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Short stature with normal growth hormone and elevated IGF-I

  • Endocrinology
  • Published:
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Abstract

We report on a Japanese girl with short stature, malar hypoplasia, up-slanting palpebral fissures, blue sclerae and thin, stiff and slightly brownish hair. Short stature started in utero and her psychomotor development was normal. Menarche appeared at 13 years 8 months. Height at 14 years 5 months was 132 cm (−4.6 SD). Her growth hormone (GH) sleep pattern and responses to insulin,l-dopa, arginine, propranolol-glucagon and growth hormone-releasing hormone were normal. Plasma insulin-like growth factor I (IGF-I) was high (2170–4860 units/l) and increased from 4860 to 7080 units/l 20 h after biosynthetic GH injection. Gel infiltration patterns of the free and protein-bound IGF-I in plasma from the patient were not different from the controls; IGF-I fraction of the high and low molecular weight binding protein and the non-protein bound fraction were 75.5%, 15.8% and 8.7%, respectively. IGF-I from the patient showed normal bioactivities when determined by [35S]sulphate and [3H]thymidine uptake into cultured rat chondrocytes, and by [3H]thymidine and [3H]α-aminoisobutyric acid uptake into the patient's skin fibroblasts. IGF-I binding to cultured skin fibroblasts from the patient was comparable to that of controls. These results suggest that tissue specific defects of IGF-I receptors may be the cause of increased IGF-I levels in the patient.

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Abbreviations

AIB:

α-aminoisobutyric acid

DMEM:

Dulbecco's minimum essential medium

GH:

growth hormone

IGF-I:

insulin-like growth factor I

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Authors and Affiliations

  1. Department of Paediatrics, Wakayama Red Cross Hospital, 4-20 Komatsubara-dori, 640, Wakayama City, Wakayama, Japan

    T. Momoi

  2. Department of Paediatrics, Kyoto University Faculty of Medicine, Sakyo-ku, 606, Kyoto City, Kyoto, Japan

    T. Momoi, C. Yamanaka, H. Sasaki, T. Yorifuji, M. Kaji & H. Mikawa

  3. The Third Department of Medicine, Shiga University of Medical Science, 520-21, Ohtsu City, Shiga, Japan

    M. Kobayashi & T. Haruta

Authors
  1. T. Momoi
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  2. C. Yamanaka
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  3. M. Kobayashi
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  4. T. Haruta
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  5. H. Sasaki
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  6. T. Yorifuji
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  7. M. Kaji
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  8. H. Mikawa
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Momoi, T., Yamanaka, C., Kobayashi, M. et al. Short stature with normal growth hormone and elevated IGF-I. Eur J Pediatr 151, 321–325 (1992). https://doi.org/10.1007/BF02113248

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  • DOI: https://doi.org/10.1007/BF02113248

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