41. Orthodontic And Orthopedic Treatment Of A Patient With Incontinentia Pigmenti Incontinentia pigmenti (IP), also called blochsulzberger syndrome or Bloch-Siemens syndrome, is a complex hereditary disease with ectodermal, neurological http://www.angle.org/anglonline/?request=get-document&issn=0003-3219&volume=073&

42. Incontinentia Pigmenti Synonyms. BlochSiemens-Sulzberger Syndrome; bloch-sulzberger syndrome; IP; Bloch-Siemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis; http://www.bchealthguide.org/kbase/nord/nord409.htm

document.write(''); var hwPrint=1; var hwDocHWID="nord409"; var hwDocTitle="Incontinentia Pigmenti"; var hwRank="1"; var hwSectionHWID="nord409"; var hwSectionTitle=""; var hwSource="cn6.0"; var hwProdCfgSerNo="wsh_html_031_s"; var hwDocType="NORD"; National Organization for Rare Disorders, Inc. Incontinentia Pigmenti Important It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Bloch-Siemens-Sulzberger Syndrome Bloch-Sulzberger Syndrome IP Bloch-Siemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis Pigmented Dermatosis, Siemens-Bloch type Disorder Subdivisions None Related Disorders List Information on the following diseases can be found in the Related Disorders section of this report: Incontinentia Pigmenti Achromians Franceschetti-Jadassohn Syndrome Caffey Disease Hypomelanosis of Ito General Discussion Incontinentia Pigmenti (IP) is a rare genetic dermatological disorder affecting the skin, hair, teeth, and central nervous system. Progressive skin changes occur in four stages, the first of which appears in early infancy or is present at birth. IP is inherited as an X-linked dominant trait. Symptoms More than 80 percent of individuals with Incontinentia Pigmenti have dental abnormalities. These abnormalities include a delay in the eruption of primary teeth; abnormal contours of teeth, giving them a peg-like or cone-shaped appearance; or the congenital absence of both primary and secondary teeth.

43. Hypomelanosis Of Ito - HITS (UK) (Specific Eye Conditions Web Site) Ectodermal Dysplasias, especially Naegeli Type; Incontinentia Pigmenti (blochsulzberger syndrome); Systematized Nevus Depigmentosus; Tuberous Sclerosis http://www.eyeconditions.org.uk/hits.htm

SP ecific E ye C ondition S www.eyeconditions.org.uk Hypomelanosis of Ito Syndrome HITS (UK) Family Support Network National Contact: Sandra Field, 33 Fernworthy Close, Torquay, Devon TQ2 7JQ Tel: London Contact: Terri Grant, Saskatchewan, 99 Great Cambridge Road, Corner of Mayfair Gardens, London N17 7LN Tel: E-Mail: tgrant@hitsuk.freeserve.co.uk Website: www.e-fervour.com/hits HITS (UK) was fully established in February 2000 and we are currently in the process of becoming a registered charity. We can offer support by letter, e-mail, internet chat site with monthly "live chats", telephone and family days. We also produce a newsletter 3 times a year. The aim of HITS (UK) is to enrich the lives of children and families affected by Hypomelanosis of Ito by encouraging communication, facilitating the flow of information between families and health professionals, and generally reducing the sense of isolation patients and families may experience. We are a voluntary, not-for-profit organisation, and we are holding the first ever "Children of Ito" Family day on July 15th 2001. We hope to hold the 2nd Children of Ito Family day in July 2003, for more information please contact Terri Grant at

44. Incontinentia Pigmenti (Diagnosen) Linksammlung Bei Medinfo.de Translate this page Synonym(s) bloch-sulzberger syndrome. Defekten Link melden (Aufnahmedatum 22.07.2003). Synonyme im weiteren Sinne Bloch-Sulzberger-Syndrom, http://www.medinfo.de/index.asp?r=706&thema=Incontinentia pigmenti

45. CASE 4—A BABY GIRL WITH LINEAR VESICLES Incontinentia Pigmenti (IP), also known as blochsulzberger syndrome, is a rare genodermatosis with X-linked dominant inheritance. http://www.med.wayne.edu/dermatology/webmichderm01/case4.htm

CASE 4—A BABY GIRL WITH LINEAR VESICLES ON THE ARMS AND THE LEGS Meena Moossavi MD, Vicky Langsam MD, George Murakawa MD PhD HISTORY A one-month old Caucasian girl of normal gestation presented with an asymptomatic rash on her arms and legs that developed at birth. The rash did not respond to treatment with oral antibiotics and topical silvadene cream. There was no significant family history. Her normal older brother, sister, and mother are unaffected. EXAMINATION The patient had multiple fluid-filled vesicles on an erythematous base distributed on the arms and legs, following the lines of Blaschko. The scalp, face and trunk were spared. The eyes were normal and the neurologic examination was age appropriate. There was no developmental delay noted. LABORATORY Genetic testing: pending. HISTOPATHOLOGY A biopsy from a vesicle on the thigh revealed a focus of intraepidermal vesicle formation containing numerous eosinophils. The surrounding epidermis showed eosinophilic spongiosis and occasional dyskeratotic epidermal cells. COURSE AND THERAPY The vesicular lesions resolved by three months of age and verrucous papules developed on the right foot, left leg and left chest at four months. No eye abnormalities were found on exam by the ophthalmologist. At five months, a slight developmental delay was noted by the pediatrician, and the patient was referred to pediatric neurology for further work up.

46. Case 7 case 7—incontinentia pigmenti. Incontinentia pigmenti (IP), also known as blochsulzberger syndrome, is an X-linked dominant, monogenic disease. http://www.med.wayne.edu/dermatology/MichDerm03/case_7diagnosis.htm

cases diagnosis/discussions case 7—incontinentia pigmenti Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is an X-linked dominant, monogenic disease. Patients can present with neurologic, ophthalmologic, dental, and cutaneous findings. IP was first reported in 1906 by Garrod, and later defined clinically by Bloch and Sulzberger. The gene responsible for 80% of IP cases is NEMO/IKK- g , located on chromosome Xq28. NEMO, short for nuclear factor- k B (NF- k B) essential modulator, is a key component in NF- k B signaling pathways affecting cellular apoptosis. Almost all NEMO mutations destroy gene function, perhaps causing enhanced sensitivity to apoptosis and dysregulation of cellular homeostasis. Extracutaneous signs are also common in IP. Delayed dentition, partial anodontia, or cone-shaped teeth may occur in 80% of cases. The absence of lateral incisors and premolars in unaffected siblings and mothers can be a clue to the diagnosis. 30% of patients demonstrate retinal pigment epitheliopathy, and this is believed to cause other ocular findings like cataracts, leukocoria, optic atrophy, strabismus, and microphthalmos. Neurologic problems include seizures, developmental delay, mental retardation, ataxia, spasticity, microcephaly, and hypoplasia of the corpus callosum. Associated cardiac abnormalities, dwarfism, spina bifida, and hemiatrophy have been described. The histopathology of IP is most specific in stage 1, which manifests as spongiotic eosinophil-filled epidermal vesicles. In stage 2, acanthosis, papillomatosis, and hyperkeratosis with dyskeratotic keratinocytes are visualized. The hyperpigmented lesions of stage 3 and the hypopigmented lesions of stage 4 exhibit nonspecific histologic findings. Moreover, the presence of eosinophils in vesicular lesions, brain tissue, and peripheral blood suggests a possible immunologic reaction to an abnormal protein expressed by the mutated gene during early life. Referrals to genetics, dentistry, ophthalmology, and neurology are key for evaluation for extracutaneous manifestations.

47. Magnetic Resonance Findings Of Central Nervous System ABSTRACT. Incontinentia pigmenti, or blochsulzberger syndrome is a rare neurocutaneous disease. The clinical manifestations begin at or shortly after birth. http://www.rsroc.org.tw/English/Ejournal/article/V25/N1/A5/A5.htm

48. Penn State Faculty Research Expertise Database (FRED) Related Terms, blochsulzberger syndrome, Bloch Sulzberger Syndrome. Syndrome, Bloch-Sulzberger, Faculty Experts, Faculty Experts of Child Terms, http://fred.hmc.psu.edu/ds/retrieve/fred/meshdescriptor/D007184

49. Early On s Disease; WerdnigHoffman Syndrome. Encephalocele Neurocutaneous Disorders bloch-sulzberger syndrome; Neurofibromatosis; Sturge-Weber http://www.ingham.k12.mi.us/page.cfm?p=309

50. Genetic Incontinentia pigmenti (blochsulzberger syndrome). X linked dominant (97% females - males die); Marble cake skin hyperpigmentation; http://www.visioneyedoctor.com/genetic1.htm

Albinism Oculocutaneous albinism Hair, skin and eyes Auto-recessive Photophobia and squinting Ocular albinism Skin normal Nystagmus Iris translucent Poor visual acuity X Linked auto-recessive Darier's disease Red to brown skin papules and scales, nail anomalies Corneal opacities Ichthyosis Skin scaling Scales on lashes and lids, SPK Some forms have white cornea stromal opacities "KID syndrome": keratitis, ichthyosis, and deafness Incontinentia pigmenti (Bloch-Sulzberger syndrome) X linked dominant (97% females - males die) "Marble cake" skin hyperpigmentation Nipple defects Retinal pigment changes Retinal vascular occlusion and neovascularization Hypomelanosis of Ito Hypopigmented whorls in skin Strabismus, pin-point pupils Xeroderma pigmentosum Extreme photosensitivity, pterygia, dry eye Carcinomas Ehlers-Danlos syndrome Hyperextensible skin and joints Skin scars easily Keratoconus, blue sclera, angiod streaks, subluxation of lens Juvinile Xanthogranuloma Rubbery skin nodules on head and neck Iris tumor, spontaneous hyphema, glaucoma

51. List Of Neurological Disorders - Wikipedia, The Free Encyclopedia Blepharospasm; blochsulzberger syndrome; Brachial plexus injury; Brain abscess; Brain injury; Brain tumor; Spinal tumor; Brown-Sequard syndrome. C http://www.phatnav.com/wiki/wiki.phtml?title=List_of_neurological_disorders

52. Incontinentia Pigmenti This Web resource on incontinentia pigmenti (also known as blochsulzberger syndrome) is produced by the National Institute of Neurological Disorders and http://omni.ac.uk/browse/mesh/C0021171L0021171.html

low graphics Incontinentia Pigmenti broader: Abnormalities, Multiple Skin Abnormalities other: Alagille Syndrome Angelman Syndrome Beckwith-Wiedemann Syndrome Branchio-Oto-Renal Syndrome ... NINDS : incontinentia pigmenti information page This Web resource on incontinentia pigmenti (also known as Bloch-Sulzberger Syndrome) is produced by the National Institute of Neurological Disorders and Stroke (NINDS). A description of incontinentia pigmenti is provided, and available treatments, prognosis, and current research activities are all discussed. Links to related organisations are provided. This resource has a US focus. Patient Education Handout [Publication Type] Incontinentia Pigmenti Last modified: 27 May 2004

53. Part 29. Disorders Of The Eye - KMLE: ¼Ò¾Æ°ú ³Ú½¼(Nelson) Á¤¸®Áý medulloepithelioma, massive retinal gliosis, retinal pseudotumor of Norrie disease, pseudoglioma of the blochsulzberger syndrome, retinal dysplasia, retinal http://www.kmle.co.kr/nelsonview.php?view=Part 29. Disorders of The Eye.htm

54. Incontinentia Pigmenti BlochSiemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis; Bloch-Siemens-Sulzberger Syndrome; bloch-sulzberger syndrome; Pigmented Dermatosis, Siemens http://www.icongrouponline.com/health/Incontinentia_Pigmenti.html

ICON Health Publications Official Health Sourcebooks The Official Parent's Sourcebook on INCONTINENTIA PIGMENTI (Bloch-Siemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis; Bloch-Siemens-Sulzberger Syndrome; Bloch-Sulzberger Syndrome; Pigmented Dermatosis, Siemens-Bloch type) Revised and Updated for the Internet Age P A P E R B A C K Paperback Book Paperback Book Order by phone: 800-843-2665 (within USA) 1-201-272-3651 (from outside USA) Paperback Book Shipped in 3 to 5 business days E B O O K Electronic File * E-Book version sent via e-mail in 2 business days Electronic File *E-Book version sent via e-mail in 2 business days Pages Price $28.95(USD) ISBN Published Synopsis A comprehensive manual for anyone interested in self-directed research on Incontinentia Pigmenti. Fully referenced with ample Internet listings and glossary. Related Conditions/Synonyms Bloch-Siemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis; Bloch-Siemens-Sulzberger Syndrome; Bloch-Sulzberger Syndrome; Pigmented Dermatosis, Siemens-Bloch type Description Table of Contents Introduction Overview Organization Scope Moving Forward PART I: THE ESSENTIALS Chapter 1. The Essentials on Incontinentia Pigmenti: Guidelines

55. Search By Disease 56 Blepharophimosis, epicanthus inversus and ptosis (BPES). 57 blochsulzberger syndrome. 58 Blood groupRhesus system Cc/Ee polypeptide. http://www.eddnal.com/directory/disease.php?letter=B&page=4

56. Blackwell Synergy - Cookie Absent The characteristic dentition of incontinentia pigmenti (blochsulzberger syndrome) a diagnostic aid. Incontinentia pigmenti (bloch-sulzberger syndrome). http://www.blackwell-synergy.com/links/doi/10.1111/j.1525-1470.2003.20607.x/full

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58. Conditions And Diseases - Incontinentia Pigmenti Top Links Incontinentia Pigmenti Web Site Links. National Library of Medicine bloch-sulzberger syndrome, the synonyms, a summary and major features. http://www.disease-resources.com/Top_Health_Conditions_and_Diseases_Genetic_Diso

Sub Categories Related Healthcare Subjects Disabilities Environmental Health Fitness Health Insurance ... Men's Health Incontinentia Pigmenti Web Site Links National Library of Medicine Bloch-sulzberger syndrome, the synonyms, a summary and major features. Drkoop.com: Medical Encyclopedia Information about incontinentia pigmenti, its causes, symptoms, tests, diagnosis, treatment and possible complications. MCW Healthlink: Incontinentia Pigmenti An article about this rare genetic disorder, its stages and symptoms. Incontinentia Pigmenti Informational sheet compiled by National Institute of Neurological Disorders and Stroke. Vanderbilt Medical Center An article about incotinentia pigmenti. Readers Digest Health: IP A general discussion about incontinentia pigmenti, with alternate names and further resources. Incontinentia Pigmenti International Foundation Details about the organization that is dedicated to research into the causes and treatment of this genetic disease. Includes information about the disease, genetics and genes. Incontinentia Pigmenti: All About Anna The authors' daughter was diagnosed with IP 6 weeks after birth. This is her story.

59. BJO -- Abstracts: Spallone 71 (8): 629 71, 629634. ORIGINAL ARTICLES. Incontinentia pigmenti (bloch-sulzberger syndrome) seven case reports from one family. A Spallone St http://www.bjophthalmol.com/cgi/content/abstract/71/8/629

HOME HELP FEEDBACK SUBSCRIPTIONS ... Email this link to a friend eLetters: Submit a response to this article Similar articles found in: BJO Online PubMed PubMed Citation This Article has been cited by: other online articles Search PubMed for articles by: Spallone, A. Alert me when: new articles cite this article Download to Citation Manager British Journal of Ophthalmology, 1987, Vol 71, 629-634 ORIGINAL ARTICLES Incontinentia pigmenti (Bloch-Sulzberger syndrome): seven case reports from one family A Spallone St Gerardo Hospital, Department of Ophthalmology, Monza, Milan, Italy. Seven members from a large family who showed signs of incontinentia pigmenti were examined. A clear X-linked dominant transmission was demonstrated, lethal in males. Study of this family shows that vascular abnormalities of the retina and disorders of the retinal pigment epithelium are the most important ocular lesions in the Bloch- Sulzberger syndrome. This article has been cited by other articles: E J Mayer, G N Shuttleworth, K L Greenhalgh, J E Sansom, R H B Grey, and S Kenwrick Novel corneal features in two males with incontinentia pigmenti Br. J. Ophthalmol., May 1, 2003; 87(5): 554 - 556.

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