Extractions: (advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Dermatology Pediatric Diseases Last Updated: January 14, 2004 Rate this Article Email to a Colleague Synonyms and related keywords: hereditary ectodermal dysplasia, ectodermal dysplasia syndrome, EDS, ED, ectodermal birth defect, ectoderm birth defect, ectodermal disorder, ectoderm disorder, hypohidrotic ectodermal dysplasia, hypohidrotic ED, hidrotic ectodermal dysplasia, hidrotic ED, anhidrotic ectodermal dysplasia, anhidrotic ED, facial defect, cleft lip, cleft palate, EEC syndrome, AEC syndrome, Rapp-Hodgkin syndrome, Hay-Wells syndrome, Christ-Siemens-Touraine syndrome, Clouston syndrome AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography
Ectrodactyly - Ectodermal Dysplasia Other characters, Ectrodactyly ectodermal dysplasia,. Print this article, See ectodermal dysplasia. HC. The Encyclopaedia of Medical Imaging Volume VII. http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/ECTRODACTYLY E
Extractions: Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Ectrodactyly - ectodermal dysplasia, a claw hand or claw foot with or without syndactyly and polydactyly which may be associated with a cleft lip and palate. The radiological manifestations are those of the claw hand and foot. There is a high association with urinary tract abnormalities, which are variable and include duplication anomalies, hydronephrosis and agenesis. See ectodermal dysplasia
Ectodermal Dysplasia Browse entry words starting with A, B, C, D, E, F, G, H, I, J, K, L, M, N, O, P, Q, R, S, T, U, V, W, X, Y, Z, Other characters, ectodermal dysplasia,. Print this article, http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/ECTODERMAL DYSPL
Extractions: Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Ectodermal dysplasia, hypohidrosis, hypotrichosis and dental abnormalities with an abnormal facies which consists of frontal bossing and prominent supraorbital ridges with protruberant lips are the clinical features. Radiologically, the main abnormalities are associated with deficient dentition.
The Ectodermal Dysplasia Society - Patient UK The ectodermal dysplasia Society Patient UK. A directory of UK that provide patient information. The ectodermal dysplasia Society. http://www.patient.co.uk/showdoc.asp?doc=26739375
Ectodermal Dysplasia - Patient UK ectodermal dysplasia Patient UK. A directory of UK health, disease medical websites that provide patient information. ectodermal dysplasia. http://www.patient.co.uk/ectodermal_dysplasia.htm
The Andy Fund ectodermal dysplasia ectodermal dysplasia is a rare group of inherited disorders. The disorders arise from disturbances in one or http://www.andy.org.mx/eng/content/ectodermal_dysplasia.htm
Extractions: Ectodermal Dysplasia is a rare group of inherited disorders. The disorders arise from disturbances in one or more ectodermal structures and their accessory appendages. The absence of, or deficient function of, at least two derivatives of the ectoderm constitutes a form of ED. Each combination of defects represents another type of ED and has a specific name. At least 150 different forms of Ed have been identified. Individuals affected by ED have at least two or more of the following manifestations: 1.Absent or malfunctioning sweat glands. The patient may suffer from dry skin, yperthermia, and unexplained high fever as a result of the deficiency of sweat glands. 2.Dental anomalies include missing or underdeveloped teeth
1996.07.29 : Gene For Anhidrotic Ectodermal Dysplasia Identified Gene for Anhidrotic ectodermal dysplasia Identified. As was noted by Darwin, the Xlinked form of ectodermal dysplasia is associated with male children. http://www.hhs.gov/news/press/1996pres/960729.html
Extractions: This is an archive page. The links are no longer being updated. Date: Monday, July 29, 1996 FOR IMMEDIATE RELEASE Contact: Wayne Little (301)496-4261 Still, little is known about what occurs at the molecular level to ultimately produce the unique clinical features of EDA. The name itself refers to the abnormal development (dysplasia) of tissues that are derived from ectoderm, the outermost of the three primary layers of the embryo from which is derived, among other structures, the skin, hair, nails, sweat glands, and teeth. The term "anhidrotic" refers to the underdevelopment or absence of sweat glands, a condition that can be life threatening because it prevents the body from cooling down during fevers or periods of excessive heat. Other features that affect the quality of life for these young children include baldness and often the need for dentures or dental implants to compensate for the absence of teeth. The investigators identified a gene within this region of the X chromosome that contains the molecular code to produce a 135-amino acid protein. This novel protein has a predicted structure compatible with a transmembrane molecule, a protein that is part of the cell membrane and falls within the category of receptors, growth factors, or adherence molecules. The protein was detected in fetal tissues and certain types of adult skin cells that are consistent with the pathology of EDA. The function of the protein remains unknown, but some exciting hints are provided by a strain of mouse called Tabby, named for its unusual pattern of hair markings. The mice have a mutation in the X chromosome region that is the mouse equivalent of the human EDA gene location. The mice are afflicted with some of the same symptoms that appear in humans, including missing sweat glands and abnormally shaped and/or missing teeth.
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Ectodermal Dysplasia Articles, Support Groups, And Resources ectodermal dysplasia articles, support groups, and resources for patients from Med Help International (www.medhelp.org). ectodermal dysplasia. http://www.medhelp.org/HealthTopics/Ectodermal_Dysplasia.html
ECTODERMAL DYSPLASIA ectodermal dysplasia A hereditary condition (most often x linked) that is characterized by the abnormal development of skin, absence of sweat glands, dry eyes http://www.medhelp.org/glossary2/new/GLS_1774.HTM
Extractions: ECTODERMAL DYSPLASIA - A hereditary condition (most often x linked) that is characterized by the abnormal development of skin, absence of sweat glands dry eyes , and abnormal development of teeth. Symptoms include absent teeth, peg teeth, inability to sweat, thin skin, and heat intolerance. Mucous membrane involvement may result in a foul-smelling nasal discharge. The inability to sweat leads to the inability to maintain normal body temperature in a warm environment. Some may exhibit fevers and will require artificial cooling.
Congenital Anomalies And Dentistry This includes, patients with congenital anodontia, ectodermal dysplasia and dentinogenesis imperfecta. More ectodermal dysplasia In http://dentalimplants-usa.com/Conditions/congen.html
Extractions: Congenital Anomalies Congenital Anomalies are a broad category of health conditions that are present at birth and is a deviation from normal anatomic growth, development, or function. The congenital anomaly may have developed in utero or may have genetic origins. Research Program: The Institute for Facial Esthetics, in cooperation with Prosthodontics Intermedica's clinical care center in Fort Washington, is conducting a research program for patients with congenitally missing teeth. This includes, patients with congenital anodontia, ectodermal dysplasia and dentinogenesis imperfecta. More information about this program Anodontia is defined as the failure of teeth to form, completely or partially. Ectodermal Dysplasia: In congenital ectodermal dysplasia, teeth are absent or conical in appearance so that from childhood, partial dentures are needed. Congenital ectodermal dysplasia is the result of an hereditary fault affecting the stage of initiation of various specialized ectodermal structures. It is characterized by one or all of the following: Anodontia - partial or total Absence of sweat or sebaceous glands. The skin is smooth, atrophic and wrinkled. The absence of sweat glands makes the child extremely susceptible to heat prostration since heat regulation by sweating is absent.
Ectodermal Dysplasia More about ectodermal dysplasia. CAUSE OF ectodermal dysplasia ectodermal dysplasia is caused by a single abnormal gene or pair of abnormal genes. http://dentalimplants-usa.com/Conditions/ectod.html
Extractions: More about Ectodermal Dysplasia CAUSE OF ECTODERMAL DYSPLASIA: Ectodermal Dysplasia is caused by a single abnormal gene or pair of abnormal genes. The chance for parents to have an affected child depends on the type of Ectodermal Dysplasia that exists in the family. In some families the mistake in the gene was a fresh mistake (mutation) in their child and the likelihood of another child being affected is very low. There are other families however that have an inherited form of Ectodermal Dysplasia with recurrence risk ranging from 25% to 50%. SWEAT GLANDS: Diminished or absent sweating is a common problem. The sweat glands are absent, reduced in number, or may not function normally. Reduced sweating may result in very high fevers, because the body regulates its temperature by sweating. Often, the first clue that the sweat glands are absent or are not functioning normally is an elevated temperature. Elevations in body temperature are often caused by high environmental temperatures, excessive
Extractions: Experimental Ophthalmology Unit Eye Problems and Ectodermal Dysplasia Feedback: ophthalmology@liv.ac.uk An ectodermal dysplasia (ED) is disorder that results from abnormal formation of the ectodermal layer in the developing embryo. The ectoderm of the embryo forms the hair, teeth, nails, sweat glands and parts of the eyes. The ectodermal dysplasias are a diverse group of conditions and over a 100 different types have been described. To understand the eye problems in ED, you must understand the basic structure and function of the eye. This page will briefly describe the anatomy of the eye and how the eye develops in the embryo, in particular the role of the ectoderm. How different structures and functions of the eye are affected by ED will be covered, leading into the treatments available for different ocular problems, and to finish, a look to the future. The eye consists not only of the globe but also the surrounding structures - the lids, the tear drainage passage and the tear or lacrimal gland. The globe of the eye consists of an outer coat, a middle layer and an inner layer. The outer coat is made up of a tough fibrous, white layer - the sclera, and a clear transparent window - the cornea. The middle layer contains pigment and forms the iris. The inner layer is the light seeing layer or retina. The lens is an oval disc which sits behind the pupil and iris.
Hypohidrotic Ectodermal Dysplasia Hypohidrotic ectodermal dysplasia Important It is possible that the main title of the report Hypohidrotic ectodermal dysplasia is not the name you expected. http://my.webmd.com/hw/health_guide_atoz/nord804.asp
Extractions: HED is usually inherited as an X-linked recessive genetic trait; in such cases, the disorder is fully expressed in males only. However, females who carry a single copy of the disease gene (heterozygote carreirs) may exhibit some of the symptoms and findings associated with the disorder. These may include absence and/or malformation of certain teeth, sparse hair, and/or reduced sweating. Researchers also have reported cases in which HED appears to be inherited as an autosomal recessive genetic trait. In such cases, the disorder is fully expressed in both males and females. Resources
Extractions: Ectrodactyly-Ectodermal Dysplasia-Cleft Lip/Palate (EEC Syndrome) is a rare form of ectodermal dysplasia inherited as an autosomal dominant genetic trait the symptoms of which can vary from mild to severe. The most common symptoms found in patients with EEC Syndrome are: missing or irregular fingers and/or toes (ectrodactyly), abnormalities of the hair and glands, cleft lip and/or palate, or unusual facial features, as well as abnormalities of the eyes and urinary tract. Resources
► Ectodermal Dysplasia Home Medical Reference Encyclopedia (English) Toggle English / Spanish ectodermal dysplasia. Alternative Names Anhidrotic ectodermal dysplasia. http://www.umm.edu/ency/article/001469.htm
Extractions: Causes, incidence, and risk factors: There are several different types of ectodermal dysplasia of which X-linked anhidrotic ectodermal dysplasia is the most common. This condition affects only males. An autosomal dominant form exists in which females and males are equally affected and is symptomatically almost identical to the X-linked form of the disease. Multiple genes have been discovered to cause ectodermal dysplasias. Ectodermal dysplasia may affect, to varying degrees, all of the structures associated with the development of the skin. Absent or decreased sweating in people with anhidrotic ectodermal dysplasia is caused by absence of sweat glands. Affected children have difficulty controlling fevers and mild illness may produce extremely high fevers, because of absent temperature regulation by sweating . Affected adults are unable to tolerate a warm environment and require special measures to maintain a normal body temperature
Ectodermal Dysplasia ectodermal dysplasia (ED) is a genetic birth defect which is defined by the irregular development of at least two structures derived from the ectoderm. http://www.parentsplace.com/expert/dentist/qas/0,,200533_106972,00.html
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Healthfinder® - Ectodermal Dysplasia Carefully selected government and nonprofit health information on ectodermal dysplasia. healthfinder® home page, National Foundation for ectodermal dysplasias. http://www.healthfinder.gov/Scripts/SearchContext.asp?topic=268
Extractions: University of California, San Francisco About UCSF Quick Links... Admissions Appointments Billing Calendar Clinical Trials Clinics Conditions and Treatments Contact a Patient Contact Us Directions and Maps For Health Professionals Jobs Make A Donation Medical Dictionary Medical Tests News Patient Profiles Specialized Services UCSF Medical Center Volunteer Services Search Treatment Signs and Symptoms Ectodermal dysplasia is a disorder involving two or more of the ectodermal structures, which include the skin, hair, nails, teeth, mucous membranes and sweat glands. How an individual is affected varies. For example, in one individual the hair and nails may be affected, while in another the disorder may involve the sweat glands and teeth. Each combination is considered a distinct type of ectodermal dysplasia. Last reviewed in June 2003 by experts at UCSF Children's Hospital.
GeneReviews: Hypohidrotic Ectodermal Dysplasia Your browser does not support HTML frames so you must view Hypohidrotic ectodermal dysplasia in a slightly less readable form. Please http://www.geneclinics.org/profiles/x-hed/
