Abstract

BackgroundMost 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).MethodsWe 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.ResultsAt 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).ConclusionThe factors of the growth response to GH treatment should be analysed separately for each population: with and without PSIS or other markers.

Highlights

  • Most patients with childhood non-organic growth hormone (GH) deficiency (GHD) produce a normal GH peak as young adults

  • Fifteen other patients seen during the same period were not included because the pituitary stalk interruption syndrome (PSIS) was associated with malformative syndrome, which can modify the parameters studied (Fanconi anemia n = 6, Blackfan-Diamond anemia n = 1), or because their GH treatment was initiated elsewhere (n = 8)

  • This study shows that the patients with transient growth hormone deficiency (GHD) have features that differ from those of patients with PSIS

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Summary

Introduction

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). GHD is diagnosed by the height of the GH peak after pharmacological stimulation. The value of these tests has been questioned [2] because they are expensive, labor intensive, occasionally risky, and their results are not very reproducible. The majority of patients who have no markers of certain GHD produce a normal GH peak response to these tests when evaluated as young adults [3]. The transient GHD may be partly responsible for the variability of the growth response to GH treatment

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