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         Bassen-kornzweig Syndrome:     more detail
  1. Bassen-Kornzweig syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Bryan, PhD Cobb, 2005

41. Bassen-Kornzweig Syndrome
Wiedemann Syndrome BlochSulzberger Syndrome. Abetalipoproteinemia(bassen-kornzweig syndrome) Pediatric Database A definition of
http://www.medlina.com/bassen-kornzweig_syndrome.htm
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Barth Syndrome

Bassen-Kornzweig Syndrome
Batten Disease

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Bloch-Sulzberger Syndrome
Abetalipoproteinemia (Bassen-Kornzweig syndrome) Pediatric Database A definition of abetalipoproteinemia, followed by epidemiology, pathogenesis, clinical features, investigations and management. WebMD/Lycos A definition of Bassen-Kornzweig syndrome, followed by a look at the alternate names, causes, symptoms, tests, treatment, prognosis and complications. Med Help A brief definition of abetalipoproteinemia. SUBCATEGORIES Up powered by A merica M edica, I nc. info@medlina.com New York City

42. Subject Topics
Abetalipoproteinemia/bassenkornzweig syndrome ?ß- ABO IncompatibilityABO Achalasia of the Cardia Achilles
http://www.kingdoctor.com/cgi-bin/showtopic.asp?topic=A

43. Abetalipoproteinemia
What is it? Abetalipoproteinemia (also known as bassenkornzweig syndrome) isan inherited disorder that affects how fats are made and used in the body.
http://rarediseases.about.com/library/weekly/aa072601a.htm
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Abetalipoproteinemia Problem with using fat in the body Related Resources Additional information
From Other Guides Retinitis pigmentosa resources
Elsewhere on the Web Discussion group By Mary Kugler, MSN, RN,C About.com Guide to Rare/Orphan Diseases July 26, 2001 What is it? Abetalipoproteinemia (also known as Bassen-Kornzweig syndrome) is an inherited disorder that affects how fats are made and used in the body. The body needs fats for healthy nerves, muscles, and digestion. Like oil and water, fats can't travel in the blood around the body by themselves-they just don't mix in. Fats attach to special proteins called lipoproteins to travel to wherever they're needed. Because of a genetic disorder, people with abetalipoproteinemia don't produce a critical "building block" (a special protein) for making lipoproteins. Without enough lipoproteins, fats can't be digested properly or travel around the body to where they're needed, which leads to serious health problems.

44. Chromosomal Disorders
produces this disorder, in which fats are absorbed but can't be brokendown by the body. Also known as bassenkornzweig syndrome.
http://rarediseases.about.com/cs/chromosomeproblem/
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Chromosomal disorders
Guide picks Chromosomes hold the genetic keys to all of the body's functions. There may be errors on any of the body's 23 pairs of chromosomes, such as broken or missing pieces or too many pieces or copies. Links below are grouped by chromosome number.
Chromosome 2 Disorders
Links to information and resources for disorders of Chromosome 2. Chromosome 3, 4, 5 disorders Links to information and resources for syndromes associated with Chromosomes 3, 4, or 5. Abetalipoproteinemia (Chromosome 4) An error on Chromosome 4 produces this disorder, in which fats are absorbed but can't be broken down by the body. Also known as Bassen-Kornzweig syndrome. Chromosome 8, 9, 10 disorders

45. IComm: File Not Found!
(Graves Disease). National Graves Disease Foundation http//www.ngdf.org.bassenkornzweig syndrome ..Abetalipoproteinemia).
http://www.icomm.ca/geneinfo/b.htm
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46. Definitions Of Genetic Disorders-B.
rdb_sum/560.htm. bassenkornzweig syndrome (Abetalipoproteinemia)http//www.stepstn.com/nord/rdb_sum/445.htm. Batten’s Disease
http://www.icomm.ca/geneinfo/def-b.htm
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47. Directory :: Look.com
Balanitis (6) Baldness (47) BardetBiedl Syndrome (6) Barth Syndrome (3) Basal GangliaDiseases (20) Basilar Migraine (3) bassen-kornzweig syndrome (5) Batten
http://www.look.com/searchroute/directorysearch.asp?p=43344

48. Directory :: Look.com
Lycos A definition of bassenkornzweig syndrome, followed by a look at the alternatenames, causes, symptoms, tests, treatment, prognosis and complications.
http://www.look.com/searchroute/directorysearch.asp?p=526699

49. Searchalot Directory For B
Diseases (20); Basilar Migraine (3); bassenkornzweig syndrome (5);Batten Disease (8); Beckwith-Wiedemann Syndrome (6); Behcet's Syndrome
http://www.searchalot.com/Top/Health/ConditionsandDiseases/B/
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50. MUMS List Of Disorders - A
Abdominal Teratoma Tumor (5); Abetalipoproteinemia (4) *; Abetalipoproteinemia(bassenkornzweig syndrome) (1) *; Absence of Arm
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  • 2 Hydroxic Gluteric Urea (1)
  • 2 Keto Adepic Aciduria (1)
  • 3 Hydroxy 3-Methylglutaryl CoAlyase Deficiency (HMG) (1) *
  • 3 Methycrotonyl CoA Carboxylase Deficiency (3MCCC) (1)
  • 3 Methylglutaconic Aciduria (4)
  • 4 Hydroxybutyric Aciduria (1)
  • 5 Alpha Reductase Deficiency (1) *
  • 5 Oxoprolinuria (Pyroglutamic Aciduria) (1)
  • 18 Hydroxylase Deficiency (Adrenal Hyperplasia) (1)
  • 21 Hydroxylase Deficiency (Adrenal Hyperplasia) (18) **
  • ACTH Deficiency (1)
  • AIDS (Acquired Immune Deficiency Syndrome) (6) **
  • Aarskog Syndrome (8) **
  • Abdominal Chylous Ascites (abdomen fills w/fluid) (4)
  • Abdominal Migraines (2)
  • Abdominal Teratoma Tumor (5)
  • Abetalipoproteinemia (4) *
  • Abetalipoproteinemia (Bassen-Kornzweig Syndrome) (1) *
  • Absence of Arm Below Elbow, Congenital (25) *
  • Absence of Arms (Severed in accident) (2) *
  • Absence of Arms, Hands attached to shoulder (1) *
  • Absence of Extensor Tendons in hands - bilateral (1) *
  • Absence of Feet and Hands, Congenital (2) *

51. Descripteur - Abetalipoproteinemia
Metabolic, Inborn Abetalipoproteinemia BASSENKORNZWEIG, SYNDROME ApolipoproteinB Deficiency Disease bassen-kornzweig syndrome Betalipoprotein Deficiency
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    MESH : Nutritional and Metabolic Diseases: Metabolic Diseases: Metabolism, Inborn Errors: Lipid Metabolism, Inborn Errors: Hypolipoproteinemia: Abetalipoproteinemia
    MESH
    : Congenital, Hereditary, and Neonatal Diseases and Abnormalities: Genetic Diseases, Inborn: Metabolism, Inborn Errors: Lipid Metabolism, Inborn Errors: Hypolipoproteinemia: Abetalipoproteinemia
    MESH
    : Hemic and Lymphatic Diseases: Hematologic Diseases: Blood Protein Disorders: Abetalipoproteinemia
    MESH
    : Nutritional and Metabolic Diseases: Metabolic Diseases: Metabolism, Inborn Errors: Brain Diseases, Metabolic, Inborn: Abetalipoproteinemia MESH : Congenital, Hereditary, and Neonatal Diseases and Abnormalities: Genetic Diseases, Inborn: Metabolism, Inborn Errors: Brain Diseases, Metabolic, Inborn: Abetalipoproteinemia MESH : Nutritional and Metabolic Diseases: Metabolic Diseases: Brain Diseases, Metabolic: Brain Diseases, Metabolic, Inborn: Abetalipoproteinemia MESH : Nervous System Diseases: Central Nervous System Diseases: Brain Diseases: Brain Diseases, Metabolic: Brain Diseases, Metabolic, Inborn:

52. Disease Reference
s esophagus Bartholin’s abscess Bartter’s syndrome Basal cell cancer Basal cellnevus syndrome Basedow's disease bassenkornzweig syndrome Bathing trunk
http://www.rwjhamilton.org/Atoz/Encyclopedia/diseaseB.asp
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53. THE MERCK MANUAL, Sec. 14, Ch. 179, Disorders Of Movement
Information about cerebellar and spinocerebellar disorders. Includes a short discussion about friedreich Category Health Conditions and Diseases Brain Diseases Cerebellar...... Abetalipoproteinemia (bassenkornzweig syndrome, vitamin E deficiency)and Refsum's disease (phytanic acid storage diseasesee Ch.
http://www.merck.com/pubs/mmanual/section14/chapter179/179g.htm
This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 14. Neurologic Disorders Chapter 179. Disorders Of Movement Topics [General] Tremor Dyskinesias Drug-Induced Movement Disorders ... Idiopathic Orthostatic Hypotension And Shy-Drager Syndrome
Cerebellar And Spinocerebellar Disorders
Disorders of the cerebellum and its inflow or outflow pathways produce deficits in the rate, range, and force of movement. Anatomically, the cerebellum has three subdivisions. The archicerebellum (vestibulocerebellum) comprises the flocculonodular lobe, helps maintain equilibrium and coordinate eye-head-neck movements, and is closely interconnected with the vestibular nuclei. The midline vermis (paleocerebellum) helps coordinate movement of the trunk and legs. Vermis lesions result in abnormalities of stance and gait. The lateral hemispheres, which make up the neocerebellum, control ballistic and finely coordinated limb movements, predominantly of the arms. Signs of cerebellar disease are listed in Table 179-3.

54. THE MERCK MANUAL, Sec. 1, Ch. 3, Vitamin Deficiency, Dependency, And Toxicity
A discussion about this vitamin and the organs which can be affected by deficiency, signs and symptoms, Category Health Conditions and Diseases...... Abetalipoproteinemia (bassenkornzweig syndrome), due to the genetic absence ofapolipoprotein B, causes serious fat malabsorption and steatorrhea, with
http://www.merck.com/pubs/mmanual/section1/chapter3/3f.htm
This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 1. Nutritional Disorders Chapter 3. Vitamin Deficiency, Dependency, And Toxicity Topics [General] Vitamin A Deficiency Vitamin A Toxicity Vitamin D Deficiency And Dependency ... Vitamin C Deficiency
Vitamin E Deficiency
Vitamin E (tocopherol) is a generic term for compounds that have a 6-chromanol ring, an isoprenoid side chain, and the biologic activity of -tocopherol. The vitamin E group contains -, and -tocopherols, which vary in the extent to which the chromanol ring is methylated. d- -Tocopherol is the only naturally occurring stereoisomer and the most potent in biologic assays (1.49 IU/mg); the totally synthetic dl- -tocopherol is completely racemic and has less biologic activity (1.1 IU/mg) than d- -tocopherol. The international standard is dl- -tocopherol acetate (1.0 IU/mg). In general, tocopherols act as antioxidants to prevent lipid peroxidation of polyunsaturated fatty acids in cellular membranes. The antioxidant activity of -tocopherol is similar to that of glutathione peroxidase, which contains selenium (see

55. CHAPTER_EIGHT
The neurologic abnormality of abetalipoproteinemia (bassenkornzweig syndrome)which includes peripheral neuropathy, ataxia and loss of posterior column
http://www.akronchildrens.org/neuropathology/CHAPTER_EIGHT.html
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CHAPTER EIGHT
NUTRITIONAL CNS DISORDERS
WERNICKE-KORSAKOFF SYNDROME
MIDLINE CEREBELLAR DEGENERATION VITAMIN E DEFICIENCY VITAMINE B12 DEFICIENCY WERNICKE-KORSAKOFF SYNDROME
The Wernicke-Korsakoff syndrome (WKS) is most common in malnourished alcoholics, but is also seen in demented people who neglect their nutrition, patients with gastric cancer, hyperemesis gravidarum, and other malnutrition settings. The classic clinical presentation is a triad of eye abnormalities (nystagmus, oculomotor paralysis, paralysis of conjugate gaze), ataxia of stance and gait, and mental symptoms , such as withdrawal, confusion, anterograde and retrograde amnesia with a tendency to fill gaps of memory with fabrications. Peripheral neuropathy (a form of Beri-Beri disease) is seen in 80 percent of patients. The syndrome may be partial and a high index of suspicion is essential for diagnosis, especially in debilitated patients with unexplained mental confusion. Administration of thiamine causes a rapid reversal of ophthalmoplegia within hours. Recovery from nystagmus and confusion is slower. Amnesia does not respond to treatment fully. The acute lesions of WKS consist of incomplete loss of neurons, loosening or vacuolization of the neuropil, and punctate hemorrhages.

56. NIH Guide: THE HEREDITARY ATAXIAS INCLUDING MACHADO-JOSEPH DISEASE
telangiectasia, hereditary spastic paraplegia, RoussyLevy and Marinesco-Sjogrensyndrome, abetalipoproteinemia (bassen-kornzweig syndrome), Refsum's disease
http://grants.nih.gov/grants/guide/pa-files/PA-92-048.html
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57. Abetalipoproteinaemia Information Page Diseases Database
Abetalipoproteinaemia Information Page. 3 synonyms or equivalents were found. Abetalipoproteinaemiaaka/or ApoB deficiency aka/or bassenkornzweig syndrome
http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=17

58. B
Biedl Syndrome; @ Barth Syndrome; @ Basal Ganglia Diseases; @ BasilarMigraine; @ bassenkornzweig syndrome; @ Batten Disease; @ Beckwith
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59. Massachusetts Eye AndEar Infirmary: Berman-Gund Laboratory
retinitis pigmentosa is a part Usher syndrome, type I and type II;LaurenceMoon-Bardet-Biedl syndrome; bassen-kornzweig syndrome;
http://www.meei.harvard.edu/research/labs/bermang.html
home ophthalmology department research in ophthalmology
Studies of Retinitis Pigmentosa and Macular Degeneration:
... Contact Information
Studies of Retinitis Pigmentosa and Macular Degeneration: Overview
The Berman-Gund Laboratory for the Study of Retinal Degenerations continues multidisciplined research on retinitis pigmentosa, macular degeneration, and other related degenerative diseases of the retina. Results from ocular examinations of more than 10,000 patients are in a computerized data bank that provides an unusual resource for research on these diseases. More than 5,000 blood samples are on file for molecular genetics research. The Laboratory is presently conducting research that includes:
  • Performing new randomized controlled study to see if a medication in addition to vitamin A can further slow the course of the common forms of retinitis pigmentosa.
    Making a concentrated effort to isolate the genes responsible for retinal degenerations.
    Studying transgenic models to define the mechanisms that lead to photoreceptor cell death.

60. Basic Text Formatting In HTML
It was first characterised in the 1950's by Bassen and Kornzweig (qv)and is sometimes referred to as the bassenkornzweig syndrome.
http://www.antipope.org/charlie/attic/webbook/ch2html/html-ch1c.html
Chapter 2: Understanding HTML
Basic Text Formatting in HTML
Comments Chapter contents Book contents HTML is the product of a collision between two schools of thought about how to create a generic text formatting language. First let's look at the uncontroversial stuff. The basic elements of an HTML document are chunks of text, aggregated into paragraphs. Because HTML is rendered to fit different-sized windows on different systems, all HTML browsers do their own word-wrapping to fit the width of the line to the width of the window. A space character or carriage return in an HTML document is basically interpreted by a browser as an opportunity to insert a space or a line break at its own discretion. If you just type text in and separate your paragraphs with blank lines, the browsers will happily ignore the blanks and concatenate your text into one monster paragraph. HTML gives us a tag, , to indicate a new paragraph. A paragraph consists of text enclosed in the tag . However, in practice, the is not necessary; the paragraph is implicitly closed by the start of the next paragraph or heading element. For example:

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