61. Tuberous Sclerosis-Koenen's Tumor tuberous sclerosis (Epiloia) Koenen s Tumor (Subungual fibromata) - Finger. For more information see the Online Mendelian Inheritance in Man Entries for http://tray.dermatology.uiowa.edu/TubScKT01.htm

Dept. of Dermatology - University of Iowa College of Medicine Tuberous Sclerosis (Epiloia) - Koenen's Tumor (Subungual fibromata) - Finger For more information see the Online Mendelian Inheritance in Man Entries for Tuberous Sclerosis-1 Tuberous Sclerosis-2 Tuberous Sclerosis-3 , and Tuberous Sclerosis-4 Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. September, 1995

62. Brain Foundation - Tuberous Sclerosis tuberous sclerosis is an uncommon and complex genetic disorder. The common feature of tuberous sclerosis is the overgrowth of “normal” tissue in the http://www.brainaustralia.org.au/AZ_of_Brain_Disorders/tuberous_sclerosis

Home About Us The Brain Healthy Brain ... Site Map DHTML_MENU_rel_path = '/squizlib/dhtml_menu/images/'; Tuberous Sclerosis Description Tuberous Sclerosis is an uncommon and complex genetic disorder. It varies in the severity with which it affects people - some people are severly affected and diagnosed early, others are only found to have the condition when their child is diagnosed. The common feature of Tuberous Sclerosis is the overgrowth of “normal” tissue in the brain and in other organs, including the kidneys, heart, liver and lungs. These growths begin to form in the brain prior to birth and can interfere with brain functioning. They can cause seizures, delayed development, mental retardation, and autistic or hyperactive behaviour. With age, these growths become hard and calcified, hence the term 'sclerosis'. However, life expectancy remains normal. Around one out of two people born with Tuberous Sclerosis will lead normal lives with no apparent intellectual dysfunction or epilepsy. Estimates vary, but it is thought that approximately one in 7,000 to 10,000 people have Tuberous Sclerosis. There is no cure. The symptoms of Tuberous Sclerosis vary from one individual to the next, depending on the severity of the condition and which areas of the body are affected.

63. Tuberous Sclerosis tuberous sclerosis (epiloia) is an autosomal dominant multi system genetic tuberous sclerosis was first diagnosed by Bournville, a French physician over http://www.skinatlas.com/tuberous.htm

Tuberous Sclerosis (epiloia) is an autosomal dominant multi system genetic condition that is characterized by multiple tuber like hamartomas of the skin, the brain, and frequently in other vital organs. The condition develops before birth and continues to do so during the person's lifetime. It occurs in approximately 1 in 10 000 people. Tuberous Sclerosis was first diagnosed by Bournville, a French physician over 100 years and classic triad of adenoma sebaceum, mental deficiency, and epilepsy is present in only 25% of patients and mildly affected individuals are often misdiagnosed. Typical symptoms of the disease can be one, some or all of the following: Epilepsy: 80% of children with Tuberous Sclerosis will be epileptic, some with many types of seizures. These seizures are often difficult to control. Intellectual Impairment: This does not always occur. Impairment can vary from mild to very severe. Mental retardation occurs in less than 50%. Behavioural Problems: Some children with Tuberous Sclerosis are very overactive and may have sleeping problems. Some may show signs of autism. Some children may develop challenging behaviours.

64. Tuberous Sclerosis Complex (TSC) tuberous sclerosis is a genetic disorder that affects various parts of the body to varying degrees of severity. Its common characteristic is the formation http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Tuberous_sclerosi

65. Tuberous Sclerosis Article describes tuberous sclerosis, its diagnosis, and treatment. http://rarediseases.about.com/cs/tuberoussclerosis/a/051703.htm

var zLb=8; var zIoa2 = new Array('Elsewhere on the Web','Information pamphlet: tuberous sclerosis','http://www.tuberous-sclerosis.org/publications/nurses.pdf','Tuberous Sclerosis Alliance','http://www.tsalliance.org','Australasian Tuberous Sclerosis Society','http://www.atss.org.au'); zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zfs=0;zCMt='a70' About Rare / Orphan Diseases Rare Diseases S - V Rare Diseases: T Tuberous Sclerosis Rare Diseases Essentials Rare Diseases: Basic Information Rare Diseases Support Groups ... Help zau(256,140,140,'el','http://z.about.com/0/ip/417/C.htm','');w(xb+xb+' ');zau(256,140,140,'von','http://z.about.com/0/ip/496/7.htm','');w(xb+xb); FREE Newsletter Sign Up Now for the Rare / Orphan Diseases newsletter! See Online Courses Search Rare / Orphan Diseases Stay up to date! Compare Prices Email to a friend ... Print this page Elsewhere on the Web Information pamphlet: tuberous sclerosis Tuberous Sclerosis Alliance Australasian Tuberous Sclerosis Society Most Popular Muscular Dystrophy ALD and Lorenzo's Oil Progeria Syndromes Leprosy (Hansen's Disease) ... The Elephant Man What's Hot Charcot-Marie-Tooth Disease Peyronie's Disease Twin-to-Twin Transfusion Synd Autoimmune Kidney Disease ... Cri-du-Chat Syndrome Related Topics Multiple Sclerosis Parenting Special Needs Sleep Disorders adunitCM(150,100,'x55')

66. Tuberous Sclerosis Aicardi J. tuberous sclerosis. International Pediatrics 1993;82171175 tuberous sclerosis. Part 1 Clinical and central nervous system findings. http://www.5mcc.com/Assets/SUMMARY/TP0959.html

Tuberous sclerosis DESCRIPTION: One of the neurocutaneous syndromes (phakomatoses). A genetic developmental disorder with variable presentations, a broad clinical spectrum and multi-organ involvement. Organ systems may be few and subtle, but can encompass multiple types of cutaneous lesions and tumor formation in the central nervous system, skin, retina, heart, lung, viscera, liver, kidney, bone, teeth and nails. Other phakomatoses include neurofibromatosis, Sturge-Weber disease, von Hippel-Lindau syndrome, and ataxia-telangiectasia. System(s) affected: Skin/Exocrine, Nervous, Cardiovascular, Pulmonary, Renal/Urologic, Musculoskeletal Genetics: Autosomal dominant with variable penetrance Two thirds are the result of a new gene mutation Two chromosomal loci are mapped; TSC1 (locus 9q34) and TSC2 (locus 16p13.3) Incidence/Prevalence in USA: Reported to affect 1 in 6,000 in the general population Predominant age: Clinical expression is variable, and diagnosis may be delayed. Usually diagnosed during the first decade. Predominant sex: Male = Female (but autism more common in males) CAUSES: Congenital Synonyms: Bourneville disease ICD-9-CM: 759.5 Tuberous sclerosis

67. Tuberous Sclerosis Complex Research Program, Congressionally Directed Medical Re tuberous sclerosis is a genetic disorder that can affect any or all systems of the Children with tuberous sclerosis may have autisticlike symptoms. http://cdmrp.army.mil/tscrp/default.htm

Home Site Map Contact Us Funding Innovation - Finding A Cure - Providing Hope Research Programs Funding Opportunities Consumer Involvement Search Awards ... Research Programs Search Site: Tuberous Sclerosis Complex Vision To lessen the impact of tuberous sclerosis. Mission To encourage innovative research aimed at understanding the role and function of proteins produced by the TSC1 and TSC2 tumor suppressor genes. Funding Opportunities Search Awards Integration Panels Peer Review Participants ... Related Tuberous Sclerosis Complex Links Congressional Appropriations $6 million in FY02-FY04 $3.2 million in FY05 Research Program Fact Sheet Funding Summary 7 awards in FY02-03 approximately 12 awards in FY04 Tuberous Sclerosis Complex News Funds for FY06 pending availability. More News... Research Highlights Coming Soon! Previous Highlights... CDMRP Home Site Map Search ... About Us Last updated June 21, 2005 (lm)

68. Related Links - Tuberous Sclerosis Complex, Congressionally Directed Medical Res to a site that is not within the CDMRP web site. CDMRP is not responsible for the content of the sites listed below. The tuberous sclerosis Alliance http://cdmrp.army.mil/resources/tsclinks.htm

Home Site Map Contact Us Funding Innovation - Finding A Cure - Providing Hope Research Programs Funding Opportunities Consumer Involvement Search Awards ... Cancer Resources Search Site: Tuberous Sclerosis Complex not within the CDMRP web site. CDMRP is not responsible for the content of the sites listed below. The Tuberous Sclerosis Alliance CDMRP Home Site Map Search ... About Us Last updated April 14, 2005 (ldm)

69. Introduction: Tuberous Sclerosis - WrongDiagnosis.com Introduction to tuberous sclerosis as a medical condition including symptoms, diagnosis, misdiagnosis, treatment, prevention, and prognosis. http://www.wrongdiagnosis.com/t/tuberous_sclerosis/intro.htm

Home Symptoms Diseases Risks ... Diseases Search our medical database Subscribe to our free Newsletter sample Send this page to a friend Condition Lists By Organ By Symptom By Class By Prevalence ... List A-Z Current chapter: Tuberous sclerosis Next sections Basic Summary for Tuberous sclerosis Prevalence and Incidence of Tuberous sclerosis Prognosis of Tuberous sclerosis Symptoms of Tuberous sclerosis ... Complications of Tuberous sclerosis Next chapters: Amyotrophic lateral sclerosis Lichen sclerosis Scleroderma Connective tissue disorders ... Feedback Introduction: Tuberous sclerosis Tuberous sclerosis: Rare genetic brain disorder causing seizures, retardation and lesions. Tuberous sclerosis: Tuberous sclerosis is a rare genetic, neurological disorder primarily characterized by seizures, mental retardation, and skin and eye lesions. In some cases, neurobehavioral problems may also occur. Researching symptoms of Tuberous sclerosis: Further information about the symptoms of Tuberous sclerosis is available including a list of symptoms of Tuberous sclerosis , or alternatively return to research other symptoms in the symptom center Treatments for Tuberous sclerosis: Various information is available about treatments available for Tuberous sclerosis , or research treatments for other diseases.

70. Tuberous Sclerosis Of The Brain tuberous sclerosis of Brain From the Virtual Pathology Museum, Department of Pathology, University of Connecticut Health Center. http://radiology.uchc.edu/eAtlas/CNS/672.htm

Search Frames Search No frames PathWeb Home Feed Back ... About Tuberous Sclerosis Click on Image to Enlarge it Tuberous Sclerosis View of the base and frontal lobes of the brain showing tubers or hard, potato like gyri at the arrows. Tubers contain malformed neurons and astrocytes which form the foci for seizures. (Description By:Margaret Grunnet, M.D. ) (Image Contrib. by:Margaret Grunnet, M.D. UCHC ) Tuberous sclerosis Etiology Tuberous sclerosis is a phacomatosis (disease that affects brain, skin and other organs) that an autosomal dominant, inherited with high penetrance. Pathogenesis /see etiology., Epidemiology The prevalance of tuberous sclerosis is 1 in 10,000 children ages 0-5. General Gross Description The brains of most tuberous sclerosis patients show tubers (hard, potato-like nodules in gyri) of the cerebral cortex and candle gutterings (irregular calcified nodules) in the lateral, third and fourth ventricles. The face often shows angiofibromas on the cheeks and forehead, the heart may have rhabdomyomas and the kidneys may show cysts or angiomyolipomas.

71. Tuberous Sclerosis Links to information about tuberous sclerosis. It is estimated that tuberous sclerosis occurs as a spontaneous mutation in 80 per cent of cases. http://www.healthinsite.gov.au/topics/Tuberous_Sclerosis

Skip to Content Advanced Search About Health Insite A-Z Health Topics ... Conditions and Diseases Topics such as asthma, diabetes, cancer, arthritis, depression Health and Wellbeing Topics such as fitness, nutrition, drugs, preventing suicide, health insurance, women's health, living with a disability Life Stages and Events Topics such as menopause, pregnancy, ageing, going into hospital Health Services Includes links to State/Territory Health Services and other services News Health Insite Newsletter ... Home Tuberous Sclerosis Links to information about tuberous sclerosis. Reviewed June 2005 Printer friendly page 5 Resources Found Results 1 to 5 displayed. Title: Tuberous sclerosis complex (TSC) Publisher: Better Health Channel Description: Tuberous sclerosis is a genetic disorder that affects various parts of the body to varying degrees of severity. Its common characteristic is the formation of tuber-like growths in the brain. There is no cure. Date: Oct 2004 Title: Tuberous sclerosis Publisher: myDr Description: Tuberous sclerosis is a genetic condition that causes tuber-like growths in the brain and other vital organs.

72. AllRefer Health - Tuberous Sclerosis (Adenoma Sebaceum) tuberous sclerosis (Adenoma Sebaceum) information center covers causes, prevention, symptoms, diagnosis, treatment, incidence, risk factors, signs, tests, http://health.allrefer.com/health/tuberous-sclerosis-info.html

AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia Web health.allrefer.com You are here : AllRefer.com Health Tuberous Sclerosis Tuberous Sclerosis Definition Prevention Treatment Expectations or Prognosis ... Go To Main Page Alternate Names : Adenoma Sebaceum Definition Tuberous sclerosis is a group of two genetic disorders characterized by problems with the skin, brain/nervous system, and kidneys, and a predisposition to tumors. The diseases are named after a characteristic abnormal growth in the brain, which takes the shape of a tuber or root. Tuberous Sclerosis, Angiofibromas - Face Tuberous Sclerosis, Hypopigmented Macule Tuberous sclerosis is inherited as an autosomal dominant trait (only one parent must pass on the bad gene for the child to get the disease). However, a high percentage of cases are due to new mutations (which occur in the sperm or egg of one of the parents that created the particular child), so there usually is no family history of the disease.

73. AllRefer Health - Tuberous Sclerosis, Hypopigmented Macule (Pictures, Images, Ph tuberous sclerosis, Hypopigmented Macule. tuberous sclerosis, Hypopigmented Macule tuberous sclerosis, Angiofibromas Face • tuberous sclerosis http://health.allrefer.com/pictures-images/tuberous-sclerosis-hypopigmented-macu

AllRefer Channels :: Yellow Pages Reference Health Home ... Medical Encyclopedia Web health.allrefer.com You are here : AllRefer.com Health Tuberous Sclerosis, Hypopigmented Macule This area of decreased pigment (hypopigmentation) is referred to as an "ash leaf macule" and is seen with the inherited disorder, tuberous sclerosis. Another finding, "confetti hypopigmentation" is evident as scattered white spots around the ash leaf spot. Related Images Tuberous Sclerosis, Angiofibromas - Face Tuberous Sclerosis, Hypopigmented Macule Vitiligo Vitiligo on the Back and Arm ... Vitiligo, Drug Induced Related Articles Tuberous Sclerosis Vitiligo Review Date : 1/1/1997 Reviewed By : Mosby Publishing Company From Our Sponsors: A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial reviewers . A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch). Par Web Solutions Health Topics: A-Al Am-Az B C-Cj ... Healthopedia.com

74. Dr. Koop - Tuberous Sclerosis tuberous sclerosis is a group of two genetic disorders characterized by problems with the skin, brain/nervous system, and kidneys, and a predisposition to http://www.drkoop.com/ency/93/000787.html

Home Health Reference Tuberous sclerosis Jul 29, 2005 Search: Dr.Koop MEDLINE Diseases Symptoms Procedures Natural Medicine ... Drug Library Inside DrKoop News Archive Animations Health Videos Health Tools ... Newsletters Tuberous sclerosis Injury Disease Nutrition Poison ... Prevention Tuberous sclerosis Definition: Tuberous sclerosis is a group of two genetic disorders characterized by problems with the skin, brain/nervous system, and kidneys, and a predisposition to tumors. The diseases are named after a characteristic abnormal growth in the brain, which takes the shape of a tuber or root. Alternative Names: Adenoma sebaceum Causes, incidence, and risk factors: Tuberous sclerosis is inherited as an autosomal dominant trait (only one parent must pass on the bad gene for the child to get the disease). However, a high percentage of cases are due to new mutations (which occur in the sperm or egg of one of the parents), so there usually is no family history of the disease. It is one of a group of diseases described as neuro-cutaneous syndromes because of extensive involvement of both the skin and the central nervous system (brain and/or spinal cord).

75. Dr. Koop - Tuberous Sclerosis, Hypopigmented Macule tuberous sclerosis, hypopigmented macule, Jul 29, 2005 referred to as an ash leaf macule and is seen with the inherited disorder, tuberous sclerosis. http://www.drkoop.com/ency/93/ImagePages/2538.html

Home Health Reference Tuberous sclerosis, hypopigmented macule Jul 29, 2005 Search: Dr.Koop MEDLINE Diseases Symptoms Procedures Natural Medicine ... Drug Library Inside DrKoop News Archive Animations Health Videos Health Tools ... Newsletters Tuberous sclerosis, hypopigmented macule This area of decreased pigment (hypopigmentation) is referred to as an "ash leaf macule" and is seen with the inherited disorder, tuberous sclerosis. Another finding, "confetti hypopigmentation" is evident as scattered white spots around the ash leaf spot. New Features Fewer Migraines Skin Cancer Atkins/Low Carb Diets Learn More About Migraines * All Health Centers * Acne Addictions AIDS/HIV Alcohol Abuse Allergies Alternative Medicine Alzheimer's Arthritis Asthma Attention Deficit Disorder (ADHD) Backache Bipolar Affective Disorder Birth Control Blood Blood Pressure Bone/Joint/Tendon Bowel Brain Breast cancer Cancer Cerebral Palsy Cholesterol Cramps Crohn's Disease Cysts Dental/Oral Depression Diabetes Diet, Fitness, Looks Disabled/Special Needs Drug Abuse Ear/Nose/Throat Eating/Appetite Eczema Encephalitis Eye/Vision Fatigue Fever Flu Food Poisoning Foot Gallbladder Gastrointestinal Genetic/Congenital GERD/Heartburn Hair Loss Hair/Scalp Headache Hearing Heart Disease, Stroke

76. Virtual Children's Hospital: Paediapaedia: Tuberous Sclerosis tuberous sclerosis. Michael P. D Alessandro, MD Peer Review Status Internally Peer Reviewed. Clinical Presentation Clinical triad is adenoma sebaceum, http://www.vh.org/pediatric/provider/radiology/PAP/NeuroDiseases/TS.html

Paediapaedia: Neurological Diseases Tuberous Sclerosis Michael P. D'Alessandro, M.D. Peer Review Status: Internally Peer Reviewed Clinical Presentation: Clinical triad is adenoma sebaceum, seizures, and mental retardation. Etiology/Pathophysiology: A hamartomatous proliferation of fibrous and connective tissue in the brain, skin, skeleton, kidneys, and heart. Pathology: The hamartomatous lesions in the brain consist of proliferating glial tissue and ganglion cells. Imaging Findings: The intracranial tubers are usually calcified and usually are in a periventricular / subependymal location but they can occasionally be found in the brain parenchyma. The tubers may undergo malignant degeneration into giant cell astrocytomas. DDX: Not applicable References: See References Chapter. Section Top Title Page Virtual Children's Hospital Home Virtual Hospital Home ... University of Iowa http://www.vh.org/pediatric/provider/radiology/PAP/NeuroDiseases/TS.html

77. Virtual Hospital: Radiology Resident Case Of The Week: Disease: Tuberous Scleros Disease tuberous sclerosis Bourneville s Disease. Jeff Goree, MD Peer Review Status Internally Peer Reviewed. Clinical Sx The classic triad of adenoma http://www.vh.org/pediatric/provider/radiology/PedRadSecTF/072895/

Radiology Resident Case of the Week: July 28, 1995 Disease: Tuberous Sclerosis - Bourneville's Disease Jeff Goree, M.D. Peer Review Status: Internally Peer Reviewed Clinical Sx: The classic triad of adenoma sebaceum (in 60-70% of patients), mental retardation, and seizures is seen in only 1/3 of patients. Other findings include: retinal hamartomas, Shagreen patches, and Ash leaf spots on physical exam. Etiology/Pathophysiology: Although it's an autosomal dominant disease involving the long arm of chromosome 9, tuberous sclerosis most commonly occurs spontaneously (60%). The incidence is about 1/150-100,000 live births. Pathology: This disease is a "shotgun blast." It hits the entire body. The intracranial changes are due to "tubers" 1-2 cm in size that can involve cortex and/or subependyma. Microscopically, they exhibit bizarre architecture with surrounding gliosis and fibrillary background. The cells are enlarged multinuclear astrocytes. The lesions have a tendency to calcify. When there are subependymal and periventricular nodules, "Candle guttering" may be seen (88% calcify) along the lateral ventricles. Cortical and subcortical tubers are most common in the frontal lobes. The subependymal lesions may degenerate to a Giant Cell Astrocytoma (2-15% of patients). This tumor, although usually benign, can grow large and causes symptoms due to its mass effect. They most commonly occur around the Foramen of Monro, and may lead to obstructive hydrocephalus.

78. LLU Health Care Tuberous Sclerosis Clinic tuberous sclerosis clinic. Sponsored by Loma Linda University Health Care. The tuberous sclerosis clinic is the first specialty clinic for patients with http://www.llu.edu/lluhc/tsc-clinic.html

Site index Contact document.write(''); document.write(''); Services Back Tuberous sclerosis clinic Sponsored by Loma Linda University Health Care The tuberous sclerosis clinic is the first specialty clinic for patients with tuberous sclerosis complex on the West Coast. This "clinic without walls" has a multi-disciplinary approach that provides appropriate medical and surgical services related to the complications of this disorder. Both children and adults with tuberous sclerosis complex will initially be evaluated by Stephen Ashwal, MD, pediatric neurologist and clinic director, and Teresa Serna, PNP, pediatric neurology nurse practitioner, and then will be referred for subspecialty evaluation as needed. Subspecialists with expertise in this disorder include members from neurology, urology, nephrology, dermatology, ophthalmology, cardiology, neurosurgery, rehabilitation medicine, orthopedics, clinical psychology, and psychiatry. If you have any questions regarding the tuberous sclerosis clinic or if you wish to make a referral, please call the pediatric neurology clinic directly at and ask for Teresa Serna, PNP.

79. Tuberous Sclerosis A case of renal cell carcinoma associated with tuberous sclerosis Kakimoto K; On the other hand, the relation between tuberous sclerosis and renal cell http://www.edae.gr/tuberous.html

HELLENIC ASSOCIATION DERMATOLOGY - VENEREOLOGY TUBEROUS SCLEROSIS - Bourneville's Disease - Epiloia A case of renal cell carcinoma associated with tuberous sclerosis Kakimoto K; Mori N; Tei N; Hara T; Koide T . Tuberous sclerosis is associated commonly with renal angiomyolipoma. On the other hand, the relation between tuberous sclerosis and renal cell carcinoma is not widely recognized. We report a case of renal cell carcinoma of the right kidney associated with tuberous sclerosis.... Tuberous Sclerosis - October 1999 A.S.T. Associazione Sclerosi Tuberosa American Brain Tumor Association Homepage American Society of Human Genetics Ash-Leaf Spots in Tuberous Sclerosis The New England Journal of Medicine June 25, 1998 Vol. 338, No. 26 Australasian Tuberous Sclerosis Association Australasian Tuberous Sclerosis Society Inc. (ATSS) The Australasian Tuberous Sclerosis Society Inc. (ATSS) was foundered in 1982, with a mission statement: "ATSS aims to support Tuberous Sclerosis families, educate an inform health professionals and the community." ATSS acts as a mutual support group for sufferers, relatives and health professionals, sharing problems and information by providing: Australasian Tuberous Sclerosis Society ATSS Autism Research Institute Autism Society of America Basic facts about Tuberous Sclerosis..

80. Tuberous Sclerosis tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by a Secondary Parathyroid Hyperplasia in tuberous sclerosis http://www.thedoctorsdoctor.com/diseases/tuberous_sclerosis.htm

Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by a variety of organ system changes. Its name is derived from the characteristic changes that occur in the brain, called tubers. Over time, these tuberous growths become hard and sclerotic and may calcify. Until recently, most of the proliferations and tumors associated with the disease were thought to be hamartomas. Recent molecular biological analysis has revealed that these are clonal processes and true neoplastic growths. The TSC1 and TSC2 genes behave as tumor suppressor genes. If a somatic mutation of the second allele occurs, abnormal cell growth and differentiation occur. The current diagnostic criteria divide the disease into diagnostic categories based upon the combination of clinical and histological findings. Definite TSC: Either 2 major features or 1 major feature and 2 minor features Possible TSC: Either 1 major feature or 2 or more minor features Major Features Minor Features Facial angiofibromas or forehead plaque Nontraumatic ungual or periungual fibroma Hypomelanotic macules (more than 3) Shagreen patch (connective tissue nevus) Multiple renal nodular hamartomas Cortical tuber* Subependymal nodule Subependymal giant cell astrocytoma Cardiac rhabdomyoma, single or multiple

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