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         Russell Silver Syndrome:     more detail
  1. Silver-Russell Syndrome - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References by Icon Health Publications, 2004-06-09
  2. Russell-Silver syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Paul Johnson, 2005
  3. Growth Disorders: Achondroplasia, Dwarfism, Acromegaly, Primordial Dwarfism, Psychosocial Short Stature, Midgetville, Silver-russell Syndrome

41. The MAGIC Foundation
One interesting and important aspect of the russellsilver syndrome is itsvariation in phenotype. In this context, a phenotype is all the physical
http://www.magicfoundation.org/www/docs/112/russell_silver_syndrome.html
Members Only
Small for Gestational Age

Underlying Conditions of Growth Abnormalities

Networking
... Russell-Silver Syndrome General Information
Note: If you have difficulty understanding terms in this (or any other document) please refer to the More Information- section (lower left table) for Dictionary terms. If we have missed some terms, which you would find helpful in the dictionary, please let us know and we will add a definition for them. Also, on the left side tables you will see other important articles and information relating to this issue. Feel free to look at them for more help. Thank you. In 1953 and 1954, Drs. Silver and Russell independently described groups of small-for-gestational-age [SGA] children whose pregnancies had been complicated by intrauterine growth retardation [IUGR]. Their common findings were short stature without catch-up growth, normal head size for age, a distinctive triangular face, low-set ears and incurving fifth fingers. These two groups of patients are now considered to have had variations of the same disorder that we now call Russell-Silver Syndrome [RSS] in the U.S. and Silver-Russell Syndrome [SRS] in Europe. One interesting and important aspect of the Russell-Silver syndrome is its variation in phenotype. In this context, a phenotype is all the physical characteristics and abnormalities found in an individual patient that are attributed specifically to RSS. Some individuals with RSS have many traits, thus a severe phenotype, while others have very few traits, thus a mild phenotype.

42. The MAGIC Foundation
Disorders Small for Gestational Age russellsilver syndrome. russell-silversyndrome. Printable Version Email this Page Larger font
http://www.magicfoundation.org/www/docs/180/russell_silver_syndrome.html
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Disorders ... Russell-Silver Syndrome Russell-Silver Syndrome Printable Version Email this Page Larger font Powered by TruePresence TM The MAGIC Foundation 1.800.362.4423 (1.800.3 MAGIC 3) info@magicfoundation.org Link Policy

43. Russell-Silver Syndrome Medical Information
russellsilver syndrome Information from Drugs.com.
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Russell-Silver syndrome
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Russell-Silver syndrome
Definition
Russell-Silver syndrome is a disorder present at birth and diagnosed no later than early childhood. It is characterized by poor growth demonstrated by low birth weight and short stature (adult height usually less than 5 feet) and, frequently, asymmetry in the size of the two halves or other parts of the body. The facial features may have a characteristic appearance:
  • triangular shape with broad forehead small pointed chin thin wide mouth
Children with Russell-Silver syndrome are at risk for developmental and motor delays as well as learning disabilities.
Alternative Names
Silver-Russell syndrome; Silver syndrome

44. Raisingkids: Ask Our Experts - Silver-Russell Syndrome
What is silverrussell syndrome, and is it associated with poor weight-gain?Our Nutrition expert, David Swain, advises.
http://www.raisingkids.co.uk/ask/ex03_pri01.asp
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Ask Our Experts: Silver-Russell Syndrome
What is Silver-Russell Syndrome, and is it associated with poor weight-gain? Our nutrition expert, David Swain, advises. Raisingkids member's problem
My son has been fed though a gastrostomy since he was 3 months old and now he won't eat. He gets 1000 ml of 'Nutrini Extrain' his feeding pump at night but he's still underweight and doesn't put on weight well. He also has Silver-Russell Syndrome and a curved spine - could this have anything to do with it? If not, have you any suggestions on how to help? He is 5 yrs-old now, and I don't get any answers from anybody. David's advice
Russell-Silver syndrome (RSS) has a number of recognised characteristics. Some individuals have many of the documented traits, while others have very few. Almost every individual with the syndrome has:
  • low birth weight (Intrauterine Growth Retardation) decreased birth length triangular shaped face (lessens with age) long narrow head at birth postnatal growth retardation poor appetite in early years
Beyond this the characteristics vary very widely. However, the early years are often the most difficult and typically appetite will improve with age.

45. Russell-Silver Syndrome - [Support Group]
This information is provided as a resource and does not constitute an endorsementfor any group. It is the responsibility of the reader to decide whether a
http://www.bchealthguide.org/kbase/shc/shc29rus.htm
var hwPrint=1;var hwDocHWID="shc29rus";var hwDocTitle="Russell-Silver Syndrome";var hwRank="1";var hwSectionHWID="shc29rus-Header";var hwSource="en-caQ2_05";var hwDocType="Shc";
Self Help Clearinghouse
Russell-Silver Syndrome
This information is provided as a resource and does not constitute an endorsement for any group. It is the responsibility of the reader to decide whether a group is appropriate for his/her needs. For evidence-based information on diseases, conditions, symptoms, treatment and wellness issues, continue searching this site.
Russell-Silver Syndrome Support Network
National network. Founded 1989.
Network and exchange of information for parents of children with Russell Silver syndrome. Information and referrals, phone support, pen pals, conferences, annual convention, literature. Newsletter $30yr.
WRITE:
Russell-Silver Syndrome Support Network
c/o MAGIC Foundation
6645 W. North Ave.
Oak Park, IL 60302
CALL: 1-800-362-4423
FAX: 708-383-0899
E-MAIL: mary@magicfoundation.org
WEBSITE: http://www.magicfoundation.org

46. Russell-Silver Syndrome
Rusellsilver syndrome is a congenital disease (present at birth) characterizedby and, frequently, asymmetry in the size of the two halves or other parts
http://adam.about.com/encyclopedia/001209trt.htm
zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zCMt='a00' About Healthcare Center Healthcare Center Essentials ... Surgeries and Procedures zau(256,152,145,'gob','http://z.about.com/5/ad/go.htm?gs='+gs,''); Allergies Arthritis Asthma Breast Cancer ... 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/496/6.htm','');w(xb+xb); Search Healthcare Center ENCYCLOPEDIA INDEX Injury Disease Nutrition Poison ... Z
Russell-Silver syndrome
Overview Symptoms Treatment Prevention Alternative Names: Silver-Russell syndrome; Silver syndrome Treatment: There is no specific treatment for Russell-Silver syndrome. Symptoms are treated as necessary. Sometimes growth hormone replacement is recommended if a growth hormone deficiency is discovered. This has had varying results. Supportive treatment for discrepancy of leg length may prevent problems. Support Groups: Additional resources are available from Little People of America (888-572-2001) and MAGIC Foundation for Children's Growth (800-362-4423). Expectations (prognosis): As the child ages, many will improve in growth and appearance. There is normal intelligence.

47. Russell-Silver Syndrome
Rusellsilver syndrome is a congenital disease (present at birth) characterizedby and, frequently, asymmetry in the size of the two halves or other parts
http://adam.about.com/encyclopedia/001209sym.htm
zJs=10 zJs=11 zJs=12 zJs=13 zc(5,'jsc',zJs,9999999,'') zCMt='a00' About Healthcare Center Healthcare Center Essentials ... Surgeries and Procedures zau(256,152,145,'gob','http://z.about.com/5/ad/go.htm?gs='+gs,''); Allergies Arthritis Asthma Breast Cancer ... 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/496/6.htm','');w(xb+xb); Search Healthcare Center ENCYCLOPEDIA INDEX Injury Disease Nutrition Poison ... Z
Russell-Silver syndrome
Overview Symptoms Treatment Prevention Alternative Names: Silver-Russell syndrome; Silver syndrome Symptoms: Signs and tests: A physical examination may show:
  • delayed closure of anterior fontanel (soft spot) side to side asymmetry (especially limbs) curved-in fifth finger (clinodactyly) normal intelligence small or "pushed-back" jaw ( micrognathia or retrognathia ) small triangular face with normal head circumference
Tests include:

48. N.C.M.G. Ireland | Silver-Russell Syndrome Service Description.
The National Centre for Medical Genetics provides a comprehensive service forall patients and families in the Republic of Ireland affected by or at risk of
http://www.genetics.ie/molecular/srs/
@import "../../css/layout.css"; home molecular genetics Silver-Russell Syndrome
National Centre for Medical Genetics
Silver-Russell Syndrome
Background and Standard Service Information
Silver-Russell syndrome, also known as Russell-Silver Syndrome or RSS is a malformation syndrome characterised by pre- and post-natal growth retardation. While the exact aetiology of RSS is unknown, 10% of cases are due to maternal uniparental disomy of the entire chromosome 7 [mUPD(7)]; that is, the patient has inherited both copies of chromosome 7 from the mother and none from the father. If present, mUPD(7) helps to make the diagnosis and indicates a low recurrence risk. Specific clinical features of RSS include:
  • Pre and post natal growth retardation. Cerebral haemorrhage. Feeding difficulties (at 16 months) triangular face. Downturned mouth, micrognathia (unusually small jaw). Broad high forehead. Pointed chin. Low prominent dysplastic ears. Clinodactyly (inward bending) of little fingers and toes and mild psychomotor developmental delay.
There is some evidence to suggest that mUPD(7) patients are less likely to have the triangular face, downturned mouth and micrognathia.

49. Russell-Silver Syndrome - General Practice Notebook
russellsilver syndrome is an inherited cause of short stature and a number ofother characteristic features. After a provocation test there is a surge in
http://www.gpnotebook.co.uk/cache/-603586547.htm
Russell-Silver syndrome Russell-Silver syndrome is an inherited cause of short stature and a number of other characteristic features. After a provocation test there is a surge in growth hormone; there is an initial response to growth hormone treatment which may not be maintained. There is an association with Wilm's tumour.
Click here for more information...

50. Silver-Russell Syndrome (Alexander Russell) (www.whonamedit.com)
silverrussell syndrome (Alexander russell) A syndrome of intra-uterine dwarfismwith characterized by short stature, skeletal asymmetry, triangular shaped
http://www.whonamedit.com/synd.cfm/2892.html

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Silver-Russell syndrome (Alexander Russell) Also known as: Russell dwarf Russell nanism Russell syndrome Russell-Silver syndrome Silver's syndrome Synonyms: Asymmetry-dwarfism syndrome. Associated persons: Alexander Russell Henry K. Silver Description: A syndrome of intra-uterine dwarfism with low birth weight characterized by short stature, skeletal asymmetry, relatively large skull with triangular shaped head, small incurved little fingers, characteristic facies, precocious sexual development. Renal and/or ureteral defects and mental retardation may occur in some cases. Café au lait macules are present in nearly half the cases. Nephroblastoma can be a complication. No sex preponderance. The mode of inheritance is unknown. Some authors separate the Russell and Silver syndromes as independent entities, the Russell syndrome being a similar disturbance, but not asymmetric. However, Russell's and Silver's are now considered the same entity. An affected individual is known as the Russell dwarf.

51. Russell-Silver Syndrome
Rusellsilver syndrome is a congenital disease (present at birth) characterizedby and, frequently, asymmetry in the size of the two halves or other parts
http://www.ehendrick.org/healthy/001209trt.htm
Injury Disease Nutrition Poison ... Prevention
Russell-Silver syndrome
Alternative Names
Silver-Russell syndrome; Silver syndrome
Treatment
There is no specific treatment for Russell-Silver syndrome. Symptoms are treated as necessary. Sometimes growth hormone replacement is recommended if a growth hormone deficiency is discovered. This has had varying results. Supportive treatment for discrepancy of leg length may prevent problems.
Support Groups
Additional resources are available from Little People of America (888-572-2001) and MAGIC Foundation for Children's Growth (800-362-4423).
Outlook (Prognosis)
As the child ages, many will improve in growth and appearance. There is normal intelligence.
Possible Complications
  • self esteem and emotional problems related to appearance chewing or speaking difficulty if jaw is very small there may be an increased risk for tumors of the liver
When to Contact a Medical Professional
There may be some indications of this condition at birth. Call your health care provider if your child does not seem to be growing normally and has asymmetry of body parts or in-turned 5th fingers.
Review Date: 1/30/2003
Reviewed By: Douglas R. Stewart, M.D., Division of Medical Genetics, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

52. Russell-Silver Syndrome
Rusellsilver syndrome is a congenital disease (present at birth) characterizedby and, frequently, asymmetry in the size of the two halves or other parts
http://www.ehendrick.org/healthy/001209sym.htm
Injury Disease Nutrition Poison ... Prevention
Russell-Silver syndrome
Alternative Names
Silver-Russell syndrome; Silver syndrome
Symptoms
Exams and Tests
A physical examination may show:
  • delayed closure of anterior fontanel (soft spot) side to side asymmetry (especially limbs) curved-in fifth finger (clinodactyly) normal intelligence small or "pushed-back" jaw ( micrognathia or retrognathia ) small triangular face with normal head circumference
Tests include:
  • A glucose tolerance test may show episodes of low blood sugar when fasting. Skeletal X-rays may show a discrepancy (delay) between "bone age" (the age that the bones appear) and the actual age or sexual development. A karyotype (examination of a person's chromosomes under a microscope) is frequently ordered. About 10% of patients will have 2 copies (instead of 1) of their mother's chromosome 7 (a situation called maternal uniparenal disomy). Rarely, duplication of genes in a certain area of the chromosome 7 inherited from the mother is seen.
Review Date: 1/30/2003
Reviewed By: Douglas R. Stewart, M.D., Division of Medical Genetics, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

53. Dwarfism Types And Definitions
russellsilver syndrome is marked by low birthweight, and appetite is often poor Read a description of russell-silver syndrome by the MAGIC Foundation.
http://www.lpaonline.org/resources_dwarftypes.html
LPA Online
Dwarfism Resources
TYPES AND DEFINITIONS
And announcing the LPA Medical Resource Center
The links on this page are intended as a starting point for the lay person new parents, teachers, and the like.
Little People of America is pleased to announce a comprehensive resource for medical information about dwarfism: the LPA Medical Resource Center. Click here to enter. This is where you should go to find information that is more in-depth. It is intended not just for the lay person, but for medical professionals as well. The Medical Resource Center is a major project overseen by LPA's medical resource director, Ericka Peasley, and technology activist David Bradford.
Information about other types of dwarfism, and additional resources for those already listed, are always welcome. Send them to the LPA Online editor, Dan Kennedy, at dkennedy@lpaonline.org

54. Chromosome 7 Clinical Table UNIPARENTAL DISOMY 7
10756351, 46, XY, upd(7) mat, russellsilver syndrome, cystic fibrosis, pter,qter, 10756351, 10789928, isodisomy, Edit. 1463018, 46, XY, upd(7) mat
http://www.chr7.org/clinical.php?breaktype=UNIPARENTAL DISOMY 7&browser=gbrowse

55. Arch Pediatr Adolesc Med -- Abstract: Russell-Silver Syndrome And Hypopituitaris
russellsilver syndrome (RSS) is a sporadic form of prenatal onset dwarfism withtypical facial features, variable asymmetry, and linear growth 3 to 4 SDs
http://archpedi.ama-assn.org/cgi/content/abstract/140/2/155
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 140 No. 2, February 1986 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager Articles in PubMed by Cassidy SB Carey DE Contact me when this article is cited
Russell-Silver syndrome and hypopituitarism. Patient report and literature review
S. B. Cassidy, O. Blonder, V. W. Courtney, S. K. Ratzan and D. E. Carey
Russell-Silver syndrome (RSS) is a sporadic form of prenatal onset dwarfism with typical facial features, variable asymmetry, and linear growth 3 to 4 SDs below the mean. Endocrinologic studies are usually normal; however, six cases of RSS with growth hormone deficiency have been reported, three of which had additional pituitary abnormalities. We describe another case, a

56. Canadian Directory Of Genetic Support Groups
russellsilver syndrome Network 448 North Macombe St. Munro, Michigan, USA, 48161Tel 313.242.2219 Canadian Liaison Kim Blais 15 Baltimore Road
http://www.lhsc.on.ca/programs/medgenet/russells.htm

Index by
support group Canadian Directory of Genetic Support Groups
Russell-Silver Syndrome Network
448 North Macombe St.
Munro, Michigan, USA, 48161
Tel: 313.242.2219
Canadian Liaison:
Kim Blais
15 Baltimore Road
Barrie, Ontario, Canada, L4M 5M6 Page revised:December 16, 2002 Maintained by: Janice Little
Index by disease/ disorder
Return to Programs and Services Questions? Comments? Contact Janice Little London Health Sciences Centre . Please read our

57. TCAG-The Centre For Applied Genomics
Statistical Analysis Microarray Facility Genome Resources, Gene Isolation Cytogenomics. russellsilver syndrome. RSS Consent Form.pdf
http://tcag.bioinfo.sickkids.on.ca/index.php?pagename=rss.php

58. Crossed Asymmetry In Russell-Silver Syndrome -- Qazi Et Al. 14 (1): 74 -- Journa
Crossed asymmetry in russellsilver syndrome report by silver et al (1953),more than 50 examples of the russell-silver syndrome have been reported.
http://jmg.bmjjournals.com/cgi/content/abstract/14/1/74

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ARTICLES
Crossed asymmetry in Russell-Silver syndrome
QH Qazi, EG Kassner and C Ganapathy
Since the initial report by Silver et al (1953), more than 50 examples of the Russell-Silver syndrome have been reported. Unilateral congenital asymmetry of the extremities has been considered one of the major features of this disorder (Silver, 1964). We recently observed a child with

59. The Spectrum Of Silver-Russell Syndrome: A Clinical And Molecular Genetic Study
The silverrussell syndrome (SRS) is characterised by severe intrauterine Unilateral disomy as a possible explanation for russell-silver syndrome.
http://jmg.bmjjournals.com/cgi/content/full/36/11/837

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The spectrum of Silver-Russell syndrome: a clinical and molecular genetic study and new diagnostic criteria
S M Price a , R Stanhope b , C Garrett c , M A Preece b , R C Trembath d a Child Health Directorate, Northampton General Hospital NHS Trust, Cliftonville, Northampton NN1 5BD, UK, b Biochemistry, Endocrinology, and Metabolism Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK, c The Kennedy-Galton Centre, North West Thames Regional Genetic Service, Level 8V, Northwick Park Hospital, Watford Road, Harrow HA1 3UJ, UK

60. Renal Abnormalities In The Russell-Silver Syndrome -- Haslam Et Al. 51 (2): 216
be included in the investigation of these patients. Pediatrics, 51216, 1973,russellsilver syndrome, INTRAUTERINE GROWTH RETARDATION, RENAL ANOMALIES.
http://pediatrics.aappublications.org/cgi/content/abstract/51/2/216
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Renal abnormalities in the Russell-Silver syndrome
RHA Haslam, W Berman and RM Heller
The John F. Kennedy Institute, 707 North Broadway, Baltimore, Maryland 21205. ABSTRACT. There has been a recent report of five children with the Russell-Silver syndrome. It was suggested that the clinical features were well-established, and the only laboratory investigation necessary was a fasting blood glucose to exclude hypoglycemia. This study is a review of the status of the genitourinary system in six patients with the Russell-Silver syndrome. Four had abnormal excretory urograms or

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