American Journal of Cardiovascular Drugs

, Volume 2, Issue 6, pp 401–413 | Cite as

Turner Syndrome and the Heart

Cardiovascular Complications and Treatment Strategies
Review Article

Abstract

Turner syndrome is a condition usually associated with reduced final height, gonadal dysgenesis, and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequently with the syndrome. With respect to cardiac function, congenital malformations of the heart and the great vessels, hypertension and ischemic heart disease, and increased risk of aortic dissection are all conditions that the pediatrician or the physician caring for females with Turner syndrome should keep in mind. Many girls and adolescents with Turner syndrome receive growth hormone (GH) treatment, which has so far been an effective and well-tolerated therapy. Nevertheless, because of the experience from acromegaly, the physician should monitor blood pressure and perform echocardiography, together with clinical examinations by a cardiologist at regular intervals. During adulthood most women with Turner syndrome are faced with premature menopause and the need for female hormone replacement therapy (HRT). During clinical evaluation of girls and women with Turner syndrome, these conditions and complications should be kept under surveillance.

Here the cardiovascular complications of Turner syndrome are reviewed. The risk of congenital heart defects such as bicuspid aortic valves, aortic coarctation, other valve abnormalities, and septal defect is increased. Likewise, the risk of aortic dissection at a young age is increased, as is the risk of hypertension, ischemic heart disease, and stroke. GH therapy does not seem to adversely affect the heart, although longer-term follow-up studies are needed. In short-term studies, HRT lowers blood pressure, while any effect on the risk of ischemic heart disease has not been evaluated. Treatment with GH and HRT are discussed in relation to the heart and great vessels. Presently, the pathophysiology of the congenital cardiovascular malformation in Turner syndrome is unexplained, although different theories exist. Recommendations for clinical practice are given, including life-long surveillance of cardiac function, aortic diameter and blood pressure.

References

  1. 1.
    Turner HH. A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology 1938; 23: 566–74CrossRefGoogle Scholar
  2. 2.
    Lippe B. Turner syndrome. Endocrinol Metab Clin North Am 1991; 20: 121–52PubMedGoogle Scholar
  3. 3.
    Sylven L, Hagenfeldt K, Brondum NK, et al. Middle-aged women with Turner’s syndrome: medical status, hormonal treatment and social life. Acta Endocrinol 1991;125:359–65PubMedGoogle Scholar
  4. 4.
    Gotzsche CO, Krag-Olsen B, Nielsen J, et al. Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child 1994;71:433–6PubMedCrossRefGoogle Scholar
  5. 5.
    Gravholt CH, Juul S, Naeraa RW, et al. Morbidity in Turner syndrome. J Clin Epidemiol 1998; 51: 147–58PubMedCrossRefGoogle Scholar
  6. 6.
    Elsheikh M, Casadei B, Conway GS, et al. Hypertension is a major risk factor for aortic root dilatation in women with Turner’s syndrome. Clin Endocrinol (Oxf) 2001;54:69–73CrossRefGoogle Scholar
  7. 7.
    Jacobs PA, Melville M, Ratcliffe S, et al. A cytogenetic survey of 11 680 newborn infants. Ann Hum Genet 1974; 37: 359–76PubMedCrossRefGoogle Scholar
  8. 8.
    Hook EB, Warburton D. The distribution of chromosomal genotypes associated with Turner’s syndrome: livebirth prevalence rates and evidence for diminished fetal mortality and severity in genotypes associated with structural X abnormalities or mosaicism. Hum Genet 1983; 64: 24–7PubMedCrossRefGoogle Scholar
  9. 9.
    Nielsen J, Wohlert M. Chromosome abnormalities found among 34,910 newborn children: results from a 13-year incidence study in Arhus, Denmark. Hum Genet 1991; 87: 81–3PubMedCrossRefGoogle Scholar
  10. 10.
    Imaizumi K, Kuroki Y. Prevalence of Turner syndrome in Japan. Int Symposium on Turner Syndrome: 1992; Japan, 3-6Google Scholar
  11. 11.
    Savendahl L, Davenport ML. Delayed diagnoses of Turner’s syndrome: proposed guidelines for change. J Pediatr 2000; 137: 455–9PubMedCrossRefGoogle Scholar
  12. 12.
    Sas TC, Muinck Keizer-Schrama SM, Stijnen T, et al. Normalization of height in girls with Turner syndrome after long-term growth hormone treatment: results of a randomized dose-response trial. J Clin Endocrinol Metab 1999; 84: 4607–12PubMedCrossRefGoogle Scholar
  13. 13.
    Haddad HM, Wilkins L. Congenital anomalies associated with gonadal aplasia: review of 55 cases. Pediatrics 1959; 23: 885–902PubMedGoogle Scholar
  14. 14.
    Lemli L, Smith DW. The XO syndrome: a study of the differentiated phenotype in 25 patients. J Pediatr 1963; 63: 577–88PubMedCrossRefGoogle Scholar
  15. 15.
    Nora JJ, Torres FG, Sinha AK, et al. Characteristic cardiovascular anomalies of XO Turner syndrome, XX and XY phenotype and XO-XX Turner mosaic. Am J Cardiol 1970; 25: 639–41PubMedCrossRefGoogle Scholar
  16. 16.
    Rainier-Pope CR, Cummingham RD, Nadas AS, et al. Cardiovascular malformations in Turner’s syndrome. Pediatrics 1964; 19: 919–25Google Scholar
  17. 17.
    Mutinelli MR, Nizzoli G, Chiumello G, et al. Echocardiographic diagnosis of congenital bicuspid aortic valves in gonadal dysgenesis. G Ital Cardiol 1986; 16: 496–8PubMedGoogle Scholar
  18. 18.
    Dawson-Falk KL, Wright AM, Bakker B, et al. Cardiovascular evaluation in Turner syndrome: utility of MR imaging. Australas Radiol 1992; 36: 204–9PubMedCrossRefGoogle Scholar
  19. 19.
    Berdahl LD, Wenstrom KD, Hanson JW. Web neck anomaly and its association with congenital heart disease. Am J Med Genet 1995; 56: 304–7PubMedCrossRefGoogle Scholar
  20. 20.
    Lacro RV, Jones KL, Benirschke K. Coarctation of the aorta in Turner syndrome: a pathologic study of fetuses with nuchal cystic hygromas, hydrops fetalis, and female genitalia. Pediatrics 1988; 81: 445–51PubMedGoogle Scholar
  21. 21.
    Clark EB. Neck web and congenital heart defects: a pathogenic association in 45 X-O Turner syndrome? Teratology 1984; 29: 355–61PubMedCrossRefGoogle Scholar
  22. 22.
    Miller MJ, Geffner ME, Lippe BM, et al. Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome. J Pediatr 1983; 102: 47–50PubMedCrossRefGoogle Scholar
  23. 23.
    Sybert VP. Cardiovascular malformations and complications in Turner syndrome. Pediatrics 1998; 101(1): E11PubMedCrossRefGoogle Scholar
  24. 24.
    Mazzanti L, Cacciari E. Congenital heart disease in patients with Turner’s syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediatr 1998; 133: 688–92PubMedCrossRefGoogle Scholar
  25. 25.
    Mazzanti L, Prandstraller D, Tassinari D, et al. Heart disease in Turner’s syndrome. Helv Paediatr Acta 1988; 43: 25–31PubMedGoogle Scholar
  26. 26.
    Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with turner syndrome. Pediatrics 1998; 102(1): e12PubMedCrossRefGoogle Scholar
  27. 27.
    Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol 1984; 53: 849–55PubMedCrossRefGoogle Scholar
  28. 28.
    Natowicz M, Chatten J, Clancy R, et al. Genetic disorders and major extracardiac anomalies associated with the hypoplastic left heart syndrome. Pediatrics 1988; 82: 698–706PubMedGoogle Scholar
  29. 29.
    Greenlee R, Hoyme H, Witte M, et al. Developmental disorders of the lymphatic system. Lymphology 1993; 26: 156–68PubMedGoogle Scholar
  30. 30.
    Barr Jr M, Oman-Ganes L. Turner syndrome morphology and morphometrics: cardiac hypoplasia as a cause of midgestation death. Teratology 2002; 66: 65–72PubMedCrossRefGoogle Scholar
  31. 31.
    Carrel L, Cottle AA, Goglin KC, et al. A first-generation X-inactivation profile of the human X chromosome. Proc Natl Acad Sci U S A 1999; 96: 14440–4PubMedCrossRefGoogle Scholar
  32. 32.
    Rao E, Weiss B, Fukami M, et al. Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in idiopathic short stature and Turner syndrome. Nat Genet 1997; 16: 54–63PubMedCrossRefGoogle Scholar
  33. 33.
    Ellison JW, Wardak Z, Young MF, et al. PHOG, a candidate gene for involvement in the short stature of Turner syndrome. Hum Mol Genet 1997; 6: 1341–7PubMedCrossRefGoogle Scholar
  34. 34.
    Fang J, Dagenais SL, Erickson RP, et al. Mutations in FOXC2 (MFH-1), a forkhead family transcription factor, are responsible for the hereditary lymphedema-distichiasis syndrome. Am J Hum Genet 2000; 67: 1382–8PubMedCrossRefGoogle Scholar
  35. 35.
    Ogata T, Muroya K, Matsuo N, et al. Turner syndrome and Xp deletions: clinical and molecular studies in 47 patients. J Clin Endocrinol Metab 2001; 86: 5498–508PubMedCrossRefGoogle Scholar
  36. 36.
    Nathwani NC, Unwin R, Brook CG, et al. Blood pressure and Turner syndrome. Clin Endocrinol (Oxf) 2000; 52: 363–70CrossRefGoogle Scholar
  37. 37.
    Gravholt CH, Naeraa RW, Nyholm B, et al. Glucose metabolism, lipid metabolism, and cardiovascular risk factors in adult Turner syndrome: the impact of sex hormone replacement. Diabetes Care 1998; 21: 1062–70PubMedCrossRefGoogle Scholar
  38. 38.
    Verdecchia P, Schillaci G, Gatteschi C, et al. Blunted nocturnal fall in blood pressure in hypertensive women with future cardiovascular morbid events. Circulation 1993; 88: 986–92PubMedCrossRefGoogle Scholar
  39. 39.
    Verdecchia P, Schillaci G, Boldrini F, et al. Sex, cardiac hypertrophy and diurnal blood pressure variations in essential hypertension. J Hypertens 1992; 10: 683–92PubMedCrossRefGoogle Scholar
  40. 40.
    Verdecchia P, Schillaci G, Guerrieri M, et al. Orcadian blood pressure changes and left ventricular hypertrophy in essential hypertension. Circulation 1990; 81: 528–36PubMedCrossRefGoogle Scholar
  41. 41.
    Ross JL, Feuillan P, Long LM, et al. Lipid abnormalities in Turner syndrome. J Pediatr 1995; 126: 242–5PubMedCrossRefGoogle Scholar
  42. 42.
    Gravholt CH, Klausen IC, Weeke J, et al. Lp(a) and lipids in adult Turner’s syndrome: impact of treatment with 17beta-estradiol and norethisterone. Atherosclerosis 2000; 150: 201–8PubMedCrossRefGoogle Scholar
  43. 43.
    Dean JW, Fowler PB. Exaggerated responsiveness to thyrotrophin releasing hormone: a risk factor in women with coronary artery disease. BMJ 1985; 290: 1555–61PubMedCrossRefGoogle Scholar
  44. 44.
    Arem R, Patsch W. Lipoprotein and apolipoprotein levels in subclinical hypothyroidism: effect of levothyroxine therapy. Arch Intern Med 1990; 150: 2097–100PubMedCrossRefGoogle Scholar
  45. 45.
    Ivarsson SA, Ericsson UB, Nilsson KO, et al. Thyroid autoantibodies, Turner’s syndrome and growth hormone therapy. Acta Paediatr 1995; 84: 63–5PubMedCrossRefGoogle Scholar
  46. 46.
    Van Campenhout J, Antaki A, Rasio E. Diabetes mellitus and thyroid autoimmunity in gonadal dysgenesis. Fertil Steril 1973; 24: 1–9PubMedGoogle Scholar
  47. 47.
    Williams ED, Engel E, Forbes AP. Thyroiditis and al dysgenesis. N Engl J Med 1964; 270: 805–10PubMedCrossRefGoogle Scholar
  48. 48.
    Pai GS, Leach DC, Weiss L, et al. Thyroid abnormalities in 20 children with Turner syndrome. J Pediatr 1977; 91: 267–9PubMedCrossRefGoogle Scholar
  49. 49.
    Bright GM, Blizzard RM, Kaiser DL, et al. Organ-specific autoantibodies in children with common endocrine diseases. J Pediatr 1982; 100: 8–14PubMedCrossRefGoogle Scholar
  50. 50.
    Germain EL, Plotnick LP. Age-related anti-thyroid antibodies and thyroid abnormalities in Turner syndrome. Acta Paediatr Scand 1986; 75: 750–5PubMedCrossRefGoogle Scholar
  51. 51.
    Fleming S, Cowell C, Bailey J, et al. Hashimoto’s disease in Turner’s syndrome. Clin Invest Med 1988; 11: 243–6PubMedGoogle Scholar
  52. 52.
    Gruneiro PL, Iorcansky S, Coco R, et al. High incidence of thyroid disturbances in 49 children with Turner syndrome. J Pediatr 1987; 111: 258–61CrossRefGoogle Scholar
  53. 53.
    Radetti G, Mazzanti L, Paganini C, et al. Frequency, clinical and laboratory features of thyroiditis in girls with Turner’s syndrome. The Italian Study Group for Turner’s Syndrome. Acta Paediatr 1995; 84: 909–12PubMedCrossRefGoogle Scholar
  54. 54.
    Marner B, Bille G, Christy M, et al. Islet cell cytoplasmic antibodies (ICA) in diabetes and disorders of glucose tolerance. Diabet Med 1991; 8: 812–6PubMedCrossRefGoogle Scholar
  55. 55.
    Emans SJ, Grace E, Hoffer FA, et al. Estrogen deficiency in adolescents and young adults: impact on bone mineral content and effects of estrogen replacement therapy. Obstet Gynecol 1990; 76: 585–92PubMedGoogle Scholar
  56. 56.
    Larizza D, Martinetti Bianchi M, et al. Autoimmunity, HLA, Gm and Km polymorphisms in Turner’s syndrome. Autoimmunity 1989; 4: 69–78PubMedCrossRefGoogle Scholar
  57. 57.
    Chiovato L, Larizza D, Bendinelli G, et al. Autoimmune hypothyroidism and hyperthyroidism in patients with Turner’s syndrome. Eur J Endocrinol 1996; 134: 568–75PubMedCrossRefGoogle Scholar
  58. 58.
    Elsheikh M, Wass JA, Conway GS. Autoimmune thyroid syndrome in women with Turner’s syndrome-the association with karyotype. Clin Endocrinol (Oxf) 2001; 55: 223–6CrossRefGoogle Scholar
  59. 59.
    Jeresaty RM, Basu SK, Franco J. Dissecting aneurysm of the aorta in Turner’s syndrome. JAMA 1972; 222: 574–6PubMedCrossRefGoogle Scholar
  60. 60.
    Strader III WJ, Wachtel HL, Lundberg Jr GD. Hypertension and aortic rupture in gonadal dysgenesis. J Pediatr 1971; 79: 473–5PubMedCrossRefGoogle Scholar
  61. 61.
    Youker JE, Roe BB. Aneurysm of the aortic sinuses and ascending aorta in Turner’s syndrome. Am J Cardiol 1969; 23: 89–93PubMedCrossRefGoogle Scholar
  62. 62.
    Cecchi F, Samoun M, Santoro G, et al. Chronic dissecting aortic aneurysm and Turner’s syndrome: apropos of a case. Arch Mal Coeur Vaiss 1992; 85: 1043–6PubMedGoogle Scholar
  63. 63.
    Kusaba E, Imada T, Iwakuma A, et al. Aortic aneurysm complicated with coarctation of the aorta and Turner syndrome. Kyobu Geka 1995; 48: 1115–7PubMedGoogle Scholar
  64. 64.
    Ota Y, Tsunemoto M, Shimada M, et al. Aortic dissection associated with Turner’s syndrome. Kyobu Geka 1992; 45: 411–4PubMedGoogle Scholar
  65. 65.
    Oohara K, Yamazaki T, Sakaguchi K, et al. Acute aortic dissection, aortic insufficiency, and a single coronary artery in a patient with Turner’s syndrome. J Cardiovasc Surg (Torino) 1995; 36: 273–5Google Scholar
  66. 66.
    Imamura M, Aoki H, Eya K, et al. Balloon angioplasty before Wheat’s operation in a patient with Turner’s syndrome. Cardiovasc Surg 1995; 3: 70–2PubMedCrossRefGoogle Scholar
  67. 67.
    Lie JT. Aortic dissection in Turner’s syndrome. Am Heart J 1982; 103: 1077–80PubMedCrossRefGoogle Scholar
  68. 68.
    Price WH, Wilson J. Dissection of the aorta in Turner’s syndrome. J Med Genet 1983; 20: 61–3PubMedCrossRefGoogle Scholar
  69. 69.
    Slater DN, Grundman MJ, Mitchell L. Turner’s syndrome associated with bicuspid aortic stenosis and dissecting aortic aneurysm. Postgrad Med J 1982; 58: 436–8PubMedCrossRefGoogle Scholar
  70. 70.
    Kostich ND, Opitz JM. Ullrich-Turner syndrome associated with cystic medial necrosis of the aorta and great vessels. Am J Med 1965; 38: 943–9PubMedCrossRefGoogle Scholar
  71. 71.
    Edwards WD, Leaf DS, Edwards JE. Dissecting aortic aneurysm associated with congenital bicuspid aortic valve. Circulation 1978; 57: 1022–5PubMedCrossRefGoogle Scholar
  72. 72.
    Asch AJ. Turner’s syndrome occurring with Horner’s syndrome: seen with coarctation of the aorta and aortic aneurysm. Am J Dis Child 1979; 133: 827–30PubMedGoogle Scholar
  73. 73.
    Ravelo HR, Stephenson LW, Friedman S, et al. Coarctation resection in children with Turner’s syndrome: a note of caution. J Thorac Cardiovasc Surg 1980; 80: 427–30PubMedGoogle Scholar
  74. 74.
    Kido G, Miyagi A, Shibuya T, et al. Turner’s syndrome with pituitary hyperplasia: a case report. No Shinkei Geka 1994; 22: 333–8PubMedGoogle Scholar
  75. 75.
    Rubin K. Aortic dissection and rupture in Turner syndrome [letter]. J Pediatr 1993; 122: 670PubMedGoogle Scholar
  76. 76.
    Ohuchi H, Kawazoe K, Kosakai Y, et al. One-staged repair for coarctation of the aorta and annuloaortic ectasia with severe aortic regurgitation in a patient with Turner syndrome. Nippon Kyobu Geka Gakkai Zasshi 1992; 40: 2247–51PubMedGoogle Scholar
  77. 77.
    Pollak H, Veit F, Enenkel W. Presumed “successful” fibrinolysis in unrecognized acute aortic dissection. Dtsch Med Wochenschr 1992; 117: 368–71PubMedCrossRefGoogle Scholar
  78. 78.
    Martin MM, Beekman RH, Rocchini AP, et al. Aortic aneurysms after subclavian angioplasty repair of coarctation of the aorta. Am J Cardiol 1988; 61: 951–3PubMedCrossRefGoogle Scholar
  79. 79.
    Bartlema KA, Hogervorst M, Akkersdijk GJ, et al. Isolated abdominal aortic dissection in a patient with Turner’s syndrome [letter]. Surgery 1995; 117: 116–7PubMedCrossRefGoogle Scholar
  80. 80.
    Apostolopoulos T, Kyriakidis M, Toutouzas P. Endarteritis of the aortic arch in Turner’s syndrome with cystic degeneration of the aorta. Int J Cardiol 1992; 35: 417–9PubMedCrossRefGoogle Scholar
  81. 81.
    Allemann J, Muller G, Legat M. Rare variant of a Turner-Ullrich syndrome. Schweiz Med Wochenschr 1982; 112: 1249–55PubMedGoogle Scholar
  82. 82.
    Buheitel G, Singer H, Hofbeck M. Aortic aneurysms in Ullrich-Turner syndrome. Klin Padiatr 1996; 208: 42–5PubMedCrossRefGoogle Scholar
  83. 83.
    Akimoto N, Shimizu T, Ishikawa M, et al. The surgical treatment of aortic dissection in a patient with Turner’s syndrome: report of a case. Surg Today 1994; 24: 929–32PubMedCrossRefGoogle Scholar
  84. 84.
    Meunier JP, Jazayeri S, David M. Acute type A aortic dissection in an adult patient with Turner’s syndrome. Heart 2001; 86(5): 546PubMedCrossRefGoogle Scholar
  85. 85.
    Anabtawi IN, Ellison RG, Yeh TJ, et al. Dissecting aneurysm of aorta associated with Turner’s syndrome. J Thorac Cardiovasc Surg 1964; 47: 750–4PubMedGoogle Scholar
  86. 86.
    Roberts WC. Aortic dissection: anatomy, consequences, and causes. Am Heart J 1981; 101: 195–214PubMedCrossRefGoogle Scholar
  87. 87.
    Spittell PC, Spittell Jr JA, Joyce JW, et al. Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). Mayo Clin Proc 1993; 68: 642–51PubMedGoogle Scholar
  88. 88.
    Prandstraller D, Mazzanti L, Picchio FM, et al. Turner’s syndrome: cardiologic profile according to the different chromosomal patterns and long-term clinical follow-up of 136 nonpreselected patients. Pediatr Cardiol 1999; 20: 108–12PubMedCrossRefGoogle Scholar
  89. 89.
    Nielsen J, Sillesen I, Hansen KB. Fertility in women with Turner’s syndrome: case report and review of literature. Br J Obstet Gynaecol 1979; 86: 833–5PubMedCrossRefGoogle Scholar
  90. 90.
    Hovatta O. Pregnancies in women with Turner’s syndrome. Ann Med 1999; 31: 106–10PubMedGoogle Scholar
  91. 91.
    Foudila T, Soderstrom AV, Hovatta O. Turner’s syndrome and pregnancies after oocyte donation. Hum Reprod 1999; 14: 532–5PubMedCrossRefGoogle Scholar
  92. 92.
    Swapp GH, Johnston AW, Watt JL, et al. A fertile woman with non-mosaic Turner’s syndrome: case report and review of the literature. Br J Obstet Gynaecol 1989; 96: 876–80PubMedCrossRefGoogle Scholar
  93. 93.
    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: 81–7PubMedCrossRefGoogle Scholar
  94. 94.
    Birdsall M, Kennedy S. The risk of aortic dissection in women with Turner syndrome [letter]. Hum Reprod 1996; 11: 1587PubMedCrossRefGoogle Scholar
  95. 95.
    Nagel TC, Tesch LG. ART and high risk patients [letter]. Fertil Steril 1997; 68: 748–9PubMedGoogle Scholar
  96. 96.
    Beauchesne LM, Connolly HM, Ammash NM, et al. Coarctation of the aorta: outcome of pregnancy. J Am Coll Cardiol 2001; 38: 1728–33PubMedCrossRefGoogle Scholar
  97. 97.
    Weytjens C, Bove T, Van Der NP. Aortic dissection and Turner’s syndrome. J Cardiovasc Surg (Torino) 2000; 41: 295–7Google Scholar
  98. 98.
    Ranke MB, Pfluger H, Rosendahl W, et al. Turner syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediatr 1983; 141:81–8PubMedCrossRefGoogle Scholar
  99. 99.
    Karlberg J, Albertsson WK, Nilsson KO, et al. Growth in infancy and childhood in girls with Turner’s syndrome. Acta Paediatr Scand 1991; 80: 1158–65PubMedCrossRefGoogle Scholar
  100. 100.
    Davenport ML, Punyasavatsut N, Gunther D, et al. Turner syndrome: a pattern of early growth failure. Acta Paediatr Suppl 1999; 88: 118–21PubMedCrossRefGoogle Scholar
  101. 101.
    Lyon AJ, Preece MA, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child 1985; 60: 932–5PubMedCrossRefGoogle Scholar
  102. 102.
    Pasquino AM, Passeri F, Pucarelli I, et al. Spontaneous pubertal development in Turner’s syndrome. Italian Study Group for Turner’s Syndrome. J Clin Endocrinol Metab 1997; 82: 1810–3PubMedCrossRefGoogle Scholar
  103. 103.
    Massarano AA, Brook CG, Hindmarsh PC, et al. Growth hormone secretion in Turner’s syndrome and influence of oxandrolone and ethinyl oestradiol. Arch Dis Child 1989; 64: 587–92PubMedCrossRefGoogle Scholar
  104. 104.
    Reiter JC, Craen M, Van Vliet G. Decreased growth hormone response to growth hormone-releasing hormone in Turner’s syndrome: relation to body weight and adiposity. Acta Endocrinol 1991; 125: 38–42PubMedGoogle Scholar
  105. 105.
    Ranke MB, Blum WF, Haug F, et al. Growth hormone, somatomedin levels and growth regulation in Turner’s syndrome. Acta Endocrinol 1987; 116: 305–13PubMedGoogle Scholar
  106. 106.
    Saenger P, Schwartz E, Wiedemann E, et al. The interaction of growth hormone, somatomedin and oestrogen in patients with Turner’s syndrome. Acta Endocrinol 1976; 81: 9–18PubMedGoogle Scholar
  107. 107.
    Wit JM, Massarano AA, Kamp GA, et al. Growth hormone secretion in patients with Turner’s syndrome as determined by time series analysis. Acta Endocrinol 1992; 127: 7–12PubMedGoogle Scholar
  108. 108.
    Hochberg Z, Aviram M, Rubin D, et al. Decreased sensitivity to insulin-like growth factor I in Turner’s syndrome: a study of monocytes and T lymphocytes. Eur J Clin Invest 1997; 27: 543–7PubMedCrossRefGoogle Scholar
  109. 109.
    Swearingen B, Barker FG, Katznelson L, et al. Long-term mortality after transsphenoidal surgery and adjunctive therapy for acromegaly. J Clin Endocrinol Metab 1998; 83: 3419–26PubMedCrossRefGoogle Scholar
  110. 110.
    Colao A, Cuocolo A, Marzullo P, et al. Is the acromegalic cardiomyopathy reversible? Effect of 5-year normalization of growth hormone and insulin-like growth factor I levels on cardiac performance. J Clin Endocrinol Metab 2001; 86: 1551–7PubMedCrossRefGoogle Scholar
  111. 111.
    Caprio S, Boulware SD, Press M, et al. Effect of growth hormone treatment on hyperinsulinemia associated with Turner syndrome. J Pediatr 1992; 120: 238–43PubMedCrossRefGoogle Scholar
  112. 112.
    Saenger P, Attie KM, DiMartino Nardi J, et al. Carbohydrate metabolism in children receiving growth hormone for 5 years: chronic renal insufficiency compared with growth hormone deficiency, Turner syndrome, and idiopathic short stature. Genentech Collaborative Group. Pediatr Nephrol 1996; 10: 261–3PubMedGoogle Scholar
  113. 113.
    Gravholt CH, Naeraa RW. Reference values for body proportions and body composition in adult women with Turner’s syndrome. Am J Med Genet 1997; 72: 403–8PubMedCrossRefGoogle Scholar
  114. 114.
    Bordeleau L, Cwinn A, Turek M, et al. Aortic dissection and Turner’s syndrome: case report and review of the literature. J Emerg Med 1998; 16: 593–6PubMedCrossRefGoogle Scholar
  115. 115.
    Sas TC, Cromme-Dijkhuis AH, de Muinck K, et al. The effects of long-term growth hormone treatment on cardiac left ventricular dimensions and blood pressure in girls with Turner’s syndrome. J Pediatr 1999; 135: 470–6PubMedCrossRefGoogle Scholar
  116. 116.
    Nathwani NC, Unwin R, Brook CG, et al. The influence of renal and cardiovascular abnormalities on blood pressure in Turner syndrome. Clin Endocrinol (Oxf) 2000; 52: 371–7CrossRefGoogle Scholar
  117. 117.
    Sack MN, Rader DJ, Cannon RO. Oestrogen and inhibition of oxidation of lowdensity lipoproteins in postmenopausal women. Lancet 1994; 343: 269–70PubMedCrossRefGoogle Scholar
  118. 118.
    Gilligan DM, Quyyumi AA, Cannon RO. Effects of physiological levels of estrogen on coronary vasomotor function in postmenopausal women. Circulation 1994; 89: 2545–51PubMedCrossRefGoogle Scholar
  119. 119.
    Losordo DW, Kearney M, Kim EA, et al. Variable expression of the estrogen receptor in normal and atherosclerotic coronary arteries of premenopausal women. Circulation 1994; 89: 1501–10PubMedCrossRefGoogle Scholar
  120. 120.
    Selzman CH, Gaynor JS, Turner AS, et al. Ovarian ablation alone promotes aortic intimai hyperplasia and accumulation of fibroblast growth factor. Circulation 1998; 98: 2049–54PubMedCrossRefGoogle Scholar
  121. 121.
    Elsheikh M, Bird R, Casadei B, et al. The effect of hormone replacement therapy on cardiovascular hemodynamics in women with Turner’s syndrome. J Clin Endocrinol Metab 2000; 85: 614–8PubMedCrossRefGoogle Scholar
  122. 122.
    Seely EW, Walsh BW, Gerhard MD, et al. Estradiol with or without progesterone and ambulatory blood pressure in postmenopausal women. Hypertension 1999; 33: 1190–4PubMedCrossRefGoogle Scholar
  123. 123.
    Gravholt CH, Naerra RW, Fisher S, et al. Body composition and physical fitness are major determinants of the growth hormone_IGF axis aberrations in adult Turner syndrome, with important modulations by treatment with 17-Beta-Estradiol. J Clin Endocrinol Metab 1997; 82: 2570–7PubMedCrossRefGoogle Scholar
  124. 124.
    Risks and benefits of estrogen plus progestin in healthy postmenopausal women: principal results from the Women’s Health Initiative randomized controlled trial. JAMA 2002; 288: 321-33Google Scholar
  125. 125.
    Hulley S, Grady D, Bush T, et al. Randomized trial of estrogen plus progestin for secondary prevention of coronary heart disease in postmenopausal women. Heart and Estrogen/progestin Replacement Study (HERS) Research Group. JAMA 1998; 280: 605–13PubMedCrossRefGoogle Scholar
  126. 126.
    Viscoli CM, Brass LM, Kernan WN, et al. A clinical trial of estrogen-replacement therapy after ischemic stroke. N Engl J Med 2001; 345: 1243–9PubMedCrossRefGoogle Scholar
  127. 127.
    Reaven GM. Banting lecture 1988. Role of insulin resistance in human disease. Diabetes 1988; 37: 1595–607PubMedCrossRefGoogle Scholar
  128. 128.
    Naeraa RW, Gravholt CH, Hansen J, et al. Mortality in Turner syndrome. In: Albertsson-Wikland K, Ranke MB, editors. Turner syndrome in a life span perspective: research and clinical aspects. Amsterdam: Elsevier, 1995: 323Google Scholar
  129. 129.
    Price WH, Clayton JF, Collyer S, et al. Mortality ratios, life expectancy, and causes of death in patients with Turner’s syndrome. J Epidemiol Community Health 1986; 40: 97–102PubMedCrossRefGoogle Scholar
  130. 130.
    Swerdlow AJ, Hermon C, Jacobs PA, et al. Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study. Ann Hum Genet 2001; 65: 177–88PubMedCrossRefGoogle Scholar
  131. 131.
    Saenger P, Wikland KA, Conway GS, et al. Recommendations for the diagnosis and management of Turner syndrome. J Clin Endocrinol Metab 2001; 86: 3061–9PubMedCrossRefGoogle Scholar

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© Adis International Limited 2002

Authors and Affiliations

  1. 1.Medical Department M (Endocrinology and Diabetes) and Medical Research Laboratories, Aarhus KommunehospitalAarhus University HospitalAarhus CDenmark

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