THE INVESTIGATION OF GROWTH-HORMONE INSENSITIVITY

Growth hormone insensitivity is a pathological stale characterized by a disturbance of the physiological relationships between GH secretion, IGF-I synthesis and the biological actions of GH. GH insensitivity ma ybe either a primary, genetically determined disorder, e.g. GH recepto r deficiency, or an acquired disorder arising from a primary disease p rocess, e.g. malnutrition, protein catabolic states. In both forms of GH insensitivity there are low circulating levels of IGF-I in the pres ence of increased GH secretion. This article will deal with primary GH insensitivity states, which are now increasingly recognized to be gen etically and clinically heterogeneous. In common with other paediatric hormone resistant syndromes, such as androgen insensitivity and pseud ohypoaldosteronism, the cardinal features are the association of a sta te of clinical hormone deficiency and the elevation of stimulatory hor mone levels. GH insensitivity can now be treated effectively with reco mbinant IGF-I (Ranke et al., 1996).