Publications by authors named "J R Bierich"

During recent years numerous reports on the favourable results of short-term trials with GH in patients with constitutional delay of growth and adolescence (CDGA) have been published, but it has been unclear whether such treatment affects final height. In the present study, the results of long-term therapy with GH in replacement doses have been evaluated in 15 patients who were treated with GH for several years (three years on average). At the start of treatment, 10 of the children were prepubertal and 5 were in puberty.

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Constitutional delay of growth and adolescence.

Baillieres Clin Endocrinol Metab

July 1992

Constitutional delay of growth and adolescence (CDGA) is characterized by simultaneous retardation of growth, skeletal maturation and sexual development. Primarily longitudinal growth is impaired. The late occurrence of puberty is a secondary phenomenon brought about by the retarded physical development.

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Growth hormone deficiency (GHD) represents an aetiologically non-uniform disorder: it can have multiple causes. Three main groups can be differentiated: (1) GHD due to manifest organic alterations of the hypothalamo-hypophyseal system; (2) so-called idiopathic cases; and (3) genetically determined forms. The first group comprises the congenital malformations, affecting the midline structures of the brain more often than the pituitary itself, and the acquired lesions, mainly tumours, which are generally suprasellar rather than intrasellar.

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Four children with severe congenital eye anomalies are described of which three had related symptoms. Two had bilateral anophthalmia, the optic nerves not detectable by computed cranial tomography and magnetic resonance imaging, and the third child had bilateral microphthalmia and coloboma iridis. The fourth patient had bilateral cryptophthalmia as part of Fraser syndrome.

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