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Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.
  1. A T Soliman,
  2. A Rajab,
  3. I AlSalmi,
  4. A Darwish,
  5. M Asfour
  1. Department of Paediatrics, Royal Hospital, Muscat, Oman.


    Short stature is a characteristic feature of pycnodysostosis. We report defective growth hormone secretion in response to provocation and low insulin-like growth factor-I (IGF-I) concentration in five out of six patients with pycnodysostosis. Physiological replacement with growth hormone increased IGF-I concentration and improved linear growth in these children.

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