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	Proteus Syndrome
		Proteus Syndrome (Wiedemann's Syndrome; Elephant Man Disease; Partial Gigantism of Hands and Feet with Nevi, Hemihypertrophy, and Macrocephaly)
		Proteus syndrome with infantile myofibromatosis and severely disturbed adipose growth and differentiation
		Proteus Syndrome: Diagnostic Criteria, Differential Diagnosis, and Patient Evaluation
	www.bdid.com /proteus.htm (57 words)

	Proteus syndrome
		Proteus Syndrome is condition which involves atypical growth of the bones, skin, head, and a variety of other symptoms.
		While evidence of this syndrome has been cited in medical literature as early as 1907, recognition has been difficult due to the variability of the manifestations and the rarity of the syndrome.
		Proteus Syndrome foundation is a not-for- profit voluntary organization, and it's ongoing existence is dependent on donations from families, professionals, businesses, and other organizations.
	www.kumc.edu /gec/support/protwww.html (500 words)

	European Journal of Human Genetics - The challenges of Proteus syndrome: diagnosis and management
		Proteus syndrome (PS) is a disorder of patchy or mosaic postnatal overgrowth of unknown etiology.
		Proteus syndrome (PS) is an extremely rare and overdiagnosed disorder of mosaic growth dysregulation, primarily involving overgrowth.
		Of course, the syndrome is not 'new' and the oldest known case is that of Mr Joseph Merrick, described by Treves in the 19th century.
	www.nature.com /ejhg/journal/v14/n11/full/5201638a.html (4563 words)

	Proteus Syndrome: Diagnostic Criteria, Differential Diagnosis, and Patient Evaluation - Office of Rare Diseases (Site not responding. Last check: 2007-11-05)
		Proteus syndrome was originally described in two patients by Cohen and Hayden [1979] as a newly recognized disorder characterized by overgrowth of multiple tissues, connective tissue nevi, epidermal nevi, and hyperostoses.
		Lipomas in Proteus syndrome are composed principally of mature adipocytes.
		Type I neurofibromatosis and Bannayan-Riley-Ruvalcaba syndrome are distinct from Proteus syndrome and are understood at the molecular level, mutations occurring respectively in the NF1 gene and the PTEN gene [Cohen, 1998a].
	rarediseases.info.nih.gov /html/workshops/workshops/proteus980319.html (3238 words)

	Proteus syndrome: Encyclopedia of Genetic Disorders
		Proteus syndrome is characterized by excessive growth of cells.
		The disorder gained wide recognition when it became publicized that Joseph (John) Merrick, the person depicted in the movie The Elephant Man, probably had Proteus syndrome.
		The excess growth of tissue that characterizes Proteus syndrome is progressive.
	health.enotes.com /genetic-disorders-encyclopedia/proteus-syndrome (140 words)

	Proteus Syndrome: Symptoms and Cases of "Elephant Man Disease"
		Proteus Syndrome is one of the most debilitating conditions known to man. It can be responsible for massive distortion of the body and may often be fatal.
		Joseph Merrick¹s pathology resembles each of the individuals who have been diagnosed with Proteus, but because he managed to survive until the age of 29 without treatment beyond some simple excisions, the condition was able to progress to such a state as to render him nearly unidentifiable as a human.
		He never experienced the mentally debilitating aspects of Proteus, however, and Treves acknowledges that he did have an intellect and an imagination that was perfectly normal, in fact, he was an avid theatre-goer, and enjoyed having intelligent discussions.
	evergreen.27names.org /academia/elephant.txt (2808 words)

	Proteus Syndrome
		Proteus syndrome is thought to be caused by a mutation in a growth regulatory gene that occurs after fertilization of the embryo.
		Klippel-Trenaunay syndrome is thought to be a hereditary disorder combining a birth mark which is the color of a port wine stain (nevus flammeus), excessive growth of soft tissue and bone, and varicose veins.
		Cowden syndrome (multiple hamartoma syndrome) is a hereditary disorder characterized by multiple nodules composed of an overgrowth of mature cells and tissues that occur in the affected part of the body.
	hw.healthdialog.com /kbase/nord/nord579.htm (1238 words)

	ORPHANET - About rare diseases - About orphan drugs (Site not responding. Last check: 2007-11-05)
		Proteus syndrome (PS) is a sporadic disorder affecting tissue derived from any germinal layer and resulting in tissue overgrowth in a mosaic pattern.
		Although germline PTEN mutations (the PTEN gene is responsible for Bannayan-Zonana syndrome) have been found in some PS cases, this mutation was not identified in most reported patients, and it seems unlikely to have a major role in the etiology of PS.
		This would explain the genetic basis of syndromes such as PS, which are often characterized by sporadic occurrence, distribution of lesions in a scattered or asymmetrical pattern, variable extent of involvement and lack of diffuse involvement of entire organs.
	www.orpha.net /consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=744 (495 words)

	Medical Dictionary: Proteus syndrome - WrongDiagnosis.com - WrongDiagnosis.com
		Proteus syndrome: An overgrowth syndrome with a wide spectrum of abnormalities, including gigantism of the hands and/or feet, pigmented nevi with variable distribution, hemihypertrophy which may be limited to face, ears, shoulder girdle, thorax, and arms or involve the entire side of the body.
		Proteus syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
		This means that Proteus syndrome, or a subtype of Proteus syndrome, affects less than 200,000 people in the US population.
	www.wrongdiagnosis.com /medical/proteus_syndrome_printer.htm (392 words)

	Proteus syndrome Case Reports Pediatric Oncall
		As the son of Poseidon, Proteus possessed the ability to transform himself into any shape to avoid prophesying the futures of the mortals who hounded him.
		The morbidity and mortality risk of pulmonary cysts in Proteus syndrome is unknown.
		The proteus syndrome: The emergence of an entity.
	www.pediatriconcall.com /fordoctor/casereports/Proteus_syndrome.asp (1034 words)

	eMedicine - Proteus Syndrome : Article by Matthew J Mahlberg, MD
		Although some evidence of this syndrome was published in the medical literature as early as 1907, the modern medical description of the disease is attributed to Cohen and Hayden, who identified the syndrome in 1979 (Cohen, 1979).
		It is characterized by a diffuse capillary blushing, warmth, and underlying arteriovenous shunts.
		Bannayan-Riley-Ruvalcaba syndrome, which is characterized by macrocephaly, lipomas, capillary malformations, and polyposis of the colon and rectum, does not cause asymmetric growth, cranial exostoses, epidermal nevi, or palmar or plantar changes.
	www.emedicine.com /derm/topic721.htm (3665 words)

	Rare Pediatric Disease Database (Site not responding. Last check: 2007-11-05)
		Proteus Syndrome is characterized by overgrowth of multiple parts of the body including the skin, bones, organs (such as the spleen), and other tissues.
		Proteus syndrome is a rare genetic disorder with less than one in a million people being affected.
		Because of changes in the vessels caused by Proteus syndrome and because of decreased mobility, affected children are also at risk for blood clots.
	www.madisonsfoundation.org /content/3/1/display.asp?did=656 (731 words)

	PROTEUS SYNDROME: Contact a Family - for families with disabled children: information on rare syndromes and disorders
		Proteus syndrome is a rare disorder first identified in 1979 but with cases described in the literature since 1856.
		The clinical features of Proteus syndrome are overgrowth/enlargement of soft tissue and bone which can affect any area of the body but often involving the hands and/or the feet, the skull and sometimes the whole of one side of the body (hemihypertrophy) and blood vessel (vascular) abnormalities.
		The abnormalities seen in Proteus syndrome are present at birth but may become more apparent and develop with age.
	www.cafamily.org.uk /Direct/p50.html (672 words)

	Diagnostic Radiology - Special Cases - Proteus Syndrome
		Proteus Syndrome is a disturbance of cell growth including benign tumors under the skin, overgrowth of the body -- often more on one side than the other (hemihypertrophy) -- and overgrowth of fingers (macrodactyly).
		The syndrome is named after the Greek god Proteus, the polymorphous who could change his appearance.
		The Elephant Man (John Merrick) of 19th century England, who was thought to have had neurofibromatosis, instead probably suffered from Proteus Syndrome.
	www.cc.nih.gov /drd/ps.html (189 words)

	genome.gov \| Biesecker Lab
		In the laboratory, his group performs classical positional cloning studies to find the genes that are altered in these syndromes, determines genotype-phenotype correlations, and uses animal models to investigate the pathogenetic mechanisms of these disorders.
		It is believed that the many manifestations of Proteus syndrome - lipomas, several types of nerve disorders, and partial gigantism (with limb or digit overgrowth) - result from somatic mosaicism of a dominant lethal gene, but the gene locus has yet to be identified.
		They are also determining the range of manifestations, severity, and natural history of Proteus syndrome with a longitudinal study and are using microarray expression analysis, representational difference analysis, and two-dimensional Southern blot analysis to characterize gene alterations.
	www.genome.gov /10000356 (638 words)

	Proteus syndrome - humpath.com - a website dedicated to human pathology.
		Proteus syndrome - humpath.com - a website dedicated to human pathology.
		Proteus syndrome is a rare, sporadic disorder that causes postnatal overgrowth of multiple tissues in a mosaic pattern.
		Various benign and malignant tumors and hamartomas may complicate the clinical course of patients with the syndrome.
	www.humpath.com /article.php3?id_article=726 (129 words)

	Proteus syndrome. DermNet NZ
		Proteus syndrome is also known as elattoproteus syndrome and elephant man disease.
		The syndrome became widely recognised after the release of the movie “The Elephant Man”, a screenplay depicting the life of Joseph Merrick who was thought to have neurofibromatosis, but is now believed to actually have had severe Proteus syndrome.
		Proteus syndrome is an extremely rare genetic disorder that is characterised by the abnormal growth of bones and blood vessels, and various skin lesions including lipomas, epidermal naevi and café au lait macules.
	www.dermnetnz.org /systemic/proteus.html (483 words)

	Alex Hoag Run for Sunshine 5K run - Home Page - The proceeds from this run benefits the Proteus Syndrome Foundation
		In the form of a pulmonary embolism, a complication of PS Alex died unexpectedly on a rainy Monday afternoon.
		The Proteus Syndrome Foundation is a worldwide support group founded by Alex Hoag's mom in 1991.
		The cause of Proteus Syndrome is unknown, the effects of Proteus Syndrome on children and adults is devastating.
	www.alexhoagrun.org /default.htm (522 words)

	Proteus syndrome (PS)
		An overgrowth syndrome with a wide spectrum of abnormalities, including gigantism of the hands and/or feet, pigmented nevi with variable distribution, hemihypertrophy which may be limited to face, ears, shoulder girdle, thorax, and arms or involve the entire side of the body.
		A localized form is referred to as the Fishman syndrome.
		Head and neck: Skull abnormalities are progressive and include macrocephaly and/or frontal bossing, hyperostoses, and prognathism which, together with unilateral cranial and facial subcutaneous tumors and asymmetric tissue overgrowth gives the face a typical bizarre appearance.
	www.nlm.nih.gov /mesh/jablonski/syndromes/syndrome540.html (433 words)

	Jordan - The Boy with Proteus Syndrome
		It is believed that Proteus Syndrome is the same condition that Joseph Merrick, the Elephant Man, suffered from.
		Proteus Syndrome is not an inherited condition, it's caused by a, little understood, fault which occurs in the genes of the developing embryo.
		Because of the nature of the condition, each child with Proteus Syndrome is affected differently.
	www.mymultiplesclerosis.co.uk /misc/proteus-syndrome.html (787 words)

	Proteus Syndrome
		Bilateral ovarian cystadenomas are regarded as having diagnostic value in Proteus syndrome when occurring within the first two decades of life.
		We describe a 3-year-old girl with Proteus syndrome who developed bilateral paraovarian villoglandular endometrioid cystadenomatous tumors of borderline malignancy (low malignant potential) of the broad ligament.
		This is the first recognized example of a cystic borderline epithelial tumor of the female genital tract and the first paraovarian tumor reported in a patient with Proteus syndrome.
	www.thedoctorsdoctor.com /diseases/proteus_syndrome.htm (407 words)

	Proteus syndrome and immunodeficiency -- Hodge et al. 82 (3): 234 -- Archives of Disease in Childhood
		Proteus syndrome and immunodeficiency -- Hodge et al.
		A 10 year old boy with Proteus syndrome presented with a pericardial effusion of unknown aetiology.
		Proteus syndrome is a sporadically occurring hamartomatous syndrome first described in 1979
	www.fetalneonatal.com /cgi/content/full/82/3/234 (878 words)

	Proteus Syndrome Foundation
		We will be featuring family stories, medical articles, and all up to date information concerning Proteus syndrome and the families it affects.
		There will be much more follow up writing on the conference and many of the presenters have donated their presentations for publication on this website which will be posted soon.
		The Proteus Syndrome Foundation has been founded to support and educate familes and professionals, and to raise money for research to find a cure for individuals living with Proteus Syndrome.
	www.proteus-syndrome.org (187 words)

	eMedicine - Proteus Syndrome : Article by Beth A Pletcher, MD (Site not responding. Last check: 2007-11-05)
		Background: Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder.
		A coagulation workup may be indicated preoperatively for patients with Proteus syndrome in light of the apparent increased risks for thrombotic events such as DVT or PE.
		Plotz SG, Abeck D, Plotz W, Ring J: Proteus syndrome with widespread portwine stain naevus.
	www.emedicine.com /ped/topic1912.htm (2902 words)

	healthfinder.gov — Proteus Syndrome Foundation
		The Proteus Syndrome Foundation is a non-profit organization founded to support and educate families and professionals, and to raise money for research to find a cure for individuals living with Proteus Syndrome.
		The main goals of the Foundation are patient and family support, public education, medical staff education, promotion and funding of research, and ultimately the treatment and cure of Proteus Syndrome.
		The organization provides consumer and professional information about Proteus Syndrome, a condition which involves atypical growth of the bones, skin, head and a variety of other symptoms.
	www.healthfinder.gov /orgs/HR3745.htm (100 words)

	MedPix 3687: Proteus Syndrome - Medical Image Database and Atlas
		Proteus Syndrome is a newly described genetic disorder characterized by abnormalities in several organ systems, including most prominently skin and musculoskeletal.
		Now, many scientist consider that he had Proteus Syndrome.
		According to the OMIM, Proteus Syndrome (PS) is characterized by "generalized, unilateral or localized overgrowth of any tissue hemihypertrophy" caused by a "somatic mosaic, sporadic" mutation.
	rad.usuhs.edu /medpix/medpix.html?mode=single&recnum=3687 (270 words)

	The Boy with Proteus Syndrome from Channel4.com (Site not responding. Last check: 2007-11-05)
		They exist to help the families affected by Proteus and provide emotional support through family meetings, quarterly newsletters, family bulletins and access to the library of medical articles on Proteus Syndrome.
		A network for families and individuals affected by the syndrome to enable them to have contact with other families in similar circumstances.
		They support ongoing research into the causes of Proteus, provide information on specialist clothing and footwear, publish regular newsletters and host family get-togethers.
	www.channel4.com /health/microsites/B/boy_in_a_million/support.html (134 words)

	Discovery Health :: Frequently Asked Questions About Proteus Syndrome
		In 1998, a panel of doctors at the National Institutes of Health surmised that Joseph Merrick's affliction may have been caused by a condition known as Proteus syndrome.
		A: Proteus syndrome is likely caused by several different genes, with PTEN being only one of them.
		When an individual with Proteus syndrome is found to have a germline PTEN mutation, he or she has a 50 percent likelihood of passing on the PTEN mutation.
	health.discovery.com /convergence/elephantman/proteusfaq.html (328 words)

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