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Silver-Russell Syndrome Summary

Description: 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 Show All >>

Networked: 92 relevant articles (0 outcomes, 10 trials/studies) for this Disease

Key Drugs and Agents for Silver-Russell Syndrome

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Growth Hormone (Somatotropin) : 5 studies in 26 results : IBA
  2. SRS-A : 2 studies in 6 results : IBA
  3. IGF Type 1 Receptor (IGF 1 Receptor) : 1 study in 3 results : IBA
  4. Untranslated RNA (Noncoding RNA) : 1 study in 1 result : IBA
  5. Insulin-Like Growth Factor Binding Proteins : 1 study in 1 result : IBA
  6. Insulin-Like Growth Factor I (IGF-1) : 5 results : IBA
  7. DNA (Deoxyribonucleic Acid) : 5 results : IBA
  8. Silver : 4 results : IBA
  9. trisomy mosaic Chromosome 7 : 3 results : IBA
  10. Myoclonic dystonia : 3 results : IBA
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Diseases Related to Silver-Russell Syndrome

  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)
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Key Therapies for Silver-Russell Syndrome

Efficacy Chart >>
  1. Ligation : 3 results
  2. Distraction Osteogenesis : 1 result
  3. Fat-Restricted Diet (Diet, Fat Restricted) : 1 result
  4. Osteotomy : 1 result
  5. Long-Term Care : 1 result
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