Skip to main content
Top
Published in: BMC Pediatrics 1/2009

Open Access 01-12-2009 | Research article

Diagnosis of growth hormone (GH) deficiency: comparison of pituitary stalk interruption syndrome and transient GH deficiency

Authors: Murielle Louvel, Mariana Marcu, Christine Trivin, Jean-Claude Souberbielle, Raja Brauner

Published in: BMC Pediatrics | Issue 1/2009

Login to get access

Abstract

Background

Most patients with childhood non-organic growth hormone (GH) deficiency (GHD) produce a normal GH peak as young adults. Our objectives were to better define this transient GHD and evaluate the factors influencing the growth response of patients with pituitary stalk interruption syndrome (PSIS).

Methods

We studied 72 prepubertal patients with a GH peak < 6.7 ng/ml after 2 stimulation tests, treated with 0.2 mg GH/kg/w for at least 3 years. Group 1 (n = 53, 4.7 ± 4.0 years) had PSIS and Group 2 (n = 19, 9.2 ± 3.0 years) had transient GHD and normal pituitary.

Results

At diagnosis, 64% of Group 1 and one Group 2 were < 5 years old. The growth rate of 59% Group 1 and two Group 2 patients was ≤ -2 SDS. The GH peak of 64% Group 1 patients and no Group 2 patients was < 3 ng/ml. The plasma insulin-like growth factor-1 of all Group 1 and all but one Group 2 patients was ≤ -2 z scores.
During the first year of GH treatment, the growth rate was ≥ 2 SDS in 81% Group 1 and 37% Group 2 patients. In Group 1, it was negatively correlated with the GH peak before treatment (P < 0.03), and with the difference between the target and adult heights (P < 0.01).
The height gain SDSs between diagnosis and adult height were 1.7 ± 1.2 in Group 1 (n = 30) and 1.08 ± 0.8 in Group 2 (n = 12, P = 0.05).

Conclusion

The factors of the growth response to GH treatment should be analysed separately for each population: with and without PSIS or other markers.
Appendix
Available only for authorised users
Literature
1.
go back to reference Argyropoulou M, Pérignon F, Brauner R, Brunelle F: Magnetic resonance imaging in the diagnosis of growth hormone deficiency. J Pediatr. 1992, 120: 886-891. 10.1016/S0022-3476(05)81955-9.CrossRefPubMed Argyropoulou M, Pérignon F, Brauner R, Brunelle F: Magnetic resonance imaging in the diagnosis of growth hormone deficiency. J Pediatr. 1992, 120: 886-891. 10.1016/S0022-3476(05)81955-9.CrossRefPubMed
2.
go back to reference Badaru A, Wilson DM: Alternatives to growth hormone stimulation testing in children. Trends in Endocrinol Metab. 2004, 15: 252-258. 10.1016/j.tem.2004.06.004.CrossRef Badaru A, Wilson DM: Alternatives to growth hormone stimulation testing in children. Trends in Endocrinol Metab. 2004, 15: 252-258. 10.1016/j.tem.2004.06.004.CrossRef
3.
go back to reference Castro C, Trivin C, Souberbielle JC, Zerah M, Brauner R: Growth hormone deficiency: permanence and diagnosis in young adults. Horm Res. 2002, 58: 165-171. 10.1159/000065489.CrossRefPubMed Castro C, Trivin C, Souberbielle JC, Zerah M, Brauner R: Growth hormone deficiency: permanence and diagnosis in young adults. Horm Res. 2002, 58: 165-171. 10.1159/000065489.CrossRefPubMed
4.
go back to reference Trivin C, Souberbielle JC, Aubertin G, Lawson-Body E, Adan L, Brauner R: Diagnosis of idiopathic growth hormone deficiency: contributions of data on the acid-labile subunit, insulin-like growth factors (IGF) I & II and IGF binding protein – 3. J Pediatr Endocrinol Metab. 2006, 19: 481-489.PubMed Trivin C, Souberbielle JC, Aubertin G, Lawson-Body E, Adan L, Brauner R: Diagnosis of idiopathic growth hormone deficiency: contributions of data on the acid-labile subunit, insulin-like growth factors (IGF) I & II and IGF binding protein – 3. J Pediatr Endocrinol Metab. 2006, 19: 481-489.PubMed
5.
go back to reference Marin G, Domené HM, Barnes KM, Blackwell BJ, Cassorla FG, Cutler GB: The effects of estrogen priming and puberty on the growth hormone response to standardized treadmill exercise and arginine-insulin in normal girls and boys. J Clin Endocrinol Metab. 1994, 79: 537-541. 10.1210/jc.79.2.537.PubMed Marin G, Domené HM, Barnes KM, Blackwell BJ, Cassorla FG, Cutler GB: The effects of estrogen priming and puberty on the growth hormone response to standardized treadmill exercise and arginine-insulin in normal girls and boys. J Clin Endocrinol Metab. 1994, 79: 537-541. 10.1210/jc.79.2.537.PubMed
6.
go back to reference Sempé M, Pédron G, Roy-Pernot MP: Auxologie, méthode et séquence. Théraplix, Paris. 1979 Sempé M, Pédron G, Roy-Pernot MP: Auxologie, méthode et séquence. Théraplix, Paris. 1979
7.
go back to reference Rolland-Cachera MF, Cole TJ, Sempé M, Trichet J, Rossignol C, Charrand A: Body mass index variations: centiles from birth to 87 years. Eur J Clin Nutr. 1991, 45: 13-21.PubMed Rolland-Cachera MF, Cole TJ, Sempé M, Trichet J, Rossignol C, Charrand A: Body mass index variations: centiles from birth to 87 years. Eur J Clin Nutr. 1991, 45: 13-21.PubMed
8.
go back to reference Tanner JM, Goldstein H, Whitehouse RH: Standards for children's height at ages 2–9 years allowing for height of parents. Arch Dis Child. 1970, 47: 755-762. 10.1136/adc.45.244.755.CrossRef Tanner JM, Goldstein H, Whitehouse RH: Standards for children's height at ages 2–9 years allowing for height of parents. Arch Dis Child. 1970, 47: 755-762. 10.1136/adc.45.244.755.CrossRef
9.
go back to reference Greulich W, Pyle S: Radiographic atlas of skeletal development of the hand and wrist. 1959, University Press, Stanford, 2 Greulich W, Pyle S: Radiographic atlas of skeletal development of the hand and wrist. 1959, University Press, Stanford, 2
10.
go back to reference Trainer PJ, Barth J, Sturgeon C, Wieringaon G: Consensus statement o the standardization of GH assays. Eur J Endocrinol. 2006, 155: 1-2. 10.1530/eje.1.02186.CrossRefPubMed Trainer PJ, Barth J, Sturgeon C, Wieringaon G: Consensus statement o the standardization of GH assays. Eur J Endocrinol. 2006, 155: 1-2. 10.1530/eje.1.02186.CrossRefPubMed
11.
go back to reference Bussières L, Souberbielle JC, Pinto G, Adan L, Noël M, Brauner R: The use of insulin like growth factor 1 reference values for the diagnosis of growth hormone deficiency in prepubertal children. Clin Endocrinol. 2000, 52: 735-739. 10.1046/j.1365-2265.2000.00999.x.CrossRef Bussières L, Souberbielle JC, Pinto G, Adan L, Noël M, Brauner R: The use of insulin like growth factor 1 reference values for the diagnosis of growth hormone deficiency in prepubertal children. Clin Endocrinol. 2000, 52: 735-739. 10.1046/j.1365-2265.2000.00999.x.CrossRef
12.
go back to reference Lemaire P, Brauner N, Hammer P, Trivin C, Souberbielle JC, Brauner R: Improved screening for growth hormone deficiency using logical analysis data. Med Sci Monit. 2009, 15 (1): MT5-10.PubMed Lemaire P, Brauner N, Hammer P, Trivin C, Souberbielle JC, Brauner R: Improved screening for growth hormone deficiency using logical analysis data. Med Sci Monit. 2009, 15 (1): MT5-10.PubMed
13.
go back to reference Loche S, Bizzarri C, Maghnie M, Faedda A, Tzialla C, Autelli M, Casini MR, Cappa M: Results of early reevaluation of growth hormone secretion in short children with apparent growth hormone deficiency. J Pediatr. 2002, 140: 445-449. 10.1067/mpd.2002.122729.CrossRefPubMed Loche S, Bizzarri C, Maghnie M, Faedda A, Tzialla C, Autelli M, Casini MR, Cappa M: Results of early reevaluation of growth hormone secretion in short children with apparent growth hormone deficiency. J Pediatr. 2002, 140: 445-449. 10.1067/mpd.2002.122729.CrossRefPubMed
14.
go back to reference Coutant R, Rouleau S, Despert F, Magontier N, Loisel D, Limal JM: Growth and adult height in GH-treated children with nonacquired GH deficiency and idiopathic short stature: the influence of pituitary magnetic resonance imaging findings. J Clin Endocrinol Metab. 2001, 86: 4649-4654. 10.1210/jc.86.10.4649.CrossRefPubMed Coutant R, Rouleau S, Despert F, Magontier N, Loisel D, Limal JM: Growth and adult height in GH-treated children with nonacquired GH deficiency and idiopathic short stature: the influence of pituitary magnetic resonance imaging findings. J Clin Endocrinol Metab. 2001, 86: 4649-4654. 10.1210/jc.86.10.4649.CrossRefPubMed
15.
go back to reference Zenaty D, Garel C, Limoni C, Czernichow P, Léger J: Presence of magnetic resonance imaging abnormalities of the hypothalamic-pituitary axis is a significant determinant of the first 3 years growth response to human growth hormone treatment in prepubertal children with nonacquired growth hormone deficiency. Clin Endocr. 2003, 58: 647-652.PubMed Zenaty D, Garel C, Limoni C, Czernichow P, Léger J: Presence of magnetic resonance imaging abnormalities of the hypothalamic-pituitary axis is a significant determinant of the first 3 years growth response to human growth hormone treatment in prepubertal children with nonacquired growth hormone deficiency. Clin Endocr. 2003, 58: 647-652.PubMed
16.
go back to reference Maghnie M, Ambrosini L, Cappa M, Pozzobon G, Ghizzoni L, Ubertini MG, di Iorgi N, Tinelli C, Pilia S, Chiumello G, Lorini R, Loche S: Adult height in patients with permanent growth hormone deficiency with and without multiple pituitary hormone deficiencies. J Clin Endocrinol Metab. 2006, 91: 2900-2905. 10.1210/jc.2006-0050.CrossRefPubMed Maghnie M, Ambrosini L, Cappa M, Pozzobon G, Ghizzoni L, Ubertini MG, di Iorgi N, Tinelli C, Pilia S, Chiumello G, Lorini R, Loche S: Adult height in patients with permanent growth hormone deficiency with and without multiple pituitary hormone deficiencies. J Clin Endocrinol Metab. 2006, 91: 2900-2905. 10.1210/jc.2006-0050.CrossRefPubMed
17.
go back to reference Blethen SL, Baptista J, Kuntze J, Foley T, LaFranchi S, Johanson A, on behalf of the Genentech Growth Study Group: Adult height in growth hormone (GH)-deficient children treated with biosynthetic GH. J Clin Endocrinol Metab. 1997, 82: 418-420. 10.1210/jc.82.2.418.CrossRefPubMed Blethen SL, Baptista J, Kuntze J, Foley T, LaFranchi S, Johanson A, on behalf of the Genentech Growth Study Group: Adult height in growth hormone (GH)-deficient children treated with biosynthetic GH. J Clin Endocrinol Metab. 1997, 82: 418-420. 10.1210/jc.82.2.418.CrossRefPubMed
18.
go back to reference Reiter EO, Price DA, Wilton P, Albertsson-Wikland K, Ranke MB: Effect of growth hormone (GH) treatment on the near-final height of 1258 patients with idiopathic GH deficiency: analysis of a large international database. J Clin Endocrinol Metab. 2006, 91: 2047-2054. 10.1210/jc.2005-2284.CrossRefPubMed Reiter EO, Price DA, Wilton P, Albertsson-Wikland K, Ranke MB: Effect of growth hormone (GH) treatment on the near-final height of 1258 patients with idiopathic GH deficiency: analysis of a large international database. J Clin Endocrinol Metab. 2006, 91: 2047-2054. 10.1210/jc.2005-2284.CrossRefPubMed
19.
go back to reference De Ridder MAJ, Stijnen T, Hokken-Koelega ACS: Prediction of adult height in growth-hormone-treated children with growth hormone deficiency. J Clin Endocrinol Metab. 2007, 92: 925-931. 10.1210/jc.2006-1259.CrossRefPubMed De Ridder MAJ, Stijnen T, Hokken-Koelega ACS: Prediction of adult height in growth-hormone-treated children with growth hormone deficiency. J Clin Endocrinol Metab. 2007, 92: 925-931. 10.1210/jc.2006-1259.CrossRefPubMed
20.
go back to reference Cole TJ, Hindmarsh PC, Dunger DB: Growth hormone (GH) provocation tests and the response to GH treatment in GH deficiency. Arch Dis Child. 2004, 89: 1024-1027. 10.1136/adc.2003.043406.CrossRefPubMedPubMedCentral Cole TJ, Hindmarsh PC, Dunger DB: Growth hormone (GH) provocation tests and the response to GH treatment in GH deficiency. Arch Dis Child. 2004, 89: 1024-1027. 10.1136/adc.2003.043406.CrossRefPubMedPubMedCentral
21.
go back to reference Broeck Van den J, Arends N, Hokken-Koelega A: Growth response to recombinant human growth hormone (GH) in children with idiopathic growth retardation by level of maximum GH peak during GH stimulation tests. Horm Res. 2000, 53: 267-273. 10.1159/000053182.CrossRefPubMed Broeck Van den J, Arends N, Hokken-Koelega A: Growth response to recombinant human growth hormone (GH) in children with idiopathic growth retardation by level of maximum GH peak during GH stimulation tests. Horm Res. 2000, 53: 267-273. 10.1159/000053182.CrossRefPubMed
22.
go back to reference Wilson DM, Frane J: A brief review of the use and utility of growth hormone stimulation testing in the NCGS: Do we need to do provocative GH testing?. Growth Horm & IGF Res. 2005, 15: S21-S25. 10.1016/j.ghir.2005.06.005.CrossRef Wilson DM, Frane J: A brief review of the use and utility of growth hormone stimulation testing in the NCGS: Do we need to do provocative GH testing?. Growth Horm & IGF Res. 2005, 15: S21-S25. 10.1016/j.ghir.2005.06.005.CrossRef
23.
go back to reference Bright GM, Julius JR, Lima J, Blethen S: Growth hormone stimulation test results as predictors of recombinant human growth hormone treatment outcomes: preliminary analysis of the National Cooperative Growth Study Database. Pediatrics. 1999, 104: 1028-1031.PubMed Bright GM, Julius JR, Lima J, Blethen S: Growth hormone stimulation test results as predictors of recombinant human growth hormone treatment outcomes: preliminary analysis of the National Cooperative Growth Study Database. Pediatrics. 1999, 104: 1028-1031.PubMed
Metadata
Title
Diagnosis of growth hormone (GH) deficiency: comparison of pituitary stalk interruption syndrome and transient GH deficiency
Authors
Murielle Louvel
Mariana Marcu
Christine Trivin
Jean-Claude Souberbielle
Raja Brauner
Publication date
01-12-2009
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2009
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/1471-2431-9-29

Other articles of this Issue 1/2009

BMC Pediatrics 1/2009 Go to the issue