Turner Syndrome Medication

  • Author: Daniel C Postellon, MD; Chief Editor: Bruce Buehler, MD   more...
 
Updated: Sep 22, 2011
 

Human growth hormone

Class Summary

These agents are the primary treatment for short stature. They stimulate growth of linear bone, skeletal muscle, and organs.

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Somatotropin (Nutropin, Genotropin, Humatrope, Norditropin, Saizen, Tev-Tropin)

 

Taller adult heights are associated with earlier treatment and with the duration of treatment prior to induced or spontaneous puberty. With treatment, approximately 50% of patients reach an adult height of 150 cm (59") or more, compared with an untreated mean adult height of 142 cm (56").

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Anabolic steroids

Class Summary

This is an adjuvant for growth hormone therapy.

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Oxandrolone (Oxandrin, Anavar)

 

Of limited use. Some endocrinologists recommend use in patients diagnosed in their teens to achieve a maximum adult height quickly. When used, often combined with growth hormone to allow a lower dose, thus decreasing the potential for adverse effects.

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Thyroid replacement therapies

Class Summary

These agents are used for treatment of hypothyroidism.

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Levothyroxine (Synthroid, Levoxyl, Levothroid, L-thyroxine)

 

Hypothyroidism is common with Turner syndrome and is treated like any other hypothyroidism. Thyroid hormones influence growth and maturation of tissues. Involved in normal growth, metabolism, and development.

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Estrogen replacement therapies

Class Summary

Almost all individuals require estrogen replacement. Estrogen is usually started at chronologic age 12 years or older. Adults usually require cyclic therapy with both estrogen and progestin. Transdermal or parenteral estrogen may be useful in limiting some adverse effects of estrogen therapy.

Estrogens

 

Available in many forms (eg, estradiol [Estrace], conjugated estrogens [Premarin]). Transdermal therapy is more physiologic than oral medications. Restore estrogen levels to concentrations that induce negative feedback at gonadotrophic regulatory centers, which, in turn, reduces release of gonadotropins from pituitary. Increases synthesis of DNA, RNA, and many proteins in target tissues.

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Antihypertensive agents

Class Summary

These products are used to control hypertension and to ultimately prevent complications such as aortic dissection. The 2 most common class of medications used for these purposes in pediatric patients are beta-blockers and ACE inhibitors. Propranolol is an example of one of the beta-blockers used in pediatrics, whereas captopril is an example of an ACE inhibitor.

Additional drug recommendations for patients aged 1-17 years may be found in The Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents. For neonatal doses, see the eMedicine article Neonatal Hypertension. For pediatric doses, see the eMedicine article Hypertension.

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Propranolol (Inderal)

 

Has membrane-stabilizing activity and decreases automaticity of contractions.

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Captopril (Capoten)

 

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

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Vitamins and minerals

Class Summary

Osteoporosis is common and is a major cause of morbidity in adults. Treatment is the same as for other adult women with osteoporosis. Monitor diet and ensure an intake of at least 1 g/d of calcium and 400 IU/d of vitamin D. Treatment with growth hormone and estrogen are also important in the prevention of osteoporosis later in life.

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Ergocalciferol (Calciferol, Drisdol)

 

Vitamin D is a micronutrient essential for normal absorption of calcium and phosphorus. Also produced in response to exposure to ultraviolet B light.

Calcium salts (acetate, carbonate, chloride, gluconate)

 

Supplemental source of dietary calcium. Calcium carbonate is 40% elemental calcium.

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Contributor Information and Disclosures
Author

Daniel C Postellon, MD  Clinical Associate Professor, College of Human Medicine, Pediatrics and Human Development, Michigan State University; Consulting Staff, Pediatric Endocrine Clinic, Helen DeVos Children's Hospital

Daniel C Postellon, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Maala S Daniel, MBBS  Attending Physician, Division of Pediatric Endocrinology, Helen DeVos Children's Hospital

Maala S Daniel, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Medical Student Association/Foundation, and Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ian Krantz, MD  Department of Pediatrics, Assistant Professor, University of Pennsylvania and Children's Hospital of Philadelphia

Ian Krantz, MD is a member of the following medical societies: 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 School of Medicine; 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.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

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.

References

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A patient with Turner syndrome is shown. This posterior view shows a low hairline and a shield-shaped chest. Note the narrow hip development.
Lymphedema of the feet in an infant is shown. The toes have the characteristic sausagelike appearance.
Hyperconvex nails in Turner syndrome. Note U-shaped cross section.
Generalized lymphedema is seen here in an infant with Turner syndrome. The loose skin folds around the neck will form a webbed neck later in life.
 
 
 
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