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Silver-Russell Syndrome

Genetically and clinically heterogeneous disorder characterized by low birth weight, postnatal growth retardation, facial dysmorphism, bilateral body asymmetry, and clinodactyly of the fifth fingers. Alterations in GENETIC IMPRINTING are involved. Hypomethylation of IGF2/H19 locus near an imprinting center region of chromosome 11p15 plays a role in a subset of Silver-Russell syndrome. Hypermethylation of the same chromosomal region, on the other hand, can cause BECKWITH-WIEDEMANN SYNDROME. Maternal UNIPARENTAL DISOMY for chromosome 7 is known to play a role in its etiology.
Also Known As:
Russell Silver Syndrome; Dwarfism, Silver Russell; Dwarfism, Silver-Russell; Silver Russell Syndrome; Syndrome, Russell Silver; Syndrome, Silver-Russell; Silver Russell Dwarfism; Silver-Russell Dwarfism
Networked: 92 relevant articles (0 outcomes, 10 trials/studies)

Disease Context: Research Results

Related Diseases

1. Fetal Growth Retardation (Intrauterine Growth Retardation)
2. Beckwith-Wiedemann Syndrome (Exomphalos Macroglossia Gigantism Syndrome)
3. Uniparental Disomy
4. Turner Syndrome (Turner's Syndrome)
5. Prader-Willi Syndrome (Syndrome, Prader-Willi)

Experts

1. Eggermann, Thomas: 8 articles (07/2015 - 09/2002)
2. Binder, Gerhard: 6 articles (07/2015 - 09/2002)
3. Begemann, Matthias: 4 articles (07/2015 - 01/2010)
4. Binder, G: 4 articles (10/2014 - 11/2006)
5. Wollmann, Hartmut A: 4 articles (03/2008 - 09/2002)
6. Ranke, Michael B: 3 articles (01/2010 - 09/2002)
7. Ranke, M B: 3 articles (01/2008 - 09/2006)
8. Eggermann, T: 3 articles (01/2008 - 09/2006)
9. Schönherr, N: 3 articles (01/2008 - 09/2006)
10. Wollmann, H A: 3 articles (01/2008 - 09/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Silver-Russell Syndrome:
1. Growth Hormone (Somatotropin)IBA
2. SRS-AIBA
3. IGF Type 1 Receptor (IGF 1 Receptor)IBA
4. Untranslated RNA (Noncoding RNA)IBA
5. Insulin-Like Growth Factor Binding ProteinsIBA
6. Insulin-Like Growth Factor I (IGF-1)IBA
7. DNA (Deoxyribonucleic Acid)IBA
8. SilverIBA
9. trisomy mosaic Chromosome 7IBA
10. Myoclonic dystoniaIBA

Therapies and Procedures

1. Ligation
2. Distraction Osteogenesis
3. Fat-Restricted Diet (Diet, Fat Restricted)
4. Osteotomy
5. Long-Term Care