The Journal of clinical endocrinology and metabolism
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J. Clin. Endocrinol. Metab. · Nov 1999
Effect of severe growth hormone (GH) deficiency due to a mutation in the GH-releasing hormone receptor on insulin-like growth factors (IGFs), IGF-binding proteins, and ternary complex formation throughout life.
Measurement of the insulin-like growth factors (IGFs) and their binding proteins has become commonplace in the indirect assessment of the integrity of the GH axis. However, the relative effect of GH deficiency (GHD) on each component of the IGF axis and the merit of any one parameter as a diagnostic test have not been defined in a homogeneous population across all ages. We therefore measured IGF-I, IGF-II, IGF-binding protein-1 (IGFBP-1), IGFBP-2, IGFBP-3, and acid labile subunit (ALS) in 27 GHD subjects (aged 5-82 yr) from an extended kindred in Northeast Brazil with an identical GHRH receptor mutation and in 55 indigenous controls (aged 5-80 yr). ⋯ Severe GHD not only reduces the amounts of IGFs, IGFBP-3, and ALS, but also modifies the distribution of the IGFs bound to each IGFBP. Diagnostic tests used in the investigation of GHD should be tailored to the age of the individual. In particular, measurement of IGF-I in the ternary complex may prove useful in the diagnosis of GHD in children and older adults, whereas free ALS may be more relevant to younger adults.
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J. Clin. Endocrinol. Metab. · Nov 1999
Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke's cleft cyst, and arachnoid cyst.
The distinction among craniopharyngioma (CR), Rathke's cleft cyst (RCC), and intrasellar arachnoid cyst (AC) remains a difficult preoperative problem. Accurate diagnosis of these rare pituitary lesions is important to determine the type of treatment and predict prognostic outcome. The majority of the literature describes the clinical manifestations and management of only one of CR, RCC, or AC, rendering comparisons difficult. ⋯ Endocrinological deficits, especially diabetes insipidus, had the worst prognosis after surgery. Low recurrence rates can be expected for RCC and AC. These data have direct implications for the management and monitoring of patients with cystic lesions of the sella turcica.