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         Silver-russell 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. Growth Disorders: Achondroplasia, Dwarfism, Acromegaly, Primordial Dwarfism, Psychosocial Short Stature, Midgetville, Silver-russell Syndrome
  3. Russell-Silver syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Paul Johnson, 2005

1. EMedicine - Silver-Russell Syndrome : Article By Ainu Prakash-Cheng, MD, PhD
silverrussell syndrome. Last Updated December 12, 2003, FULL DISCLAIMER. silver-russell syndrome excerpt. © Copyright 2004, eMedicine.com, Inc.
http://www.emedicine.com/ped/topic2099.htm
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Silver-Russell Syndrome
Last Updated: December 12, 2003 Rate this Article Email to a Colleague Synonyms and related keywords: SRS, Russell-Silver syndrome, Silver-Russell dwarfism, Silver syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Ainu Prakash-Cheng, MD, PhD , Assistant Professor of Human Genetics and Pediatrics, Department of Human Genetics, Mount Sinai School of Medicine Coauthor(s): Margaret McGovern, MD, PhD , Vice Chair, Associate Professor, Department of Human Genetics, Mount Sinai School of Medicine Ainu Prakash-Cheng, MD, PhD, is a member of the following medical societies: American Academy of Pediatrics American Society of Gene Therapy , and American Society of Human Genetics Editor(s): Elaine H Zackai, MD , Director of Clinical Genetics Center, Professor of Pediatrics, Department of Pediatrics, Division of Human Genetics and Molecular Biology, University of Pennsylvania, Children's Hospital of Philadelphia;

2. Silver-Russell Syndrome (Alexander Russell) (www.whonamedit.com)
silverrussell syndrome (Alexander Russell) A syndrome of intra-uterine dwarfism with characterized by short stature, skeletal asymmetry, triangular shaped head and other anomalies. Also known as
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.

3. EMedicine - Silver-Russell Syndrome : Article Excerpt By: Ainu Prakash-Cheng, MD
eMedicine World Medical Library. (advertisement). Excerpt from silverrussell syndrome. Please click here to view the full topic text silver-russell syndrome.
http://www.emedicine.com/ped/byname/silver-russell-syndrome.htm
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Excerpt from Silver-Russell Syndrome
Synonyms, Key Words, and Related Terms: SRS, Russell-Silver syndrome, Silver-Russell dwarfism, Silver syndrome
Please click here to view the full topic text: Silver-Russell Syndrome
Background: Silver-Russell syndrome (SRS) originally was described by Silver and colleagues in 1953 and, soon afterwards, by Russell in 1954. The first reports were in children with characteristic facies, low birthweight, asymmetry, and growth retardation. Over the past several years, more than 400 patients have been described, with phenotypes ranging from mild to classic. Some patients have maternal uniparental disomy of chromosome 7, with the possibility of imprinting (eg, inheriting 2 copies of maternal chromosome 7, with no paternal contribution). Pathophysiology: Growth failure is the primary abnormality. Patients present with intrauterine growth retardation, difficulty feeding, failure to thrive, or postnatal growth retardation that may be caused by immature osseous development through early childhood. Although catch-up growth often occurs during adolescence, the final height still is less than normal ( -3.6 SD). Older children and adults do not manifest clinical features as clearly as infants or young children.

4. Russel-Silver Syndrome
RussellSilver Syndrome (RSS) is also known as silver-russell syndrome, Silver Syndrome, and Russell Syndrome.
http://www.magicfoundation.org/rss.html
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Russell-Silver Syndrome
In 1953 and 1954, respectively, Dr. H. K. Silver and Dr. A. Russell independently described a special group of IUGR (Intrauterine Growth Retardation) children with short stature along with a small triangular face, low-set ears, incurved fifth fingers, and other characteristics. Their independent and varied findings were eventually determined to be a variation of the same syndrome. (A syndrome is a group of physical signs and symptoms which make up a specific disease or disorder.) As a result, the syndrome received its name. Russell-Silver Syndrome (RSS) is also known as Silver-Russell Syndrome, Silver Syndrome, and Russell Syndrome. One of the interesting and at the same time unsettling aspects of this syndrome is the very wide variation of its characteristics. Some individuals with RSS have many of the documented traits, while others have very few. Mental retardation is not characteristic of this disorder.
Almost, without exception, every RSS individual has:

5. SILVER-RUSSELL SYNDROME : Contact A Family - For Families With Disabled Children
printer friendly, silverrussell syndrome, silver-russell syndrome Asymmetry Dwarfism; Russell-Silver syndrome; Silver s syndrome.
http://www.cafamily.org.uk/Direct/s30.html
printer friendly SILVER-RUSSELL SYNDROME home more about us in your area conditions information ... how you can help search this site Did you find this page
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yes no Silver-Russell syndrome: Asymmetry Dwarfism; Russell-Silver syndrome; Silver's syndrome Intra-uterine growth retardation without dysmorphic features is also covered by the group. Inheritance patterns
This is not yet fully determined. It has been suggested that while most cases are sporadic, some cases may be X-linked or autosomal dominant. However there is a congenital association between siblings which may result from placental insufficiency. Such placental problems may be hereditary. Prenatal diagnosis
There is no biochemical diagnosis but serial ultrasound assessments starting from early pregnancy will confirm intra-uterine growth retardation Medical text last updated October 2001 by Dr R Stanhope, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital, London, UK. Further Online Resources
Medical texts in The Contact a Family Directory are designed to give a short, clear description of specific conditions and rare disorders. More extensive information on this condition can be found on a range of reliable, validated web sites and links to them are included in the

6. Index S: Contact A Family - For Families With Disabled Children: Information On
Cell Anaemia see Sickle Cell Disorders Sickle Cell Disorders Sick New Born Babies see Prematurity and sick newborn silverrussell syndrome Silver s syndrome
http://www.cafamily.org.uk/Idx/s.html
printer friendly home more about us in your area ... how you can help search this site Please use the Index below to access the condition on which you require information. If you do not find what you want in the Index then try our search facility in the navigator on the left. Contact a Family also has information on many other specific conditions and rare disorders. If you cannot find the information you require in The Contact a Family Directory Online , you may wish to use our Contact a Family Helpline service. SCAD see Fatty Acid Oxidation Disorders
SCH see Lissencephaly
SCID see Primary Immunodeficiencies
SED see Restricted Growth
SIDS see Cot Death
SLE see Lupus
SLOS see Smith-Lemli-Opitz syndrome
SMA see Spinal Muscular Atrophy
SOD see Septo-Optic Dysplasia
SSPE see Subacute-Sclerosing Panencephalitis
S-adenosylhomocysteine hydolase deficiency see Saccade Initiation Failure see Congenital Ocular Motor Apraxia Sacral Agenesis Saethre-Chotzen see Craniofacial Conditions Sagital Craniosynostosis see Craniofacial Conditions Salaam attacks see West syndrome Sandhoff's disease see Metabolic diseases Sanfilippo see Mucopolysaccharide diseases and associated diseases Santavuori disease see Batten disease Santavuori-Haltia disease (infantile) see Batten disease Sarcoidosis Scapulohumeral Dystrophy see

7. MAGIC FOUNDATION
are now considered to have had variations of the same disorder that we now call RussellSilver Syndrome RSS in the US and silver-russell syndrome SRS in
http://www.magicfoundation.org/divisions/rss.htm

INTRODUCTION

PDF Brochure

Links Child's Site with RSS
coltongeorge.com
INTRODUCTION
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. When first described, RSS was NOT thought to be a genetic disorder because it recurred within families rarely, and when it did recur, its pattern of transmission failed to follow a consistent genetic mode of inheritance. More recent understandings of genetic mechanisms have led scientists to conclude that RSS is genetic, but its genetics are not simple. Scientists now believe that the RSS phenotype is associated with more than one genotype.

8. OMIM - SILVER-RUSSELL SYNDROME; SRS

http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=180860

9. Entrez PubMed
Click here to read The spectrum of silverrussell syndrome a clinical and molecular genetic study and new diagnostic criteria.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

10. AllRefer Health - Russell-Silver Syndrome (Silver Syndrome, Silver-Russell Syndr
Alternate Names Silver Syndrome, silverrussell syndrome. Definition. Rusell-Silver syndrome is a congenital disease (present at
http://health.allrefer.com/health/russell-silver-syndrome-info.html
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Russell-Silver Syndrome
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Alternate Names : Silver Syndrome, Silver-Russell Syndrome Definition Rusell-Silver syndrome is a congenital disease (present at birth) characterized by short stature and, frequently, asymmetry in the size of the two halves or other parts of the body. Genetic problems are thought to cause this syndrome, although the specific gene(s) remain to be discovered. The pattern of inheritance has not been determined, but in most cases it seems to occur without any family history of the condition. Children with Russell-Silver syndrome are born small and generally achieve less than 5 feet at adult height. Side-to-side asymmetry is fairly common. For example, a left body part may be larger than the right counterpart. This asymmetry may occur anywhere including the face.

11. AllRefer Health - Russell-Silver Syndrome (Silver Syndrome, Silver-Russell Syndr
Syndrome. RussellSilver Syndrome. Provided by ADAM. Alternate Names Silver Syndrome, silver-russell syndrome. Definition. Rusell-Silver
http://health.allrefer.com/health/russell-silver-syndrome.html
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You are here : AllRefer.com Health
Russell-Silver Syndrome
Alternate Names : Silver Syndrome, Silver-Russell Syndrome Definition Rusell-Silver syndrome is a congenital disease (present at birth) characterized by short stature and, frequently, asymmetry in the size of the two halves or other parts of the body. Jump to a section Definition
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Hypoglycemia

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Other Topics Anterior Micrognathia Short Stature Sweating - Excessive 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

12. Silver-Russell Syndrome - General Practice Notebook
silverrussell syndrome. Russell-Silver syndrome is an inherited cause of short stature and a number of other characteristic features.
http://www.gpnotebook.co.uk/cache/-1543110608.htm
Silver-Russell 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...

13. What Is Russell-Silvers Syndrome?
RSSKids.com provide support and information for families with children with Russel Silvers Syndrome. RussellSilver Syndrome (RSS) is also known as silver-russell syndrome, Silver Syndrome, and
http://www.buradley.com/rss/desc.htm
Russell-Silver Syndrome In 1953 and 1954, respectively, Dr. H. K. Silver and Dr. A. Russell independently described a special group of IUGR (Intrauterine Growth Retardation) children with short stature along with a small triangular face, low-set ears, incurved fifth fingers, and other characteristics. Their independent and varied findings were eventually determined to be a variation of the same syndrome. (A syndrome is a group of physical signs and symptoms which make up a specific disease or disorder.) As a result, the syndrome received its name. Russell-Silver Syndrome (RSS) is also known as Silver-Russell Syndrome, Silver Syndrome, and Russell Syndrome. One of the interesting and at the same time unsettling aspects of this syndrome is the very wide variation of its characteristics. Some individuals with RSS have many of the documented traits, while others have very few. Mental retardation is not characteristic of this disorder.

14. [DYSPHAGIA] Child With Silver-Russell Syndrome
DYSPHAGIA Child with silverrussell syndrome. I just received a referral from a pediatrician on a 9 year-old male with silver-russell syndrome.
http://list.dysphagia.com/dysphagia/1999-June/msg00098.html
Date Prev Date Next [Chronological] [Thread] ... [Top]
[DYSPHAGIA] Child with Silver-Russell Syndrome
  • Subject [DYSPHAGIA] Child with Silver-Russell Syndrome From cpruden@media-net.net (christina pruden) Date: Tue, 15 Jun 1999 19:09:11 -0500
I just received a referral from a pediatrician on a 9 year-old male with Silver-Russell Syndrome. One of his ongoing problems is poor weight gain. He is currently working with a nutritionist in our hospital. According to his mother, he has difficulty with swallowing/chewing food.He prefers liquids and a soft diet versus meat and fruit. Any ideas before I see this child I would appreciate any input. Christina Pruden cpruden@media-net.net - To UNSUBSCRIBE from this list, please send an e-mail message to majordomo@cyberport.com with the following text as a message: unsubscribe dysphagia -

15. [DYSPHAGIA] Child With Silver-Russell Syndrome
DYSPHAGIA Child with silverrussell syndrome. Subject DYSPHAGIA Child with silver-russell syndrome; From Kari@Fabrizio.com (Kari Fabrizio);
http://list.dysphagia.com/dysphagia/1999-June/msg00127.html
Date Prev Date Next [Chronological] [Thread] ... [Top]
[DYSPHAGIA] Child with Silver-Russell Syndrome
  • Subject [DYSPHAGIA] Child with Silver-Russell Syndrome From Kari@Fabrizio.com (Kari Fabrizio) Date: Thu, 17 Jun 1999 19:49:56 -0400
I just wanted to inform everyone that a virus was attached to a message from "christina pruden <cpruden@media-net.net>" dated 6/15/99, and everyone on this listserv had it sent to your computer. The virus is called Happy99 and it is sending itself without your knowledge. If you recived a message with the attachment Happy99.exe, delete the attachment WITHOUT opening it!!! If you open the file, it will spread to anyone you email. I would STRONGLY suggest EVERYONE purchase Anit-Virus software and keep the definitions updated. You might also let anyone else you emailed know about the virus so they can protect themselves. Kari Fabrizio - To UNSUBSCRIBE from this list, please send an e-mail message to majordomo@cyberport.com with the following text as a message: unsubscribe dysphagia -

16. Karger Publishers
Genetics of silverrussell syndrome EL Wakeling a,b , S. Abu-Amero a , SM Price c , P. Stanier a , RC Trembath d , GE Moore a , MA Preece a,b a Institute of
http://content.karger.com/ProdukteDB/produkte.asp?Doi=53085

17. Disease Directory : EMedicine - Silver-Russell Syndrome : Article By Ainu Prakas
Diseases Genetic Disorders Russell Silver Syndrome eMedicine silver-russell syndrome Article by Ainu Prakash Directory Listing.
http://www.diseasedirectory.net/detailed/26147.aspx
Wednesday, June 02, 2004 Genetic Disorders
Aarskog Syndrome

Aase Syndrome

Ablepharon-Macrostomia Syndrome
... Russell Silver Syndrome : eMedicine - Silver-Russell Syndrome : Article by Ainu Prakash ...
Directory Listing
Title: eMedicine - Silver-Russell Syndrome : Article by Ainu Prakash ...
Description: Synonyms and related keywords: SRS, Russell-Silver syndrome, Silver-Russell dwarfism, Silver syndrome. Vol. I. 1997: 737-752. Jones KL: Russell-Silver syndrome.
Date Added: 2/17/2004 10:18:15 AM
URL: http://www.emedicine.com/ped/topic2099.htm

18. Disease Directory : Silver-Russell Syndrome (Alexander Russell) (www.whonamedit.
Diseases Genetic Disorders Russell Silver Syndrome silverrussell syndrome (Alexander Russell) (www.whonamedit.com). Directory Listing.
http://www.diseasedirectory.net/detailed/26145.aspx
Wednesday, June 02, 2004 Genetic Disorders
Aarskog Syndrome

Aase Syndrome

Ablepharon-Macrostomia Syndrome
... Russell Silver Syndrome : Silver-Russell syndrome (Alexander Russell) (www.whonamedit.com)
Directory Listing
Title: Silver-Russell syndrome (Alexander Russell) (www.whonamedit.com)
Description: Also known as: Russell dwarf,Russell nanism,Russell syndrome,Russell-Silver syndrome,Silver's syndrome. Silver-Russell syndrome (Alexander Russell)
Date Added: 2/17/2004 10:18:15 AM
URL: http://www.whonamedit.com/synd.cfm/2892.html

19. Swiss Society Of Neonatology
A newborn girl with suspected silverrussell syndrome Nordmann Y, Fauchère JC, Bucher HU Clinic of Neonatology, University Hospital Zurich, Switzerland.
http://www.neonet.ch/cotm/html/body_case-june03.html
A newborn girl with suspected Silver-Russell syndrome
Nordmann Y, Fauchère JC, Bucher HU
Clinic of Neonatology, University Hospital Zurich, Switzerland We present the case of a girl with suspected Silver-Russell syndrome (SRS). SRS is a rare, clinically and genetically heterogeneous disorder involving intra-uterine and post-natal growth retardation with a wide spectrum of additional dysmorphic features. Because of the clinical heterogeneity, diagnosis can be rather subjective and is not always easy to confirm. Case presentation
The girl, third child of a 30-years-old mother, was born prematurely at 31 6/7 weeks of gestation by caesarean section because of a pathologic CTG. Intrauterine growth retardation had been diagnosed at 26 6/7 weeks of gestation. At that time, amniocentesis and TORCH serologies were performed with inconspicuous results.
Fig. 1. Large appearing head, high forehead, downturning corners of the mouth. Fig. 2. Asymmetry of the legs. Fig. 3. Weight and height Fig. 4.

20. Raisingkids: Ask Our Experts - Silver-Russell Syndrome
Ask Our Experts silverrussell syndrome What is silver-russell syndrome, and is it associated with poor weight-gain? Our nutrition
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.

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