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Orphanet Journal of Rare Diseases

Ausgabe 1/2011

Inhalt (89 Artikel)

Open Access Research

Molecular analysis and intestinal expression of SAR1 genes and proteins in Anderson's disease (Chylomicron retention disease)

Amandine Georges, Jessica Bonneau, Dominique Bonnefont-Rousselot, Jacqueline Champigneulle, Jean P Rabès, Marianne Abifadel, Thomas Aparicio, Jean C Guenedet, Eric Bruckert, Catherine Boileau, Alain Morali, Mathilde Varret, Lawrence P Aggerbeck, Marie E Samson-Bouma

Open Access Research

Genome-wide analysis of Ollier disease: Is it all in the genes?

Twinkal C Pansuriya, Jan Oosting, Tibor Krenács, Antonie HM Taminiau, Suzan HM Verdegaal, Luca Sangiorgi, Raf Sciot, Pancras CW Hogendoorn, Karoly Szuhai, Judith VMG Bovée

Open Access Research

Respective implications of glutamate decarboxylase antibodies in stiff person syndrome and cerebellar ataxia

Mario U Manto, Christiane S Hampe, Véronique Rogemond, Jérome Honnorat

Open Access Case Report

Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation

Jean-Philippe Camdessanché, Véronique V Belzil, Guillemette Jousserand, Guy A Rouleau, Christelle Créac'h, Philippe Convers, Jean-Christophe Antoine

Open Access Erratum

Erratum To:Clinical and genetic characterization of chanarin-dorfman syndrome patients: first report of large deletions in the ABHD5 gene

Chiara Redaelli, Rosalind A Coleman, Laura Moro, Amalia Sertedaki, Talia Kakourou, Solaf Mohamed Elsayed, Daniele Prati, Agostino Colli, Donatella Mela, Roberto Colombo, Daniela Tavian

Open Access Case Report

Prenatal manifestation and management of a mother and child affected by spondyloperipheral dysplasia with a C-propeptide mutation in COL2A1: case report

Maria Francesca Bedeschi, Vera Bianchi, Barbara Gentilin, Lorenzo Colombo, Federica Natacci, Sabrina Giglio, Elena Andreucci, Laura Trespidi, Barbara Acaia, Andrea Superti Furga, Faustina Lalatta

Open Access Case Report

Autosomal recessive cerebellar ataxia caused by mutations in the PEX2 gene

Caroline Sevin, Sacha Ferdinandusse, Hans R Waterham, Ronald J Wanders, Patrick Aubourg

Open Access Research

Utero-vaginal aplasia (Mayer-Rokitansky-Küster-Hauser syndrome) associated with deletions in known DiGeorge or DiGeorge-like loci

Karine Morcel, Tanguy Watrin, Laurent Pasquier, Lucie Rochard, Cédric Le Caignec, Christèle Dubourg, Philippe Loget, Bernard-Jean Paniel, Sylvie Odent, Véronique David, Isabelle Pellerin, Claude Bendavid, Daniel Guerrier

Open Access Research

Lack of a synergistic effect of a non-viral ALS gene therapy based on BDNF and a TTC fusion molecule

Ana C Calvo, María Moreno-Igoa, Renzo Mancuso, Raquel Manzano, Sara Oliván, María J Muñoz, Clara Penas, Pilar Zaragoza, Xavier Navarro, Rosario Osta

Open Access Research

Mortality associated with neurofibromatosis type 1: A study based on Italian death certificates (1995-2006)

Maria Masocco, Yllka Kodra, Monica Vichi, Susanna Conti, Mark Kanieff, Monica Pace, Luisa Frova, Domenica Taruscio

Open Access Review

Hughes-Stovin Syndrome

Umair Khalid, Taimur Saleem

Open Access Case Report

Cholestasis in patients with Cockayne syndrome and suggested modified criteria for clinical diagnosis

Tawhida Y Abdel Ghaffar, Ezzat S Elsobky, Solaf M Elsayed

Open Access Case Report

Microarray based analysis of an inherited terminal 3p26.3 deletion, containing only the CHL1 gene, from a normal father to his two affected children

Cristina Cuoco, Patrizia Ronchetto, Stefania Gimelli, Frédérique Béna, Maria Teresa Divizia, Margherita Lerone, Marisol Mirabelli-Badenier, Monica Mascaretti, Giorgio Gimelli

Open Access Review

Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema

Ignacio Blanco, Beatriz Lara, Frederick de Serres

Open Access Research

Mortality Associated with Neurofibromatosis 1: A Cohort Study of 1895 Patients in 1980-2006 in France

Tu Anh Duong, Emilie Sbidian, Laurence Valeyrie-Allanore, Cédric Vialette, Salah Ferkal, Smaïl Hadj-Rabia, Christophe Glorion, Stanislas Lyonnet, Michel Zerah, Isabelle Kemlin, Diana Rodriguez, Sylvie Bastuji-Garin, Pierre Wolkenstein

Open Access Case Report

Complex chromosome rearrangements related 15q14 microdeletion plays a relevant role in phenotype expression and delineates a novel recurrent syndrome

Maria Cristina Roberti, Cecilia Surace, Maria Cristina Digilio, Gemma D'Elia, Pietro Sirleto, Rossella Capolino, Antonietta Lombardo, Anna Cristina Tomaiuolo, Stefano Petrocchi, Adriano Angioni

Open Access Review

Limitations of drug registries to evaluate orphan medicinal products for the treatment of lysosomal storage disorders

Carla EM Hollak, Johannes MFG Aerts, Ségolène Aymé, Jeremy Manuel

Open Access Research

MR spectroscopy-based brain metabolite profiling in propionic acidaemia: metabolic changes in the basal ganglia during acute decompensation and effect of liver transplantation

James E Davison, Nigel P Davies, Martin Wilson, Yu Sun, Anupam Chakrapani, Patrick J McKiernan, John H Walter, P Gissen, Andrew C Peet

Open Access Review

Clinical and animal research findings in pycnodysostosis and gene mutations of cathepsin K from 1996 to 2011

Yang Xue, Tao Cai, Songtao Shi, Weiguang Wang, Yanli Zhang, Tianqiu Mao, Xiaohong Duan

Open Access Review

X-linked disorders with cerebellar dysgenesis

Ginevra Zanni, Enrico S Bertini

Open Access Review

Congenitally corrected transposition

Gonzalo A Wallis, Diane Debich-Spicer, Robert H Anderson

Open Access Research

Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia

Maureen A Lefton-Greif, Thomas O Crawford, Sharon McGrath-Morrow, Kathryn A Carson, Howard M Lederman

Open Access Review

Glucose-6-phosphatase deficiency

Roseline Froissart, Monique Piraud, Alix Mollet Boudjemline, Christine Vianey-Saban, François Petit, Aurélie Hubert-Buron, Pascale Trioche Eberschweiler, Vincent Gajdos, Philippe Labrune

Open Access Research

Population pharmacokinetics and pharmacodynamics of hydroxyurea in sickle cell anemia patients, a basis for optimizing the dosing regimen

Ines Paule, Hind Sassi, Anoosha Habibi, Kim PD Pham, Dora Bachir, Frédéric Galactéros, Pascal Girard, Anne Hulin, Michel Tod

Open Access Review

Parental risk factors and anorectal malformations: systematic review and meta-analysis

Nadine Zwink, Ekkehart Jenetzky, Hermann Brenner

Open Access Research

Epithelial thymic tumours in paediatric age: a report from the TREP project

Elena Carretto, Alessandro Inserra, Andrea Ferrari, Massimo Conte, Andrea Di Cataldo, Roberta Migliorati, Giovanni Cecchetto, Gianni Bisogno

Open Access Research

A combination of transcriptome and methylation analyses reveals embryologically-relevant candidate genes in MRKH patients

Katharina Rall, Gianmaria Barresi, Michael Walter, Sven Poths, Karina Haebig, Karin Schaeferhoff, Birgitt Schoenfisch, Olaf Riess, Diethelm Wallwiener, Michael Bonin, Sara Brucker

Open Access Review

Autosomal dominant cerebellar ataxia type I: A review of the phenotypic and genotypic characteristics

Nathaniel Robb Whaley, Shinsuke Fujioka, Zbigniew K Wszolek

Open Access Review

Machado-Joseph Disease: from first descriptions to new perspectives

Conceição Bettencourt, Manuela Lima

Open Access Case Report

Pontine tegmental cap dysplasia: developmental and cognitive outcome in three adolescent patients

Marilena Briguglio, Lorenzo Pinelli, Lucio Giordano, Alessandro Ferraris, Eva Germanò, Serena Micheletti, Mariasavina Severino, Laura Bernardini, Sara Loddo, Gaetano Tortorella, Francesca Ormitti, Roberto Gasparotti, Andrea Rossi, Enza Maria Valente

Open Access Research

Complete exon sequencing of all known Usher syndrome genes greatly improves molecular diagnosis

Crystel Bonnet, M'hamed Grati, Sandrine Marlin, Jacqueline Levilliers, Jean-Pierre Hardelin, Marine Parodi, Magali Niasme-Grare, Diana Zelenika, Marc Délépine, Delphine Feldmann, Laurence Jonard, Aziz El-Amraoui, Dominique Weil, Bruno Delobel, Christophe Vincent, Hélène Dollfus, Marie-Madeleine Eliot, Albert David, Catherine Calais, Jacqueline Vigneron, Bettina Montaut-Verient, Dominique Bonneau, Jacques Dubin, Christel Thauvin, Alain Duvillard, Christine Francannet, Thierry Mom, Didier Lacombe, Françoise Duriez, Valérie Drouin-Garraud, Marie-Françoise Thuillier-Obstoy, Sabine Sigaudy, Anne-Marie Frances, Patrick Collignon, Georges Challe, Rémy Couderc, Mark Lathrop, José-Alain Sahel, Jean Weissenbach, Christine Petit, Françoise Denoyelle

Open Access Research

Molecular Genetic Analysis of the PLP1 Gene in 38 Families with PLP1-related disorders: Identification and Functional Characterization of 11 Novel PLP1 Mutations

Serena Grossi, Stefano Regis, Roberta Biancheri, Matthew Mort, Susanna Lualdi, Enrico Bertini, Graziella Uziel, Odile Boespflug-Tanguy, Alessandro Simonati, Fabio Corsolini, Ercan Demir, Valentina Marchiani, Antonio Percesepe, Franco Stanzial, Andrea Rossi, Catherine Vaurs-Barrière, David N Cooper, Mirella Filocamo

Open Access Research

Development of an ELISA for sensitive and specific detection of IgA autoantibodies against BP180 in pemphigoid diseases

Kinga Csorba, Sabine Schmidt, Florina Florea, Norito Ishii, Takashi Hashimoto, Michael Hertl, Sarolta Kárpáti, Leena Bruckner-Tuderman, Wataru Nishie, Cassian Sitaru

Open Access Review

Congenital neutropenia: diagnosis, molecular bases and patient management

Jean Donadieu, Odile Fenneteau, Blandine Beaupain, Nizar Mahlaoui, Christine Bellanné Chantelot

Open Access Review

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome

Hala Mégarbané, André Mégarbané

Open Access Research

Cognitive development in patients with Mucopolysaccharidosis type III (Sanfilippo syndrome)

Marlies J Valstar, Jan Pieter Marchal, Martha Grootenhuis, Vivian Colland, Frits A Wijburg

Open Access Research

"MY PKU": increasing self-management in patients with phenylketonuria. A randomized controlled trial

Amber E ten Hoedt, Carla EM Hollak, Carolien CA Boelen, N Ada P van der Herberg-van de Wetering, Nienke M ter Horst, Cora F Jonkers, Frits A Wijburg, Annet M Bosch

Open Access Research

Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Deniz Güngör, Juna M de Vries, Wim CJ Hop, Arnold JJ Reuser, Pieter A van Doorn, Ans T van der Ploeg, Marloes LC Hagemans

Open Access Research

TRPV4 related skeletal dysplasias: a phenotypic spectrum highlighted byclinical, radiographic, and molecular studies in 21 new families

Elena Andreucci, Salim Aftimos, Melanie Alcausin, Eric Haan, Warwick Hunter, Peter Kannu, Bronwyn Kerr, George McGillivray, RJ McKinlay Gardner, Maria G Patricelli, David Sillence, Elizabeth Thompson, Margaret Zacharin, Andreas Zankl, Shireen R Lamandé, Ravi Savarirayan

Open Access Case Report

9q22 Deletion - First Familial Case

Linda Siggberg, Maarit Peippo, Marjatta Sipponen, Taina Miikkulainen, Keiko Shimojima, Toshiyuki Yamamoto, Jaakko Ignatius, Sakari Knuutila

Open Access Research

Phenotypic variability of the kyphoscoliotic type of Ehlers-Danlos syndrome (EDS VIA): clinical, molecular and biochemical delineation

Marianne Rohrbach, Anthony Vandersteen, Uluç Yiş, Gul Serdaroglu, Esra Ataman, Maya Chopra, Sixto Garcia, Kristi Jones, Ariana Kariminejad, Marius Kraenzlin, Carlo Marcelis, Matthias Baumgartner, Cecilia Giunta

Open Access Research

Mutation spectrum of MLL2 in a cohort of kabuki syndrome patients

Lucia Micale, Bartolomeo Augello, Carmela Fusco, Angelo Selicorni, Maria N Loviglio, Margherita Cirillo Silengo, Alexandre Reymond, Barbara Gumiero, Federica Zucchetti, Ester V D'Addetta, Elga Belligni, Alessia Calcagnì, Maria C Digilio, Bruno Dallapiccola, Francesca Faravelli, Francesca Forzano, Maria Accadia, Aldo Bonfante, Maurizio Clementi, Cecilia Daolio, Sofia Douzgou, Paola Ferrari, Rita Fischetto, Livia Garavelli, Elisabetta Lapi, Teresa Mattina, Daniela Melis, Maria G Patricelli, Manuela Priolo, Paolo Prontera, Alessandra Renieri, Maria A Mencarelli, Gioacchino Scarano, Matteo della Monica, Benedetta Toschi, Licia Turolla, Alessandra Vancini, Adriana Zatterale, Orazio Gabrielli, Leopoldo Zelante, Giuseppe Merla

Open Access Research

Efficacy and outcome of expanded newborn screening for metabolic diseases - Report of 10 years from South-West Germany *

Martin Lindner, Gwendolyn Gramer, Gisela Haege, Junmin Fang-Hoffmann, Karl O Schwab, Uta Tacke, Friedrich K Trefz, Eugen Mengel, Udo Wendel, Michael Leichsenring, Peter Burgard, Georg F Hoffmann

Open Access Research

Oxytocin may be useful to increase trust in others and decrease disruptive behaviours in patients with Prader-Willi syndrome: a randomised placebo-controlled trial in 24 patients

Maïthe Tauber, Carine Mantoulan, Pierre Copet, Joseba Jauregui, Genevieve Demeer, Gwenaëlle Diene, Bernadette Rogé, Virginie Laurier, Virginie Ehlinger, Catherine Arnaud, Catherine Molinas, Denise Thuilleaux

Open Access Research

Incidence, Phenotypic Features and Molecular Genetics of Kallmann Syndrome in Finland

Eeva-Maria Laitinen, Kirsi Vaaralahti, Johanna Tommiska, Elina Eklund, Mari Tervaniemi, Leena Valanne, Taneli Raivio

Open Access Research

At-Risk Phenotype of Neurofibromatose-1 Patients: A Multicentre Case-Control Study

Emilie Sbidian, Sylvie Bastuji-Garin, Laurence Valeyrie-Allanore, Salah Ferkal, Jean P Lefaucheur, Alain Drouet, Pierre Brugière, Cédric Vialette, Patrick Combemale, Sébastien Barbarot, Pierre Wolkenstein

Open Access Review

Classification, diagnosis and potential mechanisms in Pontocerebellar Hypoplasia

Yasmin Namavar, Peter G Barth, Bwee Tien Poll-The, Frank Baas

Open Access Research

Evaluation of SHOX copy number variations in patients with Müllerian aplasia

Maria Sandbacka, Mervi Halttunen, Varpu Jokimaa, Kristiina Aittomäki, Hannele Laivuori

Open Access Research

Enzyme replacement therapy and/or hematopoietic stem cell transplantation at diagnosis in patients with mucopolysaccharidosis type I: results of a European consensus procedure

Minke H de Ru, Jaap J Boelens, Anibh M Das, Simon A Jones, Johanna H van der Lee, Nizar Mahlaoui, Eugen Mengel, Martin Offringa, Anne O'Meara, Rossella Parini, Attilio Rovelli, Karl-Walter Sykora, Vassili Valayannopoulos, Ashok Vellodi, Robert F Wynn, Frits A Wijburg

Open Access Review

VACTERL/VATER Association

Benjamin D Solomon

Open Access Editorial

Awakening Australia to Rare Diseases: Symposium report and preliminary outcomes

Hugh JS Dawkins, Caron M Molster, Leanne M Youngs, Peter C O'Leary

Open Access Research

Two new Rett syndrome families and review of the literature: expanding the knowledge of MECP2 frameshift mutations

Kirstine Ravn, Gitte Roende, Morten Duno, Kathrine Fuglsang, Kristin L Eiklid, Zeynep Tümer, Jytte B Nielsen, Ola H Skjeldal

Open Access Research

Genome-wide association study of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Europe

Emmanuelle Génin, Martin Schumacher, Jean-Claude Roujeau, Luigi Naldi, Yvonne Liss, Rémi Kazma, Peggy Sekula, Alain Hovnanian, Maja Mockenhaupt

Open Access Research

Understanding the impact of 1q21.1 copy number variant

Chansonette Harvard, Emma Strong, Eloi Mercier, Rita Colnaghi, Diana Alcantara, Eva Chow, Sally Martell, Christine Tyson, Monica Hrynchak, Barbara McGillivray, Sara Hamilton, Sandra Marles, Aziz Mhanni, Angelika J Dawson, Paul Pavlidis, Ying Qiao, Jeanette J Holden, Suzanne ME Lewis, Mark O'Driscoll, Evica Rajcan-Separovic

Open Access Research

Does market exclusivity hinder the development of Follow-on Orphan Medicinal Products in Europe?

Anne EM Brabers, Ellen HM Moors, Sonja van Weely, Remco LA de Vrueh

Open Access Research

Reducing selection bias in case-control studies from rare disease registries

J Alexander Cole, John S Taylor, Thomas N Hangartner, Neal J Weinreb, Pramod K Mistry, Aneal Khan

Open Access Research

Estimating the budget impact of orphan medicines in Europe: 2010 - 2020

Carina Schey, Tsveta Milanova, Adam Hutchings

Open Access Review

Atypical hemolytic uremic syndrome

Chantal Loirat, Véronique Frémeaux-Bacchi

Open Access Research

Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

Lucile Houyel, Babak Khoshnood, Robert H Anderson, Nathalie Lelong, Anne-Claire Thieulin, François Goffinet, Damien Bonnet

Open Access Review

Congenital hyperinsulinism: current trends in diagnosis and therapy

Jean-Baptiste Arnoux, Virginie Verkarre, Cécile Saint-Martin, Françoise Montravers, Anaïs Brassier, Vassili Valayannopoulos, Francis Brunelle, Jean-Christophe Fournet, Jean-Jacques Robert, Yves Aigrain, Christine Bellanné-Chantelot, Pascale de Lonlay

Open Access Research

Mutational screening of the USH2A gene in Spanish USH patients reveals 23 novel pathogenic mutations

Gema Garcia-Garcia, Maria J Aparisi, Teresa Jaijo, Regina Rodrigo, Ana M Leon, Almudena Avila-Fernandez, Fiona Blanco-Kelly, Sara Bernal, Rafael Navarro, Manuel Diaz-Llopis, Montserrat Baiget, Carmen Ayuso, Jose M Millan, Elena Aller

Open Access Review

Neuroacanthocytosis Syndromes

Hans H Jung, Adrian Danek, Ruth H Walker

Open Access Research

Therapy of Fabry disease with pharmacological chaperones: from in silico predictions to in vitro tests

Giuseppina Andreotti, Valentina Citro, Agostina De Crescenzo, Pierangelo Orlando, Marco Cammisa, Antonella Correra, Maria Vittoria Cubellis

Open Access Research

Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients

Bouwien E Smid, Saskia M Rombach, Johannes MFG Aerts, Symen Kuiper, Mina Mirzaian, Hermen S Overkleeft, Ben JHM Poorthuis, Carla EM Hollak, Johanna EM Groener, Gabor E Linthorst

Open Access Review

Xeroderma pigmentosum

Alan R Lehmann, David McGibbon, Miria Stefanini

Open Access Review

Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease

Maurizio Scarpa, Zsuzsanna Almássy, Michael Beck, Olaf Bodamer, Iain A Bruce, Linda De Meirleir, Nathalie Guffon, Encarna Guillén-Navarro, Pauline Hensman, Simon Jones, Wolfgang Kamin, Christoph Kampmann, Christina Lampe, Christine A Lavery, Elisa Leão Teles, Bianca Link, Allan M Lund, Gunilla Malm, Susanne Pitz, Michael Rothera, Catherine Stewart, Anna Tylki-Szymańska, Ans van der Ploeg, Robert Walker, Jiri Zeman, James E Wraith

Open Access Review

The hyperimmunoglobulin E syndrome - clinical manifestation diversity in primary immune deficiency

Aleksandra Szczawinska-Poplonyk, Zdzislawa Kycler, Barbara Pietrucha, Edyta Heropolitanska-Pliszka, Anna Breborowicz, Karolina Gerreth

Open Access Research

Ancient founder mutation is responsible for Imerslund-Gräsbeck Syndrome among diverse ethnicities

Cameron M Beech, Sandya Liyanarachchi, Nidhi P Shah, Amy C Sturm, May F Sadiq, Albert de la Chapelle, Stephan M Tanner

Open Access Review

Spinal muscular atrophy

Adele D'Amico, Eugenio Mercuri, Francesco D Tiziano, Enrico Bertini

Open Access Research

Sporadic and genetic forms of paediatric somatotropinoma: a retrospective analysis of seven cases and a review of the literature

Cécile Nozières, Pascale Berlier, Clémentine Dupuis, Catherine Raynaud-Ravni, Yves Morel, Françoise Borson Chazot, Marc Nicolino

Open Access Research

Exon duplications in the ATP7A gene: Frequency and Transcriptional Behaviour

Mie Mogensen, Tina Skjørringe, Hiroko Kodama, Kenneth Silver, Nina Horn, Lisbeth B Møller

Open Access Review

Benefits, challenges and obstacles of adaptive clinical trial designs

Shein-Chung Chow, Ralph Corey

Open Access Research

Coordinated multidisciplinary care for ambulatory Huntington's disease patients. Evaluation of 18 months of implementation

Ruth B Veenhuizen, Branda Kootstra, Wilma Vink, Janneke Posthumus, Pleuntje van Bekkum, Margriet Zijlstra, Jelleke Dokter

Open Access Review

Fibrodysplasia Ossificans Progressiva: Clinical and Genetic Aspects

Robert J Pignolo, Eileen M Shore, Frederick S Kaplan

Open Access Research

Population pharmacokinetics and pharmacodynamics of cysteamine in nephropathic cystinosis patients

Naïm Bouazza, Jean-Marc Tréluyer, Chris Ottolenghi, Saik Urien, Georges Deschenes, Daniel Ricquier, Patrick Niaudet, Bernadette Chadefaux-Vekemans

Open Access Review

Laryngo-tracheo-oesophageal clefts

Nicolas Leboulanger, Eréa-Noël Garabédian

Open Access Review

Type 1 autoimmune pancreatitis

Yoh Zen, Dimitrios P Bogdanos, Shigeyuki Kawa

Open Access Research

Osteogenesis imperfecta: the audiological phenotype lacks correlation with the genotype

Freya KR Swinnen, Paul J Coucke, Anne M De Paepe, Sofie Symoens, Fransiska Malfait, Filomena V Gentile, Luca Sangiorgi, Patrizia D'Eufemia, Mauro Celli, Ton JTM Garretsen, Cor WRJ Cremers, Ingeborg JM Dhooge, Els MR De Leenheer

Open Access Research

Congenital Dyserythropoietic Anemia Type II: molecular analysis and expression of the SEC23B Gene

Francesca Punzo, Aida M Bertoli-Avella, Saverio Scianguetta, Fulvio Della Ragione, Maddalena Casale, Luisa Ronzoni, Maria D Cappellini, Gianluca Forni, Ben A Oostra, Silverio Perrotta

Open Access Research

5'UTR mutations of ENG cause hereditary hemorrhagic telangiectasia

Kristy Damjanovich, Carmen Langa, Francisco J Blanco, Jamie McDonald, Luisa M Botella, Carmelo Bernabeu, Whitney Wooderchak-Donahue, David A Stevenson, Pinar Bayrak-Toydemir

Open Access Research

Biomarker analysis of Morquio syndrome: identification of disease state and drug responsive markers

Lisa Martell, Kelly Lau, Miranda Mei, Vicki Burnett, Celeste Decker, Erik D Foehr

Open Access Research

Anderson's disease/chylomicron retention disease in a Japanese patient with uniparental disomy 7 and a normal SAR1B gene protein coding sequence

Tomoo Okada, Michio Miyashita, Junji Fukuhara, Masahiko Sugitani, Takahiro Ueno, Marie-Elisabeth Samson-Bouma, Lawrence P Aggerbeck

Open Access Research

Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

Sophie Ng Wing Tin, Nadine Martin-Duverneuil, Ahmed Idbaih, Catherine Garel, Maria Ribeiro, Judith Landman Parker, Anne-Sophie Defachelles, Anne Lambilliotte, Mohamed Barkaoui, Martine Munzer, Martine Gardembas, Jean Sibilia, Patrick Lutz, Renato Fior, Michel Polak, Alain Robert, Olivier Aumaitre, Dominique Plantaz, Corinne Armari-Alla, Thierry Genereau, Perrine Marec Berard, Ghislain Nokam Talom, Jean-Loup Pennaforte, Hubert Ducou Le Pointe, Marie-Anne Barthez, Gérard Couillault, Julien Haroche, Karima Mokhtari, Jean Donadieu, Khê Hoang-Xuan

Open Access Research

Lidocaine for systemic sclerosis: a double-blind randomized clinical trial

Rachel Riera, Luís EC Andrade, Alexandre WS Souza, Cristiane Kayser, Edison T Yanagita, Virgínia FM Trevisani

Open Access Research

A locus-specific database for mutations in GDAP1 allows analysis of genotype-phenotype correlations in Charcot-Marie-Tooth diseases type 4A and 2K

Julien Cassereau, Arnaud Chevrollier, Dominique Bonneau, Christophe Verny, Vincent Procaccio, Pascal Reynier, Marc Ferré