Turner Syndrome Follow-up

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

Prognosis

Overall prognosis for patients with Turner syndrome is good.

  • Even with growth hormone therapy, most individuals are shorter than average.[29]
  • Turner syndrome is not a cause of mental retardation.[30]
  • Life expectancy is slightly shorter than average but may be improved by attention to associated chronic illnesses, such as obesity and hypertension.[31]
  • Almost all individuals are infertile, but pregnancy with donor embryos is possible.[32]
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Patient Education

For excellent patient education resources, please refer to eMedicine's Amenorrhea article, Endocrine System Center, and Women's Health Center. All these materials may be printed free of charge.

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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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  2. [Guideline] Donaldson MD, Gault EJ, Tan KW, Dunger DB. Optimising management in Turner syndrome: from infancy to adult transfer. Arch Dis Child. Jun 2006;91(6):513-20. [Medline].

  3. Natowicz M, Kelley RI. Association of Turner syndrome with hypoplastic left-heart syndrome. Am J Dis Child. Feb 1987;141(2):218-20. [Medline].

  4. [Best Evidence] Jorgensen KT, Rostgaard K, Bache I, et al. Autoimmune diseases in women with Turner's syndrome. Arthritis Rheum. Mar 2010;62(3):658-66. [Medline].

  5. [Guideline] Bondy CA. Care of girls and women with Turner syndrome: A guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab. Jan 2007;92(1):10-25. [Medline].

  6. Heinrichs C, Bourdoux P, Saussez C, Vis HL, Bourguignon JP. Blood spot follicle-stimulating hormone during early postnatal life in normal girls and Turner's syndrome. J Clin Endocrinol Metab. Apr 1994;78(4):978-81. [Medline].

  7. Conte FA, Grumbach MM, Kaplan SL. A diphasic pattern of gonadotropin secretion in patients with the syndrome of gonadal dysgenesis. J Clin Endocrinol Metab. Apr 1975;40(4):670-4. [Medline].

  8. Lyon AJ, Preece MA, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child. Oct 1985;60(10):932-5. [Medline].

  9. [Best Evidence] Ross JL, Quigley CA, Cao D, Feuillan P, Kowal K, Chipman JJ. Growth hormone plus childhood low-dose estrogen in Turner's syndrome. N Engl J Med. Mar 31 2011;364(13):1230-42. [Medline].

  10. Illig R, Tolksdorf M, Mürset G, Prader A. LH and FSH response to synthetic LH-RH in children and adolescents with Turner's and Klinefelter's syndrome. Helv Paediatr Acta. Oct 1975;30(3):221-31. [Medline].

  11. Sculerati N, Ledesma-Medina J, Finegold DN, Stool SE. Otitis media and hearing loss in Turner syndrome. Arch Otolaryngol Head Neck Surg. Jun 1990;116(6):704-7. [Medline].

  12. [Guideline] Frias JL, Davenport ML. Health supervision for children with Turner syndrome. Pediatrics. Mar 2003;111(3):692-702. [Medline].

  13. Pai GS, Leach DC, Weiss L, Wolf C, Van Dyke DL. Thyroid abnormalities in 20 children with Turner syndrome. J Pediatr. Aug 1977;91(2):267-9. [Medline].

  14. Alpman A, Cogulu O, Akgul M, et al. Prenatally Diagnosed Turner Syndrome and Cystic Hygroma: Incidence and Reasons for Referrals. Fetal Diagn Ther. Mar 12 2008;25(1):58-61. [Medline].

  15. Carr RF, Ochs RH, Ritter DA, Kenny JD, Fridey JL, Ming PM. Fetal cystic hygroma and Turner's syndrome. Am J Dis Child. Jun 1986;140(6):580-3. [Medline].

  16. Rao E, Weiss B, Fukami M, Rump A, Niesler B, Mertz A. Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in idiopathic short stature and Turner syndrome. Nat Genet. May 1997;16(1):54-63. [Medline].

  17. [Guideline] Davenport ML. Approach to the patient with Turner syndrome. J Clin Endocrinol Metab. Apr 2010;95(4):1487-95. [Medline].

  18. Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi IT. Detection of hidden Y mosaicism in Turner's syndrome: importance in the prevention of gonadoblastoma. J Pediatr Endocrinol Metab. Sep 2006;19(9):1113-7. [Medline].

  19. Birdsall M, Kennedy S. The risk of aortic dissection in women with Turner syndrome. Hum Reprod. Jul 1996;11(7):1587. [Medline].

  20. Elsheikh M, Conway GS, Wass JA. Medical problems in adult women with Turner's syndrome. Ann Med. Apr 1999;31(2):99-105. [Medline].

  21. [Best Evidence] Davenport ML, Crowe BJ, Travers SH, Rubin K, Ross JL, Fechner PY, et al. Growth hormone treatment of early growth failure in toddlers with Turner syndrome: a randomized, controlled, multicenter trial. J Clin Endocrinol Metab. Sep 2007;92(9):3406-16. [Medline].

  22. Backeljauw P. Does growth hormone therapy before 4 years of age enhance the linear growth of girls with Turner's syndrome?. Nat Clin Pract Endocrinol Metab. Feb 2008;4(2):78-9. [Medline].

  23. [Best Evidence] Bolar K, Hoffman AR, Maneatis T, Lippe B. Long-term safety of recombinant human growth hormone in turner syndrome. J Clin Endocrinol Metab. Feb 2008;93(2):344-51. [Medline].

  24. Ross JL, Quigley CA, Cao D, Feuillan P, Kowal K, Chipman JJ, et al. Growth hormone plus childhood low-dose estrogen in Turner's syndrome. N Engl J Med. Mar 31 2011;364(13):1230-42. [Medline].

  25. [Best Evidence] Ankarberg-Lindgren C, Elfving M, Wikland KA, Norjavaara E. Nocturnal application of transdermal estradiol patches produces levels of estradiol that mimic those seen at the onset of spontaneous puberty in girls. J Clin Endocrinol Metab. Jul 2001;86(7):3039-44. [Medline].

  26. [Best Evidence] Bannink EM, van der Palen RL, Mulder PG, de Muinck Keizer-Schrama SM. Long-term follow-up of GH-treated girls with Turner syndrome: metabolic consequences. Horm Res. 2009;71(6):343-9. [Medline].

  27. Zuckerman-Levin N, Frolova-Bishara T, Militianu D, Levin M, Aharon-Peretz J, Hochberg Z. Androgen replacement therapy in Turner syndrome: a pilot study. J Clin Endocrinol Metab. Dec 2009;94(12):4820-7. [Medline].

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  29. [Best Evidence] Stephure DK. Impact of growth hormone supplementation on adult height in turner syndrome: results of the Canadian randomized controlled trial. J Clin Endocrinol Metab. Jun 2005;90(6):3360-6. [Medline].

  30. Bender B, Puck M, Salbenblatt J, Robinson A. Cognitive development of unselected girls with complete and partial X monosomy. Pediatrics. Feb 1984;73(2):175-82. [Medline].

  31. Price WH, Clayton JF, Collyer S, De Mey R, Wilson J. Mortality ratios, life expectancy, and causes of death in patients with Turner's syndrome. J Epidemiol Community Health. Jun 1986;40(2):97-102. [Medline].

  32. [Best Evidence] Foudila T, Soderström-Anttila V, Hovatta O. Turner's syndrome and pregnancies after oocyte donation. Hum Reprod. Feb 1999;14(2):532-5. [Medline]. [Full Text].

  33. Garden AS, Diver MJ, Fraser WD. Undiagnosed morbidity in adult women with Turner's syndrome. Clin Endocrinol (Oxf). Nov 1996;45(5):589-93. [Medline].

  34. Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics. Jul 1998;102(1):e12. [Medline]. [Full Text].

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  36. [Best Evidence] Kaneko N, Kawagoe S, Hiroi M. Turner's syndrome--review of the literature with reference to a successful pregnancy outcome. Gynecol Obstet Invest. 1990;29(2):81-7. [Medline].

  37. Chorazy PA, Himelhoch S, Hopwood NJ, Greger NG, Postellon DC. Persistent hypothyroidism in an infant receiving a soy formula: case report and review of the literature. Pediatrics. Jul 1995;96(1 Pt 1):148-50. [Medline].

  38. Davenport ML, Punyasavatsut N, Stewart PW, Gunther DF, Savendahl L, Sybert VP. Growth failure in early life: an important manifestation of Turner syndrome. Horm Res. 2002;57(5-6):157-64. [Medline].

  39. Even L, Cohen A, Marbach N, Brand M, Kauli R, Sippell W, et al. Longitudinal analysis of growth over the first 3 years of life in Turner's syndrome. J Pediatr. Oct 2000;137(4):460-4. [Medline].

  40. Fryer SL, Kwon H, Eliez S, Reiss AL. Corpus callosum and posterior fossa development in monozygotic females: a morphometric MRI study of Turner syndrome. Dev Med Child Neurol. May 2003;45(5):320-4. [Medline].

  41. [Guideline] Gravholt C. Clinical Practice in Turner Syndrome. Nature Clinical Practice Endocrinology & Metabolism. 2005;1:41-52.

  42. Gravholt CH, Juul S, Naeraa RW, Hansen J. Morbidity in Turner syndrome. J Clin Epidemiol. Feb 1998;51(2):147-58. [Medline].

  43. Hasle H, Olsen JH, Nielsen J, Hansen J, Friedrich U, Tommerup N. Occurrence of cancer in women with Turner syndrome. Br J Cancer. May 1996;73(9):1156-9. [Medline].

  44. Health supervision for children with Turner syndrome. American Academy of Pediatrics. Committee on Genetics. Pediatrics. Dec 1995;96(6):1166-73. [Medline].

  45. Lippe BM. Primary Ovarian Failure. In: Clinical Pediatric Endocrinology. 1990. Philadelphia, Pa: WB Saunders; 325-366.

  46. Mazzanti L, Prandstraller D, Tassinari D, Rubino I, Santucci S, Picchio FM, et al. Heart disease in Turner's syndrome. Helv Paediatr Acta. Aug 1988;43(1-2):25-31. [Medline].

  47. Medeiros CC, Marini SH, Baptista MT, Guerra G Jr, Maciel-Guerra AT. Turner's syndrome and thyroid disease: a transverse study of pediatric patients in Brazil. J Pediatr Endocrinol Metab. Apr 2000;13(4):357-62. [Medline].

  48. Neumann PJ, Gharib SD, Weinstein MC. The cost of a successful delivery with in vitro fertilization. N Engl J Med. Jul 28 1994;331(4):239-43. [Medline].

  49. [Best Evidence] Sas TC, de Muinck Keizer-Schrama SM, Stijnen T, van Teunenbroek A, Hokken-Koelega AC, Waelkens JJ, et al. Final height in girls with Turner's syndrome treated with once or twice daily growth hormone injections. Dutch Advisory Group on Growth Hormone. Arch Dis Child. Jan 1999;80(1):36-41. [Medline]. [Full Text].

  50. [Best Evidence] Soriano-Guillen L, Coste J, Ecosse E, et al. Adult height and pubertal growth in Turner syndrome after treatment with recombinant growth hormone. J Clin Endocrinol Metab. Sep 2005;90(9):5197-204. [Medline]. [Full Text].

  51. Starke M, Albertsson Wikland K, Möller A. Parents' descriptions of development and problems associated with infants with Turner syndrome: a retrospective study. J Paediatr Child Health. May-Jun 2003;39(4):293-8. [Medline].

  52. Zinn AR, Ross JL. Molecular analysis of genes on Xp controlling Turner syndrome and premature ovarian failure (POF). Semin Reprod Med. Jun 2001;19(2):141-6. [Medline].

 
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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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