Isolated growth hormone (GH) deficiency is the cause of many cases of pituitary dwarfism. Although an autosomal dominant form of the complete GH deficiency is associated with a deletion of the GH structural gene, such genetic defects are believed to be responsible for only a small number of cases. The treatment of GH deficiency in adults <50 years has already been discussed above (see general hypopituitarism).
A lack of individual pituitary hormones may precede the development of a generalized hypopituitarism. Patients should be observed for signs of a lack of other pituitary hormones, and imaging of the sella in terms of a tumor should be done at regular intervals. Isolated growth hormone (GH) deficiency is the cause of many cases of pituitary dwarfism. Although an autosomal dominant form of the complete GH deficiency is associated with a deletion of the GH structural gene, such genetic defects are believed to be responsible for only a small number of cases. The treatment of GH deficiency in adults <50 years has already been discussed above (see general hypopituitarism). An isolated gonadotropin deficiency occurs in both sexes and must be distinguished from a primary hypogonadism. Men have low serum testosterone mirror and infertility, women show amenorrhea, low serum estrogen levels and infertility. A eunuchoides appearance is always to be found. However, patients with primary hypogonadism have elevated levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH), while patients with secondary (pituitary) or tertiary (hypothalamic) gonadotropin deficiency low-normal, low or non-measurable LH and FSH have mirror. Although, patients with hypogonadotropic hypogonadism in most cases a scarcity of both FSH and LH, there are rare cases in which only the secretion of a hormone is affected. Isolated gonadotropin deficiency must be distinguished from a hypogonadotropic amenorrhea due to strenuous exercise, diet or psychological stress. Although an accurate medical history can be helpful, the commitment to one of the possible diagnoses is sometimes impossible. Kallmann syndrome, for the lack of gonadotropin releasing hormone (GnRH) is specifically associated with centerline defects of the face, lip or cleft palate, a anosmia and with color blindness. Embryological studies have shown that GnRH neurons initially develop in the epithelium of the olfactory groove and then migrate early in the course of development in the septal-preoptic area of ??the hypothalamus. In a few cases, genetic defects were localized in the X-linked form on the X chromosome and KALIG-1 (Kallmann syndrome Intervallgen 1) can be mentioned, with adhesion proteins which facilitate neuronal migration detected. The administration of GnRH is not indicated. Isolated ACTH deficiency is rare. Weakness, hypoglycemia, weight loss and decreased pubic point to the diagnosis. Cortisol levels in the blood and urine are low and normalize after the substitution with ACTH. Clinical and laboratory evidence of a lack of other hormones are missing. The treatment is effected by the replacement of cortisol as in Addison's disease; replacement of mineralocorticoid is not necessary. An isolated lack of thyroid-stimulating hormone is likely when clinical signs of hypothyroidism exist, the (TSH) hormone levels are low or not increased serum Thyorid stimulating and no shortage of other pituitary hormones is composed. The serum TSH levels, which are determined using an immunoassay, are not always reduced, which leads to the presumption that the secreted TSH is biologically inactive. The administration of recombinant human TSH increases the levels of thyroid hormones (hypothyroidism. Insulated Prolactinmangel is very rarely seen in women in whom it does not come to a had taken place birth to the onset of lactation. The basal prolactin levels are low and increase after provocation such . as the administration of thyrotropin-releasing hormone not. the substitution of prolactin is not indicated.