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MeSH Review

Turner Syndrome

 
 
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Disease relevance of Turner Syndrome

 

Psychiatry related information on Turner Syndrome

 

High impact information on Turner Syndrome

 

Chemical compound and disease context of Turner Syndrome

  • Growth-promoting effect of growth hormone and low dose ethinyl estradiol in girls with Turner's syndrome [12].
  • Abnormal glucose tolerance was found in 50% of Turner's syndrome patients before and 78% during treatment with sex hormones [13].
  • Treatment with recombinant human GH (rhGH), alone or in combination with the anabolic steroid oxandrolone (OX), has been recommended for girls with Turner's syndrome to improve final height [14].
  • In an attempt to clarify this variability, we assessed growth parameters, 24-h GH profiles, arginine-stimulated serum GH levels, and plasma insulin-like growth factor-I (IGF-I) concentrations in a group of 41 girls with Turner's syndrome with a mean (+/- SD) age of 13 +/- 3 yr (range, 6.7-18.9) [15].
  • Luteinizing hormone and follicle-stimulating hormone secretory dynamics in Turner's syndrome [16].
 

Biological context of Turner Syndrome

 

Anatomical context of Turner Syndrome

 

Gene context of Turner Syndrome

  • Plasma GH responses to GHRH, arginine, L-dopa, pyridostigmine, sequential administrations of GHRH and combined administration of PD and GHRH in Turner's syndrome [26].
  • Plasma PRL levels after TRH in patients with Turner's syndrome were significantly higher (P less than 0.05) than those in age-matched males, but were not significantly different from those in age-matched females [27].
  • BACKGROUND: SHOX haplo-insufficiency is considered the molecular basis of short stature in patients with Turner's syndrome, and gives rise to the short stature with mesomelic dysplasia and Madelung deformity of patients with Leri-Weill syndrome [28].
  • We have studied the course over age of fasting insulin, sex hormone-binding globulin (SHBG), and insulin-like growth factor (IGF)-binding protein-1 (IGFBP-1) in untreated children with Turner's syndrome (TS) and measured the course of these parameters during therapy with GH alone and in combination with oxandrolone [29].
  • Reports indicate that in plasma insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) are normal in patients with Turner's syndrome (TS) [30].
 

Analytical, diagnostic and therapeutic context of Turner Syndrome

References

  1. Y chromosome sequences in Turner's syndrome and risk of gonadoblastoma or virilisation. Page, D.C. Lancet (1994) [Pubmed]
  2. Magnetic resonance imaging evaluation of adipose tissue and muscle tissue mass in children with growth hormone (GH) deficiency, Turner's syndrome, and intrauterine growth retardation during the first year of treatment with GH. Leger, J., Carel, C., Legrand, I., Paulsen, A., Hassan, M., Czernichow, P. J. Clin. Endocrinol. Metab. (1994) [Pubmed]
  3. Naevocyte triggering by recombinant human growth hormone. Piérard, G.E., Piérard-Franchimont, C., Nikkels, A., Nikkels-Tassoudji, N., Arrese, J.E., Bourguignon, J.P. J. Pathol. (1996) [Pubmed]
  4. Inversion of intron 22 of the factor VIII gene in a girl with severe hemophilia A and Turner's syndrome. Chuansumrit, A., Sasanakul, W., Goodeve, A., Treratvirapong, T., Parinayok, R., Pintadit, P., Hathirat, P. Thromb. Haemost. (1999) [Pubmed]
  5. The effects of long-term growth hormone treatment on cardiac left ventricular dimensions and blood pressure in girls with Turner's syndrome. Dutch Working Group on Growth Hormone. Sas, T.C., Cromme-Dijkhuis, A.H., de Muinck Keizer-Schrama, S.M., Stijnen, T., van Teunenbroek, A., Drop, S.L. J. Pediatr. (1999) [Pubmed]
  6. Deficits in space-form perception in patients with sex chromosome mosaicism (45,X/46,XY). Ebbin, A.J., Howell, V.V., Wilson, M.G. Developmental medicine and child neurology. (1980) [Pubmed]
  7. Detection of Y-chromosome sequences in gonadal tissue of patients with Turner's syndrome (45,X). Kocova, M., Siegel, S.F., Nalesnick, M., Trucco, M. N. Engl. J. Med. (1994) [Pubmed]
  8. A preliminary study of the effect of estrogen dose on growth in Turner's syndrome. Ross, J.L., Cassorla, F.G., Skerda, M.C., Valk, I.M., Loriaux, D.L., Cutler, G.B. N. Engl. J. Med. (1983) [Pubmed]
  9. Growth hormone deficiency in Turner's syndrome. Brook, C.G. N. Engl. J. Med. (1978) [Pubmed]
  10. X-chromosome inactivation may explain the difference in viability of XO humans and mice. Ashworth, A., Rastan, S., Lovell-Badge, R., Kay, G. Nature (1991) [Pubmed]
  11. Effects of human growth hormone therapy on melanocytic naevi. Bourguignon, J.P., Piérard, G.E., Ernould, C., Heinrichs, C., Craen, M., Rochiccioli, P., Arrese, J.E., Franchimont, C. Lancet (1993) [Pubmed]
  12. Growth-promoting effect of growth hormone and low dose ethinyl estradiol in girls with Turner's syndrome. Vanderschueren-Lodeweyckx, M., Massa, G., Maes, M., Craen, M., van Vliet, G., Heinrichs, C., Malvaux, P. J. Clin. Endocrinol. Metab. (1990) [Pubmed]
  13. Glucose metabolism, lipid metabolism, and cardiovascular risk factors in adult Turner's syndrome. The impact of sex hormone replacement. Gravholt, C.H., Naeraa, R.W., Nyholm, B., Gerdes, L.U., Christiansen, E., Schmitz, O., Christiansen, J.S. Diabetes Care (1998) [Pubmed]
  14. Long-term treatment with growth hormone has no persisting effect on lipoprotein(a) in patients with Turner's syndrome. Querfeld, U., Döpper, S., Gradehand, A., Kiencke, P., Wahn, F., Zeisel, H.J. J. Clin. Endocrinol. Metab. (1999) [Pubmed]
  15. Slow baseline growth and a good response to growth hormone (GH) therapy are related to elevated spontaneous GH pulse frequency in girls with Turner's syndrome. Kamp, G.A., Kuilboer, M.M., Wynne, H.J., Rongen-Westerlaken, C., Johnson, M.L., Veldhuis, J.D., Wit, J.M. J. Clin. Endocrinol. Metab. (1993) [Pubmed]
  16. Luteinizing hormone and follicle-stimulating hormone secretory dynamics in Turner's syndrome. Boyar, R.M., Ramsey, J., Chipman, J., Fevre, M., Madden, J., Marks, J.F. J. Clin. Endocrinol. Metab. (1978) [Pubmed]
  17. Centromere of Y chromosome in Turner's syndrome. Kocova, M., Trucco, M. Lancet (1994) [Pubmed]
  18. Bone demineralization, biochemical indices of bone remodeling, and estrogen replacement therapy in adults with Turner's syndrome. Stĕpán, J.J., Musilová, J., Pacovský, V. J. Bone Miner. Res. (1989) [Pubmed]
  19. An investigation of ring and dicentric chromosomes found in three Turner's syndrome patients using DNA analysis and in situ hybridisation with X and Y chromosome specific probes. Cooper, C., Crolla, J.A., Laister, C., Johnston, D.I., Cooke, P. J. Med. Genet. (1991) [Pubmed]
  20. Dicentric X isochromosomes in man. Howell, R.T., Roberts, S.H., Beard, R.J. J. Med. Genet. (1976) [Pubmed]
  21. Amniotic fluid alpha-fetoprotein and Turner's syndrome. Seller, M.J. Lancet (1977) [Pubmed]
  22. The effect of hormone replacement therapy on cardiovascular hemodynamics in women with Turner's syndrome. Elsheikh, M., Bird, R., Casadei, B., Conway, G.S., Wass, J.A. J. Clin. Endocrinol. Metab. (2000) [Pubmed]
  23. Mineral metabolism in Turner's syndrome: evidence for impaired renal vitamin D metabolism and normal osteoblast function. Saggese, G., Federico, G., Bertelloni, S., Baroncelli, G.I. J. Clin. Endocrinol. Metab. (1992) [Pubmed]
  24. Detection of chromosomal abnormalities in the dysmorphic fetus using fluorescence in situ hybridization: evaluation for monosomy X genotype. Slagel, D.D., Bromley, C.M., Benda, J.A. Hum. Pathol. (1995) [Pubmed]
  25. Increased spontaneous, tumor necrosis factor receptor- and CD95 (Fas)-mediated apoptosis in cord blood T-cell subsets from Turner's syndrome. Gupta, S., Chiplunkar, S., Gupta, A., Gollapudi, S. Genes Immun. (2003) [Pubmed]
  26. Plasma GH responses to GHRH, arginine, L-dopa, pyridostigmine, sequential administrations of GHRH and combined administration of PD and GHRH in Turner's syndrome. Hanew, K., Tanaka, A., Utsumi, A. J. Endocrinol. Invest. (1998) [Pubmed]
  27. Prolactin response to thyrotropin-releasing hormone in children with Turner's syndrome and hyperthyroidism. Abe, K., Matsuura, N., Fukushima, N., Nohara, Y., Fujita, H., Kato, T., Endo, M. Eur. J. Pediatr. (1982) [Pubmed]
  28. SHOX intragenic microsatellite analysis in patients with short stature. Ezquieta, B., Cueva, E., Oliver, A., Gracia, R. Journal of pediatric endocrinology & metabolism : JPEM. (2002) [Pubmed]
  29. Insulin, insulin-like growth factor-binding protein-1, and sex hormone-binding globulin in patients with Turner's syndrome: course over age in untreated patients and effect of therapy with growth hormone alone and in combination with oxandrolone. Haeusler, G., Schmitt, K., Blümel, P., Plöchl, E., Waldhör, T., Frisch, H. J. Clin. Endocrinol. Metab. (1996) [Pubmed]
  30. Insulin-like growth factors (IGF-I and IGF-II) and IGF-binding protein-3 production by fibroblasts of patients with Turner's syndrome in culture. Barreca, A., Larizza, D., Damonte, G., Arvigo, M., Ponzani, P., Cesarone, A., Lo Curto, F., Severi, F., Giordano, G., Minuto, F. J. Clin. Endocrinol. Metab. (1997) [Pubmed]
  31. A longitudinal study of the effect of subcutaneous estrogen replacement on bone in young women with Turner's syndrome. Khastgir, G., Studd, J.W., Fox, S.W., Jones, J., Alaghband-Zadeh, J., Chow, J.W. J. Bone Miner. Res. (2003) [Pubmed]
  32. Growth hormone secretion in pubertal age patients with Turner's syndrome. Lanes, R., Brito, S., Suniaga, M., Moncada, G., Borges, M. J. Clin. Endocrinol. Metab. (1990) [Pubmed]
  33. Nested polymerase chain reaction study of 53 cases with Turner's syndrome: is cytogenetically undetected Y mosaicism common? Binder, G., Koch, A., Wajs, E., Ranke, M.B. J. Clin. Endocrinol. Metab. (1995) [Pubmed]
  34. Clinical trials of GH treatment in patients with Turner's syndrome in Japan--a consideration of final height. The Committee for the Treatment of Turner's Syndrome. Takano, K., Ogawa, M., Tanaka, T., Tachibana, K., Fujita, K., Hizuka, N. Eur. J. Endocrinol. (1997) [Pubmed]
 
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