Skip to main content

Laron Syndrome

Primary Growth Hormone Resistance

  • Chapter

Part of the book series: Contemporary Endocrinology ((COE,volume 14))

Abstract

In 1996, our group reported three siblings with clinical and biochemical features of growth hormone (GH) deficiency, but who had extremely high serum GH levels (1). Within two years, we were able to collect 22 patients (2) of Oriental Jewish descent.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Laron Z, Pertzelan A, Mannheimer S. Genetic pituitary dwarfism with high serum concentration of growth hormone. A new inborn error of metabolism ? Isr J Med Sci 1966; 2: 152–155.

    PubMed  CAS  Google Scholar 

  2. Laron Z, Pertzelan A, Karp M. Pituitary dwarfism with high serum levels of growth hormone. Isr J Med Sci 1968; 4: 883–894.

    PubMed  CAS  Google Scholar 

  3. Eshet R, Laron Z, Brown M, Arnon R. Immunoreactive properties of the plasma hGH from patients with the syndrome of familial dwarfism and high plasma IR-hGH. J Clin Endocrinol Metab 1973; 37: 819–821.

    Article  PubMed  CAS  Google Scholar 

  4. Eshet R, Laron Z, Brown M, Arnon R. Immunological behaviour of hGH from plasma of patients with familial dwarfism and high IR-hGH in a radioimmunoassay system using the cross-reaction between hGH and HCS. Horm Metab Res 1974; 6: 79–81.

    Article  Google Scholar 

  5. Jacobs LS, Sneid DS, Garland JT, Laron Z, Daughaday WH. Receptor-active growth hormone in Laron dwarfism. J Clin Endocrinol Metab 1976; 43: 403–407.

    Article  Google Scholar 

  6. Eshet R, Peleg S, Josefsberg Z, Fuchs C, Arnon R. and Laron Z. Some properties of the plasma hGH activity in patients with Laron-type dwarfism determined by a radioreceptor assay using human liver tissue. Horm Res 1985; 22: 276–283.

    Article  PubMed  CAS  Google Scholar 

  7. Eshet R, Laron Z, Pertzelan A, Dintzman M. Defect of human growth hormone in the liver of two patients with Laron type dwarfism. Isr J Med Sci 1984; 20: 8–11.

    PubMed  CAS  Google Scholar 

  8. Leung DW, Spencer SA, Cachianes G, Hammonds RG, Collins C, Henzel WJ, et al. Growth hormone receptor and serum binding protein: purification, cloning and expression. Nature 1987; 330: 537–543.

    Article  PubMed  CAS  Google Scholar 

  9. Godowski PJ, Leung DW, Meacham LR, Galgani JP, Hellmiss R, Keret R, et al. Characterization of the human growth hormone receptor gene and demonstration of a partial gene deletion in two patients with Laron type dwarfism. Proc Natl Acad Sci USA 1989; 86: 8083–8087.

    Article  PubMed  CAS  Google Scholar 

  10. Elders MJ, Garland JT, Daughaday WH, Fisher DA, Whitney JE, Hughes ER. Laron’ s dwarfism: Studies on the nature of the defect. J Pediatr 1973; 83: 253–263.

    Article  PubMed  CAS  Google Scholar 

  11. Laron Z, Blum W, Chatelain P, Ranke M, Rosenfeld R, Savage M, et al. Classification of growth hormone insensitivity syndrome. J Pediatr 1993; 122: 241.

    Article  PubMed  CAS  Google Scholar 

  12. Laron Z, Parks, JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Basel, New York, 1993, pp. 367.

    Google Scholar 

  13. Bass SH, Mulkerrin MG, Wells JA. A systematic mutational analysis of hormone-binding determinants in the human growth hormone receptor. Proc Natl Acad Sci USA 1991; 88: 4498–4502.

    Article  PubMed  CAS  Google Scholar 

  14. Spencer SA, Hammonds RG, Henzel WJ, Rodriquez H, Waters MJ, Wood WI. Rabbit liver growth hormone receptor and serum binding protein: purification, characterization and sequence. J Biol Chem 1988; 263: 7862–7867.

    PubMed  CAS  Google Scholar 

  15. Barton DE, Foellmer BE, Woods WI, Francke U. Chromosome mapping of the growth hormone receptor gene in man and mouse. Cytogenet Cell Genet 1989; 50: 137–141.

    Article  PubMed  CAS  Google Scholar 

  16. Kelly PA, Goujon L, Sotiropoulos A, Dinerstein H, Esposito N, Edery M, et al. The GH receptor and signal transduction. Horm Res 1994; 42: 133–139.

    Article  PubMed  CAS  Google Scholar 

  17. Parks JS, Faase E. GH and GH-receptor genes. In: Merimee TJ, Laron Z, eds. Growth Hormone, IGF-I and Growth. New Views of Old Concepts. Modern Endocrinology and Diabetes, vol. 4. Freund Publishing House, London, Tel Aviv, 1996, pp. 23–43.

    Google Scholar 

  18. Argetsinger LS, Campbell GS, Yang X, Witthuhn BA, Silvennoinen O, Ihle JN, et al. Identification of JAK2 as a growth hormone receptor-associated tyrosine kinase. Cell 1993; 74: 237–244.

    Article  PubMed  CAS  Google Scholar 

  19. Horseman NS. Editorial: Famine to feast-growth hormone and prolactin signal transducers. Endocrinology 1994; 135: 1289–1291.

    Article  PubMed  CAS  Google Scholar 

  20. Daughaday WH. Are there direct, non-IGF-I-mediated effects of hGH? In: Laron Z, Parks JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Karger, Basel, 1993, pp. 338–345.

    Google Scholar 

  21. de Vos AM, Ultsch M, Kossiakioff AA. Human growth hormone and extracellular domain of its receptor: crystal structure of the complex. Science 1992; 255: 306–312.

    Article  PubMed  Google Scholar 

  22. Amselem S, Duquesnoy P. Attree O, Novelli G, Bousnina S, Postel-Vinay MC, et al. Laron dwarfism and mutations of the growth hormone-receptor gene. N Engl J Med 1989; 321: 989–995.

    Article  PubMed  CAS  Google Scholar 

  23. Amselem S, Sobrier ML, Duquesnoy P, Rappaport R, Postel-Vinay MS, Gourmelen M, et al. Recurrent nonsense mutations in the growth hormone receptor from patients with Laron dwarfism. J Clin Invest 1991; 87: 1098–1102.

    Article  PubMed  CAS  Google Scholar 

  24. Amselem S, Duquesnoy P, Duriez B, Dastot F, Sobrier ML, Valleix S. Spectrum of growth hormone receptor mutations and associated haplotypes in Laron syndrome. Hum Mol Genet 1993; 2: 355–359.

    Article  PubMed  CAS  Google Scholar 

  25. Meacham WR, Brown MR, Murphy TL, Keret R, Silbergeld A, Laron Z, et al. Characterization of a noncontiguous gene deletion of the growth hormone receptor in Laron’s syndrome. J Clin Endocrinol Metab 1993; 77: 1379–1383.

    Article  PubMed  CAS  Google Scholar 

  26. Berg MA, Peoples R, Perez-Jurado L, Guevara-Aguirre J, Rosenbloom AL, Laron Z, et al. Receptor mutations and haplotypes in growth hormone receptor deficiency: a global survey and identification of the Ecuadorean E180splice mutation in an oriental Jewish patient. Acta Paediatr Suppl 1994; 399: 112–114.

    Article  PubMed  CAS  Google Scholar 

  27. Rosenbloom AL, Berg MA, Kasatkina EP. Volvoka TN, Skorobogatova VF, Sokolovskaya VN, et al. Severe growth hormone insensitivity (Laron syndrome) due to nonsense mutation of the GH receptor in brothers from Russia. J Pediatr Endocrinol Metab 1995; 8: 159–166.

    Article  PubMed  CAS  Google Scholar 

  28. Counts DR, Cutler GB. Growth hormone insensitivity syndrome due to point deletion and frame shift in the growth hormone receptor. J Clin Endocrinol Metab 1995; 80: 1978–1981.

    Article  PubMed  CAS  Google Scholar 

  29. Sobrier ML, Dastot F, Duquesnoy P, Kandemir N, Yordam N, Goossens M, et al. Nine novel growth hormone receptor gene mutations in patients with Laron syndrome. J Clin Endocrinol Metab 1997; 82: 435–437.

    Article  PubMed  CAS  Google Scholar 

  30. Baumbach L, Schiavi A, Bartlerr R, Perera E, Day J, Brown MR, et al. Clinical, biochemical and molecular investigations of a genetic isolate of growth hormone insensitivity (Laron’s syndrome). J Clin Endocrinol Metab 1997; 82: 444–451.

    Article  PubMed  CAS  Google Scholar 

  31. Daughaday WH, Trivedi B. Absence of serum growth hormone binding protein in patients with growth hormone receptor deficiency (Laron dwarfism). Proc Natl Acad Sci USA 1987; 84: 4636–4640.

    Article  PubMed  CAS  Google Scholar 

  32. Baumann G, Shaw MA, Winter RJ. Absence of plasma growth hormone-binding protein in Laron-type dwarfism. J Clin Endocrinol Metab 1987; 65: 814–816

    Article  PubMed  CAS  Google Scholar 

  33. Woods KA, Fraser NC, Postel-Vinay MC, Dusquenoy P, Savage MO, Clark AJL. A homozygous splice site mutation affecting the intracellular domain of the growth hormone (GH) receptor resulting in Laron syndrome with elevated GH-binding protein. J Clin Endocrinol Metab 1996; 81: 1686–1690.

    Article  PubMed  CAS  Google Scholar 

  34. Silbergeld A, Dastot F, Klinger B, Kanety H, Eshet R, Amselem S, et al. Intronic mutation in the growth hormone (GH) receptor gene from a girl with Laron Syndrome and extremely high serum GH binding protein: extended phenotypic study in a very large pedigree. J Pediatr Endocrinol Metab 1997; 10: 265–274.

    Article  PubMed  CAS  Google Scholar 

  35. Kou K, Lajara R, Rotwein P. Amino acid substitutions in the intracellular part of the growth hormone receptor in a patient with the Laron syndrome. J Clin Endocrinol Metab 1993; 76: 54–59.

    Article  PubMed  CAS  Google Scholar 

  36. Duquesnoy P, Sobrier ML, Duriez B, Dastot F, Buchanan CR, Savage MO, et al. A single amino acid substitution in the exoplasmic domain of the human growth hormone (GH) receptor confers familial GH resistance (Laron syndrome) with positive GH-binding activity by abolishing receptor homodimeriza-tion. Embo J 1994; 13: 1386–1395.

    PubMed  CAS  Google Scholar 

  37. Duquesnoy P, Sobrier ML, Amselem S, Goossens M. Defective membrane expression of human growth hormone (GH) receptor causes Laron-type GH insensitivity syndrome. Proc Natl Acad Sci USA 1991; 88:10, 272–10, 276.

    Google Scholar 

  38. Laron Z, Klinger B, Eshet R, Kaneti H, Karasik A, Silbergeld A. Laron syndrome due to a post-receptor defect: response to IGF-I treatment. Isr J Med Sci 1993; 29: 757–763.

    PubMed  CAS  Google Scholar 

  39. Herington AC, Ymer S, Stevenson J. Identification and characterization of specific binding proteins for growth hormone in normal human sera. J Clin Invest 1986; 77: 1817–1823.

    Article  PubMed  CAS  Google Scholar 

  40. Baumann G, Stolar MN, Amburn K, Barsano CP, DeVries BC. A specific GH-binding protein in human plasma: Initial characterization. J Clin Endocrinol Metab 1986; 62: 134–141.

    Article  PubMed  CAS  Google Scholar 

  41. Silbergeld A, Lazar L, Erster B, Keret R, Tepper R, Laron Z. Serum growth hormone binding protein activity in healthy neonates, children and young adults-correlation with age, height and weight. Clin Endocrinol 1989; 31: 295–303.

    Article  CAS  Google Scholar 

  42. Laron Z Klinger B, Erster B, Silbergeld A. Serum GH binding protein activity identifies the heterozy-gous carriers for Laron type dwarfism. Acta Endocrinol 1989; 121: 603–608.

    PubMed  CAS  Google Scholar 

  43. Laron Z. Laron type dwarfism (hereditary somatomedin deficiency): a review. In: Frick P, Von Harnack GA, Kochsiek GA, Prader A, eds. Advances in Internal Medicine and Pediatrics, Springer-Verlag, Berlin, 1984, pp. 117–150.

    Google Scholar 

  44. Rosenbloom AL, Guevara-Aguirre J, Rosenfeld RG, Fielder PJ. The little women of Loja: growth hormone receptor deficiency in an inbred population of Southern Ecuador. N Engl J Med 1990; 323: 1367–1374.

    Article  PubMed  CAS  Google Scholar 

  45. Maheshwari HG, Clayton PE, Mughal Z, Price DA, Norman M. Laron type dwarfism: the GH-BP positive phenotype. In: Laron Z, Parks JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Karger, Basel, 1993, pp. 160–166.

    Google Scholar 

  46. Desai M, Choksi C, Colaco P, Ambadkar M. Familial growth hormone deficiency (GHD) and growth hormone insensitivity (GHI) in Indian children. [Abstract #442]. Horm Res 1997; 48 (Suppl. 2): 90.

    Google Scholar 

  47. Rosenfeld RG, Rosenbloom AL, Guevara-Aguirre J. Growth hormone (GH) insensitivity due to primary GH receptor deficiency. Endocr Rev 1994; 15: 369–390.

    PubMed  CAS  Google Scholar 

  48. Krzisnik C, Battelino T. Five year treatment with IGF-I of a patient with Laron Syndrome in Slovenia (a follow-up report). J Pediatr Endocrinol Metab 1997; 10: 443–447.

    Article  PubMed  CAS  Google Scholar 

  49. Pertzelan A, Adam A, Laron Z. Genetic aspects of pituitary dwarfism due to absence of biological activity of growth hormone. Isr J Med Sci 1968; 4: 895–900.

    PubMed  CAS  Google Scholar 

  50. Laron Z, Pertzelan A, Karp M, Keret R, Eshet R, Silbergeld A. Laron syndrome-A unique model of IGF-I deficiency. In: Laron Z, Parks JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Karger, Basel, 1993, pp. 3–23.

    Google Scholar 

  51. Laron Z. Prismatic cases: Laron Syndrome (primary growth hormone resistance). From patient to laboratory to patient. J Clin Endocrinol Metab 1995; 80: 1526–1531.

    Article  PubMed  CAS  Google Scholar 

  52. Daughaday WH, Laron Z, Pertzelan A, Heins JN. Defective sulfation factor generation: a possible etiological link in dwarfism. Trans Assoc Am Phys 1969; 82: 129–138.

    PubMed  CAS  Google Scholar 

  53. Laron Z, Pertzelan A, Karp M, Kowadlo-Silbergeld A, Daughaday WH. Administration of growth hormone to patients with familial dwarfism with high plasma immunoreactive growth hormone. Measurement of sulfation factor, metabolic, and linear growth responses. J Clin Endocrinol Metab 1971; 33: 332–342.

    Article  PubMed  CAS  Google Scholar 

  54. Scharf A, Laron Z. Skull changes in pituitary dwarfism and the syndrome of familial dwarfism with high plasma immunoreactive growth hormone. A roentgenologic study. Horm Metab Res 1972; 4: 93–97.

    Article  PubMed  CAS  Google Scholar 

  55. Konfino R, Pertzelan A, Laron Z. Cephalometric measurements of familial dwarfism and high plasma immunoreactive growth hormone. Am J Orthod 1975; 68: 196–201.

    Article  PubMed  CAS  Google Scholar 

  56. Laron Z, Klinger B, Grunebaum M. Laron type dwarfism. Special feature-Picture of the month. Am J Dis Child 1991; 145: 473–474.

    PubMed  CAS  Google Scholar 

  57. Abramovici A, Josefsberg Z, Mimouni M, Liban E, Laron Z. Histopathological features of the skin in hypopituitarism and Laron-type dwarfism. Isr J Med Sci 1983; 19: 515–519.

    PubMed  CAS  Google Scholar 

  58. Laron Z, Klinger B. Body fat in Laron syndrome patients: Effect of insulin-like growth factor treatment. Horm Res 1993; 40: 16–22.

    Article  PubMed  CAS  Google Scholar 

  59. Laron Z, Lilos P, Klinger B. Growth curves for Laron syndrome. Arch Dis Child 1993; 68: 768–770.

    Article  PubMed  CAS  Google Scholar 

  60. Laron Z, Sarel R. Penis and testicular size in patients with growth hormone insufficiency. Acta Endocrinol 1970; 63: 625–633.

    PubMed  CAS  Google Scholar 

  61. Laron Z, Sarel R, Pertzelan A. Puberty in Laron type dwarfism. Eur J Pediatr 1980; 134: 79–83.

    Article  PubMed  CAS  Google Scholar 

  62. Pertzelan A, Lazar L, Klinger B, Laron Z. Puberty in 15 patients with Laron Syndrome: a longitudinal study. In: Laron Z, Parks JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Karger, Basel, 1993, pp. 27–33.

    Google Scholar 

  63. Laron Z, Klinger B. IGF-I treatment of adult patients with Laron syndrome: preliminary results. Clin Endocrinol 1994; 41: 631–638.

    Article  CAS  Google Scholar 

  64. Galatzer A, Aran O, Nagelberg N, Rubitzek J, Laron Z. Cognitive and psychosocial functioning of young adults with Laron syndrome. In: Laron Z, Parks JS, eds. Lessons from Laron Syndrome (LS) 1966–1992. Pediatric and Adolescent Endocrinology, vol. 24. Karger, Basel, 1993, pp. 53–60.

    Google Scholar 

  65. Brat O, Ziv I, Klinger B, Avraham M, Laron Z. Muscle force and endurance in untreated and human growth hormone or insulin-like growth factor-I-treated patients with growth hormone deficiency or Laron Syndrome. Horm Res 1997; 47: 45–48.

    Article  PubMed  CAS  Google Scholar 

  66. Keret R, Pertzelan A, Zeharia A, Zadik Z, Laron Z. Growth hormone (hGH) secretion and turnover in three patients with Laron type dwarfism. Isr J Med Sci 1988; 24: 75–79.

    PubMed  CAS  Google Scholar 

  67. Laron Z, Pertzelan A, Doron M, Assa S, Keret R. The effect of dihydrosomatostatin in dwarfism with high plasma immunoreactive growth hormone. Horm Metab Res 1977; 9: 338–339.

    Article  PubMed  CAS  Google Scholar 

  68. Laron Z, Klinger B, Blum WF, Silbergeld A, Ranke MB. IGF binding protein 3 in patients with LTD: effect of exogenous rIGF-I. Clin Endocrinol 1992; 36: 301–304.

    Article  CAS  Google Scholar 

  69. Laron Z, Suikkari AM, Klinger B, Silbergeld A, Pertzelan A, Seppala M, et al. Growth hormone and insulin-like growth factor regulate insulin-like growth factor binding protein in Laron type dwarfism, growth hormone deficiency and constitutional growth retardation. Acta Endocrinol 1992; 127: 351–358.

    PubMed  CAS  Google Scholar 

  70. Silbergeld A, Klinger B, Schwartz H, Laron Z. Serum prolactin in patients with Laron-type dwarfism: effect of insulin-like growth factor I. Horm Res 1992; 37: 160–164.

    Article  PubMed  CAS  Google Scholar 

  71. Laron Z. (1997) Hypoglycemia due to hormone deficiencies. J Pediatr Endocrinol Metab 1998; 11: 117–120.

    Google Scholar 

  72. Laron Z, Avitzur Y, Klinger B. Insulin resistance in Laron Syndrome (primary insulin-like growth factor-I [IGF-I] deficiency) and effect of IGF-I replacement therapy. J Pediatr Endocrinol Metab 1997; 10 (Suppl 1): 105–115.

    Google Scholar 

  73. Niwa M, Sato Y, Saito Y, Uchiyama F, Ono H, Yamashita M, et al. Chemical synthesis, cloning and expression of genes for human somatomedin C (insulin like growth factor I) and 59Val somatomedin C. Ann NY Acad Sci 1986; 469: 31–52.

    Article  PubMed  CAS  Google Scholar 

  74. Eshet R, Dux Z, Silbergeld A, Koren R, Klinger B, Laron Z. Erythrocytes from patients with low serum concentrations of IGF-I have an increase in receptor sites for IGF-I. Acta Endocrinol (Copenh.) 1991; 125: 354–358.

    CAS  Google Scholar 

  75. Eshet R, Klinger B, Silbergeld A, Laron Z. Modulation of insulin like growth factor I (IGF-I) binding sites on erythrocytes by IGHF-I treatment in patients with Laron syndrome (LS). Regul Pep 1993; 48: 233–239.

    Article  CAS  Google Scholar 

  76. Laron Z, Klinger B, Erster B, Anin S. Effects of acute administration of insulin like growth factor I in patients with Laron-type dwarfism. Lancet 1988;ii:1170–1172.

    Google Scholar 

  77. Laron Z, Klinger B, Silbergeld A, Lewin B, Erster B, Gil-Ad I. Intravenous administration of recombi-nant IGF-I lowers serum GHRH and TSH. Acta Endocrinol 1990; 123: 378–382.

    PubMed  CAS  Google Scholar 

  78. Klinger B, Garty M, Silbergeld A, Laron Z. Elimination characteristics of intravenously administered rIGF-I in Laron type dwarfs (LTD). Dev Pharmacol Ther 1990; 15: 196–199.

    PubMed  CAS  Google Scholar 

  79. Takano H, Hizuka N, Asakawa K, Sukegawa J, Shizume K. Effects of s.c. administration of recombinant human insulin like growth factor-I (IGF-I) on normal human subjects. Endocrinol Jpn 1990; 37: 309–317.

    Article  PubMed  CAS  Google Scholar 

  80. Gil-Ad I. Koch, Y. Silbergeld A, Dickerman Z, Kaplan B, Weizman A et al. Differential effect of insulin-like growth factor I (IGF-I) and growth hormone (GH) on hypothalamic regulation of GH secretion in the rat. J Endocrinol Invest 1996; 19: 542–547.

    PubMed  CAS  Google Scholar 

  81. Laron Z, Klinger B, Jensen LT, Erster B. Biochemical and hormonal changes induced by one week of administration of rIGF-I to patients with Laron type dwarfism. Clin Endocrinol 1991; 35: 145–150.

    Article  CAS  Google Scholar 

  82. Laron Z, Anin S, Klipper-Aubach Y, Klinger B. Effects of insulin-like growth factor on linear growth, head circumference and body fat in patients with Laron-type dwarfism. The Lancet 1992; 339: 1258–1261.

    Article  CAS  Google Scholar 

  83. Walker JL, Van Wyk JJ, Underwood LE. Stimulation of statural growth by recombinant insulin-like growth factor I in a child with growth hormone insensitivity syndrome (Laron type). J Pediatr 1992; 121: 641–646.

    Article  PubMed  CAS  Google Scholar 

  84. Klinger B, Jensen LT, Silbergeld A, Laron Z. Insulin-like growth factor-I raises serum procollagen levels in children and adults with Laron syndrome. Clin Endocrinol 1996; 45: 423–429.

    Article  Google Scholar 

  85. Klinger B, Laron Z. Renal function in Laron syndrome patients treated by insulin-like growth factor-I. Pediatr Nephrol 1994; 8: 684–688.

    Article  PubMed  CAS  Google Scholar 

  86. Kaneti H, Karasik A, Klinger B, Silbergeld A, Laron Z. Long-term treatment of Laron type dwarfs with insulin-like growth factor I increases serum insulin-like growth factor-binding protein 3 in the absence of growth hormone activity. Acta Endocrinol 1993; 128: 144–149.

    Google Scholar 

  87. Kaneti H, Silbergeld A, Klinger B, Karasik A, Baxter RC, Laron Z. Long-term effects of insulin-like growth factor (IGF)-I on serum IGF-I, IGF-binding protein-3 and acid labile subunit in Laron syndrome patients with normal growth hormone binding protein. Eur J Endocrinol 1997; 137: 626–630.

    Article  Google Scholar 

  88. Ranke MB, Savage MO, Chatelain PG, Preece MA, Rosenfeld RG, Blum WF, et al. Insulin-like growth factor I improves height in growth hormone insensitivity: Two years’ results. Horm Res 1995; 44: 253–264.

    Article  PubMed  CAS  Google Scholar 

  89. Backeljauw PF, Underwood LE, the GHIS Collaborative Group. Prolonged treatment with recombinant insulin-like growth factor-I in children with growth hormone insensitivity syndrome-a clinical Research Center study. J Clin Endocrinol Metab 1996; 81: 3312–3317.

    Article  PubMed  Google Scholar 

  90. Klinger B, Laron Z. Three year IGF-I treatment of children with Laron Syndrome. J Pediatr Endocrinol Metab 1995; 8: 149–158.

    Article  PubMed  CAS  Google Scholar 

  91. Guevara-Aguirre J, Rosenbloom AL, Vasconez O, Martinez V, Gargosky SE, Allen L, et al. Two year treatment of growth hormone (GH) receptor deficiency with recombinant insulin-like growth factor-I in 22 children: comparison of two dosage levels and to GH treated GH deficiency. J Clin Endocrinol Metab 1997; 82: 629–633.

    Article  PubMed  CAS  Google Scholar 

  92. Guevara-Aguirre J, Vasconez O, Martinez V, Martinez AL, Rosenbloom AL, Diamond FB Jr. et al. A randomized, double blind, placebo-controlled trial on safety and efficacy of recombinant human insulin-like growth factor-I in children with growth hormone receptor deficiency. J Clin Endocrinol Metab 1995; 80: 1393–1398.

    Article  PubMed  CAS  Google Scholar 

  93. Laron Z, Klinger B. Are patients with Laron syndrome candidates for limb lengthening? In: Laron Z, Mastragostino S, Romano C, Boero S, Cohen A. eds. Limb Lengthening: For Whom, When and How?, Freund Publishing House, London, 1995, pp. 79–91.

    Google Scholar 

  94. Amselem S, Sobrier M-L, Dastot F, Duquesnoy P, Duriez B, Goossens M. Molecular basis of inherited growth hormone resistance in childhood. In: Ross RJM, Savage MO, Guest eds. Growth Hormone Resistance. Balliere’s Clin Endocrinol Metab Int Pract Res 1996; 10: 353–369.

    Google Scholar 

  95. Berg MA, Argente J, Chernausek S, Gracia R, Guevara-Aguirre J, Hopp M, et al. Diverse growth hormone receptor gene mutations in Laron syndrome. Am J Hum Genet 1993; 52: 998–1005.

    PubMed  CAS  Google Scholar 

  96. Berg MA, Guevara-Aguirre J, Rosenbloom AL, Rosenfeld RG, Francke U. Mutation creating a new splice site in the growth hormone receptor genes of 37 Ecuadorean patients with Laron syndrome. Hum Mutat 1992; 1: 124–134.

    Article  Google Scholar 

  97. Woods KA, Camach-Hubner C, Savage MO, Clark AJL. Intrauterine growth retardation and postnatal deletion of the insulin-like growth factor I gene. N Engl J Med 1996; 335: 1363–1367.

    Article  PubMed  CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1999 Springer Science+Business Media New York

About this chapter

Cite this chapter

Laron, Z. (1999). Laron Syndrome. In: Jameson, J.L. (eds) Hormone Resistance Syndromes. Contemporary Endocrinology, vol 14. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-698-0_2

Download citation

  • DOI: https://doi.org/10.1007/978-1-59259-698-0_2

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-4757-5418-6

  • Online ISBN: 978-1-59259-698-0

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics