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

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          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.

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          Most cited references24

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          Standards for children's height at ages 2-9 years allowing for heights of parents.

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            Body Mass Index variations: centiles from birth to 87 years.

            This report provides Body Mass Index (weight/height2) values for the French population from birth to the age of 87 years. BMI curves increase during the first year, decrease until the age of 6, increase again up to 65 years and decrease thereafter. These variations reflect the total changes of fat body mass during life. The 50th centile values of Wt/Ht2 at the ages of 20, 40, 60, 80 years are 21.5, 24.6, 25.4, 24.4 kg/m2 for men and 20.6, 22.6, 24.1, 23.4 kg/m2 for women. The values for the 3rd, 50th and 97th centiles in the middle years are approximately 18, 24 and 32 kg/m2. Graphs for these and four other percentiles are plotted against age, and two other graphs summarising the variation and skewness of the Wt/Ht2 distribution are provided to calculate exact percentiles and Z-scores for individuals.
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              Effect of growth hormone (GH) treatment on the near-final height of 1258 patients with idiopathic GH deficiency: analysis of a large international database.

              Treatment with GH has been used to correct the growth deficit in children with GH deficiency (GHD). Although successful in increasing height velocity, such treatment often falls short of helping patients achieve full genetic height potential. This study set out to analyze near-final height (FH) data from a cohort of GH-treated children with idiopathic GHD. Of 1258 evaluable patients in the Pfizer International Growth Database (KIGS) with GHD, 980 were of Caucasian origin, and 278 were of Japanese origin; 747 had isolated GHD (IGHD), and 511 had multiple pituitary hormone deficiencies (MPHD). Near-FH, relation to midparental height, and factors predictive of growth outcomes were the main outcome measures. Median height sd scores (SDS) at the start of treatment were -2.4 (IGHD) and -2.9 (MPHD) for Caucasian males and -2.6 (IGHD) and -3.4 (MPHD) for females, respectively; comparable starting heights were -2.9 (IGHD) and -3.6 (MPHD) for Japanese males and -3.3 (IGHD) and -4.0 (MPHD) for females, respectively. Corresponding near-adult height SDS after GH treatment were -0.8 (IGHD) and -0.7 (MPHD) for Caucasian males and -1.0 (IGHD) and -1.1 (MPHD) for females, respectively; and -1.6 (IGHD) and -1.9 (MPHD) for Japanese males and -2.1 (IGHD) and -1.8 (MPHD) for females, respectively. Differences between near-adult height and midparental height ranged between -0.6 and +0.2 SDS for the various groups, with the closest approximation to MPH occurring in Japanese males with MPHD. The first-year increase in height SDS and prepubertal height gain was highly correlated with total height gain, confirming the importance of treatment before pubertal onset. It is possible to achieve FH within the midparental height range in patients with idiopathic GHD treated from an early age with GH, but absolute height outcomes remain in the lower part of the normal range. Patients with MPHD generally had a slightly better long-term height outcome.
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                Author and article information

                Journal
                BMC Pediatr
                BMC Pediatrics
                BioMed Central
                1471-2431
                2009
                6 May 2009
                : 9
                : 29
                Affiliations
                [1 ]Université Paris Descartes and AP-HP, Hôpital Bicêtre, Unité d'Endocrinologie Pédiatrique, Le Kremlin Bicêtre, 94275, France
                [2 ]AP-HP, Hôpital Necker-Enfants Malades, Service d'Explorations Fonctionnelles, Paris, 75743, France
                Article
                1471-2431-9-29
                10.1186/1471-2431-9-29
                2684107
                19419564
                8d24bd57-7712-43b6-a451-d8b925c58137
                Copyright © 2009 Louvel et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 24 January 2009
                : 6 May 2009
                Categories
                Research Article

                Pediatrics
                Pediatrics

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