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Selective deficiencies of pituitary hormones may represent an early stage in the development of more generalized hypopituitarism. Patients must be observed for signs of other pituitary hormone deficiencies, and sellar imaging should be performed at intervals for signs of a pituitary tumor.
Isolated
growth hormone (GH) deficiency is responsible for many cases of pituitary dwarfism (see Pituitary Disorders: Hypopituitarism in Children Resulting in Short Stature). Although one autosomal dominant form of complete GH deficiency is associated with a deletion of the GH structural gene, such gene defects probably account for a minority of cases. Treatment of GH deficiency in adults < 50 yr is discussed elsewhere (see Pituitary Disorders: Generalized Hypopituitarism).
Isolated
gonadotropin deficiency occurs in both sexes and must be distinguished from primary hypogonadism; men have low serum testosterone levels and infertility, and women have amenorrhea, low serum estrogen levels, and infertility. A eunuchoid habitus is generally present. However, patients with primary hypogonadism (see Male Reproductive Endocrinology: Etiology) have elevated levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH), whereas those with gonadotropin deficiency, either secondary (pituitary) or tertiary (hypothalamic), have low-normal, low, or unmeasurable levels of LH and FSH. Although most cases of hypogonadotropic hypogonadism involve deficiencies of both LH and FSH, in rare cases the secretion of only one is impaired. Isolated gonadotropin deficiency must also be distinguished from hypogonadotropic amenorrhea secondary to exercise, diet, or mental stress (see Menstrual Abnormalities: Amenorrhea). Although the history may be helpful, differential diagnosis may be impossible.
In Kallmann's
syndrome, the specific lack of gonadotropin-releasing hormone (GnRH) is associated with midline facial defects, including anosmia and cleft lip or palate (see Endocrine Disorders in Children: Secondary), and with color blindness. Embryologic studies have shown that GnRH neurons originally develop in the epithelium of the olfactory placode and migrate into the septal-preoptic region of the hypothalamus early in development. In at least some cases, gene defects, localized to the X chromosome in the X-linked form of the disorder and termed the KALIG-1 (Kallmann's syndrome interval gene 1) gene, have been found in the adhesion proteins facilitating this neuronal migration. Administration of GnRH is not indicated.
Isolated
ACTH deficiency is rare. Weakness, hypoglycemia, weight loss, and decreased axillary and pubic hair suggest the diagnosis. Plasma and urinary steroid levels are low and rise to normal after ACTH replacement. Clinical and laboratory evidence of other hormonal deficiencies is absent. Treatment is with cortisol replacement, as for Addison's disease (see Adrenal Disorders: Treatment).
Isolated thyroid-stimulating
hormone (TSH) deficiency is likely when clinical features of hypothyroidism exist, plasma TSH levels are not elevated, and no other pituitary hormone deficiencies exist. Plasma TSH levels, as measured by immunoassay, are not always lower than normal, suggesting that the TSH secreted is biologically inactive. Administration of recombinant human-TSH increases thyroid hormone levels (see Thyroid Disorders: Hypothyroidism).
Isolated
prolactin deficiency has been noted rarely in women who fail to lactate after delivery. Basal prolactin levels are low and do not increase in response to provocative stimuli, such as thyroid-releasing hormone. Administration of prolactin is not indicated.
Last full review/revision February 2007 by Ian M. Chapman, MBBS, PhD
Content last modified February 2007
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