نتایج جستجو برای: ectodermal dysplasia

تعداد نتایج: 30772  

Journal: :Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics 2004
Miguel Peñarrocha-Diago Roberto Uribe-Origone Javier Rambla-Ferrer Juan Guarinos-Carbó

We found few references in the dental literature on implant rehabilitation of patients with hypohidrotic ectodermal dysplasia and only 1 case on zygomatic fixations and maxillary prosthesis in a patient with hypohidrotic ectodermal dysplasia. Two zygomatic fixations were placed according to the sinus slot technique, together with 3 implants in the anterior maxillary region. After 6 months, an u...

2014
Sanjay Kumar Sinha Shailesh Chandra Gupta Brajesh Kumar

Ectodermal dysplasia is a group of systemic conditions that are congenital and are all caused by errors of/in ectoderm and the tissues arising of it. It constitutes of large and complex group of diseases characterized by triad of sparse hairs, abnormal or missing teeth and inability to sweat. Out of 170 ectodermal dysplasia described, <30 have been explained at molecular level with identifi cat...

Backgrounds: Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disorder, distinguished by hypotrichosis, hypohidrosis, and hypodontia. HDE can be inherited in X-linked recessive manner as a result of mutations in the ectodysplasin A (EDA) gene as well as autosomal dominant and autosomal recessive manners both of them caused by mutations in EDA receptor (EDAR) and EDAR-associated death d...

2016
Toshiyuki Yamamoto Keiko Miura Hiroo Yokozeki

Eccrine syringofibroadenoma (ESFA) is a rare tumor originated from eccrine ductal portion [1,2]. Clinically, ESFA presents a solitary papule, nodule or plaque to multiple lesions. Cases with multiple papular or macular lesions have been often associated with hereditary ectodermal dysplasia [3]. Recent findings have classified ESFA into several groups including, solitary ESFA, multiple ESFA with...

Journal: :Indian journal of dermatology, venereology and leprology 2012
Adone Baroni Vincenzo Piccolo Rosa Di Maio Teresa Russo

2015
Mehmet-Sinan Doğan Michele Callea İzzet Yavuz Orhan Aksoy Gabriella Clarich Ayşe Günay Ahmet Günay Sedat Güven Michele Maglione Zeki Akkuş

BACKGROUND This study aimed to review the results related to head and jaw disorders in cases of ectodermal dysplasia. The evaluation of ectodermal dysplasia cases was made by clinical examination and examination of the jaw and facial areas radiologically and on cone-beam 3-dimensional dental tomography (CBCT) images. MATERIAL AND METHODS In the 36 cases evaluated in the study, typical clinica...

2015
Suja Joseph George J Cherackal Jose Jacob Alex K Varghese

Hypohydrotic ectodermal dysplasia is a hereditary disorder, which affects ectodermal derivatives. It manifests several abnormalities of the teeth, and is commonly inherited through female carriers. This case report presents a patient with compromised esthetics and function. A multidisciplinary approach was planned involving an oral pathologist, endodontist, orthodontist and a prosthodontist.

Journal: :European journal of paediatric dentistry : official journal of European Academy of Paediatric Dentistry 2014
E Sfeir N Nassif C Moukarzel

BACKGROUND Ectodermal dysplasia is a hereditary genodermatosis characterised by a congenital defect of ectodermal structures, causing tooth malformations and anomalies. Implantology has become accepted in these subjects. However cases are often complicated by a reduction in the size of the alveolar process, making the insertion of conventional implants difficult without bone grafting. The reduc...

Journal: :Acta biochimica Polonica 2003
Agnieszka Kobielak Krzysztof Kobielak Barbara Biedziak Wieslaw H Trzeciak

The structure of the EDA1 gene was investigated in a patient with anhidrotic ectodermal dysplasia. Sequence analysis revealed a novel A1270G transition in exon 9 of the EDA1 gene in the patient and his uncle, whereas the patient's mother and grandmother were heterozygotes. This mutation resulted in Tyr343Cys substitution in the extracellular domain of the EDA1 gene product - ectodysplasin-A. Th...

2003
Gilles Courtois Asma Smahi Janine Reichenbach Rainer Döffinger Caterina Cancrini Marion Bonnet Anne Puel Christine Chable-Bessia Shoji Yamaoka Jacqueline Feinberg Sophie Dupuis-Girod Christine Bodemer Susanna Livadiotti Francesco Novelli Paolo Rossi Alain Fischer Alain Israël Arnold Munnich Françoise Le Deist Jean-Laurent Casanova

The Journal of Clinical Investigation | October 2003 | Volume 112 | Number 7 Introduction Patients with anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) present with impaired development of skin appendices, resulting in sparse hair, conical teeth, and anhidrosis/hypohidrosis. Host defense is also impaired, resulting principally in multiple and severe bacterial diseases (1). X-link...

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