Silver-Russell Syndrome Treatment & Management

  • Author: Robert J Ferry Jr, MD; Chief Editor: Bruce Buehler, MD   more...
 
Updated: Jun 14, 2011
 

Medical Care

Growth

Significant effort must be undertaken to optimize caloric intake.

Growth hormone therapy should be considered in a child with Silver-Russell syndrome (SRS) who has not manifested adequate catch-up growth by age 2 years. Growth hormone was approved in the United States in 2001 by the US Food and Drug Administration for use in children born small for gestational age who have not yet manifested adequate catch-up growth by age 2 years. Recombinant human growth hormone (rhGH) is given via daily subcutaneous injections. Recommended dose is 0.48 mg/kg/wk.

Development

An early intervention program, including physical therapy, is beneficial.

Special education courses are needed when the child is older.

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Surgical Care

  • Consider enteral feeding if the patient does not tolerate oral feeding and has severe failure to thrive. Nasogastric or percutaneous endoscopic gastrostomy (PEG) feeds are needed to facilitate growth and maintenance.
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Consultations

  • Consult a clinical geneticist to differentiate Silver-Russell syndrome from etiologies of growth retardation, asymmetry, and other clinical findings.
  • Consult a gastroenterologist or nutritionist to optimize caloric intake and enhance growth and feeding therapy.
  • Developmental evaluation places the patient in early intervention, special education programs, physical therapy, and occupational therapy as early as possible.
  • Consult a pediatric endocrinologist to consider the use of recombinant human growth hormone (rhGH) in an infant nearing age 2 years who has not manifested adequate catch-up growth.
  • Consult an ophthalmologist to identify refractive errors and other abnormalities of vision commonly associated with Silver-Russell syndrome.
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Diet

  • Patients should have a nutritional evaluation to provide optimal calories for growth. As stated previously, nasogastric or PEG feeding may be required.
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Contributor Information and Disclosures
Author

Robert J Ferry Jr, MD  Professor and Chief, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN; Brigade Surgeon, 36th Sustainment Brigade, US Army; Adjunct Professor, Pediatric Surgery Department, King Saud University, Riyadh, Saudi Arabia

Robert J Ferry Jr, MD, is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society

Disclosure: Nutropin Speakers Bureau Honoraria Speaking and teaching; Genotropin Speakers Bureau Honoraria Speaking and teaching; Eli Lilly & Co. Grant/research funds Investigator; MacroGenics, Inc. Grant/research funds Investigator; Ipsen, S.A. (formerly Tercica, Inc.) Grant/research funds Investigator; NovoNordisk SA Grant/research funds Investigator; Diamyd Investigator

Specialty Editor Board

Elaine H Zackai, MD  Professor of Pediatrics, Professor of Obstetrics and Gynecology, Professor of Pediatrics in Human Genetics, University of Pennsylvania School of Medicine; Director, Clinical Genetics Center, University of Pennsylvania; Senior Physician and Director of Clinical Genetics, The Children's Hospital of Philadelphia

Elaine H Zackai, MD is a member of the following medical societies: American Cleft Palate/Craniofacial Association, American College of Medical Genetics, and American Society of Human Genetics

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Robert Anthony Saul, MD  Clinical Professor, Department of Pediatrics, University of South Carolina; Senior Clinical Geneticist, Greenwood Genetic Center

Robert Anthony Saul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics, and American College of Physician Executives

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Bruce Buehler, MD  Professor, Department of Pediatrics and Genetics, Director RSA, University of Nebraska Medical Center

Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Ainu Prakash-Cheng, MD, PhD; Lawrence A Wetterau, MD; and Margaret M McGovern, MD, PhD, to the development and writing of this article.

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Failure of growth in weight, length, and head circumference starting at birth, suggesting an organic etiology that occurred in utero.
 
 
 
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