the connatal phenotype.

On the other hand, NS patients suffer from desarroollo less harmful condition also caused by large deletions or damaging mutations resulting in loss of PLP1 protein product. The esccala protein gene and myelin disorders in man and animal models. Available for review by the Editors of this journal and are kept within the clinical records of each patient. Pelizaeus-Merzbacher disease, Pelizaeus-Merzbacher-like disease 1, and related hypomyelinating disorders.

Structure and molecular arrangement of proteolipid protein of central nervous system myelin. Survival rates in this patients have been described to be up to the seventh decade of life.

Interestingly, it has been described NS affected individuals to have a multifocal demyelinating neuropathy 812 sometimes being the only clinical feature of the syndrome; NS patients do not present jnicef nystagmus, their spastic paraplegia is mild, affecting primarily the lower limbs, and ataxic compromise may vary. Q99X nonsense variant in two ddland in one patient the c. Cerebral magnetic resonance imaging.

Most of mutations detected on individuals diagnosed with SPG2 are missense 8.

Calaméo – Saray Esther Polo Polo

Note that patients are cited on the tables with their assigned pedigree numbers. PLP1 gene is located in chromosomal region Xq22, with a 17 kb lenght, 7 exons and 6 introns. Its genetic etiology affects the expression of the Proteolipidic Protein type 1 34varying from hemizygous mutations to gene dosage alterations of the PLP1 Xq Clinical and mutational spectrum of Colombian patients with Pelizaeus Merzbacher Disease Translated title es: The espectrum of PLP1 gene mutations in patients with classical form of the Pelizaeus Merzbacher disease.

Z Ges Neurol Psychiatr. Estudios Moleculares y Resultados: Classic PMD affected males also have improved cognitive development, with acceptable speech.

In our sample, two patients had history of cerebral palsy, being an actual comorbidity in only one of them. Mutations in noncoding regions of uniccef proteolipid protein gene in Pelizaeus-Merzbacher disease.

Clinical and mutational spectrum of Colombian patients with Pelizaeus Merzbacher Disease

Molecular studies were used in the majority of the cases to confirm the diagnosis Hobson GM, Kamholz J. Case series presentation Seven individuals ages 6 months to 16 years 4 probands, 3 male relatives of the probandsdiagnosed clinically, paraclinically and molecularly as Pelizaeus Merzbacher patients, attended in different medical care centers in Colombia Fig.

Seven individuals ages 6 months to 16 years 4 probands, desardollo male relatives of the probandsdiagnosed clinically, paraclinically and molecularly as Pelizaeus Merzbacher patients, attended in different medical care centers in Colombia Fig.