CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST
Primary growth hormone insensitivity syndrome (GHIS) is a rare entity which can be due to defects in growth hormone (GH) receptor that is called type 1 Laron syndrome (T1LS) or post receptor defects (type 2 Laron syndrome ). The aim of study was determining the clinical and hormonal milieu of the pa...
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2006-02-01
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| Series: | Acta Medica Iranica |
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| Online Access: | https://acta.tums.ac.ir/index.php/acta/article/view/3139 |
| _version_ | 1818336066257551360 |
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| author | M. Razzaghy-Azar M. Nourbakhsh F. Hajighasemi |
| author_facet | M. Razzaghy-Azar M. Nourbakhsh F. Hajighasemi |
| author_sort | M. Razzaghy-Azar |
| collection | DOAJ |
| description | Primary growth hormone insensitivity syndrome (GHIS) is a rare entity which can be due to
defects in growth hormone (GH) receptor that is called type 1 Laron syndrome (T1LS) or post receptor defects (type 2 Laron syndrome ). The aim of study was determining the clinical and hormonal milieu of the patients with primary GHIS and their response to IGF-I (insulin like growth factor-I) generation test (IGT). GH, IGF-I, IGF-II, IGF binding protein 1 and 3 (BP-1 and BP-3), GH binding protein (GHBP) and anti-GH antibody were detected by ELISA and RIA methods. IGF-I and BP-3 were measured before and after IGT. Nine patients (8 males, 1 female) (mean age ± SD, 6.4 ± 5 years) with severe short stature and high GH level were studied. Height SDS was - 8.5 ± 2.6. In 7 patients GHBP was zero, IGF-I and BP-3 were low and did not increase after IGT, so they had T1LS. Two brothers did not show the hormonal milieu of GH receptor defect, and were called non Laron syndrome (NLS). Birth weight in patients with T1LS and NLS was 3.65 ± 0.2 Kg and 1.65 ± 0.2 Kg, respectively (P = 0.001).
All of the patients had typical clinical feature of GH-deficiency, but nasal bridge depression and microphallus were not seen in NLS. GH treatment of NLS, normalized their growth velocity, but without catch up growth. In conclusion IGT can differentiate Laron syndrome from other types of short stature. GH and IGF-I of fetus have no role in intrauterine growth. |
| first_indexed | 2024-12-13T14:33:24Z |
| format | Article |
| id | doaj.art-e12f14efe5574d3ab6913cf07eabd658 |
| institution | Directory Open Access Journal |
| issn | 0044-6025 1735-9694 |
| language | English |
| last_indexed | 2024-12-13T14:33:24Z |
| publishDate | 2006-02-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Acta Medica Iranica |
| spelling | doaj.art-e12f14efe5574d3ab6913cf07eabd6582022-12-21T23:41:47ZengTehran University of Medical SciencesActa Medica Iranica0044-60251735-96942006-02-01441CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST M. Razzaghy-Azar0 M. Nourbakhsh F. Hajighasemi1 Primary growth hormone insensitivity syndrome (GHIS) is a rare entity which can be due to defects in growth hormone (GH) receptor that is called type 1 Laron syndrome (T1LS) or post receptor defects (type 2 Laron syndrome ). The aim of study was determining the clinical and hormonal milieu of the patients with primary GHIS and their response to IGF-I (insulin like growth factor-I) generation test (IGT). GH, IGF-I, IGF-II, IGF binding protein 1 and 3 (BP-1 and BP-3), GH binding protein (GHBP) and anti-GH antibody were detected by ELISA and RIA methods. IGF-I and BP-3 were measured before and after IGT. Nine patients (8 males, 1 female) (mean age ± SD, 6.4 ± 5 years) with severe short stature and high GH level were studied. Height SDS was - 8.5 ± 2.6. In 7 patients GHBP was zero, IGF-I and BP-3 were low and did not increase after IGT, so they had T1LS. Two brothers did not show the hormonal milieu of GH receptor defect, and were called non Laron syndrome (NLS). Birth weight in patients with T1LS and NLS was 3.65 ± 0.2 Kg and 1.65 ± 0.2 Kg, respectively (P = 0.001). All of the patients had typical clinical feature of GH-deficiency, but nasal bridge depression and microphallus were not seen in NLS. GH treatment of NLS, normalized their growth velocity, but without catch up growth. In conclusion IGT can differentiate Laron syndrome from other types of short stature. GH and IGF-I of fetus have no role in intrauterine growth.https://acta.tums.ac.ir/index.php/acta/article/view/3139"Growth hormone insensitivity syndromeType 1 Laron syndromeIGF-I generation testIGF binding protein-3IGF binding protein-1IGF-II" |
| spellingShingle | M. Razzaghy-Azar M. Nourbakhsh F. Hajighasemi CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST Acta Medica Iranica "Growth hormone insensitivity syndrome Type 1 Laron syndrome IGF-I generation test IGF binding protein-3 IGF binding protein-1 IGF-II" |
| title | CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST |
| title_full | CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST |
| title_fullStr | CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST |
| title_full_unstemmed | CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST |
| title_short | CLINICAL AND HORMONAL MILIEU OF 9 PATIENTS WITH PRIMARY GROWTH HORMONE INSENSITIVITY SYNDROME AND THEIR RESPONSE TO IGF-I GENERATION TEST |
| title_sort | clinical and hormonal milieu of 9 patients with primary growth hormone insensitivity syndrome and their response to igf i generation test |
| topic | "Growth hormone insensitivity syndrome Type 1 Laron syndrome IGF-I generation test IGF binding protein-3 IGF binding protein-1 IGF-II" |
| url | https://acta.tums.ac.ir/index.php/acta/article/view/3139 |
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